OBJECTIVE: Variants of frontotemporal lobar degeneration (FTLD) are associated with distinct clinical, pathological, and neuroanatomical profiles. Lines of emerging research indicate a rare variant with focal atrophy of the right temporal lobe (RTLA). The objective was to present case data and discussion of an individual with RTLA in order to assist with conceptualization of this variant. METHOD: A 60-year-old, right-handed, college-educated Protestant minister with RTLA was evaluated. This patient presented with several hallmark behavioral and psychiatric features with personality changes, including hyper-religiosity, depression, and social disinhibition. Given the profession of the patient, the observed personality alterations (e.g., religiosity and pietism) were initially excused, which delayed diagnosis. RESULTS: In addition to cognitive deficits, an examination of affect processing within visual and auditory channels revealed severe impairment in emotion recognition with features of prosopagnosia. These impairments were in general more severe than the cognitive impairment observed on traditional neuropsychological measures. CONCLUSIONS: This case provides support for an FTLD right temporal lobe variant. This case also illustrates the importance of neuropsychological evaluation of affect processing in the differential diagnosis and treatment planning for FTLD and its subtypes.
OBJECTIVE: Variants of frontotemporal lobar degeneration (FTLD) are associated with distinct clinical, pathological, and neuroanatomical profiles. Lines of emerging research indicate a rare variant with focal atrophy of the right temporal lobe (RTLA). The objective was to present case data and discussion of an individual with RTLA in order to assist with conceptualization of this variant. METHOD: A 60-year-old, right-handed, college-educated Protestant minister with RTLA was evaluated. This patient presented with several hallmark behavioral and psychiatric features with personality changes, including hyper-religiosity, depression, and social disinhibition. Given the profession of the patient, the observed personality alterations (e.g., religiosity and pietism) were initially excused, which delayed diagnosis. RESULTS: In addition to cognitive deficits, an examination of affect processing within visual and auditory channels revealed severe impairment in emotion recognition with features of prosopagnosia. These impairments were in general more severe than the cognitive impairment observed on traditional neuropsychological measures. CONCLUSIONS: This case provides support for an FTLD right temporal lobe variant. This case also illustrates the importance of neuropsychological evaluation of affect processing in the differential diagnosis and treatment planning for FTLD and its subtypes.
Entities:
Keywords:
Emotion processing.; Frontotemporal dementia; Right temporal lobe; Right temporal lobe atrophy
Authors: Hulya Ulugut Erkoyun; Colin Groot; Ronja Heilbron; Anne Nelissen; Jonathan van Rossum; Roos Jutten; Ted Koene; Wiesje M van der Flier; Mike P Wattjes; Philip Scheltens; Rik Ossenkoppele; Frederik Barkhof; Yolande Pijnenburg Journal: Brain Date: 2020-09-01 Impact factor: 15.255