Pick’s disease is a variant of senile dementia with atrophic changes localized mainly in the temporal and frontal lobes of the brain. Clinically manifested by a violation of behavior with antisocial tendencies and disinhibition of instincts, progressive decay of cognitive functions. The list of diagnostic measures includes EEG, cerebral vascular ultrasound, Echo-EG, psychiatric consultation, CT, or MRI of the brain. Treatment consists in long-term administration of anticholinesterase agents, memantine, nootropics, but it does not prevent a complete intellectual-mnestic disintegration of the personality.
ICD 10
G31.0 Limited brain atrophy
General information
Pick’s disease is a rare type of frontotemporal dementia with a characteristic onset at the age of 50-60 years and predominant atrophy of the temporal and frontal lobes of the brain. It is named after Arnold Pick, who described it in 1892. Pick himself considered the pathology described by him to be only a separate clinical variant of senile dementia. However, later, after the identification of distinctive morphological features confirmed by pathological anatomical studies, Pick’s disease was isolated as an independent nosology.
Data on the prevalence of the disease have not been collected due to its rarity and difficulties of lifetime diagnosis. There is evidence in the literature that Pick dementia is 4 times less common than Alzheimer’s disease. Men get sick somewhat less often than women. The steadily and rapidly progressing nature of the pathology, the difficulties of its diagnosis and therapy make Pick’s disease an urgent problem of modern gerontology and neurology.
Causes
The etiology remains unclear. Family cases of the disease prompt researchers to think about its hereditary nature. However, sporadic cases are observed much more often than family ones, and brothers and sisters are more likely to get sick within the same family than relatives in different generations. Among the possible etiofactors are long-term effects on the brain of harmful chemicals. This can also include the use of anesthesia, especially in cases of its frequent use or inadequate dosing. Some authors believe that Pick’s disease may develop due to a previous mental disorder. Along with this, researchers are inclined to believe that factors such as intoxication, traumatic brain injuries, infections, mental disorders, vitamin g hypovitaminosis. B only plays a provocative role.
Morphologically, atrophic processes in the frontal and temporal lobes of the brain are determined, often more pronounced in the dominant hemisphere. Atrophy affects both the cortex and subcortical structures. At the same time, there are no inflammatory changes. Vascular disorders are not characteristic or poorly expressed. Senile plaques and neurofibrillary plexuses typical of Alzheimer’s disease are absent. Intra-neuronal argentophilic inclusions are considered pathognomonic for Pick’s disease.
Symptoms
In its development, Pick’s disease goes through 3 consecutive stages. In the initial stage, personality changes prevail with the loss of developed moral principles. A person becomes extremely selfish, antisocial behavior is observed. Disinhibition of instincts and loss of control over their actions lead to the immediate realization of instinct (physiological needs, sexual desire), regardless of the environment and the situation. The patient’s criticism is significantly reduced and he explains his behavior by saying that “I couldn’t resist”, “it happened”, “I couldn’t wait”. Against this background, bulimia, hypersexuality, and other disorders may develop. Speech changes are reduced to repeated repetitions of words, phrases, jokes, stories or secrets (a symptom of a “gramophone record”). Euphoria or apathy is noted.
The prevalence in the clinic of the initial period of dementia of the peak of certain manifestations depends on the localization of emerging atrophic processes in the brain. Thus, basal frontal atrophy is accompanied by personality disorders, emotional instability, changing periods of rigidity and disinhibition; convexital atrophy of the frontal lobe is a combination of antisocial behavior with apathy and abulia — loss of motives and desires, complete lack of will. With the predominance of atrophy in the left hemisphere, behavioral disorders manifest themselves against the background of depression. Right hemisphere atrophy is characterized by inappropriate behavior combined with euphoria.
In the second stage, cognitive impairments appear and increase. Sensorimotor aphasia occurs — the patient cannot formulate his thoughts and loses the ability to understand the speech of others. Alexia, agraphy and acalculia are observed — loss of reading, writing and counting skills. There is amnesia — memory loss, agnosia — a change in perception of the world and violations of praxis — the ability to consistently perform actions. At first, the disorder of cognitive functions can be episodic, then it becomes permanent and progresses until the complete intellectual destruction of the personality. In a number of patients, skin hyperalgesia (hypersensitivity) becomes a peculiar manifestation.
The third stage of Pick’s disease is deep dementia. Patients are immobilized due to extreme apraxia, disoriented, unable to perform basic self-care actions, and need constant care. This condition leads to the death of patients due to cerebral insufficiency or intercurrent infections (infection of bedsores with the development of sepsis, congestive pneumonia, ascending pyelonephritis, etc.).
Diagnostics
Of no small importance in the diagnosis is the interview of the patient and his relatives, the study of anamnesis, an objective examination. Pyramidal symptoms in the neurological status are not characteristic. When involved in the atrophic process of subcortex, it is possible to identify signs of damage to the extrapyramidal system (hyperkinesis, manifestations of secondary Parkinsonism). The somatic condition in the 1-2 stages of Pick dementia is usually satisfactory. During diagnosis, Pick’s disease should be differentiated from vascular dementia, Alzheimer’s disease, intracerebral tumors localized in the frontal lobes, arteriovenous malformation of the brain, schizophrenia.
Clinical data (age of onset of the disease, its manifestation with behavioral disorders followed by the addition of cognitive dysfunction, the absence of cerebral and pyramidal symptoms, etc.) allow the neurologist to assume Pick disease only in the second stage, when there are cognitive disorders. Changes in behavior that characterize the onset of the disease are often mistaken for mental disorders, and therefore the relatives of the patient primarily seek help from a psychiatrist.
During EEG in patients with Pick dementia, a decrease in the voltage of bioelectric activity in the frontal leads is revealed. REG and transcranial ultrasound do not reveal significant vascular disorders. According to the Echo-EG data, moderate hydrocephalus can be diagnosed. Imaging of atrophic changes in the brain allows tomographic methods: CT, MSCT and MRI. MRI of the brain reveals thinning of the cortex, areas of atrophic decrease in the density of brain matter in the frontal and temporal lobes, expansion of subarachnoid spaces.
Treatment and prognosis
Specific therapy has not been developed. The treatment regimen, as a rule, includes anticholinesterase pharmaceuticals (choline alfoscerate, galantamine, rivastigmine, donepezil), nootropics (piracetam, fonturacetam, gamma-aminobutyric acid), memantine. In the presence of aggressive behavior, neuroleptics are prescribed: alimemazine, chlorprotixen. In the initial stages, psychotherapy, participation in cognitive trainings, counseling of a psychologist are recommended; in later stages, a sensory room, art therapy, and presence simulation are recommended.
All of the above methods are aimed at slowing the progression of symptoms. However, their effectiveness in Pick’s dementia is significantly lower than in Alzheimer’s disease. Despite the ongoing therapy, the state of deep dementia occurs on average 5-6 years after the onset of the disease. The life expectancy from the onset of the disease does not exceed 10 years.
