WordNet

a pattern of symptoms indicative of some disease
a complex of concurrent things; "every word has a syndrome of meanings"
United States architect (1838-1886) (同)Henry Hobson Richardson
British stage and screen actor noted for playing classic roles (1902-1983) (同)Ralph Richardson, Sir Ralph David Richardson
English literary critic who collaborated with C. K. Ogden and contributed to the development of Basic English (1893-1979) (同)I. A. Richards, Ivor Armstrong Richards
English writer (1672-1729) (同)Sir Richrd Steele

PrepTutorEJDIC

(疾患の徴候となる一群の)症徴候,症候群 / (事件・社会的状態などのパターンを示す)徴候形態

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/08/25 07:45:40」(JST)

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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]

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 Difficulties walking
Frequent falls

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.

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] Early falls are characteristic, especially with Richardson-syndrome. ^[6]

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.^[7] 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.^[8] Their chemical composition is usually different, however, and is similar to that of tangles seen in corticobasal degeneration.^[9] 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 Parkinson's Disease.^[10]^[11]^[12]

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.^[13]^[14] Others consider them separate diseases.^[15]^[16]^[17] PSP has been shown occasionally to co-exist with Pick's disease.^[18]

Classification and treatment

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.^[19] After a few years the Parkinsonian variant tends to take on Richardson features. ^[20] Other variants have been described.^[21] ^[22] ^[23] Botox can be used to treat neck dystonia and blephrospasm, but this can aggravate dysphagia.^[24]

Two studies have suggested that rivastigmine may help with cognitive aspects, but the authors of both studies have suggested a larger sampling be used.,^[25]^[26] There is some evidence that the hypnotic zolpidem may improve motor function and eye movements, but only from small-scale studies.^[27]^[28]

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.^[29] 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.^[29] 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.^[29] 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.^[30]^[31] 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.^[30] 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.^[31] 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.^[30] This may include arising from and sitting in chairs safely.^[31]

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.^[30]

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.^[32]
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.^[33]
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.^[34]
Jon Hassler, author
Bob Gibson (musician), influential American singer-songwriter of the 1950s and 1960s, died September 28, 1996, age 64.
Richard Rainwater, investor^[35]
Geoff Miller, Co-founder, editor, and publisher of Los Angeles Magazine, 1936–2011
K. Lamar Alsop, concertmaster of the New York City Ballet Orchestra.^[36]
John Attenborough, executive at Alfa Romeo and younger brother of Sir David Attenborough^[37]
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

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.
^ 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. 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.
^ 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].". 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.
^ 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. 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.
^ "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.

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?

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. 進行性核上性麻痺（PSP） progressive supranuclear palsy psp
2. 大脳皮質基底核変性症 corticobasal degeneration
3. パーキンソン病の診断 diagnosis of parkinson disease
4. 前頭側頭型認知症：臨床的特徴および診断 frontotemporal dementia clinical features and diagnosis
5. 多系統萎縮症：臨床的特徴および診断 multiple system atrophy clinical features and diagnosis

English Journal

Mass Spectrometric Analysis of Cerebrospinal Fluid Ubiquitin in Alzheimer's Disease and Parkinsonian Disorders.

Sjödin S1, Hansson O2,3, Öhrfelt A1, Brinkmalm G1, Zetterberg H1,4,5,6, Brinkmalm A1,4, Blennow K1,4.
Proteomics. Clinical applications.Proteomics Clin Appl.2017 Oct 3. doi: 10.1002/prca.201700100. [Epub ahead of print]
PMID 28972305

Probing Conformational Dynamics of Tau Protein by Hydrogen/Deuterium Exchange Mass Spectrometry.

Huang RY1, Iacob RE2, Sankaranarayanan S3, Yang L3, Ahlijanian M3, Tao L4, Tymiak AA1, Chen G5.
Journal of the American Society for Mass Spectrometry.J Am Soc Mass Spectrom.2017 Oct 2. doi: 10.1007/s13361-017-1815-8. [Epub ahead of print]
PMID 28971440

Pain processing in atypical Parkinsonisms and Parkinson disease: A comparative neurophysiological study.

Avenali M1, Tassorelli C2, De Icco R2, Perrotta A3, Serrao M4, Fresia M5, Pacchetti C6, Sandrini G2.
Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology.Clin Neurophysiol.2017 Oct;128(10):1978-1984. doi: 10.1016/j.clinph.2017.06.257. Epub 2017 Jul 17.
PMID 28829981

Japanese Journal

The prevalence of progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) in the UK

Brain 124, 1438-1449, 2001
NAID 30018522649

Which clinical features differentiate progressive supranuclear palsy (Steele-Richardson-Olszewski syndrome) from related disorders?

Brain 120, 65-74, 1997
NAID 30018521647

Proton magnetic resonance spectroscopy in Steele-Richardson-Olszewski syndrome

Mov Disord 12, 767-771, 1997
NAID 30012625085

Related Pictures

★リンクテーブル★

リンク元	「進行性核上性麻痺」
関連記事	「syndrome」

「進行性核上性麻痺」

Progressive supranuclear palsy
	Main anatomical plans and axes applied to the sections of the brain.
Classification and external resources
Specialty	neurology
ICD-10	G23.1
ICD-9-CM	333.0
OMIM	601104
DiseasesDB	10723
MedlinePlus	000767
eMedicine	neuro/328
NCI	Progressive supranuclear palsy
Patient UK	Progressive supranuclear palsy
MeSH	D013494

　　[★]

英: progressive supranuclear palsy PSP
同: 進行性核上麻痺、スティール・リチャードソン・オルゼウスキー症候群スティール-リチャードソン-オルシェウスキィ症候群 Steele-Richardson-Olszewski syndrome
関: パーキンソン症候群

[show details]

進行性核上麻痺 : 約 5,930 件
進行性核上性麻痺 : 約 61,700 件

概念

難病であり、特定疾患治療研究事業の対象疾患である。
大脳基底核や脳幹が障害され、核上性注視麻痺とパーキンソニズムを中核症状として呈する慢性進行性変性疾患
中年期以降男性に好発し、易転倒性、核上性注視麻痺、パーキンソニズム、認知症などを特徴とする原因不明の疾患である。病理学的には淡蒼球、視床下核、小脳歯状核、赤核、黒質、脳幹被蓋の神経細胞が脱落し、異常リン酸化タウ蛋白が神経細胞内およびグリア細胞内に蓄積が見られる。(参考1)

症状

後方への転倒傾向(歩行障害。姿勢反射障害に基づく) が特徴

1. 項筋の緊張亢進：頭部背屈(項部ジストニア)
2 垂直性核上性眼球運動障害(垂直方向、特に下方への眼球運動制限)

下方注視障害により階段を降りることが困難。末期には水平運動も障害される。

3 パーキンソニズム：筋強剛が前景に出る(振戦は稀)。動作緩徐、姿勢反射障害
4 精神障害：皮質下性認知症(前頭側頭型認知症に類似)
5 偽性球麻痺：構音障害、嚥下障害

※ すくみ足が前景に出る場合はpure akinesia またはPSP-Pと呼ぶ

症状が出る順番：①歩行障害を中心とするパーキンソニズム　→　②仮性球麻痺症状　→　③認知症症状　→　④(発症から4-5年)核上性眼球運動障害、頸部ジストニア

検査

MRI：中脳被蓋部橋被蓋の萎縮、第三脳室の拡大、矢状断ではハチドリに似た所見(humming bird sign)
SPECT：前頭葉の血流低下

治療

L-ドーパやドパミンアゴニストなどのドパミン作動薬は無効
三環系抗うつ薬が有効であるという報告がある。アミトリプチリン

参考

1. パーキンソン病関連疾患 (1)進行性核上性麻痺 - 難病情報センター

http://www.nanbyou.or.jp/entry/276

2.

http://www.jsnp.jp/cerebral_14.htm

<youtube>https://www.youtube.com/watch?v=BPt3pTzbcXc</youtube>

「syndrome」

　　[★]

n.

症候群

[1] 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.

[2] 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.

[pmid18819991-3] 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.

[4] ttp://www.ncbi.nlm.nih.gov/pmc/articles/PMC2812742/

[5] 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.

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案内

Steele-Richardson-Olszewski syndrome