Idiopathic normal pressure hydrocephalus and frontotemporal dementia: an unexpected association. Issue 6 (14th December 2022)

• Record Type: Journal Article
• Authors: de Guilhem de Lataillade, Adrien; Boutoleau-Bretonnière, Claire; Aguilar-Garcia, Jesus; Pallardy, Amandine; Bigot-Corbel, Edith; Roualdes, Vincent; Leroy, Julie; Damier, Philippe; Pouclet-Courtemanche, Hélène
• Is Part Of: Brain communications. Volume 4:Issue 6(2022)
• Publication Date: 2022-12-14
• Subjects: behavioural variant of frontotemporal lobar degeneration -- Alzheimer's disease -- idiopathic normal pressure hydrocephalus; 616
• DOI: 10.1093/braincomms/fcac319
• Languages: English
• ISSNs: 2632-1297

Abstract

Abstract: Idiopathic normal pressure hydrocephalus has a complex multifactorial pathogenesis and is associated with Alzheimer's disease in many patients. To date, it is not well known if a similar association exists with behavioural variant of frontotemporal lobar degeneration. In a first step, we compare the prevalence of idiopathic normal pressure hydrocephalus in two groups of patients, one with behavioural variant of frontotemporal lobar degeneration ( n = 69) and the other with Alzheimer's disease ( n = 178). In the second step, we describe more precisely the phenotype of patients with the association of idiopathic normal pressure hydrocephalus and behavioural variant of frontotemporal lobar degeneration. Firstly, we report that the prevalence of idiopathic normal pressure hydrocephalus was far higher in the group of patients with behavioural variant of frontotemporal lobar degeneration than in the group of patients with Alzheimer's disease (7.25% and 1.1%, respectively, P = 0.02). Secondly, we show that patients with the double diagnosis share common clinical and para-clinical features of both idiopathic normal pressure hydrocephalus and behavioural variant of frontotemporal lobar degeneration patients, including CSF shunting efficacy in real-life experience. Overall, our results suggest a link between these two conditions and should encourage neurologists to look for idiopathic normal pressure hydrocephalus in their behavioural variant of frontotemporal lobar degeneration patients in the event of gait disturbances; the benefit/risk balance could indeed be in favour of shunt surgery for selected patients with this newly described entity. Abstract : De Guilhem de Lataillade et al. report an unexpected higher prevalence of idiopathic normal pressure hydrocephalus in a group of patients with behavioural variant of frontotemporal lobar degeneration than in a group with Alzheimer's disease. The results suggest a possible pathophysiological link between these entities initially thought to be unrelated. Graphical Abstract: Graphical abstract Video Abstract: