AANEM Connect

Our questions:
Is this the correct diagnosis?
Should we simply treat her with whatever rheumatology suggests (steroids plus steroid sparing agent of thier choice) or should we continue maintenance IVIg in addition to this?


62 year old woman with 6 weeks of progressive distal painful paresthesias and weakness. 
Seven weeks prior - myalgias, no weakness, ?viral illness?. No further musculoskeletal pain after this week.  
Six weeks prior - painful paresthesias in both hands and both feet. 
4-5 weeks prior - noticed progressive weakness - fell when "ankle collapsed" 
 
Exam with significant distal and proximal weakness with some distal wasting.
Absent reflexes except knees 1+.  (Exam from another MD noted present reflexes 5 days prior.)
Sensation: Stocking (to ankle) - glove (to above wrists) - LT, PP.
Decreased vibration ulnar styloid, B and medial malleolus, B.
Proprioception impaired at MP bilaterally and ankle bilaterally. 

EMG/NCS
Motor NCS - no responses - Median (APB), Ulnar (ADM), Tibial (AH), Peroneal (EDB).
Peroneal (TA) - low amplitude, no slowing of CV, no temporal dispersion, no conduction block.
Sensory NCS - no responses - Median (DIII), Ulnar (DV), Superficial peroneal and sural. 
Signs of denervation in multiple muscles with few MUPS with normal configuration, increased recruitment ratio, discrete interference patttern at full effort.

CSF
Protein - 28
Glucose normal
WBC - 4 -
52%Neutrophils
22%Lymphocytes
26%Eosinophils
RBC - 11

Awaiting VEGF results

P-ANCA 1:320
MPO (myeloperoxidase) 23.8 weakly positive (drawn after IVIg was given)
CT sinuses -  extensive paranasal sinusitis with with areas 
of hyperattenuation favored to represent inspissated secretions.
No rash
CT chest - 6 mm left apical nodule; no parenchymal abnormalities. 
CT abd/pelvis - no abnormalities.
EKG - normal
ECHO - no abnormalities
No symptoms of asthma
Peripheral eosinophil - 37%; absolute 4.7 K/uL
 

Rheumatology - given her clinical and laboratory data, they had high suspicion for eosinophilic granulomatosis with polyangiitis (EGPA - the old Churg-Strauss).  They started treating with pulse methylprednisolone 1000 mg IV daily for 3 days and now on oral steroids daily.
We gave IVIg a week prior but no response as of yet. 
 

MRI spine and brain without and with gado - no abnormalities.

Patient with some mild improvement of symptoms on steroid, noted as return of some reflexes ( 1+ Brachioradial, Biceps bilaterally and 2+ patellar bilaterally).

The rheumatologists wanted to do sural nerve biopsy and I asked them about other sites for biopsy and they said there was nowhere else to biopsy.
Sural Nerve Biopsy showed no evidence of vasculitis. Showed severe, subacute (ongoing) axonal degeneration, in the background of moderately severe axonal loss.. 

Official report summary as follows: 
This biopsy reveals severe, subacute (ongoing) axonal degeneration, in the background of moderately severe axonal loss. Numerous foct of fiber breakdown associated with phagocytotic macrophages are displayed in cross-sections. By the semi-quantitative teasing fiber analysis, 62% of the remaining fibers show active Wallerian degeneration. The demyelinating componentis displayed amongst 12% of the teased fibers in the forms of segmental demyelination/ remyelination and is thought to be indistinguishable from age-related changes. Multiple levels of sections have been investigated for possible vasculitis: this biopsied segment of the nerve shows no histological features of vasculitis.
 

Other work up: 

-B12, Mg and TSH wnl

-ESR/CRP moderately elevated

-Zn, Cu levels normal

-RPR negative

-strongllodes, toxocara, tryptase- WNL,

- Hepatitis panel negative