Antiglycine receptor-related stiff limb syndrome in a patient with chronic lymphocytic leukaemia.

Abstract

We report a 61-year-old man presenting with rapidly progressive stiffness and painful muscle spasms in the lower extremity muscles. The patient was diagnosed with chronic lymphocytic leukaemia (CLL) approximately a year before symptom onset. Electromyography displayed continuous motor unit activity and immunocytochemistry showed a positive staining for antiglycine receptor (anti-GlyR) antibodies. The clinical course was complicated by autonomic instability and cardiac arrest, but stabilised under continuous therapy with plasma exchange and symptomatic treatment with baclofen and clonazepam. Anti-GlyR antibodies induce rare, but severe, variants of stiff person syndrome that can be of paraneoplastic origin and life threatening due to autonomic dysfunction.

• Authors: Angelika Derksen, Mark Stettner, Winfried Stöcker, Rüdiger J Seitz
• Journal: BMJ case reports (BMJ Case Rep) Vol. 2013 (May 20 2013) ISSN: 1757-790X [Electronic] England
• PMID: 23696138 (Publication Type: Case Reports, Journal Article)

Chemical References

• Autoantibodies
• GABA Agents
• Receptors, Glycine
• Clonazepam
• Baclofen

Topics

• Aged
• Autoantibodies (immunology)
• Baclofen (therapeutic use)
• Clonazepam (therapeutic use)
• Electromyography
• GABA Agents (therapeutic use)
• Humans
• Leukemia, Lymphocytic, Chronic, B-Cell (complications)
• Male
• Paraneoplastic Syndromes (immunology, therapy)
• Plasma Exchange
• Receptors, Glycine
• Stiff-Person Syndrome (immunology, therapy)