Neuropediatrics 2006; 37 - THP159
DOI: 10.1055/s-2006-945982

HEMIMEGACEREBELLUM: A REPORT OF ONE CASE

C Cieuta-Walti 1, É Lavoie 1, Y Patenaude 1, B Echenne 1, G Sebire 1
  • 1Université de Médecine de Sherbrooke, Sherbrooke, QC, Canada

Objectives: We report clinical and paraclinical features of a patient with an original unilateral hemispheric malformation of cerebellum.

Methods: Clinical features, neuropsychological testing, neuroradiological, electrophysiological, metabolic and genetic investigations were performed.

Results: This girl is the only child of non consanguineous French Canadian parents. The first neurological examination was performed when she was 2.5 years old and showed a global psychomotor delay (Denver test; DQ=60%). She started to walk at 23 months. Ataxic gait was then observed. She was dysarthric. At the age of 3.5 years, speech remained immature (phonology, syntax). A non-progressive macrocephaly was noticed. Brain MRI at 2.5 years showed a diffusely prominent left cerebellar hemisphere. EEG, genetic and metabolic studies were normal. Brain MRI of the parents was normal.

Conclusion: We reported for the first time to our knowledge, an hemispheric cerebellar dysplasia characterized by an “ hemimegacerebellum ” appearance in brain MRI investigation.