Eponym : Rasmussen syndrome.
Rasmussen's syndrome (RS) is a rare acquired progressive inflammatory encephalopathy characterized by drug-resistant partial seizures and cognitive deterioration resulting from a gradual impairment and a subsequent atrophy of a single brain hemisphere. It was firstly described by Theodore Rasmussen in 1958. The original etiopathogenic hypothesis of a chronic viral infection no longer holds. Today, the presumed mechanism is a complex autoimmune process comprising an active role of both cytotoxic T lymphocytes and circulating autoantibodies, activating the subunit 3 of the ionotropic glutamate receptor (GluR3Ab). Several medical options, such as antiepileptic drugs and immunomodulatory therapies, deserve consideration to face epileptic manifestations and to slow neurologic regression. Yet, all these therapies turn out to be almost always just temporizing measures. Surgical disconnection of the affected hemisphere ("functional hemispherectomy") is the best therapeutic choice to achieve the arrest of the disease, a good seizure control, the block of neurologic decline, and the improvement of patient's quality of life.
Mastrangelo
Mario
M
Child Neurology Division, Department of Pediatrics, La Sapienza-University of Rome, Viale Regina Elena, 324 00161 Rome, Italy. mari0mastrangelo@tiscali.it
Mariani
Rosanna
R
Menichella
Alessandra
A
D000927:Anticonvulsants / Q000627:therapeutic use*; D001323:Autoantibodies / Q000097:blood; D004569:Electroencephalography; D004660:Encephalitis / Q000188:drug therapy* / Q000276:immunology / Q000601:surgery*; D004850:Eponyms; D038421:Hemispherectomy / Q000379:methods; D006801:Humans; D056747:Immunomodulation; D018091:Receptors, AMPA / Q000276:immunology; D012640:Seizures / Q000517:prevention & control*; D013602:T-Lymphocytes, Cytotoxic / Q000276:immunology; D016896:Treatment Outcome
D000927:Anticonvulsants; D001323:Autoantibodies; D018091:Receptors, AMPA; C104723:glutamate receptor ionotropic, AMPA 3