Axial myoclonus in paraproteinemic polyneuropathy.
We describe a patient with a paraproteinemic anti-myelin-associated glycoprotein (anti-MAG) antibody polyneuropathy and concomitant axial myoclonic jerks. Neurophysiological investigation revealed that axial jerks were asymmetrical and exaggerated by lying in bed. They disappeared during mental arousal and sleep. Analysis of axial myoclonus showed that the first activated muscle was the left rectus abdominis with subsequent rostral and caudal propagation of a propriospinal type. Plasmapheresis substantially reduced the frequency and intensity of axial myoclonic jerks. In our patient, propriospinal myoclonus was associated with anti-MAG polyneuropathy, but the causal relationship remains unclear.
Vetrugno
Roberto
R
Dipartimento di Scienze Neurologiche dell'Università di Bologna, Via Ugo Foscolo 7, 40123 Bologna, Italy. roberto.vetrugno@unibo.it
Liguori
Rocco
R
D'Alessandro
Roberto
R
D'Angelo
Roberto
R
Alessandria
Maria
M
Montagna
Pasquale
P
D000009:Abdominal Muscles / Q000294:innervation / Q000503:physiopathology; D000200:Action Potentials / Q000502:physiology; D001323:Autoantibodies / Q000276:immunology; D015415:Biomarkers / Q000032:analysis; D018450:Disease Progression; D004568:Electrodiagnosis; D004576:Electromyography; D005260:Female; D006801:Humans; D037102:Lectins; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009119:Muscle Contraction / Q000502:physiology; D018992:Myelin-Associated Glycoprotein; D009207:Myoclonus / Q000276:immunology* / Q000503:physiopathology; D009363:Neoplasm Proteins / Q000276:immunology*; D009431:Neural Conduction; D010265:Paraproteinemias / Q000150:complications* / Q000503:physiopathology; D010525:Peripheral Nerves / Q000276:immunology / Q000473:pathology / Q000503:physiopathology; D010956:Plasmapheresis; D011129:Polyradiculoneuropathy / Q000150:complications* / Q000276:immunology* / Q000503:physiopathology; D011187:Posture / Q000502:physiology; D016896:Treatment Outcome
D001323:Autoantibodies; D015415:Biomarkers; D037102:Lectins; C115082:MAG protein, human; D018992:Myelin-Associated Glycoprotein; D009363:Neoplasm Proteins