Cerebellar Cleft: Confirmation of the Neuroimaging Pattern

Abstract

We recently described the neuroimaging and clinical findings in 6 children with cerebellar clefts and proposed that they result from disruptive changes following prenatal cerebellar hemorrhage. We now report an additional series of 9 patients analyzing the clinical and neuroimaging findings. The clefts were located in the left cerebellar hemisphere in 5 cases, in the right in 3, and bilaterally in one child who had bilateral cerebellar hemorrhages as a preterm infant at 30 weeks gestation. In one patient born at 24 weeks of gestation a unilateral cerebellar hemorrhage has been found at the age of 4 months. Other findings included disordered alignment of the folia and fissures, an irregular gray/white matter junction, and abnormal arborization of the white matter in all cases. Supratentorial abnormalities were found in 4 cases. All but 2 patients were born at term. We confirm the distinct neuroimaging pattern of cerebellar clefts. Considering the documented fetal cerebellar hemorrhage in our first series, we postulate that cerebellar clefts usually represent residual disruptive changes after a prenatal cerebellar hemorrhage. Exceptionally, as now documented in 2 patients, cerebellar clefts can be found after neonatal cerebellar hemorrhages in preterm infants. The short-term outcome in these children was variable.

cerebellar cleft - cerebellar hemorrhage - cerebellar disruption

References

• 1 Barkovich AJ, Kjos BO. Gray matter heterotopias: MR characteristics and correlation with developmental and neurologic manifestations.  Radiology. 1992;  182 493-499
• 2 Barkovich AJ, Kuzniecky RI, Jackson GD. et al . A developmental and genetic classification for malformations of cortical development.  Neurology. 2005;  65 1873-1887
• 3 Barkovich AJ, Millen KJ, Dobyns WB. A developmental and genetic classification of midbrain-hindrain malformations.  Brain. 2009;  132 3199-3230
• 4 Battaglia G, Bassanini S, Granata T. et al . The genesis of epileptogenic cerebral heterotopia: clues from experimental models.  Epileptic Disord. 2003;  5 (S 02) 51-58
• 5 Battaglia G, Pagliardini S, Saglietti L. et al . Neurogenesis in cerebral heterotopia induced in rats by prenatal methylazoxymethanol treatment.  Cereb Cortex. 2003;  13 736-748
• 6 Boltshauser E, Steinlin M, Martin E. et al . Unilateral cerebellar aplasia.  Neuropediatrics. 1996;  27 50-53
• 7 Boltshauser E, Schneider J, Kollias S. et al . Vanishing cerebellum in myelomeningocoele.  Eur J Paediatr Neurol. 2002;  6 109-113
• 8 Boltshauser E. Cerebellum-small brain but large confusion: a review of selected cerebellar malformations and disruptions.  Am J Med Genet. 2004;  126 376-385
• 9 Clement E, Mercuri E, Godfrey C. et al . Brain involvement in muscular dystrophies with defective dystroglycan glycosylation.  Ann Neurol. 2008;  64 573-582
• 10 Curry CJ, Lammer EJ, Nelson V. et al . Schizencephaly: heterogeneous etiologies in a population of 4 million California births.  Am J Med Genet A. 2005;  137 181-189
• 11 Demaerel P, Lagae L, Casaer P. et al . MR of cerebellar cortical dysplasia.  AJNR Am J Neuroradiol. 1998;  19 984-986
• 12 Demaerel P. Abnormalities of cerebellar foliation and fissuration: classification, neurogenetics and clinicoradiological correlations.  Neuroradiology. 2002;  44 639-646
• 13 Limperopoulos C, Benson CB, Bassan H. et al . Cerebellar hemorrhage in the preterm infant: ultrasonographic findings and risk factors.  Pediatrics. 2005;  116 717-724
• 14 Maeda T, Akaishi M, Shimizu M. et al . The subclassification of schizencephaly and its clinical characterization.  Brain Dev. 2009;  31 694-701
• 15 ManciniJ, Lethel V, Hugonenq C. et al . Brain injuries in early foetal life: consequences for brain development.  Dev Med Child Neurol. 2001;  43 52-55
• 16 Mercuri E, He J, Curati WL. et al . Cerebellar infarction and atrophy in infants and children with a history of premature birth.  Pediatr Radiol. 1997;  27 139-143
• 17 Poretti A, Leventer RJ, Cowan FM. et al . Cerebellar cleft: a form of prenatal cerebellar disruption.  Neuropediatrics. 2008;  39 106-112
• 18 Poretti A, Prayer D, Boltshauser E. Morphological spectrum of prenatal cerebellar disruptions.  Eur J Paediatr Neurol. 2009;  13 397-407
• 19 Poretti A, Limperopoulos C, Roulet-Perez E. et al . Outcome of severe unilateral cerebellar hypoplasia.  Dev Med Child Neurol. 2009;  epub Oct 23
• 20 Sehgal A, El-Naggar W, Glanc P. et al . Risk factors and ultrasonographic profile of posterior fossa haemorrhages in preterm infants.  J Paediatr Child Health. 2009;  45 215-218
• 21 Soto-Ares G, Delmaire C, Deries B. et al . Cerebellar cortical dysplasia: MR findings in a complex entity.  AJNR Am J Neuroradiol. 2000;  21 1511-1519
• 22 Soto-Ares G, Devisme L, Jorriot S. et al . Neuropathologic and MR imaging correlation in a neonatal case of cerebellar cortical dysplasia.  AJNR Am J Neuroradiol. 2002;  23 1101-1104
• 23 Steggerda SJ, Leijser LM, Wiggers-de Bruine FT. et al . Cerebellar injury in preterm infants: incidence and findings on US and MR Images.  Radiology. 2009;  252 190-199
• 24 van der Knaap MS, Vermeulen G, Barkhof F. et al . Pattern of white matter abnormalities at MR imaging: use of polymerase chain reaction testing of Guthrie cards to link pattern with congenital cytomegalovirus infection.  Radiology. 2004;  230 529-536

Correspondence