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022-1340036-2083 Frontotemporal Dementias

MINISTRY OF SCIENCE, EDUCATION AND SPORTS OF THE REPUBLIC OF CROATIA (2007-2014)

Principal Investigator: Rajka Liščić

SUMMARY
Background: A group of frontotemporal dementias (FTD) is a major cause of early non-Alzheimer dementia (AD) and is increasingly being recognized as an important cause of dementia. FTD may be mistaken clinically for AD, particularly in early stage of the disease. However, FTD is behaviourally and pathologically distinct from AD and encompasses three clinical syndromes with heterogeneous neuropathology according to Neary et al. 1998. A motor neuronopathy complicating FTD has been recognized as features of amyotrophic lateral sclerosis (ALS) or motor neuron disease (MND). The disease develops gradually, where changes in personality and behaviour precede the development of symptoms and signs of anterior horn cell involvement. Patients with typical ALS may develop FTD, and vice versa ALS occurs in patients with FTD. There is, however, less awareness of the involvement of cognition and possible dementia in ALS, especially in early stage of the disease.

Hypothesis and aim: As FTD and AD have different prognoses and treatment; there is a need for accurate clinical diagnosis. The hypothesis is that patients, who present with bulbar palsy and ALS, rather than corticospinal features, may be most susceptible to the development of FTD.

Expected results:

  1. To establish criteria for FTD diagnosis.
  2. To determine if ALS patients have cognitive impairment. Follow-up study should tell us if deterioration of cognitive impairment exists and if so weather is correlated with deterioration of motoric functions or not.
  3. To determine the nature of the differing pathological entities behind these heterogeneous forms of lobar atrophy and to find a molecular biological basis that might explain them. With molecular markers becoming available, it will be possible to specify the exact nosological relationship between FTD-ALS and FTD and between FTD-ALS and the classical ALS. We’d like to establish whether or not they do indeed represent phenotypic variants of a common genotype.

Importance: We aim to distinguish between different FTD phenotypes and genotypes