frontotemporal dementia
Introduction
Introduction to frontotemporal dementia Frontotemporal dementia refers to the slow personality changes, speech disorders and behavioral abnormalities in the middle-aged and elderly patients. The neuroimaging shows that the frontotemporal lobe atrophy, but the pathological examination does not find the dementia syndrome of Pick and Pick cells. When there is no pathological evidence, Pick disease and frontotemporal dementia are difficult to identify. At present, it is recommended to classify Pick disease into frontotemporal dementia. Clinical studies have found that frontotemporal dementia may be the most common neurodegenerative dementia syndrome after Alzheimer's disease, accounting for about a quarter of all dementia patients. It has been suggested that frontotemporal dementia actually includes Pick disease with pathologically present Pick bodies, and other Pick syndromes with similar clinical manifestations of absence of Pick bodies, including frontal dementia (frontaldementia) and primary progressive Aphasia (primary progressiveaphasia) and so on. basic knowledge Sickness ratio: 0.0001% Susceptible people: no special people Mode of infection: non-infectious Complications: depression, urinary tract infection
Cause
The cause of frontotemporal dementia
Genetic factors (55%):
The cause may be neuronal cell idiopathic degeneration or axonal injury secondary cell changes, Wilhelmsen (1994) in a large family of frontotemporal dementia with extrapyramidal symptoms, localize the disease gene on chromosome 17. It has been confirmed that it is associated with tau gene mutation, and it has been found that about 20% of patients with frontotemporal dementia have this gene mutation.
Other diseases (45%):
The pathological features are similar to those of Pick disease. The gross pathology is mainly the frontal lobe and/or temporal lobe atrophy. About 1/3 of the patients have bilateral symmetry atrophy, the cerebral cortex is severely affected, the lateral ventricle anterior horn, and the horn is light to medium. When the degree is enlarged, the number of nerve cells in the atrophic cerebral cortex can be significantly reduced under the microscope, the neurons in the II and III layers are lost, the glial cells are diffusely proliferated, the remaining neurons are denatured to varying degrees, and there are no Pick bodies and Pick cells. It is the main pathological identification point of this disease and Pick disease.
Prevention
Frontotemporal dementia 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
Frontotemporal dementia complications Complications 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 frontotemporal dementia common symptoms cognitive dysfunction memory impairment dementia mutism personality change visual agnosia
1. The clinical manifestations of this disease are the same as those of Pick disease, more than 50 to 60 years old, onset of insidious onset, slow progress, early personality changes, speech disorders and behavioral abnormalities, such as Klüver-Bucy syndrome, if family history CT and MRI showed atrophy, temporal lobe atrophy, genetic examination revealed a variety of tau protein coding region or intron 10 related mutations, pathological examination without Pick body and Pick cells, can be diagnosed.
2. Primary progressive aphasia (PPA) is a progressive decline in language function for 2 years or more, and other cognitive functions remain normal, significantly different from AD and other frontotemporal dementia, and pathologically The amount of sputum leaves is mainly atrophy, and there is no Pick body.
Mesulam (1982) first reported 6 cases of chronic progressive aphasia without dementia, Weintraub et al (1990) named primary progressive aphasia, the main clinical features are:
(1) usually before the age of 65, slow progressive aphasia, without other cognitive dysfunction, combined with visual agnosia, spatial damage or misuse, etc., daily living ability can be kept intact.
(2) The course of disease can last for more than 10 years. Language barriers can exist for several years alone. In 6 to 7 years, they develop into severe aphasia or silence. They can still maintain their own self-care, and eventually dementia. In some cases, the initial performance is progressive aphasia. Rapid progress, short-term development into Pick disease, motor neuron disease or cortical basal ganglia degeneration.
(3) There was no positive sign in the neurological examination. MRI showed that the dominant hemisphere frontal lobe, temporal lobe and parietal lobe were obviously atrophied. SPECT showed a decrease in blood flow in the left temporal lobe and frontal lobe or bilateral frontal lobe.
Examine
Examination of frontotemporal dementia
Determination of cerebrospinal fluid, serum Apo E polymorphism, Tau protein quantification, amyloid fragment, have diagnostic or differential diagnostic significance.
1. EEG examination is mostly normal in the early stage, a few visible 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 spindles during sleep The wave is less, and the complex wave rarely occurs, and the slow wave is reduced.
2. CT and MRI examination showed characteristic localized frontal lobe and/or temporal lobe atrophy, cerebral gyrus narrowed, sulcus width and frontal angle were balloon-like enlargement, frontal and anterior epipolar cortex were thinned, and sacral angle was enlarged. The cleavage pool is widened, more asymmetrical, and a few are symmetrical. The disease can appear early. The SPECT examination shows asymmetry, the temporal lobe blood flow is reduced, PET shows asymmetry, and the temporal lobe metabolism is reduced. The two are more MRI than MRI. Sensitive and helpful for early diagnosis.
Diagnosis
Diagnosis and differential diagnosis of frontotemporal dementia
At present, there is no unified diagnostic standard for frontotemporal dementia and Pick 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 family history, genetic examination reveals tau gene mutation can be diagnosed; 1 to 5 items can be diagnosed as Pick disease, no Pick body And Pick cells are the main pathological identification points for frontotemporal dementia and Pick disease.
Differential diagnosis
It should be distinguished from Alzheimer's disease. Both of them are insidious onset, and the progress is slow. There are many common points in clinical practice. The most distinguishing is the chronological sequence of progressive dementia symptoms in the course of the disease. Early AD disappears and visual orientation Cognitive impairments such as impaired power and computational power, social ability and personal etiquette are relatively reserved; early manifestations of frontotemporal dementia are personality changes, speech disorders and behavioral disorders, spatial orientation and memory preservation are good, and intelligent decline and forgetting occur in the late stage. Etc., Klüver-Bucy syndrome is a manifestation of early behavioral changes in frontotemporal dementia. AD is only seen in the late stage. CT and MRI are helpful in the identification of both. AD can show extensive brain atrophy. Frontotemporal dementia shows the amount and/or sputum. Leaf atrophy; clinical diagnosis requires histopathological examination.
