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      Unilateral cerebellar hypoplasia

      case-report

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          Abstract

          Unilateral cerebellar hypoplasia is a relatively rare malformation. We report the case of a 7-year-old boy who presented with a history of a fall, which was followed by cerebellar signs. Imaging findings suggested a diagnosis of unilateral cerebellar hypoplasia. The child recovered with conservative management, probably because the cerebellar signs were due to the trauma and not the hypoplasia itself.

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          Analysis and classification of cerebellar malformations.

          Because of improved visualization of posterior fossa structures with MR imaging, cerebellar malformations are recognized with increasing frequency. Herein we attempt to describe and propose a rational classification of cerebellar malformations. MR images obtained in 70 patients with cerebellar malformations were retrospectively reviewed. The cerebellar malformations were initially divided into those with hypoplasia and those with dysplasia. They were then divided into focal and diffuse malformations. Finally, they were separated according to other features, such as brain stem involvement and cerebral involvement. All patients with diffuse cerebellar dysplasia (muscular dystrophy [n = 10], cytomegalovirus [n = 6], lissencephaly [n = 3],) had abnormalities of the cerebrum. Patients with focal cerebellar dysplasia of the Joubert (n = 12) and rhombencephalosynapsis (n = 8) types had variable cerebral dysplasia. Patients with nonsyndromic focal cerebellar dysplasia (isolated focal cerebellar cortical dysplasia [n = 2], cerebellar heterotopia with cerebellar cortical dysplasia [n = 1], idiopathic diffuse cerebellar dysplasia [n = 1], Lhermitte-Duclos syndrome [n = 1]) and those with cerebellar hypoplasia (isolated cerebellar hypoplasia [n = 6], pontocerebellar hypoplasia type 1 [n = 1]) had normal cerebra. Patients with features of Dandy-Walker malformation (n = 19) had both hypoplasia and dysplasia of the cerebellum. No notable difference was found between the cerebella of patients with large fourth ventricle cysts (Dandy-Walker malformations) and those without large fourth ventricle cysts (isolated cerebellar hypoplasia). Therefore, the Dandy-Walker malformation seems to be heterogeneous. Use of this classification system helps in the segregation and understanding of the relationship among cerebellar malformations. Although it will undoubtedly require revisions, this classification is a first step in combining imaging with molecular biology to facilitate understanding of cerebellar development and maldevelopment.
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            Differential diagnosis of cerebellar atrophy in childhood.

            Starting from the imaging appearance of cerebellar atrophy (CA) we provide checklists for various groups of CA: hereditary CA, postnatally acquired CA, and unilateral CA. We also include a list of disorders with ataxia as symptom, but no evidence of CA on imaging. These checklists may be helpful in the evaluation of differential diagnosis and planning of additional investigations. However, the complete constellation of clinical (including history and neurological examination), imaging, and other information have to be considered. On the basis of a single study distinction between prenatal onset atrophy, postnatal onset atrophy, and cerebellar hypoplasia is not always possible. Apart from rare exceptions, neuroimaging findings of CA are nonspecific. A pattern-recognition approach is suggested, considering isolated (pure) CA, CA and hypomyelination, CA and progressive white matter abnormalities, CA and basal ganglia involvement, and cerebellar cortex hyperintensity.
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              MR imaging quantification of cerebellar growth following hypoxic-ischemic injury to the neonatal brain.

              Cerebellar atrophy may occur as a result of a primary injury, such as infarction or hemorrhage. Impaired growth of a non-injured cerebellum may be seen as a secondary effect related to damage in other remote but connected areas of the brain, or so-called diaschisis. We sought to determine whether perinatal hypoxic-ischemic injury leads to poor cerebellar growth and whether such impairment occurs asymmetrically in infants with predominantly unilateral brain injury. We used a computerized quantification program to measure cerebellar size by using serial MR images. Term-born infants presenting with encephalopathy and/or seizures presumed due to a hypoxic-ischemic insult within 48 hours of delivery were included if they had two or more volume acquisition images obtained at least 3 months apart but within the first 15 months of delivery. When data were grouped by MR appearances, significant differences in total cerebellum growth were seen between infants with focal infarction and those with basal ganglia and thalamic injury (P <.001). Unilateral forebrain lesions shown on MR imaging were not predictive of asymmetric cerebellar growth. Infants with focal infarction of the cerebral hemisphere had an apparently normal pattern of growth in both cerebellar hemispheres. However, in infants with severe basal ganglia and thalamic lesions, cerebellar growth was reduced, and the vermis showed little or no growth during the first year after birth.
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                Author and article information

                Journal
                Indian J Radiol Imaging
                IJRI
                The Indian Journal of Radiology & Imaging
                Medknow Publications (India )
                0971-3026
                1998-3808
                May 2009
                : 19
                : 2
                : 146-147
                Affiliations
                Department of Pediatrics, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, India
                [1 ]Department of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, India
                Author notes
                Correspondence: Dr. Amit Agrawal, Division of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha-442 004, Maharashtra, India. E-mail: dramitagrawal@ 123456gmail.com
                Article
                IJRI-19-146
                10.4103/0971-3026.50838
                2765179
                19881071
                4bf03207-e9da-40be-8c08-7abc1ea4077c
                © Indian Journal of Radiology and Imaging

                This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                Categories
                Neuroradiology

                Radiology & Imaging
                cerebellar hypoplasia,hypoplasia,cerebellum
                Radiology & Imaging
                cerebellar hypoplasia, hypoplasia, cerebellum

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