1. Amyotrophic Lateral Sclerosis (Lou Gehrig Disease) ICD-9CM Code 335.20 Amyotrophic lateral Sclerosis A.

A Degenerative Disorder Involving The Upper and Lower Motor Neurons Of The Pyramidal System With Progressive Muscle Weakness, Atrophy (Amyotrophy) & Spasticity. B. It Can Be 1. Familial Or 2. Sporadic In Nature C. A Subset Of Familial Cases Has Been A 1. Associated With Mutations In The Gene Coding For The Enzyme, Superoxide Dismutase Located On The Long Arm Of Chromosome 21 D. Pathology: 1. ALS Is Characterized A. Loss Of Motor Neurons In The Anterior Horns Of The Spinal Cord, Brain Stem Motor Nuclei, & The Primary Motor Cortex Of The Cerebrum 2. Inclusions Are Typically Seen In Residual Spinal Motor Neurons Including Ubiquitin Positive Hyaline Inclusions : Reminiscent Of Lewy Bodies& Other Small Cytoplasmic Structures Known As Bumina Bodies E. Clinical Features: 1. Begins With A. Weakness B. Clumsiness C. Speech Difficulties 2. The Weakness Progresses To Muscle Atrophy & Fasciculations 3. Upper Motor Neuron Involvement Is Present In Most Cases Including A. Spasticity B. Hyperactive Deep Tendon Reflexes C. A Positive Babinski Sign (viz. Upgoing Toes In Response To A Painful Stimulus To The Soles Of The Feet 4. Most Patients Have Signs Of Upper & Lower Motor Neuron Disease

F. Course: 1. Relentlessly Progressive Disease With A. Median Survival Of 5Years 2. Most Patients Die From A. Respiratory Insufficiency B. Infections. G. Rx: 1. Riluzole: (Rilutek) : A Glutamate Antagonist Check ALT Levels 2. Sialorrhea: Glycopyrrolate or Amitriptyline Consider Propanolol or Metoprolol If Secretions Are Thick. 3. Spasticity: Baclofen or Tizanidine or Clonazepam 4. Pseudobulbar Affect: Amitriptyline, Sertraline (Zoloft), or Dextromethorphan