Neuromuscular Case Vignettes

Case 8: 68 year old man with progressive bilateral leg and arm weakness

68 year old man started to have increasing muscle pain and  lower limb weakness 2 weeks after he was started on atorvastatin for hyperlipidemia.  He self discontinued atorvastatin, and the myalgia improved in the next subsequent weeks, but the weakness continued to deteriorate and spread to the arms.His hand strength was affected but to a  milder degree. About a year later the weakness had progressed to the point that he was unable to ambulate. He had no sensory complaints. He also developed chronic diarrhea and severe  weight loss, 90 lbs  in a period of 2 years despite having a good appetite and oral supplementation.

Workup showed seropositivity to gliadin IgA and tissue transglutaminase antibodies (which resulted in Dx of Celiac disease).  Diarrhea improved and he became seronegative to celiac related autoantibodies after starting on a  gluten free diet but there was no improvement of weakness. He was also found to have an IgG κ paraprotein  (0.2 g/L), however a bone marrow biopsy was negative for myeloma or amyloid. CK was 28 U/L (nl: 9-185).

Exam about 2 years after the onset of weakness showed that the mental status and cranial nerves were normal.
His limb muscles were severely atrophic and he had proximal>distal, lower>upper limb weakness, with no fasciculations. The reflexes were trace to absent and the sensory exam was entirely normal. He was unable to stand or walk. Nerve conduction study and EMG was done as the next step.