Abstract Details

Title Crippling Calcium Catastrophe: A Complex Case of Calciphylaxis and Critical Myopathy

Topic Neuromuscular and Clinical Neurophysiology (EMG)

Presentation(s) P8 - Poster Session 8 (11:45 AM-12:45 PM)

Poster/Presentation
Number 9-022

Objective N/A

Background

Calciphylaxis is a rare disease characterized by small cutaneous arteriolar calcification, leading to tissue infarction, ischemia, and development of painful skin lesions. Seldomly, calciphylaxis can present neurologically as a stroke or with polyneuropathy. We describe a challenging case of muscle weakness with myositis-like features, later discovered as an atypical manifestation of calciphylaxis.

Design/Methods N/A

Results A 45-year-old man with prior hyperparathyroidism and end-stage renal disease on hemodialysis presented with myalgias and weakness. He developed a rising CK from 1,554 to 14,733 U/L over a month. A weakly positive MDA-5 antibody was present, and MRI thighs revealed extensive muscle edema. Empiric prednisone and mycophenolate were started for presumed autoimmune myositis with improvement in strength and CK to 344 U/L. Nuclear bone scan did not show evidence of calciphylaxis. Repeat MDA-5 antibody was negative. Muscle biopsy demonstrated severe type-2 myofiber atrophy without evidence of vasculitis, inflammatory, or necrotizing myositis. He presented a week later with quadriparesis with 0/5 strength proximally and 1/5 distally. CK increased to 32,900 U/L. It was thought this may have progressed as a necrotizing immune-mediated myositis or vasculitis myopathy. Repeat muscle biopsies were obtained, empiric IVIg was given, and prednisone was tapered with improvement in CK to 587 U/L. Soon after, the patient developed a violaceous retiform rash. Skin biopsy was obtained. The patient experienced critical gastrointestinal bleeding and clinically decompensated, passing away. Postmortem, skin biopsy revealed microthrombi in blood vessels, and muscle biopsy noted extensive calcium deposition in myofibers and perimysial vessels consistent with calciphylaxis.

Conclusions

Calciphylaxis is a challenging diagnosis that is often suspected through the presence of cutaneous findings. Our patient presented as progressive, painful quadriparesis that was likely secondary to tissue ischemia from calcium deposition. This case highlights an unusual presentation to demonstrate the heterogeneity of this disease and underscores the need in recognition among neurologists.

Authors/Disclosures
Haia Hazim, DO PRESENTER	Dr. Hazim has nothing to disclose.
Sathvik S. Shastry, MD (Lehigh Valley Health Network)	Dr. Shastry has nothing to disclose.
Gary W. Clauser, MD (Lehigh Neurology)	Dr. Clauser has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Bigoen . The institution of Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Genzyme. Dr. Clauser has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Genentech . Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Genzyme. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Biogen. Dr. Clauser has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Novartid.
Connie G. Tang, MD (Lehigh Valley Health Network)	Dr. Tang has nothing to disclose.

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