Eponym : Rasmussen syndrome.

Abstract:

: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.

journal_name

Eur J Pediatr

authors

Mastrangelo M,Mariani R,Menichella A

doi

10.1007/s00431-010-1148-0

subject

Has Abstract

pub_date

2010-08-01 00:00:00

pages

919-24

issue

8

eissn

0340-6199

issn

1432-1076

journal_volume

169

pub_type

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