Â鶹´«Ã½Ó³»­

Â鶹´«Ã½Ó³»­

Explore the latest content from across our publications
Join The Â鶹´«Ã½Ó³»­

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Stiff Limb Syndrome: A Rare Variant of Stiff Person's Syndrome
Neuromuscular and Clinical Neurophysiology (EMG)
P15 - Poster Session 15 (12:00 PM-1:00 PM)
1-015
Stiff-person syndrome (SPS) is a spectrum of rare autoimmune neurological disorders with a manifestation of muscle rigidity and episodic muscle spasms.  Stiff limb syndrome (SLS) is a rare variant of SPS that is more localized. These disorders are typically acquired, progressive, fluctuating, and commonly occur in young women aged 30-50 years.  In this report, we present an atypical case of an elderly woman who presented with bilateral leg muscle rigidity and ultimately diagnosed with SLS.

A 79-year-old woman presented with progressive bilateral leg weakness and cramps for three days resulting in multiple falls. On physical examination she was found to have hyperreflexia, increased gastrocnemius tone, as well as spasticity and plantarflexion in both legs.  Diagnostic work-up including imaging of the neuraxis and complete infectious and malignancy work-up was unremarkable.  Titers of antibodies to glutamic acid decarboxylase (GAD) were elevated at 124,000 U/mL (normal 0-5 U/mL). The patient was diagnosed with SLS and subsequently started on a benzodiazepine with significant improvement in her symptoms.
NA
SLS is an emerging entity that is considered a focal form of SPS in which the symptoms are confined to a distal limb, usually the legs. Our review of the literature revealed that the SPS-related spectrum disorders such as SLS has not been reported in the elderly. SPS and its variants are suspected to be related to autoantibodies to the GAD enzyme, although the pathophysiology remains poorly understood.  SLS may potentially be a distinct entity from SPS with several overlapping clinical features and diagnostic findings. 
Our case report describes a patient who presented with focal neurological symptoms and eventually diagnosed with SLS, confirmed by autoimmune work-up.  We advise clinicians to be aware of this rare entity and pursue the appropriate work-up when the clinical suspicion is high, including the elderly population. 
Authors/Disclosures
Benjamin Lin
PRESENTER 		No disclosure on file
Keithan Sivakumar, MD Dr. Sivakumar has nothing to disclose.
Hussam A. Yacoub, DO (The Lehigh Valley Health Network) Dr. Yacoub has nothing to disclose.
Jay D. Varrato, DO Dr. Varrato has nothing to disclose.
Alison L. Walsh, MD (Lehigh Valley Health Network) Dr. Walsh has nothing to disclose.
Alissa E. Romano, DO (LEhigh Valley Physician Group) Dr. Romano has nothing to disclose.