A case of Pick's disease presenting with progressive nonfluent speech.
- Author:
Sung Shin AHN
1
;
Duk L NA
;
Pil Wook JUNG
;
Jung Il LEE
;
Yeon Lim SUH
;
Sang Eun KIM
Author Information
1. Dept of Neurology, Sangsung Medical Center, College of Medicine, Sung Kyun Kwan University.
- Publication Type:Case Report
- MeSH:
Aphasia;
Atrophy;
Biopsy;
Brain;
Cytoplasm;
Eosinophils;
Female;
Frontal Lobe;
Frontotemporal Dementia;
Gliosis;
Humans;
Inclusion Bodies;
Kluver-Bucy Syndrome;
Korea;
Lewy Bodies;
Magnetic Resonance Imaging;
Neurofibrillary Tangles;
Neurons;
Neuropsychological Tests;
Pathology;
Pick Disease of the Brain*;
Plaque, Amyloid;
Temporal Lobe
- From:Journal of the Korean Neurological Association
1997;15(5):1162-1172
- CountryRepublic of Korea
- Language:Korean
-
Abstract:
BACKGROUND AND SIGNIFICANCE: Frontotemporal dementia is a behavioral disorder arising from nonAlzheimer's disease atrophy of frontal and anterior temporal lobe. Clinical manifestations include frontal lobe dysfunction. Kluver-Bucy syndrome or progressive language impairments. Two types of histological change underline, the atrophy. The commoner pathology is nerve cell loss and spongiform change with astrocytic gliosis. The second one is typical Pick-type histology characterized by intraneuronal inclusion body and astrocytic gliosis. We report a case with biopsy proved Pick's disease presenting with progressive nonfluent speech. CASE: A 41-years, old right-handed woman developed progressive language impairment over a period of 6 months. Brain MRI revealed asymmetric frontotemporal cortical atrophy more severe on the left side and 18F-FDG-brain, PET showed hypometabolism in the same area. Neuropsychological test including Korean version-Western Aphasia Battery revealed non-fluent speech as well as frontal lobe, dysfunction. A biopsy from left frontal lobe, demonstrate neuronal loss and diffuse astrogliosis. In the cytoplasm of remaining neurons are eosinophilic inclusion bodies which are neurofilament-positive with immunostaining. Senile plaque, neurofibrillary tangle and cortical Lewy body were absent. COMMENT: We report a case presenting with progressive nonfluent speech whose imaging and pathological findings are compatible with Pick's disease, which may be the first biopsy proven case in Korea.
