Peripheral autonomic failure

If the pathology of the suprasegmental department of the autonomic nervous system is represented mainly by the psychovegetative syndrome with permanent and paroxysmal vegetative disorders (vegetative crises, etc.), then the defeat of segmental (peripheral) apparatuses of the autonomic nervous system is mainly organic in nature and manifests itself as a complex of various visceral disorders and vegetative disorders on extremities. By the term "peripheral vegetative failure" is meant a complex of vegetative manifestations that arise when the peripheral (segmental) segment of the vegetative nervous system is affected (usually organic). Views on the pathology of the segmental autonomic nervous system have changed significantly. Until recently, various infections (ganglionitis, trunkite, solarite, etc.) were seen primarily as its cause. At present, it is obvious that the role of infections is very moderate, the cause of pathology is primarily endocrine, systemic and metabolic diseases.

Classification of peripheral autonomic failure

In the modern classification of vegetative disorders, primary and secondary peripheral vegetative failure are isolated ..

Classification of peripheral vegetative failure [Vein AM, 1991]

Primary peripheral autonomic failure

1. Idiopathic (isolated, "clean") autonomic failure (strongradbury-Egglestone syndrome).
2. Peripheral autonomic failure plus multiple systemic atrophies (Shy-Drager syndrome).
3. Peripheral autonomic failure plus clinical manifestations of Parkinson's disease
4. Family disautonomy (Raily-Dey);
5. Other hereditary vegetative neuropathies (with NSVN and NMSN).

Secondary peripheral autonomic failure

1. Endocrine diseases (diabetes mellitus, hypothyroidism, adrenal insufficiency).
2. Systemic and autoimmune diseases (amyloidosis, scleroderma, myasthenia gravis, Guillain-Barre syndrome).
3. Metabolic disorders (alcoholism, porphyria, hereditary beta-lipoprotein insufficiency, uremia).
4. Drug intoxication (dopasoderzhaschie drugs, alpha and beta adrenoblockers, anticholinergic drugs, etc.).
5. Toxic lesions (vincristine, arsenic, lead).
6. The defeat of the autonomic apparatus of the trunk and spinal cord (syringobulbia, syringomyelia, spinal cord tumors, multiple sclerosis).
7. Carcinomatous lesions, paraneoplastic syndromes.
8. Infectious lesions of the peripheral autonomic nervous system (AIDS, herpes, syphilis, leprosy).

Primary forms of peripheral vegetative failure are chronic slow-progredient diseases. They are based on the degenerative lesion of segmental vegetative apparatuses in isolated form ("pure" peripheral vegetative insufficiency) or parallel to the degenerative process in other structures of the nervous system (in combination with MCA, Parkinsonism). Only in relation to the above-mentioned primary forms of peripheral vegetative insufficiency is sometimes used the term "progressive vegetative failure", implying the chronic progredient nature of the course of these diseases. Primary forms include the defeat of the peripheral autonomic nervous system in some forms of hereditary polyneuropathies, when along with motor or sensory fibers vegetative neurons suffer (NMSN - hereditary motor-sensory neuropathy, NSVN - hereditary sensory-vegetative neuropathy). The etiology of the primary forms of peripheral vegetative insufficiency remains unrecognized.

Secondary peripheral vegetative failure is formed against the background of a current somatic or neurological disease. At the heart of these forms of peripheral vegetative disorders lie the pathogenetic mechanisms inherent in the disease occurring in the patient (endocrine, metabolic, toxic, autoimmune, infectious, etc.).

[1], [2], [3], [4], [5],