Focal Seizure, White Matter Lesions, and Focal Encephalitis

Abstract

Focal seizures became generalized and were associated with intellectual deterioration over a period of at least 4 years. The focal seizures initially appeared to be a tic, and white matter lesions suggested a diagnosis of multiple sclerosis. Examination of a biopsied specimen led to diagnosis. Paulson GW, Liss L. Focal seizure, white matter lesions, and focal encephalitis. J Neuroimag 1991 ;1 :23-26At age 5 years, a boy had aseptic meningitis, and later had brief seizures in which he looked to the right. The seizures were controlled by treatment with primidone, 250 mg three times daily. His development and school performance were normal. Anticonvulsant medication was discontinued at age 13. At age 28, he had lightninglike spasms of the right side of the face and the right eye, which were called "jaw myoclonus" or a "tic." Brief episodes of dysarthria, sounding like a stutter or stammer, were associated with the jerking. Different anticonvulsants were tried without success. The boy was thought to have a variant of Tourette's syndrome, but haloperidol therapy also failed. Findings on brain computed tomography (CT), cerebral angiography, and electroencephalography (EEG) were normal. Magnetic resonance imaging (MRI) showed six high-signal, white matter lesions, the largest being deep in the left frontal lobe. Facial myokymia due to multiple sclerosis was considered; an evaluation of the cerebrospinal fluid (CSF) showed 14 oligoclonal bands and · 24 lymphocytes. The total protein content was normal, as were the results of all other lab-Diagnosis: The patient had encephalitis of the Rasmussen's syndrome diagnosed by a combination of clinical, pathological, and imaging techniques.