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 behaviors 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), childlike, 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 6.5 miles, and "volunteers" at a local nursing home, making the rounds with all maintenance crews. On exam, 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 nonfluent. Verbal fluency is impaired. Mini-mental state exam 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 organization, along with declining self-care, childlike behavior, 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 exam score was 27 points and the neurologic exam was normal. Brain magnetic resonance imaging showed temporal lobe atrophy, predominantly left-sided. Three years later, his partner complains about impulsive and obstinate behavior, and excessive eating. Formal neuropsychological testing shows mini-mental state exam 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 nonfluent 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 behavioral 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. Hyperreflexia and tongue fasciculations may be present, although these are insufficient for formal diagnosis of ALS.
Use of this content is subject to our disclaimer