Peripheral autonomic failure.

If the pathology of the suprasegmental section of the autonomic nervous system is represented mainly by a psychovegetative syndrome with permanent and paroxysmal autonomic disorders (vegetative crises, etc.), then the damage to the segmental (peripheral) apparatuses of the autonomic nervous system is predominantly organic in nature and manifests itself as a complex of various visceral disorders and autonomic disorders in the extremities. The term "peripheral autonomic insufficiency" refers to a complex of autonomic manifestations that occur with damage (usually organic) to the peripheral (segmental) section of the autonomic nervous system. The ideas about the pathology of the segmental autonomic nervous system have changed significantly. Until recently, various infections (ganglionitis, truncitis, solaritis, etc.) were primarily seen as its cause. At present, it is obvious that the role of infections is quite moderate; the cause of pathology is primarily endocrine, systemic and metabolic diseases.

Classification of peripheral autonomic failure

In the modern classification of autonomic disorders, primary and secondary peripheral autonomic failure are distinguished.

Classification of peripheral autonomic failure [Vein A M., 1991]

Primary peripheral autonomic failure

1. Idiopathic (isolated, “pure”) autonomic failure (Strongradbury-Egglestone syndrome).
2. Peripheral autonomic failure plus multiple system atrophies (Shy-Drager syndrome).
3. Peripheral autonomic failure plus clinical manifestations of parkinsonism
4. Familial dysautonomia (Raily-Dey);
5. Other hereditary autonomic 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, Guillain-Barré syndrome).
3. Metabolic disorders (alcoholism, porphyria, hereditary beta-lipoprotein deficiency, uremia).
4. Drug intoxications (dopa-containing drugs, alpha- and beta-blockers, anticholinergic drugs, etc.).
5. Toxic lesions (vincristine, arsenic, lead).
6. Damage to the autonomic nervous system of the brainstem 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 autonomic failure are chronic slowly progressive diseases. They are based on degenerative damage to segmental autonomic apparatuses in isolation ("pure" peripheral autonomic failure) or in parallel with the degenerative process in other structures of the nervous system (in combination with MSA, Parkinsonism). Only in relation to the above-mentioned primary forms of peripheral autonomic failure is the term "progressive autonomic failure" sometimes used, implying the chronic progressive nature of the course of these diseases. Primary forms also include damage to the peripheral autonomic nervous system within the framework of some forms of hereditary polyneuropathies, when autonomic neurons are affected along with motor or sensory fibers (HMSN - hereditary motor-sensory neuropathy, HSVN - hereditary sensory-autonomic neuropathy). The etiology of primary forms of peripheral autonomic failure remains unclear.

Secondary peripheral autonomic failure develops against the background of a current somatic or neurological disease. These forms of peripheral autonomic disorders are based on pathogenetic mechanisms inherent in the patient's disease (endocrine, metabolic, toxic, autoimmune, infectious, etc.).

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