Request Demo

Last update 08 May 2025

Amyotrophy, Monomelic

Last update 08 May 2025

Basic Info

Synonyms

AMYOTROPHY, MONOMELIC, Amyotrophy, monomelic, Benign focal amyotrophy

+ [12]

Introduction

Monomelic amyotrophy (MA) is a rare benign lower motor neuron disorder characterized by muscular weakness and wasting in the distal upper extremities during adolescence followed by a spontaneous halt in progression and a stabilization of symptoms.

Clinical Trials associated with Amyotrophy, Monomelic

NCT05959980

/ RecruitingNot ApplicableIIT

Posterior Cervical Fixation Versus Long-term Cervical Collar for Management of Hirayama Disease: Prospective Randomized Open Blinded Endpoint (PROBE), Phase III Study

The goal of this clinical trial] is to compare cervical collar versus neck stabilization surgery in diagnosed patients of Hirayama disease who have been reporting worsening of problems in the past six months. The main question[s] it aims to answer are:
• Is cervical stabilization surgery (Posterior cervical fixation) superior to conservative management in the form of cervical collar placement in patients with progressive Hirayama disease, observed at six months after intervention
Participants will have equal chance to:
Undergo cervical fixation surgery
Cervical collar management The investigators will study and compare the efficacy of both treatments upto six months after intervention

Start Date01 Oct 2023

Sponsor / Collaborator

All India Institute of Medical Sciences

ChiCTR2000035885

/ SuspendedEarly Phase 1IIT

Exploration of the correlations between hand function, spine function, and cerebral cortex function in patients with Hirayama disease

Start Date01 Sep 2020

Sponsor / Collaborator-

ChiCTR1900021216

/ SuspendedNot ApplicableIIT

The Effect of Anterior Cervical Fusion Segment Selection on the Surgical Outcome of Hirayama Disease

Start Date01 Feb 2019

Sponsor / Collaborator-

100 Clinical Results associated with Amyotrophy, Monomelic

100 Translational Medicine associated with Amyotrophy, Monomelic

0 Patents (Medical) associated with Amyotrophy, Monomelic

495

Literatures (Medical) associated with Amyotrophy, Monomelic

01 May 2025·Annals of Plastic Surgery

Upper Extremity Surgery in Hirayama Disease

Review

Author: Holzer, Elad Y. ; Giuffre, Jennifer L. ; Clark, Tod A.

IntroductionHirayama disease (HD) is a rare, nonfamilial, monomelic amyotrophy in which patients present with muscle atrophy and weakness of the forearms and hands, either unilateral or bilateral, and without sensory loss. Current treatment guidelines describe the role of conservative treatments including cervical collars and neurotropic medications, as well as spinal surgery in select patients. Upper extremity surgery has not yet been incorporated into the treatment algorithm of HD. The objective of this study is twofold: to present a case series of HD patients treated with the incorporation of nerve and tendon transfers and joint fusions into the existing treatment algorithm and to perform a literature review of interventions.MethodsThree cases (4 limbs) of HD treated surgically with nerve and tendon transfers and fusion are retrospectively reviewed. The subjective and objective results from surgery are reported. A literature review is performed on PubMed using “Hirayama disease” and “peripheral nerve surgery,” “nerve transfer,” “tendon transfer,” “hand surgery,” or “upper extremity surgery” as search terms to identify studies describing surgical treatment of HD outside of spinal surgery.ResultsThree HD patients (4 limbs) were identified. The average age was 23 years old (range, 16–33 years). Patients presented with intrinsic muscle atrophy, hypothenar, and thenar atrophy. The disease had been present for an average of 6.5 years (range, 1.5–15 years) prior to referral. Two patients had unilateral involvement, whereas one had bilateral involvement. Two patients (3 limbs) were treated with an anterior interosseous (AIN) to ulnar motor nerve transfer, whereas a patient with delayed presentation underwent thumb metacarpophalangeal joint fusion and a Zancolli lasso to the fingers. All patients had subjective and objective improvements postoperation.The literature reveals three independent case reports of HD patients treated with upper extremity surgery. Two papers describe using a tendon transfer, whereas one paper describes an AIN to ulnar motor nerve transfer. All patients demonstrated functional improvements in follow-up.ConclusionsHD can be successfully treated with a combination of upper extremity surgery and nerve transfers. To the authors' knowledge, this is the first literature review and the largest case series presenting such interventions in HD.

01 Apr 2025·BMJ Case Reports

Electrodiagnostic studies in the diagnosis of Hirayama disease: a focus on early identification

Article

Author: Sivasubramanian, Nirangjhana ; Kumar, Yogesh ; Rai, Anand ; Bhuvanachandran, Athira Sindhu

Hirayama disease is a rare neurological disorder primarily affecting young males. This condition poses diagnostic challenges due to its subtle onset and progressive nature. Recognising the importance of early diagnosis is crucial to implementing timely interventions and improving patient outcomes. While Hirayama disease is considered rare, its exact prevalence remains unclear due to under-diagnosis and variability in presentation. Reported cases are more common in certain regions, notably East Asia, but occurrences have been documented worldwide. Despite its rarity, the impact of Hirayama disease on affected individuals can be significant, warranting increased awareness among healthcare professionals.The key message of this case report is to highlight the utility of electrodiagnostic studies, particularly electromyography and F wave analysis, in aiding the diagnosis of Hirayama disease. By emphasising the significance of recognising specific electromyographic patterns, this report aims to facilitate early identification and intervention, thereby improving patient management and quality of life.

01 Mar 2025·Neurology India

Diagnostic Crossroads: Coexistence of Myotonic Dystrophy Type 1 and Hirayama Disease in a Case Report

Article

Author: Poncha, Fali F. ; Arora, Ronak K. ; Dudhatra, Ashutosh V.

Analysis

Perform a panoramic analysis of this field.

view full example data

AI Agents Built for Biopharma Breakthroughs

Accelerate discovery. Empower decisions. Transform outcomes.

Get started for free today!

Accelerate Strategic R&D decision making with Synapse, PatSnap’s AI-powered Connected Innovation Intelligence Platform Built for Life Sciences Professionals.

Start your data trial now!

Synapse data is also accessible to external entities via APIs or data packages. Empower better decisions with the latest in pharmaceutical intelligence.

Bio

Bio Sequences Search & Analysis

Chemical

Chemical Structures Search & Analysis

Amyotrophy, Monomelic

Basic Info

Related

Analysis