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British Journal of Radiology (2008) 81, 82-84
© 2008 British Institute of Radiology
doi: 10.1259/bjr/14977064

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Case of the month

A child with cerebellar ataxia

S Gamanagatti, MD and Z Nayaz, MD

Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi-110029, India

Correspondence: Dr Shivanand Gamanagatti, Assistant Professor, c/o Vijay Kumar Uppal, 198/58 East of Kailash, Uppal's House, Ramesh market, New Delhi-110065. E-mail: shiv223{at}rediffmail.com


    Introduction
 Top
 Introduction
 Findings
 References
 
A 7-year-old boy presented with unsteadiness and wide-based gait, headache and vomiting that had lasted for 6 days. There was no history of fever, sore throat or vaccination. Neurological examination was normal except for gait disturbances. A CT scan of the head was normal; MRI was also performed (Figure 1Go). What is the diagnosis?


Figure 1
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Figure 1. MR images showing(a) bilateral symmetric hypointensity on T1 weighted sequences and hyperintensity on (b) T2 weighted and (c) fluid-attenuated inversion recovery (FLAIR) sequences, involving both cerebellar grey and white matter. (d) There was restriction of diffusion on diffusion-weighted sequences.

 

    Findings
 Top
 Introduction
 Findings
 References
 
MRI revealed bilateral symmetric hyperintensity involving both cerebellar grey and white matter on T2 weighted and fluid-attenuated inversion-recovery (FLAIR) sequences, and there was restriction of diffusion on diffusion-weighted sequences (Figure 1Go). Cerebrospinal fluid examination was normal. Clinical recovery was complete after 4 days, without any specific treatment.

Diagnosis
Acute cerebellitis

Discussion
Cerebellitis is an inflammatory syndrome resulting in acute cerebellar dysfunction, which can occur as a primary infectious, post-infectious or post-vaccination disorder; it may also occur without evidence of an antecedent or concurrent factor. Patients might be almost asymptomatic; however, they typically present with mild to moderate cerebellar signs such as abnormal spontaneous eye movement, truncal ataxia, wide-based gait and tremor associated with headache, somnolence, stiff neck and vomiting. Differential diagnosis includes acute intoxication, demyelinating processes, vasculitis and myositis. Diagnosis of acute cerebellitis can be difficult because it may be infectious, post-infectious or post-vaccination, and symptoms might be mild with (usually) a normal cerebrospinal fluid examination. Thus, imaging plays an important role in diagnosing this condition.

In acute cerebellitis, CT might be normal or may show subtle bilateral symmetrical low densities in the cerebellar hemispheres; however, these findings may be overlooked [1, 2].

MRI features are classical and bilaterally symmetric. Abnormalities in the cerebellar grey and white matter show hypointense signal on T1 weighted images and hyperintense signal on T2 weighted images. There is no need for gadolinium administration. On diffusion-weighted imaging, restriction of diffusion is seen in these areas.

The prognosis of acute cerebellitis is usually good. Even patients with severe symptoms and increased intracranial pressure can recover completely without any sequelae. Steroids are the first line of treatment when symptoms are moderate to severe; however, most patients will recover without steroids or any specific treatment [3, 4]. Sudden deaths have been reported following fulminant cerebellitis [5]. Death in acute cerebellitis is usually due to severe cerebellar swelling resulting in transtentorial and transforaminal herniations.

We conclude that MRI is an important tool in the diagnosis of acute cerebellitis, especially for cases in which the symptoms are mild and cerebrospinal fluid is normal. Although the cerebellum may show contrast enhancement, MRI findings of acute cerebellitis are very specific and there is no need for gadolinium administration.

Received for publication March 21, 2006. Revision received May 16, 2006. Accepted for publication June 14, 2006.


    References
 Top
 Introduction
 Findings
 References
 

  1. Tlili-Graiess K, Mhiri Souei M, Mlaiki B, Arifa N, Moulahi H, Jemni Gharbi H, et al. Imaging of acute cerebellitis in children. Report of 4 cases. J Neuroradiol 2006;33:38–44.[Medline]
  2. De Bruecker Y, Claus F, Demaerel P, Ballaux F, Sciot R, Lagae L, et al. MRI findings in acute cerebellitis. Eur Radiol 2004;14:1478–83.[Medline]
  3. Aylett SE, O'Neill KS, De Sousa C, Bitton J. Cerebellitis presenting as acute hydrocephalus. Child Nerv Syst 1998;14:139–41.[CrossRef][Medline]
  4. Asenbauer B, McConachie NS, Allcutt D, Farrell MA, King MD. Acute near-fatal parainfectious cerebellar swelling with favorable outcome. Neuropediatrics 1997;28:122–5.[Medline]
  5. Levy EI, Harris AE, Omalu BI, Hamilton RL, Branstetter BF 4th, Pollack IF. Sudden death from fulminant acute cerebellitis. Pediatr Neurosurg 2001;35:24–8.[CrossRef][Medline]




This Article
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