Motor neurone disease - Infobox_Disease Name Image Caption...

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Sheet1 Infobox_Disease Name = Motor neurone disease Image = Polio spinal diagram.PNG Caption = DiseasesDB = 8358 ICD10 = ICD10G122g10 ICD9 = ICD9335.2 ICDO = OMIM = MedlinePlus = eMedicineSubj = eMedicineTopic = MeshID = D016472 The '''motor neurone diseases''' (or '''motor neuron diseases''') (MND) are a group of progressive neurological disorders that d Err:510 Forms of motor neurone disease include: * amyotrophic lateral sclerosis (ALS) (sometimes called Lou Gehrig's disease) * primary lateral sclerosis (PLS) * progressive muscular atrophy (PMA) * bulbar&lt ** pseudobulbar palsy - spastic ** progressive bulbar palsy - spastic and flaccid Spinal muscular atrophy (SMA) is sometimes (but not always) considered a form of MND. Err:510 In this article, MND refers to a group of diseases which affect the motor neurones. In the United States, the term Amyotrophic Err:509 Neurological examination presents specific signs associated with upper and lower motor neurone degeneration. Signs of uppe Note that every muscle group in the body requires both upper and lower motor neurones to function. It is a common misconce Symptoms usually present between the ages of 50-70, and include progressive weakness, muscle wasting, and muscle fascic The symptoms described above may Err:510 The diagnosis of MND is a clinical one, established by a neurologist on the basis of history and neurological examination. The A set of diagnostic criteria * MND in the presence of both upper and lower motor neurone degeneration is ''ALS''. * Where the illness affects only the upper motor neurones it is ''PLS''. * Where the illness affects only the lower motor neurones it is ''PMA''. * ''Progressive bulbar palsy'' is degeneration of the lower motor neurones innervating the bulbar region (mouth, face, and thro * ''Pseudobulbar palsy'' refers to degeneration of the upper motor neurones to the same region. It it Page 1
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Sheet1 Err:510 Most cases of MND progress quite quickly, with noticeable decline occurring over the course of months. Although symptoms m MND is typically fatal within 2-5 years. Around 50% die within 14 months of diagnosis. The remaining 50% will not necessarily Mortality normally results when control of the diaphragm is impaired and the ability to breathe is lost. One exception is PLS, w Err:510 Err:510 About 90% of Of these, SOD1 mutations account for some 20% of familial MND cases. The ''SOD1'' gene codes for the enzyme superoxide It is thought that ''SOD1'' mutations confer a toxic gain, rather than a loss, of function to the enzyme. SOD1 mutations may inc Err:510 Skeletal muscles are innervated by a group of neurones (''lower motor neurones'') located in the ventral horns of the spinal co There is a role in excitotoxicity and oxidative stress, presumably secondary to
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This note was uploaded on 04/30/2008 for the course CS 3304 taught by Professor Chen during the Spring '08 term at University of Houston - Downtown.

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Motor neurone disease - Infobox_Disease Name Image Caption...

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