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monomelic amyotrophy

Taotao Hui, Zhi Bo Chang, Feng Han, Yongjun Rui
RATIONALE: Monomelic amyotrophy (MMA) is a benign motor neuron disease with bilateral muscular atrophy in asymmetry and abnormal in the electromyography (EMG). However, we report a case by the muscle biopsy which shows symptoms of slowly progressive amyotrophy despite having a normal EMG. PATIENT CONCERNS: A 51-year-old male was diagnosed with a lower limb amyotrophy, insidious at the onset and located in the distal thigh and the proximal crus near the knee, slowly progressive weakness, and wasting of his right gastrocnemius muscle for the last 20 years...
June 2018: Medicine (Baltimore)
Deb Kumar Boruah, Shantiranjan Sanyal, Arjun Prakash, Sashidhar Achar, Dhabal D Dhingani, Binod Sarma
Background: The aim of the study was to evaluate the magnetic resonance imaging (MRI) findings in bilateral symmetrical Hirayama disease and find out MRI features which are probably more indicative of symmetrical Hirayama disease, thereby help in differentiating this entity from other motor neuron disease (MND). Methods: This prospective as well as retrospective study was carried out from December 2010 to September 2016 in a tertiary care center of northeast India on 92 patients with Hirayama disease. Only 19 patients having bilateral symmetric upper limb involvement at the time of presentation were included in this study sample...
July 6, 2017: Iranian Journal of Neurology
José Manuel Matamala, Nimeshan Geevasinga, William Huynh, Thanuja Dharmadasa, James Howells, Neil G Simon, Parvathi Menon, Steve Vucic, Matthew C Kiernan
OBJECTIVE: To evaluate corticomotoneuronal integrity in monomelic amyotrophy using threshold tracking transcranial magnetic stimulation (TT-TMS). METHODS: Cortical excitability studies were prospectively performed in 8 monomelic amyotrophy patients and compared to 21 early-onset amyotrophic lateral sclerosis (ALS) patients and 40 healthy controls. Motor evoked potentials responses were recorded over abductor pollicis brevis. RESULTS: Maximal motor evoked potential (MEP/CMAP ratio) was significantly increased in monomelic amyotrophy compared with controls (monomelic amyotrophy 51...
August 2017: Clinical Neurophysiology: Official Journal of the International Federation of Clinical Neurophysiology
Halil Ay
Hirayama disease (HD) is a rare motor neuron disorder that involves a single upper extremity. It is clinically characterized by weakness and atrophy of the muscles of the hand and forearm. This article presents a 19-year-old woman who visited the orthopedics outpatient clinic with weakness and atrophy in her right hand and was clinically diagnosed with advanced stage carpal tunnel syndrome and scheduled for surgical intervention; she was later diagnosed with HD by an electrophysiological study. As a result, it has been found that a careful electrophysiological study and neurological examination can be used to diagnose HD...
2017: Neuropsychiatric Disease and Treatment
S Anuradha, Vanlalmalsawmdawngliana Fanai
Hirayama disease, also known as monomelic amyotrophy (MMA), is a rare cervical myelopathy that manifests itself as a self-limited, asymmetrical, slowly progressive atrophic weakness of the forearms and hands predominantly in young males. The forward displacement of the posterior dura of the lower cervical dural canal during neck flexion has been postulated to lead to lower cervical cord atrophy with asymmetric flattening. We report a case of Hirayama disease in a 25-year-old Indian man presenting with gradually progressive asymmetrical weakness and wasting of both hands and forearms along with unusual features of autonomic dysfunction and upper motor neuron lesion...
2016: Case Reports in Neurological Medicine
Sanjeev Jain
An early description of paralysis confined to an upper limb can be found in the casebook of Dr. Charles I Smith, who was a doctor in Bangalore in the 19th century. The historical and clinical aspects, as well as the current importance of this entity are described briefly.
January 2017: Neurology India
Ravinder-Jeet Singh, Veeramani Preethish-Kumar, Kiran Polavarapu, Seena Vengalil, Chandrajit Prasad, Atchayaram Nalini
Preferential involvement of C7, C8, T1 level anterior horn cells is a typical feature in Hirayama disease/brachial monomelic amyotrophy (BMMA). There are no clinico-electrophysiological studies to substantiate the peculiar pattern of muscle involvement. Thirty subjects, 10 in each group of BMMA, amyotrophic lateral sclerosis (ALS) and age-matched normal healthy subjects underwent detailed clinical and electrophysiological testing. Results showed that the mean age at evaluation for BMMA and ALS patients was 25...
February 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
B S Narayana Gowda, J Mohan Kumar, Praveen Kumar Basim
INTRODUCTION: Hirayama's disease is a rare benign disorder, also referred to as monomelic amyotrophy (MMA), Juvenile non progressive amyotrophy, Sobue disease. It is a focal, lower motor neuron type of disease. Mainly young males in their second and third decades of age are most commonly affected. It is seen most commonly in Asian countries like India and Japan. In majority of people cause of this disease is unknown. MRI of cervical spine in flexion will reveal the cardinal features of Hirayama disease...
July 2013: Journal of Orthopaedic Case Reports
Eman Al-Ghawi, Talal Al-Harbi, Adnan Al-Sarawi, Mohamed Binfalah
BACKGROUND: Monomelic amyotrophy is an uncommon, benign, unilateral disorder of the lower motor neurons, affecting predominantly the hand and forearm muscles. Proximal involvement of the arm and shoulder muscles is an unusual presentation that has been rarely reported in the literature. CASE PRESENTATION: A 28-year-old white man presented with insidious-onset, slowly progressive, unilateral weakness and atrophy of his left shoulder girdle and deltoid muscles. A neurological examination revealed weakness and atrophy in his left deltoid, infraspinatus and supraspinatus muscles...
March 17, 2016: Journal of Medical Case Reports
Deepti Vibha, Madhuri Behari, Vinay Goyal, Garima Shukla, Rohit Bhatia, Achal Kumar Srivastava, S Vivekanandhan
OBJECTIVES: The objective of our study was to describe the clinical characteristics, electrophysiology, MRI features and conduct viral assays in patients with Monomelic Amyotrophy (MMA) and follow them up over one year. METHODS: Consecutive patients with MMA who attended the Neurology services from April 2013 to March 2014 were included. Age and sex matched controls were taken for the purpose of viral assay analysis. The clinical evaluation was repeated at six months and one year...
December 15, 2015: Journal of the Neurological Sciences
Arjit Agarwal, Shruti Chandak, Pawan Joon
Hirayama disease is a rare type of neurological disease commonly manifesting as brachial monomelic amyotrophy in young males of Asian origin, easily understood as juvenile non-progressive cervical amyotrophy. The first case was reported by Hirayama in 1959. The pathogenesis is attributed towards chronic compression of cervical spinal cord during flexion movements of neck in cases where there is detachment of posterior dura mater. This chronic event, invariably leads to features of cord atrophy along with other MRI features...
August 2015: Journal of Clinical and Diagnostic Research: JCDR
Veeramani Preethish-Kumar, Atchayaram Nalini, Ravinder-Jeet Singh, Jitender Saini, Chandrajit Prasad, Kiran Polavarapu, Kandavel Thennarasu
Our objective was to characterize the MR imaging features in a large and distinct series of distal bimelic amyotrophy (DBMA) from India. We utilized a retrospective and prospective study on 26 cases. Results demonstrated that upper limb distal muscle wasting and weakness was predominantly symmetrical in onset. Mean age at onset was 20.9 ± 7.0 years, mean duration 83.0 ± 102.6 months. MRI carried out in 22 patients with flexion studies showed forward displacement of posterior dura in 19 (86.4%). Crescent shaped epidural enhancement on contrast was seen in 20/24 cases (83...
2015: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Kenji Sekiguchi, Takanori Saito, Ichiro Yokota, Hisatomo Kowa, Fumio Kanda, Tatsushi Toda
A 65-year-old woman experienced progressive intrinsic muscle wasting on the right hand over a period of 7 years. The distribution of muscular atrophy and weakness was consistent with the area innervated by the right C8 and Th1 nerve roots. Neurophysiological examination suggested a right lower trunk lesion. An elongated right transverse process of the C7 vertebra and an aberrant subclavian artery were detected on computed tomography images, and the right lower trunk of the brachial plexus appeared to be lifted upward on magnetic resonance images...
2015: Rinshō Shinkeigaku, Clinical Neurology
Seung Don Yoo, Hee-Sang Kim, Dong Hwan Yun, Dong Hwan Kim, Jinmann Chon, Seung Ah Lee, Sung Yong Lee, Yoo Jin Han
Monomelic amyotrophy (MMA), also known as Hirayama disease, is a sporadic juvenile muscular atrophy in the distal upper extremities. This disorder rarely involves proximal upper extremities and presents minimal sensory symptoms with no upper motor neuron (UMN) signs. It is caused by anterior displacement of the posterior dural sac and compression of the cervical cord during neck flexion. An 18-year-old boy visited our clinic with a 5-year history of left upper extremity pain and slowly progressive weakness affecting the left shoulder...
February 2015: Annals of Rehabilitation Medicine
Lisa M DeGregoris, Murray Engel
No abstract text is available yet for this article.
August 5, 2014: Neurology
Atchayaram Nalini, Mandavilli Gourie-Devi, Kandavel Thennarasu, Aravinda Hanumanthapura Ramalingaiah
Our objective was to study the clinical characteristics and natural history of monomelic amyotrophy (MMA). We used a retrospective study of 279 patients diagnosed to have either upper (Hirayama disease) or lower limb MMA. Results showed that brachial MMA (BMMA) occurred in 224 patients (male:female, 9:1). Mean age of onset was 19.5 ± 4.18 years. Progression occurred over less than five years in the majority (95.9%) of patients. Duration at the last follow-up was: up to five years in 61.4%, 5-10 in 21.3%, 10-15 in 7...
September 2014: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
Shumaila Arooj, Fatima Mubarak, Muhammad Azeemuddin, Zafar Sajjad, Wasey Jilani
Hirayama Disease is a disease of young adults lying in the age group between twenty to thirty years. It is an extremely uncommon disorder. Its other synonyms are juvenile muscular atrophy of the distal upper extremity (JMADUE) or monomelic amyotrophy (MMA). A previously healthy 25-year-old man presented with gradually increasing weakness in both hands for the past few years. There was neither history of trauma nor family history of neuromuscular disease. MRI was advised. Routine cervical sagittal MR images (Non-flexion or extension) revealed cord flattening and atrophy at C5 to C7 levels...
November 2013: JPMA. the Journal of the Pakistan Medical Association
Mário Correia de Sá, Henrique Costa, Sérgio Castro, Marta Vila Real
Hirayama disease, also known as monomelic amyotrophy or juvenile spinal muscular atrophy of the distal upper extremity features the impairment of the anterior horn cells of the distal cervical spinal cord secondary to dural sac anterior displacement during cervical flexion. We describe a case of a 17-year-old boy with a history of scoliosis, evaluated in the emergency department for decreased muscle strength and atrophy of the left upper limb with progressive worsening for about 6 months. We performed electrophysiological studies that showed severe neurogenic atrophy involving the C7-T1 left myotomes...
2013: BMJ Case Reports
Kaukab Maqbool Hassan, Hirdesh Sahni
Since its original description by Keizo Hirayama in 1959, "juvenile muscular atrophy of the unilateral upper extremity" has been described under many nomenclatures from the east. Hirayama disease (HD), also interchangeably referred to as monomelic amyotrophy, has been more frequently recognised in the west only in the last two decades. HD presents in adolescence and young adulthood with insidious onset unilateral or bilateral asymmetric atrophy of hand and forearm with sparing of brachioradialis giving the characteristic appearance of oblique amyotrophy...
2013: BioMed Research International
Feizhou Lu, Hongli Wang, Jianyuan Jiang, Wenjun Chen, Xin Ma, Xiaosheng Ma, Xinlei Xia, Lixun Wang
OBJECT: Monomelic amyotrophy (MMA) is a benign, self-limiting lower motor neuron disease. Optimal surgical strategies-discectomy decompression and fusion (DDF) or corpectomy decompression and fusion (CDF)-for patients with aggravated symptoms (within 6 months of presentation) are controversial, particularly in those who are ineligible for conventional treatment. These 2 methods of anterior cervical decompression and fusion for MMA in patients unwilling or unable to wear a conventional cervical collar long term were evaluated...
October 2013: Journal of Neurosurgery. Spine
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