Pick's disease, its difference from Nyman-Pick disease: photos, symptoms, treatment and prognosis

Peak disease is a rarely detected but severe disease of the brain, leading to a gradual atrophy of cells in the temporal and frontal lobes. Patients with Pick's disease are characterized by rapidly growing dementia, the age of patients within 50 years, cases with early and late onset of the disease are identified. The pathology of the brain is similar to all known Alzheimer's disease, although it has differences in some symptoms, the growth of the clinic, and, accordingly, in the treatment.

Peak Disease The disease was first described in 1892 by A. Peak, in the course of the studies there were revealed significant distinctive criteria with a disease with similar symptoms - Alzheimer's disease.

Differences are justified by a characteristic picture of the occurring changes:

  • Atrophic changes, as a rule, are of limited character - only the cells of the frontal and temporal lobes are affected.
  • Vascular pathologies are minor or completely absent.
  • No noticeable signs of inflammation, senile plaques in the vessels, and neurofibrils produced in patients with Alzheimer's.
For Pick's disease, the death of neurons is characteristic, which leads to thinning of the cortex in the brain regions. Increase in the size of the ventricles of the brain, the normal border between gray and white substance is erased.

Speech pathological changes and disturbances of the patient's usual, preceding illness, come to the fore in the forefront, in the last stages the patient shows total dementia-dementia.

Dementia in the

Peak Dementia Dementia is a progressive negative and non-reversible change in the structures of the brain, leading to a steady decline in intelligence, memory, loss of existing skills, the ability to learn, remember events, orientation in space.

In the photo a snapshot of the brain affected by dementia with

Peak Disease The consciousness remains for a long time from the onset of the illness clear, all signs of the disease develop about six months, although sometimes a sharp change in character and personality is observed.

The following adverse factors affecting the human body may provoke the development of dementia:

  • Prolonged, uncontrolled administration of certain pharmaceutical products.
  • Chronic avitaminosis of vitamins B.
  • Metabolic progressive disorders due to internal ailments.
  • Infections.
  • Alcoholism.
  • Tumors.
  • Bruises, craniocerebral trauma.

In the disease it is common to distinguish three, gradually passing into each other, the stage of development, the life span of a sick person rarely exceeds 10 years.

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The first stage of the disease

The character of a person changes towards egoistic orientation, there are unmotivated actions that the patient explains not by the possibility of not doing them.

  • Instinctive emancipation is the loss of the old moral principles. The patient can improve his physiological desires in any place, regardless of the environment. There is sexual promiscuity.
  • Changes in speech - the repetition of identical words, standard expressions, jokes.
  • Decreased self-criticism for their actions.
  • There are episodes of psychoemotional changes - hallucinations, delusions of jealousy, inferiority, etc.
  • Slowing down when performing movements, less often hyperkinesis.

Second stage

In the second stage, all the symptoms continue to progress and become focal.

The following signs are observed:

  • Short or persistent amnesia.
  • Aphasia - pathological disorders in speech activity. The ability to use words to express one's thoughts partially or completely ceases, and the patients also do not catch the meaning of the speech of others.
  • Apraxia - a violation of the former ability to perform actions. And there can be a violation in one link of the whole chain, for example, at the request to eat a candy - the patient unwraps the sweetness, throws out the contents and puts a wrapper in his mouth.
  • Agnosia - changes in visual, auditory perception, tactile sensations.
  • Akalkuliya - impossibility of correct execution of oral and written operations with a mathematical account.

All the symptoms of the second stage increase imperceptibly, in the beginning they can appear sporadically, then permanently.

The third stage of

The third stage of the disease is a deep, not reversible dementia, patients need psychological help and constant examination of relatives.

Pick's disease and heredity

Pick's disease is attributed to the degenerative negative changes that develop in the departments and cells of the brain. The disease is hereditary, so the presence of blood relatives with dementia, progressing after 50 years, should make you take better care of your health.

Neemann's disease of the peak in children

The similarity of the name of Pick's disease with another ailment - Niemann Peak's disease of some is misleading. The main difference in medical sources is the age of patients - Pick's disease develops at a sufficiently mature age and a characteristic symptom of the revealed pathology is dementia.

Niemann Pick's disease refers to medicine for hereditary diseases, characterized by an obvious disruption in lipid metabolism, which leads to the accumulation of lipids in the bone and brain, liver tissues, in the spleen and in the lungs.

Depending on the type of disease, the disease can manifest itself in about three months of life or in the puberty period, and children are born without signs of pathology. Inheritance occurs in an autosomal recessive type of development, that is, to transmit the disease it is necessary that both genes be mutated.

The photo shows schematically the symptoms of a child with Niman Pik's disease:

The disease is caused by a deficiency of the sphingomyelase enzyme, which leads to disruption of the entire lipid digestion process. Undivided lipids accumulate in monocytes and phagocytes, provoking their increase from the permissible norm several times.

When diagnosing all the organs of the child, the pathological growth of the liver, adrenal glands, and spleen is revealed, and their normal coloration is changed to yellow. In the lung tissues a spotted pattern is recorded.

It is accepted to classify Niemann-Pick disease on types A, B, C, they differ in development time, symptoms and course, prognosis for later life.

  • Niemann Pick's disease Type A is the most frequent form of ailment, it is typical for children of the first year of life, the prognosis is unfavorable - the sick children with all modern medicine possibilities seldom live up to 2 years. Children with mutated genes are born with the usual mass, in the first weeks they have no obvious problems in the body.
  • Type B develops after two years, involvement in the disease process of the structures of the nervous system is not detected. On the contrary, in some children, intellectual abilities and abilities are much higher than the average.

    It is considered the most favorable form of genetic disease, because with an effective and correctly developed principle of treatment, patients lead a normal lifestyle.

  • Type C - a youthful form, develops to two years, the main difference is the addition of changes in the structures and departments of the central nervous system. Lethal outcome in most patients occurs in adolescence.
Children with an identified Niemann-Pick disease occurring in type A and B rarely experience symptoms after 18 months. In type C, developing with pathological processes in the central nervous system, there may be signs of dementia.

Causes of

Peak Disease A single-valued cause leading to the development of cell atrophy and the structures of the frontal and temporal lobes is not reliably established.

Among the most likely provoking factors are:

  • The main risk factor is hereditary dependence. The disease is often detected among relatives who have a blood relationship.
  • Severe head injuries leading to the death of neurons.
  • Intoxication - chronic poisoning with salts of heavy metals, chemical compounds, alcohol. The probability of death of nerve cells rises several times in those cases when toxins act on the body for a long time. To the negative effect of toxins can be attributed and anesthesia, especially if it was applied several times.
  • Heavy mental disorders that are transmitted at any age.

Symptoms and manifestations of

The patients with Pick's disease examined have characteristic symptoms that are conventionally divided into two groups in medicine: patients have problems with written records, speech and behavioral difficulties.

Close people and relatives of the patient at the first stages of the onset of the disease note that he has a noticeable and rapidly changing personality.

Characteristic features for such patients are:

  • With atrophy in the frontal areas, noticeably increased mood without reason, carelessness with respect to household duties and work, inattention, absent-mindedness. A characteristic feature is sexual promiscuity.
  • At temporal localization of atrophic processes patients are on the contrary suspicious, there is a delirium of defectiveness, uselessness.

All patients suffer speech to some extent, stereotyped expressions, words can appear, the meaning of speech is simplified to a minimum, the grammatical construction of sentences is violated. Patients also do not understand the speech addressed to them, personal hygiene is not observed, sudden outbursts of excitement are noted.

In the middle of the development of brain atrophy, obesity is typical, followed by cachexia. In the late stages of the patient need constant supervision.
Pick's disease significantly changes a person's personality.

A modest woman with a rich vocabulary, a culture of speech, neat in clothes can turn into a creature in a few months that does not watch herself at all, does not possess tact and can not correctly and correctly associate words in the sentence.

Differences in Peak Disease from Alzheimer's Disease

Peak disease and Alzheimer's disease have one characteristic feature - dementia. But between these ailments there are a number of distinctive symptoms that help the doctor differentiate these pathologies.

  • Age. The Pick's disease develops after 50 years, whereas Alzheimer's disease is rarely detected before 60.
  • Patients with Pick's disease are already prone to vagrancy in the early stages, resisting caring for them. In Alzheimer's disease, these symptoms appear much later from the onset of the pathology.
  • In the initial stages of Pick's disease, attention, orientation in the terrain, the account does not suffer. In Alzheimer's disease, an important symptom is memory impairment.
  • Patients with Pick's disease are characterized by disinhibition, following their instincts, in Alzheimer's disease, the person's personality does not change in the early stages.
  • An early sign of Pick's disease is depletion of the vocabulary, appearance of stereotyped expressions in speech. Alzheimer's patients are characterized by a slower onset of speech problems.
  • The early loss of reading and writing skills is characteristic of patients with Alzheimer's. People with Pick's disease are able to write and read for a long time.

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Diagnosis

Diagnosis is made after examination and interview with the patient, conducting instrumental examinations. The doctor needs to talk with the patient's close relatives, revealing all the stages of personality change.

Assign the following examinations:

  • Electroencephalography - the detection of the activity of electrical impulses. With Pick's disease, abnormalities are noted.
  • Tomography - a layer-by-level examination of all parts of the brain.

Treatment of

No specific and effective treatment for Pick's disease has been developed. Pharmaceutical drugs are prescribed based on the symptoms and severity of the disease.

There are several groups of drugs prescribed for dementia.

  • Substitution treatment is performed by MAO inhibitors, antidepressants.
  • Neuroprotectors stimulate the activity of brain cells, improve ongoing metabolic processes. This contributes to the slowing of atrophic processes.
  • Anti-inflammatory therapy.
  • Drugs designed to correct identified mental disorders - sedatives, drugs that reduce aggressive manifestations.
For the patients, psychological support from relatives is important. In the late stages they can not be left alone, it is dangerous for society and for the patient himself.

Forecast

In the event of the detection of Pick's disease, the prognosis for life is unfavorable, all treatment is aimed only at improving the well-being and delaying the occurring atrophic changes.

Disease in about five years leads to a complete moral decay of the individual, marasmus occurs and a person becomes lost not only for society, but for themselves.

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