|Table of contents
Acute inflammatory polyradiculoneuropathy
Chronic inflammatory demyelinating polyradiculoneuropathy
Immunopathology of liquor and blood
Undifferentiated therapy of polineyropatiya
The differentiated therapy of polineyropatiya
Page 6 of 11
5.3. Toxic polyneuropathy
This group allocates to polyneuropathy, caused by the exogenous intoxications and endogenous toxic substances which are formed in an organism as a result of various frustration of a metabolism. Exogenous intoxications in modern conditions are important. Contacts of the person with toxics as a result of environmental pollution and their accumulation in food stuffs, and also as a result of broad consumption of medicines, development of the new, sanitarily not studied technology processes extend. Keep the activity and infections implementing neurotropic action through toxin. Allocate acute and chronic forms of toxic polineyropatiya.
The pathogeny in case of endogenous intoxications is developed insufficiently. It is difficult to define when damage of a nerve is caused by directly exchange defects and when — effect of the toxins which are formed as a result of these disturbances.
5.3.1 Toxic polyneuropathy, caused by exogenous intoxications
Diphtheritic polyneuropathy — the toxic peripheral miyelinopatiya which is characterized by periaksialny segmented demyelination of nervous trunks and ventral roots at an intact axon. PNP is typical complication of diphtheria, developing against a severe toxic form of an infection, a thicket at adults, than at children. The Neyropatichesky syndrome mixed there are motive, sensitive, vegetative frustration.
Distinctive feature - damage of cranial nerves at the early paralyzes developing between the 3rd and 14th day of a basic disease (an early form) or on the 4-7th weeks (a late form). In clinic paresis of an abductor of an eye, unstriated muscles (accommodation paralysis), and especially the muscles innervated glossopalatine and wandering nerves (a rhinolalia, an aphonia, swallowing disturbance, sometimes tachycardia) are typical. Involvement of a phrenic nerve is dangerous. Often observe amyotrophic and pseudo-tabic (atactic) disease forms.
In most cases the forecast favorable, paresis regresses in 2-4 weeks, sometimes within months. Resuming of weakness of skeletal muscles and the delayed development of symptomatology on 4-10 week of a disease is possible. The early period of polyneuropathy with threat of a cardiac standstill as a result of defeat of cordial branches of a vagus nerve or heavy aspiration pneumonia in connection with a dysphagia is dangerous. The disease is caused neurotropic effect of exotoxin — the causative agent of diphtheria.
Herpetic defeat of a peripheral nervous system
At viral diseases of polyneuropathy, as a rule, develop in the postinfectious period. Defeat is provided to a thicket immunoallergichesk caused by a polyradiculoneuropathy, is more rare a polyneuritis or a mononeuritis. The leading role belongs to viruses of family of herpes: herpes simplex virus type I and II, virus of chicken pox (varitselliya-zoster), Epstayna-Barra virus and cytomegalovirus. Primary infection occurs at children's age and leads to immunity development. The virus can persistirovat in spinal or cranial gangliya in a latent state until reactive ability of an organism is kept, however, proves in cases of the expressed decrease in immunity. Defeats in a type of ballooning colliquation and intranuclear inclusions with limfoplazmotsitarny infiltrates are observed, mainly, in neurons spinal gangliyev and back horns of a spinal cord. There are several clinical options, but in all cases neurologic signs and damages of skin are present jointly at limits of the same dermatome (more often in a zone of chest dermatomes, also necks are more rare on head skin).
Herpetic radiculoneuropathies are caused by a herpes simplex virus like I. Rashes in a face, on a mucous membrane of a nasopharynx and head skin are characteristic. Pains and paresthesias are noted in the dermatomas corresponding to distribution of rashes. The herpes simplex virus like II causes the radiculoneuropathy which is followed by rashes in genitals and a crotch and also pains, a hyperesthesia, a hyperpathia which area can be more site of rashes (a stomach, buttocks, legs).
Except neuropathy of cranial nerves (ophthalmic, ear, glossopalatine shingles) and polyradiculoneuropathies, herpetic defeat it can be shown by multiple neyropatiya. Motive frustration as sluggish paralysis with an atrophy of muscles develop after rashes in terms from several days to one month. The lower extremities are involved less than upper extremities and an abdominal wall. Sensitive frustration have radicular or neural distribution which can not match topography of rashes. The pathomorphologic picture represents inflammatory defeat of roots, spinal gangliyev and nerves. Asymmetric distribution of these frustration distinguishes them from a polyradiculoneuropathy.
Defeat of a peripheral nervous system at HIV - an infection
AIDS is the disease caused by a human immunodeficiency virus of the 1st type (VICh-1) is more rare - 2-go types (VICh-2). Neurologic disturbances occur at a half or 2/3 patients. They can arise in any stage of a disease, but a thicket - in the last.
The reasons of primary neurologic complications of AIDS are various: the direct damaging action of TsNS a virus, autoimmune processes, neurotoxic effect which is rendered by both virus components, and products of immune responses.
Secondary complications are connected with opportunistic infections and tumors, and also neurotoxic action of antiviral means. HIV - a neurotropic and lymphotropic virus is allocated from SMZh and a brain of patients with AIDS with various neurologic syndromes. It gets into a nervous system in an early phase of a disease and remains in macrophages in a latent state.
First HIV infection from a nervous system proceeds asymptomatically. However, in some cases neurologic disturbances happen the first displays of AIDS, arising during seroconversion when immunological indicators are still normal. Treat such disturbances: HIV encephalopathy, acute aseptic meningitis, neuropathy of separate cranial nerves, and also the acute ascending or cross myelitis. They are representatives of an acute infection.
Approximately at 30% of patients with AIDS at heavy disturbance of immunity AIDS - associated kognitivno - a motive complex, or AIDS - a dementive syndrome develops. It are shown by the progressing dementia of subcrustal type, sometimes with disturbance of balance and weakness in legs.
The recurrent or chronic irritation of a meninx can be followed by neuropathy of cranial nerves, especially V, VII, VIII couples. The HIV myelipathy (a vakurlyarny myelipathy) clinically reminds a funicular myelosis at deficit of B12 vitamin. Neuropathy (see tab. 3) at HIV infection meet quite often. However, they are still badly studied. Their reasons are various. Some neuropathy are probably caused by immunological reactions, others (the polyradiculopathy which is especially progressing lumbar krestsovaya) - consecutive infection. Toxic neuropathy are often observed at use of anti-retrovirus drugs (a didanozin or a zaltsitabin), the chemotherapeutic means used for treatment of sarcoma of Kaposha.
Approximately at 1/4 patients with AIDS distal symmetric neuropathy develops. Pain and decrease in sensitivity in feet are characteristic. Also multiple mononeuropathy owing to ischemic damage of peripheral nerves is possible. Often the clinical picture of a syndrome to Geyena-Barra and chronic inflammatory demyelinating polyneuropathy with a pleocytosis develops in SMZh. The lumbosacral polyradiculopathy is sometimes directly connected with HIV infection. In this case it comes to an end with spontaneous remission. More often it is curable, but potentially lethal complication of an opportunistic tsitomegalovirusny infection. Subacutely the lower paraparesis which sometimes is followed by a dorsodynia, radicular pains develops. Often soon dysfunctions of pelvic bodies join. Tendon jerks standing decrease or drop out. Anesthesia of a crotch is possible. In SMZh, unlike many other displays of AIDS, at this syndrome characteristic changes come to light: a high pleocytosis (more than 500 in 1 mkl) in which neutrophils often prevail (but not always); concentration of protein is increased; glucose level within norm or is a little reduced. From SMZh it is possible to allocate a cytomegalovirus.
The AIDS-dementive syndrome, recurrent or chronic irritation of a meninx, HIV - a myelipathy, neuropathy, a lumbosacral polyradiculopathy are representatives of chronic HIV infection.
Exogenous toxic polyneuropathy can be intoxication consequence heavy metals, organic including organophosphorous, connections and medicines. The axonal degeneration is the cornerstone of pathological process. The accompanying demyelination has segmented character and is, usually, less expressed. Depending on features of contact with toxic substances, polyneuropathy can develop sharply or chronically; in most cases has the mixed touch and motor character.
Lead polyneuropathy is morphologically characterized by segmented demyelination and a degeneration of axons of peripheral nerves. The question of dominance of these processes remains disputable. It is clinically shown by chronic motive neuropathy, motor defects, usually asymmetric. In upper extremities the beam nerve ("the hanging-down brush") and, in lower - a fibular nerve ("the hanging-down foot") is surprised. Sensitive frustration insignificant. The touch form meets at the latent and moderately expressed poisoning. The pain syndrome, distal vegetative dysfunction are possible. Lead gets to an organism through airways or a gastrointestinal tract and is deposited in bones and a liver. There are an increased fatigue, slackness, irritability, decrease in attention and memory, a "stupid" headache, easy pyramidal and extrapyramidal symptomatology (symptoms of a lead encephalopathy); sharp anemia; spastic colitis; hand and legs pains; trembling of fingers of a brush; "cock gait"; "the hanging-down brush" (the evidence of damage of beam and fibular nerves). The forecast is rather favorable. An indispensable condition is the termination of contact with lead.
Disease source — the insecticides containing arsenic, medical supplies, dyes (recently with the medical purpose or as dye arsenic is not applied). Professional intoxication is possible at founders (proceeds softly). Single influence of a high dose conducts to vascular hypotension, vomiting, disturbance of consciousness, perhaps korsakovsky type. If the patient survives, after stage of latency in 2-3 weeks polyneuropathy develops. Muscular weakness is variable, prevails in legs. At repeated influences of small amounts of arsenic distal symmetric sensomotor polyneuropathy at which pains and disturbances of sensitivity dominate develops. At chronic intoxication gastrointestinal frustration, usually, are absent, but hypersalivation is often observed. Vascular and trophic frustration are noted: rashes, pigmentation, peeling, cross white strips on nails (strips the Mass), a hyperkeratosis on palms and soles, pigmentation by plaques in the form of rain drops on a stomach. In connection with decrease in deep sensitivity the ataxy (arsenical pseudo-tabes) develops. The current is chronic, recovery lasts many months. Diagnosis is based on identification of arsenic in urine (higher than 25 mkg/days), hair and nails (it is more than 1 mkg/g).
The mechanism of development of polyneuropathy at an alcohol abuse is not up to the end clear. The leading role is assigned to frustration of a resorption of thiamin and creation of tiaminovy insufficiency in an organism. It, in huge degree, is promoted by development of a gastroduodenit at alcoholism. Also direct toxic effect of alcohol is not excluded.
The clinic allocates acute and subacute forms of a current. There is also a subclinical option when PNP-a syndrome can be revealed only at inspection of the patient. The first two forms of a course of polyneuropathy can sometimes be combined with amyotrophic and myopathic syndromes. Amyotrophy can be as diffusion, and mainly in distal departments. More often and stronger motive and sensitive fibers suffer. Meet purely motor alcoholic polyneuropathy. Alcoholic polyneuropathy is among aksonopatiya, however also segmented demyelination can meet. The clinical picture consists of disturbances of sensitivity, movements, vegetative and trophic frustration. Most of alcoholics have subclinical current polyneuropathy: small disturbance of sensitivity in feet, decrease or lack of Achilles reflexes, palpatorny morbidity of gastrocnemius muscles. The developed clinical picture corresponds to symmetric sensomotor polyneuropathy (symmetric paresis and an atrophy of muscles, mainly sgibately foot and fingers, sensitive disturbances of a type of "gloves" and "socks", constants or the shooting pains and paresthesias in shins and feet, kramp, thermalgias in soles, puffiness, hyperpegmentation of extremities, ulcers). Allocate a pseudo-tabic form of a disease. Polyneuropathy can sometimes be combined with cerebral manifestations (alcoholic dementia, a cerebellar degeneration, an epileptiform syndrome). Recovery flows slowly, the forecast depends on return to alcohol or refusal of it.
The patient With, 17 years, was on hospitalization from 14.02.04 till 13:03. 04 g.
Complaints: at revenues to weakness in legs and hands, a reduced background of mood.
Anamnesis of an illness: Considers himself as the patient during the last 3-x weeks when pains and paresthesias in muscles of legs for the first time began to disturb. Further began to note emergence of weakness in legs (" legs began to give away in knees"), and in hands, within the last week weakness amplified. The last two months abused alcohol, badly ate that is connected with the postponed psychoemotional injury.
Anamnesis of life: From the postponed diseases — ORZ. Denies an infectious disease, a diabetes mellitus, tuberculosis, rheumatism, malaria, venereal diseases. Epidanamnez and the allergological anamnesis are not burdened. Does not smoke.
Somatic status: Correct constitution, subnutrition. Integuments, visible mucous pale. Lymph nodes are not palpated. In lungs vesicular breath of ChDD — 16 in 1 min. Action of the heart is rhythmical, cordial tones clear ChSS — 120 ud. in 1 min., sufficient filling, the ABP — 120/80 mm Hg. Heart borders within age norm. The stomach is soft, it is palpatorno painless. The liver, a spleen are not increased. Pasternatsky's symptom is negative with 2-x the parties. There are no peripheral hypostases. A chair and an urination without features.
Neurologic status: The consciousness is clear. In space and time it is oriented, on questions gives adequate answers. Palpebral fissures of D = S. Pupils of the correct form and size D = S, photoreactions of moderate degree of vivacity. Corneal reflexes are kept. Movements of eyeglobes in full. Convergence, accommodation are not broken. There is no nystagmus. The palpation of points of an exit of a trifacial with 2-x the parties is painless. Sensitivity on a face is not broken. Tension of masseters when chewing uniform. Mimic muscles at rest and at the active movements without asymmetry with 2-x the parties. The hearing is kept. Phonation and swallowing are not broken. Language on the average line, palatine velums strain evenly with 2-x the parties. Elements bulbar or pseudobulbar
syndrome are not revealed.
The volume of active movements is limited in all extremities. Muscle strength is reduced in proximal departments of hands to 3,5 points, legs - to 2 points, in distal departments 4-x extremities to 3,5 points. Hypotonia of muscles in all extremities. Hypotrophy of muscles of a shoulder and pelvic girdle, back and proximal departments of extremities. Tendon and periosteal jerks from hands of D> S, average vivacity; knee D <S, torpid, are quickly exhausted; akhillova are absent with 2-x the parties. Pathological signs are not caused. Belly reflexes live. Cerebellar symptoms do not manage to be defined from - for paresis. Meningeal signs no, of Sensitive disturbances are not revealed. The expressed vegetative reactions in the form of the general hyperhidrosis with accent in brushes and feet, pilomotor skin reactions. Tremor century and fingers of outstretched arms. Emotional lability. Sphincteric disturbances are absent.
Additional methods of inspection:
Wedge, blood test: Ayr. — 5,0-10 12/l; Hb — 150 g/l; L. — 6,9-109/l; aa. — 6%; the item — 1%; with — 51%; l. — 40%; m — 2%. SOE — 4 mm/h, tsv. pok. — 0,9.
— wiped blood on RW.
Wedge, analysis of urine: quantity — 200 ml; ud. weight — 1012, prozr., reaction — subacidic, protein — is not present, sugar does not, leyk. — 1-2 in p / зр., an epithelium — transitional, places.
Blood biochemistry: urea — 5,4 µmol/l, creatinine — 69 µmol/l, crude protein — 71 g/l, general bilirubin — 14 µmol/l, is direct, bilirubin — 4 µmol/l, is not direct, bilirubin — 10 µmol/l, an alkaline phosphatase — 1199 pieces.
Blood immunology: heterophyllous hemolysins — 0,47 (N 0,6); limfotsitotoksichesky antibodies of 5% (N of 15%); complement level — 1,12 (N 0,9-1,2); T - lymphocytes — 49% (40-60%); In - limotsita — 9% (N of 10-20%); The CEC — 3,5% of PEG — 0,020, 7% of PEG-0,035; T - a help. — 42%; T - supr. — 16%.
Kone, oculist: the eyeground without features, indicators of an oftalmoskopiya is normal.
ECG: sinus tachycardia, a sinoatrial rate (100 in 1 min.).
EEG: the expressed irritation dientsefalno - trunk structures.
REG: level of a pulse krovenapolneniye is reduced, tachycardia to 100 ud. in min., the angiodystonia on hypotonic type, elasticity is normal.
EKHOEG: there is no M shift - an echo, internal hydrocephaly.
The central hemodynamics — sharply expressed hyperkinetic type of circulation.
EMG: the item peroneus dextra et sinistra decrease in amplitude on M - the answer to 1,5 mV, above a popliteal space — norm. SRV — 7,5 m/s, decrease in SRV in upper extremities to 4,9 m/s.
Conclusion: Considering data of complaints, the anamnesis, a clinical picture of a disease the diagnosis is made: dismetabolichesky polyneuropathy (alcoholic) with moderate tetraparesis.
Treatment: Haemodesum, glucose, ascorbic acid, Essentiale, vitamin B 1, vitamin B 12, ATF, prozerin, retabolil, amitriptyline, enkad. Against the conducted medical course the condition of the patient considerably improved.
Adequate therapy led to clinical recovery.
Polyneuropathy at FOS poisonings
Organophosphorous connections (FOS) are widely used as insecticides, plasticizers, lubricating oils. They are resistant inhibitors of cholinesterase in vegetative nodes, the terminations of parasympathetic nerves, in neuromuscular synapses. Acute poisoning flows as cholinergic crisis: hypersalivation, perspiration, a miosis, intestinal gripes, vomiting, diarrhea, a headache, fastsikulyation, an urine incontience, a bronchospasm, spasms, a depression of breath, a lump, in hard cases death. It should be noted that only insecticides cause at the use peripheral neuropathy. Sometimes cases of polyneuropathy are noted after suicide attempts at reception of a trikhlorfon (Diptereks). Several days later after stopping of poisoning distal sensomotor PNP with the expressed dominance of motive defects, existence of pyramidal signs owing to defeat not only peripheral nerves, but also TsNS (front horns, motive kernels of a trunk, back cords, cortical and medullispinal ways) develops. Paralyzes are recovered badly.
Are of special interest medicamentous polyneuropathy. Amiodaronum (kordaron), dapsone, Teturamum, gold drugs, an isoniazid, metronidazole, mizonidazol, pergeksilen, Cisplatinum (platinum drug), a thalidomide, periwinkle alkaloids — here the list of those medicines which can cause polyneuropathy.
Polyneuropathy at reception of a pergeksilen Pergeksilen (apply to treatment of coronary insufficiency) in a dose of 200-400 mg/days leads to development of the PNP-syndrome several weeks later. A polyneuropathy basis — segmented demyelination, to a lesser extent - defeat of the axial cylinder. The beginning is noted by painful paresthesias, decrease in superficial sensitivity, joint muscle sense (ataxy), paresis from extremities, distal to proximal muscular groups, develops further. Drug withdrawal leads to regress of symptomatology. The mechanism of neurotropic action is not clear. Perhaps, it is result of exchange disturbances owing to anoreksogenny effect of the medicine.
Izoniazidny polyneuropathy of Izoniazidnaya polyneuropathy of sensomotor type — a consequence of deficit of the B6 vitamin caused by drug in persons with genetically determined disturbance of a metabolism of this vitamin. An axiom of tuberculostatic therapy — appointment along with an isoniazid of 25-30 mg/day of a pyridoxine inside. PNP of mainly axonal type.
Polyneuropathy at surplus of a pyridoxine
At newborns and children of early age the rare form of epilepsy which will respond to treatment high doses of a pyridoxine is observed. Surplus of a pyridoxine (at a daily dose of 50-300 mg) also leads to development of touch polyneuropathy with a headache, irritability, fatigue. Inspection reveals Lermitt's symptom.
Polyneuropathy at long reception of a gidrolazin (apressine)
Prolonged use of drug of hypotensive action of a gidrolazin (apressine) promotes removal from an organism of pyridoxal phosphate and development of polyneuropathy of dismetabolichesky type. Purpose of this drug demands periodic use of B6 vitamin.
Polyneuropathy at reception of Teturamum Teturamum (it is used at treatment of alcoholism), which toxic effects should be distinguished from alcoholic polyneuropathy, implements the neurotropic action at e/dn reception of 1,0-1,5 g. The leading symptoms — disturbances of sensitivity against moderate paresis, defeat of optic nerves is possible (neuritis). The axonal degeneration with dominance of segmented demyelination is the cornerstone of polyneuropathy.
Polyneuropathy as a result of treatment kordarony
Segmented demyelination with involvement of axial cylinders is characteristic of the PNP-syndrome as a result of reception of a kordaron (drug for treatment of coronary insufficiency) in a dose of 400 mg/days and above lasting more than 1 year.
Polyneuropathy at disvitaminoza of Ve and E.
Are of special interest polyneuropathy at disvitaminoza of B6 and E. O B6 hypovitaminosis at the persons accepting an isoniazid and gidrolazin it was already told above. However, Cycloserinum, Penicillaminum and Pyrazinamidum can also conduct to its shortcoming. B6 hypovitaminosis caused by food disturbances is exclusively rare. Symmetric distal sensomotor polyneuropathy is characteristic of a hypovitaminosis of B6, is more rare - epileptic seizures and encephalopathy. B6 hypervitaminosis in turn can also cause touch polyneuropathy therefore reception of a pyridoxine should be conducted strictly in necessary doses.
Vitamin E — fat-soluble vitamin, its deficit develops at a syndrome of the broken absorption. As other reasons of a hypovitaminosis E serve hereditary diseases (abetalipoproteidemiya), a resection of intestines, an illness of a liver and bilious ways. The clinical picture is provided by PNP, an ataxy and weakness of proximal muscles. With the medical purpose in this case select the vitamin E dose equal 60-70 ME inside or in oil. Overdoses and - tocopherol cause a syndrome of a myopathy which at adequate treatment is exposed to involution.
5.3.2 Toxic polyneuropathy, caused by endogenous intoxications
For maintenance of normal structurally functional integrity
peripheral nervous system a number of hormones is necessary. Deficit or surplus of some of them can lead to disturbance of work of peripheral nerves. The knowledge of this group of polyneuropathy is obviously important as the last usually are reversible frustration on condition of timely correction of the broken hormonal state. Damage of a thyroid gland (a hypothyroidism, a hyperthyroidism), pituitary frustration (an acromegalia, a hypopituitarism), damage of adrenal glands, disorder of function of a pancreas, parathyroid and gonads to some extent can act as the reasons of development of certain forms of polyneuropathy.
Diabetic polyneuropathy — one of the most frequent diseases of a peripheral nervous system in the developed countries. Meets at lungs and at heavy displays of an illness, has a number of features. Lack of permanent compensation of a diabetes mellitus, lability of a day-night rhythm of content of glucose in blood predetermine changes in structure of nerve fibril. The pathogeny of diabetic polineyropatiya is difficult and up to the end is not clear. The metabolic theory pays much attention to accumulation in neurons of sorbitol and decrease in a mioinozitol as a result of activation of a pentozny way of utilization of glucose. The genetic theory approves hereditary conditionality of these disturbances regardless directly from a lack of insulin. In recent years development of diabetic polyneuropathy is coordinated to microvascular changes.
At the beginning schwannian cells, further myelin covers, axial cylinders are surprised. In blood vessels of peripheral nerves thickenings of capillaries an endonevriya and expansion of connective tissue membranes of a perineurium, occlusion of small vessels are defined. Most likely the nerve heart attack owing to vascular disorders is the cornerstone of the isolated and multiple neuropathy at a diabetes mellitus.
Clinical displays of diabetic polyneuropathy can vary over a wide range. The beginning of development can be acute or gradual, and progressing — fast or slow.
PNP can proceed asymptomatically or lead to a heavy invalidism. At diabetes three main neyropatichesky syndromes develop.
Classification of diabetic neyropatiya by topography
I. Distal symmetric polyneuropathy.
1) The mixed motor and touch and vegetative neuropathy.
2) Mainly touch neuropathy:
a) with preferential defeat of thick fibers;
b) with defeat of thick and thin fibers;
c) with preferential defeat of thin fibers.
3) Mainly motor neuropathy.
4) Mainly vegetative neuropathy.
II. Symmetric proximal motor neuropathy.
III. Local and multiple neuropathy.
1) Asymmetric proximal motor neuropathy.
2) Neuropathy of cranial nerves.
3) Mononeuropathy of intercostal nerves, etc. mononeuropathy.
4) Tunnel neuropathy.
The most widespread clinical type of diabetic polyneuropathy is mixed sensorno - motorno - vegetative. It begins gradually, is characterized by symmetric sensitive disturbances, mainly in distal departments of legs, in the form of the pains and paresthesia amplifying at night, hypalgesias as "socks" and "gloves", decrease in all types of sensitivity. Lack of knee and Achilles jerks is observed. The expressed decrease in painful and proprioceptive sensitivity quite often causes development of painless ruptured ulcers. Weakness and atrophies of muscles can not be expressed. Nature of disturbances of functions of a vegetative nervous system can vary from subclinical changes before heavy frustration.
Touch polyneuropathy meets rather often at a diabetes mellitus. Both thick, and thin fibers at the same time can be involved in pathological process. Disturbance of sensitivity is varied from easy numbness of toes before deep anesthesia with neyropatichesky ulcers and an arthropathy. Sensitivity of a cornea and hearing can be surprised. Disorder of sensitivity from the level of "gloves" and "socks" can rise proksimalny inguinal area and shoulders. The expressed decrease in deep types of sensitivity can lead to a sensitive ataxy — a "pseudo-tabic" form of diabetic polyneuropathy. Intolerable spontaneous pains, dizesteziya and paresthesias are often observed. The paradoxical combination of spontaneous pains and lack of sensitivity to painful irritants does not find a sufficient explanation yet.
Symmetric proximal motor polyneuropathy (diabetic amyotrophy) — the phenomenon, rare for diabetes. It is characterized by weakness mainly of muscles of hips and a pelvic girdle. Weakness of muscles develops sharply or subacutely within several weeks, is sometimes combined with the aching pains in the field of damage of muscles. The nature of diabetic amyotrophy remains not clear, however, there is an assumption that metabolic and vascular disorders are the cornerstone of a syndrome. The relative proof of it is the possibility of satisfactory recovery in several months of function of the affected muscles against therapy by insulin.
Vegetative polyneuropathy — is observed only at a small number of patients with diabetes when selectively vegetative nervous system is involved in pathological process. Young patients with diabetes of the I type prevail (insulin-dependent). Vegetative disturbances include gastroparez, a diarrhea, tachycardia, orthostatic hypotension, sweating disturbances, neurogenic dysfunction of a bladder and impotence. Patients with this type of neuropathy have a risk of bezbolevy myocardial infarctions, a cardiac standstill and sudden death in a dream.
Among local multiple neyropatiya at a diabetes mellitus femoral, locking, sciatic nerves most often are surprised, is more rare — median and elbow. In this case neuropathy develops sharply or subacutely, the forecast favorable, regress of symptomatology comes in 6-12 months. It is characteristic also for diabetic neyropatiya of a third cranial nerve (the III couple).
Analyzing clinical features of the most often met types of polineyropatiya at a diabetes mellitus, it is possible to allocate a number of the patterns characteristic of diabetic polyneuropathy:
- sensitive and reflex functions suffer earlier and stronger, than motive;
- vibration and discrimination sensitivity are surprised earlier and stronger, than muscular and joint sensitivity;
- akhillova reflexes decrease earlier, than bottom;
- sensitivity of the back of foot is broken earlier and stronger, than a bottom surface;
- also moderately expressed asymmetry in damage of the nerves of the same name is noted poorly;
- at achrestic diabetes the widespread hypesthesia develops;
- distal departments of peripheral nerves suffer more, than proximal. — median and elbow. In this case neuropathy develops sharply or subacutely, the forecast favorable, regress of symptomatology comes in 6-12 months. It is characteristic also for diabetic neyropatiya of a third cranial nerve (the III couple).
The patient To., 57 years, was on hospitalization in a neurology unit from 29.06.98 for 22.07.98.
Complaints: at revenues to numbness in legs, the weakness in them increasing when walking.
Anamnesis of an illness: In February, 1998 pains and paresthesias in muscles of legs began to disturb. Further noted emergence of weakness in legs (" legs give away"). Within the last week weakness amplified.
Anamnesis of life: 10 years suffer from a diabetes mellitus of the II type, accept maninil. Denies tuberculosis, malaria, venereal diseases, an infectious disease. Epidanamnez and the allergological anamnesis are not burdened, has no addictions.
Somatic status: Correct constitution, subnutrition. Integuments are pale, dry. Lymph nodes are not increased. In lungs vesicular breath, ChDD — 16 in 1 min. Action of the heart is rhythmical, cordial tones are clear to ChSS — 76 ud. in min., sufficient filling, the ABP — 90/60 mm Hg. Heart borders within age norm. The stomach is soft, it is palpatorno painless. The liver, a spleen are not increased. Pasternatsky's symptom is negative with 2-x the parties. There are no peripheral hypostases. The chair, diuresis is normal.
Neurologic status: The consciousness is clear. In space and time it is oriented, on questions gives adequate answers. Palpebral fissures of D = S, pupils of the correct form and size D = S, photoreactions of moderate degree of vivacity. Corneal reflexes are kept. Movements of eyeglobes in full. Convergence, accommodation are not broken. There is no nystagmus. The palpation of points of an exit of a trifacial with 2-x the parties is painless. Sensitivity on a face is not broken. Tension of masseters when chewing uniform. Mimic muscles at rest and at the active movements without asymmetry with 2-x the parties. The hearing is kept. Phonation and swallowing are not broken. Language on the average line, palatine velums strain evenly with 2-x the parties. Elements of a bulbar or pseudobulbar syndrome are not revealed.
The volume of active movements is limited in the lower extremities. Muscle strength is reduced in proximal departments of legs to 2,5 points, in distal departments of legs to 3,5 points. Hypotonia of muscles in all extremities. Hypotrophy of muscles of a shoulder and pelvic girdle, proximal and distal departments of extremities (more legs). Tendon and periosteal jerks from hands of D = S, average vivacity; knee D - S, torpid; akhillova are absent with 2-x the parties. Pathological signs are not caused. Belly reflexes live. Carries out Paltsenosovy test well. Other koordinatorny tests do not manage to be defined from - for lower a paraparesis. There are no meningeal signs. The hypesthesia as "high socks" is defined. Pilomotor skin reactions, trophic disturbances (a hyperkeratosis, a peeling, fragility of nails) in the field of feet with 2-x the parties. Sphincteric disturbances are absent.
Additional methods of inspection:
Wedge, blood test: Ayr. — 5,0-1012/l; Hb — 131 g/l; Leyk. — 6,7-109/l; aa. — 6%; the item — 1%; page — 58%; l. — 40%; m — 3%. SOE — 2 mm/h.
Blood glucose — 15,2 mmol/l.
— wiped blood on RW.
Wedge, analysis of urine: quantity — 100 ml; ud. weight — 1030; prozr., reaction — ate. acid; there are no squirrels —; sugar — 222,04 mmol/l; leyk. — 1-2 in p / зр.; an epithelium — transitional, places; slime — moderate quantity.
Blood biochemistry: urea — 4,5 mmol/l, creatinine — 83 mmol/l, crude protein — 67 g/l, general bilirubin — 14,5 µmol/l, is direct. bilirubin — 4,5 µmol/l, is not direct, bilirubin — 10 µmol/l, lipoproteids — 7,5.
Endocrinologist (13.07.98): diabetes mellitus, II type, dekompensirovanny.
Reovazografiya of the lower extremities of 23.06.98: moderate decrease in a volume blood-groove on shins is more on the right, sharply expressed asymmetry on feet.
ECHO - EG: there is no shift of average structures. Hydrocephaly.
Oculist (6.07.98): diabetic angiopatiya of a retina, unripe cataract of both eyes.
Conclusion: Considering data of complaints, the anamnesis, a clinical picture of a disease, these laboratory researches, the diagnosis is stated: diabetic polyneuropathy with the lower paraparesis against a diabetes mellitus of the II type, dekompensirovanny.
Treatment: insulin therapy course, trental, Essentiale, prozerin, Tioctacidum, ATP, enkad, laser therapy course. Against the conducted medical course the condition of the patient considerably improved.
Polyneuropathy at a paraproteinemia and a disproteinemia
The multiple myeloma or multiple myeloma of Kallera-Rustitsky can be followed by development of the progressing sensory-motor polyneuropathy quite often with defeat of respiratory and bulbar muscles. More often polyneuropathy arises at osteosclerotic, than at osteolytic, a myeloma form. The prelum of peripheral nerves and textures owing to extradural accumulation of myelome masses is possible. Also demyelinating influence of the immunoglobulins produced by myeloma cells is not excluded.
The multiple myeloma develops equally often at men and women 50 years are more senior. Average age of initial displays of neuropathy — 52 years.
The first sign are pains which are sclerolanguid in the beginning, caused by irritation of bone, fibrous receptors. Further there are radicular pains, is more often in lumbar area and both legs, akhillova and knee jerks decrease. Patients complain of paresthesias and feeling of numbness in feet, then in brushes. Further there are objective distal disorders of sensitivity with moderate paresis and hypotrophies. Carrying out speed on peripheral nerves decreases. In liquor the proteinaceous and cellular dissociation is noted.
Macroglobulinemia (Valdenstrem's illness) — disease of people of usually advanced age. Proliferation of lymphocytic cells with an infiltration of marrow, lymph nodes and other fabrics is noted. Peripheral polyneuropathy develops at every fourth patient. The degeneration of the myelinized fibers with preferential segmented demyelination is the cornerstone. Sensitive frustration in distal departments of extremities, paresthesia, pain, kramp, distal sluggish paresis, sometimes to paralyzes, gait — "steppage" are characteristic. The PNP-syndrome develops gradually and progrediyentno. In the initial stages the symptomatology can be expressed unevenly, but further usually extends rather symmetrically. In liquor the proteinaceous and cellular dissociation can be registered. Speed of carrying out excitement on sensory nerves decreases, on motive significantly does not change. Concentration of IgM increases in blood.
Polyneuropathy at diffusion diseases of connecting fabric
Pathology of connecting fabric which, on the one hand, is part of a vascular wall is the cornerstone of this group of diseases, and with another - is a part of nerve fibrils and surrounds them. In this regard defeat of PNS is often observed at diseases of connecting fabric and can be one of the first clinical manifestations at some of them. Damage of peripheral nerves at the same time can be primary or secondary against defeat of organ systems (uraemic, hepatic neuropathy, etc.), and can be result of the carried-out treatment (gold drugs, etc.). Damages of one nerve, multiple mononeuropathy, symmetric touch or sensomotor polyneuropathy can be observed. Most often polyneuropathy occurs at a nodular periarteritis (at 25% of patients), a pseudorheumatism (at 10% of patients) and at a system lupus erythematosus (in 5% of cases).
Clinical options of polyneuropathy at a nodular periarteritis are provided by mononeuropathy and multiple mononeuropathy. With different sequence sciatic, tibial, median and elbow nerves often asymmetrically are surprised. Symmetric polyneuropathy meets less often. In far come phase of an illness of defeat can become symmetric. Sometimes polyneuropathy precedes other manifestations of a periarteritis.
The pseudorheumatism can be followed by development of four types of polyneuropathy: distal symmetric touch polyneuropathy, multiple mononeuropathy, the mixed sensomotor polyneuropathy of a heavy current, tunnel neuropathy. As a rule, polyneuropathy are part of a clinical picture of a generalized vasculitis.
System lupus erythematosus
Defeats of PNS at patients with a system lupus erythematosus are shown in a look: symmetric detailed sensory-motor polyneuropathy, multiple mononeuropathy, .porazheniye of cranial nerves (especially trigeminal) and syndrome to Giyena-Barra. Defeat of PNS is usually observed at patients with already diagnosed system lupus erythematosus against the subacute or chronic course of a disease with high activity of process during one of aggravations. Defeats of PNS at this disease of connecting fabric often generalized, differ in big expressiveness and firmness. Symmetric disturbance of mainly superficial types of sensitivity (also deep types of sensitivity occasionally suffer) with insignificant motive frustration in the form of weakness in distal departments of extremities, increased fatigue of legs when walking and at transfer of weights dominate in most cases clinical implication of defeat of PNS at a system lupus erythematosus. Vegetative disturbances are especially expressed at children.
At a scleroderma there can be a progressing trigeminal touch neuropathy or generalized sensomotor polyneuropathy of an easy current.
Uraemic polyneuropathy develops only at the long course of a chronic renal failure when the level of glomerular filtering falls lower than 20 ml/min., and concentration of creatinine in plasma is higher than 0,53 mmol/l. Mainly touch disturbances are characteristic of this polyneuropathy. Dizesteziya and numbness in distal departments of extremities are early symptoms, however, kramp and a syndrome of "uneasy legs" can precede them. PNP often is followed by pain and a burning sensation in feet and quite often leads to an invalidism. Reduction of the threshold of perception of vibration even in the absence of change of speed of carrying out on nerves acts as a sign of aggravation of a state at uraemic polyneuropathy.
Pathogenetic aspects of this polyneuropathy are fully not clear. The positive effect of a hemodialysis is noted. Successful transplantation of a kidney leads to permanent remission of polyneuropathy.
Hepatic polyneuropathy is shown by sensomotor disturbances. In most cases as the reason serves not pathology of a liver, but alcoholism, the diabetes and medicinal therapy which are its cornerstone. It should be noted that during recovery and at earlier stages of infectious hepatitis development of a syndrome to Giyena-Barra is possible. PNP at primary biliary cirrhosis is connected with xanthomatous deposits in peripheral nerves. There is an easy form of hepatic polyneuropathy which develops in an initial stage of cirrhosis. At the beginning of a disease limited defeats of one segment or one nerve are typical, is more rare than all extremity. Several weeks later, more often than months process extends to other nerves. Allocate the motive, sensitive, atactic and mixed polyneuropathy forms. Most often the sensitive and mixed forms meet.
Motive frustration can be expressed in different degree — from easy weakness to deep paresis. On hands mainly radial type of motive frustration, standing — peroneal is observed. At most of patients pains and paresthesias, muscular atrophies in distal departments of extremities, decrease in a tone, fading of tendon jerks, vasculomotor and trophic disturbances (change of coloring, temperature of skin, sweating, thinning of skin, a hyperkeratosis) come to light.
The acute form of hepatic polyneuropathy is observed at hypertrophic cirrhosis without ascites. The mixed disturbances — motor and touch are characteristic of it. The chronic form of hepatic polyneuropathy complicates a cachectic stage of cirrhosis. Mainly motor disturbances are characteristic of it. However, tendon jerks go down, but do not disappear.
Defeat of a nervous system at zheludochno - intestinal diseases
The question of peripheral neyropatiya at a gastrointestinal tract disease is connected with concept of alimentary avitamonous polyneuropathy when into the forefront in the causal relation pathology of a stomach and intestines acts.
As a result of malabsorption after a gastrectomy, at a carcinoma of a stomach, a resection of a small site of intestines, tropical to a spr, a regional ileocecal ileitis, an intestinal lipodystrophy (Whipple's illness), obturatsionny impassability of intestines, a divertuculosis of a lean and ileal gut and the phenomena of stagnation of contents gastrointestinal and other fistulas insufficiency of a number of vitamins (B1, B12, E, folic acid) and other alimentary factors can develop. Pathological changes in PNS are provided at the same time by an axonal degeneration with secondary segmented demyelination.
The illness of the operated stomach has a typical set of neurologic complications: dismetabolichesky encephalopathy, Vernike-Korsakov's syndrome, the progressing myelipathy, an atrophy of optic nerves and the mixed polyneuropathy. At the heart of the provided complications — pernicious vomiting and, mainly, a vitamin deficiency of B1, B12, E, folic acid.
Gee's disease (glyutenchuvstvitelny enteropathy) — the illness connected with disturbance of assimilation of the cereals supporting squirrels gliadine. It is characterized by the resistant steatorrhea, loss of weight, multiple scarce states connected with changes of a mucous membrane of a jejunum. The disease is complicated by defeat of TsNS in the form of disturbance of terms of myelination, a delay of psychomotor development; the myopathy and polyneuropathy are possible. At the heart of complications — malabsorption with a vitamin deficiency of B1, B12, E, folic acid.
Whipple's illness (intestinal lipodystrophy) is the multisystem disease of not clear genesis connected with accumulation in cells of a small bowel of CHIC-positive inclusions with bacteria or without them. Is complicated by defeat of TsNS in the form of dementia, hypothalamic disturbances, chronic meningitis. In liquor protein can be raised. Occasionally there are polyneuropathy, a myopathy, a papilledema, epileptic seizures, myoclonias, an ataxy, a nadjyaderny ophthalmoplegia. On the computer tomography (CT) the centers of the lowered density are defined.