Neuropathological spectrum of Rasmussen encephalitis
نویسندگان
چکیده
منابع مشابه
Neuropathological spectrum of Rasmussen encephalitis.
BACKGROUND Rasmussen encephalitis (RE) is a chronic epileptic disorder of unknown etiology, and is clinically characterized by progressive neurological deterioration, focal seizures often progressing to intractable epilepsy, cognitive decline and hemispheric atrophy. AIMS We report the spectrum of neuropathological changes seen in RE, and discuss the evolutionary mechanisms of this disease. ...
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Rasmussen encephalitis (RE) is a rare neurologic disorder of childhood characterized by unihemispheric inflammation, progressive neurologic deficits, and intractable focal epilepsy. The pathogenesis of RE is still enigmatic. Adenosine is a key endogenous signaling molecule with anticonvulsive and anti-inflammatory effects, and our previous work demonstrated that dysfunction of the adenosine kin...
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Rasmussen encephalitis is a chronic, progressive inflammation of the brain of unknown origin. Early diagnosis and treatment with immunoactive agents and/or hemispherectomy are sought to prevent the progressive cognitive decline that accompanies this disease. Combined anatomic and functional neuroimaging may serve to focus the diagnostic workup and to hasten brain biopsy for definitive diagnosis...
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Results 9 patients presented with LSCS, followed in 5 of them by progressive hemifacial atrophy (PFH) within 2 years. 3 patients presented with PFH, all 3 had additional scleroderma lesions. Their median(range) age at presentation was 8(4-17)yrs. Extracutaneous manifestations were equally found in LSCS and PFH+LSCS patients. They comprised asymmetry of tooth arches/missing teeth(1), ophthalmolo...
متن کاملLimited chronic focal encephalitis: another variant of Rasmussen syndrome?
OBJECTIVE To describe a more limited and less malignant form of Rasmussen encephalitis (RE). METHODS Three subjects (all women; 37, 31, and 32 years of age) developed childhood or late onset chronic focal encephalitis, with a relatively nonprogressive form of the disorder. RESULTS In our patients, clinical features were dominated by partial seizures without marked focal motor deficit and in...
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ژورنال
عنوان ژورنال: Neurology India
سال: 2005
ISSN: 0028-3886
DOI: 10.4103/0028-3886.16396