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Camptocormia or Pisa syndrome in multiple system atrophy
Jarosław Sławek1, Mirosława Derejko, Piotr Lass
1Department of Neurosurgery, Division of Functional Neurosurgery and Movement Disorders, Medical University, ul. Debinki 7, 80-211 Gdańsk, Poland. jaroslawek@amg.gda.pl
Clinical Neurology and Neurosurgery
|August 23, 2005
Summary
Extreme forward trunk flexion, known as camptocormia or Pisa syndrome, can indicate multiple system atrophy (MSA) in parkinsonism cases. Early misdiagnosis as Parkinson
Area of Science:
- Neurology
- Movement Disorders
- Neurodegenerative Diseases
Background:
- Parkinsonism typically presents with mild stooped posture, but extreme forward trunk flexion (camptocormia, Pisa syndrome, antecollis) is less common.
- These axial dystonia presentations are often associated with extrapyramidal disorders.
Observation:
- Two cases of probable multiple system atrophy (MSA) with predominant parkinsonism and Pisa syndrome/camptocormia are presented.
- Both patients were initially misdiagnosed with idiopathic Parkinson's disease (PD).
Findings:
- Diagnosis of MSA was confirmed by typical clinical presentation, rapid progression, lack of response to dopaminergic medication, and characteristic MRI findings of putaminal pathology.
- Camptocormia, Pisa syndrome, and antecollis may represent a spectrum of axial dystonia, potentially linked to specific lesions within the putamen.
Implications:
- The diagnostic accuracy for multiple system atrophy remains low, highlighting the importance of recognizing unusual clinical presentations.
- Severe forward trunk flexion in parkinsonism warrants consideration of multiple system atrophy (MSA) in the differential diagnosis.
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