A 26-year-old patient of limited cutaneous sclerosis presented to us with insidious-onset posterior fossa symptoms of headache, vomiting, vertigo and gait imbalance, progressing over a period of 3 weeks. A diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids was made by combining the clinical features with radiological evidence showing punctate infiltration of the pons, brainstem and cerebellum. Relevant differentials in the form of neurosarcoid, infections, central nervous system (CNS) lymphoma and Neuro-Behcet's disease were ruled out by history and investigations. The patient responded dramatically to steroid therapy, and had no neurological deficits after 18 months of follow-up. This case highlights the rare association of a not-so-common immunological disease with a rare neurological disease.
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