title: [Clinico-pathological investigation of two patients with dementia with motor neuron disease].
abstract: Dementia with motor neuron disease (D-MND) is characterized clinically by frontal and neurological signs, and pathologically by localized atrophy of the fronto-temporal lobes and neuronal ubiquitin(Ub)-positive inclusions. In this study, we compared the clinico-pathological findings of two patients with D-MND. Case 1 (55-year-old male): At the age of 51, he developed personality change and disinhibition, lacking neurological signs. Brain MRI exhibited localized atrophy of the frontal lobes. At the age of 54, he showed dysphagia and died after a disease duration of 4 years. Neuropathologically, the cerebrum showed localized atrophy of the dorsal area of the frontal lobes. The atrophied cerebral cortex demonstrated moderate neuronal loss with spongy change and gliosis in the superficial layers. The brainstem and spinal cord revealed moderate neuronal loss in the substantia nigra, severe neuronal loss with Bunina bodies in the hypoglossal nucleus, and moderate neuronal loss in the cervical anterior horn. There were some Ub-positive neuronal inclusions in the atrophied cortex and many in the dentate gyrus. Case 2 (68-year-old female): At the age of 64, she developed personality change, and then gait disturbance and dysarthria. Brain MRI exhibited localized atrophy of the fronto-temporal lobes. At the age of 67, she showed dysphagia with Babinski signs and died after a disease duration of 4 years. Neuropathologically, the cerebrum showed localized atrophy of the basal area of the temporal lobes, especially on the right side. The atrophied cerebral cortex demonstrated moderated neuronal loss with spongy change and gliosis in the superficial layers. The pre-central cortex revealed severe loss of Betz cells. The brainstem and spinal cord showed mild neuronal loss without Bunina bodies in the hypoglossal nucleus and cervical anterior horn, accompanied by severe degeneration of the bilateral pyramidal tracts. There were many Ub-positive neuronal inclusions with a few neurites in the atrophied cortex and some in the dentate gyrus. Cases 1 and 2 were clinically diagnosed as Pick's disease (PiD) and D-MND, respectively, although pathological diagnoses were both D-MND. Case 1 showed neuropathological findings typical to D-MND, whereas case 2 showed neuropathological findings common to atypical Pick's disease (aPiD). D-MND and aPiD are should be clinico-pathologically differentiated, although they are included in the frontotemporal lobar degeneration with motor neuron disease-type inclusions.
authors: Department of Psychiatry, Juntendo Tokyo Koto Geriatric Medical Center, Juntendo University School of Medicine, Tokyo, Japan.
authors: Ryoko
authors: R
authors: Yamamoto
authors: Eizo
authors: E
authors: Iseki
authors: Norio
authors: N
authors: Murayama
authors: Michiko
authors: M
authors: Minegishi
authors: Michihiro
authors: M
authors: Kimura
authors: Ko
authors: K
authors: Eto
authors: Heii
authors: H
authors: Arai
authors: Sadayoshi
authors: S
authors: Ohbu
authors: Daisuke
authors: D
authors: Hatanaka
authors: Hiroaki
authors: H
authors: Hino
authors: Koshiro
authors: K
authors: Fujisawa
mesh_terms: D000368:Aged; D003704:Dementia / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D003937:Diagnosis, Differential; D017809:Fatal Outcome; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D045586:Intranuclear Inclusion Bodies / Q000378:metabolism; D008297:Male; D008875:Middle Aged; D016472:Motor Neuron Disease / Q000150:complications / Q000175:diagnosis / Q000473:pathology*; D020774:Pick Disease of the Brain / Q000175:diagnosis / Q000473:pathology; D013702:Temporal Lobe / Q000473:pathology*; D025801:Ubiquitin / Q000378:metabolism
chemical_list: D025801:Ubiquitin