53 yo man with an 80 pack yr smoking hx and history of Etoh abuse was admitted
with a 5 week hx of progressive ataxia, diplopia, dysarthria,
and dysphagia. Initially w/u revealed unremarkable MRI brain, and a CT
C/A/P showing prevascular lymph nodes with some calcifications and left hilar borderline enlarged lymph nodes, and a renal cyst not further characterized on that study.
DDx included subacute cerebellar degeneration and progressive supranuclear palsy.
Multiple CSF and serum labs were pending at time of discharge
He was readmitted a week later for progressive dysphagia for liquids in addition to solids, worsening gait s/p falls, and continued dysarthria and diplopia. IV IgG was given empirically without improvement. Repeat LP and additional infectious
serologies were negative. A dedicated renal CT was done to further characterize the indeterminate lesion on the previous CT, showing only a
simple cyst.
What would you do next? <<<
Addendum added after case presented:
These are the EBUS images from the bronch including and the core biopsy from the TBNA. The answer was that these little lymph nodes were small cell carcinoma. The special stain is a cytokeratin stain of the malignant cells.
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Not sure if the calcified nodes are red herring from old histo? Calcification usually takes years to develop. If concerned perhaps a mediastinoscopy. As for the neuro symptoms with negative CSF. Did you get an EMG? What is his HIV status? Some funky neuro manifestations from AIDS can occur and involve supranuclear structures as well. However, most of these diseases I think would also have some cortical involvemet which would be inconsistent witht he negative MRI...
Jeff, it looks like its just the two of us ;-)
When he got re-admitted, he was found to have an anti-calcium channel antibody, and another, underfined anti-neuronal antibody. They asked us to bronch him after getting a PET scan which showed some uptake in these mediastinal LNs. I'll send you the pictures from the bronch including the core biopsy, and tell you the answer in that e-mail (oooh the suspense).
SO that's Eaton lambert syndrome? How did they think to look for this antibody? Did they get an EMG first?
Yes it;s just the two of us for now (cozy eh?), but Carlos will be joining soon - rumor has it).
And if it's E-L syndrome, maybe he has an underlying cancer (found in your core bx?) with paraneoplastic syndrome.
Turns out he had sub-acute cerebellar degeneration, which is worse because (unlike LEMS) it does not get better after you treat the cancer. He had small cell. Which surprised me given the size of the lymph nodes. This year alone we have found four cases of small cell in lymph nodes as small as 8 mm. GGOtta love the EBUS scope, but Olympus has serious quality problems. We have had to sent the scope out for repair twice in the last 8 months. When we used Pentax scopes, we sent them out twice in 10 years.
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