Hyper-immunoglobulin E (IgE) is a disorder of immune system characterized by assemblage of symptoms including recurrent skin and pulmonary infections, skeletal anomalies and elevated serum IgE (>2000 IU/ml). More recently, apart from the typical features, neurological manifestations have also been described including central nervous system infections, facial nerve palsy and demyelinating disorders. Here we describe three patients with hyper IgE syndrome having diverse neurological manifestation varying from CNS infection to basal ganglia stroke and progressive leukoencephalopathy.
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