Pick disease and frontotemporal dementia
Introduction
Introduction to Pick disease and frontotemporal dementia Pick disease is a rare and slowly progressing cognitive and behavioral disorder, clinical manifestations of behavioral abnormalities, aphasia and cognitive disorders. Many patients in clinical clinics cannot be identified with Pick disease, and no typical Pick body is found in pathological examination. Dementia syndrome, characterized by atrophy of frontal lobe, is called frontotemporal dementia, which accounts for about a quarter of all dementia patients. The etiology and pathogenesis of Pick disease and frontotemporal dementia are still unclear. Most believe that this disease is an idiopathic degenerative disease that invades the neuronal cell body, and it is also considered to be a secondary cell body change after axonal injury. There is currently no effective treatment, acetylcholinesterase inhibitors are usually ineffective, and small amounts of diazepam, selective serotonin reuptake inhibitors or propranolol are used cautiously for those with behavioral disorders such as aggressive behavior, irritability and aggressiveness. medical treatement. Conditional caregivers can be given appropriate life and behavioral guidance and symptomatic treatment by trained caregivers. basic knowledge The proportion of illness: 0.002% Susceptible population: the age of onset is 30-90 years old, 60 years old is the peak, more than 70 years old, more women than men Mode of infection: non-infectious Complications: multiple lung infections acne
Cause
Pick disease and the cause of frontotemporal dementia
The etiology and pathogenesis of Pick disease and frontotemporal dementia are still unclear. Most believe that this disease is an idiopathic degenerative disease that invades the neuronal cell body, and it is also considered to be a secondary cell body change after axonal injury.
Prevention
Pick disease and frontotemporal dementia prevention
There is no effective prevention method, and symptomatic treatment is an important part of clinical medical care. Early diagnosis and early treatment may slow the irreversible progression of dementia. Should eat celery, day lily, leeks, melon, ebony, dried persimmon, sesame, lotus seeds, sea cucumber. Diet regulation: It is necessary to prevent high-fat foods from causing elevated cholesterol, as well as essential nutrients such as protein, inorganic salts, amino acids and multivitamins, especially vitamins B1, B2 and B6, vitamin C and vitamin E.
Complication
Pick disease and frontotemporal dementia complications Complications multiple lung infections acne
More deaths from complications such as lung infections, urinary tract infections and hemorrhoids.
Symptom
Pick disease and frontotemporal dementia symptoms Common symptoms Dementia daily living ability reduction... Cognitive dysfunction, forgetful aphasia, anger
The age of onset is 30-90 years old, 60 years old is the peak, more than 70 years old, more women than men, about half of the patients have a family history, insidious onset, slow progress, clinically obvious personality, behavioral change and cognition Obstacles are characteristic. Early manifestations of personality and emotional changes, such as irritability, anger, stock index, emotional apathy and emotional depression, gradual behavioral behavior abnormalities, misbehavior, no aggressiveness, indifference to things and impulsive behavior, some patients with behavioral abnormalities appear Kluver-Bucy syndrome, dullness, electromechanism, obedience, visual dissonance and rapid thinking change, excessive mouth activity, hunger, excessive diet, obesity, putting everything in hand into the mouth to test, can be accompanied There are forgetfulness, aphasia and convulsions.
Cognitive impairment occurs with the progression of the disease, but it is very atypical compared with the cognitive impairment of Alzheimer's disease. In particular, spatial orientation preservation is better, memory impairment is lighter, and behavior, judgment, and speech ability are significantly impaired. Signs of the nervous system such as absorption and reflex, strong grip reflex can occur early, and the late stage has myoclonic, pyramidal and extrapyramidal signs, such as Parkinson's disease syndrome.
Primary progressive aphasia refers to a progressive decline in language function for 2 years or more, and other cognitive functions remain normal, which is the difference between Alzheimer's disease and frontotemporal dementia.
Early EEG was more normal in Pick disease and frontotemporal dementia, and late background activity was low. CT and MRI showed characteristic localized frontal lobe and anterior temporal lobe atrophy, narrow brain stenosis, sulcus width and frontal angle into balloon-like enlargement.
Examine
Examination of Pick disease and frontotemporal dementia
Laboratory inspection
Determination of cerebrospinal fluid, serum ApoE polymorphism Tau protein quantitation, amyloid beta fragment, have diagnostic or differential diagnostic significance.
Other auxiliary inspection
1. Most of the early EEG examinations are normal. The visible wave amplitude reduces the wave reduction; the late background activity has low wave or no irregular wave amplitude wave. In a few patients, the spindle wave rarely appears when there is sharp wave sleep. integrated wave, slow wave reduction.
2, CT and MRI examination showed characteristic localized frontal lobe and / or temporal lobe atrophy, cerebral gyrus, sulcus wide and frontal angle balloon-like enlarged frontal pole and anterior tibial cortex thinning angle expansion of lateral sulcus pool A wide, multi-asymmetric minority can be symmetrical, and the disease can appear early. SPECT examination showed asymmetry, decreased temporal lobe blood flow, PET showed asymmetry, and the reduction of temporal lobe metabolism was more sensitive than MRI for early diagnosis.
Diagnosis
Diagnosis of Pick disease and frontotemporal dementia
diagnosis
Diagnosis can be based on medical history, clinical symptoms, and laboratory tests.
Differential diagnosis
It should be distinguished from Alzheimer's disease, both of which are insidious, slow progress, and have many in common in clinical practice. The most discriminating is the chronological sequence of progressive dementia symptoms in the course of the disease. Early cognitive impairment such as amnesia, spatial orientation and impaired computational power, relative retention of social ability and personal etiquette; early manifestation of frontotemporal dementia For personality changes, speech disorders and behavioral disorders, spatial orientation and memory are well preserved, and intelligent decline and forgetting occur in the late stage. Klüver-Bucy syndrome is a manifestation of early behavioral changes in frontotemporal dementia, and AD is only seen in advanced stages. CT and MRI are helpful for the differentiation of the two. AD can show extensive brain atrophy. Frontotemporal dementia shows atrophy of the frontal and/or temporal lobe; clinical diagnosis requires histopathological examination.
