Graham Hughes1 and Shirish Sangle2
(1)
The London Lupus Centre, London Bridge Hospital, London, UK
(2)
Louise Coote Lupus Unit, St Thomas’ Hospital, London, UK
Abstract
Transverse myelitis is one of the most feared complications of Hughes Syndrome. Varying in severity from the localised, often fluctuating spinal cord features through to acute full blown paraplegia, the lesion is seen on MRI as a variable length ischaemic/inflammatory pattern (Fig. 8.1a, b).
8.1 Myelitis
Transverse myelitis is one of the most feared complications of Hughes Syndrome. Varying in severity from the localised, often fluctuating spinal cord features through to acute full blown paraplegia, the lesion is seen on MRI as a variable length ischaemic/inflammatory pattern (Fig. 8.1a, b).
Figure 8.1
(a, b) Transverse myelitis in Hughes Syndrome; high density signals (white areas shown by arrows) indicate ischaemic lesions
As with epilepsy, myelitis has long been a recognised feature of lupus, and it is still uncertain in lupus patients as to how major a part aPL plays.
Common features are bilateral leg weakness, sensory level, bladder disturbance. There may be clonus and upgoing plantars.
The pathogenesis is still uncertain. Other antibodies are also being associated with myelitis, including Devic’s disease – neuromyelitis optica.
Traditionally, at least in lupus, the emergency treatment for this potentially disastrous condition has been steroids and cyclophosphamide.
However, in view of the strong association with aPL, perhaps anticoagulation should be considered as well.
One of my ablest research fellows, Dr. Aziz Gharavi, studied a mouse model of APS. Some mice developed paraplegia with spastic back legs. Histological examination of their spinal cords revealed…….thrombosis.
8.2 Multiple Sclerosis
In view of the wide spectrum of neurological manifestations of Hughes Syndrome, it is perhaps not surprising that some cases are originally considered to have multiple sclerosis (MS).
This differential diagnosis was such a recurring theme in our patients’ histories that we carried out a standard questionnaire which included the question, “Did any of your doctors consider a diagnosis of MS in your case?”
The result was striking. Of those patients with positive aPL tests, 32% responded “yes”, compared with 8% of aPL-negative patients.
The differential diagnosis can be difficult. In our studies, the clinical overlaps were considerable and the MRI (read “blind” by neuro-radiologists) were indistinguishable.
Clearly there is a need for further studies. How commonly does Hughes Syndrome masquerade as “MS”? How many Hughes Syndrome patients are currently being treated as “MS”? Is it 1%, or 5%, or more?
Important, as the treatments are very different.
8.3 Peripheral Neuropathy
As peripheral neuropathy is relatively uncommon in lupus, we were surprised at its frequency in Hughes Syndrome.
Again the clinical severity varies widely from mild sensory neuropathy to more severe generalised disease.
Interestingly, pressure neuropathy such as carpal tunnel (and perhaps some cases of “spinal stenosis”, in aPL-positive individuals) are seen.
Perhaps there is a parallel with diabetes where partially ischaemic nerves are more vulnerable to pressure damage.
8.4 Autonomic Neuropathy
As might be expected, we have seen a series of APS patients with variable autonomic abnormalities. Perhaps we are missing milder cases.
8.5 Trigeminal Neuropathy
This appears not uncommonly in our Hughes Syndrome patients. Whether it reflects an ischaemic basis is uncertain. Cranial neuropathies were described in Sjögren’s patients many decades ago; it is also possible that Sjögren’s provides the common link.