Biomaterial Database

[Clinico-pathological investigation of two patients with dementia with motor neuron disease]. 2007

Ryoko Yamamoto, and Eizo Iseki, and Norio Murayama, and Michiko Minegishi, and Michihiro Kimura, and Ko Eto, and Heii Arai, and Sadayoshi Ohbu, and Daisuke Hatanaka, and Hiroaki Hino, and Koshiro Fujisawa

Department of Psychiatry, Juntendo Tokyo Koto Geriatric Medical Center, Juntendo University School of Medicine, Tokyo, Japan.

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.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008875	Middle Aged	An adult aged 45 - 64 years.	Middle Age
D003704	Dementia	An acquired organic mental disorder with loss of intellectual abilities of sufficient severity to interfere with social or occupational functioning. The dysfunction is multifaceted and involves memory, behavior, personality, judgment, attention, spatial relations, language, abstract thought, and other executive functions. The intellectual decline is usually progressive, and initially spares the level of consciousness.	Senile Paranoid Dementia,Amentia,Familial Dementia,Amentias,Dementia, Familial,Dementias,Dementias, Familial,Dementias, Senile Paranoid,Familial Dementias,Paranoid Dementia, Senile,Paranoid Dementias, Senile,Senile Paranoid Dementias
D003937	Diagnosis, Differential	Determination of which one of two or more diseases or conditions a patient is suffering from by systematically comparing and contrasting results of diagnostic measures.	Diagnoses, Differential,Differential Diagnoses,Differential Diagnosis
D005260	Female		Females
D005625	Frontal Lobe	The part of the cerebral hemisphere anterior to the central sulcus, and anterior and superior to the lateral sulcus.	Brodmann Area 8,Brodmann's Area 8,Frontal Cortex,Frontal Eye Fields,Lobus Frontalis,Supplementary Eye Field,Area 8, Brodmann,Area 8, Brodmann's,Brodmanns Area 8,Cortex, Frontal,Eye Field, Frontal,Eye Field, Supplementary,Eye Fields, Frontal,Frontal Cortices,Frontal Eye Field,Frontal Lobes,Lobe, Frontal,Supplementary Eye Fields
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D013702	Temporal Lobe	Lower lateral part of the cerebral hemisphere responsible for auditory, olfactory, and semantic processing. It is located inferior to the lateral fissure and anterior to the OCCIPITAL LOBE.	Anterior Temporal Lobe,Brodmann Area 20,Brodmann Area 21,Brodmann Area 22,Brodmann Area 37,Brodmann Area 38,Brodmann Area 52,Brodmann's Area 20,Brodmann's Area 21,Brodmann's Area 22,Brodmann's Area 37,Brodmann's Area 38,Brodmann's Area 52,Inferior Temporal Gyrus,Middle Temporal Gyrus,Parainsular Area,Fusiform Gyrus,Gyrus Fusiformis,Gyrus Temporalis Superior,Inferior Horn of Lateral Ventricle,Inferior Horn of the Lateral Ventricle,Lateral Occipito-Temporal Gyrus,Lateral Occipitotemporal Gyrus,Occipitotemporal Gyrus,Planum Polare,Superior Temporal Gyrus,Temporal Cortex,Temporal Gyrus,Temporal Horn,Temporal Horn of the Lateral Ventricle,Temporal Operculum,Temporal Region,Temporal Sulcus,Anterior Temporal Lobes,Area 20, Brodmann,Area 20, Brodmann's,Area 21, Brodmann,Area 21, Brodmann's,Area 22, Brodmann,Area 22, Brodmann's,Area 37, Brodmann,Area 37, Brodmann's,Area 38, Brodmann,Area 38, Brodmann's,Area 52, Brodmann,Area 52, Brodmann's,Area, Parainsular,Areas, Parainsular,Brodmanns Area 20,Brodmanns Area 21,Brodmanns Area 22,Brodmanns Area 37,Brodmanns Area 38,Brodmanns Area 52,Cortex, Temporal,Gyrus, Fusiform,Gyrus, Inferior Temporal,Gyrus, Lateral Occipito-Temporal,Gyrus, Lateral Occipitotemporal,Gyrus, Middle Temporal,Gyrus, Occipitotemporal,Gyrus, Superior Temporal,Gyrus, Temporal,Horn, Temporal,Lateral Occipito Temporal Gyrus,Lobe, Anterior Temporal,Lobe, Temporal,Occipito-Temporal Gyrus, Lateral,Occipitotemporal Gyrus, Lateral,Operculum, Temporal,Parainsular Areas,Region, Temporal,Sulcus, Temporal,Temporal Cortices,Temporal Gyrus, Inferior,Temporal Gyrus, Middle,Temporal Gyrus, Superior,Temporal Horns,Temporal Lobe, Anterior,Temporal Lobes,Temporal Lobes, Anterior,Temporal Regions
D016472	Motor Neuron Disease	Diseases characterized by a selective degeneration of the motor neurons of the spinal cord, brainstem, or motor cortex. Clinical subtypes are distinguished by the major site of degeneration. In AMYOTROPHIC LATERAL SCLEROSIS there is involvement of upper, lower, and brainstem motor neurons. In progressive muscular atrophy and related syndromes (see MUSCULAR ATROPHY, SPINAL) the motor neurons in the spinal cord are primarily affected. With progressive bulbar palsy (BULBAR PALSY, PROGRESSIVE), the initial degeneration occurs in the brainstem. In primary lateral sclerosis, the cortical neurons are affected in isolation. (Adams et al., Principles of Neurology, 6th ed, p1089)	Anterior Horn Cell Disease,Familial Motor Neuron Disease,Lateral Sclerosis,Motor Neuron Disease, Lower,Motor Neuron Disease, Upper,Lower Motor Neuron Disease,Motor Neuron Disease, Familial,Motor Neuron Disease, Secondary,Motor System Disease,Primary Lateral Sclerosis,Secondary Motor Neuron Disease,Upper Motor Neuron Disease,Lateral Scleroses,Lateral Scleroses, Primary,Lateral Sclerosis, Primary,Motor Neuron Diseases,Motor System Diseases,Neuron Disease, Motor,Neuron Diseases, Motor,Primary Lateral Scleroses,Scleroses, Lateral,Scleroses, Primary Lateral,Sclerosis, Lateral,Sclerosis, Primary Lateral