Rasmussen’s encephalitis

Encefalitis de Rasmussen

Introduction: Rasmussen´s encephalitis (RE) is a rare, chronic, progressive, inflammatory, neurodegenerative disease affecting only one cerebral hemisphere and usually appearing during childhood. Case presentation: 4-year-old female patient with focal tonic-clonic seizures and loss of strength on left side of the body, afebrile, conscious, oriented, cognitive impairment, and slurred speech. Upon physical exam, left facial and upper motor paralysis, entire left hemiparesis, as well as, global aphasia, muscle atrophy, spasticity, hemidystonia and ipsilateral positive Babinski sign, were found. MRI showed right cerebral hemisphere diffuse atrophy with ipsilateral ventricle enlargement and prominence of the Sylvian fissure, suggestive of Rasmussen´s encephalitis. Discussion: the disease is characterized by progressive unilateral focal neurological deterioration, drug-refractory focal seizures, hemiparesis, cognitive impairment, and hemispheric atrophy. Refractory epilepsia partialis continua is the common denominator. Diagnosis is based on clinical, electroencephalographic and neuroimaging findings. MRI may demonstrate focal or unilateral hemispheric involvement. Several immunomodulatory treatments can slow but not halt progression, the treatment of choice being surgical hemispherectomy aimed to reduce neurological deficits. Conclusion: Rasmussen´s encephalitis accurate diagnosis and timely treatment is hindered by its unknown underlying cause and variable clinical features.