On the early diagnosis of IVIg-responsive chronic multifocal acquired motor axonopathy.
Multifocal acquired motor axonopathy (MAMA) is a treatable, immune mediated motor neuropathy with purely axonal electrophysiological features. Distinction from degenerative neuronopathies such as progressive muscular atrophy (PMA) or early motor neuron disease (MND) can be difficult because of the similar clinical and electrophysiological findings. Here, we report the clinical, electrophysiological and laboratory findings in 6 patients with MAMA. Electrophysiological testing showed purely axonal findings with evidence of pathological spontaneous activity and chronic neurogenic changes. Of particular note, pathological spontaneous activity in paraspinal myotoms was not detectable in any of the patients even though it had been documented in peripheral muscles of the corresponding myotome(s). Elevated serum ganglioside antibody levels,most frequently anti-GD1a antibodies, were present in all 6 patients. IV Ig treatment led to clinical improvement in all but one patient, who showed an allergic response when exposed to IVIg. Our findings indicate that paraspinal EMG and anti-GD1a antibodies can facilitate the early identification of treatable, IVIg responsive, patients with MAMA.
Fischer
Dirk
D
Department of Neurology, University of Bonn, Sigmund-Freud-Str. 25, 53105 Bonn, Germany.
Grothe
Christoph
C
Schmidt
Stephan
S
Schröder
Rolf
R
D000200:Action Potentials / Q000187:drug effects / Q000502:physiology; D000328:Adult; D000906:Antibodies / Q000097:blood; D004576:Electromyography / Q000379:methods; D005260:Female; D005733:Gangliosidoses / Q000276:immunology; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D009431:Neural Conduction / Q000502:physiology; D010523:Peripheral Nervous System Diseases / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome
D000906:Antibodies; D016756:Immunoglobulins, Intravenous