Presentation
Delayed mental and motor milestones.
Patient Data
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Left cerebellar hemispehere and inferior vermis aplasia with hypoplastic dysplastic right cerebellar hemisphere and superior cerebellar vermis.
Hypoplastic pons with an elongated rostrocaudal length of the ventral pons
Small asymmteric middle and superior cerebellar peduncles.
Loading images...
Left cerebellar hemispehere and inferior vermis aplasia with hypoplastic dysplastic right cerebellar hemisphere and superior cerebellar vermis.
Hypoplastic pons with an elongated rostrocaudal length of the ventral pons
Small asymmteric middle and superior cerebellar peduncles.
Bilateral cerebral subcortical small hypomylination patches of abnormal signal.
Case Discussion
Pontocerebellar hypoplasia is a rare autosomal recessive neurodegenerative disease that implies a reduced volume of pons and cerebellum (not necessarily affecting the brainstem and cerebellum equally).
The midsagittal view is particularly important for assessing brainstem morphology, vermian size and lobulation, and some supratentorial anomalies, e.g. agenesis/dysgenesis of the corpus callosum, reduced white matter volume, and a simplified gyral pattern.
This case is courtesy of Dr. Tamer El Bakry. MD of Radiodiagnosis
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