monomelic Amyotrophy

Spinomuscular atrophy characterized by insidious onset of weakness and wasting of the muscles of the hand and forearm. It is usually unilateral, but can be bilateral and occurs most commonly as a sporadic condition in young men. It follows a relatively benign course after a few years of progression. Molecular basis is unknown, but polymorphisms in KIAA1377 and C5ORF42 genes have been identified. OMIM: 602440

Also Known As:

Networked: 51 relevant articles (6 outcomes, 2 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Rare Diseases (Rare Disease)
2.	Spinal Muscular Atrophies of Childhood (Werdnig Hoffman Disease)
3.	Spinal Cord Diseases
4.	Disease Progression
5.	Neuromuscular Diseases (Neuromuscular Disease)

Experts

1.	Jiang, Jianyuan: 4 articles (02/2022 - 10/2013)
2.	Lu, Feizhou: 4 articles (02/2022 - 10/2013)
3.	Ma, Xiaosheng: 4 articles (02/2022 - 10/2013)
4.	Xia, Xinlei: 4 articles (02/2022 - 10/2013)
5.	Lu, Xiao: 2 articles (02/2022 - 09/2021)
6.	Zou, Fei: 2 articles (02/2022 - 01/2021)
7.	Jiang, Jian-Yuan: 2 articles (09/2021 - 08/2018)
8.	Song, Jian: 2 articles (09/2021 - 08/2018)
9.	Wang, Hongli: 2 articles (01/2021 - 10/2013)
10.	Zheng, Chaojun: 2 articles (01/2021 - 11/2017)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to monomelic Amyotrophy:

1.	SteroidsIBA 01/01/2020 - "Early Diagnosed Hirayama Disease with Unusual Symptoms Improved by Steroid Pulse Therapy."
2.	Immunoglobulin E (IgE)IBA 03/01/2010 - "Specifically, he had elevated serum total immunoglobulin E level, which had been postulated to be a precipitating factor in Hirayama disease. " 01/01/2005 - "In patients whose history of Hirayama disease was less than five years, serum total IgE was higher than in those with the disease for five years or more (p = 0.05). " 01/01/2005 - "To investigate whether IgE is a contributing factor in Hirayama disease. " 06/01/2001 - "The frequency of mite antigen specific IgE was significantly higher in the present patients with Hirayama disease than in 82 healthy controls (26/82, p<0.005). "
3.	1- cyclohexyl- 3- (2- (4- morpholinyl)ethyl)carbodiimideIBA 11/01/2017 - "To investigate central motor conduction time (CMCT) in patients with Hirayama disease (HD) and to analyse the role of motor nerve root lesions in the pathogenesis of HD. " 09/01/2004 - "To determine the Cortical threshold intensity (TI) and central motor conduction time (CMCT) in patients with monomelic amyotrophy (MMA). " 04/01/1995 - "In 7 patients with Hirayama disease, central motor conduction to upper (CMCT-ADM) and lower limbs (CMCT-TA) was evaluated. "
4.	ChemokinesIBA 01/01/2017 - "To identify cytokine and chemokine profiles in Hirayama disease, serum samples were analyzed using multiplex magnetic bead-based assay. " 01/01/2017 - "Serum cytokine and chemokine profiles in patients with juvenile muscular atrophy of distal upper extremity (Hirayama disease)." 12/01/2020 - "In addition to these mechanical factors, immunological abnormalities such as hyperIgEemia and upreguration of some cytokines and chemokines in serum/CSF of patients with Hirayama disease have been reported. "
5.	CytokinesIBA 01/01/2017 - "To identify cytokine and chemokine profiles in Hirayama disease, serum samples were analyzed using multiplex magnetic bead-based assay. " 01/01/2017 - "Serum cytokine and chemokine profiles in patients with juvenile muscular atrophy of distal upper extremity (Hirayama disease)." 12/01/2020 - "In addition to these mechanical factors, immunological abnormalities such as hyperIgEemia and upreguration of some cytokines and chemokines in serum/CSF of patients with Hirayama disease have been reported. "
6.	Immunoglobulin M (IgM)IBA 12/01/2006 - "To evaluate IgM anti-GM1 antibody titers in patients of monomelic amyotrophy. " 12/01/2006 - "IgM anti-GM1 antibody titers in patients with monomelic amyotrophy." 08/15/2005 - "IgM antibodies to N-acetylgalactosaminyl GD1a in benign monomelic amyotrophy of the lower limb."
7.	AntibodiesIBA 12/01/1994 - "[Electrodiagnosis of neuromuscular diseases--a syndrome of monomelic amyotrophy with spinal hemiatrophy, anti-GM1 antibodies and abnormal F-waves (MASH)]." 08/15/2005 - "IgM antibodies to N-acetylgalactosaminyl GD1a in benign monomelic amyotrophy of the lower limb."
8.	Therapeutic UsesIBA 01/01/2000 - "The technique described here would be useful for evaluating the vulnerability of cervical motor neurons and the therapeutic effect of the neck collar in Hirayama disease."
9.	Pyruvic Acid (Pyruvate)IBA 04/02/2010 - "On the other hand, Hirayama disease patients had significantly higher median concentrations of pyruvate (P<0.05), glutamate (P<0.001), formate (P<0.05) and lower median concentrations of N-acetyl derivatives. "
10.	Chemokine CCL5IBA 01/01/2017 - "Eotaxin, MCP-1 and RANTES levels were significantly higher in Hirayama disease (N=11) than in normal controls (N=12). "

Therapies and Procedures

1.	Decompression 09/01/2018 - "Posterior cervical decompression with coagulation of epidural venous plexus is a technique that seems effective in Hirayama disease in young subjects. " 10/01/2013 - "Efficacy of anterior cervical decompression and fusion procedures for monomelic amyotrophy treatment: a prospective randomized controlled trial: clinical article." 02/01/2019 - "A Severe Case of Hirayama Disease Successfully Treated by Posterior Cervical Fixation without Decompression and Fusion." 08/01/2018 - "We, therefore explored the changes in cortical activation before and after anterior cervical decompression and fusion surgery in patients with Hirayama disease (HD). " 08/01/2018 - "Cortical Activation Changes in Hirayama Disease After Anterior Cervical Decompression and Fusion."
2.	Tendon Transfer 01/01/2023 - "Outcome of Tendon Transfer for Monomelic Amyotrophy (Hirayama Disease)." 01/01/2004 - "We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. " 01/01/2023 - "We present a patient with Hirayama disease whose hand function was improved dramatically by a tendon transfer after nearly 3 years without a definitive diagnosis and call attention to the hand surgeon's role in identifying this rare disease to enable timely functional restoration."
3.	Diskectomy (Discectomy) 02/01/2023 - "Efficacy of Anterior Cervical Discectomy and Fusion for Female Patients with Hirayama Disease." 09/01/2021 - "Anterior cervical discectomy and fusion (ACDF) is a common surgical method used to treat patients with Hirayama disease. " 09/01/2021 - "The Relationship Between Preoperative Cervical Sagittal Balance and Clinical Outcome of Patients With Hirayama Disease Treated With Anterior Cervical Discectomy and Fusion." 01/01/2021 - "The Short-Term to Midterm Follow-Up of Patients with Hirayama Disease After Anterior Cervical Discectomy and Fusion." 01/01/2020 - "We present the case of a 19-year-old boy who had the classic radiologic and clinical presentations of Hirayama disease treated with anterior cervical diskectomy and fusion (ACDF). "
4.	Therapeutics 11/22/2016 - "Objective: To summarize the therapeutic approaches of patients with Hirayama disease and investigate the patient's compliance to cervical collar therapy and its influencing factors. " 11/22/2016 - "[Investigation of the compliance of cervical collar therapy in 73 patients with Hirayama disease]." 04/01/2012 - "Clinical and radiological profile of Hirayama disease: A flexion myelopathy due to tight cervical dural canal amenable to collar therapy." 04/01/2001 - "[Cervical collar therapy for juvenile muscular atrophy of distal upper extremity (Hirayama disease): results from 38 cases]." 01/01/2020 - "Early Diagnosed Hirayama Disease with Unusual Symptoms Improved by Steroid Pulse Therapy."
5.	Activities of Daily Living (ADL) 01/01/2004 - "Advanced Hirayama disease with successful improvement of activities of daily living by operative reconstruction." 01/01/2004 - "We found that tendon transfer improved the activities of daily living (ADL) of a patient with advanced Hirayama disease. " 01/01/2004 - "An operative reconstruction can be valuable, even in patients with Hirayama disease who have developed impaired ADL due to extensive intrinsic hand muscle atrophy."