好色先生

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Abstract Details

Cramp-Fasciculation Syndrome in Patients with and without Neural Autoantibodies
Peripheral Nerve
(-)
005
CFS is a mild peripheral nerve hyperexitability syndrome. Clinical and electrophysiologic comparisons between CFS patients with and without neural autoantibodies have not been studied.
We reviewed the Mayo Clinic clinical and electromyography database from January 1, 2000 to December 31, 2011 to identify clinically-defined CFS patients who underwent comprehensive neural autoantibody testing. Stored sera of patients who were seropositive for VGKC-complex antibodies were tested further for LGI1 or CASPR2 antibodies.
Thirty-seven CFS patients were identified. Twelve patients were seropositive for neural autoantibodies (9 VGKC-complex antibodies, 1 striational antibodies, 1 muscle and 1 ganglionic acetylcholine receptor antibodies). Clinical manifestations were similar in both seropositive and seronegative patients except for the presence of higher rates of central or autonomic neuronal hyperexcitability symptoms in seropositive cases (33% and 0% respectively, p = 0.008). Concomitant autoimmune disorders were present in 40% of seropositive and in 12% of seronegative patients (p = 0.08). None of the CFS patients had evidence of malignancy. Repetitive tibial nerve stimulation at 10-Hz revealed afterdischarges with a mean duration of 535.50 milliseconds in seropositive and 67.63 milliseconds in seronegative patients (p = 0.03). Neuromyotonic and myokymic discharges were absent in all patients. Stored sera were available in 7 of the 9 patients with VGKC-complex autoimmunity. One patient had both LGI1 and CASPR2 antibodies and one patient had LGI1 antibodies alone. Only 2 of the 12 seropositive patients required immunotherapy.
VGKC-complex autoimmunity was present in 24% of CFS patients. The target antigen within the VGKC-complex in most of these patients remains unknown. Tumor association was absent in our series. Repetitive nerve stimulation is of limited value in the diagnosis of CFS. Most patients were successfully managed symptomatically without immunotherapy.
Authors/Disclosures
Teerin Liewluck, MD, FAAN (Department of Neurology, Mayo Clinic)
PRESENTER 		Dr. Liewluck has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sarepta Therapeutics. Dr. Liewluck has received publishing royalties from a publication relating to health care.
Christopher J. Klein, MD, FAAN (Mayo Clinic) Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sangamo Therapeutics . Dr. Klein has received personal compensation in the range of $500-$4,999 for serving as a Consultant for NMD Pharma.
Lyell K. Jones, MD, FAAN (Mayo Clinic) Dr. Jones has received personal compensation in the range of $100,000-$499,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for 好色先生. Dr. Jones has received publishing royalties from a publication relating to health care. Dr. Jones has a non-compensated relationship as a member of the AAN Board of Directors with AAN that is relevant to AAN interests or activities. Dr. Jones has a non-compensated relationship as a Chair of the Mayo Clinic ACO Board of Directors with Mayo Clinic that is relevant to AAN interests or activities.
Friedemann Paul (Charite Universitatsmedizin in Berlin) Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Hexal. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Hexal. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Hoffmann-La Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bristol-Myers Squibb GmbH & Co. KGaA (BMS. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Hexal. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Merck. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Teva. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Horizon Therapeutics GmbH. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Hexal. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Paul has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for UCB. Dr. Paul has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Dr. Paul has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for UCB. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for sanofi. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche . Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for F&U Confirm. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Bristol Myers Squibb GesmbH. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for MICE Service. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for VINDICO medical education. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for UCB. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Beijing Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sandoz Internation GmbH. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Novartis. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche Thailand. Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Roche . Dr. Paul has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Alexion.