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Primitive megalencephaly in children: natural history, medium term prognosis with special reference to external
B Laubscher1, T Deonna, A Uske
1Department of Paediatrics (Neuropaediatric Unit), Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland.
Insights
Primitive megalencephaly in children often presents at birth. Idiopathic external hydrocephalus may be a variant, potentially leading to subdural hematomas.
Area of Science:
- Pediatric Neurology
- Developmental Neuroscience
Background:
- Primitive megalencephaly is a condition characterized by an abnormally large brain.
- Understanding its association with other neurological conditions is crucial for diagnosis and management.
Observation:
- A retrospective study of 74 children with primitive megalencephaly was conducted.
- Key aspects examined included familial history, birth details, head growth, developmental outcomes, and subdural hematoma development.
Findings:
- Fifty percent of children were megalencephalic at birth.
- Developmental delay was observed in 50% of cases, transient in 18.
- Eight children (10.8%) had intellectual disability; 7 attended special schools.
- No significant differences were noted between children with normal or enlarged pericerebral subarachnoid space (SAS).
- Three children developed subdural hematomas, with some cases linked to minor trauma or spontaneous occurrence.
Implications:
- Idiopathic external hydrocephalus may represent a variant of primitive megalencephaly, possibly involving transient increased intracranial pressure.
- This condition could increase the risk of developing idiopathic subdural hematomas.
Abstract:
We studied 74 children with primitive megalencephaly retrospectively with attention directed to familial megalencephaly, birth history, enlarged pericerebral subarachnoid space (SAS) (idiopathic external hydrocephalus), head and statural growth dynamics, developmental and school prognosis, morphological findings and development of subdural haematoma. In the megalencephalic children, no significant differences were found between those with normal or those with enlarged pericerebral SAS. Out of 62, 31 children (50%) were already megalencephalic at birth. Of 74, 37 children (50%) showed variable degrees of developmental delay which in 18 was transient. Eight out of 74 were mentally retarded. Of 52 children at school age, 42 attend normal schools and 10, of whom 7 are mentally retarded, attend special schools. Three children showed subdural haematoma resulting from apparently minor trauma or occurring spontaneously. We suggest that idiopathic external hydrocephalus represents a variant of primitive megalencephaly with transient increase of intracranial pressure and that it could predispose to the development of idiopathic (spontaneous or non-traumatic) subdural haematoma.