2017년 2월 16일 목요일

Pick's disease

Pick's disease

Pick's disease
Pick's disease.png
Brain MRI in Pick's disease
A classification and outside reference information
Clinical department ・
Academic field
Neurology
ICD-10 G31.0, F02.0
ICD-9-CM 331.11
OMIM 172700
DiseasesDB 10034
MedlinePlus 000744
eMedicine neuro/311
MeSH D020774

It is maegashira both sides of the head model dementia (FTD), and a special personality change, action are abnormal, and Pick's disease (pick thumbtack, Pick's disease, PiD) is a presenile neurodegenerative disease indicating the language function obstacle. By the way, I may show the motor neurone disease symptom.

Table of contents

History

In 1892 Arnold Pick of the Czech Prague Karel University "was aging-related brain atrophy and an autopsy example titled connection with the aphasia, and reported it as a mental disease with the significant atrophy of the frontal lobe and the temporal lobe." The pick did a similar report by 1906, and a series of diseases came to be known as localized brain kraurosis of Pick. On the pathology form side, Aloys Alzheimer of the Munich University, Germany reported "嗜銀性神経細胞内封入体" (Pick corpuscle) and "a swelling cell" (Pick cell) in 1911. 大成潔 of the former Manchurian medical college and Hugo Spatz of the Munich University, Germany settled a characteristic in 1926 and named it "Pick disease". A term maegashira temporal lobe degeneration (FTLD) was proposed in 1996 by a group (Lund and Manchester Groups) of the Swedish ルンド University and Manchester University, the U.K.

Pathology

The brain dissection pathological diagnosis of Pick's disease with the pick ball it means. A definition of the pick ball is to be dyed with 3 antirepeat tau antibodies. As for maegashira both sides of the head model dementia (FrontoTemporal Dementia, FTD) that the pick globe was not accompanied by later, an ubiquitin-positive tau-negative inclusion body was recognized, and it was in FTLD-U. Pick's disease was defined as one of タウオパチー again as a result that FTLD-U and FTLD-TDP which became clear afterwards were separated. The Pick cell is said to be ballooned neuron.

Symptom

A personality change and behavior disorder are outstanding.

Lack of the consciousness of disease
We are running out of consciousness of disease from the early period of illness. Do not notice a change of oneself at all; for own obstacle is unconcerned.
The company movement that it is the way it goes
The company movement that it is the way it goes is accepted frequently from the early period of illness. A stereotyped tour to walk around the same course is often outstanding in the everyday life. I can come home without orientation and the visual space recognition having possibilities to be kept, and losing the way unless it becomes in an advanced stage.
Disinhibition, antisocial behavior
I do not mind how it is thought by courtesy and conventional wisdom, other people at all, and an action to go our way proceeding to of the instinct becomes characteristic. I perform shoplifting without a harm and may cause the neighborhood and a trouble [1]. I do it blankly without minding it for attention and the instruction really. I may run about for an impulsive action as a result of disinhibition. When an initiative drop advances, I do not often attract attention.
It is hard to maintain rolling-related sthenia of the attention
Mind deviates immediately and I continue one act and cannot continue it. An attention disorder, association with motor impersistence are thought about. Not only I react for stimulation of the outside world excessively, but also do not necessarily calm down without stimulation of the outside world. Leave the room suddenly without any notice; leave, and the action is often observed, too.
Sthenia of cover influence characteristics
It is processing, a reacting symptom reflectively without considering for external stimulation. I appear for the act to read an imitation act of another person and the letter which it gets into eyes aloud.
Thought laziness
I answer it without thinking for a question carefully, and I think about the symptom ignoring, and it is said with laziness. When there is thought laziness, it may be misunderstood "I do not know it" with an obstacle of the memory to answer immediately saying "I forgot it". As for the examination of nerve psychology, a result does not reflect the actual situation either.
Indifference, initiative drop
I become indifferent for both the self and the neighborhood and do not take a bath voluntarily, and the symptom becoming unconcerned about an appearance is relatively recognized from the early period of illness. When time comes when I think whether you spent it idly without doing anything, I go to the walk every day and coexist with the company movement that it is the way it goes in the illness early days. When an initiative drop goes, it finally becomes the akinesia silence state.
Dietary behavior abnormality
I may show the company movement that it is the way it goes to continue eating specific food, and to continue making the dish of the same menu. It is in an advanced stage, and the lips tendency that is going to carry all the things to pick up to the entrance appears.

Laboratory findings

Examination for image

Head MRI
The atrophy of the maegashira temporal lobe is strong and is described with lobation or the wavy shape (knife-blade) of the knife. In addition, there is often laterality in atrophy in acknowledgment of expansion of the lateral ventricle anterior horn with caudate atrophy. The atrophy of occipital lobe and the parietal lobe is not outstanding. I can confirm frontal convex, orbital surface, atrophy of the temporal pole with the coronal section. The disinhibition appears in frontal lobe orbital surface, the indifference for a maegashira Maeno lateral surface disorder. The sea lion is kept relatively good, but there is the atrophy of the amygdaloid body and enlarges the lateral ventricle inferior horn.
Function image
I accept an agreed significant bloodstream drop by the brain blood flow SPECT in the atrophy part, and an example and the striatal bloodstream having laterality decrease, too.

Examination of menstruation

Brain waves
I often show normalcy in comparison with cognitive functional disorder until last years. The mixture of the slow wave is accepted only after I become bedridden.

Examination for neuropsychology

The drop of the frontal lobe function is accepted.

FAB (frontal assessment battery)
FBI (frontal behavioral inventory)
NPI (neuropsychiatric inventory)
It is said to differentiation with Alzheimer's dementia effectively. I evaluate NPI in the information from a caregiver.

Pathology

Pick ball
The pick ball is spherical, and or an oval is the inclusion body in the nerve cells cell of the pseudomorph a little. I show 嗜銀性 by ボジアン dyeing. It appears most commonly on a granule cell of cerebrocortical second, V, the VI layer followed by the III layer or the sea lion dentate gyrus, a sea lion cone cell. I appear to a claustrum, an almond nucleus, a caudate nucleus, a putamen, Meynert basal nucleus, a locus coeruleus nucleus with basal nuclei, brainstem well. Phosphorylation tau, a phosphorylation neurofilament are positive for the immunohistology. 3 repeat tau accumulates biochemically. It is easy to be found in an atrophy part and may be accompanied by ballooned neuron (I called it Pick cell once).
Cerebrocortical nerve cells falling off
The moderate above-mentioned denaturation is often accepted with the cerebral neocortex by cortex, inferior temporal gyrus, middle temporal gyrus of 前頭葉弯隆面 and the orbital surface.
Basal nucleus, nerve cells falling off of the brainstem
An almond nucleus is affected most highly frequently. As for the caudate nucleus and the putamen, a moderate obstacle is accepted.

Footnote

References

Allied item

Outside link

This article is taken from the Japanese Wikipedia Pick's disease

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