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? chorea in HIV

By Erin Meier | 06 Feb, 2013

I know this is about HIV prevention, but didn't know if there were people out there with more HIV experience than I who might be able to help. I have a 25 yo female who was recently diagnosed with HIV and has a 3 month history of chorea of her L arm. No other symptoms or manifestations of rheumatic fever. Husband reports some confusion in the past 2 months, but on exam was AAOx3, answered all questions appropriately, showed no signs of confusion or dementia. CD4 is still pending. Has anyone seen chorea in any specific HIV OIs? I can only do basic labs, LP and basic X-ray - nothing fancy.

Any thoughts are appreciated.

Replies

 

Francoise LOUIS Replied at 8:12 AM, 6 Feb 2013

Hi Erin
I have never seen such a presentation during my HIV clinical practice
Is you patient having fever? violent headache? I guess the rest of the neurological C/E is without other sign.
We could think of
1) a PML because of the episode of confusion, but it actually subsided
2) Any other involving a hemipallidum
We also need not to forget the diseases that are not HIV related:
1) Genetic (we need to get a family history); in your context you will only be able to know whether it is familial or not (Huntington, Wilson (check the liver functions but there is no cure other than transplantation....), Neuroacanthosis (no comment)
2) Infectious: CJD, therefore no available treatment
3) drug induced (L dopa, anticonvulsivants, antipsychotics especially the dopaminergic ones, which if it is the case, is the only etiology for which there is a solution (to reduce the drug dosing or to stop the drug)
4) RF (you have checked, but there are a lot of faalse positive and negatives with this test)- any episode of tonsilitis followed by arthralgia etc etx?
5) pregnancy (check the LMP)
I wish it helps somehow. Good luck to you and your patient.
http://emedicine.medscape.com/article/1149854-overview#a0104

Omar Sued Replied at 12:59 PM, 6 Feb 2013

Hi Erin
Chorea used to be a common symptom in the early years of the epidemic. I
had the opportunity of care for 2 patients with chorea. The cause is
irritation of the basal ganglia or thalamus, so, it can be a spectrum of
potential causes. Most commonly referred in the literature are
toxoplasmosis, LMP or HIV encephalitis. But also we can see it in
Shyphilis, Lympnhoma. We had a Chagoma (chagas disease, fortunately not
present in Africa).
I would suggest VDRL, LP (if fundoscopic exam is normal) with indian ink,
gram, giemsa, start ART as soon as possible (if this is LMP or encephalitis
it will resolve with HAART) and evaluate referral for CT scan. If not
improvment at 1 month and no CT scan evaluate empirical toxo treatment.
Best



*Dr. Omar Sued*

Director del Área de Investigaciones Clínicas

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Aaron Berkowitz Replied at 11:11 AM, 8 Feb 2013

Dear Erin,

If possible, could you please let us know a bit more about her history and examination to help us think through the case with you?

Was the onset of her chorea sudden or did it develop gradually and worsen?
Did she or does she have headache?
Is it -and has it always been- limited only to her left arm with no symptoms in any other limbs?
Does she have any weakness, sensory loss, or changes in her reflexes in the affected arm or anywhere else?
Is her toe upgoing (Babinski sign) on the side of the hemichorea?
Are her basic labs (glucose, chemistries) normal?
She is not pregnant by pregnancy test? (thinking of chorea gravidarum)
Are you able to send us an iPhone video by chance so we have a sense of her examination?


If the deficit is focal and came on sub-acutely, a mass lesion in the right subthalamic nucleus is likely. Given her HIV, toxoplasmosis, lymphoma, or tuberculoma would be the most concerning and potentially treatable etiologies, with toxoplasmosis most likely.

If the deficit came on suddenly, this could be from a small-vessel stroke or hemorrhage in the region of the subthalamic nucleus. Given her young age and HIV status, one could consider tuberculous or cryptococcal meningitis, as both can cause basilar meninigitis, which can cause strokes at the base of the brain such as this. Presumably she would have had headache and other symptoms (such as cranial nerve abnormalities) prior to the stroke and ongoing if she had/has meningitis, so this may be lower on the differential if none of these are/have been present.

While neurodegenerative, post-infectious, and metabolic etiologies tend to be more symmetrical, they can present asymmetrically. Many of the reported cases of hyperglycemia-induced chorea are in fact unilateral (see for example: http://www.ncbi.nlm.nih.gov/pubmed/12127677

An LP would certainly be useful, with India ink and acid fast testing. While the HIV-related infectious etiologies are most likely (and toxo most likely amongst those), if her CD4 count turns out to be higher than what one would expect for the OIs above, and no other etiology is apparent, one could consider ANA for lupus, if available.

This is most likely toxoplasmosis, and she should probably be empirically treated for this while awaiting more data. The attached review of movement disorders in AIDS has a nice review of hemichorea, and goes so far as to say that hemichorea in AIDS is "virtually pathognomonic for toxoplasmosis"

For symptomatic relief while she undergoes her evaluation, you could use low dose clonazepam (such as 0.25 TID) if you have it? Or a low dose of haldol could be attempted under observation to assure that she does not worsen

Please keep us posted and let us know how we can help. If she is referred for brain CT, we are happy to help you interpret the images.


Best wishes,
Aaron Berkowitz (Neurology resident, ) and Jennifer Lyons (Neuro-infectious disease fellow)

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