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. Author manuscript; available in PMC: 2020 Aug 1.
Published in final edited form as: J Neurol. 2019 May 17;266(8):1998–2009. doi: 10.1007/s00415-019-09362-5

Table 3 –

Features of patients who changed from bvFTD diagnoses due to lack of progression and psychiatric symptomatology

Age at first diagnosis Sex Follow-up (yrs) Cognitive testing Clinician’s neuroimaging interpretation Family history Psychiatric features Behavior comments
Patient 1 47 M 10 Low-average verbal memory that improved through follow-up Differing interpretations given – mild frontal and insular atrophy or normal Autosomal dominant FTD Depression (onset in 30s, moderate to severe on initial presentation), one prior possible hypomanic episode Marital discord; spouse reported decrease in empathy, pornography viewing initially reported as compulsive, later deemed longstanding
Patient 2 66 M 4 Mild executive dysfunction, but no clear progression over time. Dorsal frontal atrophy, but no progression over follow-up Late-life dementia in both parents Depression/anxiety (onset in 40s), possible auditory hallucinations in childhood, episodes suggestive of mania throughout disease course. At first visit, spouse reported loss of empathy, rigidity, impulsivity, obsessions, sweet craving. Separated from spouse after year 1. No behavioral symptoms reported by family at subsequent visits.
Patient 3 65 M 1 Mild executive dysfunction. Improved at subsequent testing. No definitive Atrophy Bipolar disorder and substance abuse Depression (onset in 30s), diagnosed with bipolar disorder in 50s Prior episodes of impulsivity. Developed constant impulsivity, hypersexuality, apathy, poor judgment for over two years. Behaviors improved by time of year 2 visit.
Patient 4 54 F 3 Low average executive function initially. No decline over time. Initially called mild biparietal atrophy. Later interpreted as normal. AD vs FTD in one family member, psychiatric illness in others Depression since teens, signs of functional movement disorder developed after behavior change. Self-endorsed apathy, social withdrawal, sweet preference. Mild loss of empathy and compulsions reported by spouse.
Patient 5 47 M 1 Low average executive function initially. No decline over time. No definitive atrophy. Parent with bipolar illness and dementia diagnoses, sibling with early onset dementia. Mood disorder since college, potential history of hypomanic episodes, longstanding history of risk taking Spouse reported loss of empathy, mild disinhibition, intensified and narrow interests. He endorsed marital discord.
Patient 6 63 M 1 Impaired executive function, mild verbal memory impairment. Stable to mildly improved over time. Mild generalized atrophy for age. Late-life dementia, mental illness Lifelong personality differences (little empathy, excessive joking, collecting) Decompensation of longstanding traits – more distant, worse hoarding, overeating, more frequently crosses social boundaries
Patient 7 75 M 1 Within normal limits at both time points. Frontal, parietal, and temporal atrophy identified at first visit. Thought normal at second visit. Parent with late-life impulsivity, alcohol abuse in the other parent Day to day fluctuations in mood, energy level, and cognitive function from elated to lethargic, suggestive of, but excessively fast for bipolar illness Mild loss of empathy, disinhibition, repetitive behavior. Non-progressive, consistent with some longstanding traits..