Presentation, Etiology, Diagnosis, and Management of Camptocormia

Camptocormia (bent spine syndrome, cyphose hystérique) is an abnormality characterized by severe forward flexion of the thoracolumbar spine which typically increases during walking or standing and completely disappears in supine position. Camptocormia can be due to central nervous system diseases, such as Parkinson’s disease, dystonia, multisystem atrophy, or Alzheimer’s disease, due to peripheral nervous system diseases, such as primary myopathy, secondary myopathy, motor neuron disease, myasthenia, or chronic inflammatory demyelinating polyneuropathy, due to side effects of drug treatment, due to disc herniation, arthritis or spinal trauma, or due to paraneoplasia. Only rarely may camptocormia be attributable to psychiatric disease. The diagnosis is based on clinical findings, imaging of the cerebrum or spine, needle electromyography, or muscle biopsy. Treatment options are limited and frequently futile and rely on conservative measures, such as psychotherapy, physiotherapy, use of orthoses, drugs, injection of botulinum toxin, withdrawal of causative drugs, electroconvulsive therapy, or invasive measures, such as surgical correction or deep brain stimulation. The outcome is generally fair. Some patients profit from therapy whereas others do not respond to treatment and become progressively immobile. Copyright © 2010 S. Karger AG, Basel Received: February 26, 2010 Accepted: April 30, 2010 Published online: June 8, 2010 Josef Finsterer, MD, PhD Postfach 20 AT–1180 Vienna (Austria) Tel. +43 1 71165 92085, Fax +43 1 478 1711 E-Mail fifigs1 @ yahoo.de © 2010 S. Karger AG, Basel 0014–3022/10/0641–0001$26.00/0 Accessible online at: www.karger.com/ene Finsterer /Strobl Eur Neurol 2010;64:1–8 2 by the French neurologists Souques and Rosanoff-Saloff who described the abnormality in World War 1 soldiers traumatized by shell shocks [8, 9] . The first described soldier was wounded by a bullet which entered along the axillary border of the scapula and emerged near the spine, resulting in a trunk being bent almost at a right angle [9] . To the ‘poilu’ (nickname of the French WW1 soldier) this condition was known as ‘cintrage’ (aching). The man was ‘cured’ by application of plaster corsets [9] . In 1919, two further cases were described by Roussy and Lhermitte [10] . The first was an infantryman, blown into the air by a bursting shell, who experienced violent pain and remained stooped to the right after regaining consciousness [10] . He was also cured by a plaster corset. The second case was a ‘chasseur’ who experienced respiratory distress, mutism and camptocormia after being buried in an explosion. He recovered after a single séance of electrical treatment [10] . Though these cases were classified as hysterical, the traumatic injury alone may sufficiently explain camptocormia [11] . The association between camptocormia and Parkinson’s disease was first described by Djaldetti et al. [12] in 1999. Camptocormia due to a genetic disease was first described in a patient with myotonic dystrophy type 2 carrying a ZNF9 gene mutation [13] . The first patient with multisystem atrophy (MSA) and camptocormia was reported by Reichel et al. [14] in 2001.