Kamptokormie

Camptocormia (bent spine syndrome or cyphose hystérique) is a rare syndrome characterized by involuntary flexion of the thoracolumbar spine with weight-bearing which reduces when laying down, and is due to isolated atrophy of the paraspinal muscles.

Epidemiology

In a small case series (n=16), 2/3 patients Parkinson disease, however camptocormia is only seen in ~10% of Parkinson patients .

Associations

These conditions may be associated :

• Parkinson disease
• dystonic disorders
• neuromuscular disorders
  • motor neuron disease
  • myasthenia gravis
• inclusion body myositis
• Graves disease
• may be drug-induced
• as part of a paraneoplastic syndrome 
• trauma
• multisystem atrophy
• Alzheimer disease
• chronic inflammatory demyelinating polyneuropathy 

In just under half of patients the condition may be idiopathic

Clinical presentation

Marked thoracolumbar kyphosis, usually worse when upright (vs supine) or ambulatory. Back pain is common however a significant number of patients are asymptomatic .

Pathology

Recent research suggests that - at least in Parkinson disease - the problem originates centrally in the brain, and not at the level of the muscles themselves. The condition may arise due to a dysfunction in proprioception .

Radiographic features

MRI

Diffuse isolated atrophy and fatty replacement of the paraspinal muscles, with high T1 and T2 signal which suppresses on fat-saturated sequences

History and etymology

Camptocormia was first described in 1815 by Earle, and the original case descriptions of the shaking palsy in 1817 by James Parkinson (1755-1824) included this phenomenon .

Siehe auch:

• Multisystematrophie
• Morbus Parkinson