WordNet
- a condition marked by uncontrollable tremor
- affect with palsy
- become friends; act friendly towards (同)pal up, chum up
PrepTutorEJDIC
- 麻痺(まひ),中風 / …‘を'麻痺させる(paralyze)
- 《話》『友達』,仲間,親友 / (…と)仲よくなる《[『up』]『with』+『名』》
Wikipedia preview
出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/01/17 13:04:23」(JST)
wiki en
| Progressive supranuclear palsy | |
|---|---|
|
Main anatomical plans and axes applied to the sections of the brain.
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| Classification and external resources | |
| Specialty | neurology |
| ICD-10 | G23.1 |
| ICD-9-CM | 333.0 |
| OMIM | 601104 |
| DiseasesDB | 10723 |
| MedlinePlus | 000767 |
| eMedicine | neuro/328 |
| Patient UK | Progressive supranuclear palsy |
| MeSH | D013494 |
Progressive supranuclear palsy (PSP; or the Steele-Richardson-Olszewski syndrome, after the physicians who described it in 1963) is a degenerative disease involving the gradual deterioration and death of specific volumes of the brain.[1][2]
Males and females are affected approximately equally and there is no racial, geographical or occupational predilection. Approximately 6 people per 100,000 population have PSP.
It has been described as a tauopathy.[3]
Contents
- 1 Symptoms and signs
- 2 Differential diagnosis
- 3 Cause
- 4 Pathophysiology
- 5 Classification and treatment
- 5.1 Rehabilitation
- 6 Prognosis
- 7 Notable cases
- 8 Support groups
- 9 References
- 10 External links
Symptoms and signs
This patient presented with progressive dementia, ataxia and incontinence. A clinical diagnosis of normal pressure hydrocephalus was entertained. Imaging did not support this, however, and on formal testing abnormal nystagmus and eye movements were detected. A sagittal view of the CT/MRI scan shows atrophy of the midbrain, with preservation of the volume of the pons. This appearance has been called the "Hummingbird sign" or "Penguin sign". There is also atrophy of the tectum, particularly the superior colliculi. These findings suggest the diagnosis of progressive supranuclear palsy.[4]
The initial symptoms in two-thirds of cases are loss of balance, lunging forward when mobilizing, fast walking, bumping into objects or people, and falls.
Other common early symptoms are changes in personality, general slowing of movement, and visual symptoms.
Later symptoms and signs are dementia (typically including loss of inhibition and ability to organize information), slurring of speech, difficulty swallowing, and difficulty moving the eyes, particularly in the vertical direction. The latter accounts for some of the falls experienced by these patients as they are unable to look up or down.
Some of the other signs are poor eyelid function, contracture of the facial muscles, a backward tilt of the head with stiffening of the neck muscles, sleep disruption, urinary incontinence and constipation.
The visual symptoms are of particular importance in the diagnosis of this disorder. Patients typically complain of difficulty reading due to the inability to look down well. Notably, the ophthalmoparesis experienced by these patients mainly concerns voluntary eye movement and the inability to make vertical saccades which is often worse with downward saccades. Patients tend to have difficulty looking down (a downgaze palsy) followed by the addition of an upgaze palsy. This vertical gaze paresis will correct when the examiner passively rolls the patient's head up and down in what is known as an oculocephalic maneuver. Involuntary eye movement, as elicited by Bell's phenomenon, for instance, may be closer to normal. On close inspection, eye movements called "square-wave jerks" may be visible when the patient fixes at distance. These are fine movements, that can be mistaken for nystagmus, except that they are saccadic in nature, with no smooth phase. Difficulties with convergence (convergence insufficiency), where the eyes come closer together while focusing on something near, like the pages of a book, is typical. Because the eyes have trouble coming together to focus at short distances, the patient may complain of diplopia (double vision) when reading.
Cardinal manifestations:
- Supranuclear ophthalmoplegia
- Neck dystonia
- Parkinsonism
- Pseudobulbar palsy
- Behavioral and cognitive impairment
- Imbalance and walking difficulty
- Frequent falls
Differential diagnosis
PSP is frequently misdiagnosed as Parkinson's disease because of the slowed movements and gait difficulty, or as Alzheimer's disease because of the behavioral changes. It is one of a number of diseases collectively referred to as Parkinson plus syndromes. A poor response to levodopa along with symmetrical onset can help differentiate this disease from PD.[5] Also, patients with the Richardson variant tend to have an upright or arched-back posture as opposed to the stooped-forward posture of other Parkinsonian disorders, although PSP-Parkinsonism (see below) may show the stooped posture.[6] Early falls are characteristic, especially with Richardson-syndrome.[7]
Cause
The cause of PSP is unknown. Fewer than 1 percent of those with PSP have a family member with the same disorder. A variant in the gene for tau protein called the H1 haplotype, located on chromosome 17, has been linked to PSP.[8] Nearly all people with PSP received a copy of that variant from each parent, but this is true of about two-thirds of the general population. Therefore, the H1 haplotype appears to be necessary but not sufficient to cause PSP. Other genes, as well as environmental toxins, are being investigated as other possible contributors to the cause of PSP.
Pathophysiology
The affected brain cells are both neurons and glial cells. The neurons display neurofibrillary tangles, which are clumps of tau protein, a normal part of a brain cell's internal structural skeleton. These tangles are often different from those seen in Alzheimer's disease, but may be structurally similar when they occur in the cerebral cortex.[9] Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration.[10] Lewy bodies are seen in some cases, but it is not clear whether this is a variant or an independent co-existing process, and in some cases PSP can coexist with corticobasal degeneration, Parkinson's and/or Alzheimer's Disease, particularly with older patients.[11][12][13][14][15]
The principal areas of the brain affected are:
- the basal ganglia, particularly the subthalamic nucleus, substantia nigra and globus pallidus;
- the brainstem, particularly the portion of the midbrain where "supranuclear" eye movement resides;
- the cerebral cortex, particularly that of the frontal lobes;
- the dentate nucleus of the cerebellum;
- and the spinal cord, particularly the area where some control of the bladder and bowel resides.
Some consider PSP, corticobasal degeneration, and frontotemporal dementia to be variations of the same disease.[16][17] Others consider them separate diseases.[18][19][20] PSP has been shown occasionally to co-exist with Pick's disease.[21]
Classification and treatment
| This section requires expansion. (November 2010) |
PSP cases are often split into two subgroups, PSP-Richardson, the classic type, and PSP-Parkinsonism, where a short-term response to levodopa can be obtained.[22] Amantadine is also sometimes helpful.[23] After a few years the Parkinsonian variant tends to take on Richardson features.[24] Other variants have been described.[25][26][27] Botox can be used to treat neck dystonia and blephrospasm, but this can aggravate dysphagia.[28]
Two studies have suggested that rivastigmine may help with cognitive aspects, but the authors of both studies have suggested a larger sampling be used.,[29][30] There is some evidence that the hypnotic zolpidem may improve motor function and eye movements, but only from small-scale studies.[31][32]
Rehabilitation
Patients with PSP usually seek or are referred to occupational therapy, speech-language pathology for motor speech changes typically a spastic-ataxic dysarthria, and physical therapy for balance and gait problems with reports of frequent falls.[33] Evidence-based approaches to rehabilitation in PSP are lacking, and currently the majority of research on the subject consists of case reports involving only a small number of patients.
Case reports of rehabilitation programs for patients with PSP generally include limb-coordination activities, tilt-board balancing, gait training, strength training with progressive resistive exercises and isokinetic exercises and stretching of the neck muscles.[33] While some case reports suggest that physiotherapy can offer improvements in balance and gait of patients with PSP, the results cannot be generalized across all patients with PSP as each case report only followed one or two patients.[33] The observations made from these case studies can be useful, however, in helping to guide future research concerning the effectiveness of balance and gait training programs in the management of PSP.
Individuals with PSP are often referred to occupational therapists to help manage their condition and to help enhance their independence. This may include being taught to use mobility aids.[34][35] Due to their tendency to fall backwards, the use of a walker, particularly one that can be weighted in the front, is recommended over a cane.[34] The use of an appropriate mobility aid will help to decrease the individual’s risk of falls and make them safer to ambulate independently in the community.[35] Due to their balance problems and irregular movements individuals will need to spend time learning how to safely transfer in their homes as well as in the community.[34] This may include arising from and sitting in chairs safely.[35]
Due to the progressive nature of this disease, all individuals eventually lose their ability to walk and will need to progress to using a wheelchair.[34] Severe dysphagia often follows, and at this point death is often a matter of months.[36]
Prognosis
There is currently no effective treatment or cure for PSP, although some of the symptoms can respond to nonspecific measures. The average age at symptoms onset is 63 and survival from onset averages 7 years with a wide variance. Pneumonia is a frequent cause of death.[37]
Notable cases
- Nobel Laureate Dr Abdus Salam (1926–1996), a world-renowned scientist and first and only Muslim to win the Nobel Prize in Physics (1979) for his gauge unification of weak and electromagnetic interactions, which forms the basis of the Standard Model in particle physics.
- Teel Bivins (1947–2009), a former U.S. Ambassador to Sweden and a former member of the Texas State Senate from Amarillo, Texas, died at the age of sixty-one of progressive supranuclear palsy, first diagnosed in 2006, while he was in Stockholm, Sweden.[38]
- Leonard Krieger (1918–1990), was an American historian of modern Europe, particularly known as an author on Germany. He was influential as an intellectual historian, and particularly for his discussion of historicism. Krieger taught at Columbia University and The University of Chicago until his death from PSP.
- Abel John "Buddy" McNamara, died December 2, 2014 at the age of 78 after a long battle with supranuclear palsy. From 1976 to 1980, he was a member of the Louisiana House of Representatives; from 1982 to 2001, he was a judge of the United States District Court for the Eastern District of Louisiana in his native New Orleans.[39]
- Musician/actor Dudley Moore, who suffered from progressive supranuclear palsy, increased public awareness of this disease. He died on March 27, 2002 at the age of 66 from its complications.
- Country singer Teddy Wilburn, of The Wilburn Brothers, was diagnosed with PSP in the late 1990s. He died of congestive heart failure, and PSP complications, on November 24, 2003.
- Lee Philips, the 1950s actor-turned-director of such shows as Peyton Place and The Ghost & Mrs. Muir, also died after suffering from this disease.
- American singer Teresa Brewer ("Music! Music! Music!"), 76, died of this disease on October 17, 2007.
- Film director and playwright Joshua Logan
- Abe Pollin, D.C. sports mogul, longest tenured owner of an NBA franchise (since 1964) Washington Wizards, 85, died of this disease on November 24, 2009.
- Dr. Anne Turner, who was the basis of the BBC drama A Short Stay in Switzerland about assisted suicide.[40]
- Jon Hassler, author
- Bob Gibson (musician), influential American singer-songwriter of the 1950s and 1960s, died September 28, 1996, age 64.
- Richard Rainwater, investor[41]
- Geoff Miller, Co-founder, editor, and publisher of Los Angeles Magazine, 1936–2011
- K. Lamar Alsop, concertmaster of the New York City Ballet Orchestra.[42]
- John Attenborough, executive at Alfa Romeo and younger brother of Sir David Attenborough[43]
- Actor Phyllis Frelich, She died on April 10, 2014 at age 70.
- Musician Jeff Golub, died on January 1, 2015 at age 59.
- Musician, Peter Sarstedt, initially diagnosed with dementia, was diagnosed with PSP in 2015
- Richard Frank, past leader of nonprofit Population Services International and first husband of Jane Harman, died 20 April 2014, age 77[44]
- Eric Jansson, Chess master and musician.[45]
Support groups
Several international organizations serve the needs of patients with PSP and their families and support research. The Foundation for PSP, CBD and Related Brain Diseases is based in the US and the PSP Association (PSP-Europe Association) is based in the UK. The PSP-France association is based in Paris. With the help of the PSP Association based in the United States, in 2014/15 Canada will have its own CUREPSP organization.
References
- ^ Richardson JC, Steele J, Olszewski J (1963). "Supranuclear ophthalmoplegia, pseudobulbar palsy, nuchal dystonia and dementia. A clinical report on eight cases of 'heterogeneous system degeneration'". Transactions of the American Neurological Association 88: 25–9. PMID 14272249.
- ^ Steele JC, Richardson JC, Olszewski J (April 1964). "Progressive supranuclear palsy: a heterogeneous degeneration involving brain stem, basal ganglia and cerebellum with vertical gaze and pseudobulbar palsy, nuchal dystonia and dementia". Archives of Neurology 10: 333–59. doi:10.1001/archneur.1964.00460160003001. PMID 14107684.
- ^ Rizzo G, Martinelli P, Manners D, et al. (October 2008). "Diffusion-weighted brain imaging study of patients with clinical diagnosis of corticobasal degeneration, progressive supranuclear palsy and Parkinson's disease". Brain 131 (Pt 10): 2690–700. doi:10.1093/brain/awn195. PMID 18819991.
- ^ http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2812742/
- ^ Litvan I, Campbell G, Mangone CA, Verny M, McKee A, Chaudhuri KR, Jellinger K, Pearce RK, D'Olhaberriague L. (Jan 1997). "Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders? A clinicopathological study". Brain. 120 (1): 65–74. doi:10.1093/brain/120.1.65. PMID 9055798.
- ^ David P. Moore, Basant K. Puri. Textbook of Clinical Neuropsychiatry and Behavioral Neuroscience. pp. 400–401. ISBN 9781444164947.
- ^ Williams DR1, Watt HC, Lees AJ. (April 2006). "Predictors of falls and fractures in bradykinetic rigid syndromes: a retrospective study". J Neurol Neurosurg Psychiatry 77 (4): 468–73. doi:10.1136/jnnp.2005.074070. PMID 16543524.
- ^ Online 'Mendelian Inheritance in Man' (OMIM) 601104
- ^ Amano N, Iwabuchi K, Yokoi S (January 1989). "[The reappraisal study of the ultrastructure of Alzheimer's neurofibrillary tangles in three cases of progressive supranuclear palsy]". Nō to Shinkei (in Japanese) 41 (1): 35–44. PMID 2655673.
- ^ Luc Buée; André Delacourte (1999). "Comparative biochemistry of tau in progressive supranuclear palsy, corticobasal degeneration, FTDP-17 and Pick's disease" (PDF). Brain Pathology 9 (4): 681–93. doi:10.1111/j.1750-3639.1999.tb00550.x. PMID 10517507.
- ^ Uchikado H, DelleDonne A, Ahmed Z, Dickson DW (April 2006). "Lewy bodies in progressive supranuclear palsy represent an independent disease process". Journal of Neuropathology and Experimental Neurology 65 (4): 387–95. doi:10.1097/01.jnen.0000218449.17073.43. PMID 16691119.
- ^ Keith-Rokosh J, Ang LC (November 2008). "Progressive supranuclear palsy: a review of co-existing neurodegeneration". The Canadian Journal of Neurological Sciences 35 (5): 602–8. PMID 19235444.
- ^ Heather B. Rigby MD, Brittany N. Dugger PhD, Joseph G. Hentz MS, Charles H. Adler MD, PhD1, Thomas G. Beach MD, PhD, Holly A. Shill MD, Erika Driver-Dunckley MD, Marwan N. Sabbagh MD, Lucia I. Sue2 andJohn N. Caviness MD (March 2015). "Clinical Features of Patients with Concomitant Parkinson's Disease and Progressive Supranuclear Palsy Pathology". Movement Disorders Clinical Practice 2 (1): 33–38. doi:10.1002/mdc3.12104.
- ^ Gearing M1, Olson DA, Watts RL, Mirra SS. (June 1994). "Progressive supranuclear palsy: neuropathologic and clinical heterogeneity". Neurology 44 (6): 1015–24. doi:10.1212/wnl.44.6.1015. PMID 8208392.
- ^ Brittany N. Dugger,* Charles H. Adler, Holly A. Shill, John Caviness, Sandra Jacobson, Erika Driver-Dunckley, Thomas G. Beach, and the Arizona Parkinson’s Disease Consortium (May 2014). "Concomitant pathologies among a spectrum of parkinsonian disorders.". Parkinsonism Relat Disord. 20 (5): 525–9. doi:10.1016/j.parkreldis.2014.02.012. PMC 4028418. PMID 24637124.
- ^ Kertesz A, Munoz D (2004). "Relationship between frontotemporal dementia and corticobasal degeneration/progressive supranuclear palsy". Dementia and Geriatric Cognitive Disorders 17 (4): 282–6. doi:10.1159/000077155. PMID 15178937.
- ^ Katsuse, O; Iseki, E; Arai, T; Akiyama, H; Togo, T; Uchikado, H; Kato, M; Andrew Lees; et al. (September 2003). "4-repeat tauopathy sharing pathological and biochemical features of corticobasal degeneration and progressive supranuclear palsy". Acta Neuropathologica 106 (3): 251–60. doi:10.1007/s00401-003-0728-8. PMID 12802605.
- ^ Hattori M, Hashizume Y, Yoshida M (August 2003). "Distribution of astrocytic plaques in the corticobasal degeneration brain and comparison with tuft-shaped astrocytes in the progressive supranuclear palsy brain". Acta Neuropathologica 106 (2): 143–9. doi:10.1007/s00401-003-0711-4. PMID 12732936.
- ^ Komori, T; Arai, N; Oda, M; Nakayama, H; Mori, H; Yagishita, S; Takahashi, T; Komori T, Arai N, Oda M, Nakayama H, Mori H, Yagishita S, Takahashi T, Amano N, Murayama S, Murakami S, Shibata N, Kobayashi M, Sasaki S, Iwata M.; et al. (Oct 1998). "Astrocytic plaques and tufts of abnormal fibers do not coexist in corticobasal degeneration and progressive supranuclear palsy". Acta Neuropathologica 96 (4): 401–8. doi:10.1007/s004010050911. PMID 9797005.
- ^ "[Glial abnormalities in progressive supranuclear palsy and corticobasal degeneration].". Zhonghua Bing Li Xue Za Zhi 33: 125–9. Apr 2004. PMID 15132848.
- ^ Wang, LN; Zhu, MW; Feng, YQ; Wang, JH (2006). "Pick's disease with Pick bodies combined with progressive supranuclear palsy without tuft-shaped astrocytes: a clinical, neuroradiologic and pathological study of an autopsied case". Neuropathology : official journal of the Japanese Society of Neuropathology 26 (3): 222–30. doi:10.1111/j.1440-1789.2006.00671.x. PMID 16771179.
- ^ Williams, DR; De Silva, R; Paviour, DC; Pittman, A; Watt, HC; Kilford, L; Holton, JL; Williams DR, de Silva R, Paviour DC, Pittman A, Watt HC, Kilford L, Holton JL, Revesz T, Lees AJ.; Lees, AJ (Jun 2005). "Characteristics of two distinct clinical phenotypes in pathologically proven progressive supranuclear palsy: Richardson's syndrome and PSP-parkinsonism". Brain 128 (6): 1247–58. doi:10.1093/brain/awh488. PMID 15788542.
- ^ Brooks DJ. (March 2002). "Diagnosis and management of atypical parkinsonian syndromes.". J Neurol Neurosurg Psychiatry 72 (Suppl): 1:I10–I16. PMC 1765580. PMID 11870198.
- ^ What is Progressive Supranuclear Palsy?
- ^ Orphanet article on Progressive Supranuclear Palsy
- ^ What's New in Progressive Supranuclear Palsy?
- ^ NORD article on PSP
- ^ Barsottini OG1, Felício AC, Aquino CC, Pedroso JL. (December 2010). "Progressive supranuclear palsy: new concepts.". Arq Neuropsiquiatr. 68 (6): 938–46. doi:10.1590/s0004-282x2010000600020. PMID 21243256.
- ^ Nijboer H, Dautzenberg PL. (Jun 2009). "[Progressive supranucleair palsy: acetylcholineeserase-inhibitor a possible therapy?]". Tijdschr Gerontol Geriatr. 40 (3): 133–7. PMID 19731749.
- ^ Liepelt I, Gaenslen A, Godau J, Di Santo A, Schweitzer KJ, Gasser T, Berg D. (Jan 2010). "Rivastigmine for the treatment of dementia in patients with progressive supranuclear palsy: Clinical observations as a basis for power calculations and safety analysis". Alzheimers Dement. 6 (1): 70–4. doi:10.1016/j.jalz.2009.04.1231. PMID 20129321.
- ^ Abe, K (January 2008). "Zolpidem therapy for movement disorders.". Recent patents on CNS drug discovery 3 (1): 55–60. doi:10.2174/157488908783421519. PMID 18221242.
- ^ Barsottini, OG; Felício, AC; Aquino, CC; Pedroso, JL (December 2010). "Progressive supranuclear palsy: new concepts.". Arquivos de neuro-psiquiatria 68 (6): 938–46. doi:10.1590/S0004-282X2010000600020. PMID 21243256.
- ^ a b c Zampieri, Cris; Di Fabio, Richard (June 2006). "Progressive Supranuclear Palsy: Disease Profile and Rehabilitation Strategies". Physical Therapy 86 (6): 870–80. PMID 16737412.
- ^ a b c d van Balken, Irene; Litvan, Irene (May 2006). "Current and Future Treatments in Progressive Supranuclear Palsy". Current Treatment Options in Neurology 8 (3): 211–23. doi:10.1007/s11940-006-0012-z. PMID 16569380.
- ^ a b c Golbe, Lawrence (November 2001). "Progressive Supranuclear Palsy". Current Treatment Options in Neurology 3 (6): 473–477. doi:10.1007/s11940-001-0010-0. PMID 11581524.
- ^ O'Sullivan SS1, Massey LA, Williams DR, Silveira-Moriyama L, Kempster PA, Holton JL, Revesz T, Lees AJ. (May 2008). "Clinical outcomes of progressive supranuclear palsy and multiple system atrophy.". Brain 131 (5): 1362–72. doi:10.1093/brain/awn065. PMID 18385183.
- ^ Satoshi Tomita,1,2 Tomoko Oeda,1,2 Atsushi Umemura,1,2 Masayuki Kohsaka,1,2 Kwiyoung Park,1,2 Kenji Yamamoto,1,2 Hiroshi Sugiyama,2 Chiaki Mori,3 Kimiko Inoue,3 Harutoshi Fujimura,3 and Hideyuki Sawada1,2,* Oscar Arias-Carrion, Editor (August 13, 2015). "Impact of Aspiration Pneumonia on the Clinical Course of Progressive Supranuclear Palsy: A Retrospective Cohort Study.". PLOS ONE 10 (8): e0135823. doi:10.1371/journal.pone.0135823. PMC 4536232. PMID 26270456. line feed character in
|authors=at position 207 (help) - ^ "Teel Bivins Services Held Today in Amarillo, October 29, 2009". Texas Insider. Retrieved October 30, 2009.
- ^ "Retired U.S. District Court Judge A.J. "Buddy" McNamara, 78, died Tuesday". New Orleans Times-Picayune. Retrieved December 3, 2014.
- ^ "Dr Anne Turner". Dignity in Dying. Retrieved 2009-01-25.
- ^ Elkind, Peter; Sellers, Patricia; Burke, Doris (November 21, 2011). "The Fight of Richard Rainwater's Life". Fortune 164 (8): 126–140.
- ^ "K. Lamar Alsop, Violinist in a Musical Family, Dies at 85". Retrieved 2014-02-09.
- ^ "John Attenborough, youngest brother of David and Richard Attenborough (obituary)". Daily Echo (Bournemouth). 31 December 2012. Retrieved 8 December 2013.
- ^ Richard Frank obituary, Washington Post
- ^ http://textuploader.com/oiub
External links
- The Foundation for PSP | CBD and Related Brain Diseases ("CurePSP")
- The PSP Association (UK)
- psp at NINDS
- Medical Notes at BBC
- Houston PSP Review at Baylor College
- 02040 at CHORUS
- synd/3806 at Who Named It?
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UpToDate Contents
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- 1. 進行性核上性麻痺（PSP） progressive supranuclear palsy psp
- 2. 小児における核上性注視異常 supranuclear disorders of gaze in children
- 3. 成人における詳細な神経学的検査 the detailed neurologic examination in adults
- 4. 核間性眼筋麻痺 internuclear ophthalmoparesis
- 5. 大脳皮質基底核変性症 corticobasal degeneration
English Journal
- Are postural responses to backward and forward perturbations processed by different neural circuits?
- Nonnekes J, Scotti A, Oude Nijhuis LB, Smulders K, Queralt A, Geurts AC, Bloem BR, Weerdesteyn V.SourceRadboud University Medical Centre, Nijmegen Centre for Evidence Based Practice, Department of Rehabilitation, Nijmegen, The Netherlands. Electronic address: j.nonnekes@reval.umcn.nl.
- Neuroscience.Neuroscience.2013 Aug 15;245:109-20. doi: 10.1016/j.neuroscience.2013.04.036. Epub 2013 Apr 24.
- Startle pathways may contribute to rapid accomplishment of postural stability. Here we investigate the possible influence of a startling auditory stimulus (SAS) on postural responses. We formulated four specific questions: (1) can a concurrent SAS shorten the onset of automatic postural responses ;
- PMID 23624061
- Perry syndrome: A disorder to consider in the differential diagnosis of Parkinsonism.
- Aji BM, Medley G, O'Driscoll K, Larner AJ, Alusi SH.SourceWalton Centre for Neurology and Neurosurgery, Liverpool, United Kingdom.
- Journal of the neurological sciences.J Neurol Sci.2013 Jul 15;330(1-2):117-8. doi: 10.1016/j.jns.2013.04.008. Epub 2013 Apr 28.
- A patient with a mood disorder and a Parkinsonian syndrome with frontal cognitive impairment thought to resemble progressive supranuclear palsy defied precise diagnosis until the development of respiratory compromise, prompting consideration of the diagnosis of Perry syndrome. A mutation in the dyna
- PMID 23628468
- Variation in tau isoform expression in different brain regions and disease states.
- Majounie E, Cross W, Newsway V, Dillman A, Vandrovcova J, Morris CM, Nalls MA, Ferrucci L, Owen MJ, O'Donovan MC, Cookson MR, Singleton AB, de Silva R, Morris HR.SourceMRC Centre for Neuropsychatric Genetics and Genomics, Cardiff University School of Medicine, Cardiff, UK.
- Neurobiology of aging.Neurobiol Aging.2013 Jul;34(7):1922.e7-1922.e12. doi: 10.1016/j.neurobiolaging.2013.01.017. Epub 2013 Feb 19.
- Progressive supranuclear palsy (PSP) is the most common atypical parkinsonian disorder. Abnormal tau inclusions, in selected regions of the brain, are a hallmark of the disease and the H1 haplotype of MAPT, the gene encoding tau, is the major risk factor in PSP. A 3-repeat and 4-repeat (4R) tau isof
- PMID 23428180
Japanese Journal
- 進行性核上性麻痺および大脳皮質基底核変性症における精神症状 (特集 脳変性疾患の臨床神経病理学から精神症状を再考する)
- 老年精神医学雑誌 27(1), 42-50, 2016-01
- NAID 40020724442
- Disappearance of the Hummingbird Sign after Shunt Surgery in a Case of Idiopathic Normal Pressure Hydrocephalus
- Internal Medicine 55(7), 815-817, 2016
- NAID 130005141964
- Niemann-Pick病C型 (NPC):—病態と診断・治療のポイント—
- 脳と発達 48(2), 81-88, 2016
- NAID 130005140058
Related Links
- Progressive Supranuclear Palsy (PSP) information sheet compiled by the National Institute of Neurological Disorders and Stroke (NINDS). ... What is progressive supranuclear palsy? Progressive supranuclear palsy ...
- Progressive supranuclear palsy — Comprehensive overview covers symptoms and self-care of this Parkinson's disease-like condition. ... Progressive supranuclear palsy, also called Steele-Richardson-Olszewski syndrome, is an ...
Related Pictures
★リンクテーブル★
| リンク元 | 「核上性麻痺」 |
| 拡張検索 | 「progressive supranuclear palsy」 |
| 関連記事 | 「palsy」「supranuclear」 |
「核上性麻痺」
「progressive supranuclear palsy」
「palsy」
- n.
「supranuclear」
- adj.
- 核上の、核上性の