Unilateral Cerebellar Aplasia: Report of an Asymptomatic Adult Case

We report the case of a 24-year-old woman showing an exceptional unilateral cerebellar absence in an otherwise normal Cenral Nervous System, accidentally discovered on CT and MRI. The patient underwent emergency CT scan after head injury and subsequently MRI. These examinations showed no cranio-ence...

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Bibliographic Details
Published in:Rivista di neuroradiologia 2001-12, Vol.14 (6), p.659-664
Main Authors: Scuotto, A., Accardo, C., De Martino, A., Guarracino, A.
Format: Article
Language:English
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Summary:We report the case of a 24-year-old woman showing an exceptional unilateral cerebellar absence in an otherwise normal Cenral Nervous System, accidentally discovered on CT and MRI. The patient underwent emergency CT scan after head injury and subsequently MRI. These examinations showed no cranio-encephalic traumatic lesion but revealed the absence of the right cerebellar hemisphere and inferior vermis both totally replaced by cerebrospinal fluid. Neither involvement of the brain stem nor associated supratentorial abnormality was seen. On MR angiographic sequences a mild hypoplasia of the right vertebral artery was seen; the superior cerebellar artery, anterior cerebellar artery and posteroinferior cerebellar artery were not identifiable on the right side. CT and MR findings along with anamnestic details and clinical picture suggest that the unusual cerebellar anomalies found in the present case might result from a destructive lesion, whose exact time and nature are unknown. We named this finding “aplasia” even if the most appropriate term may be “atrophia”. The presence both of a symmetrically normalsized posterior fossa and a normal-located tentorium suggests the previous development of a part of hindbrain, that was subsequently destroyed. This circumstance may even explain the absence of symptoms. In fact, a complete functional recovery may occur in response to destructive lesions resulting in atrophy, thanks to enhanced cerebellar plasticity. Selective hemispheric impairment can result from intrauterine or perinatal exposure to many agents, including hypoxic-ischemic conditions, or metabolic diseases expressed early in fetal life. However, no significant events emerged in our case. A vascular disease might also play a role because, more than the remnant factors, it could escape clinical detection. We review the classification of cerebellar abnormalities from an embryological standpoint, pointing out the role of atrophic conditions on the clinical picture.
ISSN:1971-4009
1120-9976
2385-1996
DOI:10.1177/197140090101400609