Classification of autonomic disorders

Nothing is more difficult than creating medical classifications. They should be scientifically grounded, convenient for the practical doctor, created according to certain principles. Features of clinical vegetology exacerbate common difficulties, since most often - it's syndromes that occur in various diseases. It is also difficult that we can not enjoy the fruits of our predecessors. In the world and national literature there are no detailed and complete classifications of vegetative disorders. In fact, when discussing the work of our predecessors, we can catch a certain principle of the classification. In the domestic literature, the topical principle dominated: cortical, subcortical, diencephalic, stem, spinal, sympathetic, plexus, peripheral nerves lesions. Separately described vegetative manifestations with neuroses (GI Markelov, AM Grinshtein, II Rusetsky, NS Chetverikov). Vegetative syndromes were also described as manifestations of vegetative regulation disorders of individual systems - cardiac, respiratory, gastrointestinal, urogenital, etc. [Greenstein A. Mi, Popova NA, 1971, and others]. R. Bannister has created a classification of the syndrome of progressive autonomic failure. When there are no full-scale classifications of a particular area of pathology, a legitimate question may arise: is there a real need for this? We do not have any doubts about the need, and only with great objective difficulties we explain the absence of even attempts to create a universal full rubrication.

Now about the principles that form the basis of classification. Ideally, it should be formed using a single principle. However, we did not succeed, and we had to use several approaches. The first of these is the division of the pathology of supra-segmental and segmental autonomic disorders. They are fundamentally different in their pathogenesis (this will be discussed in the corresponding section), and, what is especially important, in the main clinical manifestations. The basis of supra-segmental disorders is made up of various variants of the psycho-vegetative syndrome. Segmental disorders are manifested by the syndrome of progressive vegetative insufficiency (when visceral vegetative fibers are involved in the process) and vegetative-vascular trophic disorders on the hands and feet (with the interest of autonomic fibers of spinal cord, plexus and peripheral nerves). However, quite often, as it happens in medicine, there are mixed syndromes, which combine nassegmental and segmental vegetative disorders.

The second principle is the primary and secondary nature of vegetative disorders. And this question is not simple for permission. Most often, vegetative disorders are syndromes of various diseases and, thus, are secondary. And yet we identified the situation when a nosological characteristic of vegetative disorders is possible.

Supra-segmental (cerebral) vegetative disorders

Syndrome of vegetative dystonia of permanent and (or) paroxysmal nature, generalized and (or) local, manifested mainly psycho-vegetative and neuroendocrine syndromes.

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

Segmental (peripheral) vegetative disorders

Syndrome of vegetative dystonia of 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, hypothyroidism, hyperthyroidism, hyperparathyroidism, Addison's disease, etc.).
  • Systemic and autoimmune diseases (amyloids, rheumatism, scleroderma, Guillain-Barre disease, myasthenia gravis, rheumatoid arthritis).
  • Metabolic disorders (porphyria, hereditary beta-lipoprotein insufficiency, Fabry's disease, cryoglobulinemia).
  • Vascular diseases (arteritis, arteriovenous aneurysms, vascular obliteration, thrombophlebitis, vascular insufficiency).
  • Organic diseases of the brainstem and spinal cord (syringomyelia, tumors, vascular diseases).
  • Carcinomatous autonomic neuropathies.
  • Infectious lesions (syphilis, herpes, AIDS).
• Combined nonspecific and segmental autonomic disorders
  • Primary (manifested primarily by the syndrome of progressive autonomic failure (PVN)).
    • Idiopathic (IVF).
    • Multiple system atrophy and PID.
    • Parkinsonism and PVN.
    • Family disautonomy (Riley - Dey).
  • Secondary
    • Somatic diseases that involve in the process both supra-segmental and segmental vegetative systems.
    • A combination of somatic and mental (in particular, neurotic) disorders.

It is necessary to give the necessary explanations. This requires the classification itself because of the contradictions that remain within it, which we have not overcome until the end.

Let's start with the primary super-segmental disorders. It seems that constitutional disorders, having a family character and manifesting from an early age, will not cause special discussions. It is more difficult with the second point, however, rather not in essence, but 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, then such states are designated as psychophysiological and refer to primary. There is no doubt that under certain conditions in the future these disorders can fundamentally develop into a certain psychosomatic disease. Hence, the importance of timely identification of these conditions and active intervention for the prevention of 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: pseudomigrogenic attacks - for brain tumors or vertebrogenic pathology, Raynaud's syndrome - in scleroderma, erythromelalgia syndrome - in systemic autoimmune diseases .

Secondary supra-segmental vegetative disturbances are more obvious. The neurotic disorders are dominant, in which vegetative manifestations are obligatory. Among psychic syndromes, a special place is occupied by anxiety-depressive disorders. The group of organic diseases of the brain includes the so-called hypothalamic syndromes with leading neuroendocrine disorders. In the picture of psychosomatic diseases, there is always a different intensity of psycho-vegetative syndrome, which forms the pathogenetic basis of these diseases. It is clear that the vegetative disorders are related to hormonal perturbations, that is, pathological manifestations of puberty and menopause.

Among the segmental vegetative disorders, we practically did not single out the primary ones, it is essentially about somato-neurologic syndromes. Exception was made only by genetically conditioned 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 peripheral segmental vegetative apparatuses. Among the rarer, amyloidosis, in which peripheral vegetative insufficiency is found in 80% of cases. A very modest place is occupied by infections, which makes the term "neuropathy" more optimal than "neuritis."

There is an undoubted need to designate a division of the combined supra-segmental and segmental disorders. The primary group includes a group of diseases manifested by the syndrome of progressive autonomic failure, one of the bright signs of which is orthostatic hypotension. It is based on a degenerative lesion of cerebral systems and peripheral vegetative neurons.

Secondary combined disorders are also obvious. This, first, simultaneous damage, for example, in systemic diseases, nasegmental and segmental systems; secondly, the probability of mental reactions to a physical illness.

The proposed classification seems to be realistic and convenient for clinical practice, it formed the basis for the clinical and pathogenetic concepts of the book. At the same time, we are sure that the work on creating the classification is not completed and will continue. 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]