P106 PROSPERA (PROgressive Supranuclear Palsy and the Effect of Rasagiline)

Lorenzl, Stefan; Hensler, Mira; Abright, C.; Deutschenbaur, Lorenz; Paul, Serene S.; Pfannschmidt, C.; Baumgartner, Annette

doi:10.1016/j.baga.2011.01.026

Cited by 1 publication

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“… 105 This first clinical trial in bvFTD will most probably unveil potential difficulties with studies in bvFTD: the lack of appropriate outcome parameters; problems with recruitment; diminished patient compliance; behavioral disturbances that hamper the examinations, interviews, and neuroimaging; and concomitant psychiatric medication in high doses. A number of tau-based disease-modifying therapies have already been investigated in clinical trials: 106 the glycogen synthase kinase inhibitors lithium (NCT00703677) 107 and tideglusib (NCT01350362, NCT01049399); 108 , 109 riluzole, a sodium channel blocker; 110 coenzyme Q10, which improves mitochondrial function; 111 rasagiline, a monoamine oxidase inhibitor (NCT01187888); 112 and davunetide, a microtubule stabilizer (NCT01110720, NCT01056965). 113 , 114 Beyond that, preclinical studies are under way that aim at the identification of agents with the potential to normalize GRN levels either by increased production or reduced clearance in FTLD-TDP cases that are caused by loss of function mutations in the GRN gene.…”

Section: Future Directionsmentioning

confidence: 99%

Frontotemporal lobar degeneration: current perspectives

Riedl¹,

Mackenzie²,

Förstl³

et al. 2014

NDT

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The term frontotemporal lobar degeneration (FTLD) refers to a group of progressive brain diseases, which preferentially involve the frontal and temporal lobes. Depending on the primary site of atrophy, the clinical manifestation is dominated by behavior alterations or impairment of language. The onset of symptoms usually occurs before the age of 60 years, and the mean survival from diagnosis varies between 3 and 10 years. The prevalence is estimated at 15 per 100,000 in the population aged between 45 and 65 years, which is similar to the prevalence of Alzheimer’s disease in this age group. There are two major clinical subtypes, behavioral-variant frontotemporal dementia and primary progressive aphasia. The neuropathology underlying the clinical syndromes is also heterogeneous. A common feature is the accumulation of certain neuronal proteins. Of these, the microtubule-associated protein tau (MAPT), the transactive response DNA-binding protein, and the fused in sarcoma protein are most important. Approximately 10% to 30% of FTLD shows an autosomal dominant pattern of inheritance, with mutations in the genes for MAPT, progranulin (GRN), and in the chromosome 9 open reading frame 72 (C9orf72) accounting for more than 80% of familial cases. Although significant advances have been made in recent years regarding diagnostic criteria, clinical assessment instruments, neuropsychological tests, cerebrospinal fluid biomarkers, and brain imaging techniques, the clinical diagnosis remains a challenge. To date, there is no specific pharmacological treatment for FTLD. Some evidence has been provided for serotonin reuptake inhibitors to reduce behavioral disturbances. No large-scale or high-quality studies have been conducted to determine the efficacy of non-pharmacological treatment approaches in FTLD. In view of the limited treatment options, caregiver education and support is currently the most important component of the clinical management.

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