Stiff Person Syndrome: Case Series
Laxmi Khanna1, Mandaville Gourie-Devi2
1Neurophysiology, Sir Ganga Ram Hospital, 2Neurophysiology, Sir Ganga Ram hospital
Objective:

Our objectives were to describe the varied clinical presentations, course and management of three patients of Stiff-Person Syndrome with different etiologies and outcome.

Background:
Stiff-Person Syndrome is a rare and disabling neurological condition with an incidence of 1 case per million per year. Diagnosis is clinical supported by high titers of anti-GAD antibodies and electromyography.
Design/Methods:

This was a retrospective study of three patients who fulfilled the criteria of Stiff-Person Syndrome during a time span of two years [2022– 2024].

Results:

The first case was a 50-year-old male, non-smoker, diabetic with Classic Stiff-Person Syndrome who experienced insidious onset muscle spasms while walking. There was a taut facial expression, excess sweating, stiffness of abdominal muscles, exaggerated lumbar lordosis, protuberant abdomen, elevated serum Anti GAD antibody and continuous motor unit activity on electromyography. He responded to plasmapheresis, intravenous immunoglobulins, diazepam and baclofen initially only to relapse after a few months later when Rituximab infusions were given.

The second case was a 63-year-old man, non-smoker with paraneoplastic stiff person syndrome who had right upper limb dystonia, pain, stiffness, gait ataxia, sleep and action myoclonus causing repetitive falls. His anti GAD antibodies were elevated and brain FDG PET-CT scans revealed encephalitis. Lymph node biopsy revealed a metastatic small-cell carcinoma from the lung. He received chemotherapy with four cycles of cisplatin and etoposide with clonazepam but unfortunately, he expired due to dissemination of malignancy.

The third patient, a 51-year-old man, non-smoker had focal stiff-person syndrome with stiffness of both legs, stimulus induced muscle spasms, and elevated anti GAD antibodies. He responded to treatment with gabapentin, diazepam and baclofen.

Conclusions:

Stiff-Person Syndrome has a plethora of manifestations that can be overlooked in busy clinical practice. Our aim was to illustrate the different clinical presentations to improve clinician and public awareness of this rare condition.

10.1212/WNL.0000000000210511
Disclaimer: Abstracts were not reviewed by Neurology® and do not reflect the views of Neurology® editors or staff.