Stiff Person Syndrome (SPS) - Diagnosis and Management Summary
Clinical manifestations:
 • Progressive muscle stiffness, rigidity, spasm- axial muscles
 • Severely impaired ambulation
 • Excessive muscle rigidity-lumbar, trunk, proximal muscles-sustained muscle contractions (agonist and antagonist muscles)
 • Wide based gait with tendency to fall
 • Triggers- sudden movement, noise, emotional upset
 • Spasms- visualized- tight, rock-hard, board like feel
 • Autonomic dysfunction- hyperpyrexia, diaphoresis, high HR
 • Sudden death- diaphragm spasm- apnea.
Diagnosis - High index of suspicion + Presence of:
 • stiffness in axial and limb muscles resulting in impairment of ambulation
 • presence of superimposed spasms triggered by noise, movement or emotions
 • A+ therapeutic response to diazepam or findings of continuous motor unit activity on EMG
 • absence of other neurological disorders
 • testing anti GAD (positive in 2/3 of pts)
 • therapeutic trial of diazepam
 • EMG testing
Treatment
 • Initial/symptomatic therapy: BDZ- Diazepam or clonazepam
 • Resistant to initial symptomatic therapy: Baclofen
 • Severe disease/resistant: IVIG
 • If resistant to IVIG: Rituximab (anti-CD20), plasma exchange, or others (botox, sodium valproate, vigabatrin, propofol)

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