Muscular Atrophy Of Upper Extremities Research Articles

Do patients with Hirayama disease require surgical treatment? A review of the literature.

The main clinic characteristic of Hirayama disease (HD) is atrophy of the distal muscles in the upper limbs. Recently, an increasing number of HD cases have been reported. Many HD patients have persistently progressive symptoms and conservative treatments failed. This article aims to review the current status of the field and summarizes the main surgical treatment options for patients with HD. A comprehensive search of the PubMed and the Web of Science databases was conducted from their inception to September 15th, 2022. Search terms included "juvenile muscular atrophy of upper extremity", "Hirayama disease" and "surgery". A total of 169 relevant publications were identified and 29 articles were finally reviewed. Current surgical treatments for HD are either anterior cervical surgery or posterior cervical surgery. The two approaches can effectively stop the disease. However, no studies have compared the advantages and limitations of the two surgical methods. The previous view that HD can be improved with conservative treatment has been challenged. In many studies, surgical treatment has been shown to improve the hand function in patients with HD. However, there is still controversy about the methods of anterior and posterior cervical surgery. Future research could focus on exploring the advantages and limitations of different surgeries.

Advances in research of cervical spondylotic amyotrophy

Cervical spondylotic amyotrophy (CSA) has attracted more and more attention in recent years, according to early studies, it is based on cervical degeneration, and mainly characterized by segmental, asymmetric and stable muscular atrophy of upper extremities, with or without mild sensory abnormalities and spinal cord lesions (manifested as lower extremity symptoms and gait abnormalities), also known as Keegan's cervical spondylosis. This review will focusing on recent research progress of CSA, and summarize and analyze the definition, pathogenesis, clinical manifestations, diagnosis and treatment of cervical spondylosis muscular atrophy.

Hirayama disease: analysis of cases in Russia.

The fourteen cases of Hirayama disease (HD) are presented in this article. HD is seldom disease characterized by juvenile muscular atrophy of upper extremities and benign course. All cases were diagnosed in the Research Center of Neurology (Moscow, Russia) during the year 2015-2017. Such methods as MRI (magnetic resonance imaging), EMG (electromyography), and NCS (nerve conduction studies) have been used to confirm diagnosis of HD. Transcranial magnetic stimulation was used to exclude upper motor neuron involvement in two cases. The original scale of neurological disturbances in HD has been proposed by authors to reveal correlations of HD severity with age of patients and duration of disease.Most of patients with HD are young males with common clinical signs. Detected MRI and EMG data were also comparable with previous publications. Independence of HD severity from age and duration of the disease may be the result of individual physical characteristics of dura mater and other structures of the cervical vertebra. In some our cases, amyotrophic lateral sclerosis and other neurological disorders were misdiagnosed before. In view of different prognosis in these pathologies and possible correction of HD, early diagnosis is very important.

AB1273 Virtual anatomy of cervical nerve roots by 3-D MRI/CT fusion imaging

BackgroundBoth CT and MRI are widely known to be important tools in diagnosing spinal disorders. However, even when three-dimensional data are collected, images are often presented in two dimensions and...

Segmental muscular atrophy of the distal upper extremity (Hirayama disease): An atypical case with anterior dural detachment

Segmental non-progressive spinal muscular atrophy of the upper extremity, also known as Hirayama disease (HD), is a disorder seen mainly in young male patients of Asian ancestry. On MRI loss-of-attachment between the posterior dural sac and adjacent lamina has been described as the only significantly important predictor of HD. We herein present a patient with HD who showed an atypical anterior detachment of the dura mater from the adjacent vertebral body.

Lack of serum neutralizing antibody against poliovirus in patients with juvenile distal spinal muscular atrophy of upper extremities

Serum neutralizing antibodies for type 1, 2 and 3 poliovirus were studied in 15 Chinese patients in Taiwan with juvenile distal spinal muscular atrophy of the upper extremities and 15 age-matched normal subjects. Significantly lower serum antibody titers were found in the study group, suggesting that patients with this chronic focal form of acquired motor neuron disease are, to some degree, immunologically unresponsive to the neutralizing epitope of poliovirus.

Computed tomographic myelography characteristics of spinal cord atrophy in juvenile muscular atrophy of the upper extremity

Although atrophy of the lower cervical and upper thoracic cord in juvenile muscular atrophy of distal upper extremity has been reported, the atrophic patterns of the cord, especially in the transverse section, have not been studied extensively. The aim of this study is to clarify the atrophic patterns of the cord by CT myelography (CTM) and to discuss the pathogenesis of cord atrophy. Sixteen patients with juvenile muscular atrophy of distal upper extremity were examined by CTM. Atrophy of the lower cervical and upper thoracic cord, consistent with the segmental weakness, was seen in all patients. Flattening of the ventral convexity was a characteristic atrophic pattern of the cord. Bilateral cord atrophy with dominance on the clinical side. There was no correlation between the degree of cord atrophy and duration of symptoms. Flattening of the ventral convexity, associated with purely motor disturbances, reflects selective atrophy of the anterior horns in the cord, which is attributable to chronic ischemia. Cord atrophy proved to precede clinical manifestations. The characteristic atrophy of the cord provides useful information to confirm the diagnosis without long-term observation.

Juvenile distal spinal muscular atrophy of upper extremities in Chinese males: a single fibre electromyographic study of arms and legs.

Single fibre electromyography (SFEMG) was performed on six young Chinese males with distal spinal muscular atrophy of the upper extremities. Abnormal SFEMG findings of increased fibre density, jitter and blocking were recorded over both arms and legs in all patients, suggesting a more generalised disturbance than would appear clinically.

Chronic segmental spinal muscular atrophy of upper extremities in identical twins

We present the 1st report of chronic segmental spinal muscular atrophy confined to the upper extremities in identical male twins. This occurrence in identical twins, together with reports of siblings and parent-child pairs of a disorder phenotypically similar to the more common sporadic form in the literature, suggests a genetic etiology in some cases.

Non familial juvenile distal spinal muscular atrophy of upper extremity.

An uncommon variety of non familial, juvenile onset, spinal muscular atrophy with asymmetric distal upper extremity affection is described. One hundred and two patients with a one to 14 year follow up are analysed. Spinal muscular atrophies with a distal distribution are rare. However, in the past three decades, previously unrecognised varieties of neurogenic muscular atrophy have been described in Asia (Japan, India, Sri Lanka and Singapore) under a variety of names. These provide interesting data for discussion of Asian neurogenic muscular atrophies with distal affection, in the context of diseases of the motor neuron.

Benign juvenile focal muscular atrophy of upper extremities — A familial case

A 53-year-old man and his 18-year-old son, both suffering from benign focal muscular atrophy of upper extremities are described. To our knowledge this is the first familial case of this disease in Caucasians.

Separation and establishment of a disease entity--proposal of a disease entity "juvenile type of distal and segmental muscular atrophy of upper extremities"

Separation and establishment of a disease entity--proposal of a disease entity "juvenile type of distal and segmental muscular atrophy of upper extremities"

Juvenile type of distal and segmental muscular atrophy of upper extremities.

Seventy-one cases of distal and segmental muscular atrophy of the upper extremities with juvenile onset were studied. The clinical features consisted of: juvenile onset, male preponderance, unique distribution of the muscular atrophy in the hand and forearm, tendon reflexes hypoactive in most cases but hyperactive in some, no definite sensory disturbances, no involvement of the cranial nerves, and autonomic nerve disorders in the affected region. There was rapid progression during the 2 to 3 years after onset with a slowly progressive course thereafter. There were no abnormal laboratory findings except for electrophysiological and morphological findings of the affected muscles. The site of lesion was surmised to be from the C5 to T1 spinal segments with intramedullary involvement. The cause is unknown.