The frontotemporal dementia (FTD) is used for sporadic and hereditary diseases before that affect the frontal and temporal lobes, incl. M. Pick.
Dementia is a chronic, global, usually irreversible deterioration of cognition. The frontotemporal dementia accounts for up to 10% of the dementias. The age of onset is typically younger (55-65 years) than in Alzheimer’s disease. The FTD affects men and women equally.
The frontotemporal dementia (FTD) is used for sporadic and hereditary diseases before that affect the frontal and temporal lobes, incl. M. Pick. Dementia is a chronic, global, usually irreversible deterioration of cognition. The frontotemporal dementia accounts for up to 10% of the dementias. The age of onset is typically younger (55-65 years) than in Alzheimer’s disease. The FTD affects men and women equally. M. Pick is a term used to describe the pathological changes FTD, including severe atrophy, loss of neurons, gliosis, and the presence of abnormal neurons (Pick cells) containing inclusions (Pick bodies). About half of FTD are inherited; most mutations affect the chromosome 17q21-22 and lead to abnormalities of the microtubular binding of the tau protein; therefore, the FTD are also known as tauopathies. Some experts classify the supranuclear palsy and corticobasal degeneration together with the FTD, because all have a similar pathology and gene mutations that affect the tau protein. Symptoms, gene mutations and pathological changes can not match. The same mutation can eg. As cause for a family member symptoms of FTD, in another one, symptoms of corticobasal degeneration. Dementia should not be confused with delirium although the perception is impaired in both. The following helps to distinguish these: Dementia mainly affects memory, is usually caused by anatomical changes in the brain shows a slow start and is usually irreversible. A Dellir mainly concerns the attention is usually caused by an acute illness or drug or drug toxicity (sometimes fatal) and is often reversible. Other characteristics contribute to distinguish dementia and delirium (see Table: Differences Between Delirium and Dementia *). Symptoms and complaints generally relates to a frontotemporal dementia (FTD) more personality, behavior, and usually also language functions (syntax and liquid) and less memory, as is the case in Alzheimer’s dementia. Abstract thinking and attention (maintenance and replacement) are disturbed; the reactions are disorganized. The orientation is obtained, but the information retrieval may be affected. The motor skills are generally retained. Patients have difficulty with the sequencing of activities, although the visuospatial and constructive tasks are less affected. Frontal disinhibition characters (gripping, suction, Schnauz-, Palmomentalreflex and glabellar character How to assess reflexes) appear late in the course of the disease, but are also used in other dementias ago. Some patients develop motor neuron disease with generalized muscle atrophy, weakness, fasciculations, Bulbärsymptomen (z. B. dysphagia, dysphonia, difficulty chewing) and an increased risk of aspiration pneumonia and early death. Behavoriale (frontal) variant FTD The social behavior and personality change because the orbitobasale frontal lobe is affected. Patients are impulsive and lose their social inhibitions (z. B. they commit shoplifting, they neglect their personal hygiene). Some have a jib-Bucy syndrome, emotional numbing, hyper sexuality, Hyperoralität (z. B. bulimia, sucking and smacking of the lips) and visual agnosia includes. Instability (disabled concentration), inertia and mental rigidity may occur. The behavior is repetitive and stereotyped (z. B. the patients can go every day to the same place). Patients can indiscriminately pick up objects without reason and use (so-called. Utilisationsverhalten). The verbal statements are reduced; there are echolalia, perseveration (inappropriate repetition of a response) and finally mutism vor.Primär progressive aphasia The language functions deteriorate because of an asymmetric (more on the left) anterolateral atrophy of the temporal lobe. The hippocampus and memory are less affected. Patients have word finding difficulties. The attention (eg. As Zählspanne) may be severely impaired. Many patients have aphasia with reduced flow of language and difficulties in comprehension; a hesitation in speech production and dysarthria are also common. In some patients, aphasia is about ? 10 years the only symptom; in other global deficits develop within a few years. Semantic dementia is a form of primary progressive aphasia. If the left side of the brain is more affected, the ability to understand words is being lost. The language is fluid, but it loses the meaning; a general or similar designation is used instead of the specific name for an object. If the right side is most affected, patients have a progressive anomie (inability to name objects) and prosopagnosia (inability to recognition of familiar faces). You can not remember topographical relationships. Some patients with semantic dementia have Alzheimer’s disease. Diagnosis generally similar to the diagnosis of other dementias Additional clinical evaluation for the differentiation of some other dementias A general diagnosis of dementia requires each of the following: cognitive or behavioral (neuropsychiatric) symptoms that have the ability to function at work and perform usual activities of daily living, affect. These symptoms represent a decline of previous functional levels. These symptoms can be explained not by delirium or major mental disorder. A diagnosis of FTD by typical clinical findings (eg. as social disinhibition or impaired speech function with lower memory function). As with other forms of dementia, the cognitive deficits are evaluated. For clarification involves taking a history from the patient and from a person who knows the patient, plus carrying out an investigation in mental status, at the bedside or if the investigation at the bedside is not revealing a formal neuropsychological testing (Dementia: Assessment of cognitive Function) . To determine the localization and the degree of brain atrophy, and for the exclusion of other possible causes (for. Example, brain tumors, abscesses, stroke) are carried out a CT and a MRI. FTD are characterized by marked atrophy with paper thin gyri in the temporal and frontal lobes. However, MRI or CT can show these changes late in the course of FTD. Thus, FTD and Alzheimer’s disease are generally easier to differentiate on the basis of clinical criteria. For example, the primary progressive aphasia by Alzheimer’s disease differs in that memory and visuospatial function are preserved, but syntax and fluency are disabled. A PET with fluorine-18 (18F) -labeled deoxyglucose (FDG or fluorodeoxyglucose) can help to distinguish Alzheimer’s dementia of FTD, by presenting the localization of hypometabolic areas. In Alzheimer’s disease, these areas are located in the posterior temporoparietal association cortex and the posterior cingulate cortex; at FTD they are in the front areas-in the frontal lobes, the anterior temporal cortex and the anterior cingulate. FTD forecast deteriorate mostly gradual, but the rate of progression may vary. If symptoms on speaking and language are limited, the progression to a general dementia may be slower. Treatment Supportive tests There is no specific treatment. Treatment is usually supportive. For example, the environment should be bright, friendly and familiar, and it should be designed so that an orientation is reinforced (eg. As placement of large clocks and calendars in the room). Measures to ensure the safety of the patient (eg., Signal monitoring systems for patients who walk) should be initiated. Symptoms are treated as appropriate.