Degenerative dementia with a clinical syndrome resembling diffuse neurofibrillary tangles with calcification(DNTC)but only slight calcification in the basal ganglia

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  • Igarashi Takeshi
    Department of Speech Therapy, School of Health Sciences, Niigata University of Health and Welfare.
  • Sato Atsushi
    Division of Speech Therapy, Department of Rehabilitation, Niigata Rehabilitation Hospital
  • Imamura Toru
    Department of Speech Therapy, School of Health Sciences, Niigata University of Health and Welfare. Department of Neurology, Niigata Rehabilitation Hospital

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  • Diffuse neurofibrillary tangles with calcification (DNTC) 類似の臨床症状を呈するが基底核の石灰沈着が軽微な変性疾患性痴呆の一例

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We reported a patient who showed a history and neurobehavioral and neuropsychological features resembling those of diffuse neurofibrillary tangles with calcification(DNTC), but who exhibited only slight calcification of the basal ganglia in the brain CT. A 73-year-old right-handed retired nurse presented to us with a 12-year history of abnormal behavior. At the age of 63, she repeatedly complained of anal pain, for which the psychiatric diagnosis was “psychosomatic”. At 67 years of age, she showed visual and auditory hallucinations. The complaint of anal pain subsided at the age of 70. She developed forgetfulness and delusions of persecution at the age of 71, and showed apathy, indifference to self-hygiene and timetable-like stereotyped behavior at the age of 72. A neurological examination was normal. She scored 28/30 on the MMSE and 7/70 on the Alzheimer's Disease Assessment Scale(ADAS). Amnesia and constructional disturbances were minimal, and she showed no aphasia, ideomotor or ideational apraxia, or visuocognitive disturbances. On the other hand, executive dysfunction was apparent and extensive on various tasks including the Porteus Maze, Wisconsin Card Sorting Test and Behavioural Assessment of the Dysexecutive Syndrome(BADS). In daily living, disinhibition, irritability, stereotyped behavior and timetable-like daily living were observed. A brain CT revealed frontal atrophy and spotty calcifications of minimal degree in the bilateral basal ganglia. A single photon emission CT(SPECT)showed medial frontal and anterior temporal hypoperfusions. The patient's current neurobehavioral and neuropsychological syndrome is consistent with the clinical features of frontotemporal dementia(FTD). However, her history, including extremely slow progression and hallucinations and delusions in the early disease stage, is inconsistent with frontotemporal lobar degeneration(FTLD). Taken together, the clinical features rather resemble those of DNTC. We discussed possible clinical diagnosis of the patient as DNTC associated with slight brain calcification in CT.

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