Camptocormia in a young man with anti-GAD-seropositive stiff-person syndrome

Stiff-person syndrome (SPS) usually manifests as an autoimmune neuromuscular disorder characterised by pronounced and advancing rigidity, primarily affecting the trunk and proximal muscles. There are various clinical subtypes like classic SPS (truncal stiffness, generalised rigidity and muscle spasms), partial SPS (stiff-limb syndrome) and uncommon forms including progressive encephalomyelitis with rigidity and myoclonus. Camptocormia, defined as forward flexion of the spine in the upright position that disappears in the supine position, without fixed deformity, has been described only in two cases as an initial presentation of Anti glutamic acid decarboxylase (GAD) autoimmunity. We encountered a young male presenting with a progressive forward-leaning posture and involuntary rhythmic movements in the lower limb. Diagnostic workup included MRI, blood routines, autoimmune screening, genetic testing, lumbar puncture and electromyography. Elevated serum anti-GAD antibody levels, inflammatory CSF and certain other clinical features supported the diagnosis of SPS. Treatment involved benzodiazepines, muscle relaxants and immunotherapy with intravenous immunoglobulin. This case underscores the importance of considering immune-mediated causes, such as SPS, in patients presenting with camptocormia.

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Publication Details

Journal
BMJ Case Reports
Published
2024-08-01
DOI
https://doi.org/10.1136/bcr-2024-262122
Citations
2
Primary Topic
Parkinson's Disease and Spinal Disorders
Type
article
Field-Weighted Citation Impact
0.74
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article

Camptocormia in a young man with anti-GAD-seropositive stiff-person syndrome

Deepa Avadhani, Philo Hazeena, Vijayashankar Paramanandam, Sundar Shanmugam et al.
2 citations
BMJ Case Reports
Parkinson's Disease and Spinal Disorders
0.74
article

Camptocormia in a young man with anti-GAD-seropositive stiff-person syndrome

Deepa Avadhani, Philo Hazeena, Vijayashankar Paramanandam, Sundar Shanmugam, Rithvik Ramesh, Lakshmi Narasimhan Ranganathan, Mekala Anusha, Kannan Karthik Kailash
article en
2 citations

Abstract

Stiff-person syndrome (SPS) usually manifests as an autoimmune neuromuscular disorder characterised by pronounced and advancing rigidity, primarily affecting the trunk and proximal muscles. There are various clinical subtypes like classic SPS (truncal stiffness, generalised rigidity and muscle spasms), partial SPS (stiff-limb syndrome) and uncommon forms including progressive encephalomyelitis with rigidity and myoclonus. Camptocormia, defined as forward flexion of the spine in the upright position that disappears in the supine position, without fixed deformity, has been described only in two cases as an initial presentation of Anti glutamic acid decarboxylase (GAD) autoimmunity. We encountered a young male presenting with a progressive forward-leaning posture and involuntary rhythmic movements in the lower limb. Diagnostic workup included MRI, blood routines, autoimmune screening, genetic testing, lumbar puncture and electromyography. Elevated serum anti-GAD antibody levels, inflammatory CSF and certain other clinical features supported the diagnosis of SPS. Treatment involved benzodiazepines, muscle relaxants and immunotherapy with intravenous immunoglobulin. This case underscores the importance of considering immune-mediated causes, such as SPS, in patients presenting with camptocormia.

BMJ Case ReportsVol. 17(8)
Sri Ramachandra Institute of Higher Education and Research (IN)
Good health and well-being
Openalex Percentile: Top 27%
Parkinson's Disease and Spinal Disorders
0.74
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Camptocormia in a young man with anti-GAD-seropositive stiff-person syndrome — Deepa Avadhani, Philo Hazeena, et al. · BMJ Case Reports (2024) | TGRS Research Map | TGRS