Pick's disease: one step from behavioral abnormalities to dementia

Pick's disease is one of the types of senile dementia in which atrophic changes occur, most often in the frontal and temporal lobes of the brain. It occurs mainly in older people over 50 years of age. Clinical manifestations are similar to Alzheimer's disease, but are less common and more malignant. Women are diagnosed twice as often as men.

Disease history

Pick's disease has been little studied and is rarely diagnosed. From the moment of detection, 6–8 years pass until the end of life. The disease is based on atrophic processes in the brain, and this is not due to a hereditary predisposition, although doctors previously considered genetics to be the main factor in the development of the disease.

Czech doctor Albert Pick was the first to describe the symptoms of the disease in 1892, after which the pathology was named after him. It was he who suggested a hereditary connection - he discovered that relatives of people who suffer from dementia are also likely to develop it.

Arnold Pick did not consider pathology a separate disease, but assumed that it was only a variant of senile dementia. When the development of the disease was studied pathologically, distinctive morphological features were discovered, and it was identified as an independent nosology.

1.General information

In the psychoneurological branches of medical science, a special category consists of diseases, the general essence of which is the degeneration of nerve cells - neurons. Performing fundamental control and regulatory functions on the scale of the entire organism, the neural tissue of the spinal cord and brain with the development of such pathological processes loses its inherent complex structure and organization, decreases in volume (atrophies) and copes with the tasks assigned to it increasingly poorly. Most of these diseases are caused by inherited genetic defects, but for some neurodegenerative processes the causes are still completely unclear. In the popular consciousness, such diseases are associated primarily with old age, however, only senile dementia itself can be associated with purely age-related atrophy of the cerebral cortex, while other diseases of this kind begin, as a rule, in adulthood and old age (and sometimes in young), proceeding faster and more catastrophically. Almost all neurodegenerative diseases of the central nervous system (spinal cord and brain), whether focal or diffuse, in the terminal stage lead the patient to a vegetative existence and total dementia (dementia).

Diseases in this group are fortunately quite rare, but published statistics are often contradictory and may be underestimated. At the very least, terms such as Parkinson's syndrome or Alzheimer's disease are constantly heard, and sometimes even in a highly specialized medical environment there is a tendency towards a broad, undifferentiated interpretation of presenile (presenile) atypical dementias as different variants of the same Alzheimer's disease. However, this approach is apparently still incorrect: both clinically and pathomorphologically, significant differences are found even between very similar diseases.

In particular, Pick's disease is an independent neurodegenerative process with its own specific symptoms and dynamics of development.

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Description of the disease

According to the literature, Alzheimer's disease is four times more common than Pick's dementia. Due to the rarity and complexity of diagnosis (at least intravital), there is not much data on the disease. It is known for sure that men get sick less often, and the pathology is progressive.

Senile dementia is characterized by severe mental impairment and neurological pathologies. The brain is affected mainly in the frontotemporal area, resulting in impaired behavior and speech.

Pick's disease is often diagnosed against the background of mental disorders, but they are not considered the root cause of the disease, although they provoke its development. Not only the temporal and frontal lobes can undergo atrophy - sometimes it goes beyond and affects the subcortex. In this case, there is no inflammation, vascular disorders are either absent or mild.

The neurodegenerative disease progresses slowly at first, but in the initial stages entire groups of nerve cells die. Over 5-7 years, they can spread to the entire brain, although the focus is observed mainly in the temporal and frontal lobes. First, destructive processes affect the second and third convolutions, and a little later - the first. Less commonly, the parietal lobes may be affected.

The manifestation of Pick's disease begins in the anterior frontal regions of the brain, which immediately affects a person's behavior and personal characteristics. Later, the temporal lobes, which are responsible for intelligence, memory, and concentration, undergo changes. The cortex of the speech analyzer is also destroyed, which immediately affects speech abilities.

Unlike other neurodegenerative pathologies in psychiatry, Pick's disease is characterized by deep damage to the entire lobe of the brain, and not just part of it. First, the upper layers are affected, then the process spreads deeper.

Examination of brain tissue under a microscope showed that patients experience “swelling” of cells, the thickness of the gray matter decreases, the transition from gray to white matter is blurred, and the boundaries are unclear. Another characteristic feature is the separation of the cerebral vascular endothelium. This causes circulatory disorders, stagnation of cerebrospinal fluid and, as a result, enhances neurodegenerative processes and atrophy. Moreover, separation of the endothelium always occurs in Pick's disease, but in Alzheimer's disease - only in 25% of cases.

Prognosis and treatment of the disease

Since the original source of the anomaly has not yet been identified, therapeutic measures are aimed primarily at symptomatic treatment. In case of aggressive behavioral reactions, medications are prescribed that are designed to activate processes that inhibit the psyche—neuroleptic drugs. At the initial stage, psychotherapy and work with a psychologist are carried out. Further, to preserve partially remaining cognitive functions, it is recommended to work in the sensory room, stimulate the feeling of presence, and draw.

The described measures are methods that slow down degeneration, but they do not stop the progression of the disease, which proceeds much faster compared to Alzheimer's disease. Complete destruction of personality occurs within five to six years after the identification of primary signs. The average life expectancy of such patients is 10 years, despite concurrent therapy.

Causes of the disease

The etiology of Pick's disease is still unclear. Although there are “familial” episodes of the disease, more often it is not transmitted from parents to children, but is diagnosed in siblings. However, the hereditary factor has been completely refuted.

Etiological factors are considered:

• negative effects on humans, in particular on the brain, of chemicals;
• incorrect dosage and/or frequent use of anesthesia;
• mental disorders.

Not the main, but provoking factor can be considered a lack of B vitamins, traumatic brain injuries, and intoxication. Some experts believe that a provoking factor may also be a failure in the metabolism of tau protein, a component that is an element of the cell membranes of neurons.

In this disease, brain cells undergo atrophy and destruction - they lose functionality, decrease in size or are completely destroyed. Regardless of the combination of symptoms, it is always accompanied by dementia due to a violation of the higher function of the cerebral cortex. In this case, dementia is acquired.

The risk group includes patients over 50–55 years of age, females, and in whose family there are cases of this type of dementia. Traumatic brain injuries do not serve as a provoking factor for infection, but significantly accelerate and aggravate the course of the disease.

The disease can be diagnosed in younger people as early as 40–45 years of age. But at this age it progresses slowly, the symptoms are mild, so patients rarely see a doctor, and at the time of diagnosis, the second stage is already detected. The onset most often is not so pronounced and can manifest itself in increased anxiety, loss of control over one’s actions, and depressive states, which the patients themselves usually justify as stress or other similar reasons.

Reasons for the development of the disorder

The exact causes that can cause Pick's disease have not been established. However, probable provoking factors may be:

1. Heredity
   . Very often, pathology can be diagnosed in the patient’s closest relatives.
2. Head injuries
   that lead to the death of neurons.
3. Intoxication
   - in this case, neuronal death occurs due to prolonged systematic exposure to alcohol and chemicals that have a toxic effect on the body. The same applies to anesthesia, which was used many times.
4. Previously suffered mental disorders
   .

Difference between Pick's disease and Alzheimer's disease

Alzheimer's and Pick's diseases are similar, but they are two separate forms of pathology with their own clinical manifestations. Common to the disease is dementia. But when diagnosing Pick's disease:

• memory is partially preserved;
• concentration remains;
• changes in personality characteristics are more pronounced - absence of emotions and a decrease in the frequency of their manifestation, apathy, indifference;
• if the basal cortex of the brain is damaged, the concepts of morality and morality disappear - a person loses his sense of tact and does not comply with generally accepted norms of behavior;
• if the brain is simultaneously affected in the area of ​​the forehead and temples, speech changes, it becomes illegible, clumsiness appears in movements.

Unlike Alzheimer's disease, Pick's disease has difficulty navigating a new environment. He may go very close to home, but is unable to find his way back. In addition, disorientation in time is characteristic - it is difficult to determine what time of day it is.

In general, Alzheimer's disease is milder and its symptoms are not as pronounced. This affects the patient’s quality of life and its duration. Patients, as a rule, live shorter lives, their condition is more severe, and due to the progression of the disease they quickly lose their human appearance.

4.Treatment

There is currently no etiopathogenetic treatment for Pick's disease. Palliative symptomatic therapy is carried out in the form of neurotrophic stimulants (tissue nutrition), antipsychotics to relieve severe behavioral disorders and affective instability, and antiparkinsonian drugs according to indications (symptoms of parkinsonism often develop). However, the effectiveness of all measures taken is low, and, as a rule, it is not possible to significantly slow down the progression of the disease. The prognosis is unfavorable.

Stages of the disease

Pick's disease is divided into three stages. From detection to the end of the third stage, a maximum of 10 years passes, more often 6-7. Types of pathology:

1. First stage. Characterized by disinhibition and actions that have no motive. For example, the patient may have neutralized basic instincts and lack control over his actions and emotions. Another characteristic feature of the onset of the disease is selfishness. In addition, libido sometimes increases and sexual disinhibition occurs. There are also changes in speech - repeated repetition of the same words, sentences or stories occurs. The emotional sphere at this stage is relatively stable - most often there is euphoria, which manifests itself in high spirits, but it can suddenly give way to complete apathy. There is no memory impairment yet, but at this stage the person cannot explain his strange behavior or justifies it with impatience.
2. Second stage. All the symptoms described above get worse. Focal signs of dementia appear, speech becomes even less intelligible, it is difficult for the patient to understand and assimilate information, he may forget events that happened a long time ago or very recently. It may be difficult for him to count and perform targeted movements - fine motor skills are impaired. At this stage, the disease is almost identical to Alzheimer's disease, but there are no epileptic seizures. Another characteristic feature is imitating the movements/facial expressions of other people and automatically repeating new words. The pain threshold also increases (cutaneous hyperalgesia).
3. Third stage. It is considered final. It is characterized by complete insanity, decreased muscle tone and, ultimately, a vegetative state of the patient. He cannot move, solve everyday issues, for example, eat, relieve natural needs, or dress. At this stage, incurable disability is diagnosed, which ends in death.

Stage 3 dementia is irreversible. At this time, the patient needs constant care - he himself is not able to perform even the simplest tasks, since his capacity, both mental and physical, is lost.

Niemann-Pick disease in children

Pick disease should be distinguished from Niemann-Pick disease, which is a hereditary disease of lipid metabolism. Other names: BNP, sphingomyelinosis, sphingomyelin lipidosis.

LBP is accompanied by pathological accumulation of lipids in lysosomal organelles. Niemann-Pick disease affects the liver, spleen, lungs, bone marrow, and brain.

The disease is classified as an autosomal recessively inherited storage pathology or LSD (lysosomal storage disease).

Niemann-Pick disease is divided into three types: A, B, C.

The occurrence of all types of Niemann-Pick disease is based on genetic mutations. Types A and B are caused by a mutation in the SMPD-I gene, located at the 11p15.4-p15.1 locus. This gene encodes the enzyme acid sphingomyelinase.

Type C is caused by mutations in the NPC1 (18q11-q12 locus) and NPC2 (14q24 locus) genes. These genes encode carrier proteins involved in the transport of cholesterol and other lipids within the cell. The pathology is inherited in an autosomal recessive manner.

Symptoms

The severity of symptoms, as well as their range, depend on the stage of Pick’s disease. Main signs of the disease:

1. Loss of moral principles. The patient may relieve his natural needs in inappropriate places, not control his behavior and statements, selfish character traits become aggravated, and overall behavior becomes asocial.
2. Immediate realization of instincts. This also applies to sexual desire - with increased libido, frequent casual sexual contacts are possible, including with strangers. The possibility of waiting also disappears - all the physiological needs of the patient must be fulfilled as quickly as possible. For example, bulimia may develop - uncontrolled eating of large amounts of food even after satiety.
3. Change in speech. It becomes indistinct, multiple repetitions of jokes, words, and individual phrases can be traced. This is called the "gramophone record" sign.
4. Mood swings. It can range from euphoria to complete apathy, and only a few minutes can pass in between.
5. Dementia. Its severity depends on how atrophic processes develop in the brain. But in general, emotional instability, periods of disinhibition and rigidity, loss of desires, goals, and complete lack of will can be traced. It is possible to develop depression, and sometimes a combination of inappropriate behavior with euphoria.

The more Pick's disease progresses, the more severe the symptoms become:

• misunderstanding the speech of others;
• loss of basic numeracy, writing and reading skills;
• inadequate perception of what is happening around;
• memory losses;
• inability to perform actions consistently and logically;
• at the end, deep dementia, disorientation, and loss of self-care skills develop.

If at first such violations are episodic in nature, then by the end of the second and third stages they become permanent, progressing until the complete destruction of the intellect.

The death of patients with Pick's disease is most often due to a combination of a number of factors:

• cerebral insufficiency;
• infection of bedsores followed by sepsis;
• congestive pneumonia due to constant exposure to a horizontal position;
• ascending pyelonephritis, etc.

Changes affect the physical and mental spheres of a person. Some develop obesity or, on the contrary, exhaustion, intelligence suffers greatly - vocabulary gradually decreases, a person loses previously acquired knowledge, even if before the disease he was a specialist in his field with an above-average level of intelligence.

Diagnostics

Diagnosis usually begins with interviewing family and friends, collecting anamnesis and examining the patient. Diagnostic measures are divided into several stages:

1. Differentiation of the disease from other pathologies, for example, Alzheimer's disease. It is also necessary to exclude oncology, vascular disorders, schizophrenic syndrome, and tumor processes in the frontal zone of the brain.
2. Additional consultations with narrow specialists. For example, consultations with a neurologist or psychiatrist may be required.
3. EEG. A decrease in the bioelectrical signature emanating from the frontal areas of the brain is recorded.
4. REG, ultrasound. This diagnosis allows you to exclude or confirm vascular changes.
5. EchoEG. Allows you to detect the presence of fluid in the brain.
6. MRI. Necessary for clear visualization of atrophic changes in brain tissue.

Laboratory tests, as a rule, are not required for the patient. They have no diagnostic value. It is extremely rare that a biochemical blood test may be prescribed.

Worth knowing!

Quite often, Pick's disease is confused with Niemann-Pick's disease. The main difference between these two ailments is the age indicator. If the first is detected after fifty years, the second is diagnosed in childhood, and this illness is not a mental disorder.

Niemann Pick disease is hereditary. It occurs due to a violation of lipid metabolism, as a result of which these natural compounds accumulate in the brain, liver, spleen and lungs.

Depending on the form of the disease, the first manifestations of pathology can be detected in a child as early as several months or in adolescence. Pathologies in a child are not diagnosed at birth.

The mechanism of inheritance occurs through mutation of certain genes. The disease is caused by a deficiency of sphingomyelanin, which is responsible for the breakdown of lipids. As a result, the process is disrupted, and lipids accumulate in the cells, enlarging them.

Diagnosis of the disease reveals pathological enlargement of the spleen, adrenal glands, and liver. The color of the organs changes, becomes yellowish, and spots appear on the lungs.

Treatment

Unfortunately, there is currently no effective therapy for Pick's disease. The main task of the doctor after diagnosing this disease is to support the patient’s body and slow down the destructive processes in the brain. Accordingly, treatment is selected based on the stage of the pathology and the presence of certain symptoms. It can be:

• antidepressants;
• MFO inhibitors;
• neuroprotectors to stimulate metabolic processes in the brain and increase cell activity, which helps slow down atrophic processes;
• sometimes anti-inflammatory therapy is needed;
• sedatives stabilize the psycho-emotional background and reduce aggressiveness.

One of the stages of helping a patient is constant care in the final stages. When a patient loses self-care skills, not only is he unable to perform most common household tasks, but he may also cause harm to himself or others. The solution to this problem is professional nursing care if relatives cannot be with the patient around the clock.

There is also no specific prevention for Pick's disease. General recommendations relate to maintaining a healthy lifestyle, promptly consulting a doctor if you suspect brain pathologies, and taking activities that improve cognitive function. People who have relatives with a similar disease or other types of dementia should be especially careful.

The prognosis for Pick's disease is poor. The duration of the disease until the patient’s death is influenced by age, general health, and the adequacy of the prescribed therapy.

Rapid progress of the disease

Pick's disease develops rapidly. These are the stages of disease development.

Initial stage of development

At the first stage, character changes occur. A person becomes egoistically oriented and performs actions that are atypical for him:

• emotional emancipation - moral principles are devalued, sexual promiscuity arises;
• changes in speech - the patient often repeats the same jokes and words;
• loss of self-control;
• disturbances in the psycho-emotional sphere - hallucinations, increased feelings of jealousy and inferiority occur.

Second stage

Symptoms progress unnoticed, may occur periodically, and then become constant:

• onset of amnesia;
• speech disorders, the patient becomes unable to express himself clearly using words, and there is no understanding of the meaning of other people’s speech.
• impairment of the ability to perform even the simplest actions;
• deformation of visual, auditory, tactile perception;
• loss of ability to perform oral and written actions, counting.

At the last stage, dementia takes on a deep, irreversible form. The patient becomes unable to self-care and requires full care.