Extensive Cerebral Cortical Involvement in Aquaporin 4 Antibody Positive Neuromyelitis Optica Spectrum Disorder.

Neuromyelitis optica spectrum disorders (NMOSDs) usually manifest with features of long-segment myelitis and/or optic neuritis. Area postrema involvement presents with nausea, vomiting, and intractable hiccups. Cerebral cortical involvement is a relatively uncommon phenomenon. This report describes an interesting case of aquaporin 4 antibody positive NMOSD with extensive cerebral cortical involvement in addition to area postrema and cervicomedullary lesions observed on magnetic resonance imaging. Following immunosuppressive therapy, good clinical response and near-complete resolution of brain imaging abnormalities were observed.

Pulmonary Tuberculosis with Longitudinally Extensive Transverse Myelitis.

Longitudinally extensive transverse myelitis (LETM) is described in neuromyelitis optica spectrum disorders. Simultaneous active pulmonary tuberculosis in these disorders is a relatively rare phenomenon. We report a 16 year-old boy diagnosed as LETM with clinicoradiological correlation. Further evaluation revealed active pulmonary tuberculosis. He had good recovery following the combination of antituberculosis regimen with corticosteroids.

Clinical, electrophysiological and laboratory parameters, and outcome in patients with biopsy proven systemic and nonsystemic vasculitic neuropathy.

AIM: To describe the clinical and laboratory findings of patients with biopsy proven vasculitic neuropathy. INTRODUCTION: Peripheral neuropathies form one of the most common disorders of the nervous system. However, more than 50% of them are labelled as 'idiopathic' and, therefore, treatment options become less. In this study, we tried to evaluate the phenotypic as well as laboratory characteristics and outcome of patients with biopsy proven vasculitic neuropathy. PATIENTS AND METHODS: Review of biopsy proven definite or probable vasculitic neuropathy was done. RESULTS: The cohort consisted of 67 subjects. There were 21 patients of systemic vasculitis (SVS) and 46 of non-systemic vasculitic neuropathy (NSVN). The nerve biopsy revealed definite vasculitis in 37 and probable vasculitis in 30 patients. The symptoms at onset were paraesthesia (68.7%), and paraesthesia and weakness (28.4%). Diffuse polyneuropathy occurred in 70.1% patients. The course was chronic in the majority (80.59%) of patients. Electrophysiology revealed mononeuritis multiplex in 32.84%, and polyneuropathy in 67.16% of patients. Pure sensory neuropathy was present in 16.42%. Among the patients who had undergone bilateral nerve conduction studies, the majority (71.05%) of patients had an asymmetric neuropathy. An elevated erythrocyte sedimentation rate (ESR) was observed in 80.59% (mean 71.57 ± 30.81 mm/1hr [in SVS] and 35.24 ± 21.62mm/1 hr in NSVN) of patients. The treatment included steroids, other immunomodulators, and symptomatic medications. The mean follow up was 10.98 ± 9.58 months. The outcome was good in 73.46% (43.8% with SVS and 87.88% with NSVN) patients, with those having a NSVN having a significantly better outcome. CONCLUSION: Vasculitis is a potentially treatable cause of peripheral neuropathy. The clinical features, electrophysiology, laboratory results and nerve biopsy may help in the diagnosis and categorization of patients into non-systemic and systemic vasculitic neuropathies. The long-term outcome is better in patients with NSVN compared to those with systemic vasculitis.