3.%20%20Motor_ALS

3.%20%20Motor_ALS - ALS Anterior Lateral SClerosis Dr Brett...

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11/14/2009 1 ALS – Anterior Lateral SClerosis Dr Brett Lillie 2009. Dr Brett Lillie 2009. Neuromuscular Diseases CNS | UMN }ALS + SMA barb2down LMN } / Peripheral N.Syst. } Guillian-Barre Syndrome barb2down / Motor endplate } Myaesthenia Gravis barb2down Mm (End organ) } Muscular Dystrophy, eg. Duchenne Muscle Dr Brett Lillie 2009. Neuromuscular Diseases ALS occurs = muscular dystrophy 3 x more common than Myaesthenia Gravis Dr Brett Lillie 2009. Neuromuscular Diseases Adult Motor Neuron Disease (or Amyotrophic Lateral Sclerosis-ALS) Progressive Muscular Atrophy Primary Lateral Sclerosis Amyotrophic Lateral Sclerosis } classified to Progressive Bulbar Palsy type of initial Pseudobulbar Palsy clinical features Dr Brett Lillie 2009. Adult Motor Neuron Disease ALS (Lou Gehrig’s Disease) Relentless degenerative terminal disease that affects both UMN + LMN 1. Massive loss of Ant. Horn cells = amyotrophy of s.cord + motor C.N. of b. stem 2. Demtelination of corticosp tr. + corticobulbar tr Caused by degeneration in = lateral motor cortex sclerosis Results in upper motor neuron symptoms Dr Brett Lillie 2009.
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11/14/2009 2 Adult Motor Neuron Disease Clinically Most common form of motor system disease. Depends primarily on identifying a collection of motor system changes According to World Federation of Neurology: Must have UMN + LMN signs in 3 spinal regions or 2 spinal regions + bulbar signs Excluded if a sensory or ANS dysfunction, CN3 or sign. mvt disorder. Dr Brett Lillie 2009. Adult Motor Neuron Disease Clinically Progression of signs and symptoms is essential for diagnosis. Charcot first described ALS. Usually sporadic In 5-10% of patients there is autosomal dominant inheritance.
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