Zusammenfassung
Obwohl die frontotemporalen Demenzen (FTD) nur einen Anteil von 5–7% aller Demenzformen ausmachen, sind sie aufgrund des präsenilen Beginnes eine der häufigsten Demenzformen in der Altersgruppe der 45- bis 65-Jährigen. Neben den selteren genetischen Varianten werden die FTD in die klinischen Entitäten der Verhaltensvariante einer FTD, die primär progredienten Aphasien und eine FTD-Variante, welche mit den Symptomen einer Motorneuronerkrankung (FTD-ALS) einhergeht, untergliedert. Die pathobiochemische Klassifikation hat sich in den letzten Jahren durch die Charakterisierung des pathologisch vermehrten TAR-DNA-bindendes Protein 43 als gemeinsames Merkmal vieler FTD-Varianten gewandelt. Neue klinische Diagnosekriterien für die Verhaltensvariante der FTD fordern die Anwesenheit von mindestens 3 von 6 klinischen Merkmalen (Disinhibition, Apathie, gestöre Empathie, perseverative/stereotype Verhaltensmuster, Veränderungen des Essverhaltens und typische neuropsychologische Veränderungen). Die primär progressiven Aphasien werden nach klinischen und radiologischen Merkmalen in eine nichtflüssige Variante, eine logopenische Variante und in eine semantische primär progressive Aphasie gegliedert, wobei die logopenische Variante histopathologisch vermutlich eine Form der Alzheimer-Demenz darstellt. Gelegendlich werden die FTD auch von Bewegungsstörungen wie einem Parkinsonismus begleitet. Klinisch gibt es Übergänge zu den Krankheitsbildern der progressiven supranukleären Paralyse und der kortikobasalen Degeneration. Es gibt keine kausale Therapie. Die medikamentöse Behandlung ist symptomorientiert und beinhalted die Serotonin-Reuptake-Hemmer, trizyklische Antidepressiva, atypische Neuroleptika, Carbamazepin, Valproat, Lamotrigen und ggf. auch Acetylcholineterase-Inhibitoren, wobei der Evidenzgrad für diese Substanzen insgesamt niedrig ist. Wichtig sind nicht pharmakologische Therapien, wie Physio-/Ergotherapie, Logopädie sowie eine sorgfältige Aufklärung über Krankheitssymptome und ein Beratung der Angehörigen zum Umgang mit den Patienten im Wohnumfeld und Alltag.
Summary
Frontotemporal dementias (FTD) account for only 5–7% of all dementia aetiologies. However, FTD is one common form of dementia in the presenile period with a symptom onset between an age of 45 and 65 years. FTD are clinically classified into a group of rare genetic variants, the behavioural variant, primary progressive aphasias and a variant including motor neuron symptoms (FTD-MNS). In recent years the pathobiological characteristics of some FTD variants was clarified, demonstrating a pathological accumulation of TAR-DNA binding protein 43 (TDP-43) as a common pathological substrate. The revised diagnostic criteria of the behavioural variant of the FTD require at least three of six clinically discriminating features (disinhibition, apathy, loss of sympathy, perseverative behaviours, hyperorality and dysexecutive neuropsychological profile). The primary progressive aphasias are classified in a nonfluent/agrammatic variant, a logopenic variant and a semantic variant according to clinical and imaging features. Movement disorders and more precisely a Parkinsonian syndrome can be part of the FTD spectrum. Some clinical features overlap the clinical diagnosis of a progressive supranuclear paralysis and the corticobasal ganglionic degeneration. A causal therapy does not exist and medical treatment is directed at the patient’s key symptoms. Different agents such as serotonin reuptake inhibitors, tricyclic antidepressants, atypical neuroleptics, carbamazepine, valproate, lamotrigine and when indicated also acetylcholinesterase inhibitors are potentially helpful. All together, theses medical treatments have a low level of evidence. Non-pharmacological therapies such as physiotherapy, occupational therapy, speech therapy and disease-specific education of the patient and their relatives are important to ensure a safe residential environment and daily routine.
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Witt, K., Deuschl, G. & Bartsch, T. Frontotemporale Demenzen. Nervenarzt 84, 20–32 (2013). https://doi.org/10.1007/s00115-012-3477-x
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DOI: https://doi.org/10.1007/s00115-012-3477-x
Schlüsselwörter
- Frontotemporale Demenz
- Primär progressive Aphasien
- TAR-DNA-bindendes Protein 43
- Diagnosekriterien
- Therapie