POLYNEURITIS (polyneuritis; grech, poly is a lot of + neuritis; synonym: multiple neuritis, peripheral symmetric neuritis) — the multiple defeat of peripheral nerves which is characterized by preferential symmetric sluggish paresis of muscles which was more expressed in distal departments of extremities, disturbance of a trophicity and disorders of sensitivity on peripheral type in the same zones. Distribution patol, process on motive craniocereberal is in some cases observed (cranial, T.) nerves.
Items conditionally subdivide on primary (spontaneously arising at people healthy before) and secondary (arising as a complication of various diseases). Secondary P. depending on etiol, a factor can be hypovitaminic (see. Beriberi ), alcoholic, diabetic (at a diabetes mellitus), toxic (at poisoning with arsenic, lead, etc.), infektsionnotoksichesky (at diphtheria, botulism, etc.), traumatic, infectious and allergic, autoallergichesky (at disturbances of exchange processes). In some cases P. is a consequence of long influence of adverse professional factors (e.g., vibrations, coolings, steady muscular stress, etc.). Many neuropathologists consider that in P.'s genesis the factor of an inflammation does not play a crucial role in this connection they call this disease a polyneuropathy (see. Neuropathy ).
In structure of the pathogenetic mechanism P. of various nature many processes — allergic, destructive, intoksikatsionny, infectious, etc. intertwine. A part is played by disturbances microcirculation (see), fabric exchange, immediate effect of toxic factors on nerve fibrils, autoallergichesky processes (see. Autoallergy ), conducting to demyelination, trophic disturbances, etc. Most often in P.'s pathogeny a part is played by not one any factor, but their various combination leading to a number of metabolic disturbances, development and strengthening of hypovitaminoses.
Researches of a number of neuropathologists revealed a possibility of development of P. owing to defeat of cells of front columns of a spinal cord. F. Raymond designated similar P. the term «tsellyulonevrit».
Despite P.'s polietiologichnost, its various forms the similar pathogenetic lines caused by hl have. obr. existence of demyelination, defeat of axial cylinders and others patomorfol, changes (see. Nerve fibrils ).
The experimental allergic polyneuritis was reproduced for the first time by Vaksman and Adams (V. of Waksman, R. Adams) after introduction to rabbits of tissue of homologous or heterological nerves with Freynd's stimulator (see. Adjuvants ). Experimental P. receive a lab at a large number. animals after the single intradermal injection not only nevritogenny suspension containing tissue of a nerve, but also the cleared myelin or its main protein.
After 2 — a 3 weeks incubation interval at animals defeats of a peripheral nervous system with sluggish paralyzes, an areflexia, ataxy, disorders of sensitivity develop. Nevrol, symptoms decide by development of periaksonalny (demyelinating) process on characteristic fine-grained disintegration of a myelin. Phagocytosis of decomposition products is carried out by monocytes and lemmocytes. Damage of both cranial, and spinal nerves, and also their nodes and roots is observed.
Some researchers [Lampert, Visnevski (R. of W. Lampert, H. M of Wisniewski)] consider that destruction of a myelin results from penetration of sensibilized mononuklear through a basal membrane of a lemmocyte to a normal myelin cover, to-ruyu they lyse. Other researchers [Yu. M. Zhabotinsky, V. I. Ioffe, Molnar (G. To. Mo1pyg)] believe that destruction of a myelin is caused by humoral factors (miyelinotoksichesky antibodies), perhaps, carrying out a role of a releaser, activating hydrolases. Inflammatory infiltration, in their opinion, should be considered as reactive process on primary changes in a myelin cover.
Miyelinotoksichesky factors cause destruction of a myelin in organotipichesky cultures of fabric of a peripheral nervous system and have diagnostic value. This model is used for studying immunol, reactions of the slowed-down and immediate type in development of the experimental allergic Item.
Introduction by an animal in an incubation interval of a disease of a number of drugs (AKTG, cyclophosphamide, antilimfotsi-tare serum) suppresses development of process. Lech. the nevritogenny main protein of a myelin used in mix with an incomplete stimulator of Freynd possesses action.
In patomorfol. P.'s relation divide on parenchymatous and intersticial. Among parenchymatous P. distinguish forms with preferential change of a myelin and lemmocytes (periaksonalny P.) and forms with dominance of damage of an axon (axonal P.). Intersticial P. are characterized by changes in covers and vessels of peripheral nerves. Forms with involvement of roots and spinal ganglions are called polyradiculoneurites. Depending on an etiology of process patomorfol, the picture has a number of differences. At the acute primary P. of infectious and allergic genesis which are clinically proceeding as ascending Landry's paralysis (see. Landry the ascending paralysis ) or Giyen's syndrome — Barret — Shtrolya, observes preferential defeat of myelin covers. At early stages of these diseases accumulation of mononuklear near myelin covers which are exposed to disintegration is observed. At the same time there is a proliferation of lemmocytes, and their number increases by 8 — 14 times. Myelin covers bulk up, get a sotovidny structure, there is a formation of myelin spheres and places — dissolution of a myelin. In the centers of demyelination which are especially located perivenozno focal necroses (fig. 1) with accumulation of the macrophages containing fat form. Inflammatory perivenous and perikapillyarny infiltrates consist of lymphoid cells, polymorphic mononuklear, macrophages (fig. 2). The axon increases in sizes, there is its tape-like swelling, on the course of an axon chetkoobrazny swellings form, there come lysis of fibrils and proliferation of lemmocytes (fig. 3). Death of nerve fibrils leads to development vallerovsky degenerations (see. Valera regeneration ). Owing to destruction of a myelin endoneural intervals are pulled together, and perineural spaces are expanded also infiltrirovana lymphoid elements. Inflammatory and demyelinating process at primary P. is reversible. Remyelination comes on 2 — 3rd week from an onset of the illness, as a rule, is followed by regeneration of nerve fibrils. In rare instances axons perish and on their place collagenic fibrilla and amorphous substance an endonevriya form.
At hypovitaminic, diabetic, toxic, porphyrinic, alcoholic P. preferential axonal defeat is observed, a cut is followed by a degeneration of axons, proliferation of lemmocytes, swelling of motive motor trailer plates. In nerve fibrils various stages of death of axons are traced, up to their total disappearance. The died on the spot nerve fibrils there are randomly located fibers an endonevriya, macrophages (fig. 4). Along with a degeneration of an axon it is possible to track processes of regeneration when the died on the spot fibers there is a formation of new small fibrils. In late stages of regeneration in myelin nerve fibrils separate axons reach a normal amount, their most part remains atrofichny. The axonal neuropathy is followed by a degeneration of a myelin, is more often in the form of segmented demyelination. Such mixed pathology is explained by involvement in process of lemmocytes and disturbance of process of a miyelinoobrazovaniye. At an ultrastructural research disturbance of metabolic processes in axons is shown by thinning of axoplasmatic organellas, vacuolation and a reduction of diameter of an axon, accumulation of a glycogen in lemmocytes.
Intersticial P. are observed at systemic infections: a viral hepatitis, a leprosy, shingles (see. Herpes ), etc., and also at arteritis, pseudorheumatisms, ischemia, an amyloidosis and other diseases. Patol. process at the same time is characterized by increase in endoneural collagen with formation of inflammatory changes in intersticial fabric of peripheral nerves. Moderate proliferation of lemmocytes is noted.
Morfol. differentiation of various forms P. presents considerable difficulties and shall be supplemented with electronic microscopic examinations of the material taken by a biopsy of cutaneous nerves (e.g., a sural nerve), with definition of nature of changes in an axon, lemmocytes and myelin covers. The particularly important becomes determination of caliber and density of fibers, their lengths between Ranvye's interceptions, and also morphometric assessment of the nerve fibrils deprived of a myelin.
The clinical picture
the Beginning of a disease is more often gradual. In some cases to P.'s development fever precedes various degree of manifestation, but quite often the disease develops against the background of the seeming wellbeing. Within several days, sometimes weeks at the patient symmetrically accruing paresis of muscles of distal departments of extremities with decrease in a tone and an atrophy is noted (see. Atrophy muscular ), with decrease or fading tendon jerks (see). In some cases preferential damage of muscles of proximal departments of extremities can be noted. Degree of manifestation of motive frustration is various — from slight paresis to paralyzes (see. Paralyses, paresis ). Development muscular is sometimes possible contractures (see) even at early stages of a disease. Quite often development of motive frustration is preceded by various disturbances of sensitivity on peripheral type in a look paresthesias (see), pains on the course of nervous trunks, decrease in sensitivity, sometimes hyperesthesias (see Sensitivity). Disorders of sensitivity have characteristic distal distribution in the form of «socks» and «gloves».
In some cases in the field of brushes and feet various trophic and vegetative disturbances in the form of dryness and thinning of skin, a peeling of epidermis, an akrogiper-hydrosis (see the Hyperhidrosis), fragilities of nails are noted. Function of pelvic bodies usually does not suffer; in some cases during the first two-three days of a disease the ischuria and a chair can be observed.
In cerebrospinal liquid (see) patol, changes, as a rule, are not observed. Also any specific changes are not noted and at a blood analysis though in some cases there can be a low leukocytosis.
At a research of electroexcitability partial or full reaction of degeneration and variously expressed reduction in the rate of carrying out an impulse on nervous trunks comes to light (see. Electromyography ). In process of recovery of motive functions data elektrofiziol. researches are gradually normalized.
The course of diseases is characterized by a considerable variety. In most cases since the end 2 — 3rd week of a disease there comes the slow involution of paresis beginning more often with the departments involved in process by the last.
Recovery of sensitivity usually begins earlier and happens quicker. There are atrophies and vegetative disturbances much longer. Duration of the recovery period sometimes drags on before half a year and more. Sometimes partial reduction, the resistant residual phenomena and contractures more expressed in distal departments of extremities, resulting in disability are noted. Possibly and a recurrent current with a periodic exacerbation and repeated increase of symptoms of a disease.
A special form is P. which is developed as ascending Landry's paralysis (see. Landry the ascending paralysis ). At the same time process proceeds very sharply, causes paralysis of legs. then trunks, hands, taking also cranial nerves. More often symmetrically is surprised facial nerve (see), quite often vagus nerve (see), glossopharyngeal nerve (see) etc. The ascending distribution of process can be followed by the heavy disturbances of breath demanding the emergency resuscitation actions.
Primary P.'s kind, at a cut not only peripheral nerves, but also spinal roots are surprised, the wedge, a syndrome for the first time described in 1916 by G. Guillain and Barra (J. А.Barre), and in 1917 — A. Strohl and called them a polyradiculoneuritis with proteinaceous and cellular dissociation in cerebrospinal liquid is. Sluggish paralyzes of top and bottom extremities with preferential defeat of proximal groups of muscles, smaller expressiveness of amyotrophy and sensitive frustration are characteristic of this syndrome. In hard cases muscles of a trunk and a neck can be surprised that leads sometimes to disturbance of breath.
Rather isolated group is made by the so-called vegetative P. which are characterized by sharp dominance of the trophic and vegetative disturbances usually more clearly expressed in upper extremities. These disturbances are shown by cyanosis of brushes and feet, a regional hyperemia of palms, an atrophy and thinning of skin or its hyperkeratosis, dryness or the increased perspiration of skin, puffiness of finger-tips, thinning of their skin, on a cut cracks can be observed. The atrophy of interosseous muscles of brushes is in some cases noted.
F. Raymond described the peculiar alcoholic defeat of peripheral nerves designated as «a cachectic form of a polyneuritis» (P.'s combination to quickly progressing general exhaustion of an organism) and the received name of a disease of F. Raymond. At hron, alcoholism P.'s combination to damage of a liver is sometimes observed and korsakovsky syndrome (see) — Klippel's syndrome.
Diagnosis establish on the basis a wedge, symptoms, data of the anamnesis and special researches. Differential diagnosis is carried out with myelitis (see), to acute epidemic poliomyelitis (see), a tick-borne encephalitis (see. viral tick-borne Encephalitises ), neural amyotrophy of Sharko — Mari (see. Amyotrophy ), miositis (see), hypertrophic neuritis of Dezherin — Sotta (see. Dezherina — Sotta hypertrophic neuritis ).
At differential diagnosis it is necessary to consider that, despite a certain community a wedge, manifestations, at nek-ry forms P. certain nerves are surprised. So, e.g., P. arising at diphtherias (see), it is characterized by preferential defeat of caudal group of cranial nerves, at botulism (see) the third cranial nerve more often suffers, at poisoning with arsenic and lead beam and fibular nerves are surprised more often.
Treatment in all cases complex, based on carrying out pathogenetic and causal treatment. Since in P.'s pathogeny the leading role belongs to autoimmune mechanisms, in perhaps earlier terms appoint glucocorticoids (Prednisolonum at the rate of 1 — 1,5 mg to 1 kg of weight of the patient within 7 — 14 days with the subsequent gradual slow decrease in a dosage by 5 mg each 3 days). At the same time appoint salts of potassium, a proteinaceous diet, high doses of vitamins of group B (B1, V2, V3, V6, V12, B15), and also ascorbic to - that. The antihistaminic drugs and drugs improving neuromuscular conductivity are used (e.g., prozerin, Galantaminum, etc.). Carry out disintoxication therapy (see) — dehydration in combination with hydration, enter Haemodesum, appoint cocarboxylase, ATP. At a vegetative form P. Ganglioblokiruyushchy means are shown. If necessary carry out treatment by antibiotics. At the ascending forms P. which are followed by heavy disturbance of breath performing resuscitation is shown (see. Artificial respiration ). At paralyzes the importance is attached to timely functional laying of extremities, use of massage and LFK (see. Paralyses, paresis, treatment ).
At all stages of recovery therapy widely apply methods of a fiziobalneoterapiya. For the purpose of reduction of pains locally apply sollyuks (see. Phototherapy ), novocaine electrophoresis (see. Electrophoresis ), inductothermy (see). Further appoint an electrophoresis of other pharmaceuticals (a prozerin, Galantaminum, iodine, oryzamin), carry out prozerin-diadinamoforez in combination with electrostimulation (see) the affected muscles. Widely use paraffin therapy (see), ozoceritotherapy (see) and mud cure (see), and also chamber hydroplating baths (see. Galvanization ). Prescribe patients with dominance of trophic frustration the general or local sulphidic bathtubs, and in the presence of a pain syndrome and disorders of sensitivity — radonic bathtubs (see. Bathtubs ). In necessary cases the dignity is carried out. - hens. treatment.
Forecast and Prevention
Forecast in most cases favorable. However sometimes at long disease the persistent paresis and contractures of muscles of extremities resulting in disability are possible. In the cases proceeding as ascending Landry's paralysis the lethal outcome is possible.
Prevention. P.'s Polietiologichnost does not allow to develop the general recommendations about their prevention; it is carried out taking into account features specific etiol, the factor causing P.'s development
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