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Abstract Details

Involuntary Movements, Fasciculations, and Ion Channel Dysfunction in Multifocal Motor Neuropathy (MMN)
Neuromuscular and Clinical Neurophysiology (EMG)
P2 - Poster Session 2 (8:00 AM-9:00 AM)
1-014
We aimed at investigating the spatial extent of ion-channel dysfunction in MMN with involuntary movements and proximal conduction block (CB) of the median and ulnar nerves.
Ephaptic connections have been considered the cause of fasciculations, myokymia, and pseudodystonia in MMN1, 2. However, axonal excitability studies just distal to CB have indicated changes in membrane potential and ion-channel function3.
EMG, ultrasound (US), nerve conduction studies, and axonal excitability by threshold tracking and mathematical modelling4 were studied in a 35-year-old woman with a 5-year duration of progressive involuntary movements and weakness but no atrophy of the right hand.

EMG showed neurogenic MUPs, profuse fibrillations, fasciculations, trains of discharges, and loss of motor units in the right abductor pollicis brevis (APB). Motor CB and temporal dispersion were present in the ulnar and median nerves at the axilla, whereas sensory conduction was normal. The US of the nerves at the axilla and brachial plexus was normal.

Repeated excitability testing of the median nerve at wrist over 3 years showed a progressive 80% decrease in fast potassium conductance (GKfN), 360% increase in inward rectification (GH), and 25% increase in Barrett-Barrett conductance (GBB).

MMN is considered a focal disorder. However, this study indicated widely distributed paranodal abnormalities and ion-channel dysfunction far distal to the site of CB that can be involved in spontaneous activity.

1.         Roth G, Magistris MR. Neuropathies with prolonged conduction block, single and grouped fasciculations, localized limb myokymia. Electroencephalogr Clin Neurophysiol 1987;67:428-438.

2.         Garg N, Heard RNS, Kiers L, Gerraty R, Yiannikas C. Multifocal motor neuropathy presenting as pseudodystonia. Mov Disord Clin Pract 2017;4:100-104.

3.         Garg N, Park SB, Howells J, et al. Conduction block in immune-mediated neuropathy: paranodopathy versus axonopathy. Eur J Neurol 2019;26:1121-1129.

4.         Kiernan MC, Bostock H, Park SB, et al. Measurement of axonal excitability: Consensus guidelines. Clin Neurophysiol 2019.

Authors/Disclosures
Christian Krarup, MD, DMSc, FRcP, FÂé¶¹´«Ã½Ó³»­ (Rigshospitalet)
PRESENTER
Dr. Krarup has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Shire/Takeda. Dr. Krarup has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Shire/Takeda. Dr. Krarup has received publishing royalties from a publication relating to health care.
No disclosure on file
Tina Jeppesen, MD (NMRU) No disclosure on file
No disclosure on file