Apallichesky syndrome — the clinical simptomokompleks including lack of signs of sensibleness in the presence of opening of eyes, dream/wakefulness alternation. According to neurologists, it is connected with extensive defeat of bark of a brain. Can be transition state at an exit from a coma. It is diagnosed exclusively clinically by means of scales for consciousness level assessment. Tool inspections (EEG, MRT, MSKT, PET, UZDG) are auxiliary, allow to establish causal pathology. Treatment is directed to restoration stimulation, maintenance of the vital functions, the prevention of complications, reduction of hydrocephaly.
The new bark (neocortex) consisting of 6 layers of neurons covers a surface of cerebral hemispheres and has the anatomic name "pallium" — a raincoat. Respectively, apallicheskiya means lack of a pallium, its functional "switching off". The term "apallichesky syndrome" is offered by the German doctor Kretchmer in 1940, is used in Germany, the CIS countries. In English-speaking sources on neurology the term "vegetative state" entered in 1972 is used. The Apallichesky Syndrome (AS) occurs at 25-100 patients on 1 million people in population, in many countries the tendency to increase in number of cases is noted. The EXPERT is observed at persons of both sexes of various age groups – from chest babies to very old men.
Reasons of an apallichesky syndrome
The EXPERT is the cornerstone total or subtotal damage of neocortex at preservation of function of a trunk of a brain. Act as Etiologichesky factors:
- Craniocereberal trauma. Traumatic genesis has the majority of cases the EXPERT of young age.
- Cerebral hypoxia. It is noted at poisoning with carbon monoxide, asphyxia, heavy arterial hypotonia, cardiac arrest owing to heart diseases, during surgeries.
- Neuroinfections. The Apallichesky syndrome can become the result of sharp infectious processes with extensive defeat of brain bark, arise at a final stage of slow infections of TsNS (a leykoentsefalit of Shilder, the progressing krasnushny pan-encephalitis).
- Brain tumors. In some cases extensive tumoral processes bring to the EXPERT, caused by a hypoxia, neurotoxicosis, brain hypostasis.
- The progressing degenerate diseases. Are the main reason the EXPERT at patients of advanced age. The syndrome is observed at a late stage of Alzheimer's disease, Kreyttsfeldta-Jacoba, Peak, vascular dementia, alcoholic encephalopathy.
- Sharp dismetabolichesky states. An uraemic, hypoglycemic, hepatic coma can pass into an apallichesky state.
- Anomalies of a brain: mikrotsefaliya, heavy cortical dysplasia. Cause an apallichesky syndrome at children of younger age.
The morphological picture of cortical defeat at the EXPERT is not specific, differs at different patients. Hypoxemic damages are followed by a necrosis, traumatic — diffusion aksonalny damage, degenerate — an atrophy. Variability of volume and the nature of defeat, lack in some cases of considerable morphological changes, demonstrates the functional violations leading to bark "switching off". Pathophysiological bases of these processes are in a studying stage.
Transition from a coma to an apallichesky state is characterized by restoration of function of a retikulyarny formation, subcrustal structures. Renewal of communications of a subcortex and bark leads to further restoration of consciousness. If cortical communication is not reestablished, not formed again or formed incorrectly, there is a chronic vegetative state — an apallichesky syndrome. In the outcome of the progressing cortical degeneration the EXPERT develops owing to mass loss of the communications providing interaction of neurons in bark and with underlying structures.
Symptoms of an apallichesky syndrome
The basis of a clinical picture is made by a paradox: existence of visible signs of consciousness in the absence of objective criteria of understanding by the patient of and the world around. The patient opens eyes, moves them in a condition of wakefulness, reacts to painful irritations, passes the cycles "dream wakefulness" that forms impression of sensibleness. However no signs of conscious activity, purposeful activity are observed. The movements are spontaneous, emotional and conscious reactions are absent. Movement of eyes is chaotic, reaction of tracking is not observed. Alternation of a dream and condition of wakefulness does not depend on time of day.
Face of the patient masklike, without mimicry. Chewing and swallowing are slowed down, the chewing movements, blinking, yawning can be noted. In response to painful incentives there is not purposeful motive reaction which is followed by increase of ChSS and breath, expansion of pupils. Function of pelvic bodies is not controlled. Epileptic paroxysms are possible. Increase in a muscular tone is typical: brushes are compressed, feet are bent and given in a condition of plantar bending, an extremity. Functioning of a hypothalamus and a brain trunk provides maintenance of necessary haemo dynamics, respiratory activity, vegetative function. In a phase of wakefulness prevalence of sympathetic nervous system (increase of ChSS, AD, the fever is possible), in a condition of a dream — parasympathetic is characteristic (decrease in AD, ChSS, the increased sweating).
The Apallichesky syndrome of traumatic, hypoxemic, dismetabolichesky genesis arises sharply, is often observed after the previous coma. The Apallichesky simptomokompleks at degenerate processes develops gradually, sometimes within several years. In the first case restoration of consciousness which first signs are fixing of a look, tracking by eyes behind a subject, implementation of simple instructions requests is possible (to close eyes, to squeeze fingers). As cases of emergence of tracking for a look without further restoration are known, it is considered to be this symptom criterion of an exit from an apallichesky state, only if it is combined with other signs of expansion of consciousness.
Owing to a constant spastic condition of extremities contractures of joints develop. Long finding of the patient in a bed with sharp restriction of physical activity promotes developing of decubituses, stagnant pneumonia. Accession of a secondary infection of urinary tract with development of pyelonephritis is possible. The Apallichesky syndrome can lead to final fading of all brain functions with a lethal outcome. Deadly to the patient can become the infectious complications passing into sepsis. The correct leaving, food, the supporting therapy are capable to delay emergence of complications, to increase life expectancy of the patient.
Due to the lack of accurate criteria of consciousness and sensibleness to diagnose an apallichesky syndrome not easy. According to the standard rules, at babies the diagnosis is established after three-months age as earlier it is impossible to differentiate conscious and reflex behavior authentically. Neurologists, intensivists, neurophysiologists, if necessary — neurosurgeons participate in diagnosis. The following diagnostic events are held:
- Neurologic survey. The contact with the patient completely is absent. The spontaneous movement of eyeballs, not purposeful reaction to painful incentives is noted. Kranialny and spinal reflexes, vegetative function are kept. The hyper tone of muscles of extremities with plasticity signs, increase in tendinous reflexes, existence of symmetric pathological reflexes is observed.
- Electroencephalography. Low-wave EEG, delta or a theta rhythm is characteristic. At 10-20% of patients it is noted alpha or a beta rhythm. Restoration is followed by emergence of a distinct alpha rhythm.
- Brain MRT. MRT and other ways of neurovisualization (KT, MCKT) do not reveal specific morphological changes. The picture corresponds to the main disease: the centers of a necrosis, a hematoma, tumor, intracerebral degenerate processes are defined, swelled brain substance. At 75% of patients hydrocephaly is noted. The majority of cases the EXPERT are followed by atrophic changes of bark, however the similar atrophy is observed at the patients who are in consciousness with dementia.
- Brain PET-KT. Allows to diagnose decrease in cortical metabolism by 40-50%. Restoration proceeds with the activation of a metabolism registered on PET.
- Transkranialny UZDG. It is directed to assessment of cerebral haemo dynamics. Has auxiliary value, it is used mainly at the solution of a question of expediency of the shunting operation at patients with hydrocephaly.
It is necessary to differentiate an apallichesky syndrome from a coma, conditions of the minimum consciousness, a sopor. The differentiation is carried out by means of clinical scales. Tool methods cannot precisely specify the level of consciousness of the patient, allow to establish the nature of defeat of bark, to judge the level of metabolism of cerebral fabrics.
Treatment of an apallichesky syndrome
Therapy is directed to life support of the patient, prevention of complications and restoration of consciousness. The uniform standard of maintaining patients is absent. Treatment is carried out is long, sometimes for months. Conservative and surgical methods are applied:
- Stimulation of recovery processes. The medicamentous component includes powerful nootropic, vitamin, vascular therapy, endolyumbalny introduction of oxygen. Regular touch stimulation with use of all range of incentives is in parallel carried out: acoustical, tactile, visual, olfactory.
- Artificial nutrition. It is carried out through gastrosty as probe food often is followed by complications: aspiration of food in airways, a gastroezofagealny reflux, ulcerations mucous in places of contact with the probe.
- Prevention of complications. For the purpose of reduction of a spastichnost and prevention of contractures miorelaksant appoint. The best prevention of decubituses and hypoventilating pneumonia is the adequate leaving including change of linen, change of a pose, jumping by means of special orthopedic devices, passive physiotherapy exercises, massage. Familiarizing with leaving of relatives of the patient is expedient.
- The shunting operations. Are shown at the expressed hydrocephaly. Lyumboperitonealny and ventrikuloperitonealny shunting is most widespread.
- Deep electrostimulations of a brain. It is carried out by stereotaksichesky introduction of microelectrodes by means of which stimulation of the activating systems of a trunk is carried out.
- Neurotransplantation. The EXPERT is a new experimental technique of treatment. Activates regeneration of cerebral fabrics, provides material for reconstruction of the damaged sites. Introduction of embryonic nervous cages is made intraventrikulyarno (in brain ventricles), intratserebralno (in bark or the damaged deep sites of a brain).
Forecast and prevention
The outcome depends on the nature of the brain defeat which caused an apallichesky syndrome, age of the patient, duration of a coma, existence of convulsive attacks. Sharply developed EXPERT can come to the end with consciousness restoration, but in most cases patients remain disabled people owing to the expressed psychoorganic syndrome. In case of the progressing cerebral degeneration the apallichesky syndrome is a terminal stage and terminates letalno. Emergence prevention the EXPERT consists in the prevention of injuries, neuroinfections, intoxications, timely treatment of cardiovascular pathology.