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Descritor Inglês:   Diffuse Cerebral Sclerosis of Schilder 
Descritor Espanhol:   Esclerosis Cerebral Difusa de Schilder 
Descritor Português:   Esclerose Cerebral Difusa de Schilder 
Sinônimos Inglês:   Alper Disease
Alper Syndrome
Alper's Disease
Alper's Syndrome
Alpers Diffuse Degeneration of Cerebral Gray Matter with Hepatic Cirrhosis
Alpers Disease
Alpers Huttenlocher Syndrome
Alpers Progressive Infantile Poliodystrophy
Alpers Syndrome
Alpers' Disease
Alpers' Syndrome
Alpers-Huttenlocher Syndrome
Balo Concentric Sclerosis
Balo's Concentric Sclerosis
Cerebral Sclerosis, Diffuse
Concentric Sclerosis, Balo
Concentric Sclerosis, Balo's
Diffuse Cerebral Scleroses
Diffuse Cerebral Sclerosis
Diffuse Scleroses, Myelinoclastic
Diffuse Sclerosis, Myelinoclastic
Disease, Alpers'
Disease, Schilder
Disease, Schilder's
Encephalitis Periaxialis
Encephalitis Periaxialis Concentrica
Encephalitis Periaxialis Diffusa
Myelinoclastic Diffuse Scleroses
Myelinoclastic Diffuse Sclerosis
Neuronal Degeneration Of Childhood With Liver Disease, Progressive
Poliodystrophia Cerebri
Progressive Neuronal Degeneration of Childhood with Liver Disease
Progressive Sclerosing Poliodystrophies
Progressive Sclerosing Poliodystrophy
Schilder Disease
Schilder's Disease
Schilders Disease
Scleroses, Balo's Concentric
Scleroses, Myelinoclastic Diffuse
Sclerosis, Diffuse Cerebral
Sclerosis, Myelinoclastic Diffuse
Sudanophilic Cerebral Sclerosis
Syndrome, Alpers
Syndrome, Alpers-Huttenlocher  
Categoria:   C10.114.375.112
Definição Inglês:   A rare central nervous system demyelinating condition affecting children and young adults. Pathologic findings include a large, sharply defined, asymmetric focus of myelin destruction that may involve an entire lobe or cerebral hemisphere. The clinical course tends to be progressive and includes dementia, cortical blindness, cortical deafness, spastic hemiplegia, and pseudobulbar palsy. Concentric sclerosis of Balo is differentiated from diffuse cerebral sclerosis of Schilder by the pathologic finding of alternating bands of destruction and preservation of myelin in concentric rings. Alpers' Syndrome refers to a heterogeneous group of diseases that feature progressive cerebral deterioration and liver disease. (From Adams et al., Principles of Neurology, 6th ed, p914; Dev Neurosci 1991;13(4-5):267-73) 
Nota Histórica Inglês:   2000(1963) 
Qualificadores Permitidos Inglês:  
BL blood CF cerebrospinal fluid
CI chemically induced CL classification
CO complications CN congenital
DI diagnosis DG diagnostic imaging
DH diet therapy DT drug therapy
EC economics EM embryology
EN enzymology EP epidemiology
EH ethnology ET etiology
GE genetics HI history
IM immunology ME metabolism
MI microbiology MO mortality
NU nursing PS parasitology
PA pathology PP physiopathology
PC prevention & control PX psychology
RT radiotherapy RH rehabilitation
SU surgery TH therapy
UR urine VE veterinary
VI virology  
Número do Registro:   2589 
Identificador Único:   D002549 

Ocorrência na BVS: