Gayet-Wernicke encephalopathy is an acute encephalopathy caused by a deficiency of vitamin B1, which is necessary for adequate metabolism of cerebral cells. Gayet-Wernicke encephalopathy is characterized by three clinical syndromes: mixed ataxia, oculomotor function disorder and violation of the integrative function of the central nervous system. Verification of the diagnosis is achieved by studying the anamnesis, assessing the neurological status and comparing them with the data of EEG, REG, biochemical blood test, cerebrospinal fluid examination, MRI of the brain. Treatment consists in emergency administration and subsequent long-term therapy with vitamin B1, the appointment of psychotropic, antioxidant, nootropic, vascular pharmaceuticals.
General information
Gayet-Wernicke encephalopathy is named after the French doctor Gaye and the German neuropsychiatrist Wernicke. The first described the disease, which he called “diffuse encephalopathy”, the second described a similar clinical picture called “upper acute polioencephalitis”, because he believed that it was based on inflammatory processes. Gayet-Wernicke encephalopathy occurs mainly in men aged 30-50 years, most often in the period from 35 to 45 years. However, in the modern literature on neurology, one can find isolated descriptions of the development of the syndrome before the age of 30. Although alcohol abuse is not the only cause, Gayet-Wernicke encephalopathy most often occurs precisely on the basis of alcoholism, and therefore it is attributed to acute alcoholic encephalopathy.
Causes
According to modern ideas, Gayet-Wernicke encephalopathy is caused by a pronounced deficiency of thiamine (vitamin B1) in the body. The latter is a cofactor of several enzymes involved in various metabolic processes of the human body. As a result of B1 deficiency, the biochemical reactions provided by these enzymes are inhibited. The consequence is a decrease in glucose utilization by nerve cells, i.e. their energy starvation, and extracellular accumulation of glutamate. Glutamate acts as an activator of receptors of ion channels of cerebral neurons. Its increased concentration leads to hyperactivation of the channels through which calcium ions enter the nerve cell. An excess of intracellular calcium entails the activation of a number of enzymes that destroy the structural elements of the cell, primarily mitochondria, and initiate apoptosis (self-destruction) of neurons.
Characteristic is the periventrivular lesion of cerebral tissues in the region of the III and IV ventricles, the sylvian aqueduct. The structures of the brain stem and the intermediate brain, the cerebellar worm, and the mediodorsal nucleus of the thalamus suffer mainly. The pathology of the latter is associated with the accompanying Gayet-Wernicke encephalopathy of memory disorders.
The cause of the disease can be any pathological processes that lead to thiamine deficiency. For example, hypovitaminosis, gastrointestinal diseases with malabsorption syndrome, prolonged fasting, inadequate parenteral nutrition, constant vomiting, AIDS, some helminthiasis, oncological diseases leading to cancerous cachexia, etc. However, in most cases, Gayet-Wernicke encephalopathy is associated with chronic alcoholism. The duration of alcohol abuse before the onset of the syndrome varies from 6 to 20 years, in women it can be only 3-4 years. As a rule, Gayet-Wernicke encephalopathy manifests in the third or at the end of the second stage of alcoholism, when daily alcohol consumption lasts for months. At the same time, 30-50% of patients already have a history of alcoholic psychosis.
Symptoms
As a rule, Gayet-Wernicke encephalopathy debuts after a prodromal period, the duration of which varies on average from several weeks to months. In the prodrome, asthenia, anorexia with aversion to certain dishes, instability of the stool (constipation alternating with diarrhea), nausea and vomiting, abdominalgia, sleep disorders, cramps in the fingers of the extremities and calf muscles, decreased vision, dizziness can be observed. In some cases, Gayet-Wernicke encephalopathy debuts without a prodrome against the background of acute somatic or infectious pathology, withdrawal syndrome.
The characteristic triad of the manifestation of acute Gayet-Wernicke encephalopathy is considered to be: confusion, ataxia, oculomotor disorders (ophthalmoplegia). However, it is observed only in 35% of patients. In most cases, there is an inability to concentrate attention, apathy, disorientation, lack of coherent thinking, a disorder of understanding and perception. If Gayet-Wernicke encephalopathy debuts against the background of withdrawal syndrome, alcoholic delirium is possible, accompanied by psychomotor agitation. Ataxia is usually expressed to the extent of abasia — the inability to walk independently and even stand. It is mixed: cerebellar, vestibular and sensitive. The latter is caused by polyneuropathy, the presence of which is stated in 80% of cases. Ocular disorders include strabismus, horizontal nystagmus, drooping of the upper eyelid, discoordination of friendly movements of the eyeballs; in the later stages — myosis.
In the absence of treatment, Gayet-Wernicke encephalopathy often leads to coma and death. Against the background of ongoing therapy, there is a gradual regression of the above symptoms. First of all, ophthalmoplegia disappears. This may occur a few hours after the start of vitamin B1 administration, in some cases — after 2-3 days. In the absence of such dynamics, the diagnosis should be reviewed. Disorders of consciousness regress more slowly. Against this background, problems with the assimilation of new information and memory disorders (fixation amnesia, false memories) that make up the Korsakov syndrome clinic are gradually emerging.
Of oculomotor disorders, half of the patients persistently have horizontal nystagmus, which occurs when the gaze is shifted to the side. A similar vertical nystagmus can be observed for 2-4 months. The reverse development of ataxia and vestibular disorders can take from weeks to months. Approximately 50% of patients retain persistent residual ataxia, walking remains clumsy and slow.
Diagnostics
The neurologist can diagnose “Gaye-Wernicke encephalopathy” with anamnesis data, a characteristic clinical picture, and regression of symptoms against the background of thiamine therapy. During the examination, attention is paid to signs of chronic malnutrition (reduced body weight, dry skin and reduced elasticity, deformed nails, etc.). In the neurological status, there is a disorder of consciousness, mixed ataxia, ophthalmoplegia, polyneuropathy, signs of autonomic dysfunction (hyperhidrosis, tachycardia, arterial hypotension, orthostatic collapse).
In the biochemical analysis of blood, a significant decrease in the activity of transcetolase and an increase in the concentration of pyruvate is possible. After the lumbar puncture, the examination of the cerebrospinal fluid determines that it corresponds to the norm. When detecting pleocytosis or a protein content of more than 1000 g / l, one should think about the development of complications. A caloric test diagnoses the presence of almost symmetrical bilateral vestibular disorders.
In half of the cases, Gayet-Wernicke encephalopathy is accompanied by generalized slowing of waves during EEG. REG often determines a diffuse decrease in cerebral blood flow. CT of the brain, as a rule, does not register pathological changes in cerebral tissues. MRI of the brain allows you to identify hyperintensive areas in the medial nuclei of the thalamus, mamillary corpuscles, walls of the III ventricle, reticular formation, gray matter surrounding the Sylvian aqueduct, the roof of the midbrain. The affected areas tend to accumulate contrast, introduced during additional contrast during MRI. Petechial hemorrhages and signs of cytotoxic edema can be detected in these areas.
Treatment and prognosis
Gayet-Wernicke encephalopathy is an urgent condition. Urgent hospitalization and early initiation of vitamin B1 therapy are required. Thiamine is administered intravenously drip twice a day, then switch to intramuscular injection. In parallel with this, other vitamins are prescribed: B6, C, PP. Delirium is an indication for the appointment of barbamil, chlorpromazine, diazepam, haloperidol, etc. psychotropic drugs. Cytoflavin infusions are used as an antioxidant treatment. If necessary, therapy is carried out with the participation of a psychiatrist or a narcologist. In the future, to improve mnestic functions, courses of nootropics (piracetam, gamma-aminobutyric acid, ginkgo biloba extract) and vascular pharmaceuticals (pentoxifylline, vinpocetine) are prescribed, repeated courses of thiamine therapy are carried out.
Gayet-Wernicke encephalopathy has a serious prognosis. In 15-20% of cases, hospitalized patients die, most often from liver failure or intercurrent infections (pulmonary tuberculosis, severe pneumonia, sepsis). If Gaye-Wernicke encephalopathy has an alcoholic genesis, then its transformation into Korsakov psychosis is observed, which is a persistent psycho-organic syndrome and associated with chronic alcoholic encephalopathy.