Pick disease

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Classification according to ICD-10
G31.0 Localized Brain Atrophy
- Pick's Disease
F02.0 * Pick disease dementia
ICD-10 online (WHO version 2019)

The Pick's disease or frontotemporal dementia (FTD) is a neurodegenerative disease which the frontal and temporal lobe destroys the brain. It usually occurs before the age of 60. With this type of dementia, a progressive change in personality and social behavior is in the foreground; impaired memory performance rather less.

Names previously used for the disease are also Pick's disease or Pick's disease, which are increasingly being replaced by the newer term frontotemporal dementia .

As dementia, it is less common than Alzheimer's disease or senile dementia of the Alzheimer's type ( SDAT or DVAT ). These diseases are also included in the group of neurodegenerative diseases. A familial occurrence is observed more often in Pick's disease (in about forty percent of the illnesses, information on familial frequency fluctuates). An autosomal dominant inheritance can be found in less than ten percent of cases.

The FTD must be distinguished from the so-called " frontal brain syndrome ", which is done in the corresponding article. This term describes the consequences of damage or disorder located in the frontal lobe. However, this is not a separate clinical picture, but only a description of an unspecific symptom pattern .

Historical

Pick's disease was described for the first time in 1892 by the Prague neurologist Arnold Pick , who found atrophy (tissue loss) of the frontal and temporal brain lobes during the autopsy of a so-called “feeble-minded person” who died early and classified this as a special disease. Further cases were described in the 1920s, and therefore the picture was named Pick's disease or Pick's disease after the first person to describe it.

Emergence

Exactly what factors cause the occurrence of the disease is still largely unexplored. As one of the triggers for the could tau protein coding MAPT - gene are identified. Another causative mutation in the PSEN1 gene coding for presenilin -1 has also been proven. The pathological accumulation of a protein called TDP-43 in the nerve cells is proven in around half of the cases. These cases occur in families. In addition, mutations were found in the TMEM106B gene, which favor the disease as a strong risk factor (chromosome 7p21). An association with the accumulation of TDP-43 is suspected, but has not yet been proven. The only reliable morphological feature that can be shown after the patient's death is characteristic inclusion bodies (in this case called “Pick bodies”), which were described by Alois Alzheimer in frontotemporal dementia in 1911 ( tauopathy ).

frequency

The prevalence is assumed to be 3.4 / 100,000. Pick's disease affects women and men equally often. Pick's disease is a rare type of dementia with a frequency of 3–9% (depending on the source). Assuming there are around 1.4 million dementia patients in Germany, it is estimated that at least 42,000 people would be affected by Pick's disease.

Symptoms

Pick's disease patients generally suffer from behavioral problems, personality changes, speech and memory disorders and the loss of learned rules of conduct. The course of the disease is rather slow. Usually the first two symptoms are preceded by a memory disorder.

Clinically, two symptom complexes can be observed - either "passive" appearing signs such as:

or opposing symptoms such as:

In the further course of the disease, language and orientation disorders are added until the full picture of frontotemporal dementia with muscle stiffness ( rigor ), need for care and urinary or fecal incontinence has developed.

Depending on the affected brain region, the occurrence of sub-forms of frontotemporal dementia, such as is progressive (progressive) aphasia or semantic dementia possible.

course

The disease usually begins between the ages of 50 and 60, although the range is very large (20–85 years). The mean survival time is 8 years. It should be borne in mind, however, that it is often difficult to determine the appearance of the first symptoms in retrospect and that all estimates of the duration of the disease course are likely to be inaccurate.

diagnosis

Brain PET showing decreased glucose metabolism in the left temporal lobe
Associated MRI with corresponding atrophy

In addition to clinical suspicion patterns may in vivo a nuclear medicine diagnosis of brain perfusion (with showing 99m Tc -HMPAO) ( SPECT ) or the glucose metabolism of the brain with 18 FDG ( PET help) - typically are in the brain sections (frontotemporal) the disease name correspond, the blood flow and the glucose metabolism decreased.

A reliable diagnosis of this rare form of dementia is only possible after death . However, the disease can be suspected on the basis of the symptom constellation and narrowed down by clarifying possible exclusion diagnoses.

Under the microscope, degenerative changes ("nerve cell loss ") are found in the area of ​​the temporal lobe ( temporal lobe ) and the frontal lobe , which were caused by ganglion cell loss and gliosis below the cerebral cortex. The role of the so-called “Pick body” (spherical accumulations of the τ protein) in the nerve cells of the frontal or temporal brain is unclear. In any case, no linear relationship can be established between the number of Pick's bodies and the occurrence of dementia; Pick's bodies were also found in patients not suffering from Pick's disease.

Classification according to course form

Clinically, this dementia can often be divided into 3 distinct types, which are particularly distinguishable in the early stages. They merge later in the course:

  • Frontal / frontotemporal waveform with leading personality change (main type)
  • Primary progressive aphasia ( i.e. a leading non- liquid aphasia (speech disorder) and possibly left temporal atrophy)
  • Semantic dementia (leading bitemporal atrophy, deficit of knowledge about word and object meanings, incorrect syntactic language processing, deficit of “world knowledge” on general facts, visual-gnostic disorders).

forecast

The average duration of illness until death is given as eight years. However, there are big differences between the forms, from very rapid to very slow.

therapy

Neither a cure nor a specific drug therapy are currently possible (status: 7/2013) . The acetylcholinesterase inhibitors, which were temporarily effective against Alzheimer's disease, proved to be ineffective in the treatment of frontotemporal dementias. Psychotic side effects of Pick's disease can, however, be alleviated by the administration of neuroleptics (such as pipamperon and levomepromazine ).

Continuous hospitalization of Pick patients with intensive care is necessary as the progress progresses. In view of the serious symptoms, psychological / psychotherapeutic support for relatives is essential.

Complementary

The Munich psychiatrist Hans Förstl voiced the suspicion that King Ludwig II of Bavaria , in addition to a schizotypic disorder, also suffered from Pick's disease in the last years of his life; he derived this from the autopsy findings of 1886, which had found a significant shrinkage of the frontal lobe in Ludwig. With the French composer Maurice Ravel (1875–1937) it is also possible, due to the traditional symptoms, that he suffered from Pick's disease, but some symptoms were clearly not present in Ravel's case (neglect of personal hygiene: Ravel paid careful attention to his appearance until the end; or disinhibition in social behavior).

literature

  • A. Brun et al .: Clinical and neuropathological criteria for frontotemporal dementia. In: J Neurol Neurosurg Psychiatry. 57 (1994), pp. 416-418.
  • J. Diehl: Frontotemporal dementia. In: Geriatrics Journal. The elderly patient in the practice and clinic. 4 (2002), S, pp. 36-37.
  • J. Diehl, A. Kurz: Frontotemporal dementia. In: Wiener Medical Wochenschrift. 152 (2002), pp. 92-97.
  • J. Diehl, IR Mackenzie, H. Förstl, A. Kurz: Frontotemporal dementia: results of the "Frontotemporal Dementia & Pick's Disease Conference". In: Neurologist. 74 (2003), pp. 785-788.

Web links

Individual evidence

  1. ↑ J. Greck, N. Lautenschlager, A. Kurz: Clinical aspects of frontotemporal dementia. In: Fortschr. Neurol. Psychiatr. 2000 Oct; 68 (10), pp. 447-457.
  2. ↑ Pick A. On the Relationship of Senile Brain Atrophy to Aphasia. Prague Med Wchschr 1892; 17: 165-167
  3. ^ Frontotemporal dementia.  In: Online Mendelian Inheritance in Man . (English).
  4. ↑ Pick's disease.  In: Online Mendelian Inheritance in Man . (English).
  5. ↑ VM Van Deerlin, PM Sleiman, M. Martinez-Lage, A. Chen-Plotkin, LS Wang et al .: Common variants at 7p21 are associated with frontotemporal lobar degeneration with TDP-43 inclusions. In: Nat Genet. 2010 Feb 14. PMID 20154673
  6. ↑ Society and Dementia ( Memento of the original from November 29, 2014 in the Internet Archive ) Info: The archive link was automatically inserted and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. accessed on March 22, 2014 at www.wegweiser-demenz.de @1@ 2Template: Webachiv / IABot / www.wegweiser-demenz.de
  7. ↑ a b deutsche-alzheimer.de (PDF; 90 kB) German Alzheimer's Society, Die Frontotemporale Demenz, information sheet 2013.
  8. ↑ J. Diehl, IR Mackenzie, H. Förstl, A. Kurz: The frontotemporal dementia: Results of the "Frontotemporal Dementia & Pick's Disease Conference". In: Neurologist. 2003, 74, pp. 785-788.
  9. ↑ JL Cummings. The Neuropsychiatry of AD and Related Dementias. Taylor & Francis, London 2003.
  10. ↑ Information sheet from the German Alzheimer's Society at deutsche-alzheimer.de (pdf) , accessed on July 25, 2017
  11. ↑ Tanja Schmidhofer: Ludwig II of Bavaria - not schizophrenic, but ... Klinikum rechts der Isar of the Technical University of Munich, press release of October 11, 2007 from the Science Information Service (idw-online.de), accessed on January 27, 2014.
  12. ^ Deutscher Ärzteverlag GmbH, editor of the Deutsches Ärzteblatt: Maurice Ravel (1875–1937): Between hope and discouragement. August 10, 2007, accessed January 17, 2019 .