Dementia with Levi bodies is a progressive disease of the central nervous system, manifested by cognitive impairment, the development of psychotic symptoms and extrapyramidal disorders.
Among all causes of dementia development, this disease is on the third place, yielding to Alzheimer's disease and combined dementia.
The term "dementia" in Latin means dementia.
Content
- 1Causes
- 2Clinical picture
- 3Diagnostics
- 4Treatment
- 5Forecast
Causes
To date, the cause of the disease has not been reliably detected. In some cases dementia with Levi bodies is hereditary, but, as a rule, occurs sporadically.
At the heart of the disease is a violation of the protein exchange of a-synuclein, which is synthesized in neurons. Under the influence of unfavorable factors, a mechanism is triggered, which leads to a disruption in the processing of this protein and its accumulation inside cells, followed by the death of neurons. The accumulations of a-synuclein are called the "Levi body". The largest accumulation of Lewy bodies is found in the cortical cells of the cerebral hemispheres and subcortical structures.
The disease is equally common among women and men, often recorded at the age of 60-80 years.
Clinical picture
The clinical picture is represented by several groups of symptoms.
- Mental and psychotic disorders.
Psychiatric disorders are primarily a change in cognitive function. This is manifested by inhibition, inattention, decrease in cognitive functions. A person finds it difficult to perform a complex sequential action (dressing himself, going for a walk, etc.). Visually - spatial disorders appear, for example, the patient can not draw a clock correctly and specify the specified time with arrows or draw a geometric figure. In the late stages of the disease, problems are identified with the identification of surrounding people and / or self reflection in the mirror. Such patients do not recognize close people or confuse them with other people, take them for strangers. Attention becomes distracted, there are pauses in conversation or activity. Deficiency of cognitive functions leads to a sharp restriction of professional opportunities and the development of social disadaptation.
Another important symptom of mental disorders is the appearance of fluctuations, which are registered in 60-80% of patients. Fluctuations are fluctuations in the mental state, which are manifested by the alternation of episodes of confused consciousness and areactivity. In the initial period of hesitation a person is awake, but does not understand what surrounds him and where he is. Gradually wakefulness gives way to apathy, drowsiness and inhibition. The patient ceases to come into contact, becomes silent and withdrawn. After such an episode, enlightenment sets in: the orientation in place and time is restored, cognitive functions are partially returned, contact with a person is normalized. By duration, fluctuations can occur from a few seconds to several hours. In the same person, mental fluctuations can be of different duration.
Psychotic disorders are visual hallucinations. They occur in 80% of people with dementia with Levy bodies. Patients "see" three-dimensional images of people and animals in different colors, in some cases trying to come into contact with them. Criticism to visual hallucinations in most people is preserved, that is, it makes it possible to distinguish a real object from an imaginary one. Some patients categorically deny the presence of hallucinations, so they are identified with a thorough examination and may not be suspected of the relatives of the patient. Imagination can be accompanied by delirium, more often this description of what a person sees. Some patients believe that their relatives are replaced by twins (Kapgra syndrome). The presence of visual hallucinations is in direct proportion to the concentration of Lewy bodies in the brain amygdala, the para-hippocampal cortex and the cortex of the temporal lobes. Provoke the progression of psychotic disorders of infectious diseases, a change in the scheme of drug therapy and other conditions, but in some cases progression can be spontaneous.
- Extrapyramidal disorders.
Movement disorders are often manifested in the form of gait disturbance, it becomes shaky, leading to frequent falls. There is impoverishment of mimicry, the face becomes less expressive. At a part of patients at survey reveal a paresis of a sight (immovability of eyeballs).
- Vegetative changes.
For people with dementitsa with Levi bodies, jumps of arterial pressure, orthostatic hypotension, which is manifested by syncope when the body position changes, are characteristic. Violated urination, evacuation of the intestine in the form of constipation.
- Sleep disturbance.
Sleep becomes superficial. It becomes difficult for a person to fall asleep, he often wakes up at night.
Approximately in 60% of patients, the disease makes its debut with a picture of cognitive impairment. As the disease progresses, motor disorders become attached.
Diagnostics
- An examination of a neurologist, a psychiatrist.
Features of the clinical picture can in vivo suggest and / or diagnose the disease.
- MRI or CT scan.
Are additional methods of research. The images reveal a moderate diffuse atrophy of the brain substance, there are no specific changes.
- Electroencephalogram (EEG).
- The gold standard of diagnosis is a pathomorphological study of the brain after the death of the patient.
Treatment
Specific treatment of dementia with Lewy bodies is absent. The disease has a progressive nature, so the task of drug therapy is to alleviate the symptoms. Depending on which symptomatology prevails, the preparations are selected. It should be noted that the choice of a drug and the selection of its dose should be carried out by a specialist, since different groups of drugs can simultaneously exert a weakening effect on some symptoms and strengthen others (for example, antipsychotics weaken hallucinations, but enhance extrapyramidal disorders).
Medicamentous treatment includes the following groups of drugs:
- Correctors of cognitive disorders (dementia): memantine, galantamine.
- Antiparkinsonics are used to correct extrapyramidal disorders: levodopa.
- Correctors of mental abnormalities: galantamine, clozapine.
- Antidepressants: citalopram, sertraline.
- When urination is abnormal, a throsis is prescribed.
Forecast
Dementia with Levy bodies is a progressive disease, therefore the average life expectancy after debut of the disease is on the average 5 years.