Pick's disease belongs to the group of frontotemporal dementias. For years it was considered a rare disease. However, since the publication of a landmark clinical and pathology paper in 1987, Pick's disease is considered to be the third most common cause of dementia after Alzheimer's disease and Lewy body dementia. What are the symptoms? Is there a treatment?

Pick's disease(Pick′s disease, PiD) is a rare neurodegenerative disease, a type of frontotemporal dementia (FTD). Pick's disease belongs to tautopathies, a group of diseases caused by abnormalities in the microtubules of the tau protein. The nauropathological picture shows insoluble deposits of the posttranslationally modified MAP-tau protein.

Pick's disease: symptoms

A characteristic feature of the disease is the sequential appearance of symptoms.

  • Getting started

Mood disorders (depressive states, manic states) and anxiety disorders appear. For this reason, patients may be referred for a psychiatric consultation, and their clinical symptoms prove resistant to treatment. In addition, there are also characterological changes, at first subtle in the form of a more relaxed approach to social norms, loss of good manners, and unusual behavior. Withdrawal and apathy are less common. In some patients, inadequate care for their own he alth can be observed, resulting in numerous medical consultations.

  • Intermediate phase

Emotional symptoms cease to dominate, but behavioral disorders increase. Patients completely do not respect social norms, behave inappropriately, and ignore the feelings of others. Changes in their appearance are observed: neglect of personal hygiene, lack of care for clothing. Deterioration of eating habits and sleep problems. In addition, there is a gradual development of a language deficit, as well as cognitive and memory functions disorders. There are neurological symptoms: deliberative (Marinesco-Radovici symptom), primitive reflexes (e.g. sucking, urinary incontinence, less frequently stool).

  • Advanced frontotemporal dementia

Significant behavioral disorders: disinhibition, irritability, emotional lability, rendering behavior, stereotypes, inactivity, emotional indifference, loss of interests.Complex cognitive deficit: language, abstraction and planning, memory, visual-spatial and motor functions disorders. Neurological symptoms: motor stiffness, less frequently tremors (postural), primitive reflexes (grasping, sucking, scouting), urinary and stool incontinence.

Pick's disease: diagnosis

Criteria for recognition (McKann et al., 2001) - development of behavioral and cognitive deficits that are manifested as follows:

  • early and progressive personality changes characterized by difficulty in adapting behavior, which often results in inadequate reactions and actions or
  • early and progressive speech disorders characterized by difficulty expressing or having significant difficulty naming and understanding words
  • deficits cause significant problems in social functioning and are noticeable compared to the previous level of functioning
  • the course is characterized by a gradual onset and a constant deterioration of the function
  • deficits are not caused by other CNS disorders, not metabolic or induced by ingested substances

Deficits are not only present during delirium and cannot be explained by any other psychiatric diagnosis (e.g. depression, schizophrenia). Differential diagnosis includes: systemic disease, Huntington's disease, substance abuse. The diagnosis of Alzheimer's disease (AD) is often wrongly diagnosed, especially in cases with a slow onset in old age.

Pick's disease: diagnostic tests

Blood and cerebrospinal fluid tests are normal. Until the late stages of the disease, there may be no changes in the EEG test. Later, a slowing of the rhythm is observed in the front and front leads. MRI (magnetic resonance imaging) and CT (computed tomography) can reveal atrophy of the frontal and temporal cortex.

Pick's disease: treatment

There is no effective treatment improving cognitive functions in patients. Since disorders of the neurotrasmitters - serotonin and noradrenaline are observed, attempts are made to use serotonin, norepinephrine and trazodone reuptake inhibitors to modify mental disorders and behavior. Cholinesterase inhibitors are not used.

Category:

Neurology