Adult dermatomyositis with severe polyneuropathy: does neuromyositis exist?
Peripheral nerve involvement in dermatomyositis (DM) has been known as neuromyositis. However, the pathogenic mechanism is not clear, and the association between DM and peripheral neuropathy is still controversial. Our patient exhibited symptomatic polyneuropathy that was documented electrophysiologically in addition to typical features of DM. The sural nerve biopsy showed evidence of a continuing neuropathic process of axonal type. There was no finding of inflammatory cells infiltrating the vessels. Neither methylprednisolone nor intravenous immunoglobulin (IVIg) improved neurological symptoms including muscle weakness and sensory disturbance. Clinical, electrophysiological, and neuropathological features in our case demonstrate the association of DM and polyneuropathy. The possibility that the same pathological process affecting skin and skeletal muscles also affected peripheral nerves in our patient should be considered.
Department of Neurology, Teikyo University Chiba Medical Center, 3426-3 Anesaki, Ichihara, Chiba, 299-0111, Japan.
Makoto
M
Nomura
Takeshi
T
Watanabe
Hirotsugu
H
Mikami
Hiroaki
H
Ishikawa
Kanako
K
Yasui
Toshihiro
T
Yamazaki
Togo
T
Irie
Megumi
M
Suzuki
Seiitsu
S
Ono
D000893:Anti-Inflammatory Agents / Q000627:therapeutic use; D003882:Dermatomyositis / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D007155:Immunologic Factors / Q000627:therapeutic use; D007564:Japan; D008775:Methylprednisolone / Q000627:therapeutic use; D008875:Middle Aged; D018482:Muscle, Skeletal / Q000473:pathology / Q000503:physiopathology; D011115:Polyneuropathies / Q000188:drug therapy / Q000473:pathology / Q000503:physiopathology*; D011693:Purpura / Q000473:pathology; D012720:Severity of Illness Index; D012867:Skin / Q000473:pathology; D013497:Sural Nerve / Q000473:pathology / Q000648:ultrastructure; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D016756:Immunoglobulins, Intravenous; D007155:Immunologic Factors; D008775:Methylprednisolone