Abstract:
IMPORTANCE:Documentation of muscle pathology compatible with targeting of sarcolemmal aquaporin-4 (AQP4) by complement-activating IgG implies involvement of organs beyond the central nervous system in neuromyelitis optica spectrum disorders. OBSERVATIONS:We report on a 51-year-old woman who had relapsing optic neuritis, transverse myelitis, AQP4-IgG seropositivity, and recurrent myalgias with hyperCKemia. A muscle biopsy revealed scattered myofibers with internal nuclei, atrophy, and regeneration but no necrosis. Mild inflammatory exudates, in endomysial and perivascular spaces, consisted of lymphocytes, histiocytes, and scattered eosinophils. The sarcolemma exhibited loss of AQP4 and deposition of IgG and complement activation products, characteristics not seen in control biopsy samples of healthy muscle and immune-mediated myopathies. CONCLUSIONS AND RELEVANCE:Recurrent hyperCKemia accompanying AQP4-IgG seropositivity reflects targeting of skeletal muscle AQP4 by pathogenic IgG. The entity of autoimmune AQP4 myopathy extends the neuromyelitis optica spectrum beyond the central nervous system.
journal_name
JAMA Neuroljournal_title
JAMA neurologyauthors
Guo Y,Lennon VA,Popescu BF,Grouse CK,Topel J,Milone M,Lassmann H,Parisi JE,Pittock SJ,Stefoski D,Balabanov R,Lucchinetti CFdoi
10.1001/jamaneurol.2014.775subject
Has Abstractpub_date
2014-08-01 00:00:00pages
1025-9issue
8eissn
2168-6149issn
2168-6157pii
1877427journal_volume
71pub_type
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