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Motor Neuron Diseases

Motor Neuron Diseases

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Published by Louis Fortunato

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Published by: Louis Fortunato on Mar 19, 2012
Copyright:Attribution Non-commercial


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1. Amyotrophic Lateral Sclerosis(Lou Gehrig Disease) ICD-9CM Code335.20 Amyotrophic lateral SclerosisA. A Degenerative Disorder Involving The Upper and Lower Motor  Neurons Of The Pyramidal System With Progressive Muscle Weakness,Atrophy (Amyotrophy) & Spasticity.B. It Can Be1. Familial Or 2. Sporadic In NatureC. A Subset Of Familial Cases Has Been A1. Associated With Mutations In The Gene Coding For The Enzyme,Superoxide Dismutase Located On The LongArm Of Chromosome 21D. Pathology:1. ALS Is CharacterizedA. Loss Of Motor Neurons In The Anterior HornsOf The Spinal Cord, Brain Stem Motor Nuclei, & The PrimaryMotor Cortex Of The Cerebrum2. Inclusions Are Typically Seen In Residual Spinal Motor  Neurons Including Ubiquitin Positive Hyaline Inclusions : ReminiscentOf Lewy Bodies& Other Small Cytoplasmic Structures Known AsBumina BodiesE. Clinical Features:1. Begins WithA. WeaknessB. ClumsinessC. Speech Difficulties2. The Weakness Progresses To Muscle Atrophy & Fasciculations3. Upper Motor Neuron Involvement Is Present In Most Cases IncludingA. SpasticityB. Hyperactive Deep Tendon ReflexesC. A Positive Babinski Sign (viz. Upgoing Toes In Response To APainful Stimulus To The Soles Of The Feet4. Most Patients Have Signs Of Upper & Lower Motor Neuron Disease

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