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ABSTRACT
Introduction: Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder associated with muscle rigidity and spasms. A number of antibodies have been associated with disorder, including anti-glutamic acid decarboxylase and anti-amphiphysin.
Case report; In this report, we present a rare case of a 79-year-old woman who presented with bilateral lower extremity weakness who was ultimately diagnosed with stiff-limb syndrome, a rare variant of SPS. Extensive laboratory and CSF studies were unrevealing. Electromyography showed significant peroneal motor neuropathy and complex repetitive discharges in the left tibialis anterior muscle. Antibodies to glutamic acid decarboxylase were significantly elevated at 124 units/mL. She was subsequently started on oral diazepam with significant improvement in her symptoms.
Conclusion: The presentation of SPS can vary based on epidemiologic factors, clinical symptoms, and associated disorders. These forms can have overlapping features which may make the categorization of patients into one of these forms challenging.
Declaration of interest
No potential conflict of interest was reported by the author(s).
Declaration of financial/other relationships
The contents of the paper and the opinions expressed within are those of the authors, and it was the decision of the authors to submit the manuscript for publication.
The author(s) report no conflicts of interest.
A reviewer on this manuscript has disclosed that they receive clinical trial support from Covance, Neuroderm, Blue Rock Therapeutics, Prevail Therapeutics and NIH. I have served as a consultant for Accorda, CALA, Novo Nordisk. Peer reviewers on this manuscript have no other relevant financial relationships or otherwise to disclose.