SEVERE TRANSITORY ENCEPHALOPATHY WITH REVERSIBLE LESIONS OF THE CLAUSTRUM

Reversible bilateral lesions of the claustrum and external capsule in a 12-year-old girl suffering from a severe, transitory encephalopathy are reported. After a prodromal stage of feeling uncomfortable a sudde n onset of status epilepticus occurred, followed by recurrent complex partial and myoclonic seizures for 3 weeks, with psychotic symptoms an d temporary loss of vision, speech and hearing, After treatment with p henytoin the patient became free of seizures and recovered completely without neurological deficit. The initial cranial CT was normal; howev er, cranial MRI7 days later showed bilateral selective lesions of the claustrum and external capsule, which disappeared completely 5 weeks l ater. The aetiology of these lesions remains obscure; repeated cerebro spinal fluid and blood tests were negative for herpes simplex virus an d other infectious agents. The clinical and radiological improvement w ere concomitant. This may indicate a functional disturbance of the cla ustrum grey matter, rather than lesions of the white matter of the ext ernal and extreme capsules.