Suicide Attempt as the Presenting Symptom of C9orf72 Dementia
A 72-year-old German man without significant previous medical or psychiatric illnesses was admitted after trying to hang himself. An adjustment disorder was assumed at first, but psychiatric examination revealed a striking lack of concern about the suicide attempt, frequent irrelevant answers, and inappropriate jocularity without any signs of depression. His wife reported a 2-year history of subtle behavioral disinhibition (short episodes of socially inappropriate behavior and impulsive reactions) and deficits in short-term memory and face recognition in her husband, but history and observation did not reveal additional symptoms necessary for a diagnosis of possible behavioral frontotemporal dementia (5). (For details of the diagnostic workup, see the data supplement that accompanies the online edition of this letter.) Neuropsychological testing results revealed mild deficits in the domains of verbal episodic memory and visuospatial abilities but not in executive functions such as strategic thinking and executive flexibility (see the online data supplement). MRI and [18F]fluorodeoxyglucose positron emission tomography (FDG-PET) revealed several abnormalities, including temporal atrophy with hippocampal degeneration and biparietal and temporomesial hypometabolism, but no frontal atrophy or hypometabolism (Figure 1). Although behavioral frontotemporal dementia can present with anterior temporal atrophy without frontal atrophy or hypometabolism (5), this imaging pattern was considered to be atypical for behavioral frontotemporal dementia or, at least, to be unspecific. The patient’s sister died by suicide at age 50, and his mother began to show late-onset progressive nonfluent aphasia at age 85. Based on this family history, C9orf72 genotyping was initiated, revealing a pathogenic expansion of more than 50 repeats (2, 3). A written informed consent was obtained from the patient after the procedures and the publication had been fully explained to him and his family.
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