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Hemimegaloencephaly with periventricular heterotopia--case report
S Sugiyama1, M Fujii, S Nomura
1Department of Neurosurgery, Yamaguchi University School of Medicine.
Neurologia Medico-Chirurgica
|August 1, 1994
Summary
This case study highlights hemimegaloencephaly with periventricular heterotopia in a child, presenting with severe seizures. Magnetic resonance imaging proved effective in diagnosing this rare brain malformation.
Area of Science:
- Neurology
- Medical Imaging
- Pediatric Neurology
Background:
- Hemimegaloencephaly is a rare congenital brain malformation characterized by unilateral enlargement of the cerebral hemisphere.
- Periventricular heterotopia involves the abnormal migration of neurons, often leading to neurological deficits.
Observation:
- A 7-year-old girl presented with intractable seizures and cognitive impairment.
- Clinical examination revealed symptoms consistent with a significant neurological anomaly.
Findings:
- Magnetic resonance (MR) imaging demonstrated left hemispheric hypertrophy and left ventricular dilatation.
- Proton density-weighted MR imaging identified a periventricular lesion with signal intensity similar to gray matter, indicative of heterotopia.
Implications:
- This case underscores the importance of advanced neuroimaging in diagnosing complex brain malformations.
- Accurate diagnosis of hemimegaloencephaly and periventricular heterotopia is crucial for appropriate patient management and prognosis.