child with cerebellar ataxia by jennyyingdi


									                                                                    The British Journal of Radiology, 81 (2008), 82–84


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

                                                                                                 Received 21 March 2006
                                                                                                 Revised 16 May 2006
                                                                                                 Accepted 14 June 2006

                                                                                                 DOI: 10.1259/bjr/14977064

                                                                                                 ’ 2008 The British Institute of

  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 1). What is
the diagnosis?

Address correspondence to: 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@

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Case of the month: A child with cerebellar ataxia

                          (a)                                                                 (b)

                          (c)                                                                 (d)

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.

The British Journal of Radiology, January 2008                                                                         83
                                                                                              S Gamanagatti and Z Nayaz

Findings                                                     show hypointense signal on T1 weighted images and
                                                             hyperintense signal on T2 weighted images. There is no
   MRI revealed bilateral symmetric hyperintensity           need for gadolinium administration. On diffusion-
involving both cerebellar grey and white matter on T2        weighted imaging, restriction of diffusion is seen in
weighted and fluid-attenuated inversion-recovery             these areas.
(FLAIR) sequences, and there was restriction of diffusion       The prognosis of acute cerebellitis is usually good. Even
on     diffusion-weighted      sequences      (Figure 1).
                                                             patients with severe symptoms and increased intracranial
Cerebrospinal fluid examination was normal. Clinical
                                                             pressure can recover completely without any sequelae.
recovery was complete after 4 days, without any specific
                                                             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
Diagnosis                                                    [5]. Death in acute cerebellitis is usually due to severe
  Acute cerebellitis                                         cerebellar swelling resulting in transtentorial and transfor-
                                                             aminal 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
  Cerebellitis is an inflammatory syndrome resulting in      normal. Although the cerebellum may show contrast
acute cerebellar dysfunction, which can occur as a           enhancement, MRI findings of acute cerebellitis are very
primary infectious, post-infectious or post-vaccination      specific and there is no need for gadolinium adminis-
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 sponta-        References
neous eye movement, truncal ataxia, wide-based gait          1. Tlili-Graiess K, Mhiri Souei M, Mlaiki B, Arifa N, Moulahi H,
and tremor associated with headache, somnolence, stiff          Jemni Gharbi H, et al. Imaging of acute cerebellitis in
neck and vomiting. Differential diagnosis includes acute        children. Report of 4 cases. J Neuroradiol 2006;33:
intoxication, demyelinating processes, vasculitis and           38–44.
myositis. Diagnosis of acute cerebellitis can be difficult   2. De Bruecker Y, Claus F, Demaerel P, Ballaux F, Sciot R,
because it may be infectious, post-infectious or post-          Lagae L, et al. MRI findings in acute cerebellitis. Eur Radiol
vaccination, and symptoms might be mild with (usually)          2004;14:1478–83.
a normal cerebrospinal fluid examination. Thus, imaging      3. Aylett SE, O’Neill KS, De Sousa C, Bitton J. Cerebellitis
                                                                presenting as acute hydrocephalus. Child Nerv Syst
plays an important role in diagnosing this condition.
  In acute cerebellitis, CT might be normal or may show      4. Asenbauer B, McConachie NS, Allcutt D, Farrell MA, King
subtle bilateral symmetrical low densities in the cere-         MD. Acute near-fatal parainfectious cerebellar swelling with
bellar hemispheres; however, these findings may be              favorable outcome. Neuropediatrics 1997;28:122–5.
overlooked [1, 2].                                           5. Levy EI, Harris AE, Omalu BI, Hamilton RL, Branstetter BF
  MRI features are classical and bilaterally symmetric.         4th, Pollack IF. Sudden death from fulminant acute
Abnormalities in the cerebellar grey and white matter           cerebellitis. Pediatr Neurosurg 2001;35:24–8.

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