WordNet

a brief treatise on a subject of interest; published in the form of a booklet (同)pamphlet
an extended area of land (同)piece of land, piece of ground, parcel of land, parcel
a system of body parts that together serve some particular purpose
a drawing created by superimposing a semitransparent sheet of paper on the original image and copying on it the lines of the original image (同)trace
the act of drawing a plan or diagram or outline
the discovery and description of the course of development of something; "the tracing of genealogies"
of or near the head end or toward the front plane of a body
earlier in time (同)prior

PrepTutorEJDIC

広大な土地(地域),(土地・海・空などの)広がり《+of+名》 / (器官の)管,(神経の)索
(おもに宗教・政治などの宣伝用の)小冊子,パンフレット
跡を追うこと,追跡;透写,複写 / 〈C〉 / 透写(複写)によってできたもの(地図・図案など),透写図
(場所などが)前の,前部の;(…より)前に位置する《+『to』+『名』》 / (時・事件などが)以前の,先の;(…より)前の《+『to』+『名』》
神経単位,神経細胞,ニューロン,ノイロン

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1. 女性の骨盤臓器脱：後膣壁脱の外科的修復 pelvic organ prolapse in women surgical repair of anterior vaginal wall prolapse
2. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の診断 diagnosis of amyotrophic lateral sclerosis and other forms of motor neuron disease
3. 筋萎縮性側索硬化症およびその他の運動ニューロン疾患の臨床的特徴 clinical features of amyotrophic lateral sclerosis and other forms of motor neuron disease
4. 筋萎縮性側索硬化症の疫学および病因 epidemiology and pathogenesis of amyotrophic lateral sclerosis
5. ポリオおよび前角細胞への感染性疾患 polio and infectious diseases of the anterior horn

English Journal

An autopsy case of sporadic amyotrophic lateral sclerosis associated with the I113T SOD1 mutation.

Nakamura S, Wate R, Kaneko S, Ito H, Oki M, Tsuge A, Nagashima M, Asayama S, Fujita K, Nakamura M, Maruyama H, Kawakami H, Kusaka H.Author information Department of Neurology, Kansai Medical University, Hirakata, Japan.AbstractA 64-year-old man noticed weakness in his arms and dyspnea upon exertion. Four months later he was admitted to our hospital, where muscle atrophy and hyperactive deep tendon reflexes in the arms were observed upon examination. A needle electromyograph study revealed acute and chronic denervation in the extremities, and he was diagnosed as having amyotrophic lateral sclerosis (ALS). Seven months after onset of the disease, he died of respiratory failure. Neuropathologically, neuronal cell loss was observed in the motor cortex, hypoglossal nuclei, cervical and lumbar anterior horns and Clarke's nuclei. Some of the remaining neurons contained neurofilamentous conglomerate inclusions (CIs). A small number of Lewy body-like hyaline inclusions (LBHIs) were also observed. No the Bunina bodies, skein-like inclusions or basophilic inclusions were detectable. Tract degeneration was moderate in the dorsal and ventral spinocerebellar tracts, mild in the pyramidal tract, but not discerned in the posterior column. Immunohistochemical examinations revealed that the CIs were strongly positive for phosphorylated neurofilament and moderately positive for ubiquitin and Cu/Zn superoxide dismutase 1 (SOD1). Moreover, a number of phosphorylated tau protein-positive globose neurofibrillary tangles (NFTs) and threads were observed in the periaqueductal gray matter, oculomotor nuclei and trochlear nuclei. Although the family history was negative for neuromuscular diseases, the neuropathological findings indicated features of familial ALS with a SOD1 mutation. In fact, DNA analysis of frozen-brain tissue revealed the presence of the I113T SOD1 mutation. This case represents the first one of this mutation in a patient who showed CIs as well as LBHIs in the motor neurons at the same time, in addition to the NFTs in the mesencephalic tegmentum.
Neuropathology : official journal of the Japanese Society of Neuropathology.Neuropathology.2013 Jun 17. doi: 10.1111/neup.12049. [Epub ahead of print]
A 64-year-old man noticed weakness in his arms and dyspnea upon exertion. Four months later he was admitted to our hospital, where muscle atrophy and hyperactive deep tendon reflexes in the arms were observed upon examination. A needle electromyograph study revealed acute and chronic denervation in
PMID 23773010

Spinocerebellar ataxia type 7: clinical course, phenotype-genotype correlations, and neuropathology.

Horton LC, Frosch MP, Vangel MG, Weigel-DiFranco C, Berson EL, Schmahmann JD.Author information Ataxia Unit, Cognitive and Behavioral Neurology Unit, Laboratory for Neuroanatomy and Cerebellar Neurobiology, Department of Neurology, Massachusetts General Hospital and Harvard Medical School, Suite 340, Charles River Plaza South, 175 Cambridge Street, Boston, MA 02114, USA.AbstractSpinocerebellar ataxia type 7 is a neurodegenerative polyglutamine disease characterized by ataxia and retinal degeneration. The longitudinal course is unknown, and relationships between repeat expansion, clinical manifestations, and neuropathology remain uncertain. We followed 16 affected individuals of a 61-member kindred over 27 years with electroretinograms, neurological examinations including the Brief Ataxia Rating Scale, neuroimaging in five, and autopsy in four cases. We identified four stages of the illness: Stage 0, gene-positive but phenotypically silent; Stage 1, no symptoms, but hyperreflexia and/or abnormal electroretinograms; Stage 2, symptoms and signs progress modestly; and Stage 3, rapid clinical progression. CAG repeat length correlated inversely with age of onset of visual or motor signs (r = -0.74, p = 0.002). Stage 3 rate of progression did not differ between cases (p = 0.18). Electroretinograms correlated with Brief Ataxia Rating Scale score and were a biomarker of disease onset and progression. All symptomatic patients developed gait ataxia, extremity dysmetria, dysarthria, dysrhythmia, and oculomotor abnormalities. Funduscopy revealed pale optic discs and pigmentary disturbances. Visual acuity declined to blindness in those with longer CAG expansions. Hyperreflexia was present from Stage 1 onwards. Restless legs syndrome and sensory impairment were common. Neuropathological hallmarks were neuronal loss in cerebellar cortex, deep cerebellar nuclei, inferior olive, and anterior horns of the spinal cord, and axonal loss in spinocerebellar tracts, dorsal nerve roots, and posterior columns. Retinal pathology included photoreceptor degeneration and disruption of retinal pigment epithelium. Spinocerebellar ataxia type 7 evolves through four clinical stages; neuropathological findings underlie the clinical presentation; electroretinograms are a potential biomarker of disease progression.
Cerebellum (London, England).Cerebellum.2013 Apr;12(2):176-93. doi: 10.1007/s12311-012-0412-4.
Spinocerebellar ataxia type 7 is a neurodegenerative polyglutamine disease characterized by ataxia and retinal degeneration. The longitudinal course is unknown, and relationships between repeat expansion, clinical manifestations, and neuropathology remain uncertain. We followed 16 affected individua
PMID 22915085

Early-onset LBSL: how severe does it get?

Steenweg ME, van Berge L, van Berkel CG, de Coo IF, Temple IK, Brockmann K, Mendonça CI, Vojta S, Kolk A, Peck D, Carr L, Uziel G, Feigenbaum A, Blaser S, Scheper GC, van der Knaap MS.Author information Department of Child Neurology, VU University Medical Center, Amsterdam, The Netherlands.AbstractAIM: Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is known as a relatively mild leukoencephalopathy. We investigated the occurrence of severe variants of LBSL with extensive brain magnetic resonance imaging (MRI) abnormalities.
Neuropediatrics.Neuropediatrics.2012 Dec;43(6):332-8. doi: 10.1055/s-0032-1329395. Epub 2012 Oct 12.
AIM: Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL) is known as a relatively mild leukoencephalopathy. We investigated the occurrence of severe variants of LBSL with extensive brain magnetic resonance imaging (MRI) abnormalities.METHOD: MRIs of approximat
PMID 23065766

Japanese Journal

近位筋優位遺伝性運動感覚ニューロパチー（HMSN-P）の神経病理

吉田眞理
臨床神経学 53(11), 1200-1202, 2013
Proximal-dominant hereditary motor and sensory neuropathy（HMSN-P）である滋賀家系の神経病理所見では，末梢神経の有髄線維の高度脱落に加えて，下位運動ニューロン優位の運動ニューロン疾患の病理像を示した．脊髄前角の高度の細胞脱落とグリオーシス，ubiquitin陽性，TDP-43陽性，TFG陽性封入体の出現，後索中間根帯-Clarke柱-脊 …
NAID 130004505386

ラットの小脳前葉と後葉へ投射する脊髄小脳路細胞の定量的考察　　ＨＲＰの逆行性標識法による研究:HRPの逆行性標識法による研究

北村泰子
日本医科大学雑誌 55(4), 361-370, 1988
… of neurons sending their axons into the anterior and posterior cerebellar lobes and to analyse quantitatively the number of spinocerebellar tract neurons, horseradish peroxidase (HRP)-labeled neurons were examined throughout the length of the spinal cord after HRP injection into the anterior or posterior cerebellar lobe.<BR>The distribution areas of labeled neurons sending their axons into the anterior and posterior cerebellar lobes were …
NAID 130003753026