Grand Rounds

WHAT IS THE CAUSE OF CHOREOATHETOSIS _?


Medical Sciences Department, Pediatric Oncall, Mumbai

Address for Correspondence: Dr Ira Shah, 1, B Saguna, 271, B St Francis Road, Vile Parle {W}, Mumbai 400056. India.
Email: info@pediatriconcall.com


Clinical Problem :
A 12 years old HIV infected boy presented with sudden onset left sided choreoathetoid movements which have remained same for past 4 months. There was no fever, headache, vomiting, seizures or altered sensorium. Both parents had died due to AIDS. On examination, apart from left choreoathetosis, there were no other abnormalities. MRI brain showed multiple foci of hyperintense signals in right basal ganglia. CSF examination was normal.

What could be the cause of choreoathetosis _?


Discussion :
Basal ganglia involvement in HIV infected children can occur due to:
1. HIV virus itself
2. Granuloma – TB, Toxoplasmosis
3. Vascular infarcts due to thrombotic tendency
4. Progressive multifocal leukoencephalopathy {PML}.

In this child, CSF is normal and MRI does not show any granuloma. Thus, it is unlikely to be TB or toxoplasmosis. Also MRI does not show an infarct, thus it is unlikely to be a thrombus. PML is caused by JC virus, has a progressively downhill course unless treated with ART especially protease inhibitors {PI}. It has characteristic neuroimaging findings which are not present in this child. Also, he has remained the same for past 4 months and not worsened without treatment.

Thus, most likely, it is the HIV virus itself causing the basal ganglia affection. Thus, in an HIV infected child with extrapyramidal disease, one should consider several differential diagnosis.

Last Updated: 1st March 2010. Vol 7 Issue 3 Art No. 18

Correct Answers :  yes 100%
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