Juvenile Muscular Atrophy Localized to Arms

Singh, Nidhi; Sachdev, Kuldip K.; Susheela, A. K.

doi:10.1001/archneur.1980.00500540075011

Cited by 70 publications

(27 citation statements)

References 12 publications

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“…As a disease entity JLCSMA has been re ported for 30 years, since the first description by Hirayama et al [5], Except for 20 cases reported elsewhere [l-4], most cases have been described in Asians, including 122 cases from Japan [5][6][7][8][9], 102 cases from Sri Lanka [10], 37 cases from India [11,12], 19 cases from Malaysia [13], and 6 cases from Hong Kong [14], Ethnic factors likely exist. How ever, the incidence among Chinese, one of the largest populations, has been strangely rare, comprising only 7 cases from Malaysia [13] and 6 cases from Hong Kong [14], Further more, no case among descendants of Asian immigrants has been reported from the rest of the world.…”

Section: Discussionmentioning

confidence: 99%

Juvenile Lower Cervical Spinal Muscular Atrophy in Taiwan: Report of 27 Chinese Cases

Kao¹,

Wu²,

Chern³

1993

Neuroepidemiology

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Clinical observations as well as electromyographic studies were carried out on 27 Taiwanese Chinese patients deemed to be suffering from a rare, unique and self-limiting variant of lower motor neuron disease affecting the upper limbs with juvenile onset and a marked male preponderance. The results agree with previous descriptions of the disease except for the higher incidence of triceps affection. The term ''juvenile lower cervical spinal muscular atrophy'', which describes the disease more accurately, is being proposed. The geographic relevance is discussed.

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Section: Discussionmentioning

confidence: 99%

Juvenile Lower Cervical Spinal Muscular Atrophy in Taiwan: Report of 27 Chinese Cases

Kao¹,

Wu²,

Chern³

1993

Neuroepidemiology

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“…The disorder, called juvenile-type dis tal and segmental muscular atrophy of upper extremi ties, had such characteristic features that it has become regarded as an established clinical entity [6,7,15,16,18]. In addition, three groups of patients have been reported with clinical involvement other than the distal arm muscles [4,13,20], In Western countries the disorder is rare.…”

Section: Discussionmentioning

confidence: 99%

“…Despite considerable variations in the distribu tion of muscle weakness and wasting, these subtypes of spinal muscular atrophy have much in common: spo radic occurrence; male preponderance; insidious onset; apparent non-progressive course, and lack of involve ment of the cranial nerves, brain stem, pyramidal tracts and sensory system [1,[3][4][5][6][7][8][9][11][12][13][14][15][16][17][18][19][20]. There is an even more localized form where only one limb is affected, most of the patients described being Japanese, Malay sian and Indian [4,6,7,13,15,16,18,20], So far, to our knowledge, only 25 patients have been reported in West ern countries with one limb affected [1,3,5,8,9,11,12,14,17], The present study was undertaken to investigate whether electromyography (EMG) and computed to mography (CT) of the skeletal musculature could offer useful additional information on muscle involvement in monomelic spinal muscular atrophy.…”

Section: Introductionmentioning

confidence: 99%

Electromyographic and Computed Tomographic Findings in Five Patients with Monomelic Spinal Muscular Atrophy

Visser

Visser²,

Verbeeten

1988

Eur Neurol

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Five patients with monomelic spinal muscular atrophy are described. Clinical features included insidious onset of wasting and weakness of one limb, lack of involvement of the cranial nerves, brain stem, pyramidal tracts and sensory system, and a stable condition over a period of 4–20 years. Clinical findings, electromyography and/or muscle biopsy were consistent with anterior horn cell lesion. Central cavities were excluded by magnetic resonance imaging studies of the spinal cord. Computed tomography of skeletal musculature and electromyography indicated more diffuse lower motor neuron involvement by revealing abnormalities in clinically unaffected muscles in 4 of the 5 patients. Myokymic discharges were found in the affected limb of 1 patient.

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“…Previous case reports of CFM emanate predominantly from Japan; reports of non-Japanese patients are very rare [2,5,7,39,40,42,45]. However, CFM can be inappropriately treated as entrapment neuropathy, such as cubital tunnel syndrome, mainly due to muscular atrophy of the C7-T1 myotomes, or as a motor neuron disease due to the absence of sensory disturbance.…”

Section: Surgical Indication and Methodsmentioning

confidence: 99%

Pathophysiology and treatment for cervical flexion myelopathy

et al. 2002

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IntroductionReid [43,44] and Breig and colleagues [3,4], on the basis of a cadaveric study, hypothesized that spinal cord compression by the vertebral bodies and intervertebral discs during neck flexion caused cervical myelopathy. Later, the pincer mechanism of the cervical spine became emphasised, and neck extension was thought to be the predominant pathology of cervical myelopathy [41]. HowAbstract Previous studies have suggested that spinal cord compression by the vertebral bodies and intervertebral discs during neck flexion cause cervical flexion myelopathy (CFM). However, the exact pathophysiology of CFM is still unknown, and surgical treatment for CFM remains controversial. We examined retrospectively patients with CFM based on studies of the clinical features, neuroradiological findings, and neurophysiological assessments. The objectives of this paper are to investigate the pathophysiology of CFM, and to examine an optimal surgical treatment. Twenty-three patients (20 male, three female) with age of onset ranging from 11 to 23 years (mean 15.7 years) were examined for the study. All patients were inspected by magnetic resonance imaging (MRI), myelogram, or computed tomographic myelogram (CTM) of the cervical spine. In eight patients, dynamic motor evoked potentials (MEP) studies were performed. Five patients underwent surgical treatment; two patients had cervical duraplasty with laminoplasty, two patients had musculotendinous transfer, one patient had both of these procedures, and the remaining 18 patients were treated conservatively. Amyotrophy of the hand intrinsic and flexor muscle group of the forearm except the brachioradial muscle was observed hemilaterally in 20 patients and bilaterally in three patients. In three patients, T1-weighted MRI with neck flexion showed linear high intensity regions in the epidural space. In all patients, axial MRI/CTM demonstrated flattening of the spinal cord with the posterior surface of the dura mater shifting anteriorly. The amplitude of MEPs decreased after cervical flexion in two patients with progressive muscular atrophy. In three patients, dysesthesia of the upper extremities disappeared following cervical duraplasty. Musculotendinous transfer for three patients significantly improved the performance of their upper extremity. The findings of this study suggest that degenerative changes of the dura mater may be a characteristic pathology of CFM. Cervical duraplasty with laminoplasty is effective for cases at an early stage, and musculotendinous transfer should be selected in patients at a late stage.

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Juvenile Muscular Atrophy Localized to Arms

Cited by 70 publications

References 12 publications

Juvenile Lower Cervical Spinal Muscular Atrophy in Taiwan: Report of 27 Chinese Cases

Juvenile Lower Cervical Spinal Muscular Atrophy in Taiwan: Report of 27 Chinese Cases

Electromyographic and Computed Tomographic Findings in Five Patients with Monomelic Spinal Muscular Atrophy

Pathophysiology and treatment for cervical flexion myelopathy

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