Monomelic Amyotrophy

Gourie‐Devi, M; Suresh, T N; Shankar, S. K.

doi:10.1001/archneur.1984.04050160050015

Cited by 146 publications

(135 citation statements)

References 3 publications

(4 reference statements)

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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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Section: Surgical Indication and Methodsmentioning

confidence: 99%

Pathophysiology and treatment for cervical flexion myelopathy

et al. 2002

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“…Someauthors suspect the disease to be a variant of motor neuron disease (37,39,41,53,57,58), which had been discussed in Japan, but was disregarded after the first autopsy described above. Some authors (42,46,49,50,53,57) include the disease as "monomelicamyotrophy" that means muscular wasting limited in any one of upper or lower limbs and implies focal motor neuron degeneration. Wehad a similar argument of "glove and stocking type amyotrophy" in 1960's (59).…”

Section: Reports From Other Countriesmentioning

confidence: 99%

Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease).

2000

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This disease is characterized by initially progressive muscular weakness and wasting of the distal upper limb(s) in young people predominantly in men, followed by a spontaneous arrest within several years. This disease has been thought to be separate from motor neuron diseases, yet some authors still consider the illness a variant of motor neuron disease. However, the pathological evidence of ischemic changes in the lower cervical anterior horn should facilitate differentiation of the disorder from degenerative motor neuron disease. Recent radiological investigations proved compressive flattening of the lower cervical cord due to forward displacement of the cervical dural sac and spinal cord induced by neck flexion. These findings suggest that sustained or repeated neck flexion may cause ischemic changes in the cervical anterior horn. Application of a cervical collar to minimize neck flexion prevents progressive muscular weaknessin an early stage of the disease.

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“…It is inherited as an autosomal recessive disorder although sporadic cases do occur [7]. Despite continued interest in the developed countries, there is little information on SMA in the Arabian peninsula and developing communities [8][9][10][11][12][13][14][15][16]. This communication reports the results of a clinical study of SMA type I conducted in a teaching hospital in the Eastern Province of Saudi Arabia.…”

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confidence: 99%

Werdnig Hoffman'S Disease (Spinal Muscular Atrophy Type I): A Clinical Study of 25 Saudi Nationals in Al-Khobar

et al. 1992

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We describe the clinical features of 25 cases of Werdnig Hoffman's disease (spinal muscular atrophy (SMA) type I) seen prospectively over a two-year period at the King Fahd Hospital of the University (KFHU), Al-Khobar. The hospital incidence rate was 1.93 per 1,000 live births (95% confidence limits, 0.80-3.06/1,000). The estimated prevalence rate for the community was 0.92/10,000 with 0.59-1.25 per 10,000 children as its 95% confidence limits. The male to female ratio was 2:3. Reduced fetal movements were reported by six mothers; 8 children (32%) had symptoms at birth, and 24 (96%) had symptoms by the time they were six months old. Other features apart from hypotonia, muscle weakness, and absent deep tendon reflexes included head lag with inability to achieve head control at six months (88%), respiratory problems consisting of difficulty with breathing or frequent chest infections (44%), and difficulty with feeding (40%). Wasting with fasciculations of the tongue was seen in 64%. Death occurred within six months of presentation in 75% of the cases. The parents were consanguineous in 64% of the cases. This high consanguinity rate was probably the major cause for the high population prevalence rate. Al-Khobar. 1992; 12(1): 67-71 Spinal muscular atrophy (SMA) consists of a group of disorders that present with generalized muscle weakness, muscle atrophy and areflexia secondary to degeneration of the anterior horn cells and bulbar motor nuclei without involvement of the pyramidal tracts [1]. Although recent studies have defined the genetic locus for SMA on chromosome 5q, distinct syndromes are still recognized [2][3][4][5]. The criteria that have been used to classify SMA include the age of onset, mode of inheritance and subsequent clinical course. The resulting heterogenous group of disorders have varying degrees of morbidity and mortality.SMA is the second most common neuromuscular disorder in children [1]. Werdnig Hoffman'sdisease (SMA type I), its most severe form, presents in infants, accounts for 25% of all cases and runs a progressive clinical course culminating in death by the age of three years in the majority of cases [6]. It is inherited as an autosomal recessive disorder although sporadic cases do occur [7]. Despite continued interest in the developed countries, there is little information on SMA in the Arabian peninsula and developing communities [8][9][10][11][12][13][14][15][16]. This communication reports the results of a clinical study of SMA type I conducted in a teaching hospital in the Eastern Province of Saudi Arabia. Patients and MethodsAll Saudi children who presented with muscle weakness or hypotonia at the Pediatric and other outpatient clinics of the King Fahd Hospital of the University (KFHU), Al-Khobar, Saudi Arabia between January 1, 1986 and December 31, 1987 were evaluated by the Department of Neurology, KFHU, Al-Khobar.The families, of children with the diagnosis of SMA type I were re-interviewed to obtain detailed family and social histories. Relatives of the parents of the index...

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Monomelic Amyotrophy

Cited by 146 publications

References 3 publications

Pathophysiology and treatment for cervical flexion myelopathy

Pathophysiology and treatment for cervical flexion myelopathy

Juvenile Muscular Atrophy of Distal Upper Extremity (Hirayama Disease).

Werdnig Hoffman'S Disease (Spinal Muscular Atrophy Type I): A Clinical Study of 25 Saudi Nationals in Al-Khobar

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