AANEM Connect

I have a very interesting patient that has been a conundrum for me in the interpretation. I would love to hear how the literati and gliterati of the EDX world would interpret this patient's study.

Patient is 47 year old who is an inpatient in the rehab center. He was referred for evaluation of qudriplegia, atrophy and to rule out possible critical illness neuiroapthy. He has extensive history initially presented with right sided weakness to a peripheral hospital and imaging revealed left intraprenchymal hge with subacute right caudate infarct along with multivessel stenosis and left anterior cerebral artery occlusion. He developed additional left sided weakness prompting referral to the academic hospital for work up of vasculitis. He had multiple areas of diffuse intracranial vasculopathy, bilateral anterior cerebrl artery occlusion and sever posterior circulation stenosis.. Further imaging revvealed new areas of diffusion restriction along with watershed area area restriction and chronic micro hges. Another imaging almost one month later after initail presentation revealed a new infarct in left cerbellar hemishpere in PICA distribution. Thoracic and cervical MRIs normal. Work up for treponema pallidum, HIV, Sjogren, double stranded DNA, ANA, ANCA, lupus, anicardiolipin, IgA, IgG, IgG, cryoglobulin, CSF immunoglobulin panel, CSF VDRL, paraneoplastic panel CSF PCR were all normal. Sed rate was increased at 83, with increased C reactive protein. CSF electrophoresis with positive for small alpha-1 globulin and beta globulin spikes. Brain biopsy negative for vasculitis. Hence had extensive studies. exam with left cortical thumb, right gaze preference, diffuse muscle atrophy in all areas, he is unable to answer questions about sensation and has no active ROM in the limbs. Rflexes generally 3/4 in uppers, 2/4 in patellar tendons and right Achilles and 3/4 in left Achilles. Babinski flexor. Glabellar and snout reflexes are present. 

Sorry I could not get the tables into the format. Hence I am giving descriptive information. Sensory latencies were borderline prolonged but normal amplitudes. The CV in superficial peronel nerves were at 33 with latencies of 4.3. All of the CMAPs revealed temporal dispersion of more than 10ms, normal upper CV, borderline to slow CV in the LL in the range of 31 to 33, normal amplitudes of tibial and peroneal to TA but significantly small peroneal to EDB on right (0.1 mA) and 0.9mA on left. There was 40% drop of the CMAP across the fibular head on right when recording from TA. Left median F latency was 31.7 with H-reflexes obtainable, the left tibial F latency was 70.9 with presence of A waves. Blink reflex studies were normal. Upper limb temperature was 34 degree C and lower limb temperature was 29.5 degree C

The MUPs were obtained mostly by facilitation techniques generally with one or two units in most of the areas or no activity. Recruitment was very good in the medial hamstrings and gastroc on the right but none in the lateral hamstring, TFL, TA and single polyphasic units in PL. There were single polyphasic units in the left TA and EDB and none in the gastroc, VM and TFL. There were fibs and PSWs in the right peroneal muscles but none in other muscles.  The recruitment in the right hand muscles was normal with normal units. FCR showed spasticity and normal units. There was reduced recruitment of MUPs in clavicular PM with signle unit pattern in the deltoid (facilitated).

Is this CIDP or other motor more than sensory demyelinative neuropathy because of temporal dispersion? Reflexes are brisk in the setting of multiple strokes as outlined. I could rule out CIP as there was no generlized axon loss process. Lack of albuminocytological dissociation may be against CIDP or AIDP. Could this be a outlier and be Castleman's disease but he does not have vasculitis with brain biopsy. Normal blink reflexes rules out possibility of brainstem pathology.  Am I missing some diagnosis here. I would love the input from you all in this complex patient.