Parkinson's disease — slowly progressing degenerate disease of the central nervous system which main displays are such motive violations as a gipokineziya, a rigidnost of muscles, a rest tremor, posturalny frustration. Besides at Parkinson's disease vegetative, affective and other frustration develop. Distinguish true parkinsonism (Parkinson's disease) and a syndrome of parkinsonism which can accompany many neurologic diseases (ChMT, brain tumors, strokes, encephalitis and so forth). At suspicion of Parkinson's disease the patient needs to pass an electroencephalography, a rheoencephalography, brain MRT.
Parkinson's disease — slowly progressing degenerate disease of the central nervous system which main displays are such motive violations as a gipokineziya, a rigidnost of muscles, a rest tremor, posturalny frustration. Besides at Parkinson's disease vegetative, affective and other frustration develop.
Classification of a disease of Parkinson
Classification of a disease of Parkinson is based on age of an onset of the illness:
- juvenile (juvenile parkinsonism)
- with the early beginning
- with a late debut
Various classifications of a syndrome of parkinsonism are also known:
However these classifications of a disease of Parkinson and a syndrome of parkinsonism are not considered as faultless. Therefore for today the standard approach in this question does not exist.
Etiology and pathogenesis of a disease of Parkinson
By modern medicine it is made a certain progress in understanding of molecular and biochemical mechanisms of a disease of Parkinson. Despite it, the unknown has a true etiology of sporadic forms of this disease. Genetic predisposition and factors of the external environment are of great importance. The combination and interaction of these two factors initiate process of a degeneration in pigmentsoderzhashchy, and subsequently and other neurons of a trunk of a brain. Such process, having arisen once, becomes irreversible and begins expansive distribution on all brain. More other proteinaceous substances of nervous system are exposed to the greatest destruction alpha . At the cellular level the mechanism of this process looks as insufficiency of respiratory functions of mitochondrions, and also an oxidizing stress — the main reason for apoptosis of neurons. However other factors which functions remain not opened still also take part in pathogenesis of a disease of Parkinson.
Clinical picture of a disease of Parkinson
There is a tetrad of motive symptoms of a disease of Parkinson: tremor, rigidnost, gipokineziya, violations of posturalny regulation. A tremor — the most obvious and easily revealed symptom. The rest tremor is most typical for parkinsonism, however also other types of a tremor are possible, for example: posturalny tremor or intentsionny tremor. The muscular rigidnost can be hardly noticeable at initial stages, a thicket at a drozhatelny form of a disease of Parkinson, but obvious at the expressed parkinsonism syndrome. Detection of the minimum asymmetry of a tone in extremities as asymmetry of symptoms — a characteristic sign of all stages of a disease of Parkinson is of great importance earlier.
Gipokineziya is an obligate symptom of parkinsonism of any etiology. At initial stages of a disease of Parkinson identification of a gipokineziya can is complicated therefore resort to demonstrative receptions (for example, quickly to squeeze and unclench a fist). Early manifestations of a gipokineziya can be observed in the elementary actions directed to self-service (shaving, toothbrushing, fastening of small buttons, etc.). Gipokineziya is a bradikineziya (slowness of movements), an oligokineziya (reduction of quantity of movements), and also reduction of amplitude of movements and decrease in their speed. Because of a gipokineziya at Parkinson's disease individual "language of a body", including gestures, a mimicry, the speech and plasticity of motility is broken.
Posturalny violations at Parkinson's disease are shown early enough (for example, asymmetry of the hands extended forward). However most often they draw attention of doctors already in a dezadaptatsionny stage (the III stage). The fact that posturalny violations in comparison with other symptoms of a disease of Parkinson are less specific to it can serve as an explanation for it.
Except above-mentioned main displays of parkinsonism, Parkinson's disease also other symptoms which in certain cases can come to the forefront of a clinical picture accompany. And extent of disadaptation of the patient in such cases at all not less. Let's list only some of them: salivation, dizartriya and/or dysphagy, lock, dementia, depression, sleep disorders, dizurichesky frustration, syndrome of uneasy legs and others.
Distinguish five stages of a disease of Parkinson, each of which reflects severity of a disease. The greatest distribution was gained by the classification offered in 1967 by Hen and Yar:
- 0 stage — motive manifestations are absent
- The I stage — unilateral displays of a disease
- The II stage — bilateral symptoms without posturalny violations
- The III stage — moderate posturalny instability, but the patient does not need assistance
- The IV stage — considerable loss of physical activity, but the patient is able to stand and move without support
- The V stage — for lack of assistance the patient is chained to a chair or a bed
Diagnosis of a disease of Parkinson
Clinical diagnosis of a disease of Parkinson takes place in three stages.
The 1st stage — recognition of a syndrome of parkinsonism and its sindromalny differentiation from the neurologic and psychopathological syndromes, anyway similar to true parkinsonism. True parkinsonism is a gipokineziya in combination with one of the following symptoms: a rest tremor (4-6 Hz), a muscular rigidnost, the posturalny instability which is not connected with primary vestibular, visual and cerebellar violations.
The 2nd stage — an exception of other diseases which can be shown by parkinsonism syndrome. There are several criteria of an exception of a disease of Parkinson:
- okulogirny crises
- therapy by neuroleptics before a disease debut
- existence in the anamnesis of repeated strokes with stupeneobrazny progressing of symptoms of parkinsonism, reliable encephalitis or repeated ChMT
- long remission
- exclusively unilateral manifestations within more than 3 years
- cerebellar symptoms
- nadjyaderny paralysis of a look
- earlier bright display of dementia
- earlier bright manifestation of vegetative insufficiency
- Babinsky's symptom
- tumor of a brain or open hydrocephaly
- inefficiency of high doses of a levodopa
- MFTP intoxication
The 3rd stage — identification of the symptoms confirming Parkinson's disease. For this purpose existence of at least three of following criteria is necessary:
- unilateral manifestations in a disease debut
- existence of a tremor of rest
- asymmetry of symptoms (with bigger degree of expressiveness on side of a body with which the disease began)
- 70-100% reactions for therapy by a levodopa
- the progressing course of a disease
- efficiency of a levodopa within 5 years and more
- duration of a disease is 10 years and more
Parkinson's disease needs to be differentiated from all diseases which are followed by parkinsonism syndrome: secondary parkinsonism, pseudo-parkinsonism, "parkinsonism plus". About 80% of cases of a syndrome of parkinsonism are the share of Parkinson's disease.
It is necessary to remember certain clinical features of parkinsonism which have to raise doubts in the diagnosis Parkinson's disease, for example: inefficiency of a levodopa, lack of a tremor, symmetry of motive violations, early manifestations of signs of peripheral vegetative insufficiency.
Treatment of a disease of Parkinson
Ways of treatment of a disease of Parkinson significantly differ at early and late stages of a disease therefore they should be considered separately.
Treatment of a disease of Parkinson at early stages.
Earlier diagnosing of a disease of Parkinson not always means the immediate beginning of any medicinal therapy. For determination of terms of the beginning of medicinal treatment it is necessary to consider disease severity, disease duration, rate of its progressing, any associated diseases, and also "personal factors" (professional, social and marital status of the patient, a mental state, features of the personality etc.). The purpose of such therapy — restoration (sufficient regress) of the broken functions by means of minimum possible doses.
Medicinal therapy at an early stage of a disease of Parkinson means use of the medicines increasing synthesis of dopamine in a brain, stimulating its emission and blocking its return absorption, oppressing disintegration of dopamine, stimulating dopamine receptors and the interfering death of neurons. The MAO-B selective inhibitors (, etc.), agonists of dopamine receptors belong to such medicines (piribedit, , etc.). Use of the above-stated medicines both in the form of monotherapy (more often), and in various combinations is allowed.
Above-mentioned medicines considerably concede by efficiency to levodopa medicines, however at early stages they quite are suitable for treatment of a disease of Parkinson. Theoretically at early stages of a disease of Parkinson agonists of dopamine receptors are capable to delay purpose of a levodopa, and at late stages — to lower its dose. However not in their advantage tells a large amount of side effects (stomach ulcer, orthostatic hypotension, mental violations, an eritromelalgiya, retroperitoneal fibrosis, etc.) and ability to reduce sensitivity of postsynaptic dopamine receptors.
The accurate criteria determining optimum time of an initiation of treatment by levodopa medicines no. Nevertheless, it is necessary to consider age of the patient (whenever possible after 60-70 years), to avoid early purpose of a levodopa, at selection of a dose to be guided by the patient's "otklikayemost" on medicine, improvements in its professional and social activity.
Treatment of a disease of Parkinson at late stages.
In independence of character of a course of disease of Parkinson surely there is a gradual transformation of a clinical picture of a disease. Over time already present violations progress and appear new, the majority of which hardly gives in to therapy, making thereby strong stressorny impact on the patient. Besides, the habitual effect of a levodopa changes — efficiency of medicine decreases, accrue medicinal dyskinesia (as result of hypersensitivity of dopamine receptors).
Decrease in efficiency of therapy is shown by decrease in term of medical action of each rod of a levodopa. The phenomenon of "inclusion switching off", the only way of fight against which is a gradual increase in a dose of a levodopa, is formed, and it in turn starts the vicious circle creating new problems against which it becomes heavier to fight. Real help in this case can be given in two ways: purpose of an additional dose of a levodopa for reduction of intervals between receptions; addition in the scheme of treatment of KOMT inhibitor and transfer of the patient to therapy by the combined medicine of a levodopa and entakapon.
Side effects of therapy by a levodopa. One of manifestations of reduction of the threshold of sensitivity to some side effects — a tendency to emergence oral (or others) giperkinez against the background of giperkineziya symptoms. Thus, in a clinical picture of a disease of Parkinson paradoxically symptoms of excess of dopamine (oral giperkineza) and its deficiency are combined (gipokineziya). Decrease in a dose of a levodopa in such situation gives only temporary elimination of a giperkinez, after a while it appears again. Orthostatic arterial hypotension at Parkinson's disease is shown usually by rather sharp lowering of arterial pressure soon after reception of a levodopa. Both the levodopa, and agonists of dopamine receptors therefore after definition of the reason of side effect it is necessary to reduce a dose of the corresponding medicine have such side effect.
Mental violations at Parkinson's disease can be shown in the form of a depression, alarm, apathy, visual hallucinations, agitation. Besides, emergence of the memorable, bright dreams is typical. Over time all above-mentioned violations progress and sooner or later are shown also in a condition of wakefulness. Therapy of such mental violations needs to be carried out together with the psychiatrist. Sometimes it is enough to save the patient from alarm and fear as they provoke more gross mental violations. The majority of medicinal diskineziya is shown at peak of effect of medicine. The most reliable way of their elimination — reduction of a single dose of a levodopa with preservation of a daily dose of medicine. Therefore fractional reception of small doses of a levodopa — the best way of prevention of diskineziya of this kind.
In a terminal stage of a disease of Parkinson the main difficulties are connected with a kakheksiya, loss of ability to standing, walking and self-service. At this time carrying out the whole complex of the rehabilitation actions aimed at providing optimum conditions for daily household activity of the patient is necessary. It is necessary to remember that at late stages Parkinson's disease becomes a heavy burden not only for the patient, but also for his family by which members also the specialized help can be required not only therapeutic, but sometimes.
Surgical treatment of a disease of Parkinson consists in carrying out stereotaksichesky destruction of a ventrolateralny kernel of a thalamus and a subtalamichesky kernel, and also deep stimulation of a brain. In case of the expressed akinetiko-rigidny syndrome the pallidotomiya, and also deep electrostimulation of a pale sphere and subtalamichesky kernel is recommended.
The forecast at Parkinson's disease
Steady increase of the expressed symptomatology is characteristic of Parkinson's disease. In 25% of cases within the first five years of a disease comes invalidizations or death. At 89% of the patients who endured 15 years of a course of disease of Parkinson inevitably there comes heavy degree of disability or death. Decrease in death rate of patients with Parkinson's disease in connection with the beginning of application of a levodopa, and also life expectancy growth is noted.