Infant onset subacute necrotizing encephalomyelopathy (Leigh's disease)
J Paediatr Child Health
; 29(5): 363-7, 1993 Oct.
Article
em En
| MEDLINE
| ID: mdl-8240864
ABSTRACT
We reviewed six cases of proven or probable subacute necrotizing encephalomyelopathy with an onset under 12 months of age. All children had been investigated at the Adelaide Children's Hospital in the period 1975-90. Seizures (five of six) and cortical blindness (five of six) were more prominent clinical features at presentation than the literature would suggest, while respiratory abnormalities and developmental delay were also frequent. Flash visual evoked responses, brain-stem auditory evoked responses, and the interictal electroencephalogram did not contributed to diagnosis. Computerized tomography brain scans were abnormal in three of four cases with typical basal ganglia lesions in one case and brain atrophy in two cases. The diagnosis was suspected in four cases with raised blood or cerebrospinal fluid lactate concentrations. The importance of obtaining a blood or cerebrospinal fluid lactate in all infants with unexplained seizures, cortical blindness or apnoea is emphasized.
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Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Doença de Leigh
Tipo de estudo:
Etiology_studies
Limite:
Female
/
Humans
/
Infant
/
Male
Idioma:
En
Revista:
J Paediatr Child Health
Assunto da revista:
PEDIATRIA
Ano de publicação:
1993
Tipo de documento:
Article
País de afiliação:
Austrália