WordNet

the process of declining from a higher to a lower level of effective power or vitality or essential quality (同)devolution
passing from a more complex to a simpler biological form (同)retrogression

PrepTutorEJDIC

退歩,退化,墜落

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2017/03/05 04:05:03」(JST)

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Olivopontocerebellar atrophy (OPCA) is the degeneration of neurons in specific areas of the brain – the cerebellum, pons, and inferior olives.^[1] OPCA is present in several neurodegenerative syndromes, including inherited and non-inherited forms of ataxia (such as the hereditary spinocerebellar ataxia known as Machado–Joseph disease) and multiple system atrophy (MSA), with which it is primarily associated.^[1]

OPCA may also be found in the brains of individuals with prion disorders and inherited metabolic diseases. The characteristic areas of brain damage that indicate OPCA can be seen by imaging the brain using CT scans or MRI studies.^[1]

The term was originally coined by Joseph Jules Dejerine and André Thomas.^[2]^[3]

Signs and symptoms

OPCA is characterized by progressive cerebellar ataxia, leading to clumsiness in body movements, veering from midline when walking, wide-based stance, and falls without signs of paralysis or weakness.^[4]^[5] Clinical presentation can vary greatly between patients, but mostly affects speech, balance and walking.^[6] Other possible neurological problems include spasmodic dysphonia, hypertonia, hyperreflexia, rigidity, dysarthria, dysphagia and neck dystonic posture.^[5]

Cause

Olivopontocerebellar atrophy is hereditary, but has an unknown genetic basis. There are two forms:

Number	OMIM	Alt. name	Inheritance
OPCA type 2	258300	Fickler^[7]-Winkler^[8] type OPCA	autosomal recessive
OPCA type 5	164700	OPCA with dementia and extrapyramidal signs	autosomal dominant

A few non-hereditary diseases formerly categorized as olivopontocerebellar atrophy have been reclassified as forms of multiple system atrophy^[9] as well as to four hereditary types, that have been currently reclassified as four different forms of spinocerebellar ataxia:

Hereditary OPCA type	OPCA name	SCA #	Gene	OMIM
OPCA type 1	"Menzel type OPCA"	SCA1	ATXN1	164400
OPCA type 2, autosomal dominant	"Holguin type OPCA"	SCA2	ATXN2	183090
OPCA type 3	"OPCA with retinal degeneration"	SCA7	ATXN7	164500
OPCA type 4	"Schut-Haymaker type OPCA"	SCA1	ATXN1	164400

Diagnosis

Treatment

Physiotherapy intervention aims to improve balance and gait of OPCA patients, by stimulating neuroplastic changes in the atrophied neural structure. A challenge-oriented treatment program has previously been shown to be beneficial for individuals with ataxia from OPCA. The treatment program was composed of repetitive training with task challenges (e.g. obstacle course) and/or novel motor skills acquisition over a 12-week period under the supervision of a physiotherapist. Task challenges were progressed only when the patient showed mastery of a task.^[4]

Overground harness systems may be used to allow OPCA patients to challenge their balance without chance of falling. Furthermore, home exercise programs and/or aquatic exercises are used to allow more repetitions to facilitate balance learning. Treatment programs should be frequently monitored and adjusted based on a patient's progress. Outcome measures such as the Berg Balance Scale, Dynamic Gait Index and activities-specific balance confidence scales are useful to assess patient’s progress over time.^[4]

References

^ ^a ^b ^c "NINDS Olivopontocerebellar Atrophy Information Page". Retrieved 7 Feb 2012.
^ synd/1903 at Who Named It? - "Dejerine-Thomas atrophy"
^ J. J. Dejerine, A. Thomas. L’atrophie olivo-ponto-cérébelleuse. Nouvelle iconographie de la Salpêtrière, Paris, 1900, 13: 330-370. 1912, 25: 223-250.
^ ^a ^b ^c Landers, M; Adams M; Acosta K; Fox A. (2009). "Challenge-oriented gait and balance training in sporadic olivopontocerebellar atrophy: a case study.". J Neurol Phys Ther. 33 (3): 160–168. doi:10.1097/npt.0b013e3181b511f4.
^ ^a ^b Berciano, J; Boesch S; Pérez-Ramos JM; Wenning GK (2006). "Olivopontocerebellar atrophy: toward a better nosological definition.". Mov Disord. 21 (10): 1607–13. doi:10.1002/mds.21052.
^ http://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001765/
^ Fickler, A. Klinische und pathologisch-anatomische Beitraege zu den Erkrankungen des Kleinhirns. Dtsch. Z. Nervenheilk. 41: 306-375, 1911.
^ Winkler, C. A case of olivo-pontine cerebellar atrophy and our conceptions of neo- and palaio-cerebellum. Schweiz. Arch. Neurol. Psychiat. 13: 684-702, 1923.
^ MeSH Result

External links

-113967058 at GPnotebook - "olivopontocerebellar atrophy"
-429195218 at GPnotebook - "lethal olivopontocerebellar atrophy"
opca at NINDS
OPCA Awareness

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. 多系統萎縮症：臨床的特徴および診断 multiple system atrophy clinical features and diagnosis
2. 成人における小脳性運動失調の概要 overview of cerebellar ataxia in adults
3. 多系統萎縮症：予後および治療 multiple system atrophy prognosis and treatment
4. アルコールの慢性神経学的合併症の概要 overview of the chronic neurologic complications of alcohol
5. パーキンソン病による認識機能障害および認知症 cognitive impairment and dementia in parkinson disease

English Journal

Young-onset multiple system atrophy: Its rarity and heterogeneity.

Kim HJ, Jeon B.
Movement disorders : official journal of the Movement Disorder Society. 2019 Jul;34(7)1085-1086.
PMID 31322774

New Radiologic Findings of Hypertrophic Olivary Degeneration in 2 Patients with Brainstem Lymphoma.

Sato S, Shibahara I, Inoue Y, Hide T, Kumabe T.
World neurosurgery. 2019 Mar;123()464-468.e1.
Hypertrophic olivary degeneration (HOD) is a rare neurological condition of trans-synaptic degeneration caused by disruption of the dentatorubro-olivary pathway. We present new radiologic findings of HOD in 2 cases of brainstem lymphoma. A 35-year-old woman (Case 1) and a 69-year-old man (Case 2) pr
PMID 30496930
PMID 30725641

Japanese Journal

皮質性小脳萎縮症とは何か

吉田邦広
神経治療学 35(3), 320-325, 2018
… <p>"Cortical cerebellar atrophy (CCA)" is a neuropathologically–defined disease entity, which is characterized by pure cerebello–olivary degeneration. … There is no specific biomarker for cerebello–olivary degeneration, therefore, the diagnosis of CCA is largely dependent on the exclusion of other diseases with cerebellar ataxia at present. …
NAID 130007550061

Neuro-otological Examinations in Patients With Spinocerebellar Degeneration

Shibata Michiko,Hamamura Ryoji,Aoi Noriaki,Kimura Mitsuhiro,Yamaguchi Shuhei,Kawauchi Hideyuki
Shimane Journal of Medical Science 34(1), 21-26, 2017-07-31
… Spinocerebellar-degeneration （SCD） has been reported to show a variety of abnormal neurootological findings on electronystagmography （ENG） rather in a late stage of disease developing process, but not in an earlier stage.Purpose: We have analysed ENG findings derived from 79 patients confirmed with SCD, in order to assess a diagnostic value of ENG and determine which manifestations are helpful in diagnosing SCD.Results: We observed a high incidence of saccadic pursuit, severely impaired optokinetic nystagmus, and impaired visual …
NAID 120006343745

Multiple system atrophy: clinicopathological characteristics in Japanese patients

OZAWA Tetsutaro,ONODERA Osamu
Ｐｒｏｃｅｅｄｉｎｇｓ　ｏｆ　ｔｈｅ　Ｊａｐａｎ　Ａｃａｄｅｍｙ．　Ｓｅｒ．　Ｂ：　Ｐｈｙｓｉｃａｌ　ａｎｄ　Ｂｉｏｌｏｇｉｃａｌ　Ｓｃｉｅｎｃｅｓ 93(5), 251-258, 2017
… Clinical examples include MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P), whereas olivopontocerebellar atrophy and striatonigral degeneration represent pathological variants. … A comparative study of MSA pathology showed that the olivopontocerebellar-predominant pathology was more frequent in Japanese MSA than in British MSA. …
NAID 130006770760

Related Pictures

Olivopontocerebellar Degeneration Neuroradiology On the Net: Olivopontocerebellar degeneration Olivopontocerebellar Atrophies; Dejerine-Thomas Syndrome; Olivopontocerebellar Atrophy

★リンクテーブル★

リンク元	「オリーブ橋小脳萎縮症」
関連記事	「degeneration」「olivopontocerebellar」

「オリーブ橋小脳萎縮症」

Olivopontocerebellar atrophy
	Sagittal section through right cerebellar hemisphere. The right olive has also been cut sagittally.
Classification and external resources
Specialty	neurology
ICD-10	G23.8
ICD-9-CM	333.0
DiseasesDB	2012 9208
MedlinePlus	000758
eMedicine	neuro/282
MeSH	D009849
	[edit on Wikidata]

　　[★]

英: olivopontocerebellar atrophy, OPCA, olivopontocerebellar atrophies
同: デジュリーヌ・トーマ病 Dejerine-Thomas disease、オリーブ・橋・小脳萎縮症 olivo-ponto-cerebellar atrophy、オリーブ橋小脳変性症 olivopontocerebellar degeneration
関: spinocerebellar ataxia, SCA1、多系統萎縮症、脊髄小脳変性症

概念

脊髄小脳変性症との関係

脊髄小脳変性症 spinocerebellar degeneration SCD

1. 孤発性脊髄小脳変性症

A. 多系統萎縮症 multiple system atrophy MSA

オリーブ核・橋・小脳萎縮症 OPCA

多系統萎縮症

			小脳失調	自律神経症状	錐体外路症状
オリーブ橋小脳萎縮症	OPCA	MSA-C	○	△	△
Shy-Drager症候群	SDS		△	○	△
線条体黒質変性症	SND	MSA-P	△	△	○

症状

YN.J-124

歩行障害→運動失調→平衡障害→構音障害
1. 小脳症状
2. 錐体路症状
3. 錐体外路症状
4. 自立心液晶状
5. 知能低下

治療

酒石酸プロチレリン(TRH)

予後(YN.J-124)

緩徐進行。発症後5-10年で死亡

国試

099A045

「degeneration」

　　[★]

n.

変性、変性症、逆行変性、縮退、退化

関: degeneracy、degenerate、degenerative、denaturation、denature、involution、retract、retrograde degeneration、retrograde degradation、unfolding

「olivopontocerebellar」

　　[★]

adj.

オリーブ橋小脳の

[nidsopca-1] "NINDS Olivopontocerebellar Atrophy Information Page". Retrieved 7 Feb 2012.

[2] synd/1903 at Who Named It? - "Dejerine-Thomas atrophy"

[3] J. J. Dejerine, A. Thomas. L’atrophie olivo-ponto-cérébelleuse. Nouvelle iconographie de la Salpêtrière, Paris, 1900, 13: 330-370. 1912, 25: 223-250.

[Landers-4] Landers, M; Adams M; Acosta K; Fox A. (2009). "Challenge-oriented gait and balance training in sporadic olivopontocerebellar atrophy: a case study.". J Neurol Phys Ther. 33 (3): 160–168. doi:10.1097/npt.0b013e3181b511f4.

[Berciano-5] Berciano, J; Boesch S; Pérez-Ramos JM; Wenning GK (2006). "Olivopontocerebellar atrophy: toward a better nosological definition.". Mov Disord. 21 (10): 1607–13. doi:10.1002/mds.21052.

[6] ttp://www.ncbi.nlm.nih.gov/pubmedhealth/PMH0001765/

[7] Fickler, A. Klinische und pathologisch-anatomische Beitraege zu den Erkrankungen des Kleinhirns. Dtsch. Z. Nervenheilk. 41: 306-375, 1911.

[8] Winkler, C. A case of olivo-pontine cerebellar atrophy and our conceptions of neo- and palaio-cerebellum. Schweiz. Arch. Neurol. Psychiat. 13: 684-702, 1923.

[9] MeSH Result

匿名

検索

案内

案内

olivopontocerebellar degeneration