Pathologic forward tilt of the torso

Pathological forward bending of the trunk (camptocormia in a broad sense) can be permanent, periodic, paroxysmal, rhythmic ("bows"). It can cause pain, postural instability, cause or aggravate dysbasia, lead to falls. It is sometimes very difficult to determine the nosological affiliation of this postural syndrome, especially when it is the only or main manifestation of the disease. Forward bending of the trunk is always a symptom, not a disease. Therefore, identifying other symptoms against which the forward bending of the trunk appeared is often the key to diagnosis. Sometimes the situation is complicated by the fact that this phenomenon develops against the background of not one, but two (or more) neurological diseases. For diagnosis, it is important to distinguish between permanent (and progressive) forward bending of the trunk and transient-episodic bending.

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I. Constant and progressive forward bending of the trunk

A. Diseases of the spine and large joints.

B. Postural disorders in late stages of Parkinson's disease and parkinsonism.

C. Progressive weakness of the trunk extensor muscles:

1. Myopathy.
2. Amyotrophic lateral sclerosis.
3. Progressive spinal amyotrophy.
4. Dermatomyositis and poliomyositis.
5. Glycogenosis, type 2.
6. Carnitine deficiency.

D. Tilted spine syndrome in the elderly.

II. Transient episodic and repetitive forward bending of the trunk

A. Spasm of the trunk flexor muscles:

1. Axial dystonia.
2. Paroxysmal dystonia.
3. Myoclonus of the axial muscles of the trunk.
4. Epilepsy.
5. Neuroleptic syndrome.

B. Forward bending of the trunk in the picture of mental (psychogenic and endogenous) diseases:

1. Psychogenic camptocormia.
2. Periodic bowing in the picture of conversion or compulsive disorders.
3. Stereotypes in mental illness.
4. Depression in endogenous mental illnesses.

C. Forward bending of the torso as a compensatory (voluntary) reaction to the threat of falling:

1. Transient weakness in the legs with transient spinal circulatory insufficiency.
2. Lipothymic conditions in the picture of orthostatic circulatory disorders, including progressive autonomic failure (walking in the “skater” pose).

I. Constant and progressive forward bending of the trunk

A. Diseases of the spine and large joints

Diseases of the spine and large joints are usually accompanied by pain syndrome and (or) form a mechanical cause of the trunk tilt. Vertebral syndrome occurs. (Pathological kyphosis and skeletal deformities in spondylitis, ankylosing spondylitis, injuries, tumors and congenital diseases of the spine, coxarthrosis, rheumatoid arthritis, reflex muscular-tonic syndromes).

The diagnosis is confirmed by neuroorthopedic, radiological or neuroimaging studies.

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B. Postural disturbances in late stages of Parkinson's disease and parkinsonism

Standing and walking in a "flexor posture", accelerated step with a forward bend of the body in the context of other manifestations of Parkinsonism (hypokinesia, resting tremor, muscle rigidity, postural disorders). A combination of both of the above causes (joint diseases and Parkinsonism) is possible.

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C. Progressive weakness of the extensor muscles

Myopathy involving the pelvic girdle muscles and paraspinal muscles is mentioned here rather conditionally, since it is not the entire torso that is tilted forward, but only the pelvis. The patient's pelvis tilts forward due to the weakness of the extensors, and the patient, in order to maintain an upright posture, tilts backward, forming hyperlordosis. In fact, the torso is constantly tilted backward (hyperextension). Without such compensation, the torso would be constantly tilted forward.

Other diseases accompanied by weakness of the trunk extensor muscles, such as amyotrophic lateral sclerosis (proximal forms or rare onset of the disease with weakness of the back extensor muscles); progressive spinal amyotrophy; dermatomyositis; glycogenosis (type 2, Pompe disease); carnitine deficiency - for the same reasons are rarely accompanied by a constant forward bend of the trunk. Patients experience difficulties in straightening the trunk (for example, after bending over to pick something up) and help themselves with "myopathic techniques".

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D. Tilted spine syndrome in the elderly

This syndrome is observed only when standing and walking in people over 60 years of age ("strong spine syndrome"). The syndrome should be differentiated from vertebral syndrome (kyphosis), but passive extension of the trunk in these patients is normal. Some patients may experience pain in the lumbar region, but it is transient and usually passes spontaneously as the disease progresses. CT of the paraspinal muscles reveals hypodensity (decreased muscle density). Sometimes a slight increase in CPK is possible. EMG shows nonspecific, weak signs of myopathy (not in all patients). The disease is slowly progressive. Its nature and nosological independence have not been fully established.

II. Transient episodic and repetitive forward bending of the trunk

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A. Spasm of the trunk flexor muscles

Dystonia (torsion spasm) axial sometimes manifests itself as a persistent postural defect (torso flexion) - dystonic camptocormia. This dystonic syndrome often presents great difficulties for its diagnostic interpretation. Here it is important to search for the dynamism of symptoms that is characteristic of dystonia (dependence of torsion spasm on changes in body position, time of day, rest - activity, the effect of alcohol, corrective gestures, paradoxical kinesia) and to exclude other possible causes of postural disorder.

Trunk tilts ("bows") in the picture of paroxysmal dystonia attacks. Paroxysmal dystonia (kinesiogenic and non-kinesiogenic) is extremely rarely manifested by this form of attacks, and if it is manifested, then always in the context of other, fairly typical manifestations (short, usually movement-induced, dystonic postures in the limbs, not accompanied by impaired consciousness with a normal EEG).

Myoclonus of the trunk flexor muscles has a syndromic appearance that is difficult to confuse with any other syndrome. These are short, fast, jerky flexion movements of the trunk, usually of small amplitude, stereotypical. Visually, short contractions of the abdominal press are sometimes visible, synchronous with flexion movements of the upper half of the trunk. Full-scale flexion does not have time to develop here, there is only a hint of it. The source of myoclonus and its nature need to be clarified in each individual case (spinal myoclonus, startle reactions, etc.). It is necessary to exclude the epileptic nature of myoclonus.

Epilepsy (infantile spasms, some seizures in supplementary epilepsy) sometimes manifests itself with rapid flexion movements or slower postural (including flexion) phenomena. Persistent searches for other clinical and EEG signs of epilepsy are necessary (prolonged and deep hyperventilation, sleep deprivation at night, polygraphic recording of sleep at night, video recording of seizures).

"Pseudosalam convulsions" in the picture of acute dystonic reactions (neuroleptic syndrome) develop acutely in response to the administration of a neuroleptic and are usually accompanied by other dystonic phenomena (oculogyric crises, blepharospasm, trismus, protrusion of the tongue, dystonic spasms in the limbs, etc., relieved by anticholinergics or spontaneously stopping when the neuroleptic is discontinued).

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B. Forward bending of the trunk in the picture of mental (psychogenic and endogenous) disorders

Psychogenic camptocormia is characterized by a typical posture in the form of a body bent forward at a right angle with freely hanging arms (“anthropoid posture”) and is most often observed in the picture of polysyndromic hysteria (multiple movement disorders, sensory, autonomic and emotional-personality disorders).

Periodic bowing in the picture of conversion or compulsive disorders is a type of camptocormia, characterized by paroxysmal manifestations and usually observed in a picture of bright demonstrative manifestations reminiscent of a pseudo-seizure.

Stereotypes in mental illnesses can take on a variety of forms, such as elementary meaningless movements, including stereotypically repeated torso tilts. Stereotypes can also have a neuroleptic origin ("tardive stereotypes").

Severe depression in endogenous mental illnesses is characterized by reduced efficiency, hypomimia, psychomotor retardation, and a hunched posture in the picture of other emotional, cognitive, and behavioral manifestations of mental illness. Here we are not talking about a pronounced bending of the trunk, but rather about a hunched (slouched) "lowered" posture. There is no camptocormia as such here.

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C. Forward bending of the trunk as a compensatory (voluntary) reaction to the threat of falling

Transient weakness in the legs with spinal circulatory insufficiency may be accompanied by flexion of not only the legs, but also the trunk, and is part of the picture of "myelogenous intermittent claudication" (transient weakness in the legs, often provoked by walking, with a feeling of heaviness and numbness in them), usually against the background of a systemic vascular disease. Bending of the trunk here is a reflex or voluntary reaction aimed at maintaining balance and stability of the body, preventing injuries from falling.

Lipothymic conditions in the picture of orthostatic circulatory disorders, especially with progressive autonomic failure, may be accompanied by persistent arterial hypotension with constant dizziness and a real threat of postural syncope. The presence of pyramidal, extrapyramidal and cerebellar signs (for example, in the picture of Shy-Drager syndrome) increases postural instability and can lead to a characteristic dysbasia in the "skater's pose" (tilt of the head and body forward; walking with wide, slightly to the side, steps).

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