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NEURORADIOLOGY Table of Contents   
Year : 2009  |  Volume : 19  |  Issue : 2  |  Page : 146-147
Unilateral cerebellar hypoplasia


1 Department of Pediatrics , Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, India
2 Department of Surgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha, India

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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.

Keywords: Cerebellum; cerebellar hypoplasia; hypoplasia

How to cite this article:
Vagh J D, Gadekar A, Agrawal A, Deshmukh K. Unilateral cerebellar hypoplasia. Indian J Radiol Imaging 2009;19:146-7

How to cite this URL:
Vagh J D, Gadekar A, Agrawal A, Deshmukh K. Unilateral cerebellar hypoplasia. Indian J Radiol Imaging [serial online] 2009 [cited 2017 Nov 22];19:146-7. Available from: http://www.ijri.org/text.asp?2009/19/2/146/50838

   Introduction Top


Unilateral cerebellar hypoplasia is a relatively rare malformation. [1],[2],[3],[4] We report an unusual case of unilateral cerebellar hypoplasia that was detected following head injury in a previously asymptomatic child.


   Case Report Top


A 7-year-old male child presented with a history of fall from a height 8 days earlier. Following the fall, the child had a nasal bleed and multiple episodes of vomiting. He had difficulty in walking and his speech was slurred, with a nasal intonation.

His past history revealed that following delivery he had been admitted in the intensive care unit because of low birth weight and respiratory distress and had recovered.

On examination he had gait ataxia, hypotonia, and slurred speech. The plantar reflexes were extensor. The deep tendon reflexes were exaggerated on the right side.

A CT scan showed hypoplasia of the left cerebellar hemisphere, a large cisterna magna, and asymmetry of the posterior fossa: the left side being smaller than the right [Figure 1]. The smaller size of the left cerebellar hemisphere could be better appreciated on MRI [Figure 2]. Based on these imaging findings, a diagnosis of unilateral cerebellar hypoplasia was made. The child was managed conservatively for the head injury and he recovered.


   Discussion Top


On the basis of MRI findings, cerebellar malformations can be divided into those associated with hypoplasia and those with dysplasia; each type can show either focal or diffuse malformations. Focal cerebellar hypoplasia can be further subdivided into isolated vermis hypoplasia or hypoplasia of one cerebellar hemisphere. [3],[4] Pathologic evidence of cerebellar injury due to birth asphyxia is well described and, because of its high metabolic activity, the vermis is the structure that is most commonly involved. [5] The clear demonstration of cerebellar hypoplasia, associated with hypoplasia or aplasia of the cerebellar or vertebral arteries, favors the concept of an intrauterine vascular etiology for cerebellar hypoplasia / aplasia. [2],[6] Genetic mutations with somatic mosaicism may also have a role to play. [7] Also, as in our case, unilateral cerebellar hypoplasia may be an incidental finding in a patient with no previous evidence of neuromuscular or metabolic disease and no past history of trauma or anoxia. [1] However, cases with unilateral cerebellar hypoplasia can present with severe grand mal seizures, persistent headache, [8] or with psychomotor retardation without cerebellar symptomatology. [2]

CT scan shows posterior fossa asymmetry with underlying unilateral cerebellar hemisphere hypoplasia. [1],[2] When available, MRI angiography may demonstrate the vascular anomalies in the cerebellar and / or vertebral arteries in most of the patients. [2] In the present case, the child's symptoms were primarily due to the head injury, which responded well to conservative management; the cerebellar hypoplasia was an incidental finding.

 
   References Top

1.Mendelsohn DB, Hertzanu Y, Glass RB, Spiro F. Unilateral cerebellar hypoplasia. J Comput Assist Tomogr 1983;7:1077-8.  Back to cited text no. 1  [PUBMED]  
2.Granados-Alzamora V, Pascual-Pascual SI, Pascual-Castroviejo I. Unilateral cerebellar hypoplasia: An alteration of vascular origin? [Article in Spanish] Rev Neurol 2003;36:841-5.   Back to cited text no. 2    
3.Patel S, Barkovich AJ. Analysis and classification of cerebellar malformations. AJNR Am J Neuroradiol 2002;23:1074-87.  Back to cited text no. 3  [PUBMED]  [FULLTEXT]
4.Poretti A, Wolf NI, Boltshauser E. Differential diagnosis of cerebellar atrophy in childhood. Eur J Paediatr Neurol 2008;12:155-67.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]
5.Le Strange E, Saeed N, Cowan FM, Edwards AD, Rutherford MA. MR imaging quantification of cerebellar growth following hypoxic-ischemic injury to the neonatal brain. AJNR Am J Neuroradiol 2004;25:463-8.  Back to cited text no. 5  [PUBMED]  [FULLTEXT]
6.Sargent MA, Poskitt KJ, Roland EH, Hill A, Hendson G. Cerebellar vermian atrophy after neonatal hypoxic-ischemic encephalopathy. AJNR Am J Neuroradiol 2004;25:1008-15.  Back to cited text no. 6  [PUBMED]  [FULLTEXT]
7.Barkovich AJ. Genetic mutations with somatic mosaicism may be another possible cause. In Pediatric Neuroimaging. Philadelphia, USA: Lippincott Williams and Wilkins; 2005. p. 390-1.  Back to cited text no. 7    
8.Simon M, Kafritsas D. Unilateral cerebellar hypoplasia. Clin Neuropathol 1992;11:71-3.  Back to cited text no. 8  [PUBMED]  

Top
Correspondence Address:
Amit Agrawal
Division of Neurosurgery, Datta Meghe Institute of Medical Sciences, Sawangi, Meghe, Wardha- 442 004, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0971-3026.50838

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    Figures

  [Figure 1], [Figure 2]

This article has been cited by
1 Fetal MRI and antenatal diagnosis of unilateral cerebellar hypoplasia
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[Pubmed] | [DOI]



 

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures

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