Camptocormia: Difference between revisions

[[File:Camptocormia.png|thumb|A clinical photograph showing a patient with the flexed posture. It can be abated when lying down.<ref name="Shinjo" />]]

 

== History and society ==

[[File:Alexandre Achille Souques.jpg|thumb|Alexandre-Achille Souques was one of the first major researchers of camptocormia and created the definition and name used for the condition to this day.|227x227px]]

 

When the disorder was first clinically studied around the time of [[World War I|First World War]], it was believed to be a psychogenic [[conversion disorder]] that resulted from the severe trauma of war. Souques and others treated patients with psychological therapy and early versions of [[Electroconvulsive therapy|electrotherapy]]. Samuel A. Sandler used a similar approach to treat soldiers during the [[World War II|Second World War]].<ref name="Thibaut" /> The view of BSS as a conversion disorder led to a lack of awareness about the conditions and few diagnoses by physicians.

 

=== Camptocormia in the elderly ===

 

The primary symptom of camptocormia is abnormal forward bending of the [[torso]]. This bending becomes worse while walking but does not appear when the affected individual is lying down in a horizontal position. This alleviation of the condition indicates that it is a manifestation of another disease or ailment and is not due to a spine that is actually bent.<ref name="Thibaut" /> This is somewhat ironic, since the medically accepted name for the condition is bent spine syndrome.

 

 

== Pathology ==

[[myopathy|Myopathic]] origin BSS can be secondary to various muscular disorders or occur as a primary [[idiopathy]].<ref name="Thibaut">{{cite journal|last2=Guedj|first2=Nathalie|last3=Boulu|first3=Philippe|last4=Guigui|first4=Pierre|last5=Benoist|first5=Michel|date=2010-03-19|title=Camptocormia: the bent spine syndrome, an update|journal=European Spine Journal|language=en|volume=19|issue=8|pages=1229–1237|doi=10.1007/s00586-010-1370-5|issn=0940-6719|pmc=2989190|pmid=20300781|last1=Lenoir|first1=Thibaut}}</ref> These etiologies are termed secondary and primary BSS respectively. Idiopathic primary BSS is a late-onset myopathy with progressive muscular weakness that is detected on the spinal [[Extensor Muscle|extensor muscles]] in elderly patients and is more predominant in females.<ref name="Thibaut" /> The pathogenesis of primary BSS is typically related to [[fibrosis]] and fatty infiltration of muscular tissues and to [[Mitochondrion|mitochondrial]] changes due to the aging process.<ref name="Thibaut" /> Specifically, weakening occurs in the paravertebral muscles of patients. These paravertebral muscles have a great influence over the walking stance and [[gait]] of a patient, so fatty infiltration and degradation of these muscle lead to the characteristics that easily define BSS, such as the anterior flexion of the back combined with an ability to keep upright with any kind of support (e.g., holding onto a table).<ref name="Azher" />

 

 

=== Neurological origin ===

A multitude of neurological disorders cause BSS, including [[motor neuron disease]], [[Central nervous system|CNS]] disorders, and early [[amyotrophic lateral sclerosis]].<ref name="Thibaut" /> Usually, the bent spine is caused by dysfunctioning extensor spinal muscles with a neurological cause.

 

 

=== Gene mutations ===

 

== Diagnosis ==

[[File:Deposit of fibrosis and fatty infiltration.jpg|thumb|Spinal-muscle biopsy showing intense endomysial deposit of [[fibrosis]] (green) and fatty infiltration. Also observable is irregular distribution of involuted muscle fibers. (Trichrome, original magnification ×9100)<ref name="Thibaut" />|220x220px]]

Camptocormia is becoming progressively found in patients with [[Parkinson's disease]].<ref name="Azher" />

Patients with camptocormia present with reduced strength and stooped posture when standing due to weakened paraspinous muscles (muscles parallel to the spine). Clinically, limb muscles show fatigue with repetitive movements.<ref name=":2" /> Paraspinous muscles undergo fat infiltration. [[Electromyography]] may be used as well in diagnosis. On average, the paraspinous muscles of affected individuals were found to be 75% myopathic, while limb muscles were 50% percent myopathic.<ref name=":2" /> [[Creatine kinase]] activity levels in skeletal muscle are a diagnostic indicator that can be identifiable through blood tests.

 

 

=== Treatment and management ===

Treatment of the underlying cause of the disease can alleviate the condition in some individuals with secondary BSS. Other treatment options include drugs, injections of [[botulinum toxin]], [[electroconvulsive therapy]], [[deep brain stimulation]], and surgical correction.<ref name=":3" /> Unfortunately, many of the elderly individuals affected by the BSS are not treated surgically due to age-related physical ailments and the long postoperative recovery period.<ref name="Azher" />

 

This condition can lead to excess pressure on the spine, causing pain and discomfort.<ref name=":2" /> If the spine is bent too far, a patient may have difficulties breathing because of the pressure of the spine pressed against the lungs. Camptocormia may also lead to muscle weakness in the upper back and to [[arthritis]] and other bone-degeneration diseases.<ref name=":2" /> Because of loss of bone strength, injury to the spine and slipped discs become increasingly significant. Camptocormia can lead to infection, tumors, and diseases of the endocrine system and connective tissues. The success of the treatment method is largely dependent on the patient, but response to therapeutic methods is generally low.

 

 

=== Deep brain stimulation ===

 

== References ==

 

[[Category:Aging-associated diseases]]