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Abstract

We performed a double-blind, placebo-controlled trial of intrathecal baclofen (ITB) in stiff-man syndrome. Three patients, unresponsive to current therapy, received 50 mu g of ITB or placebo on sequential days. Following ITB, all patients demonstrated improvement in reflex EMG activity. The mean reduction in total EMG activity (from all muscles) following stimulation of the medial plantar nerve (cutaneous flexor reflex) was 72% following 50 mu g of ITB compared with 18% following placebo (ANOVA: significance of F, p less than 0.0001). The mean latency to onset of the response was also significantly prolonged for all muscles following ITB (ANOVA: significance of F, p less than 0.05). Although reflex EMG activity was reduced in all patients, clinical improvement was evident in only one patient, who differed from the others studied by a longer duration of disease, greater severity of stiffness, less fear of falling, and greater electrophysiologic improvement.
NEUROLOGY 1995;45: 1893-1897

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REFERENCES

1.
McEvoy KM. Stiff-man syndrome. Mayo Clin Proc 1991;66:300-304.
2.
Solimena M, Folli F, Aparisi R, Pozza G, De Camilli P. Autoantibodies to GABA-ergic neurons and pancreatic beta cells in stiff-man syndrome. N Engl J Med 1990;322:1555-1560.
3.
Williams AC, Nutt JG, Hare T. Autoimmunity in stiff man syndrome [letter]. Lancet 1988;2:222.
4.
Knutsson E, Lindblom U, Martensson A. Plasma and cerebrospinal fluid levels of baclofen (Lioresal) at optimal therapeutic responses in spastic paresis. J Neurol Sci 1974;23:473-484.
5.
Whelan JL. Baclofen in treatment of the "stiff-man" syndrome. Arch Neurol 1980;37:600-601.
6.
Miller F, Korsvik H. Baclofen in the treatment of stiff-man syndrome. Ann Neurol 1981;9:511-512.
7.
Lorish TR, Thorsteinsson G, Howard FM Jr. Stiff-man syndrome updated. Mayo Clin Proc 1989;64:629-636.
8.
Kroin JS, Penn RD. Cerebrospinal fluid pharmacokinetics of lumbar intrathecal baclofen. In: Lakke JPWF, Delhaas EM, Rutgers AWF, eds. Parenteral drug therapy in spasticity and Parkinson's disease. Pearl River, NY: Parthenon Publishing Group, 1990:67-77.
9.
Penn RD, Mangieri EA. Stiff-man syndrome treated with intrathecal baclofen. Neurology 1993;43:2412.
10.
Meinck H-M, Ricker K, Hulser P-J, Schmid E, Peiffer J, Solimena M. Stiff man syndrome: clinical and laboratory findings in eight patients. J Neurol 1994;241:157-166.
11.
Ford B, Fahn S. Intrathecal baclofen [letter]. Neurology 1994;44:1367-1368.
12.
Meinck H-M, Ricker K, Conrad B. The stiff-man syndrome: new pathophysiological aspects from abnormal exteroceptive reflexes and the response to clomipramine, clonidine, and tizanidine. J Neurol Neurosurg Psychiatry 1984;47:280-287.
13.
Brashear HR, Phillips LH II. Autoantibodies to GABAergic neurons and response to plasmapheresis in stiff-man syndrome. Neurology 1991;41:1588-1592.
14.
Ashworth B. Preliminary trial of carisoprodol in multiple sclerosis. Practitioner 1964;192:540-542.
15.
Matsumoto JY, Caviness JN, McEvoy KM. The acoustic startle reflex in stiff-man syndrome. Neurology 1994;44:1952-1955.
16.
Davidoff RA. Antispasticity drugs: mechanisms of action. Ann Neurol 1985;17:107-116.
17.
Blaxter TJ, Carlen PL. Pre- and postsynaptic effects of baclofen in the rat hippocampal slice. Brain Res 1985;341:195-199.

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Published In

Neurology®
Volume 45Number 10October 1995
Pages: 1893-1897
PubMed: 7477988

Publication History

Published online: October 1, 1995
Published in print: October 1995

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Affiliations & Disclosures

P.L. Silbert, MBBS, FRACP
P.G. McManis, MBBS, FRACP
From the Departments of Neurology (Drs. Silbert, Matsumoto, McManis, and McEvoy), Physical Medicine and Rehabilitation (Dr. Stolp-Smith), and Anesthesiology (Dr. Elliott), Mayo Clinic and Mayo Foundation, Rochester, MN.
Presented in part at the annual meetings of the American Academy of Electrodiagnostic Medicine, San Francisco, CA, September 1994, and the American Neurological Association, San Francisco, CA, October 1994.
Received August 22, 1994. Accepted in final form March 3, 1995.
Address correspondence and reprint requests to Dr McEvoy, Department of Neurology, Mayo Clinic, Rochester, MN 55904.

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