Chronic inflammatory demiyeliniziruyushchy polyneuropathy
The Chronic Inflammatory Demiyeliniziruyushchy Polyneuropathy (CIDP) — the acquired defeat of peripheral nervous system of autoimmune character. In classical option it is shown by typical clinic of a symmetric sensomotorny polyneuropathy with slow monotonous or step progress. HVDP according to clinical data, ENMG to criteria, results of MRT of a backbone or ultrasonography of nervous trunks is diagnosed. Treatment is carried out is long with application of glucocorticosteroids, sessions of a plasma exchange and intravenous immunotherapy. In most cases HVDP forecast favorable.
Chronic inflammatory demiyeliniziruyushchy polyneuropathy
The Chronic Inflammatory Demiyeliniziruyushchy Polyneuropathy (CIDP) finally received the name in 1982. Before various terms were applied to it. Because of similarity of symptomatology clinical physicians long time considered HVDP a chronic form of a syndrome to Giyena-Barra. At the end of the 20th century experts in the field of neurology allocated accurate neurophysiological signs and diagnostic criteria of HVDP are developed.
The disease meets mainly at adult age. Frequency children — 0,5 cases on 100 thousand people, at adults have 1-2 cases on 100 thousand. Males get sick more often. The peak of incidence of HVDP is the share of age of 40-50 years. And at persons 50 years are more senior heavier current and the smaller response to therapy is observed. HVDP is often combined with other diseases: HIV infection, sarkoidozy, rheumatoid arthritis, hard currency, amiloidozy, chronic glomerulonefrit, diabetes, tumoral defeats. Along with other symptoms of HVDP can make clinic of a paraneoplastic syndrome.
Causes of HVDP
HVDP belongs to inflammatory polyneuropathies. The basis of pathological process is made by an inflammation of peripheral nervous trunks. Its autoimmune character does not raise doubts, however is still rather not studied. HLA genes often are found in patients with HVDP, in 70% antibodies to r-tubulinu come to light.
Unlike a syndrome to Giyena-Barra, in most cases HVDP communication of a debut with any previous disease or a state is not traced (a SARS, the vaccination undergone by an operation, etc.). Perhaps such communication exists, but because of the hidden slow beginning of HVDP it is difficult to track it.
The autoimmune inflammation leads to destruction of a myelin cover of a nerve. Demiyelinization at HVDP has scattered character: certain sites of a nerve are surprised; process extends on diameter, on a dlinnik of a nervous trunk; changes can affect touch, motive fibers. It causes big polymorphism of clinical manifestations and certain difficulties in diagnostics of HVDP.
Classification of HVDP
Due to the polymorphism of symptomatology allocate a typical (classical) form and atypical options of HVDP. The HVDP classical form means the symmetric muscular weakness of both disteel, and proximal departments of all 4 extremities which is combined with touch violations and increasing during more than 2 months. Has the monotonous or step slowly progressing current against the background of which separate aggravations are possible.
Treat the HVDP atypical forms: disteel with primary defeat of brushes, feet, forearms and shins, asymmetric with asymmetric involvement of extremities, focal — the isolated damage of one or several nerves, a humeral or lumbar and sacral texture, isolated motive — defeat only of motor fibers, isolated sensitive — defeat only of touch fibers.
The basis of clinical picture HVDP is made by a sensomotorny polyneuropathy. It develops gradually, often patients cannot even specify the beginning of a disease approximately. The first address to the doctor is usually dictated by weakness in extremities which complicates walking on a ladder, rise on a footboard in city transport, small work as fingers of hands, etc. Patients note unsteadiness and a sleep of extremities. Usually at HVDP muscular weakness is symmetric and progresses on the ascending type. In most cases its slow increase takes more than 2 months. However at 16-20% of patients with HVDP sharper beginning with development of weakness during the period about one month is noted.
Motive violations progress and occupy proximal departments of extremities. Are followed by decrease and loss of reflexes, it is the most frequent - Achilles. Muscular atrophies develop not at once, and only at a long current of HVDP without treatment. Touch frustration are noted in 85% of cases of HVDP. They prevail over motive only at 10% of the diseased. As a rule, sleeps of feet and brushes are noted. In some cases HVDP because of defeat of deep types of sensitivity develops a sensitive ataxy. At certain patients the pain syndrome is observed.
Quite often at HVDP the posturalny tremor of brushes — trembling at deduction of hands in a certain pose is observed. Damage of craniocereberal nerves is possible: glazodvigatelny, front, trigeminal. Bulbarny paralysis at HVDP develops seldom. Involvement of respiratory muscles with development of respiratory insufficiency is observed only in some cases. Vegetative frustration are not characteristic of HVDP.
Current of HVDP
About 70-75% of cases of HVDP make options with a monophase and chronic current. In the first case the symptomatology slowly progresses to a maximum, and then its full or partial regress without the subsequent retsidivirovaniye is observed. The chronic progressing current of HVDP is characterized by continuous smooth or step aggravation of symptoms. At 25-30% of patients the recidivous-remitiruyushchee current with accurately allocated aggravation periods is noted.
Separately allocate HVDP option with a sharp debut which is diagnosed often as a syndrome to Giyena-Barra (a sharp inflammatory demiyeliniziruyushchy polyneuropathy). However its subsequent chronic progressing current allows to expose the diagnosis of HVDP finally.
Diagnostics of HVDP
Patients with symptoms of a polyneuropathy undergo inspection at the neurologist. In the neurologic status they reveal muscular weakness of disteel departments of extremities, decrease in sensitivity (gipesteziya) as "stockings and gloves", loss of tendinous reflexes. At the HVDP atypical forms of change can have asymmetric character or come to light only in a zone of an innervation of separate nerves or textures. Diagnostics like polyneuropathy is performed by means of an elektroneyromiografiya (ENMG), a magnetic and resonant tomography and a research of tserebrospinalny liquid.
ENMG is carried out by the neurophysiologist and in most cases diagnoses changes, typical for a demiyelinization of peripheral nerves. Further on stimulation EMG signs of aksonalny defeat can be found. The initial ENMG-research has to include not less than 4 nerves.
The Lyumbalny puncture with the analysis of a likvor at HVDP is now carried out less frequently. In classical option it allows to exclude infectious defeat of TsNS. High level of protein (> 1 g/l) in tserebrospinalny liquid in the absence of a tsitoz (the increased maintenance of cellular elements) is typical for HVDP. Existence of a tsitoz indicates, first of all, probability of HIV or Lyme's disease.
Backbone MRT at patients with HVDP reveals strengthening of the MR-signal from spinal backs, branches of a lumbar or humeral texture which testifies to their thickening. Almost the cerebral centers of a demiyelinization are diagnosed for 50% of patients when carrying out MRT of a brain. Now in diagnostics of polineyropatiya ultrasonography of a nerve is more and more actively used. This method is much simpler and cheaper than MRT. Also allows to reveal a thickening of a nervous trunk and it can be applied in differential diagnostics of HVDP with multifocal motor neuropathy.
As in 10-20% of HVDP is secondary, accompanying a system disease, it is necessary to examine carefully patients for an exception of such option. In certain cases symptoms of the main disease appear in several months after emergence of HVDP. Therefore inspection of patients needs to be repeated. Comprehensive examination includes blood test on glucose, a proteinaceous range, anti-nuclear antibodies, hepatic tests, onkomarker; diagnosis of HIV and viral hepatitises, X-ray analysis of lungs and so forth.
Treatment of HVDP
Today therapy of HVDP has 3 components: reception of corticosteroids, introduction of immunoglobulin and plasma exchange. Kortikosteroidny therapy usually begins with a high dose of Prednisolonum. With effect the dose is gradually reduced and passed to its reception every other day. During from 1 to 1,5 years of therapy at most of patients with HVDP almost full regress of symptomatology is observed. For the prevention of a recurrence kortikosteroidny therapy is prolonged for several years. A part of patients even in 2-3 years against the background of attempts of cancellation of therapy has recurrence of HVDP and then treatment it is necessary to continue.
Long reception of corticosteroids has to take place under control HELL, density of a bone tissue (densitometry), sugar of blood, cholesterol, level of potassium and calcium. The accompanying courses of gastroprotektor, calcium medicines are obligatory. As an alternative to corticosteroids at HVDP immunosupressor act. They are applied in cases of small efficiency of steroids, at their bad shipping or at impossibility of decrease in a dosage.
Allows to lower doses and duration of glucocorticoid therapy at patients with HVDP additional application of a plasma exchange and immunoglobulin. Intravenous therapy by immunoglobulin is effective at 50% of sick HVDP. However its action is short therefore courses of an immunotherapy need to be repeated constantly. The plasma exchange is carried out with a frequency of 2 times a week to clinical improvement (about 1,5 months). Then sessions gradually urezhat up to 1 time a month.
Forecast of HVDP
Adequate treatment of HVDP allows to achieve full or almost full regress of symptoms of a polyneuropathy. Only at 10% of patients preservation or aggravation of clinic is noted. In 85% of cases 5 years later from a debut there is the minimum neurologic deficiency.
Important predictive value has duration of primary increase of symptoms of HVDP. If it more than 3 months, then recovery can take only 1 year. However the majority of sick HVDP needs long-term treatment and faces return of symptoms at its cancellation.