J Korean Foot Ankle Soc.  2019 Sep;23(3):131-134. 10.14193/jkfas.2019.23.3.131.

Stiff-Person Syndrome: Diagnostic Difficulty

• Lee JK
• Kang C
• Kim SB
• An BK
• Lee GS

• KMID: 2458015
• DOI: http://doi.org/10.14193/jkfas.2019.23.3.131

Abstract

Stiff-person syndrome is a rare disorder, and the natural history of the syndrome has not been completely ascertained. The symptoms range from mild to severe and can progress over time: the final result can be significant disability. However, this syndrome is often misdiagnosed due to a lack of understanding of the clinical manifestations. We report the case of a patient who presented with slowly progressing gait disturbance and lower extremity pain and was later diagnosed as suffering from stiff-person syndrome. The patient experienced symptomatic improvement after the administration of benzodiazepines. No recurrence of symptoms has been reported. If the characteristic clinical features and electromyography findings of the syndrome are accurately interpreted, diagnosis of patients with abnormal muscle tension can be effectively done.

Keyword

Stiff-person syndrome; Leg; Spasm; Autoimmune disease; Autoantibodies

MeSH Terms

Autoantibodies
Autoimmune Diseases
Benzodiazepines
Diagnosis
Electromyography
Gait
Humans
Leg
Lower Extremity
Muscle Tonus
Natural History
Recurrence
Spasm
Stiff-Person Syndrome*
Autoantibodies
Benzodiazepines

Figure

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  Figure 1 Clinical image (A), standing anteroposterior and lateral foot X-ray images (B, C) showing hyperextension of the metatarsophalangeal joint and flexion of the interphalangeal joint, respectively.

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  Figure 2 Spine magnetic resonance image. No specific findings other than mild bulging of the disc were observed. (A, B) Lumbar spine T1-weighted, T2-weighted and (C) cervical-thoracic-lumbar sagittal image.

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