Figure 1
CT scan lumbo-sacral region, sagittal views
A rare case of Camptocormia (bent spine syndrome)
SectionMusculoskeletal system
Case TypeClinical Cases
AuthorsYallapa, Sachin; Bashir, Omar; Sheik, Mujeeb.
Connected authors
76 years, female
Clinical History
The patient presented with discomfort in lower back after prolonged standing or walking for 3 years, progressive difficulty to maintain an erect posture. Difficulty to stand up straight from stooped posture and difficulty walking. No history of any neurology in upper or lower limbs. No family history of any neuromuscular disorder.
Imaging Findings
Disc degeneration throughout the lumbar region with a varying degree of disc space narrowing and disc signal loss. There is some bulging of these discs but no evidence of disc protrusion or root compression. The bony canal and the lateral root canals are adequate. Bone marrow signal is unremarkable. There is no intradural abnormality.
Significantly, gross fatty replacement of the erector spinae musculature was noted throughout the lumbar spine.
Significantly, gross fatty replacement of the erector spinae musculature was noted throughout the lumbar spine.
Discussion
Discussion:
Camptocormia (bent spine syndrome) refers to an abnormal posture with marked flexion of the thoraco-lumbar spine which increases during walking and resolves in the supine position. Originally attributed to psychogenic disorders (war hysteria), this symptom is an increasingly recognised feature of parkinsonian and dystonic disorders, but it may also be caused by neuromuscular disorders [3, 1] as well as paraneoplastic syndrome [4], inclusion body myositis [5] drug-induced camptocormia [4] and Grave’s disease [7].
Camptocormia is a syndrome confined to the extensor muscles of the spine. There are usually no other motor or sensory symptoms. Lumbar radicular nerves are unaffected. However, some patients may suffer mild discomfort in the lower back. Moreover, some authors suggest that symptoms may not occur unless there is also hip flexion contracture [2].
Radiological analysis of the thoraco-lumbar spine by MRI or CT can reveal paraspinal muscle atrophy heterogeneous in appearance, with progressive replacement of paraspinal muscles with fat. Electromyography findings are not uniform. Both myogenic and neurogenic features of the disease have been reported [2].
Some pharmacologic treatments attempted so far include anticholinergics, amantadine, dopamine agonists, muscle relaxants, and tetrabenazine, but these have not improved the posture [1]. There is some evidence of bilateral pallidal stimulation providing functional benefit for the severely disabling condition. Physiotherapy with classical and leather arthesis has also been tried in few cases.
However, most cases have failed to respond fully to any treatment. No treatment modality has proved to be effective so far.
Camptocormia (bent spine syndrome) refers to an abnormal posture with marked flexion of the thoraco-lumbar spine which increases during walking and resolves in the supine position. Originally attributed to psychogenic disorders (war hysteria), this symptom is an increasingly recognised feature of parkinsonian and dystonic disorders, but it may also be caused by neuromuscular disorders [3, 1] as well as paraneoplastic syndrome [4], inclusion body myositis [5] drug-induced camptocormia [4] and Grave’s disease [7].
Camptocormia is a syndrome confined to the extensor muscles of the spine. There are usually no other motor or sensory symptoms. Lumbar radicular nerves are unaffected. However, some patients may suffer mild discomfort in the lower back. Moreover, some authors suggest that symptoms may not occur unless there is also hip flexion contracture [2].
Radiological analysis of the thoraco-lumbar spine by MRI or CT can reveal paraspinal muscle atrophy heterogeneous in appearance, with progressive replacement of paraspinal muscles with fat. Electromyography findings are not uniform. Both myogenic and neurogenic features of the disease have been reported [2].
Some pharmacologic treatments attempted so far include anticholinergics, amantadine, dopamine agonists, muscle relaxants, and tetrabenazine, but these have not improved the posture [1]. There is some evidence of bilateral pallidal stimulation providing functional benefit for the severely disabling condition. Physiotherapy with classical and leather arthesis has also been tried in few cases.
However, most cases have failed to respond fully to any treatment. No treatment modality has proved to be effective so far.
Differential Diagnosis List
Camptocormia (bent spine syndrome)
Spinal deformity
Dystonic disorders
Final Diagnosis
Camptocormia (bent spine syndrome)
References
[1] Azher SN, Jankovic J (2005) Camptocormia. Pathogenesis, classification, and response to therapy. Journal of Neurology 65:355-359 (PMID: 16087897)
[2] Samuel Katsuyuki Shinjo, Silvia Carolina Ramos Torres, and Ari Stiel Radu (2008) Camptocormia: A Rare Axial Myopathy Disease. Clinics (Sao Paulo) 63:416–417 (PMID: 18568258)
[3] Karbowski K (1999) The old and the new camptocormia. Spine (Phila Pa 1976) 24:1494-1498 (PMID: 10423797)
[4] Zwecker M, Iancu I, Zeilig G, Ohry A (1998) Camptocormia: a case of possible paraneoplastic aetiology. Clinic Rehabil 12:157–60 (PMID: 9619658)
[5] Hund E, Heckl R, Goebel H, Meinck HM (1995) Inclusion body myositis presenting with isolated erector spinae paresis. Neurology 45:993–4 (PMID: 7746422)
[6] Kiuru S, Iivanainen M (1987) a new side effect of sodium valproate. Epilepsy Res. Epilepsy Res 1:254–7
[7] Loh LM, Hum AY, Teoh HL, Lim EC (2005) Graves’ disease associated with spasmodic truncal flexion. Parkinsonism Relat Disord 11:117–9 (PMID: 15734671)
Case information
| URL: | https://www.eurorad.org/case/9543 |
| DOI: | 10.1594/EURORAD/CASE.9543 |
| ISSN: | 1563-4086 |
