Autonomic dysregulation in frontotemporal dementia

Autonomic symptoms are associated with a number of neurodegenerative conditions.1 Cerebral structures mediating these symptoms include the anterior cingulate cortex, insular, amygdala, hypothalamus and brainstem.1 ,2 Of the cerebral structures implicated in autonomic control, the hypothalamus3 and insula4 undergo pathological changes in frontotemporal dementia (FTD). Given the locus of the pathology, it is likely that other hypothalamic functions such as blood pressure control, thermoregulation and urinary control may be affected in FTD through the process of autonomic dysfunction. Cardiac dysfunction,5 urinary dysfunction6 and thermoregulatory dysfunction7 have been previously reported in FTD; features which were included in the original 1998 diagnostic criteria.8 Despite these reports, investigations into the integrity of the autonomic system and whether dysfunction results in clinically significant symptoms have been limited. Carers of 69 patients with dementia: 28 behavioural-variant FTD (bvFTD; 10 female (F), 18 male (M)), 20 semantic dementia (SD; 9 F, 11 M), 21 Alzheimer's disease (AD; 10 F, 11 M) from the FTD clinic at Neuroscience Research Australia, completed the Autonomic Symptoms Questionnaire (ASQ), which comprises 44 questions that examine physical symptoms related to autonomic functions including blood pressure/cardiovascular function, gastrointestinal, temperature regulation and sweating, urinary symptoms and sleep. The survey was validated in the Autonomic unit at the National Hospital for Neurology and Neurosurgery, London (see online supplementary file S1). Carers were asked to rate the frequency of …