Looking for advice on this one

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53 y/o female with 2 years BLE weakness, more proximal than distal, no numbness. Pt is a nurse with cirrhosis from hep B from supposed needle stick in the 80's, HTN, recent pericardial effusion. Pt referred from rheum, who saw pt for pos ANA, FHx Sjorgrens and RA. Previous EMG RLE done last year reported small amplitudes of peroneal and tibial motors, normal sural sensory, absent H, no tests on the left. Needle showed IIA (increased instertional activity) and 1+PSW's in quad and gastroc, with decreased recuitment of same + tib Ant. EHL reported as normal IA with occ fib and decr recruitment.

My exam of BLE today shows low amplitude peroneals to EDB and TA bilateral, normal amplitude tibials, all with normal DL's and NCV's. Sural sensories normal amp and DL, peroneals absent. Saph and tibial sensories also absent. H reflexes bilaterally absent. I did femorals and got nothing, but I haven't done them for a while, so I wouldn't completely hang my hat on that.

Needle exam got interesting. 2+ Fibs and PSW's with some IIA bilaterally in the Vast Med and Lat. Same in the Add Long and Bic Fem bilaterally, with some mild variations. All had good motor recuitment. Bil Tib Ant were normal, but bil gastrocs showed 2+ fibs and PSWs again with IIA and decreased recuitment. Interestingly the quad and gastroc muscles bilaterally felt much firmer than normal during the needle exam - like I was pushing the needle through clay. EDB's were normal at rest, no spontaneous activity, but decreased rectruitment, and the FDIP's showed fibs and PSWs with decreased recruitment.

Paraspinals bilat upper- mid- and lower- showed diffuse 1-2+ more PSWs than fibs, but all were small.

At this point I was out of time, needed to get to the next patient, so I told here I would discuss this with the referring doc, might need to bring her back.

My initial thought is PPN with superimposed polyradiculopathy, but this is wierd - denervation most everywhere, except peroneal distribution, while the peroneals were lowest amplitude. She has reportedly had an MRI of the L-Spine, I haven't seen it or the report yet, but the patients reports no stenosis of HNP's acording to what she was told.

BTW PE showed normal str to MMT, nl sensation, but absent MSRs in the knees and ankles. Obviously, I need to check UE's, but what would you guys suggest for further testing, EMG and otherwise? Something here is ringing a bell of something I saw in residency, but that set of neurons has been dormant for too long.

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Anterior horn cell? Fasics? Reflexes? Wasting?
 
No fasics noted, only tested LE reflexes. No wasting, just kind of a "puffiness" in the ankles in feet - not pitting edema, more adipose, but not in proportion to the rest of her - she's not heavy for age/height.
 
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Proximal > distal weakness, no numbness – think myopathy.

Absent MSRs LE – think neuropathy, polyradic

NCS – absent sensories except sural, low amplitude peroneal motor, normal tibial motor. Femoral technically unreliable (maybe). – maybe consistent with PN, possible mono multiplex (given sparing of sural, which you would expect to be gone in a neuropathy of this character). Polyradic is still possible, since the absent sensories tested are technically harder.

EMG – fibs everywhere, proximal and distal. Reduced recruitment distal, normal (?) proximal, despite fibs. What did the MUPs look like? Polyphasic? Large? Mixed population large and small?

Weird fibrous consistency during needle exam…

Just a hunch - consider something infiltrative affecting both nerve and muscle. Inclusion body myositis, amyloidosis, sarcoidosis. I would check a CPK, ESR, SPEP/UPEP. When you do the f/u EMG in the UEs, try to limit needling to one limb. Pt may need a muscle (or nerve) biopsy down the road. Can't biopsy the LE muscles since needle trauma may screw up the reading. May need to tell the referring doc to get a biopsy on the other side (either biceps or deltoid easily accessible for biopsy).

May want to repeat the MRI w/ contrast
 
Got a hold of recent labs - CPK 1534 on 6/17 and 1097 on 6/20. AST 46 (sl inc), ALT nl, aldolase 14.2, bunch of other rheum labs nl, including CRP, CBC and rest of CMP.

Right now I'm leaning towards inclusion body myositis - except for the MUAPs being fairly normal, it seems to fit very well. We'll see what rheum says when he sees the pt back.
 
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