Case history
Case history #1
A 55-year-old man who worked as a technician developed difficulty finding words 2 years earlier, which has evolved into dysfluency, frequent repetition of remarks and questions, stereotypies (purposeless behaviours or fragments of speech frequently repeated, without regard to context), and echolalia (reflexive repetition of another's speech). In the past year, he has also become forgetful. His work efficiency deteriorated due to his poor comprehension, reasoning, planning, and completion, resulting in disability leave. He also became unfeeling, intrusive (indiscriminately approaching strangers), child-like, and impulsive. He developed rigid routines (e.g., insistence on the same TV shows) and coarse manners (e.g., eating out of serving bowls, jumping queues, and walking away from conversation). Restlessness is marked: each day he bikes, swims many laps, runs 10 km, and 'volunteers' at a local nursing home, making the rounds with all maintenance crews. On examination, he is pleasant and cooperative. Given opportunity, he quizzes the examiner on trivial facts (such as listing capital cities). Depression is not evident, and he does not have euphoria, psychosis, or paranoia. Speech is mildly non-fluent. Verbal fluency is impaired. Mini-mental state examination score is 29 points.
Case history #2
A 58-year-old male teacher developed dysnomia, spelling errors, impaired comprehension of reading and conversation, and diminished singing ability. He also has impaired attention, planning, and organisation, along with declining self-care, child-like behaviour, and altered social habits (e.g., eating meals with his fingers). He developed anxiety. Two years into the illness, a neurologist suspected early dementia. Mini-mental state examination score was 27 points and the neurological examination was normal. Brain magnetic resonance imaging showed temporal lobe atrophy, predominantly left-sided. Three years later, his partner complains about impulsive and obstinate behaviour, and excessive eating. Formal neuropsychological testing shows mini-mental state examination score of 28, impaired memory and learning, impaired word and sentence comprehension, marked dysnomia, grammatical and spelling errors, and poor copying of a complex figure.
Other presentations
Language presentations are well established, with patients presenting with progressive loss of fluency, syntax, and grammar (primary non-fluent aphasia) that progresses to mutism, or with progressive loss of word (and object) knowledge that results in profound agnosia. Apathetic presentations are also commonly observed, with absent or markedly diminished emotions, self-neglect, and social detachment the predominant features. Parkinsonism may accompany the behavioural or language features, or dominate the presentation with unilateral parkinsonism, apraxia, and alien hand (a phenomenon in which the upper limb performs complex involuntary movements, and may be seen in corticobasal degeneration [CBD]), or with bilateral parkinsonism, falling, and vertical gaze palsy (progressive supranuclear palsy [PSP]). Sometimes an overlapping CBD/PSP presentation is seen. FTD may also present with features of amyotrophic lateral sclerosis (ALS); with a strong male family history of ALS; and presenting features of several months of aloofness, emotional detachment, irritability, rudeness, forgetfulness, and excessive or binge eating. Hyper-reflexia and tongue fasciculations may be present, although these are insufficient for formal diagnosis of ALS.
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