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Familial camptocormia: from dystonia to myopathy
  1. Karen M Doherty1,
  2. Alastair J Noyce1,
  3. Laura Silveira-Moriyama1,
  4. Angus Nisbet2,
  5. Niall Quinn1,
  6. Andrew J Lees1
  1. 1National Hospital for Neurology and Neurosurgery, Queen Square, London, UK
  2. 2Hurstwood Park Neurological Centre, Sussex, UK
  1. Correspondence to Professor A J Lees, Reta Lila Weston Institute of Neurological Studies, 1 Wakefield Street, London WC1N 1PJ, UK; a.lees{at}

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Camptocormia or bent spine syndrome is clinically defined as a marked (>45°) thoracolumbar spinal flexion that occurs when a patient stands or walks but completely abates in the supine position.1 The underlying cause remains elusive in many patients, but it has been associated with various muscle diseases and movement disorders. Jankovic's series of 16 patients with camptocormia presenting to a movement disorder clinic comprised 11 patients with Parkinson's disease, 2 with primary axial dystonia, 2 with secondary dystonia (disc surgery, syringomyelia) and 1 with Tourette syndrome.1 Laroche et al reviewed 63 patients presenting to a rheumatology clinic with camptocormia.2 Only 23 of them received a neurological diagnosis (including Parkinson's disease, limb girdle muscular dystrophy and myotonic dystrophy), whereas 40 received a final label of delayed-onset paraspinal myopathy, a diagnosis of exclusion.

Case report

The patient presented to our clinic at the age of 70 years. She had a 11-year history of back pain, not helped by a lumbar laminectomy, but for 1 year she had become increasingly bent the further she walked. Her elder sister, who had Parkinson's disease, also had a flexed posture when …

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  • Note Since this letter was accepted Jordan et al have described six further patients with FSHD presenting with camptocormia. Beevor's sign was present in half. The size of the 4q35 fragments was not given.12

  • Competing interests None.

  • Patient consent Obtained.

  • Provenance and peer review Not commissioned; externally peer reviewed.