Primary angiitis of the spinal cord presenting as a conus mass: long-term remission.

BACKGROUND: Primary angiitis of the central nervous system (CNS) is a rare vasculitic disorder that typically involves the brain and, less frequently, the spinal cord without involvement of the blood vessels outside the CNS. CASE DESCRIPTION: We present a case of a 52-year-old woman who developed a conus syndrome linked to an enhancing mass of her lower thoracic spinal cord, lumbar cord, and conus. Spinal cord biopsy performed for diagnostic purposes in the setting of progressive neurological deficit confirmed angiitis of the spinal cord. Therapy with steroid and cyclophosphamide was associated with long-term (3 years) clinical and imaging remission of the lesion. CONCLUSIONS: The prognosis of primary CNS angiitis is dismal with most cases progressing to death. Long-term remission is unusual. Aggressive therapy with steroid and cytotoxic agents may improve survival.

Calgary experience with West Nile virus neurological syndrome during the late summer of 2003.

BACKGROUND: Between August 25 and September 25, 2003 seven patients with West Nile virus neurological manifestations were identified through the hospital neurology consultation services in Calgary, Alberta, Canada. Three of the seven patients were treated with interferon alpha-2b (IFN alpha-2b). In this report we document the clinical characteristics of these seven cases. METHODS: Clinical and laboratory information was obtained from a retrospective review of patient hospital and clinic charts. Patients were included if they had serological evidence of West Nile virus infection and had clinical evidence of aseptic meningitis, encephalomyelitis, cerebellar syndrome or motor neuronopathy. Three patients received a treatment course of three million units IFN alpha-2b, administered by subcutaneous injection once per day for 14 days. RESULTS: Four patients had cerebellar signs without change in consciousness, two had both encephalitis and neuromuscular weakness, and one patient had focal lower motor neuron arm weakness. The mean age was 52 (range 24 - 73). All patients had flu-like illness and fever as presenting symptoms and six had severe headaches. Two patients were immunocompromised prior to infection. Two patients with cerebellar signs (one with opsoclonus-myoclonus) improved spontaneously and exhibited only mild residual deficits on discharge. The other two patients with cerebellar findings developed brainstem involvement, one coinciding with and one subsequent to the cerebellar symptoms. Within one week of treatment with IFN alpha-2b these latter two patients showed marked improvement. One patient with encephalitis and neuromuscular weakness, was treated with IFN alpha-2b and subsequently recovered. INTERPRETATION: In this case review of seven patients, multiple neurological symptoms occurred in each patient and the neurological presentation was varied. Four patients had predominant cerebellar findings and one patient had opsoclonus-myoclonus, not previously reported. The marked improvement in three patients who received IFN alpha-2b raises preliminary optimism towards this potential treatment.