Hirayama disease

WordNet

1. an impairment of health or a condition of abnormal functioning
2. caused by or altered by or manifesting disease or pathology; "diseased tonsils"; "a morbid growth"; "pathologic tissue"; "pathological bodily processes" (同)morbid, pathologic, pathological

PrepTutorEJDIC

1. (体の)『病気』,疾患 / (精神・道徳などの)病気,病弊
2. 女性の話術芸人 =diseur
3. 病気にかかった / 病的な,不健全な(morbid)

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2014/05/19 15:17:24」(JST)

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Monomelic amyotrophy (MMA), also known as Hirayama disease, Sobue disease, juvenile non-progressive amyotrophy and juvenile asymmetric segmental spinal muscular atrophy (JASSMA) — is an untreatable, focal motor neuron disease that primarily affects young (15–25 year old) males in India and Japan. MMA is marked by insidious onset of muscular atrophy, which stabilizes at a plateau after two to five years from which it neither improves nor worsens. There is no pain or sensory loss associated with MMA. Unlike other lower motor neuron diseases, MMA is not believed to be hereditary and fasciculations (involuntary muscle twitches) are rare.

EMG tests reveal loss of the nerve supply, or denervation, in the affected limb without conduction block (nerve blockage restricted to a small segment of the nerve). Increased sweating, coldness and cyanosis have been reported for a few patients, indicating involvement of the sympathetic nervous system.

While MMA will cause weakness and/or wasting in only one limb, EMG and NCV tests often show signs of reinnervation in the unaffected limbs.

Treatment

There is no cure for MMA. Treatment consists of muscle strengthening exercises and training in hand coordination.

Prognosis

The symptoms of MMA usually progress slowly for one to two years before reaching a plateau, and then remain stable for many years. Disability is generally slight. Rarely, the weakness progresses to the opposite limb. There is also a slowly progressive variant of MMA known as O'Sullivan-McLeod syndrome, which only affects the small muscles of the hand and forearm and has a slowly progressive course.

Epidemiology

MMA occurs in males between the ages of 15 and 25. Onset and progression are slow. MMA is seen most frequently in Asia, particularly in Japan and India; it is much less common in North America.

Further reading

• Di Muzio, A.; Pizzi, C. D.; Lugaresi, A.; Ragno, M.; Uncini, A. (1994). "Benign monomelic amyotrophy of lower limb: A rare entity with a characteristic muscular CT". Journal of the Neurological Sciences 126 (2): 153–161. doi:10.1016/0022-510X(94)90266-6. PMID 7853021. 
• Di Guglielmo, G.; Brahe, C.; Di Muzio, A.; Uncini, A. (1996). "Benign monomelic amyotrophies of upper and lower limb are not associated to deletions of survival motor neuron gene". Journal of the Neurological Sciences 141 (1–2): 111–113. doi:10.1016/0022-510X(96)00154-2. PMID 8880702. 
• Münchau, A.; Rosenkranz, T. (2000). "Benign Monomelic Amyotrophy of the Lower Limb – Case Report and Brief Review of the Literature". European Neurology 43 (4): 238–240. doi:10.1159/000008183. PMID 10828656. 
• Freitas, M. R. G. D.; Nascimento, O. J. M. (2000). "Benign monomelic amyotrophy: A study of twenty-one cases". Arquivos de Neuro-Psiquiatria 58 (3B): 808–813. doi:10.1590/S0004-282X2000000500003. PMID 11018815. 
• Gourie-Devi, M.; Nalini, A. (2001). "Sympathetic skin response in monomelic amyotrophy". Acta Neurologica Scandinavica 104 (3): 162–166. doi:10.1034/j.1600-0404.2001.00016.x. PMID 11551236. 
• Neves, M. A. O.; Freitas, M. R. G. D.; Mello, M. P. D.; Dumard, C. H.; Freitas, G. R. D.; Nascimento, O. J. M. (2007). "Benign monomelic amyotrophy with proximal upper limb involvement: Case report". Arquivos de Neuro-Psiquiatria 65 (2b): 524–527. doi:10.1590/S0004-282X2007000300032. PMID 17665029. 

External links

• monomelic_amyotrophy at NINDS

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. 上肢末梢神経症候群の概要 overview of upper extremity peripheral nerve syndromes
• 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. 家族性筋萎縮性側索硬化症 familial amyotrophic lateral sclerosis
• 4. 肘および手首の尺骨神経障害 ulnar neuropathy at the elbow and wrist

English Journal

• Hirayama disease: Three cases.

• Dejobert M, Geffray A, Delpierre C, Chassande B, Larrieu E, Magni C.SourceCHR Orléans, 14, avenue de l'Hôpital, 45000 Orléans, France; CHU de Tours, 2, boulevard Tonnelé, 37000 Tours, France. Electronic address: maelle.d@hotmail.fr.
• Diagnostic and interventional imaging.Diagn Interv Imaging.2013 Jan 14. pii: S2211-5684(12)00342-7. doi: 10.1016/j.diii.2012.10.008. [Epub ahead of print]
• Hirayama disease is a rare, lower cervical myelopathy affecting young adults. It is responsible for pure distal motor impairment of the upper limbs, with slow progressive development in the metameric territories of C7 to T1. It is thought to be caused by movements involved in flexing the neck. Neutr
• PMID 23332131

• Hirayama disease in Austria.

• Finsterer J, Löscher W, Wanschitz J, Baumann M, Quasthoff S, Grisold W.SourceKrankenanstalt Rudolfstiftung, Postfach 20, 1180 Vienna, Austria. Electronic address: fifigs1@yahoo.de.
• Joint, bone, spine : revue du rhumatisme.Joint Bone Spine.2012 Dec 10. pii: S1297-319X(12)00283-7. doi: 10.1016/j.jbspin.2012.10.013. [Epub ahead of print]
• OBJECTIVES: Hirayama disease (HD) is a segmental cervical myelopathy which affects the C7-D1 myotomes and presents with unilateral or asymmetric upper limb weakness/wasting. The study aimed at systematically collecting cases of HD in Austria and at describing and discussing their presentation on cli
• PMID 23237996

Japanese Journal

• 臨床研究・症例報告 若年性一側上肢筋萎縮性(平山病)の女児例

• 有馬 ふじ代,池上 千晶,込山 修 他
• 小児科臨床 66(1), 85-88, 2013-01-00
• NAID 40019522516

• Clivus Metastasis From Gastric Signet Ring Cell Carcinoma After a 10-Year Disease-Free Interval : Case Report

• FUKUSHIMA Masamichi,KATAYAMA Yoichi,SHIGEMORI Yutaka,MIYAKE Hiroshi,HIRAYAMA Teruyasu,KOTANI Akio
• Neurologia medico-chirurgica = 神経外科 52(10), 751-753, 2012-10-15
• NAID 10030873489

Related Links

• Hirayama's Disease - Facts and Information - Disabled World

8 Mar 2010 ... Hirayama's disease, also referred to as Monomelic amyotrophy, MMS, Sobue disease or Juvenile nonprogressive amyotrophy.

• Case 68: Hirayama Disease1

In a young patient with clinical evidence of hand and/or forearm muscle atrophy and asymmetric thinning of the cervical spinal cord, a diagnosis of Hirayama disease (juvenile muscular atrophy of the distal upper extremity) should always be ...

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★リンクテーブル★

リンク元	「若年性一側上肢筋萎縮症」
関連記事	「disease」

「若年性一側上肢筋萎縮症」

　　[★]

英

juvenile muscularatrophy of unilateral upper extremity

同

平山病 Hirayama disease、若年性一側性上肢筋萎縮症、若年性一側性上肢遠位筋萎縮症 juvenile muscularatrophy of distal upper extremity

関

運動ニューロン疾患

• 神経系 091102IV

病因

• 頚椎の前屈により、後方から拡張した静脈叢によって頚部硬膜管が圧迫され、脊髄の前角細胞を中心とした圧迫ないし循環障害による壊死性病変による。

疫学

• 10-20歳代。男:女=95%：5%

症状

• 筋萎縮：一側上肢前腕尺側から手指に欠けて限局。進行性。

「disease」

　　[★]

• n.

疾患：illnessより厳密な概念。「ある臓器に明確な障害が確認され、それによって症状が出ているとはっきり説明できる場合」 (PSY.9)

特定の原因、病態生理、症状、経過、予後、病理組織所見が全てそろった場合 (PSY.9)

• something that is very wrong with people's attitudes, way of life or with society.

関

ail、ailment、disease entity、disorder、ill、illness、malady、sick、sickness

• 注意

disease ≠ illness ≠ disorder