interesting case

[gallant2m]

if you guys have time, would love to get your input

44 yo hispanic female with no significant history presents with worsening generalized weakness,ataxia, gait instability. Found to have marked opsoclonus, ataxia, diffuse weakness on exam. Initial head CT negative, initial mri showed flair t2 hyperintensities which looks like ischemic hits, an mri brain 7 days later showed more hyperintensities this time in the cerebellum. Doesnt enhance post-infusion. LP demonstrated 150 wbc, lymphs mostly (tb, hsv, lyme, wnv negative), cx negative, cytology pending. paraneoplastics are pending. any initial thoughts or questions. just wanted to get some opinions on what im dealing with here.

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[Strokeguy]

With what I have seen during residency, would also consider ruling out HIV, neurosyphilis, sarcoidosis, vasculitis.
Would also do urine tox screen for any cocaine (Most of these lie during history).
I also saw a couple of patients with similar presentation and negative HSV serology. One of them had a relapse of symptoms and repeat tap was positive for HSV. On the second we could still not make a diagnosis, but EEG showed PLEDs so we treated empirically with acyclovir.

 

[danielmd06]

I also saw a couple of patients with similar presentation and negative HSV serology. One of them had a relapse of symptoms and repeat tap was positive for HSV.

Interestingly, I have too. In my case it was the third LP that actually came up positive.

 

[neurologist]

#1. What's the time course of her symptoms? Days? Weeks? Months?
#2. What was the distribution of the initial "ischemic looking hits" on her first MRI?
#3. Any mental status changes?
#4. History of measles?
#5. Anything suggestive of seizures? If so, what's EEG?
#6. At this point I'd lean toward paraneoplastic. Smoker? PET scan or other body imaging? Breast exam? CXR? Mammo?

 

[Phantom Spike]

Interestingly, I have too. In my case it was the third LP that actually came up positive.

Do you mean an HSV-encephalitis? Would that give you ataxia and generalized weakness? Would you still be thinking of that as a possibility if the OP's patient had had no seizures or mental status changes?

Or perhaps you mean an HSV-myelitis? Might explain the ataxia and the weakness. Is the latter possible even with no signal changes in the cord?

Can HSV give you a "disseminated" CNS infection of the type the OP's patient seems to have, and cause the MRI changes described?

I ask because I currently have a patient very, very similar to the OP's, but want to make sure I'm not missing anything while I wait for the paraneoplastic panel to come back (my patient has a long-standing history of smoking, so I'm thinking paraneoplastic in him, as well).

To the OP: what are the reflexes like? Is there a sensory level? Have you imaged the cord? A myelopathy might explain some of her symptoms like ataxia and weakness, although admittedly not the opsoclonus. Could there be an acute sensory neuropathy/neuronopathy (also possibly paraneoplastic)? If that's a possibility, you might consider an EMG/NCV.

Primary CNS vasculitis is a strong possibility as well, but it would be a hard diagnosis to make, especially without mental status changes or seizures. A definitive diagnosis would require a leptomeningeal biospy. The esr might be normal (as my patient's is).

 

[danielmd06]

Do you mean an HSV-encephalitis? Would that give you ataxia and generalized weakness? Would you still be thinking of that as a possibility if the OP's patient had had no seizures or mental status changes?

HSV encephalitis. But...

In the case I saw, the MRI demonstrated a single area of restricted diffusion on DWI (unlike the OP's case), but interestingly not on ADC - the patient had actually been originally admitted as an ostensible stroke.

The patient's physical exam was curiously similar to that described above - minus the opsoclonus, and with the addition of a fluctuating depressed level of consciousness.

The mental status changes played a role in the differential of my patient. I was surprised that it took multiple LP's to prove the diagnosis.

I wonder how often it happens in AIDS patients with relatively exotic neuroinfectious disease? Anyone?

 

[gallant2m]

thanks for the replies. will gather all info about things asked and will post tomm. As for now, eeg was normal sleep/awake. ESR 27, ACe negative in csf and serum,

 

[gallant2m]

Right-handed Hispanic female, mother of four, who has been progressively getting
weaker, shakier, and now developed jerky involuntary brisk movements of the eyes
for the last two days. She is practically unable to walk or eat. She has been
nauseated, vomiting. She has been sick over several weeks; that has
progressively gotten worse, to the point that she cannot get out of bed now.
She was seen here about a week ago, where she was evaluated with a CAT scan.
She was sent home. Since then, she deteriorated. She denies past medical history. She is on no medication.

MRI brain intially showed increased flair in the R thalamus. Repeat MRI 7 days later showed improvement in r thalamus lesion, but new lesion in post. limb of R internal capsule, L thalamus, vermis, L medial cerebellar hemisphere.

UDS negative for cocaine but positive for barbiturates?? dont know where that came from
LFT initially normal, but most recently GPT slightly elevated at 75
repeat esr 7, b12 folate nl
ana negative, c3,c4 negative, ss-a,ss-b negative'
hiv nonreactive
csf vdrl negative,
csf flow cytometry likely normal but report below

Final Pathologic Diagnosis:
Laser Scanning Immunophenotyping Report Comment
Immunophenotypic analysis shows the cerebrospinal fluid to contain a population
of small cells that strongly express CD45. These are T and B cells with a
ratio of 7 to 1. The T cells express CD2, CD3, CD5 and CD7 is weakly
expressed. The T helper to T suppressor ratio of 3.5 to 1. The B cells express
CD19 and CD20 without expression of CD5 or CD10. The surface membrane light
chain immunoglobulins are polyclonal. These immunophenotypic findings show no
evidence of a B-lineage non-Hodgkin's lymphoproliferative disorder. These
immunophenotypic findings show no evidence of a B-lineage non-Hodgkin's
lymphoproliferative disorder. The vast majority of the lymphocytes are T cells
with a predominance of T helper cells. The weak expression of CD7 may
represent a normal variation however, a T-lineage abnormality cannot be
completely excluded. Clinical correlation is suggested.