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[Frontal lobe dementia. Clinical-pathologic case reports]
C Zachhuber1, F Leblhuber, C Bancher
1Abteilung für Neurologisch-Psychiatrische Gerontologie, Landesnervenklinik Wagner-Jauregg, Linz.
Abstract:
Frontal lobe dementia (FLD) is characterised clinically by personality changes and a progressive speech disorder finally leading to mutism. In the course of the disease also other neurological syndromes may occur such as parkinsonism, a partial Klüver-Bucy-syndrome or a degeneration of motoneurons (FLD + MND). The latter leads to death within about three years. The clinical diagnosis of FLD is supported by functional (SPECT) and morphological (CT, MRI) investigations. From 1988 to 1997, 9 cases of FLD (6 female, 3 male) were clinically diagnosed at our department of Gerontology, LNK Linz. In two of these cases the clinical diagnosis was confirmed histopathologically. Characteristically, all except one patients showed a presenile beginning of the disease. The clinical course was slowly progressive with a mean duration of about 10 years. Special attention was given to additional signs and symptoms of motor neuron disease, parkinsonism and hyperorality. Six patients suffered from FLD + MND; parkinsonism (rigid-akinetic type) and a partial Klüver-Bucy-syndrome were diagnosed in 5 cases each. In histopathological investigations the incidence of FLD seems to increase. This type of dementia should be considered as an important diagnosis differential of presenile dementia-syndromes.
Insights
Frontal lobe dementia (FLD) presents with personality changes and speech decline. This study highlights FLD
Area of Science:
- Neurology
- Gerontology
Background:
- Frontal lobe dementia (FLD) is a neurodegenerative disorder.
- Characterized by personality shifts and progressive speech impairment leading to mutism.
Observation:
- FLD can co-occur with other neurological syndromes like parkinsonism, Klüver-Bucy syndrome, and motor neuron disease (MND).
- FLD with MND has a rapid, fatal course within approximately three years.
- Clinical diagnosis is aided by SPECT, CT, and MRI.
- Nine FLD cases were diagnosed between 1988-1997, with most showing presenile onset.
Findings:
- Six of nine patients presented with FLD + MND.
- Parkinsonism and partial Klüver-Bucy syndrome were observed in five cases each.
- Histopathological studies suggest an increasing incidence of FLD.
Implications:
- FLD should be considered in the differential diagnosis of presenile dementia syndromes.
- Understanding FLD's varied presentations is crucial for accurate diagnosis and management.
- Further research into FLD's increasing incidence and pathological mechanisms is warranted.