Slurred speech with walking difficulty

 
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Medical Sciences Department, Pediatric Oncall, Mumbai

Address for Correspondence: Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056.


Clinical Problem :
Case Report: - A 6 year old girl born of non consanguineous marriage presented with gradual difficulty in getting up and difficulty in walking. There was no fever, altered sensorium, change in higher functions or cranial nerve involvement. There was no contact with TB. On examination she had sutural separation with positive Macewan’s sign. There were no focal neurological deficit but she had hypertonia with brisk reflexes and bilateral extensor planters. She had wide based gait and slurred speech. Fundus showed blurred disc margins.

Investigations showed:
• CBC = Normal
• ESR = 95 mm at end of 1 hour
• CSF = 7 cells {100percent lymphocytes} with normal sugar and proteins.
• HIV = Negative
• Chest X-Ray = Normal


Question :
What is the diagnosis_?

Expert Opinion :
This child has presented with gradual spasticity suggestive of pyramidal tract involvement and has signs of raised ICT in form of blurred optic discs. The commonest cause of pyramidal tract involvement without delayed development is hydrocephalus. Other causes that come with predominantly pyramidal tract involvement are white matter disease such as Metaleukodystrophy and Krabbe’s and also adrenoleukodystrophy {X-web addressed} but they also have neuroregression.
This child also has cerebellar signs and thus involvement of cerebellum. Hence, likely diagnosis in this child would be cerebellar space occupying lesion with obstructive hydrocephalus and raised ICT.
Cerebellar astrocytoma and tuberculoma are common cause of cerebellar SOL. However, with CSF showing 100percent lymphocytes, this child would be more likely to have tuberculoma. MRI showed cerebellar tuberculoma with obstructive hydrocephalus that was treated with antituberculous therapy, shunt surgery and steroids.

E-published April 2009, Vol 6 Issue 4 Art No. 20


Correct Answers :  yes 98%
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