title: Pseudobulbar dysarthria in the initial stage of motor neuron disease with dementia: a clinicopathological report of two autopsied cases.
abstract: We retrospectively analyzed the clinical features of two cases of neurodegenerative disease, whose initial symptoms were motor speech disorder and dementia, brought to autopsy. We compared the distributions of pathological findings with the clinical features. The main symptom of speech disorder was dysarthria, involving low pitch, slow rate, hypernasality and hoarseness. Other than these findings, effortful speech, sound prolongation and initial difficulty were observed. Moreover, repetition of multisyllables was severely impaired compared to monosyllables. Repetition and comprehension of words and sentences were not impaired. Neither atrophy nor fasciculation of the tongue was observed. Both cases showed rapid progression to mutism within a few years. Neuropathologically, frontal lobe degeneration including the precentral gyrus was observed. The bilateral pyramidal tracts also showed severe degeneration. However, the nucleus of the hypoglossal nerve showed only mild degeneration. These findings suggest upper motor neuron dominant motor neuron disease with dementia. We believe the results indicate a subgroup of motor neuron disease with dementia whose initial symptoms involve pseudobulbar palsy and dementia, and which shows rapid progression to mutism.
authors: Department of Neurology, Showa University School of Medicine, Tokyo, Japan. k-ishihara@mvj.biglobe.ne.jp
authors: Kenji
authors: K
authors: Ishihara
authors: Shigeo
authors: S
authors: Araki
authors: Nami
authors: N
authors: Ihori
authors: Yoshio
authors: Y
authors: Suzuki
authors: Jun-ichi
authors: J
authors: Shiota
authors: Nobutaka
authors: N
authors: Arai
authors: Imaharu
authors: I
authors: Nakano
authors: Mitsuru
authors: M
authors: Kawamura
mesh_terms: D000368:Aged; D001344:Autopsy; D001921:Brain / Q000000981:diagnostic imaging / Q000378:metabolism / Q000473:pathology*; D004268:DNA-Binding Proteins / Q000378:metabolism; D003704:Dementia / Q000150:complications* / Q000175:diagnosis; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications* / Q000175:diagnosis; D020828:Pseudobulbar Palsy / Q000175:diagnosis / Q000209:etiology*; D034702:RNA-Binding Protein FUS / Q000378:metabolism; D012189:Retrospective Studies; D013194:Staining and Labeling; D015899:Tomography, Emission-Computed, Single-Photon
chemical_list: D004268:DNA-Binding Proteins; D034702:RNA-Binding Protein FUS