Classification of vegetative disorders

There is nothing more difficult than creating medical classifications. They must be scientifically substantiated, convenient for a practicing physician, created according to certain principles. The peculiarities of clinical vegetology aggravate the general difficulties, since most often these are syndromes that arise in various diseases. It is also difficult that we cannot use the fruits of our predecessors. In the world and domestic literature there are no detailed and complete classifications of vegetative disorders. In essence, discussing the works of our predecessors, we can grasp a certain principle of rubrication. In domestic literature, the topical principle dominated: lesions of the cortex, subcortical, diencephalic, stem, spinal, sympathetic chain, plexuses, peripheral nerves. Vegetative manifestations in neuroses were described separately (G. I. Markelov, A. M. Grinshtein, I. I. Rusetsky, N. S. Chetverikov). Vegetative syndromes were also described as manifestations of disorders of the vegetative regulation of individual systems - cardiac, respiratory, gastrointestinal, genitourinary, etc. [Grinshtein A. Mi, Popova N. A., 1971, and others]. R. Bannister created a classification of the syndrome of progressive vegetative failure. When there are no complete detailed classifications of a certain area of pathology, a legitimate question may arise: is there a real need for this? We have no doubts about the need, and only by great objective difficulties can we explain the absence of even attempts to create a universal complete rubrication.

Now about the principles that underlie the classification. Ideally, it should be formed using one principle. However, we failed to do so, and had to use several approaches. The first of them is the division of the pathology of suprasegmental and segmental vegetative disorders. They differ fundamentally in their pathogenesis (this will be discussed in the relevant section), and, most importantly, in their main clinical manifestations. The basis of suprasegmental disorders is made up of various variants of psycho-vegetative syndrome. Segmental disorders are manifested by the syndrome of progressive vegetative insufficiency (with the involvement of visceral vegetative fibers in the process) and vegetative-vascular-trophic disorders in the arms and legs (with the involvement of vegetative fibers of the spinal cord roots, plexuses and peripheral nerves). However, often, as happens in medicine, there are also mixed syndromes that combine suprasegmental and segmental autonomic disorders.

The second principle is the primary and secondary nature of vegetative disorders. And this issue is not easy to resolve. Most often, vegetative disorders are syndromes of various diseases and, thus, are secondary. And yet, we have identified situations when the nosological characteristics of vegetative disorders are possible.

Suprasegmental (cerebral) autonomic disorders

A syndrome of vegetative dystonia of a permanent and/or paroxysmal nature, generalized and/or local, manifested mainly by psychovegetative and neuroendocrine syndromes.

• Primary
  • Vegetative-emotional syndrome of constitutional nature.
  • Vegetative-emotional syndrome (reaction) to acute and chronic stress (psychophysiological vegetative dystonia).
  • Migraine.
  • Neurogenic syncope.
  • Raynaud's disease.
  • Erythromelalgia.
• Secondary
  • Neuroses.
  • Mental illnesses (endogenous, exogenous, psychopathy).
  • Organic diseases of the brain.
  • Somatic (including psychosomatic) diseases.
  • Hormonal changes (puberty, menopause).

Segmental (peripheral) autonomic disorders

A syndrome of vegetative dystonia of a permanent and/or paroxysmal nature, generalized and/or local, manifested by a syndrome of progressive vegetative insufficiency and vegetative-vascular-trophic disorders in the extremities.

• Primary
  • Hereditary neuropathies (sensory, Charcot-Marie-Tooth).
• Secondary
  • Compression lesions (vertebrogenic, tunnel, additional ribs).
  • Endocrine diseases (diabetes mellitus, hypothyroidism, hyperthyroidism, hyperparathyroidism, Addison's disease, etc.).
  • Systemic and autoimmune diseases (amyloidosis, rheumatism, scleroderma, Guillain-Barré disease, myasthenia, rheumatoid arthritis).
  • Metabolic disorders (porphyria, hereditary beta-lipoprotein deficiency, Fabry disease, cryoglobulinemia).
  • Vascular diseases (arteritis, arteriovenous aneurysms, vascular obliterations, thrombophlebitis, vascular insufficiency).
  • Organic diseases of the brainstem and spinal cord (syringomyelia, tumors, vascular diseases).
  • Carcinomatous autonomic neuropathies.
  • Infectious lesions (syphilis, herpes, AIDS).
• Combined suprasegmectal and segmental autonomic disorders
  • Primary (manifested primarily by progressive autonomic failure syndrome (PAFS)).
    • Idiopathic (PVN).
    • Multiple system atrophy and PVN.
    • Parkinsonism and PVN.
    • Familial dysautonomia (Riley - Deja).
  • Secondary
    • Somatic diseases that simultaneously involve the suprasegmental and segmental autonomic systems.
    • A combination of somatic and mental (in particular, neurotic) disorders.

The necessary explanations should be given. This is required by the classification itself due to the contradictions remaining within it, which we have not fully overcome.

Let's start with primary suprasegmental disorders. It seems that constitutional disorders that run in families and manifest themselves from an early age will not cause any particular discussions. The second point is more difficult, although not in essence, but rather in connection with its unconventionality. Vegetative disorders are clearly manifested in acute and chronic emotional stress, and since at a certain stage the disease is absent, such conditions are designated as psychophysiological and are considered primary. There is no doubt that under certain conditions these disorders can in principle develop into a certain psychosomatic disease. Hence the importance of timely identification of these conditions and active intervention in order to prevent organic diseases.

The next group consists of vascular-vegetative diseases: migraine, neurogenic syncope, Raynaud's disease, erythromelalgia. It would seem that there are no problems here, but they consist in the fact that often these forms of pathology are not idiopathic diseases, but syndromes: pseudo-migraine attacks - with brain tumors or vertebrogenic pathology, Raynaud's syndrome - with scleroderma, erythromelalgia syndrome - with systemic autoimmune diseases.

Secondary suprasegmental vegetative disorders are more obvious. Neurotic disorders, in which vegetative manifestations are obligatory, dominate. Among mental syndromes, anxiety-depressive syndromes occupy a special place. The group of organic diseases of the brain also includes the so-called hypothalamic syndromes with leading neuroendocrine disorders. In the picture of psychosomatic diseases, there is always a psychovegetative syndrome of varying intensity, which is the pathogenetic basis of these diseases. The connection between vegetative disorders and hormonal perturbations, i.e. pathological manifestations of puberty and menopause, is clear.

Among segmental vegetative disorders, we have practically not identified primary ones; we are essentially talking about somato-neurological syndromes. The only exceptions were genetically determined forms. I would like to highlight certain "leaders". In connection with the frequency and prevalence of vertebrogenic and endocrine (primarily diabetes mellitus) forms, they are the leading factors affecting the peripheral segmental vegetative apparatus. Among the rarer ones, amyloidosis should be noted, in which peripheral vegetative insufficiency is detected in 80% of cases. Infections occupy a very modest place, which makes the term "neuropathy" more optimal than "neuritis".

There is an undoubted need to designate the section of combined suprasegmental and segmental disorders. Primary disorders include a group of diseases manifested by the syndrome of progressive vegetative failure, one of the striking signs of which is orthostatic hypotension. It is based on degenerative damage to the cerebral systems and peripheral vegetative neurons.

Secondary combined disorders are also obvious. Firstly, this is simultaneous damage, for example in systemic diseases, to suprasegmental and segmental systems; secondly, the likelihood of mental reactions to somatic disease.

The proposed classification seems realistic and convenient for clinical practice, it formed the basis of the clinical and pathogenetic concepts of the book. At the same time, we are sure that the work on creating the classification is not finished and will be continued. Only a certain stage of progress along the path of studying the pathology of the autonomic nervous system has been recorded.

[ 1 ], [ 2 ], [ 3 ], [ 4 ], [ 5 ], [ 6 ], [ 7 ]