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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Weakness of the hand is a common problem in the elderly. When joint and vascular diseases have been ruled out, a systematic anatomic approach to the possible neurologic causes can be used to sort through nonspecific symptoms and the complexity of hand anatomy. Syndromes of the peripheral nerves supplying the hand (ie, median, ulnar, radial) are common and are generally caused by compression of these nerves in their pathway through the elbow, forearm, or wrist. Other common causes of hand weakness include lower motor neuron syndromes involving the brachial plexus; cervical radiculopathy amyotrophic lateral sclerosis; and myasthenia gravis. Along with a careful history and physical examination, several tests are helpful in discerning the nature and location of lesions. Electromyography, magnetic resonance imaging, computed tomography, and myelography may all be used to exclude or support a diagnosis.
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PMID:Hand weakness in elderly patients. 253 9

Morphologic study of the spinal cord of a patient with generalized motor deficits revealed changes in the anterior horns characterized by the selective loss of large motor neurons, gliosis and the abnormal accumulation of 10 nm filaments which appeared as argyrophilic spheroids in the perikarya and axons of motor neurons. The ventral roots were predominantly affected and showed a variable loss of axons. The remaining axons displayed prominent onion-bulb formations, frequent axonal sprouting and occasionally evidence of active demyelination. The coexistence of a demyelinating motor radiculopathy and anterior horn changes simulating those of amyotrophic lateral sclerosis (ALS) may contribute to our understanding of the unresolved question of whether the neuronal perikaryon or its axon is the primary target in the pathogenesis of ALS. These observations also indicate that a rigid separation of pathogenetic mechanisms into neuronopathy, axonopathy and myelinopathy may not be always possible.
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PMID:Anterior horn changes of motor neuron disease associated with demyelinating radiculopathy. 301 67

Spondylotic degeneration can give rise to concurrent stenosis of the lumbar and cervical portions of the spinal canal in tandem. Symptomatic tandem spinal stenosis (TSS) is characterized by the triad of intermittent neurogenic claudication, progressive gait disturbance, and findings of mixed myelopathy and polyradiculopathy in both the upper and lower extremities. Nineteen patients with clinically symptomatic and myelographically proven disease were studied retrospectively. Surgical intervention was directed at decompression of the stenotic lesions in both the cervical and lumbar regions. The most symptomatic level was usually treated first. After a mean follow-up period of 22 months, an excellent outcome was obtained in five patients (26%), four improved (21%), five deteriorated despite initial improvement (26%), and one was unchanged. Three patients could not be traced for follow-up review, and there was one postoperative death. Postoperative improvement correlated inversely with symptom duration. Sphincter disturbance, radiculopathy, myelography, cerebrospinal fluid analysis, and electrophysiological data were not prognostically significant. The presentation of TSS mimics amyotrophic lateral sclerosis and other forms of motor-neuron disease. In contrast to these conditions, however, TSS is amenable to treatment. Operative sequence and technique could not be related to outcome. Functional recovery in TSS depends on early diagnosis and timely surgical intervention.
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PMID:Tandem lumbar and cervical spinal stenosis. Natural history, prognostic indices, and results after surgical decompression. 357 15

Somatosensory evoked potentials (SEPs) following median, ulnar and tibial nerve stimulation were recorded from sites over the shoulders, neck and scalp in 34 patients with cervical spondylosis. Twenty control subjects were matched for sex and age. Detailed clinical and radiological data were assembled, with particular attention to the sensory modalities impaired and the locus and severity of cord compression. The patients were divided clinically into 4 groups: combined myelopathy and radiculopathy (6 cases), myelopathy alone (15), radiculopathy (6) and neck pain (7). Four cases are described in detail. SEP abnormalities were strongly correlated with clinical myelopathy, but not with radiculopathy. Median and ulnar nerve responses were less often affected than tibial, even with myelopathy above C6 level. Tibial nerve SEP abnormalities were strongly correlated with posterior column signs on the same side of the body, but not with anterolateral column sensory signs. In myelopathy cases, the SEP examination appeared to be more sensitive to sensory pathway involvement than clinical sensory testing. SEP abnormalities were infrequent in cases of radiculopathy and neck pain, bearing no relation to the clinical locus of root lesions. Abnormal SEPs consistent with subclinical posterior column involvement, however, were recorded in 1 patient with radiculopathy and 2 with neck pain. Follow-up recordings made postoperatively in 7 myelopathy cases reflected the clinical course (improvement, deterioration or no change) in 4, but failed to reflect improvement in 3. The correlation of SEP findings with radiological data was generally poor. SEP abnormalities were detected in 6 out of 8 patients with clinical myelopathy but no radiological evidence of posterior cord compression, suggesting that impairment of the blood supply may be an important factor contributing to cord damage. An application for SEPs in the clinical management of cervical spondylosis may lie in the detection of posterior column involvement and the differential diagnosis from disorders such as multiple sclerosis and amyotrophic lateral sclerosis.
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PMID:Somatosensory evoked potentials in cervical spondylosis. Correlation of median, ulnar and posterior tibial nerve responses with clinical and radiological findings. 400 25

We studied the rate of initial "misdiagnosis', along with factors than might distinguish such patients, in 64 patients with amyotrophic lateral sclerosis (ALS) who completed a survey of 34 questions. Announcement of the survey was made by electronic newsletter and users group bulletin board directed at ALS patients. The questionnaire was distributed to interested ALS patients via electronic mail (e-mail), and 64 ALS patients (81% from the USA) returned their completed questionnaires via reply e-mail or postal mail. Seventeen patients (27% of total group) indicated at least 1 prior misdiagnosis, most commonly spinal stenosis/radiculopathy; 5 listed unnecessary and costly surgical treatments (laminectomy, endarterectomy). Misdiagnosis appeared to be more common in patients above age 60 and may be more common in patients originating in cities versus smaller communities. Mean time from onset of first symptom until definitive diagnosis of ALS was prolonged in patients with initial misdiagnosis (19 months) compared with non-misdiagnosed patients (10 months). Such a previous misdiagnosis may decrease a patient's chance of acceptance into multicenter ALS drug trials.
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PMID:The amyotrophic lateral sclerosis (ALS) patient perspective on misdiagnosis and its repercussions. 889 69

The term motor neurone disease encompasses combined upper and lower motor neurone disorders (amyotrophic lateral sclerosis), pure lower motor neurone disorders (spinal muscular atrophies, multifocal motor neuropathies, post irradiation lumbosacral radiculopathy, post-polio syndrome, hereditary bulbar palsy) and pure upper motor neurone disorders (primary lateral sclerosis, hereditary spastic paraplegia, neurolathyrism, Konzo). The chief clinical and electrophysiological criteria for these different disorders are discussed, with particular attention to diagnostically distinctive characteristics of each. Age of onset, and inheritance are considered as additional diagnostic features.
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PMID:Classification and clinical features of motor neurone diseases and motor neuropathies in adults. 1039 61

Traumatic axonal injury-induced apoptotic motor neuron cell death in neonatal rats is an established animal model used to study potential therapeutic agents in amyotrophic lateral sclerosis (ALS). In an analogous manner, trauma causing motor neuron axonal injury (which included focal neuropathy, plexopathy, and radiculopathy) preceded the onset of ALS in nine young adult men (age range, 28-43 years). The latency between the traumatic axonal injury and the onset of ALS symptoms in these patients ranged from 5 to 42 months (mean, 14.6 months).
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PMID:The latency between traumatic axonal injury and the onset of amyotrophic lateral sclerosis in young adult men. 1151 28

Cervical spondylosis is associated with myelopathy and radiculopathy, which sometimes mimic clinical manifestations of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder that affects upper and lower motor neurons. Cervical spondylosis may coexist with ALS because both diseases preferentially affect individuals of middle or old age. We investigated 63 patients with ALS to clarify the complications of cervical spondylosis and other spinal disorders and to explore the history of surgical treatment for them. We found cervical spondylosis in 30 patients (48%), lumbar spondylosis in 7 (13%), ossification of the posterior longitudinal ligament (OPLL) in 4 (6.3%), and ossification of the yellow ligament (OYL) in 4 (6.3%). Surgery was performed early in the course of the disease on the cervical spine in five patients (7.9%) and on the lumbar spine in one (1.6%); these patients consulted our clinic because their motor symptoms had progressed even after the spinal surgery. Our results indicate that nearly half of all ALS patients have their disease complicated by cervical spondylosis, and that a careful differential diagnosis for ALS is necessary before making decisions about spinal surgery.
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PMID:Amyotrophic lateral sclerosis: frequent complications by cervical spondylosis. 1464 82

Initial symptoms of amyotrophic lateral sclerosis (ALS) may mimic radiculopathy, myelopathy, mononeuropathy, or arthropathy. A retrospective review of 260 consecutive patients with ALS evaluated between 1996 and 2004 revealed that 55 (21%) had had surgery within the 5 years prior to ALS diagnosis. Thirty-four of these 55 (61%) had surgery for symptoms and signs that retrospectively were attributable to early manifestations of ALS. Misdiagnosis of early ALS may lead to unnecessary surgeries with their attendant potential complications.
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PMID:Inappropriate surgeries resulting from misdiagnosis of early amyotrophic lateral sclerosis. 1660 78

The aim of this review is to present the status of electrodiagnostic standards and guidelines in neuromuscular disorders. Electrodiagnostic guidelines are developed on the background of medical technology assessment, wherefore a short presentation of medical technology assessment is given covering: (1) Evidence-based medicine, i.e. "to do the right thing", describing practice parameters and the STARD initiative which introduces evidence-based medicine in electrodiagnostic medicine, (2) Continuous quality improvement, i.e. "to do the thing right", describing variation among laboratories in methods and interpretation of tests, and the need for medical audit and implementation of electrodiagnostic guidelines, (3) Outcome studies, i.e. "is it worthwhile to do the right thing right?". In electrodiagnostic medicine there are very few outcome studies. Standards and guidelines described in the literature for different neuromuscular disorders are presented, often as figures or tables. These cover guidelines developed in detail for CIDP by expert consensus multicentre groups by AAN, INCAT, EFNS/PNS and for other inflammatory demyelinating neuropathies are described, as well as guidelines differentiating between demyelinating pathophysiology and axonal loss by motor and sensory nerve conduction studies. Furthermore, electrodiagnostic guidelines for ALS as detailed in the El Escorial, the modified El Escorial and the recent supplementary Awaji criteria are described and presented in a comprehensive table. Only few electrodiagnostic guidelines are published for nerve entrapment, cervical radiculopathy and neuromuscular transmission failure whereas none are known for myopathy. If no electrodiagnostic criteria for a given disorder exist, criteria for the electrodiagnostic examination are described if present. It is concluded that future research is needed in order to develop more electrodiagnostic guidelines in neuromuscular disorders by international expert consensus groups. Such research should use an evidence-based medicine approach and medical technology assessment and include continuous quality development and outcome studies.
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PMID:Current status on electrodiagnostic standards and guidelines in neuromuscular disorders. 2067 84


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