1/2. Acute necrotizing encephalopathy: combined therapy and favorable outcome in a new case.BACKGROUND: Acute necrotizing encephalopathy (ANE) is a rare disease characterized by multiple, symmetrical brain lesions, affecting thalami, brainstem tegmentum, and cerebellar medulla; more inconstantly, other structures are involved, i.e., internal capsules, posterolateral putamen, and deep periventricular white matter. FEATURES: The clinical picture consists of rapidly deteriorating acute monophasic encephalopathy preceded by prodromal febrile illness; the symptoms include hyperpyrexia, convulsions, recurrent vomiting, and coma within 24 h. prognosis: The outcome is usually poor and approximately 70% of the patients die within a few days from the onset of fever. There is no specific therapy for ANE but, in some patients, the clinical status improved with steroid treatment.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |
2/2. Acute necrotizing encephalopathy of childhood (infantile bilateral thalamic necrosis): two non-Japanese cases.This report describes two Spanish cases of acute necrotizing encephalopathy of childhood, a rare disease first described in japan by Mizuguchi and colleagues. Similar cases have been reported from taiwan and other countries of the far east. Two cases have been reported from the UK and one from the USA. The disease affects young children and is characterized by acute encephalopathy after a viral illness, with seizures and decreased levels of consciousness. The hallmark of the disease is diffuse and symmetrical CNS lesions of both thalami, internal capsules, upper brainstem tegmentum, and cerebral white matter. The aetiology is unknown but an infectious or parainfectious process seems likely. Because of the predominant involvement of the thalami, we propose the term 'infantile bilateral thalamic necrosis', a more specific term and one which distinguishes the entity from other basal ganglia diseases in childhood.- - - - - - - - - - ranking = 1keywords = rare disease (Clic here for more details about this article) |