Non-convulsive status epilepticus associated with neuronal intranuclear inclusion disease: A case report and literature review

We report a case of neuronal intranuclear inclusion disease (NIID) confirmed by detection of intranuclear inclusions in a skin biopsy specimen. Brain magnetic resonance imaging showed mild cerebral atrophy and linear hyperintensities at the corticomedullary junction on diffusion-weighted images. This patient developed non-convulsive status epilepticus with generalized periodic discharge on electroencephalography after recurrent symptoms of paroxysmal nausea and slowly progressive cognitive decline. There have been no previous reports of NIID with non-convulsive status epilepticus. Since adult patients with NIID display a wide variety of clinical manifestations, skin biopsy should be recommended in patients who have leukoencephalopathy of unknown origin.
Source: Epilepsy and Behavior Case Reports - Category: Neurology Source Type: research