Clinical Features and Diagnosis of Frontotemporal DementiaKertesz A.
St. Joseph’s Hospital, University of Western Ontario, London, Ont., Canada
Frontotemporal degeneration (FTD), formerly known as Pick’s disease has become recognized as adistinct and relatively common entity encompassing behavioural (bvFTD language (PPA) andextrapyramidal (CBD/PSP) presentations. Further clinical subdivisions such as semantic dementia(SD), and pathological subtypes such as mesial temporal sclerosis increase the complexity of diagnosis.The relatively younger age of onset, the typical presentations of syndromes and focal asymmetricalfrontotemporal atrophy on imaging allows experienced clinicians to make the diagnosisconfidently as long as the overlap between the syndromes is recognized. There is also an overlapwith ALS pathologically and clinically. The underlying histology in FTD/Pick complex is ubiquitinpositive tau and synuclein negative neuronal inclusions (FTLD-U) in more than half of autopsies andtau positive CBD/PSP/ Pick bodies (FTLD-T) in the rest. The clinical syndromes of bvFTD and SD arelikely associated with FTLD-U and PPA/CBDS/PSP with FTLD-T, but there is too much overlap to predictthe pathology from the clinical syndromes reliably. The ubiquitin-tau pathological dichotomy isbest considered under the Pick complex umbrella to allow for the significant overlap. So far trazodonein behavior and galantamine in aphasia had symptomatic benefit in small trials and SSRI-sand antipsychotics in uncontrolled reports were used as symptomatic therapies. Recent discoveriesof tau and progranulin (in the ubiquitin-positive cases) mutations on chromosome 17 and othermutations on chromosome 3 and 9 in the high incidence of autosomal dominant families and acommon protein abnormality, the TDP-43 in FTLD-U and ALS are likely to be important in findingtherapeutic targets.
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