Inflammatory polyneuropathy — multiple defeat of nervous trunks as which substratum inflammatory reaction of autoimmune genesis acts. It is shown quickly or slowly progressing sluggish paresis with disorder of sensitivity, sometimes with damage of cranial nerves, paralysis of respiratory muscles. Diagnostics is carried out with application of electrophysiological researches, the analysis of tserebrospinalny liquid, blood test and immunological researches. Therapy pathogenetic (glucocorticosteroids, immunoglobulin G, plasma exchange, cytostatics) and symptomatic (anesthetics, antikholinesterazny, lipoic acid, physical therapy).
Inflammatory polyneuropathy — the inflammatory process affecting at once several peripheral nervous trunks. Inflammatory damage of nerves has mainly autoimmune character and often is followed by a demiyelinization — destruction of a myelin cover of nerves. The inflammatory polyneuropathy includes several nozologiya: sharp and chronic inflammatory demiyeliniziruyushchy polyneuropathies, serumal neuropathy. Incidence is widespread among children and adults. For inflammatory demiyeliniziruyushchy polyneuropathies it makes about 1,7 people on 100 thousand population though some atypical options meet much less often — according to some information to 2 cases on 1 million population. Prevalence of serumal polyneuropathy among persons to whom heterological serums were entered is at the level of 2-5%. The inflammatory polyneuropathy of any type is a serious disease with risk of a lethal outcome or an invalidization therefore search of new more effective methods of its therapy makes one of priority problems of modern neurology and immunology.
Reasons of an inflammatory polyneuropathy
Etiopatogenez of polyneuropathies of inflammatory character is up to the end not clear. Most of researchers are inclined to consider the main pathogenetic mechanism autoimmune process. In perinevralny fabrics inflammatory perivaskulyarny processes, activation of macrophages, congestions of mononuklear are observed. In blood anti-myelin antibodies are found, on peripheral nerves the complement and immunoglobulins, deposits of membranolitichesky complexes come to light. The developing autoimmune inflammation leads to an otsloyka and destruction of a myelin with reduction of thickness of a nervous trunk almost twice. The violation of carrying out nervous impulses which is clinically expressed in motive and touch frustration is result. Besides a demiyelinization in bioptata of the affected peripheral nerves multiple inflammatory infiltrates and expansion of podobolochechny space morphologically are defined.
Sharp Inflammatory Demiyeliniziruyushchy Polyneuropathy (SIDP)
It is characterized by a sharp debut against the background of a subfebrilitet. To the forefront there are motive frustration — sluggish tetraparesis of various expressiveness: muscular weakness takes all extremities and is followed by muscular hypotonia and a tendinous hyporeflection. At a half of patients the pain syndrome is noted. Eventually hypotrophies of denervirovanny muscles are formed. Sfinkterny functions are usually not broken. Damage of the craniocereberal nerves (CN), mimic muscles is possible. Violations of sensitivity meet not in all cases. At 30% of patients paresis of respiratory muscles with the disorder of respiratory function demanding IVL is observed.
As typical example of a sharp inflammatory polyneuropathy the syndrome to Giyena-Barra acts. Treat more rare forms atypical and symptomatic. The first differ in prevalence of selective defeat of these or those types of nervous fibers according to which allocate vegetative, motor, kraniobulbarny, touch, OVDP. Also Fischer's syndrome representing a combination of sluggish tetraparesis, glazodvigatelny frustration (oftalmoplegiya) and an ataxy belongs to atypical forms. Symptomatic options of a sharp inflammatory polyneuropathy can arise against the background of intoxications and infectious diseases (for example, at diphtheria).
Chronic Inflammatory Demiyeliniziruyushchy Polyneuropathy (CIDP)
Has the gradual beginning with symmetric development of the sluggish paresis which is followed by disorders of sensitivity. In some cases (about 15%) the beginning sharper that allows some clinical physicians to consider HVDP option of a current of a syndrome to Giyena-Barra. In typical cases muscular weakness arises in legs in the beginning, then extends to hands. The long progressing borrowing more than 2 months is characteristic. In hard cases at peak of a disease the full obezdvizhennost of the patient with paralysis of respiratory muscles is noted. IVL is required to about 10% of patients. In 15% of cases defeat of ChMN (a trigeminal nerve, bulbarny and/or glazodvigatelny groups) is observed.
The chronic inflammatory polyneuropathy can have several options of a current. At monophase option clinical symptoms after achievement of peak of the manifestation partially or completely regress without further aggravations or a recurrence. At the progressing current steady gradual or step increase of symptomatology is noted. To 30% of cases of a chronic inflammatory polyneuropathy have recidivous a current at which a recurrence (the periods of increase and regress of manifestations) alternate with temporary stabilization of a state — remission.
The HVDP atypical forms are presented by disteel option with defeat of mainly disteel departments of peripheral nerves, asymmetric option (Lewis-Sumner's syndrome, a multifocal motor or touch neuropathy), focal option with the defeat of separate nervous trunks (for example, with clinic of a humeral pleksit, neuritis of several nerves of one extremity, a lumbar and sacral pleksit) isolated by option with selective involvement in inflammatory process only sensitive or only motive nerves. The chronic inflammatory polyneuropathy of symptomatic character can be observed at system diseases (a nodular periarteriit, hard currency, Shegren's disease, system vaskulita), chronic infections (HIV, viral hepatitis C, a HTVL infection), an oncopathology (a gepatotsellyulyarny carcinoma, an adenocarcinoma of a thick gut), a sarkoidoza of lungs, a chronic glomerulonefrit, endocrine pathology (a gipertireoza, diabetes).
Most often develops after vaccination against tetanus. Debuts in 7-10 days from introduction of a vaccine from pains in a humeral belt, temperature increase and the itching rashes in a shoulder as a small tortoiseshell. From the first days of a disease there is a sleep of hands, then the weakness of the top extremities which was more expressed in their proximal departments gradually increases. At most of patients atrophies of proximal muscles of hands and muscles of a humeral belt are formed. In a quarter of cases there are artralgiya, in a third — disorders of sensitivity in a zone of an innervation of an axillary nerve. At 30% of patients lymphadenitis comes to light.
Diagnostics of an inflammatory polyneuropathy
As key feature of a clinical picture of an inflammatory polyneuropathy damage of both disteel, and proximal muscular groups of extremities acts that allows to otdifferentsirovat it from polyneuropathies of other genesis: toxic, dismetabolichesky (hepatic, uraemic, diabetic neuropathy) and hereditary (Refsum's disease, neural amyotrophy of Sharko-Mari-Tuta, Dezherin-Sotta's syndrome). Existence of touch frustration distinguishes an inflammatory polyneuropathy from motor-neuron diseases (the BASS, primary side sclerosis, spinal amiotrofiya) and primary and muscular defeats (miotoniya, myopathies). The dissociation which is usually observed in clinic of an inflammatory polyneuropathy between considerable muscular weakness and not rough atrophy of muscles has to draw attention of diagnosticians.
Along with clinical signs carrying out an elektroneyromiografiya allows to establish polyneural type of defeat. Inspection includes at least a research of middle, elbow, small and tibial nerves. In favor of the diagnosis of an inflammatory polyneuropathy detection of the increased protein content and proteinaceous and cellular dissociation at a research of the tserebrospinalny liquid received at a lyumbalny puncture testifies. At OVDP protein content reaches 5 g/l, proteinaceous and cellular dissociation is more expressed, can be observed , however concentration of lymphocytes usually does not exceed 20 pieces in 1 . At HVDP proteinaceous and cellular dissociation is observed generally in the period of a debut and an aggravation.
In the sharp inflammatory period in blood also acceleration of SOE can take place . The analysis on antibodies to glycosides has no specificity and high sensitivity. However at some forms of an inflammatory polyneuropathy (Fischer's syndrome, a multifocal polyneuropathy) they can be indicative. In difficult diagnostic cases neurologists resort to a nerve biopsy with the subsequent electronic microscopy of medicine which reveals characteristic demiyeliniziruyushchy processes. At separate sick HVDP on MRT of a brain define the demiyeliniziruyushchy centers located periventrikulyarno and subkortikalno demonstrating distribution of process of a demiyelinization on TsNS.
Treatment of an inflammatory polyneuropathy
Therapy consists of pathogenetic and symptomatic treatment. As means of the first line of a pathogenetic component of treatment glucocorticosteroids (Prednisolonum, methylprednisolonum), immunoglobulin human a class G and a plasma exchange act. It should be noted that in different clinical cases these methods reveal various efficiency. So, at typical forms of an inflammatory demiyeliniziruyushchy polyneuropathy the good result is shown by kortikosteroidny therapy, in atypical cases — treatment by immunoglobulin. In case of a serumal neuropathy in addition appoint antihistaminic pharmaceuticals.
The plasma exchange causes significant improvement in 80% of patients and is used in a combination with corticosteroids or immunoglobulin. However at the multifocal motor neuropathy (MMN) the plasma exchange does not render effect, and corticosteroids can aggravate expressiveness of paresis; immunoglobulin introduction remains the only way of therapy of the first line. Medicines of the second line are cytostatics (, , , a methotrexate). Their application it is recommended in the absence of desirable results from therapy by means of the first line. Tsiklofosfamid is successfully used at MMN.
The symptomatic component of treatment can include antikholinesterazny pharmaceuticals (, , ), means of removal of neuropathic pains (, , ), lipoic acid medicines at sensitive frustration, IVL at respiratory insufficiency. For the purpose of reduction of motive deficiency and in the recovery period LFK, massage and physical therapy are shown.
Forecast of an inflammatory polyneuropathy
OVDP have mainly good-quality current with almost complete recovery during the period from several weeks to 12 months. However in some cases the severe form with paresis of respiratory muscles and risk of death develops. The lethality makes about 5%, cases of resistant residual symptomatology — to 15%. At HVDP in the conditions of adequate treatment stabilization of a state is reached at 70% of patients, an absolute recovery — at 10-15%. Other 10-15% of cases differ in the steady progressing or a remittiruyushchy current which is poorly giving in to therapy. The serumal neuropathy at in due time begun treatment, as a rule, has the forecast, favorable for recovery.