RASMUSSEN´S ENCEPHALITIS. CASE REPORT.
Encefalitis de Rasmussen
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Abstract
INTRODUCTION: Rasmussen's encephalitis is a rare, chronic, progressive, inflammatory, neurodegenerative disease that affects a single cerebral hemisphere and usually appears during childhood. A case of Rasmussen's encephalitis is presented.
CASE PRESENTATION: A 4-year-old female patient presented with focal tonic-clonic seizures with loss of left hemibody strength. Patient was afebrile, conscious, oriented, but with slow cognitive development and slurred speech. Left facial and upper motor paralysis was found. Neurological examination showed complete left hemiparesis with global aphasia, muscle atrophy, spasticity, dystonia and ipsilateral Babinski's sign. MRI demonstrated diffuse atrophy of the right cerebral hemisphere with ipsilateral ventricular dilatation and prominence of the Sylvian fissure. The findings were consistent with the diagnosis of Rasmussen's encephalitis.
DISCUSSION: Rasmussen's encephalitis is characterized by progressive unilateral focal neurologic deterioration and drug-refractory focal seizures, associated with hemiparesis, cognitive dysfunction, and hemispheric atrophy. Refractory continuous partial epilepsy is the common denominator of the disease. Diagnosis is based on clinical, electroencephalographic and neuroimaging features. MRI may demonstrate focal or unihemispheric involvement, excluding other pathologies. Various immunomodulatory treatments can slow, but not stop, disease progression. The treatment of choice is surgical hemispheric disconnection to decrease neurological deficits.
CONCLUSION: Rasmussen's encephalitis is of unknown etiology. Its variable presentations make accurate diagnosis and timely treatment difficult.
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