Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0002736 (amyotrophic lateral sclerosis)
 19,048 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 21-year-old Thai man presented with progressive weakness and atrophy of both hands for 2 years. His left leg was atrophic and weak secondary to poliomyelitis since he was 8 months old. Physical examination showed that there was atrophy of both hands and forearms as well as left leg. Sensation was normal. Deep tendon reflexes were normal except for hyporeflexia of left leg. EMG showed chronic denervation pattern in the recent atrophic muscles and in normal power muscles. Motor and sensory nerve conductions were normal. Biopsy of forearm muscle revealed degeneration and fibrosis of muscle fibers. These findings were compatible with post-polio syndrome (PPS) which presents in patients with late paralysis following poliomyelitis. It is not a form of amyotrophic lateral sclerosis although some clinical similarities exist. Weakness from PPS may lead to musculoskeletal pain and increased functional disability. Proper rehabilitation program is to maintain his functional status and allow him to continue to live as independently as possible by regular exercise without stressing joints and muscles.
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PMID:Post-polio syndrome: a review and case report. 130 92

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

Generalized weakness, intermittent dysphagia, and a 40-pound weight loss developed in an elderly man over a six-month period. Examination revealed weakness, atrophy and fasciculations of extremity musculature, pseudobulbar speech, hyperactive upper extremity reflexes, and extensor toe signs without sensory loss. Results of electrodiagnostic studies were consistent with an axonal polyneuropathy. Endocrinologic results were compatible with hyperthyroidism. Radioiodine therapy resulted in resolution of clinical neurologic symptoms and signs within seven months. This case illustrates a previously undescribed concurrence of hyperthyroid associated polyneuropathy and pyramidal tract dysfunction that led to an initial clinical diagnosis of amyotrophic lateral sclerosis.
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PMID:Pyramidal tract deficits and polyneuropathy in hyperthyroidism, Combination clinically mimicking amyotrophic lateral sclerosis. 299 Feb 4

A case of motor neuron disease with clinical and pathological resemblance to amyotrophic lateral sclerosis (ALS) in a woman who was severely bitten on the ankle by a cat is described. Weakness first appeared at the ankles and relentlessly advanced proximally, terminating in death from pulmonary failure in a year. A number of unusual features that are uncharacteristic of ALS were found that included a markedly elevated antinuclear antibody titre in the serum and the presence of prominent oligoclonal bands in the cerebrospinal fluid. The spinal cord showed loss of anterior horn cells and pyramidal degeneration that are characteristic of ALS, but an extraordinary finding was the presence of transmural granulomatous inflammation of numerous small and medium sized vessels, especially veins, in the subarachnoid space around the cord. There were also inflammatory changes in the brainstem and spinal cord consisting of microglial and astrocytic nodules and perivenous cuffing by mononuclear cells. Ill-defined hyaline eosinophilic cytoplasmic inclusions were seen in some remaining anterior horn cells. No viral particles were found by electron microscopy despite an extensive search. Virological studies were inconclusive but there is reason to believe that this patient's illness was caused by an as yet unidentified virus.
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PMID:An unusual form of motor neuron disease following a cat bite. 371 65

Amyotrophic lateral sclerosis (ALS) is a progressive neurodegenerative disease characterized by significant speech and swallowing problems resulting from upper and lower motor neuron loss. Weakness is the primary ALS disease-related sign, and measures of muscle strength have revealed nonuniform patterns of muscle weakness in orofacial muscles. To a large extent, muscle strength measures in these studies have not been evaluated in terms of functional significance, and few researchers have addressed the relation between weakness and motor neuron loss. This study addressed whether multiple measures, including static isometric maximum voluntary contraction (MVC), a dynamic measure of the peak rate of change of force (PRCF), an upper motor neuron (UMN) index, and a functional disability score (FDS) might enhance understanding of speech dysfunction in ALS. Ten males diagnosed with sporadic ALS showing mild speech impairment and an equal number of matched controls were studied. Tongue MVC and PRCF were more impaired than those of the lip and jaw, irrespective of the time post onset and site of initial symptoms. Results also suggested that disproportionate tongue impairment may be related to UMN deficits. However, impairments in the rate of contraction did not appear to be related to UMN deficits. Tongue weakness and tongue and lower lip PRCF were related to the degree of speech severity, but none of the measures was related to speech intelligibility. The value of a functional outcome measure like speech intelligibility and its role in characterizing orofacial involvement in the early stages of ALS bulbar impairment are discussed.
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PMID:Multiple orofacial indices in amyotrophic lateral sclerosis. 811 82

A 63-year-old woman had suffered from progressive weakness and wasting of the right lower limb for seven years. In the lower limbs, there were profound muscular atrophy and weakness on the right, and mild weakness with spasticity on the left. Muscle strength of the upper limbs was normal. Sensory examination was normal except for paresthesias below the right knee. Anti-HTLV-I antibody titer was raised both in serum and in the CSF. Motor nerve conduction studies of the lower limbs showed small compound muscle action potentials and somewhat slow conduction velocities, more evident in the right side. F-wave was evoked only in the left tibial nerve and its latency was increased. Sensory nerve conduction studies were normal in the lower limbs. Somatosensory evoked potentials (SEPs) after tibial nerve stimulation at the ankles showed increased interpeak latencies between lumbar N20 and scalp P37. Nerve conduction studies in the upper limb were normal. Single fiber electromyography suggested anterior horn involvement not only in the lumbosacral cord but also in the cervical cord. Weakness and spasticity improved after oral administration of prednisolone. The SEPs findings and a favorable response to prednisolone excluded the possibility of amyotrophic lateral sclerosis. This case is a clinical variant of HTLV-I-associated myelopathy presenting profound atrophy of unilateral lower limb.
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PMID:[HTLV-I associated myelopathy presenting with profound atrophy of unilateral lower limb]. 825 34

Riluzole, a benzothiazole, affects neurons by 3 mechanisms: by inhibiting excitatory amino acid release, inhibiting events following stimulation of excitatory amino acid receptors and stabilising the inactivated state of voltage-dependent sodium channels. It has demonstrated neuroprotective activity in vivo and in vitro. Results from 2 randomised double-blind placebo-controlled trials in patients with amyotrophic lateral sclerosis (ALS; motor neuron disease) have demonstrated that riluzole can extend survival and/or time to tracheostomy. After 18 months, the relative risk of death or tracheostomy with riluzole 100 mg/day was reduced by 21%. Although riluzole slowed the rate of deterioration in muscle strength in the first trial, this was not confirmed in the second, larger trial. Riluzole had no effect on any other functional or secondary variable. Gastrointestinal effects, anorexia, asthenia, circumoral paraesthesia and dizziness were reported more frequently with riluzole than placebo. Elevated alanine aminotransferase levels were observed in 10.6 versus 3.8% of patients treated with riluzole 100 mg/day versus placebo, leading to treatment withdrawal in 3.8 versus 2.1% of patients. In conclusion, riluzole is the first drug that has been shown to have an effect on survival in patients with ALS. Although the effect of riluzole was modest, it has allowed some insight into the pathogenesis of ALS from which future gains may be made.
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PMID:Riluzole. A review of its pharmacodynamic and pharmacokinetic properties and therapeutic potential in amyotrophic lateral sclerosis. 889 67

ALS is a progressive motor neuron disease with no effective treatment. The anti-excitotoxic drug riluzole (100 mg/day) has been shown to decrease mortality and muscular deterioration in ALS patients. To confirm and extend the therapeutic effect of riluzole, we performed a double-blind, placebo-controlled, multicenter, international, dose-ranging (50, 100, 200 mg/day), stratified study in 959 ALS outpatients treated for up to 18 months. Primary efficacy criterion was survival and the effect of treatment was analyzed before (Wilcoxon and log rank tests) and after adjustment on prognostic factors (Cox model). Secondary efficacy criterion was disease progression assessed through change in functional measures. Tracheostomy-free survival rates were: 50.4% (placebo), 55.3% (50 mg riluzole) (p = 0.23, Wilcoxon test; p = 0.25, log-rank test), 56.8% (100 mg riluzole) (p = 0.05, Wilcoxon test; p = 0.076, log-rank test), and 57.8% (200 mg riluzole) (p = 0.061, Wilcoxon test; p = 0.075, log-rank test). At the end of the 18-month study, there was a significant dose-related decrease in risk of death or tracheostomy (p = 0.04). Adjustment for baseline prognostic factors showed a 35% decreased risk of death with the 100-mg dose compared with placebo (p = 0.002). No significant treatment effects were detected for the functional assessments. The most frequent dose-related adverse events included nausea, asthenia, and elevated liver enzyme levels. This study confirms the therapeutic effect of riluzole in a large representative ALS sample, over an 18-month period. Riluzole is well tolerated and decreases the risk of death or tracheostomy in ALS patients.
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PMID:A confirmatory dose-ranging study of riluzole in ALS. ALS/Riluzole Study Group-II. 895 96

Preclinical investigations indicated that recombinant human ciliary neurotrophic factor (rhCNTF) may have potential as therapy for amyotrophic lateral sclerosis (ALS). We evaluated the safety and efficacy of rhCNTF in a prospective, double-blind, placebo-controlled trial in 570 patients with ALS. Patients were randomized to receive 0.5, 2, or 5 micrograms/kg/day rhCNTF, or placebo, for 6 months. The primary efficacy end point was the change from baseline to the last on-treatment value of a combination megascore for limb strength (maximum voluntary isometric contraction) and pulmonary function. Secondary end points included individual arm and leg megascores, pulmonary function tests, an activities-of-daily-living outcome measure, and survival. The four treatment groups were similar at baseline with respect to age, sex, disease duration, and muscle strength values. At all doses tested, rhCNTF had no beneficial effect on the primary or secondary end points. Certain adverse events, as follows, appeared to be dose related: injection site reactions, cough, asthenia, nausea, anorexia, weight loss, and increased salivation. There was an increased number of deaths at the highest dose level. rhCNTF had no beneficial effect on any measure of ALS progression. There were increased adverse events in the 5 micrograms/kg group and increased deaths.
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PMID:A placebo-controlled trial of recombinant human ciliary neurotrophic (rhCNTF) factor in amyotrophic lateral sclerosis. rhCNTF ALS Study Group. 896 57

Amyotrophic lateral sclerosis (ALS) is a rapidly fatal degenerative disorder of the motoneurones which was without any effective therapy until 1997. Riluzole (Rilutek) has been the first patented drug used in its specific treatment. In order to evaluate the tolerability profile of this molecule, a Pharmacovigilance study was undertaken in the Department of Neurology B at the Montpellier University Hospital. A total of 153 patients were studied and all observed side-effects were listed in the French bank of Pharmacovigilance. Riluzole induced one or more adverse effects in 50.3 per cent of patients. The most frequent were gastrointestinal disturbances, hepatotoxicity and asthenia. Dermatological, haematological, neuropsychiatric and metabolic side-effects were also reported. This study shows an acceptable safety profile for riluzole. Due to its mode of action, riluzole could potentially be used in the treatment of other neurodegenerative diseases involving glutamate excitotoxicity. Subsequently, Pharmacovigilance will have to be carried out to establish the proper use of riluzole.
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PMID:[Adverse efects of riluzole (Rilutek) in the treatment of amyotrophic lateral sclerosis]. 1096 3


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