若年性進行性球麻痺
WordNet
- gradually advancing in extent
- a tense of verbs used in describing action that is on-going (同)progressive tense, imperfect, imperfect tense, continuous tense
- favoring or promoting reform (often by government action) (同)reformist, reform-minded
- (of a card game or a dance) involving a series of sections for which the participants successively change place or relative position; "progressive euchre"; "progressive tournaments"
- (of taxes) adjusted so that the rate increases as the amount of income increases
- advancing in severity; "progressive paralysis"
- favoring or promoting progress; "progressive schools"
- a modified bud consisting of a thickened globular underground stem serving as a reproductive structure
- anything with a round shape resembling a teardrop
- a rounded part of a cylindrical instrument (usually at one end); "the bulb of a syringe"
- a rounded dilation or expansion in a canal or vessel or organ
- of or relating to or characteristic of or appropriate for children or young people; "juvenile diabetes"; "juvenile fashions"
- a young person, not fully developed (同)juvenile person
- involving the medulla oblongata; "bulbar paralysis"
- loss of the ability to move a body part (同)palsy
PrepTutorEJDIC
- (行列などが)『前進する』,進んで行く / (事態が)進展する,進行する / 『進歩的な』,革新的な / (病気・暴力などが)次第に悪くなる(広がる) / (課税が)累進的な / (文法で)進行[形]の / 進歩的な人,革新主義者
- 『球根』(ユり・タマネギなどの地下茎),りん(鱗)茎,球茎 / 球根状のもの;電球,真空管
- 少年少女向きの / 少年の,少女の,若い / 子供じみた,未熟な / 少年,少女 / 子役の俳優
- (体の)麻痺(まひ),中風 / (…の)停滞《+『of』+『名』》
UpToDate Contents
全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.
- 1. 脊髄性筋萎縮症spinal muscular atrophy [show details]
…most common cause of acute flaccid paralysis in healthy infants and children. GBS has several variant forms. The classic presentation of GBS is one of ascending paralysis with progressive, mostly symmetric …
- 2. 消失性白質疾患(Vanishing white matter disease)vanishing white matter disease [show details]
… occur. Most affected children die within a few years after onset, although some have lived only a few months, while others have survived for several decades . The late childhood/juvenile form presents between …
- 3. 筋萎縮性側索硬化症への疾患修飾療法disease modifying treatment of amyotrophic lateral sclerosis [show details]
…(100 mg/day) compared with controls (74 versus 58 percent) . For the subset of patients with bulbar-onset ALS, an even greater advantage for survival at 12 months emerged for the riluzole group (73 versus…
- 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 [show details]
… a mouse model of ALS halted neuronal loss and prevented axonal degeneration, symptom onset, and paralysis . In addition, survival was extended. These findings suggest that inhibition of apoptosis is a possible…distinguishable from other MNDs such as primary lateral sclerosis, primary muscular atrophy, and progressive bulbar palsy, based upon the location of first symptom and the extent to which anterior horn cells or … a disease of neonatal or childhood onset characterized by degeneration of the anterior…
- 5. 筋電図検査の概要overview of electromyography [show details]
… dystrophy, myotonia congenita, paramyotonia congenita), Schwartz-Jampel syndrome, hyperkalemic periodic paralysis, and glycogen storage diseases (most often glycogen storage disease II or Pompe disease) . Both … One study in children found needle EMG pain to be equivalent to that of a venipuncture , while others recommend topical anesthetic use in children .…
English Journal
- Niemann-Pick disease type C1 predominantly involving the frontotemporal region, with cortical and brainstem Lewy bodies: an autopsy case.
- Chiba Y1, Komori H, Takei S, Hasegawa-Ishii S, Kawamura N, Adachi K, Nanba E, Hosokawa M, Enokido Y, Kouchi Z, Yoshida F, Shimada A.
- Neuropathology : official journal of the Japanese Society of Neuropathology.Neuropathology.2014 Feb;34(1):49-57. doi: 10.1111/neup.12047. Epub 2013 May 27.
- Niemann-Pick disease type C (NPC) is an autosomal recessive neurovisceral lipid storage disorder. Two disease-causing genes (NPC1 and NPC2) have been identified. NPC is characterized by neuronal and glial lipid storage and NFTs. Here, we report a man with juvenile-onset progressive neurological defi
- PMID 23711246
- The first ALS2 missense mutation associated with JPLS reveals new aspects of alsin biological function.
- Panzeri C1, De Palma C, Martinuzzi A, Daga A, De Polo G, Bresolin N, Miller CC, Tudor EL, Clementi E, Bassi MT.
- Brain : a journal of neurology.Brain.2006 Jul;129(Pt 7):1710-9. Epub 2006 May 2.
- Primary lateral sclerosis (PLS) is a rare progressive paralytic disorder that results from dysfunction of the upper motoneurons. Although PLS is a sporadic disorder of adult middle age, it has also been described in children as juvenile PLS or JPLS. The causative gene for JPLS was found to be ALS2,
- PMID 16670179
- Brown-Vialetto-Van Laere syndrome; variability in age at onset and disease progression highlighting the phenotypic overlap with Fazio-Londe disease.
- Dipti S1, Childs AM, Livingston JH, Aggarwal AK, Miller M, Williams C, Crow YJ.
- Brain & development.Brain Dev.2005 Sep;27(6):443-6. Epub 2004 Dec 15.
- We report four siblings showing features of a pontobulbar palsy, a mixed spinal and upper motor neuropathy and variable deafness. The observation of affected males and females born to consanguineous first cousin parents suggests autosomal recessive inheritance. Two children presented in the first 16
- PMID 16122634
★リンクテーブル★
[★]
- 英
- juvenile progressive bulbar paralysis
- 同
- ファツィオ・ロンド病 Fazio-Londe disease
[★]
- 関
- eyeball、globe、medullary、ocular
- 関
- medulla oblongata 延髄
[★]
- 関
- juvenilis、young
[★]
- 関
- ball、globe、globus、sphere、olfactory bulb
[★]
- 関
- progressively