Cerebellar cleft: a form of prenatal cerebellar disruption.
In contrast to malformations, cerebellar disruptions have attracted little interest in the literature. We draw attention for the first time to the hypothesis that cerebellar clefts are residual changes following a prenatal cerebellar insult, and represent disruptions. We reviewed the clinical records and MR findings of six patients with a cerebellar cleft, two of whom also had prenatal MRI at 24 weeks of gestation. The clefts were located in the left cerebellar hemisphere in five cases, in the right in one patient. Other typical findings included disorderly alignment of the cerebellar folia and fissures, irregular gray/white matter junction, and abnormal arborization of the white matter in all patients. The cerebellar cleft extended into the fourth ventricle in three cases, and in two children cystic cortical lesions were seen. Supratentorial schizencephaly was found in two patients. In two patients there was a documented fetal cerebellar hemorrhage at 24 weeks of gestation. We conclude that cerebellar clefts are residual changes resulting from a prenatal cerebellar insult and consequently represent disruptions rather than primary malformations. The supratentorial findings are also in agreement with an acquired lesion. The outcome in these children was variable, mainly depending of the presence of supratentorial lesions.
Poretti
A
A
Department of Neurology, University Children's Hospital of Zurich, Zurich, Switzerland.
Leventer
R J
RJ
Cowan
F M
FM
Rutherford
M A
MA
Steinlin
M
M
Klein
A
A
Scheer
I
I
Huisman
T A G M
TA
Boltshauser
E
E
D002531:Cerebellum / Q000002:abnormalities*; D002648:Child; D002675:Child, Preschool; D004108:Dilatation, Pathologic / Q000175:diagnosis / Q000209:etiology*; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D005865:Gestational Age; D006801:Humans; D007091:Image Processing, Computer-Assisted; D007223:Infant; D020300:Intracranial Hemorrhages / Q000150:complications / Q000473:pathology; D008279:Magnetic Resonance Imaging; D008297:Male; D054220:Malformations of Cortical Development / Q000150:complications / Q000473:pathology; D011247:Pregnancy; D011296:Prenatal Diagnosis / Q000379:methods*