Case 3

A 53-year-old woman developed subacute tetraplegia. Brain and spinal MRI was normal and CSF acellular. She made a partial recovery over 6 months after intravenous high-dose corticosteroids. Eighteen months later, she developed severe bilateral sequential optic neuritis and a left hemiplegia. Limb weakness partially improved, and her vision remained poor despite a further course of corticosteroids. Repeat MRI showed two infratentorial white matter lesions. MRI cervical cord showed an extensive cervical cord lesion extending over three levels. VEPs were delayed asymmetrically. She had unmatched CSF oligoclonal bands in addition to matched bands in the serum and CSF. She had a past history of two first-trimester miscarriages, an unprovoked ileofemoral deep vein thrombosis (DVT) for which she was on long-term warfarin, migraine, and one positive antiphospholipid antibody result. Her antineu-trophil antibody (ANA) was raised without more specific lupus autoantibodies. Three months on, she presented with a right hemiplegia and a left optic neuritis, with no further recovery after 2 months. Partial improvement followed a course of plasma exchange. The patient subsequently developed keratoconjunctivitis sicca, xerostomia, and a photosensitive rash. Anti-Ro and anti-La antibodies and a Schirmer test were positive. A minor salivary gland biopsy confirmed Sjögren syndrome, and she was started on a pulsed cyclophosphamide regime.

Over the next 2 years, she had no further relapses and made a partial but slow recovery.

Discussion

• The initial differential included Devic's disease and antiphospholipid (APL) syndrome. CSF-specific oligoclonal bands, an acellular CSF, and brain MRI lesions are not typical in Devic's disease.

• Livedo reticularis, thrombocytopenia, and a repeat positive antiphospholipid antibody would have provided further support for a diagnosis of APL syndrome, but CSF oligoclon-al bands would be unusual.

• Cervical cord lesions extending over more than one vertebral body length would not be expected in MS. Matched bands in the serum and CSF and poor clinical recovery after an attack are also against an optico-spinal presentation of MS.

• A number of other conditions may present with a Devic's-type clinical picture including Sjögren syndrome, sarcoidosis, Behcet disease, SLE, and ADEM. Review of the history, longitudinal follow-up, and additional tests should be considered in all cases with atypical features.

Behget Disease (BD) The prevalence of BD is high in the Mediterranean basin and Japan, and it has been linked with the human leucocyte antigen B51 in these countries. Neurologic involvement occurs in approximately 5% of patients. Thrombophlebitic involvement can lead to cerebral venous thrombosis, but it is the parenchymal CNS involvement that can occasionally mimic MS. This is usually characterized by a relapsing brainstem syndrome and may be accompanied by sphincter disturbance and cognitive-behavioral changes. Optic neuritis and progressive transverse myelitis also is described. Diagnosis is made by highlighting a history of recurrent oral ulceration (greater than three episodes per year) in association with a combination of genital ulceration, skin lesions (typically papulopustular lesions, pseudofolli-culitis, or erythema nodosum), pathergy, and uveitis (Figure 4.5). Relapses may be associated with fever, headache, and a raised erythro-cyte sedimentation rate (ESR), all of which should prompt the search for a systemic disorder. MRI scanning usually shows characteristic brainstem and basal ganglia involvement, and the CSF is usually pleocystic with a slightly raised protein level. Oligoclonal bands are reported to be less common but can occur. A combination of immunosuppression usually is advocated, although a monophasic presentation is recognized.

Neurosarcoidosis The nervous system is affected in about 5% of patients with sarcoid disease and may rarely be the presenting and only feature. A steroid-responsive optic neuritis, brainstem disorder, or spinal cord disease may mimic MS. Cranial nerves, in particular the optic and facial nerves, commonly are affected. Sarcoid disease also may cause a meningitic-radicular syndrome, a brain-stem-cerebellar syndrome, cognitive deterioration, and hypopituitarism. The diagnosis is suggested by associated meningeal, psychiatric, peripheral nerve, and muscle involvement and the presence of systemic disease. Cranial MRI may show multiple white matter lesions, but the presence of meningeal enhancement is helpful (when present) in distinguishing neurosarcoidosis from MS. The CSF often shows a significantly raised protein and cell count in active disease. CSF-specific oligoclonal bands are not usual. Data presented in abstract form (see references) from the National Hospital for Neurological Diseases found CSF-specific bands in only 5% of biopsy-proven cases. Serum and CSF angiotensin converting enzyme (ACE) levels do not routinely appear sensitive or specific. Subclinical hilar lymphadenopathy should be sought with a chest radiograph and, if possible, with high resolution computed tomography (CT). Abnormal respiratory function tests, particularly reduced transfer factor, are found in most cases of systemic sarcoidosis. 67Gallium single-photon emission computed tomography (SPECT) scanning may show characteristic tracer uptake in salivary and lacrimal glands, chest and spleen; a positive Kveim test, if available, may provide addi

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