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1/6. Problems and pitfalls in the diagnosis of ALS.

    Although misdiagnosis of amyotrophic lateral sclerosis (ALS) is rare, it may be more difficult to make a diagnosis in some groups of patients than in others. If a patient presents in the later stages of the disease, only a small number of alternative diagnoses need to be considered. These include spinal muscular atrophies of adult onset, inclusion body myositis and motor neuropathies with conduction block. The latter group in particular may present a serious diagnostic problem, as several groups have recently reported patients suffering from lower motor neuron syndrome without detectable conduction block, who responded unexpectedly to treatment with immunoglobulins. As recent laboratory results suggest that a lengthy pre-clinical period may precede clinical ALS, there is increased pressure for clinicians to make an early diagnosis so that the maximum effect can be achieved from neuroprotective drugs. Thus, diseases such as distal motor amyotrophies, pressure palsies of motor branches of hand nerves, and cervical myelopathies, which can be differentiated mainly by their time-course, may be relevant in the differential diagnosis of ALS in some patients. During recent years, a few patients have been seen in our clinic who presented with pure motor deficits but later developed a more complex pattern of vulnerability suggestive of multisystem degeneration. The existence of patients with a disease that borders the spectrum of motor neuron diseases cannot be disputed. These patients include those carrying the Huntington mutation and those suffering from guam and new guinea disease ('ALS/PD'). From our experience, however, these 'difficult' diagnoses represent less than 10% of the patients seen in our clinic.
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2/6. Exon 5 encoded domain is not required for the toxic function of mutant SOD1 but essential for the dismutase activity: identification and characterization of two new SOD1 mutations associated with familial amyotrophic lateral sclerosis.

    Two new mutations in the gene encoding cytoplasmic Cu,Zn superoxide dismutase (SOD1) have been discovered in patients with familial amyotrophic lateral sclerosis (FALS). These mutations result in the truncation of most of the polypeptide segment encoded by exon 5, one by the formation of a stop codon in codon 126 (L126Z) and the other by inducing alternative splicing in the mRNA (splicing junction mutation). These two mutants of SOD1 result in a FALS phenotype similar to that observed in patients with missense mutations in the SOD1 gene, establishing that exon 5 is not required for the novel toxic functions of mutant SOD1 associated with ALS. These mutant enzymes are present at very low levels in FALS patients, suggesting elevated toxicity compared to mutant enzymes with single site substitutions. This increased toxicity likely arises from the extreme structural and functional changes in the active site channel, beta-barrel fold, and dimer interface observed in the mutant enzymes, including the loss of native dismutase activity. In particular, the truncation of the polypeptide chain dramatically opens the active site channel, resulting in a marked increase in the accessibility and flexibility of the metal ions and side chain ligands of the enzyme active site. These structural changes are proposed to cause a decrease in substrate specificity and an increase in the catalysis of harmful chemical reactions such as peroxidation.
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3/6. neostigmine: an alternative treatment for constipation.

    constipation is a common complication of amyotrophic lateral sclerosis (ALS), especially as the disease progresses. While ALS patients may experience disturbed gastrointestinal motility due to the nature of the disease and decreased physical activity, the constipation is not usually caused by mechanical obstruction. Acute colonic pseudo-obstruction (ACPO) is a syndrome characterized by massive dilation of the colon without mechanical obstruction. Recent studies have shown neostigmine may be an effective treatment for ACPO. Through a case study, the author discusses the use of neostigmine and its nursing implications on a patient with ALS.
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4/6. An intraoral prosthesis to control drooling in a patient with amyotrophic lateral sclerosis.

    Many alternatives exist for treating chronic drooling. The treatment of a patient with amyotrophic lateral sclerosis who complained of chronic drooling and lack of retention of fluids while drinking is presented. In this case, an alternative type of intraoral prosthesis, not previously reported in the dental literature, was used to treat chronic drooling. A lip plumper prosthesis was fabricated to approximate the partially incompetent lips and create an oral seal. The results of the prosthesis therapy for this patient are encouraging both physically and psychologically.
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5/6. Treatment efficacy: dysarthria.

    The dysarthrias form a group of diverse, chronic motor speech disorders. The disorders of Parkinson's disease, stroke, traumatic brain injury, amyotrophic lateral sclerosis, and cerebral palsy are reviewed because they represent important clinical diagnoses in which dysarthria is a frequent and debilitating symptom. The roles played by speech-language pathologists include participation in differential diagnosis, provision of speech treatment, staging of treatment, and timely education so that clients and families can make informed decisions about communication alternatives. Both scientific and clinical evidence is presented that suggests that individuals with dysarthria benefit from the services of speech-language pathologists. Group-treatment studies, single-subject studies, and case reports illustrate the effectiveness of various types of speech treatment. research into the effectiveness of augmentative and alternative communication systems for individuals with cerebral palsy is also presented.
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6/6. Temporary amelioration of symptoms with intravenous cyclophosphamide in amyotrophic lateral sclerosis.

    Based on the evidence that autoimmunity may play a role in the pathogenesis of amyotrophic lateral sclerosis (ALS), a variety of immunomodulating agents have been used in the treatment. In an uncontrolled trial we treated 44 patients of ALS with intravenous cyclophosphamide (IVCP) at a total dose of 1.5 g/m2 given over a period of 8 to 10 days. The patients were evaluated using neurological score which included bulbar, motor and daily activity scores before and following treatment. Twenty three patients showed a significant improvement (P=<0.001) in the composite and the individual scores. The improvement persisted only for 2 to 3 months. Amongst them the severely (7) and moderately (16) affected (score less than or more than 150) showed almost a similar response to treatment. A comparison of the improved group of 23 patients with the unimproved group of 21 patients did not reveal any significant factors which influenced the response to IVCP. However, there was a suggestion that patients below the age of 60 years and a duration of illness less than 12 months may respond to the drug. In conclusion, treatment with intravenous cyclophosphamide resulted in mild and temporary improvement in clinical status of the patients with amyotrophic lateral sclerosis. This may be considered as an alternative method of treatment in developing countries where newer drugs are not available and affordable.
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