History
Age
Chief Complaint
History of Present Illness
Past Medical History
Family History
Social History
Review of Systems
Summary
General Physical Examination
Vital Signs
Skin
Head, Eyes, Ears, & Neck
Chest & Cardiovascular
Abdomen
Extremities & Back
Rectal & Pelvic
Neurologic Examination
Mental Status Examination
Cranial Nerves
Motor Function
Sensory Function
Coordination
Reflexes
Stance & Gait
Neurologic Examination in Special Settings
Coma
“Screening” Neurologic Examination
Diagnostic Formulation
Principles of Diagnosis
Anatomic Diagnosis: Where Is the Lesion?
Etiologic Diagnosis: What Is the Lesion?
Laboratory Investigations
References
A thorough but directed history and neurologic examination are the keys to neurologic diagnosis and treatment. Laboratory studies, discussed in Chapter 2, can provide valuable additional information, but cannot replace the history and exam.
HISTORY
Taking a history from a patient with a neurologic complaint is fundamentally the same as taking any history.
Age
The patient’s age can be a major clue to the likely causes of a neurologic problem. For example, epilepsy, multiple sclerosis, and Huntington disease usually have their onset by middle age, whereas Alzheimer disease, Parkinson disease, brain tumors, and stroke predominantly affect older individuals.
Chief Complaint
The patient’s problem (chief complaint) should be defined as clearly as possible, because it will guide subsequent evaluation toward—or away from—the correct diagnosis. In eliciting the chief complaint, the goal is to describe the nature of the problem in a word or phrase.
Common neurologic complaints include confusion, dizziness, weakness, shaking, numbness, blurred vision, and spells. Each of these terms means different things to different people, so it is critical to point evaluation of the problem in the right direction by getting as much clarification as possible regarding what the patient is trying to convey.
A. Confusion
Confusion reported by the patient or family members may include memory impairment, getting lost, difficulty understanding or producing spoken or written language, problems with numbers, faulty judgment, personality change, or combinations thereof. Symptoms of confusion may be difficult to characterize, and asking for specific examples can be helpful in this regard.
B. Dizziness
Dizziness can mean vertigo (the illusion of movement of oneself or the environment), imbalance (unsteadiness due to extrapyramidal, vestibular, cerebellar, or sensory deficits), or presyncope (light-headedness resulting from cerebral hypoperfusion).
C. Weakness
Weakness is the term neurologists use to mean loss of power from disorders affecting motor pathways in the central or peripheral nervous system or skeletal muscle. However, patients sometimes use this term when they mean generalized fatigue, lethargy, or even sensory disturbances.
D. Shaking
Shaking may represent abnormal movements such as tremor, chorea, athetosis, myoclonus, or fasciculation (see Chapter 11, Movement Disorders), but the patient is unlikely to classify his or her problem according to this terminology. Correct classification depends on observing the movements in question or, if they are intermittent and not present when the history is taken, asking the patient to demonstrate them.
E. Numbness
Numbness can refer to any of a variety of sensory disturbances, including hypesthesia (decreased sensitivity), hyperesthesia (increased sensitivity), or paresthesia (“pins and needles” sensation). Patients occasionally also use this term to signify weakness.
F. Blurred vision
Blurred vision may represent diplopia (double vision), ocular oscillations, reduced visual acuity, or visual field cuts.
G. Spells
Spells imply episodic and often recurrent symptoms such as may be seen with epilepsy or syncope (fainting).
History of Present Illness
The history of present illness should provide a detailed description of the chief complaint, including the following features.
A. Quality of Symptoms
Some symptoms, such as pain, may have distinctive features that are diagnostically helpful. Neuropathic pain—which results from direct injury to nerves—may be described as especially unpleasant (dysesthetic) and may be accompanied by increased sensitivity to pain (hyperalgesia) or touch (hyperesthesia), or by the perception of a normally innocuous stimulus as painful (allodynia), in the affected area. The quality of symptoms includes their severity—although individual thresholds for seeking medical attention for a symptom vary, it is often useful to ask a patient to rank the present complaint in relation to problems he or she has had in the past.
B. Location of Symptoms
The location of symptoms is critical to neurologic diagnosis, and patients should be encouraged to localize their symptoms as precisely as possible. The spatial distribution of weakness, decreased sensation, or pain helps to assign the underlying disease process to a specific site in the nervous system. This provides an anatomic diagnosis, which is then refined to identify the cause.
C. Time Course
It is important to determine when the problem began, whether it came on abruptly or insidiously, and if its subsequent course has been characterized by improvement, worsening, or exacerbation and remission (Figure 1-1). For episodic disorders, such as headache or seizures, the time course of individual episodes should also be determined.
Figure 1-1. Temporal patterns of neurologic disease and examples of each.
D. Precipitating, Exacerbating, and Alleviating Factors
Some symptoms may appear to be spontaneous, but in other cases, specific precipitating factors can be identified. Through observation and experimentation, patients often become aware of factors that worsen symptoms, and which they can avoid, or factors that prevent symptoms or provide relief.
E. Associated Symptoms
Associated symptoms can assist with anatomic or etiologic diagnosis. For example, neck pain accompanying leg weakness suggests a cervical myelopathy (spinal cord disorder), and fever in the setting of headache raises concern about meningitis.
Past Medical History
Certain aspects of the past medical history may be especially relevant to a neurologic complaint.
A. Illnesses
Many preexisting illnesses can predispose to neurologic disease, including hypertension, diabetes, heart disease, cancer, and human immunodeficiency virus (HIV) disease.
B. Operations
Open heart surgery may be complicated by stroke or a confusional state. Entrapment neuropathies (disorders of a peripheral nerve due to local pressure) affecting the upper or lower extremity may complicate the perioperative course.
C. Obstetrical History
Pregnancy can worsen epilepsy, at least partly due to altered metabolism of anticonvulsant drugs. The frequency of migraine attacks may increase or decrease. Pregnancy is a predisposing condition for benign intracranial hypertension (pseudotumor cerebri) and entrapment neuropathies, especially carpal tunnel syndrome (median neuropathy) and meralgia paresthetica (lateral femoral cutaneous neuropathy). Traumatic neuropathies affecting the obturator, femoral, or peroneal nerve may result from pressure exerted by the fetal head or obstetrical forceps during delivery. Eclampsia is a life-threatening syndrome in which generalized tonic-clonic seizures complicate the course of pre-eclampsia (hypertension with proteinuria) during pregnancy.
D. Medications
A wide range of medications can cause adverse neurologic effects, including confusional states or coma, headache, ataxia, neuromuscular disorders, neuropathy, and seizures.
E. Immunizations
Vaccination can prevent several neurologic diseases, including poliomyelitis, diphtheria, tetanus, rabies, and meningococcal meningitis. Vaccinations may be associated with postvaccination autoimmune encephalitis, myelitis, or neuritis (inflammation of the brain, spinal cord, or peripheral nerves).
F. Diet
Dietary deficiency and excess can both lead to neurologic disease. Deficiency of vitamin B1 (thiamin) is responsible for the Wernicke-Korsakoff syndrome and polyneuropathy in alcoholics. Vitamin B3(niacin) deficiency causes pellagra, which is characterized by dementia. Vitamin B12 (cobalamin) deficiency usually results from malabsorption associated with pernicious anemia and produces combined systems disease (degeneration of corticospinal tracts and posterior columns in the spinal cord) and dementia (megaloblastic madness). Inadequate intake of vitamin E (tocopherol) can also lead to spinal cord degeneration. Conversely, hypervitaminosis A can produce intracranial hypertension (pseudotumor cerebri) with headache, visual deficits, and seizures, whereas excessive intake of vitamin B6 (pyridoxine) is a cause of polyneuropathy. Excessive consumption of fats is a risk factor for stroke. Finally, ingestion of improperly preserved foods containing botulinum toxin causes botulism, a disorder of acetylcholine release at autonomic and neuromuscular synapses, which presents with descending paralysis.
G. Tobacco, Alcohol, and Other Drug Use
Tobacco use is associated with lung cancer, which may metastasize to the central nervous system or produce paraneoplastic neurologic syndromes. Alcohol abuse can produce withdrawal seizures, polyneuropathy, and nutritional disorders of the nervous system. Use of intravenous drugs may suggest HIV disease or drug-related neurologic complications of infection or vasculitis.
Family History
This should indicate any past or current diseases in the spouse and first- (parents, siblings, children) and second-(grandparents, grandchildren) degree relatives. Several neurologic diseases are inherited in Mendelian or more complex patterns, such as Huntington disease (autosomal dominant), Wilson disease (autosomal recessive), and Duchenne muscular dystrophy (X-linked recessive) (Figure 1-2).
Figure 1-2. Simple Mendelian patterns of inheritance. Squares represent males, circles females, and filled symbols affected individuals.
Social History
Information about the patient’s education and occupation help in the interpretation of whether his or her cognitive performance is background-appropriate. The sexual history may indicate risk for sexually transmitted diseases that affect the nervous system, such as syphilis or HIV disease. The travel history can document possible exposure to infections endemic to particular geographic areas.
Review of Systems
Non-neurologic complaints elicited in the review of systems may point to a systemic cause of a neurologic problem.
1. General—Weight loss or fever may indicate a neoplastic or infectious cause of neurologic symptoms.
2. Immune—Acquired immune deficiency syndrome (AIDS) may lead to dementia, myelopathy, neuropathy, myopathy, or infections (eg, toxoplasmosis) or tumors (eg, lymphoma) affecting the nervous system.
3. Hematologic—Polycythemia and thrombocytosis may predispose to ischemic stroke, whereas thrombocytopenia and coagulopathy are associated with intracranial hemorrhage.
4. Endocrine—Diabetes increases the risk for stroke and may be complicated by polyneuropathy. Hypothyroidism may lead to coma, dementia, or ataxia.
5. Skin—Characteristic skin lesions are seen in certain disorders that also affect the nervous system, such as neurofibromatosis and postherpetic neuralgia.
6. Eyes, ears, nose, and throat—Neck stiffness is a common feature of meningitis and subarachnoid hemorrhage.
7. Cardiovascular—Ischemic or valvular heart disease and hypertension are major risk factors for stroke.
8. Respiratory—Cough, hemoptysis, or night sweats may be manifestations of tuberculosis or lung neoplasm, which can disseminate to affect the nervous system.
9. Gastrointestinal—Hematemesis, jaundice, and diarrhea may direct the investigation of a confusional state toward hepatic encephalopathy.
10. Genitourinary—Urinary retention or incontinence, or impotence, may be manifestations of peripheral neuropathy or myelopathy.
11. Musculoskeletal—Muscle pain and tenderness may accompany the myopathy of polymyositis.
12. Psychiatric—Psychosis, depression, and mania may be manifestations of a neurologic disease.
Summary
Upon completion of the history, the examiner should have a clear understanding of the chief complaint, including its location and time course, and familiarity with elements of the past medical history, family and social history, and review of systems that may be related to the complaint. This information should help to guide the general physical and neurologic examinations, which should focus on areas suggested by the history. For example, in an elderly patient who presents with the sudden onset of hemiparesis and hemisensory loss, which is likely to be due to stroke, the general physical examination should stress the cardiovascular system, because a variety of cardiovascular disorders predispose to stroke. On the other hand, if a patient complains of pain and numbness in the hand, much of the exam should be devoted to examining sensation, strength, and reflexes in the affected upper extremity.
GENERAL PHYSICAL EXAMINATION
In a patient with a neurologic complaint, the general physical examination should focus on looking for abnormalities often associated with neurologic problems.
Vital Signs
A. Blood Pressure
Elevated blood pressure may indicate chronic hypertension, which is a risk factor for stroke and is also seen acutely in the setting of hypertensive encephalopathy, ischemic stroke, or intracerebral or subarachnoid hemorrhage. Blood pressure that drops by ≥20 mm Hg (systolic) or ≥10 mm Hg (diastolic) when a patient switches from recumbent to upright signifies orthostatic hypotension (Figure 1-3). If the drop in blood pressure is accompanied by a compensatory increase in pulse rate, sympathetic autonomic reflexes are intact, and the likely cause is hypovolemia. However, the absence of a compensatory response is consistent with central (eg, Parkinson disease) or peripheral (eg, polyneuropathy) disorders of sympathetic function or an adverse effect of sympatholytic (eg, antihypertensive) drugs.
Figure 1-3. Test for orthostatic hypotension. Systolic and diastolic blood pressure and heart rate are measured with the patent recumbent (left) and then each minute after standing for 5 min (right). A decrease in systolic blood pressure of ≥20 mm or in diastolic blood pressure of ≥10 mm indicates orthostatic hypotension. When autonomic function is normal, as in hypovolemia, there is a compensatory increase in heart rate, whereas lack of such an increase suggests autonomic failure.
B. Pulse
A rapid or irregular pulse—especially the irregularly irregular pulse of atrial fibrillation—may point to a cardiac arrhythmia as the cause of stroke or syncope.
C. Respiratory Rate
The respiratory rate may provide a clue to the cause of a metabolic disturbance associated with coma or a confusional state. Rapid respiration (tachypnea) can be seen in hepatic encephalopathy, pulmonary disorders, sepsis, or salicylate intoxication; depressed respiration is observed with pulmonary disorders and sedative drug intoxication. Tachypnea may also be a manifestation of neuromuscular disease affecting the diaphragm. Abnormal respiratory patterns are also observed in coma: Cheyne-Stokes breathing (alternating deep breaths, or hyperpnea, and apnea) can occur in metabolic disorders or with hemispheric lesions, whereas apneustic, cluster, or ataxic breathing (see Chapter 3, Coma) implies a brainstem disorder.
D. Temperature
Fever (hyperthermia) occurs with infection of the meninges (meningitis), brain (encephalitis), or spinal cord (myelitis). Hypothermia can be seen in ethanol or sedative drug intoxication, hypoglycemia, hepatic encephalopathy, Wernicke encephalopathy, and hypothyroidism.
Skin
Jaundice (icterus) suggests liver disease as the cause of a confusional state or movement disorder. Coarse dry skin, dry brittle hair, and subcutaneous edema are characteristic of hypothyroidism. Petechiae are seen in meningococcal meningitis, and petechiae or ecchymoses may suggest a coagulopathy as the cause of subdural, intracerebral, or paraspinal hemorrhage. Bacterial endocarditis, a cause of stroke, can produce a variety of cutaneous lesions, including splinter (subungual) hemorrhages, Osler nodes (painful swellings on the distal fingers), and Janeway lesions (painless hemorrhages on the palms and soles). Hot dry skin accompanies anticholinergic drug intoxication.
Head, Eyes, Ears, & Neck
A. Head
Examination of the head may reveal signs of trauma, such as scalp lacerations or contusions. Basal skull fracture may produce postauricular hematoma (Battle sign), periorbital hematoma (raccoon eyes), hemotympanum, or cerebrospinal fluid (CSF) otorrhea or rhinorrhea (Figure 1-4). Percussion of the skull over a subdural hematoma may cause pain. A vascular bruit heard on auscultation of the skull is associated with arteriovenous malformations.
Figure 1-4. Signs of head trauma include periorbital (raccoon eyes, A) or postauricular (Battle sign, B) hematoma, each of which suggests basal skull fracture. (From Knoop KJ, Stack LB, Storrow AB, et al. The Atlas of Emergency Medicine. 3rd ed. New York, NY: McGraw-Hill; 2010.)
B. Eyes
Icteric sclerae are seen in liver disease. Pigmented (Kayser-Fleischer) corneal rings—best seen by slit-lamp examination—are produced by deposition of copper in Wilson disease. Retinal hemorrhages (Roth spots) may occur in bacterial endocarditis, which is also associated with septic emboli that may produce stroke. Exophthalmos is observed with hyperthyroidism, orbital or retro-orbital masses, and cavernous sinus thrombosis.
C. Ears
Otoscopic examination shows bulging, opacity, and erythema of the tympanic membrane in otitis media, which may spread to produce bacterial meningitis.
D. Neck
Meningeal signs (Figure 1-5), such as neck stiffness on passive flexion or thigh flexion upon flexion of the neck (Brudzinski sign), are seen in meningitis and subarachnoid hemorrhage. Restricted lateral movement (lateral flexion or rotation) of the neck may accompany cervical spondylosis. Auscultation of the neck may reveal a carotid bruit consistent with predisposition to stroke.
Figure 1-5. Signs of meningeal irritation. Kernig sign (A) is resistance to passive extension at the knee with the hip flexed. Brudzinski sign (B) is flexion at the hip and knee in response to passive flexion of the neck. (From LeBlond RF, DeGowin RL, Brown DD. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
Chest & Cardiovascular
Signs of respiratory muscle weakness—such as intercostal muscle retraction and the use of accessory muscles—may occur in neuromuscular disorders. Heart murmurs may be associated with valvular heart disease predisposing to stroke and with infective endocarditis and its neurologic sequelae.
Abdomen
Abdominal examination may reveal a source of systemic infection or suggest liver disease and is always important in patients with the new onset of back pain, because a variety of pathologic intra-abdominal processes (eg, pancreatic carcinoma or aortic aneurysm) may produce pain that radiates to the back.
Extremities & Back
Resistance to passive extension of the knee with the hip flexed (Kernig sign) is seen in meningitis. Raising the extended leg with the patient supine (straight leg raising, or Lasègue sign) stretches the L4-S2 roots and sciatic nerve, whereas raising the extended leg with the patient prone (reverse straight leg raising) stretches the L2-L4 roots and femoral nerve and may reproduce radicular pain in patients with lesions affecting these structures (Figure 1-6). Localized pain with percussion of the spine may be a sign of vertebral or epidural infection. Auscultation of the spine may reveal a bruit due to spinal vascular malformation.
Figure 1-6. Signs of lumbosacral nerve root irritation. The straight leg raising or Lasègue sign (top) is pain in an L4-S2 root or sciatic nerve distribution in response to raising the extended leg with the patient supine. The reverse straight leg raising sign (bottom) is pain in an L2-L4 root or femoral nerve distribution in response to raising the extended leg with the patient prone. (From LeBlond RF, DeGowin RL, Brown DD. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill, 2009.)
Rectal & Pelvic
Rectal examination can provide evidence of gastrointestinal bleeding, which is a common precipitant of hepatic encephalopathy. Rectal or pelvic examination may disclose a mass lesion responsible for pain referred to the back.
NEUROLOGIC EXAMINATION
The neurologic examination should be tailored to each patient’s specific complaint. Each area of the exam—mental status, cranial nerves, motor function, sensory function, coordination, reflexes, and stance and gait—should always be covered, but the relative emphasis among and within areas will differ. The patient’s history should have raised questions that the examination can now address. For example, if the patient’s complaint is weakness, the examiner seeks to determine its distribution and severity and whether it is accompanied by deficits in other areas, such as sensation and reflexes. The goal is to obtain the information necessary to generate an anatomic diagnosis on completion of the examination.
Mental Status Examination
The mental status examination addresses two key questions: (1) Is level of consciousness (wakefulness or alertness) normal or abnormal? (2) If the level of consciousness permits more detailed examination, is cognitive functionnormal, and if not, what is the nature and extent of the abnormality?
A. Level of Consciousness
Consciousness is awareness of the internal or external world, and the level of consciousness is described in terms of the patient’s apparent state of wakefulness and response to stimuli. A patient with a normal level of consciousness is awake (or can be awakened), alert (responds appropriately to visual or verbal cues), and oriented (knows who and where he or she is and the approximate date or time).
Abnormal (depressed) consciousness represents a continuum ranging from mild sleepiness to unarousable unresponsiveness (coma, see Chapter 3). Depressed consciousness short of coma is sometimes referred to as a confusional state, delirium, or stupor, but should be characterized more precisely in terms of the stimulus–response patterns observed. Progressively more severe impairment of consciousness requires stimuli of increasing intensity to elicit increasingly primitive (nonpurposeful or reflexive) responses (Figure 1-7).
Figure 1-7. Assessment of level of consciousness in relation to the patient’s response to stimulation. A normally conscious patient responds coherently to visual or verbal stimulation, whereas a patient with impaired consciousness requires increasingly intense stimulation and exhibits increasingly primitive responses.
B. Cognitive Function
Cognitive function involves many spheres of activity, some of which are localized and others dispersed throughout the cerebral hemispheres. The strategy in examining cognitive function is to assess a range of specific functions and, if abnormalities are found, to evaluate whether these can be attributed to a specific brain region or require more widespread involvement of the brain. For example, discrete disorders of language (aphasia) and memory (amnesia) can often be assigned to a circumscribed area of the brain, whereas more global deterioration of cognitive function, as seen in dementia, implies diffuse or multifocal disease.
1. Bifrontal or diffuse functions—Attention is the ability to focus on a particular sensory stimulus to the exclusion of others; concentration is sustained attention. Attention can be tested by asking the patient to immediately repeat a series of digits (a normal person can repeat five to seven digits correctly), and concentration can be tested by having the patient count backward from 100 by 7. Abstract thought processes like insight and judgment can be assessed by asking the patient to list similarities and differences between objects (eg, an apple and an orange), interpret proverbs (overly concrete interpretations suggest impaired abstraction ability), or describe what he or she would do in a hypothetical situation requiring judgment (eg, finding an addressed envelope on the street). Fund of knowledge can be tested by asking for information that a normal person of the patient’s age and cultural background would be expected to possess (eg, the name of the President, sports stars, or other celebrities, or of major events in the news). This is not intended as a test of intelligence, but to determine whether the patient has been incorporating new information normally in the recent past. Affect is the external behavioral correlate of the patient’s (internal) mood and may be manifested by talkativeness or lack thereof, facial expression, and posture. Conversation with the patient may also reveal abnormalities of thought content, such as delusions or hallucinations, which are usually associated with psychiatric disease, but can also exist in confusional states (eg, alcohol withdrawal) or complex partial seizures.
2. Memory—Memory is the ability to register, store, and retrieve information and can be impaired by either diffuse cortical or bilateral temporal lobe disease. Memory is assessed clinically by testing immediate recall, recent memory, and remote memory, which correspond roughly to registration, storage, and retrieval, respectively. Tests of immediate recall are similar to tests of attention (see earlier discussion) and include having the patient immediately repeat a list of numbers or objects. To test recent memory, the patient can be asked to repeat the same list 3 to 5 minutes later. Remote memory is tested by asking the patient about important items he or she can be expected to have learned in past years, such as personal or family data or major historic events. Confusional states typically impair immediate recall, whereas memory disorders (amnesia) are characteristically associated with predominant involvement of recent memory, with remote memory preserved until late stages. Personal and emotionally charged memories tend to be preferentially spared, whereas the opposite is true in psychogenic amnesia.Inability of an awake and alert patient to remember his or her own name strongly suggests a psychogenic disorder.
3. Language—The key elements of language are comprehension, repetition, fluency, naming, reading, and writing, all of which should be tested when a language disorder (aphasia) is suspected. There are a variety of aphasia syndromes, each characterized by a particular pattern of language impairment (Table 1-1) and often correlating with a specific site of pathology (Figure 1-8). Expressive, nonfluent, motor, or Broca aphasia is characterized by paucity of spontaneous speech and by the agrammatical and telegraphic nature of the little speech that is produced. Language expression is tested by listening for these abnormalities as the patient speaks spontaneously and answers questions. Patients with this syndrome are also unable to write normally or to repeat (tested with a content-poor phrase such as “no ifs, ands, or buts”), but their language comprehension is intact. Thus, if the patient is asked to do something that does not involve language expression (eg, “close your eyes”), he or she can do it. The patient is typically aware of the disorder and frustrated by it. In receptive, fluent, sensory, or Wernicke aphasia, language expression is normal, but comprehension and repetition are impaired. A large volume of language is produced, but it lacks meaning and may include paraphasic errors (use of words similar to the correct word) and neologisms (made-up words). Written language is similarly incoherent, and repetition is defective. The patient cannot follow oral or written commands, but can imitate the examiner’s action when prompted by a gesture to do. These patients are usually unaware of and therefore not disturbed by their aphasia. Global aphasia combines features of expressive and receptive aphasia—patients can neither express, comprehend, nor repeat spoken or written language. Other forms of aphasia include conduction aphasia, in which repetition is impaired whereas expression and comprehension are intact; transcortical aphasias, in which expressive, receptive, or global aphasia occurs with intact repetition; and anomic aphasia, a selective disorder of naming. Language is distinct from speech, the final motor step in oral expression of language. A speech disorder (dysarthria) may be difficult to distinguish from aphasia, but always spares oral and written language comprehension and written expression.
Table 1-1. Aphasia syndromes.
Figure 1-8. Brain areas involved in language function include the language comprehension (Wernicke) area, the motor speech (Broca) area, and the arcuate fasciculus. Lesions at the numbered sites produce aphasias with different features: (1) expressive aphasia, (2) receptive aphasia, (3) conduction aphasia—although the role of the arcuate fasciculus has been questioned, (4) transcortical expressive aphasia, and (5) transcortical receptive aphasia. See also Table 1-1. (Modified from Waxman SG. Clinical Neuroanatomy. 26th ed. New York, NY: McGraw-Hill; 2010.)
4. Sensory integration—Sensory integration disorders result from parietal lobe lesions and are manifested by misperception of or inattention to sensory stimuli on the side of the body contralateral to the lesion, even though primary sensory modalities (eg, touch) are intact on that side. Patients with parietal lesions may exhibit any of a number of signs. Astereognosis is the inability to identify by touch an object placed in the hand. With his or her eyes closed, the patient is asked to identify items such as coins, keys, and safety pins. Agraphesthesia is the inability to identify by touch a number written on the hand. Failure of two-point discrimination is the inability to differentiate between a single stimulus and two simultaneously applied, adjacent but separated, stimuli that can be distinguished by a normal person (or on the normal side). For example, the points of two pens can be applied together on a fingertip and then gradually separated until they are perceived as separate objects; the distance at which this occurs is then recorded. Allesthesia is misplaced (typically more proximal) localization of a tactile stimulus. Extinctionis the failure to perceive a visual or tactile stimulus when it is applied bilaterally, even though it can be perceived when applied unilaterally. Neglect is failure to attend to space or use the limbs on one side of the body. Anosognosia is unawareness of a neurologic deficit. Constructional apraxiais the inability to draw accurate representations of external space, such as in filling in the numbers on a clock face or copying geometric figures (Figure 1-9).
Figure 1-9. Unilateral (left-sided) neglect in a patient with a right parietal lesion. The patient was asked to fill in the numbers on the face of a clock (A) and to draw a flower (B). (Reproduced from Waxman SG. Clinical Neuroanatomy. 26th ed. New York, NY: McGraw-Hill; 2010.)
5. Motor integration—Praxis is the application of learning, and apraxia is the inability to perform previously learned tasks despite intact motor and sensory function. Typical tests for apraxia involve asking the patient to demonstrate how he or she would use a key, comb, or fork, without props. Unilateral apraxias are commonly caused by contralateral premotor frontal cortex lesions. Bilateral apraxias, such as gait apraxia, may be seen with bifrontal or diffuse cerebral lesions.
Cranial Nerves
A. Olfactory (I) Nerve
The olfactory nerve mediates the sense of smell (olfaction) and is tested by asking the patient to identify common scents, such as that of coffee, vanilla, peppermint, or cloves. Normal function of the nerve can be assumed if the patient detects the smell, even if he or she cannot identify it correctly. Each nostril is tested separately. Irritants such as alcohol should not be used because they may be detected as noxious stimuli independent of olfactory receptors.
B. Optic (II) Nerve
The optic nerve transmits visual information from the retina, through the optic chiasm (where fibers from the nasal, or medial, sides of both retinas, conveying information from the temporal, or lateral, halves of both visual fields, cross), and then via the optic tracts to the lateral geniculate nuclei of the thalami. Optic nerve function is assessed separately for each eye and involves inspecting the back of the eye (optic fundus) by direct ophthalmoscopy, measuring visual acuity, and mapping the visual field.
1. Ophthalmoscopy—This should be conducted in a dark room to dilate the pupils, which makes it easier to see the fundus. Mydriatic (sympathomimetic or anticholinergic) eye drops are sometimes used to enhance dilation, but this should not be done until visual acuity and pupillary reflexes are tested, nor in patients with untreated closed angle glaucoma or an intracranial mass lesion that might lead to transtentorial herniation. The normal optic disk (Figure 1-10) is a yellowish, oval structure situated nasally at the posterior pole of the eye. The margins of the disk and the blood vessels that cross it should be sharply demarcated, and the veins should show spontaneous pulsations. The macula, an area paler than the rest of the retina, is located about two disk diameters temporal to the temporal margin of the optic disk and can be visualized by having the patient look at the light from the ophthalmoscope. In patients with neurologic problems, the most important abnormality to identify on ophthalmoscopy is swelling of the optic disk resulting from increased intracranial pressure (papilledema). In early papilledema (Figure 1-11), the retinal veins appear engorged and spontaneous venous pulsations are absent. The disk may be hyperemic with linear hemorrhages at its borders. The disk margins become blurred initially at the nasal edge. In fully developed papilledema, the optic disk is elevated above the plane of the retina, and blood vessels crossing the disk border are obscured. Papilledema is almost always bilateral, does not typically impair vision except for enlargement of the blind spot, and is not painful. Another abnormality—optic disk pallor—is produced by atrophy of the optic nerve. It can be seen in patients with multiple sclerosis or other disorders and is associated with defects in visual acuity, visual fields, or pupillary reactivity.
Figure 1-10. The normal fundus. The diagram shows landmarks corresponding to the photograph. (Photo by Diane Beeston; reproduced with permission from Vaughan D, Asbury T, Riordan-Eva P. General Ophthalmology. 15th ed. Stamford, CT: Appleton & Lange; 1999.)
Figure 1-11. Appearance of the fundus in papilledema. A: In early papilledema, the superior and inferior margins of the optic disk are blurred by the thickened layer of nerve fibers entering the disk. B:Moderate papilledima with disk swelling. C: In fully developed papilledema, the optic disk is swollen, elevated, and congested, and the retinal veins are markedly dilated; swollen nerve fibers (white patches) and hemorrhages can be seen. D: In chronic atrophic papilledema, the optic disk is pale and slightly elevated, and its margins are blurred. (Photos courtesy of Nancy Newman.)
2. Visual acuity—This should be tested under conditions that eliminate refractive errors, so patients who wear glasses should be examined with them on. Acuity is tested in each eye separately, using a Snellen eye chart approximately 6 m (20 ft) away for distant vision or a Rosenbaum pocket eye chart approximately 36 cm (14 in) away for near vision. The smallest line of print that can be read is noted, and acuity is expressed as a fraction: 20/20 indicates normal acuity, with the denominator increasing as vision worsens. More severe impairment can be graded according to the distance at which the patient can count fingers, discern hand movement, or perceive light. Red–green color vision is often disproportionately impaired with optic nerve lesions and can be tested using colored pens or hatpins or with color vision plates.
3. Visual fields—Visual fields are tested for each eye separately, most often using the confrontation technique (Figure 1-12). The examiner stands at about arm’s length from the patient, the patient’s eye that is not being tested and the examiner’s eye opposite it are closed or covered, and the patient is instructed to fix on the examiner’s open eye, superimposing the monocular fields of patient and examiner. Using the index finger of either hand to locate the peripheral limits of the patient’s field, the examiner then moves the finger slowly inward in all directions until the patient detects it. The size of the patient’s central scotoma (blind spot), located in the temporal half of the visual field, can also be measured in relation to the examiner’s. The object of confrontation testing is to determine whether the patient’s visual field is coextensive with—or more restricted than—the examiner’s. Another approach to confrontation testing is to use the head of a hatpin as the visual target. Subtle field defects may be detected by asking the patient to compare the brightness of colored objects presented at different sites in the field or by measuring the fields using a pin with a red head as the target. Gross visual field abnormalities can be detected in less than fully alert patients by determining whether they blink when the examiner’s finger is brought toward the patient’s eye from various directions. In some situations (eg, following the course of a patient with a progressive or resolving defect), it is valuable to map the visual fields more precisely, which can be done using perimetry techniques, such as tangent screen or automated perimetry testing. Common visual field abnormalities and their anatomic correlates are shown in Figure 1-13.
Figure 1-12. Confrontation testing of the visual field. A. The left eye of the patient and the right eye of the examiner are aligned. B. Testing the superior nasal quadrant. C. Testing the superior temporal quadrant. D. Testing the inferior nasal quadrant. The procedure is then repeated for the patient’s other eye. E. Testing the inferior temporal quadrant.
Figure 1-13. Common visual field defects and their anatomic bases. 1. Central scotoma caused by inflammation of the optic disk (optic neuritis) or optic nerve (retrobulbar neuritis). 2. Total blindness of the right eye from a complete lesion of the right optic nerve. 3. Bitemporal hemianopia caused by pressure exerted on the optic chiasm by a pituitary tumor. 4. Right nasal hemianopia caused by a perichiasmal lesion (eg, calcified internal carotid artery). 5. Right homonymous hemianopia from a lesion of the left optic tract. 6. Right homonymous superior quadrantanopia caused by partial involvement of the optic radiation by a lesion in the left temporal lobe (Meyer loop). 7. Right homonymous inferior quadrantanopia caused by partial involvement of the optic radiation by a lesion in the left parietal lobe. 8. Right homonymous hemianopia from a complete lesion of the left optic radiation. (A similar defect may also result from lesion 9.) 9. Right homonymous hemianopia (with macular sparing) resulting from posterior cerebral artery occlusion.
C. Oculomotor (III), Trochlear (IV), and Abducens (VI) Nerves
These three nerves control the action of the intraocular (pupillary sphincter) and extraocular muscles.
1. Pupils—The diameter and shape of the pupils in ambient light and their responses to light and accommodation should be ascertained. Normal pupils average ≈3 mm in diameter in a well-lit room, but can vary from ≈6 mm in children to <2 mm in the elderly, and can differ in size from side to side by ≈1 mm (physiologic anisocoria). They should be round and regular in shape. Normal pupils constrict briskly in response to direct illumination, and somewhat less so to illumination of the pupil on the opposite side (consensual response), and dilate again rapidly when the source of illumination is removed. When the eyes converge to focus on a nearer object such as the tip of one’s nose (accommodation), normal pupils constrict. Pupillary constriction (miosis) is mediated through parasympathetic fibers that originate in the midbrain and travel with the oculomotor nerve to the eye. Interruption of this pathway, such as by a hemispheric mass lesion producing coma and compressing the nerve as it exits the brainstem, produces a dilated (≈7 mm) unreactive pupil. Pupillary dilation is controlled by a three-neuron sympathetic relay, from the hypothalamus, through the brainstem to the T1 level of the spinal cord, to the superior cervical ganglion, and to the eye. Lesions anywhere along this pathway result in constricted (≤1 mm) unreactive pupils. Other common pupillary abnormalities are listed in Table 1-2.
Table 1-2. Common pupillary abnormalities.
2. Eyelids and orbits—The eyelids (palpebrae) should be examined with the patient’s eyes open. The distance between the upper and lower lids (interpalpebral fissure) is usually ≈10 mm and approximately equal in the two eyes. The upper lid normally covers 1 to 2 mm of the iris, but this is increased by drooping of the lid (ptosis) due to lesions of the levator palpebrae muscle or its oculomotor (III) or sympathetic nerve supply. Ptosis occurs together with miosis (and sometimes defective sweating, or anhidrosis, of the forehead) in Horner syndrome. Abnormal protrusion of the eye from the orbit (exophthalmos or proptosis) is best detected by standing behind the seated patient and looking down at his or her eyes.
3. Eye movements—Movement of the eyes is accomplished by the action of six muscles attached to each globe, which act to move the eye into each of six cardinal positions of gaze (Figure 1-14). Equal and opposed actions of these six muscles in the resting state place the eye in mid- or primary position, that is, looking directly forward. When the function of an extraocular muscle is disrupted, the eye is unable to move in the direction of action of the affected muscle (ophthalmoplegia) and may deviate in the opposite direction because of the unopposed action of other extraocular muscles. When the eyes are thus misaligned, visual images of perceived objects fall on a different region of each retina, creating the illusion of double vision, or diplopia. The extraocular muscles are innervated by the oculomotor (III), trochlear (IV), and abducens (VI) nerves, and defects in eye movement may result from either muscle or nerve lesions. The oculomotor (III) nerve innervates all the extraocular muscles except the superior oblique, which is innervated by the trochlear (IV) nerve, and the lateral rectus, which is innervated by the abducens (VI) nerve. Because of their differential innervation, the pattern of ocular muscle involvement in pathologic conditions can help to distinguish a disorder of the ocular muscles per se from a disorder that affects a cranial nerve.
Figure 1-14. The six cardinal positions of gaze for testing eye movement. The eye is adducted by the medial rectus and abducted by the lateral rectus. The adducted eye is elevated by the inferior oblique and depressed by the superior oblique; the abducted eye is elevated by the superior rectus and depressed by the inferior rectus. All extraocular muscles are innervated by the oculomotor (III) nerve except the superior oblique, which is innervated by the trochlear (IV) nerve, and the lateral rectus, which is innervated by the abducens (VI) nerve.
Eye movement is tested by having the patient look at a flashlight held in each of the cardinal positions of gaze and observing whether the eyes move fully and in a yoked (conjugate) fashion in each direction. With normal conjugate gaze, light from the flashlight falls at the same spot on both corneas. Limitations of eye movement and any disconjugacy should be noted. If the patient complains of diplopia, the weak muscle responsible should be identified by having the patient gaze in the direction in which the separation of images is greatest. Each eye is then covered in turn and the patient is asked to report which of the two (near or far) images disappears. The image displaced farther in the direction of gaze is always referable to the weak eye. Alternatively, one eye is covered with translucent red glass, plastic, or cellophane, which allows the eye responsible for each image to be identified. For example, with weakness of the left lateral rectus muscle, diplopia is maximal on leftward gaze, and the leftmost of the two images seen disappears when the left eye is covered.
4. Ocular oscillations—Nystagmus, or rhythmic oscillation of the eyes, can occur at the extremes of voluntary gaze in normal subjects. In other settings, however, it may be due to anticonvulsant or sedative drugs, or reflect disease affecting the extraocular muscles or their innervation, or vestibular or cerebellar pathways. The most common form, jerk nystagmus, consists of a slow phase of movement followed by a fast phase in the opposite direction (Figure 1-15). To detect nystagmus, the eyes are observed in the primary position and in each of the cardinal positions of gaze. If nystagmus is observed, it should be described in terms of the position of gaze in which it occurs, its direction and amplitude (fine or coarse), precipitating factors such as changes in head position, and associated symptoms, such as vertigo. The direction of jerk nystagmus is, by convention, the direction of the fast phase (eg, leftward-beating nystagmus). Jerk nystagmus usually increases in amplitude with gaze in the direction of the fast phase (Alexander law). A less common form of nystagmus is pendular nystagmus, which usually begins in infancy and is of equal velocity in both directions.
Figure 1-15. Nystagmus. A slow drift of the eyes away from the position of fixation (indicated by the broken arrow) is corrected by a quick movement back (solid arrow). The direction of the nystagmus is named from the quick component. Nystagmus from the primary position is more likely to be pathologic than that from the end position. (From LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
D. Trigeminal (V) Nerve
The trigeminal nerve conveys sensory fibers from the face and motor fibers to the muscles of mastication. Facial touch and temperature sensation are tested by touching and by placing the cool surface of a tuning fork on both sides of the face simultaneously in the distribution of each division of the trigeminal nerve—ophthalmic (V1, forehead), maxillary (V2, cheek), and mandibular (V3, jaw) (Figure 1-16). The patient is asked if the sensation is the same on both sides and, if not, on which side the stimulus is felt less well, or as less cool. To test the corneal reflex, a wisp of cotton is swept lightly across the lateral surface of the eye (out of the subject’s view). The normal response, which is mediated by a reflex arc that depends on trigeminal (V1) nerve sensory and facial (VII) nerve moor function, is bilateral blinking of the eyes. With impaired trigeminal function, neither eye blinks, whereas unilateral blinking implies a facial nerve lesion on the unblinking side. Trigeminal motor function is tested by observing the symmetry of opening and closing of the mouth; on closing, the jaw falls faster and farther on the weak side, causing the face to look askew. More subtle weakness can be detected by asking the patient to clench his or her teeth and attempting to force the jaw open. Normal jaw strength cannot be overcome by the examiner.
Figure 1-16. Trigeminal (V) nerve sensory divisions. (From Waxman SG. Clinical Neuroanatomy. 26th ed. New York, NY: McGraw-Hill; 2010.)
E. Facial (VII) Nerve
The facial nerve supplies the facial muscles and mediates taste sensation from about the anterior two-thirds of the tongue (Figure 1-17). To test facial strength, the patient’s face should be observed for symmetry or asymmetry of the palpebral fissures and nasolabial folds at rest. He or she is then asked to wrinkle the forehead, squeeze the eyes tightly shut (looking for asymmetry in the extent to which the eyelashes protrude), and smile or show his or her teeth. Again the examiner looks for symmetry or asymmetry. With a peripheral (facial nerve) lesion, an entire side of the face is weak, and the eye cannot be fully closed. With a central (eg, hemispheric) lesion, the forehead is spared, and some ability to close the eye is retained. This discrepancy is thought to result from dual cortical motor input to the upper face. The traditional view has been that there is bilateral cortical representation of the upper face, but it has also been suggested that dual inputs arise from the same hemisphere, one within the distribution of the middle cerebral artery and the other in the anterior cerebral artery territory. Bilateral facial weakness cannot be detected by comparison between the two sides. It is tested for instead by asking the patient to squeeze both eyes tightly shut, press the lips tightly together, and then puff out his or her checks. If strength is normal, the examiner should not be able to pry open the eyelids, force apart the lips, or force air out of the mouth by compressing the cheeks. Facial weakness may be associated with dysarthria that is most pronounced for m sounds. If the patient is normally able to whistle, this ability may be lost with facial weakness. To test taste sensation, cotton-tipped applicators are dipped in sweet, sour, salty, or bitter solutions and placed on the protruded tongue, and the patient is asked to identify the taste.
Figure 1-17. Facial (VII) nerve. A. Central and peripheral motor innervation of the face. The forehead receives motor projections from both hemispheres and the lower face (eyes and below) from the contralateral hemisphere only. B. Somatic afferent (SA, touch) and visceral afferent (VA, taste) innervation of the tongue. (From Waxman SG. Clinical Neuroanatomy. 26th ed. New York, NY: McGraw-Hill; 2010.)
F. Acoustic (VIII) Nerve
The acoustic nerve has two divisions—auditory and vestibular—which are involved in hearing and equilibrium, respectively. Examination should include otoscopic inspection of the auditory canals and tympanic membranes, assessment of auditory acuity in each ear, and Weber and Rinne tests performed with a 512-Hz tuning fork. Auditory acuity can be tested crudely by rubbing thumb and forefinger together approximately 2 in from each ear.
If the patient complains of hearing loss or cannot hear the finger rub, the nature of the hearing deficit should be explored. To perform the Rinne test (Figure 1-18), the base of a lightly vibrating, high-pitched tuning fork is placed on the mastoid process of the temporal bone until the sound can no longer be heard; the tuning fork is then moved near the opening of the external auditory canal. In patients with normal hearing or sensorineural hearing loss, air in the auditory canal conducts sound better than bone, and the tone can still be heard. With conductive hearing loss, the patient hears the tone longer with the tuning fork on the mastoid process than the air-conducted tone. In the Weber test (Figure 1-18), the handle of the vibrating tuning fork is placed in the middle of the forehead. With conductive hearing loss, the tone will sound louder in the affected ear; with sensorineural hearing loss, the tone will be louder in the normal ear.
Figure 1-18. Tests for hearing loss.
In patients who complain of positional vertigo, the Nylen-Bárány or Dix-Hallpike maneuver (Figure 1-19) can be used to try to reproduce the precipitating circumstance. The patient is seated on a table with the head and eyes directed forward and is then quickly lowered to a supine position with the head over the table edge, 45 degrees below horizontal. The test is repeated with the patient’s head and eyes turned 45 degrees to the right and again with the head and eyes turned 45 degrees to the left. The eyes are observed for nystagmus, and the patient is asked to note the onset, severity, and cessation of vertigo.
Figure 1-19. Test for positional vertigo and nystagmus. The patient is seated on a table with the head and eyes directed forward (A) and is then quickly lowered to a supine position with the head over the table edge, 45 degrees below horizontal. The patient’s eyes are then observed for nystagmus, and the patient is asked to report any vertigo. The test is repeated with the patient’s head and eyes turned 45 degrees to the right (B), and again with the head and eyes turned 45 degrees to the left.
G. Glossopharyngeal (IX) and Vagus (X) Nerves
Motor function of these nerves is tested by asking the patient to say “ah” with his or her mouth open and looking for full and symmetric elevation of the palate. With unilateral weakness, the palate fails to elevate on the affected side; with bilateral weakness, neither side elevates. Patients with palatal weakness may also exhibit dysarthria, which affects especially k sounds. Sensory function can be tested by the gag reflex. The back of the tongue is stimulated on each side in turn using a tongue depressor or cotton-tipped applicator, and differences in the magnitude of gag responses elicited in this manner are noted.
H. Spinal Accessory (XI) Nerve
The spinal accessory nerve innervates the sternocleidomastoid and trapezius muscles. The sternocleidomastoid is tested by asking the patient to rotate his or her head against resistance provided by the examiner’s hand, which is placed on the patient’s jaw. Sternocleidomastoid weakness results in decreased ability to rotate the head away from the weak muscle. The trapezius is tested by having the patient shrug his or her shoulders against resistance and noting any asymmetry.
I. Hypoglossal (XII) Nerve
The hypoglossal nerve innervates the tongue muscles. Its function can be tested by having the patient push his or her tongue against the inside of the cheek while the examiner resists by pressure on the outside of the cheek. In some cases, there may be also deviation of the protruded tongue toward the weak side, but facial weakness may result in false-positive tests. Tongue weakness also produces dysarthria with prominent slurring of labial (l) sounds. Finally, denervation of the tongue may be associated with wasting (atrophy) and twitching (fasciculation).
Motor Function
Motor function is governed by both upper and lower motor neurons. Upper motor neurons arise in cerebral cortex and brainstem and project onto lower motor neurons in the brainstem and anterior horn of the spinal cord. They include the projection from cortex to spinal cord (corticospinal tract) and the part of the corticospinal tract that crosses (decussates) in the medulla (pyramidal tract). The motor examination includes evaluation of muscle bulk, tone, and strength. Lower motor neurons project from brainstem and spinal cord, via motor nerves, to innervate skeletal muscle. Lesions of either upper or lower motor neurons produce weakness. As discussed later, upper motor neuron lesions also cause increased muscle tone, hyperactive tendon reflexes, and Babinski signs, whereas lower motor neuron lesions produce decreased muscle tone, hypoactive reflexes, muscle atrophy, and fasciculations.
A. Bulk
The muscles should be inspected to determine whether they are normal or decreased in bulk. Reduced muscle bulk (atrophy) is usually the result of denervation from lower motor neuron (spinal cord anterior horn cell or peripheral nerve) lesions. Asymmetric atrophy can be detected by comparing the bulk of individual muscles on the two sides, by visual inspection, or by the use of a tape measure. Atrophy may be associated with fasciculations, or rapid muscle twitching that resembles wormlike writhing under the skin.
B. Tone
Tone is resistance of a muscle to passive movement at a joint. With normal tone, there is little such resistance. Abnormally decreased tone (hypotonia or flaccidity) may accompany muscle, lower motor neuron, or cerebellar disorders. Increased tone takes the form of rigidity, in which the increase is constant over the range of motion at a joint, or spasticity, in which the increase is velocity-dependent and variable over the range of motion. Rigidity is associated classically with diseases of the basal ganglia and spasticity with diseases affecting the corticospinal tracts. Tone at the elbow is measured by supporting the patient’s arm with one hand under his or her elbow, then flexing, extending, pronating, and supinating the forearm with the examiner’s other hand. The arm should move smoothly in all directions. Tone at the wrist is tested by grasping the forearm with one hand and flopping the wrist back and forth with the other. With normal tone, the hand should rest at a 90-degree angle at the wrist. Tone in the legs is measured with the patient lying supine and relaxed. The examiner places one hand under the knee, then pulls abruptly upward. With normal or reduced tone, the patient’s heel is lifted only momentarily off the bed or remains in contact with the surface of the bed as it slides upward. With increased tone, the leg lifts completely off the bed. Axial tone can be measured by passively rotating the patient’s head and observing whether the shoulders also move, which indicates increased tone, or by gently but firmly flexing and extending the neck and noting whether resistance is encountered.
C. Strength
Muscle strength, or power, is graded on a scale according to the force a muscle can overcome: 5, normal strength; 4, decreased strength but still able to move against gravity plus added resistance; 3, able to move against gravity but not added resistance; 2, able to move only with the force of gravity eliminated (eg, horizontally); 1, flicker of movement; 0, no visible muscle contraction. What is normal strength for a young person cannot be expected of a frail, elderly individual, and this must be taken into account in grading muscle strength. Strength is tested by having the patient execute a movement that involves a single muscle or muscle group and then applying a gradually increasing opposing force to determine whether the patient’s movement can be overcome (Figure 1-20). Where possible, the opposing force should be applied using muscles of similar size (eg, the arm for proximal and the fingers for distal limb muscles). The emphasis should be on identifying differences from side to side, between proximal and distal muscles, or between muscle groups innervated by different nerves or nerve roots. In pyramidal weakness (due to lesions affecting the corticospinal tract), there is preferential weakness of extensor muscles in the upper and flexor muscles in the lower extremity. Fine finger movements, such as rapidly tapping the thumb and index finger together, are slowed. With the arms extended, palms up, and eyes closed, the affected arm falls slowly downward and the hand pronates (pronator drift). Bilaterally symmetrical distal weakness is characteristic of polyneuropathy, whereas bilaterally symmetrical proximal weakness is observed in myopathy. Tests of strength for selected individual muscles are illustrated in the Appendix.
Figure 1-20. Technique for testing muscle strength. In the example shown (biceps), the patient flexes the arm and the examiner tries to overcome this movement. (From LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
Sensory Function
Somatic sensation is mediated through large sensory fibers that travel from the periphery to the thalamus in the posterior columns of the spinal cord and brainstem medial lemniscus and small sensory fibers that ascend to the thalamus in the spinothalamic tracts. Light touch sensation is conveyed by both pathways, vibration and position sense by the large-fiber pathway, and pain and temperature sense by the small-fiber pathway. Because most sensory disorders affect distal more than proximal sites, screening should begin distally (ie, at the toes in the lower and fingers in the upper limb) and proceed proximally, until the border of any deficit is reached. If the patient complains of sensory loss in a specific area, sensory testing should begin in the center of that area and proceed outward until sensation is reported as normal. Comparing the intensity of or threshold for sensation on the two sides of the body is useful for detecting lateralized sensory deficits. When sensory deficits are more limited, such as when they affect a single limb or truncal segment, their distribution should be compared with that of the spinal roots and peripheral nerves (see Chapter 10, Sensory Disorders) to determine whether involvement of a specific root or nerve can explain the deficit observed. Some tests of somatosensory function are illustrated in Figure 1-21.
Figure 1-21. Tests of somatosensory function. A. Touch (using finger or dull end of safety pin) and pain (sharp end of safety pin). B. Joint position sense. C. Vibration sense (using 128-Hz tuning fork). (Modified from LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
A. Light Touch
Touch perception is tested by applying a light stimulus—such as a wisp of cotton, the teased-out tip of a cotton swab, or a brushing motion of the fingertips—to the skin of a patient whose eyes are closed and asking him or her to indicate where the stimulus is perceived. If a unilateral deficit is suspected, the patient can be asked to compare how intensely a touch stimulus is felt when applied at the same site on the two sides.
B. Vibration
Vibration sense is tested by striking a low-pitched (128-Hz) tuning fork and firmly placing its base on a bony prominence, such as a joint; the fingers of the examiner holding the tuning fork serve as a control for normal vibration sense. The patient is asked to indicate whether the vibration is felt and, if so, when the feeling goes away. Testing begins distally, at the toes and fingers, and proceeds proximally from joint to joint until sensation is normal.
C. Position
To test joint position sense, the examiner grasps the sides of the distal phalanx of a finger or toe and slightly displaces the joint up or down. The patient, with eyes closed, is asked to report any perceived change in position. Normal joint position sense is exquisitely sensitive, and the patient should detect the slightest movement. If joint position sense is diminished distally, more proximal limb joints are tested until normal position sense is encountered. Another test of position sense is to have the patient close his or her eyes, extend the arms, and then touch the tips of the index fingers together.
D. Pain
A disposable pin should be used to prick (but not puncture) the skin with enough force for the resulting sensation to be mildly unpleasant. The patient is asked whether the stimulus feels sharp. If a safety pin is used, the rounded end can be used to demonstrate to the patient the intended distinction between a sharp and dull stimulus. Depending on the circumstance (eg, a complaint referable to a specific site or screening in the absence of sensory symptoms), the examiner should compare pain sensation from side to side, distal to proximal, or dermatome to dermatome, and from the area of deficit toward normal regions.
E. Temperature
This can be tested using the flat side of a cold tuning fork or another cold object. The examiner should first establish the patient’s ability to detect the cold sensation in a presumably normal area. Cold sensation is then compared on the two sides, moving from distal to proximal, across dermatomes, and from abnormal toward normal areas.
Coordination
Impaired coordination (ataxia), which usually results from lesions affecting the cerebellum or its connections, can affect the eye movements, speech, limbs, or trunk. Some tests of coordination are illustrated in Figure 1-22.
Figure 1-22. Tests of cerebellar function: finger-to-nose test (left), test for rebound (center), and heel-knee-shin test (right). (From LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
A. Limb Ataxia
Distal limb ataxia can be detected by asking the patient to perform rapid alternating movements (eg, alternately tapping the palm and dorsum of the hand on the patient’s other hand, or tapping the sole of the foot on the examiner’s hand) and noting any irregularity in the rate, rhythm, amplitude, or force of successive movements. In the finger-to-nose test, the patient moves an index finger back and forth between his or her nose and the examiner’s finger; ataxia may be associated with intention tremor, which is most prominent at the beginning and end of each movement. Impaired ability to check the force of muscular contraction can also often be demonstrated. When the patient is asked to raise the arms rapidly to a given height—or when the arms, extended and outstretched in front of the patient, are displaced by a sudden force—there may be overshooting (rebound). This can be demonstrated by having the patient forcefully flex the arm at the elbow against resistance—and then suddenly removing the resistance. If the limb is ataxic, continued contraction without resistance may cause the hand to strike the patient. Ataxia of the lower limbs can be demonstrated by the heel-knee-shin test. The supine patient is asked to run the heel of the foot smoothly up and down the opposite shin from ankle to knee. Ataxia produces jerky and inaccurate movement, making it impossible for the patient to keep the heel in contact with the shin.
B. Truncal Ataxia
To detect truncal ataxia, the patient is asked to sit on the side of the bed or in a chair without lateral support, and any tendency to list to one side is noted.
Reflexes
A. Tendon Reflexes
A tendon reflex is the reaction of a muscle to being passively stretched by percussion on a tendon and depends on the integrity of both afferent and efferent peripheral nerves and their inhibition by descending central pathways. Tendon reflexes are decreased or absent in disorders that affect any part of the reflex arc, most often by polyneuropathies, and increased by lesions of the corticospinal tract. Tendon reflexes are graded on a scale according to the force of the contraction or the minimum force needed to elicit the response: 4, very brisk, often with rhythmic reflex contractions (clonus); 3, brisk but normal; 2, normal; 1, minimal; 0, absent. In some cases, tendon reflexes are difficult to elicit, but may be brought out by having the patient clench the fist on the side not being tested or interlock the fingers and attempt to pull them apart. The main goal of reflex testing is to detect asymmetry. However, symmetrically absent reflexes suggest a polyneuropathy and symmetrically increased reflexes may indicate bilateral cerebral or spinal cord disease. The commonly tested tendon reflexes and the nerve roots they involve are as follows: biceps and brachioradialis (C5-6), triceps (C7-8), quadriceps (L3-4), and Achilles (S1-2). Methods for eliciting these tendon reflexes are shown in Figure 1-23.
Figure 1-23. Methods to elicit the tendon reflexes. Techniques for eliciting the quadriceps reflex in both seated and supine patients are shown. (Modified from LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.)
B. Superficial Reflexes
The superficial reflexes are elicited by stimulating the skin, rather than tendons, and are altered or absent in disorders affecting the corticospinal tract. They include the plantar reflex, in which stroking the sole of the foot from its lateral border near the heel toward the great toe results in plantar flexion of the toes. With corticospinal lesions, the great toe dorsiflexes (Babinski sign), which may be accompanied by fanning of the toes, dorsiflexion at the ankle, and flexion at the thigh (Figure 1-24). Several superficial reflexes that are normally present in infancy, and subsequently disappear, may reappear with aging or frontal lobe dysfunction. The palmar grasp reflex, elicited by stroking the skin of the patient’s palm with the examiner’s fingers, causes the patient’s fingers to close around those of the examiner. The plantar grasp reflex consists of flexion and adduction of the toes in response to stimulation of the sole of the foot. The palmomental reflex is elicited by scratching the palm of the hand and results in contraction of ipsilateral chin (mentalis) and perioral (orbicularis oris) muscles. The suck reflex consists of involuntary sucking movements following stimulation of the lips. The snout reflex is elicited by gently tapping the lips and results in their protrusion. In the rooting reflex, stimulation of the lips causes them to deviate toward the stimulus. The glabellar reflex is elicited by repetitive tapping on the forehead; normal subjects blink only in response to the first several taps, whereas persistent blinking is an abnormal response (Myerson sign).
Figure 1-24. Extensor plantar reflex (Babinski sign). (Modified from LeBlond RF, Brown DD, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009)
Stance & Gait
The patient should be asked to stand with feet together and eyes open to detect instability from cerebellar ataxia. Next, the patient should close his or her eyes; instability occurring with eyes closed but not open (Romberg sign) is a sign of sensory ataxia. The patient should then be observed as he or she walks normally, on the heels, on the toes, and in tandem (one foot placed directly in front of the other), to identify any of the following classic gait abnormalities (Figure 1-25).
Figure 1-25 Gait abnormalities. Left to right: hemiplegic gait, paraplegic gait, parkinsonian gait, steppage gait, dystrophic gait. (From Springhouse. Handbook of Signs & Symptoms. 3rd ed. Ambler, PA: Lippincott Williams & Wilkins; 2006.)
1. Hemiplegic gait—The affected leg is held extended and internally rotated, the foot is inverted and plantar flexed, and the leg moves in a circular direction at the hip (circumduction).
2. Paraplegic gait—The gait is slow and stiff, with the legs crossing in front of each other (scissoring).
3. Cerebellar ataxic gait—The gait is wide-based and may be associated with staggering or reeling, as if one were drunk.
4. Sensory ataxic gait—The gait is wide based, the feet are slapped down onto the floor, and the patient may watch his or her feet.
5. Steppage gait—Inability to dorsiflex the foot, often due to a peroneal nerve lesion, results in exaggerated elevation of the hip and knee to allow the foot to clear the floor while walking.
6. Dystrophic gait—Pelvic muscle weakness produces a lordotic, waddling gait.
7. Parkinsonian gait—Posture is flexed, starts are slow, steps are small and shuffling, there is reduced arm swing, and involuntary acceleration (festination) may occur.
8. Choreic gait—The gait is jerky and lurching, but falls are surprisingly rare.
9. Apraxic gait—Frontal lobe disease may result in loss of the ability to perform a previously learned act (apraxia), in this case the ability to walk. The patient has difficulty initiating walking and may appear to be glued to the floor. Once started, the gait is slow and shuffling. However, there is no difficulty performing the same leg movements when the patient is lying down and the legs are not bearing weight.
10. Antalgic gait—One leg is favored over the other in an effort to avoid putting weight on the injured leg and causing oneself pain.
NEUROLOGIC EXAMINATION IN SPECIAL SETTINGS
Although the neurologic examination is always tailored to a patient’s specific situation, it is sufficiently distinctive to deserve mention in two special settings: examination of the comatose patient and “screening” examination of a patient without neurologic complaints.
Coma
The comatose patient cannot cooperate for a full neurologic examination. Fortunately, however, a great deal of information can be derived from much more limited examination of comatose patients, focused on three elements: the pupillary reaction to light, eye movements induced by oculocephalic (head turning) or oculovestibular (cold water caloric) stimulation, and the motor response to pain. Examination of the comatose patient is discussed at length in Chapter 3, Coma.
“Screening” Neurologic Examination
1. Mental status—Observe whether the patient is awake and alert, confused, or unarousable. Test for orientation to person, place, and time. Screen for aphasia by asking the patient to repeat “no ifs, ands, or buts.”
2. Cranial nerves—Examine the optic disks for papilledema. Test the visual fields by confrontation. Confirm the patient’s ability to move the eyes conjugately in the six cardinal directions of gaze. Have the patient close his or her eyes tightly and show his or her teeth to assess facial strength.
3. Motor function—Compare the two sides with respect to speed of fine finger movements, strength of extensor muscles in the upper limb, and strength of flexor muscles in the lower limb, to detect corticospinal tract lesions.
4. Sensory function—Ask the patient to sketch out any area of perceived sensory deficit. Test light touch and vibration sense in the feet and, if impaired, determine the upper limit of impairment in both the lower and upper limbs.
5. Reflexes—Compare the two sides for activity of the biceps, triceps, quadriceps, and Achilles tendon reflexes, as well as the plantar responses.
6. Coordination, stance, and gait—Watch the patient stand and walk and note any asymmetry or instability of stance or gait.
DIAGNOSTIC FORMULATION
Principles of Diagnosis
Once the history and examination are completed, evaluation of a neurologic problem proceeds with the formulation of a provisional diagnosis. As discussed next, this is divided into two stages: anatomic diagnosis and etiologic diagnosis. The diagnostic process should always be guided by the law of parsimony, or Occam’s razor: the simplest explanation is most likely to be correct. This means that a single, unifying diagnosis should be sought in preference to multiple diagnoses, each accounting for a different feature of the patient’s problem.
Anatomic Diagnosis: Where Is the Lesion?
Anatomic diagnosis takes advantage of neuroanatomic principles to localize a lesion in space. The precision with which localization can be achieved varies, but it should always be possible at least to state the highest and lowest levels of the nervous system at which a lesion could produce the clinical picture under consideration.
A. Central Versus Peripheral Nervous System
Making this distinction is typically the first step in anatomic diagnosis. Many symptoms and signs can be produced by both central and peripheral processes, but some symptoms and signs are more definitive. For example, cognitive abnormalities, visual field deficits, hyperreflexia, or extensor plantar responses (Babinski signs) point to the central nervous system, whereas muscle atrophy, fasciculation, or areflexia usually results from peripheral nervous system disorders.
B. Valsalva Doctrine
Unilateral brain lesions typically produce symptoms and signs on the opposite (contralateral) side of the body. This doctrine helps localize most focal cerebral lesions. However, exceptions occur. For example, hemispheric mass lesions that cause transtentorial herniation may compress the contralateral cerebral peduncle in the midbrain, producing hemiparesis on the same side as the mass. Brainstem lesions can produce crossed deficits, with weakness or sensory loss over the ipsilateral face and contralateral limbs. Thus a unilateral lesion in the pons can cause ipsilateral facial weakness due to involvement of the facial (VII) nerve nucleus, with contralateral weakness of the arm and leg from involvement of descending motor pathways above their crossing (decussation) in the medulla. Wallenberg syndrome, usually due to a stroke in the lateral medulla, is associated with ipsilateral impairment of pain and temperature sensation over the face due to involvement of the descending tract and nucleus of the trigeminal (V) nerve, with contralateral pain and temperature deficits in the limbs from interruption of the lateral spinothalamic tract. Lesions of a cerebellar hemisphere produce ipsilateral symptoms and signs (eg, limb ataxia), due partly to connections with the contralateral cerebral cortex. Finally, the spinal accessory (XI) nerve receives bilateral input from motor cortex, with ipsilateral input predominating, so a cortical lesion can produce ipsilateral sternocleidomastoid muscle weakness.
C. Anatomic Patterns of Involvement
Anatomic diagnosis of neurologic lesions can be facilitated by recognizing patterns of involvement characteristic of disease at different sites (Figure 1-26). Hemispheric lesions are suggested by contralateral motor and sensory deficits affecting face, arm, and leg, as well as by cognitive or visual field abnormalities. Brainstem lesions should be suspected with crossed deficits (motor or sensory involvement of the face on one side of the body and the arm and leg on the other) or cranial nerve (eg, ocular) palsies. Spinal cord lesions produce deficits below the level of the lesion and, except for high cervical cord lesions affecting the spinal tract and nucleus of the trigeminal (V) nerve, spare the face. The relative involvement of upper motor neurons, lower motor neurons, and various sensory pathways depends on the site and extent of the lesion in the horizontal plane. Polyneuropathies produce distal, symmetric sensory deficits and weakness, which usually affect the lower more than the upper limbs, and are associated with areflexia. Myopathies (disorders of muscle) produce proximal weakness, which may affect the face and trunk as well as the limbs, without sensory loss.
Figure 1-26. Anatomic patterns of involvement resulting from disorders affecting different sites in the nervous system. Filled areas are affected.
Etiologic Diagnosis: What Is the Lesion?
A. Revisit the History
Once an anatomic diagnosis is arrived at, the next step is to identify the cause. Often the patient’s prior history contains clues in this regard. Preexisting diseases such as hypertension, diabetes, heart disease, cancer, and AIDS are each associated with a spectrum of neurologic complications. Numerous medications and drugs of abuse (eg, alcohol and tobacco) have neurologic side effects. The family history may point to a genetic disease.
B. Consider General Categories of Disease
Neurologic disease can be produced by the same kinds of pathologic processes that cause disease in other organ systems (Table 1-3). Once a neurologic problem has been localized, it can be helpful to run through each of these categories to generate a list of possible etiologies.
Table 1-3. Etiologic categories of neurologic disease.
C. Time Course Is a Clue to Etiology
The time course of a disorder is an important clue to its etiology (Figure 1-1). For example, only a few processes produce neurologic symptoms that evolve within minutes—typically ischemia, seizure, or syncope. Neoplastic and degenerative processes, on the other hand, give rise to progressive, unremitting symptoms and signs, whereas inflammatory and metabolic disorders may wax and wane.
D. Common Diseases Are Common
Sometimes the anatomic syndrome is sufficiently distinctive that the cause is obvious. More often, however, an anatomic syndrome can have multiple etiologies. When this is the case, it is important to remember that common diseases are common and that even unusual presentations of common diseases occur more frequently than classic presentations of rare diseases. Figure 1-27 illustrates the relative prevalence of several neurologic diseases. It is helpful diagnostically to have a general sense of how common different diseases are and whether they tend to affect particular populations (ie, ages, sexes, or ethnic groups) disproportionately. For example, multiple sclerosis usually has its onset between the ages of 20 and 40 years, affects women more often than men, and preferentially affects individuals of north European descent.
Figure 1-27. Prevalence of selected neurologic diseases (US). (Data from Ropper A, Samuels M. Adams and Victor’s Neurology. 9th ed. New York, NY: McGraw-Hill; 2009.)
LABORATORY INVESTIGATIONS
After the history is taken, the general physical and neurologic examinations are completed, and a preliminary diagnosis is formulated, laboratory investigations are often undertaken to obtain additional diagnostic information. These investigations are addressed in Chapter 2.
REFERENCES
Axer H, Axer M, Sauer H, Witte OW, Hagemann G. Falls and gait disorders in geriatric neurology. Clin Neurol Neurosurg. 2010; 112:265-274.
Baron R, Binder A, Wasner G. Neuropathic pain: diagnosis, pathophysiological mechanisms, and treatment. Lancet Neurol. 2010;9:807-819.
Bernal B, Ardila A. The role of the arcuate fasciculus in conduction aphasia. Brain. 2009;132:2309-2316.
Campbell WW. DeJong’s The Neurologic Examination. 6th ed. Philadelphia, PA: Lippincott Williams & Wilkins; 2005.
Cattaneo L, Saccani E, De Giampaulis P, Crisi G, Pavesi G. Central facial palsy revisited: a clinical-radiological study. Ann Neurol. 2010;68:404-408.
Doty RL. The olfactory system and its disorders. Semin Neurol. 2009;29:74-81.
Erman AB, Kejner AE, Hogikyan ND, Feldman EL. Disorders of cranial nerves IX and X. Semin Neurol. 2009;29:85-92.
Faber RA. The neuropsychiatric mental status examination. Semin Neurol. 2009;29:185-193.
Fridriksson J, Kjartansson O, Morgan PS, et al. Impaired speech repetition and left parietal lobe damage. J Neurosci. 2010;30:11057-11061.
Gilchrist JM. Seventh cranial neuropathy. Semin Neurol. 2009;29:5-13.
Kerr NM, Chew SSL, Eady EK, Gamble GD, Danesh-Meyer HV. Diagnostic accuracy of confrontation visual field tests. Neurology. 2010;74:1184-1190.
LeBlond R, Brown D, DeGowin RL. DeGowin’s Diagnostic Examination. 9th ed. New York, NY: McGraw-Hill; 2009.
Lin HC, Barkhaus PE. Cranial nerve XII: the hypoglossal nerve. Semin Neurol. 2009;29:45-52.
Massey EW. Spinal accessory nerve lesions. Semin Neurol. 2009;29:82-84.
Moore FGA, Chalk C. The essential neurologic examination: what should medical students be taught? Neurology. 2009;72:2020-2023.
O’Brien M. Aids to the Examination of the Peripheral Nervous System. 5th ed. New York, NY: Saunders; 2010.
Patten J. Neurological Differential Diagnosis. 2nd ed. New York, NY: Springer Verlag; 1996.
Sanai N, Mirzadeh Z, Berger MS. Functional outcome after language mapping for glioma resection. N Engl J Med. 2008;358:18-27.
Schutta HS, Abu-Amero KK, Bosley TM. Exceptions to the Valsalva doctrine. Neurology. 2010;74:329-335.
Selhorst JB, Chen Y. The optic nerve. Semin Neurol. 2009;29:29-35.
Singerman J, Lee L. Consistency of the Babinski reflex and it variants. Eur J Neurol. 2008;15:960-964.
Snyderman D, Rovner BW. Mental status examination in primary care: a review. Am Fam Physician. 2009;80:809-814.
Waxman SG. Clinical Neuroanatomy. 26th ed. New York, NY: McGraw-Hill; 2010.