Head drop and camptocormia
- T Umapathi1,
- V Chaudhry2,
- D Cornblath2,
- D Drachman2,3,
- J Griffin2,3,4,
- R Kuncl2,4
- 1Department of Neurology, National Neuroscience Institute, Singapore
- 2Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, USA
- 3Department of Neuroscience, Johns Hopkins University School of Medicine, Baltimore, USA
- 4Department of Pathology, Johns Hopkins University School of Medicine, Baltimore, USA
- Correspondence to: Dr T Umapathi, Department of Neurology, National Neuroscience Institute, Singapore 308433; tumapathi{at}yahoo.com
The spectrum of bent spine disorders
Head ptosis (drop) results from weakness of the neck extensor, or increased tone of the flexor muscles. It is characterised by marked anterior curvature or angulation of the cervical spine and is associated with various neuromuscular (table 1) and extrapyramidal disorders.12–15 Camptocormia or the bent spine syndrome was first described in hysterical soldiers in 1915 by the French neurologist Souques.16 Typically there is marked anterior curvature of the thoracolumbar spine. In some patients the spine is angulated forward, the arms propped against the thigh for support. More cases, all among soldiers, were reported during the first and second world wars. These patients responded well to psychotherapy. Recently camptocormia arising as a result of weakness or abnormality in the tone of the paraspinal muscles has been described (table 2). In contrast with other skeletal disorders of the spine such as kyphosis, the deformity in head ptosis and camptocormia is not fixed and is corrected by passive extension or lying in the supine position. It is not possible to straighten the neck or back voluntarily. The evaluation of these disorders can indeed be challenging and often no definite diagnosis is made, as illustrated by four cases of head ptosis and camptocormia seen by us at the Johns Hopkins Hospital.
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Neuromuscular causes for head ptosis
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Causes of camptocormia
CASE A
An 80 year old man developed head ptosis insidiously over a period of few weeks. A week before this he had an upper respiratory tract infection and also experienced transient sharp pain over the left and then the right shoulder. He had no diplopia, dysarthria, dysphagia, limb weakness, or fatiguability. Examination showed severe neck extensor weakness, Medical Research Council (MRC) grade 2. Muscle strength was normal in all other cranial, proximal, and distal limb muscles. Serum …
