II. Epidemiology
- Prevalence: 5-7 per 100,000 worldwide
- Gender: More common in men by ratio of 1.5 to 1
- Ages affected: 50 to 70 years old
III. Etiology
- Idiopathic in most cases
- Autosomal Dominant inheritance in 5-10% of cases
IV. Pathophysiology
- Upper Motor Neuron and Lower Motor Neuron degeneration
- Affects anterior horn cells
- Subtypes
- Progressive Bulbar Palsy
- Primary Lateral Sclerosis
- Spinal Muscular Atrophy
V. Symptoms
- Muscle aches and muscle cramps
- Weakness of distal upper limbs
- Weakness progresses inferiorly (towards feet)
- Dysarthria
- Dysphagia
- Drooling
- No sensory deficits
VI. Signs
- Muscle fibrillation and atrophy (upper limbs)
- Hyperreflexia
- Spasticity of lower limbs
VII. Diagnosis: Electromyogram (EMG)
- Muscle fibrillation on mechanical stimulation
- Increased duration and amplitude of action potentials
VIII. Course
- Majority of patients die within 1-3 years of diagnosis
- Only 10% survive beyond 5 years
IX. Management
- Riluzole 50 mg bid
- Anti-glutamate properties
- Only modest effect at best (extended life 3 months)
- Best effect if used early
- Very expensive ($700/month)
-
Vitamin E and Vitamin C
- Shown effective in rats but not proven in humans
- Immunosuppressants not effective or indicated
- Treat at ALS center
- Physical Therapy
- Occupational Therapy
- Dietitian
- Neurologist
- Symptomatic treatment
- Progressive Pseudobulbar palsy
- Spontaneous laugh (Tricyclic Antidepressants)
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| Definition (MEDLINEPLUS) |
Amyotrophic lateral sclerosis (ALS) is a nervous system disease that attacks nerve cells called neurons in your brain and spinal cord. These neurons transmit messages from your brain and spinal cord to your voluntary muscles - the ones you can control, like in your arms and legs. At first, this causes mild muscle problems. Some people notice
Eventually, you lose your strength and cannot move. When muscles in your chest fail, you cannot breathe. A breathing machine can help, but most people with ALS die from respiratory failure. The disease usually strikes between age 40 and 60. More men than women get it. No one knows what causes ALS. It can run in families, but usually it strikes at random. There is no cure. Medicines can relieve symptoms and, sometimes, prolong survival. NIH: National Institute of Neurological Disorders and Stroke |
| Definition (NCI) | An autosomal dominant inherited form of amyloidosis. |
| Definition (MSH) | A degenerative disorder affecting upper MOTOR NEURONS in the brain and lower motor neurons in the brain stem and SPINAL CORD. Disease onset is usually after the age of 50 and the process is usually fatal within 3 to 6 years. Clinical manifestations include progressive weakness, atrophy, FASCICULATION, hyperreflexia, DYSARTHRIA, dysphagia, and eventual paralysis of respiratory function. Pathologic features include the replacement of motor neurons with fibrous ASTROCYTES and atrophy of anterior SPINAL NERVE ROOTS and corticospinal tracts. (From Adams et al., Principles of Neurology, 6th ed, pp1089-94) |
| Definition (CSP) | progressive degeneration of the neurons that give rise to the corticospinal tract and of the motor cells of the brain stem and spinal cord, resulting in a deficit of upper and lower motor neurons. |
| Concepts | Disease or Syndrome (T047) |
| MSH | D000690 |
| ICD9 | 335.20 |
| ICD10 | G12.21 |
| SnomedCT | 86044005 |
| English | Gehrig Disease, Gehrig's Disease, Gehrigs Disease, Lou Gehrig Disease, Lou Gehrig's Disease, ALS, Sclerosis, Amyotrophic Lateral, ALS - Amyotroph lat sclerosis, Amyotrophic Lateral Sclerosis/Progressive Muscular Atrophy, Amyotrophic Lateral Sclerosis, LOU GEHRIGS DIS, GEHRIGS DIS, LOU GEHRIG DIS, MOTOR NEURON DIS AMYOTROPHIC LATERAL SCLEROSIS, amyotrophic lateral sclerosis, amyotrophic lateral sclerosis (diagnosis), ALS (amyotrophic lateral sclerosis), ALS (Amyotrophic Lateral Sclerosis), Motor Neuron Disease, Amyotrophic Lateral Sclerosis, Amyotrophic sclerosis, Lou-Gehrigs Disease, Disease, Lou-Gehrigs, Amyotrophic Lateral Sclerosis [Disease/Finding], amyotrophic laterals sclerosis, lou gehrig disease, lou gehrigs disease, lou gehrig's disease, Lou Gehrig's disease (ALS), Amytrophic lateral sclerosis, Charcot Disease, Amyotrophic lateral sclerosis, Bulbar motor neuron disease, Lou Gehrig's disease, ALS - Amyotrophic lateral sclerosis, Amyotrophic lateral sclerosis (disorder), creeping; palsy, lateral sclerosis; amyotrophy, palsy; creeping, sclerosis; spinal, lateral (amyotrophic), amyotrophy; lateral sclerosis, spinal; sclerosis, lateral (amyotrophic), Motor neuron disease, bulbar |
| Spanish | ALS, enfermedad bulbar de la neurona motora, enfermedad de Lou Gehrig, esclerosis lateral amiotrófica (trastorno), esclerosis lateral amiotrófica, Esclerosis lateral amiotrófica, Enfermedad de la Neurona Motora de la Esclerosis Amiotrófica Lateral, Enfermedad de Lou Gehrig, Esclerosis Amiotrófica Lateral |
| Japanese | ＡＬＳ, ｷﾝｲｼｭｸｾｲｿｸｻｸｺｳｶｼｮｳ, ALS, Gehrig病, 筋萎縮性側索硬化症, Lou Gehrig病, 側索硬化症-筋萎縮性, 筋萎縮側索硬化, 筋萎縮側索硬化症, 筋萎縮性側索硬化 |
| Swedish | Amyotrofisk lateralskleros |
| Czech | amyotrofická laterální skleróza, Amyotrofická laterální skleróza, ALS, Charcotova choroba, Charcotova nemoc, Lou Gehrigova nemoc |
| Finnish | Amyotrofinen lateraaliskleroosi |
| Russian | SKLEROZ BOKOVOI AMIOTROFICHESKII, BOKOVOI SKLEROZ, GERIGA BOLEZN', SHARKO BOLEZN', БОКОВОЙ СКЛЕРОЗ, ГЕРИГА БОЛЕЗНЬ, СКЛЕРОЗ БОКОВОЙ АМИОТРОФИЧЕСКИЙ, ШАРКО БОЛЕЗНЬ |
| Italian | Morbo di Lou Gehrig, SLA, Malattia del neurone motore, Sclerosi laterale amiotrofica |
| Croatian | AMIOTROFIČNA LATERALNA SKLEROZA |
| Polish | Stwardnienie boczne zanikowe, Choroba Lou Gehringa |
| Hungarian | Amyotrophiás lateral sclerosis, ALS |
| Norwegian | ALS, Amyotrofisk lateralsklerose, Amyotrofisk lateral sklerose |
| French | Maladie de Gehrig, Sclérose latérale amyotrophique, Maladie de Charcot, Maladie de Lou Gehrig, SLA (Sclérose Latérale Amyotrophique) |
| Dutch | amyotrofie; laterale sclerose, creeping; palsy, laterale sclerose; amyotrofie, palsy; creeping, sclerose; spinaal, lateraal (amyotrofisch), spinaal; sclerose, lateraal (amyotrofisch), amyotrofe laterale sclerose, ALS, Amyotrofische laterale sclerose (ALS), Lou-Gehrig-ziekte, Motoneuronziekte, Sclerose, amyotrofische laterale, Sclerose, laterale, amyotrofische (ALS) |
| Portuguese | Esclerose lateral amiotrófica, Doença de Lou Gehrig, Doença do Neurônio Motor em Esclerose Lateral Amiotrófica, Esclerose Amiotrófica Lateral |
| German | amyotrophische Lateralsklerose, ALS, Amyotrophische Lateralsklerose, Gehrig-Krankheit, Myatrophische Lateralsklerose, Lateralsklerose, amyotrophische, Lateralsklerose, myatrophische, Loe-Gehrig-Krankheit, Motoreuronerkrankung, amyotrophe Lateralsklerose, Charcot-Syndrom II |
