Pick disease
Introduction
Introduction to Pick disease Pick's disease is a rare, slowly progressive cognitive and behavioral disorder. Pick (1892) first described a group of patients with frontotemporal atrophy as a pathological feature, characterized by behavioral abnormalities, aphasia and recognition. Knowing the obstacles, he reported the first 71-year-old man with progressive mental decline and extremely severe aphasia. Autopsy found that the left epipolar cortical atrophy was extremely serious; the four cases reported later highlighted the patient's progressive language disorder. basic knowledge The proportion of illness: 0.005% Susceptible population: mostly middle-aged and elderly people, the age of onset is 30-90 years old, the peak is 60 years old, more than 70 years old, more women, 40% of patients have a family history Mode of infection: non-infectious Complications: Dementia Depression Urinary tract infection
Cause
The cause of Pick's disease
(1) Causes of the disease
The etiology of Pick's disease is still unclear. In recent years, studies have shown that up to 40% of patients with Pico disease have a family history, mostly autosomal dominant inheritance, suggesting that this disease is closely related to genetic factors.
(two) pathogenesis
The pathogenesis is not yet clear. Some literature reports suggest that it is mainly the idiopathic degeneration of the neuronal cell body. Some scholars believe that it is the secondary cell body change after axonal injury.
Gross pathological observation of brain weight reduction, female average 1050g, male 1075g, brain atrophy amount, temporal lobe (54%) or invasive frontal lobe (25%) or temporal lobe (21%), about 1/3 of patients double Side symmetry is involved, about 1/2 of the left hemisphere is severely affected, 1/5 is mainly affected by the right hemisphere, and 2/3 of the iliac crest is often unaffected by one of the characteristics of Pick's disease. The amygdala is more affected than the hippocampus. The nucleus and basal nucleus can be involved, gray matter and white matter can be involved, the anterior horn of the lateral ventricle, the horn angle is slightly moderately enlarged, and the number of neurons in the atrophic cerebral cortex is significantly reduced under light microscope, and the II and III layers are significant. V, VI layer is lighter; some patients can see the loss of cervical and thoracic motor neurons, diffuse hyperplasia of cortical and subcortical white matter astrocytes, with spongiform changes, residual neuronal degeneration and atrophy, partial nerve The cytoplasm contains a well-defined silver-plated Pick body, and some of the neurons are expanded and denatured. The Pick body is clearly defined by electron microscopy. It is round or oval, without a capsule, and is 5-15 m in diameter. Silver-containing inclusion body, consisting of 10nm filament, ribosome, vesicle, lipopoly Short segment and 24nm twisted straight and microtubules and neurofilaments, Pick bodies forming mechanism is unclear, Golgi impregnation method cortical pyramidal cell dendritic spines seen almost completely disappeared.
Prevention
Pico disease prevention
There is no effective prevention method. Symptomatic treatment is an important part of clinical medical care. Early diagnosis and early treatment may slow the irreversible process of dementia.
Complication
Pico disease complications Complications Dementia depression urinary tract infection
With the development of the disease, in addition to the obvious dementia (cognitive disorder), the common combination of patients with aphasia, depression, severe mental behavior abnormalities, etc., in addition, should pay attention to secondary lung infections, urinary tract infections.
Symptom
Symptoms of Pico's disease Common symptoms Dementia Depression and daily living ability are reduced... Memory disorder, anger, irritability, mutism, personality, change, anxiety, strong grip, reflex
1. Picking disease is insidious, the course progresses slowly, mostly in the middle-aged and old age. The age of onset is 30-90 years old, the peak is 60 years old, more than 70 years old, more women, 40% of patients have family history The rest is distributed.
2. The clinical course of Pick's disease can be divided into three stages. The early stage is characterized by obvious personality changes, emotional changes and behavioral abnormalities. It is characterized by irritability, anger, stubbornness, apathy and depression, and abnormal behavior. Personality change, inappropriate behavior, lack of initiative, indifference to things and impulsive behavior; some patients have Klüver-Bucy syndrome, manifested as dullness, apathy, obedience, visual agnosia, hyperorality, thinking change Hypermetamorphosis, good hunger and excessive diet, put everything you find, such as waste paper, garbage and feces, into the mouth to test and eat, and have emotional depression, anxiety, physical abnormalities and fragmentary delusions.
3. With the progress of the disease, cognitive impairment can occur, gradually unable to think, attention and memory loss, compared with Alzheimer's disease, cognitive impairment is not typical, spatial orientation preservation is better, memory impairment is lighter, speech disability disorder is more obvious, gradually Reduced speech, poor vocabulary, stereotyped language, imitative language and aphasia, late stage of mutism.
4. Neurological signs such as sucking reflexes, strong grip reflexes can occur early in the course of the disease, late stage myoclonus, pyramidal tract and extrapyramidal damage such as Parkinson's disease syndrome.
Examine
Pick disease check
Determination of cerebrospinal fluid, serum Apo E polymorphism, Tau protein quantification, amyloid fragment, have diagnostic or differential diagnostic significance.
EEG examination
Most of the early period is normal, a few visible wave amplitudes are reduced, waves are reduced; late background activity is low, waves are rare or absent, irregular wave amplitude waves may be present, a few patients have sharp waves, and spindle waves are less during sleep, rarely appearing integrated wave, slow wave reduction.
2. CT and MRI examination
It can be seen that the characteristic frontal lobe and/or temporal lobe atrophy, the brain is narrow, the sulcal width and frontal angle are balloon-like enlargement, the frontal and anterior epipolar cortex are thinner, the temporal angle is enlarged, and the lateral fissure pool is widened. Asymmetry, a few can be symmetrical, the disease can appear early, SPECT examination is asymmetrical, the temporal lobe blood flow is reduced, PET shows asymmetry, the temporal lobe metabolism is reduced, the two are more sensitive than MRI, help early diagnosis.
Diagnosis
Diagnosis and identification of Pick disease
diagnosis
At present, there is no unified diagnostic standard for frontotemporal dementia and Pico disease. The following standards can be used as reference:
1. Middle-aged and elderly people (usually 50 to 60 years old) slowly appear personality changes early, emotional changes and misbehavior, and gradually appear abnormal behavior, such as Klüver-Bucy syndrome.
2. Speech disorder appeared early, such as reduced speech, poor vocabulary, stereotyped language and imitative language, followed by obvious aphasia, early computational power preservation, memory impairment, and relative spatial orientation.
3. Late stage of intelligent decline, forgetting, urinary incontinence and mutism.
4. CT and MRI showed asymmetry atrophy of the frontal and/or temporal lobe.
5. Pathological examination revealed Pick bodies and Pick cells.
Having 1 to 4 items, excluding other dementia diseases, clinical diagnosis can be diagnosed as frontotemporal dementia. If there is a family history, genetic examination reveals that tau gene mutation can be diagnosed; 1 to 5 items can be diagnosed as Pico disease.
Differential diagnosis
The disease of Pick should be distinguished from Alzheimer's disease. Both of them are insidious, and the progress is slow. There are many common points in clinical practice. The most distinguishing is the time sequence of symptoms of progressive dementia in the course of the disease. Cognitive impairments such as spatial orientation and impaired computational power, social ability and personal etiquette are relatively reserved; early manifestations of Pico disease are personality changes, speech disorders and behavioral disorders, spatial orientation and memory preservation are better, and intelligence occurs in the late stage. Recession and forgetting, Klüver-Bucy syndrome is a manifestation of early behavioral changes in Pick disease. AD is only seen in the late stage. CT and MRI are helpful in the identification of both. AD shows extensive brain atrophy, and Pico disease shows the sum of Or) axillary atrophy; clinical diagnosis requires histopathological examination, identification of Alzheimer's disease and Pick's disease.
