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Motor Neuron Dz
CM Neurology
| Question | Answer |
|---|---|
| ALS (amyotrophic lateral sclerosis) | UMN and LMN damage |
| PLS (Primary lateral sclerosis) | UMN damage |
| PMA (progressive Muscular atrophy) | LMN damage, as well as in Kennedy's dz |
| This divides the primary motor cortex from the parietal lobes | Central Sulcus |
| UMN damage signs | Loss of dexterity,Increased muscle tone,Spasticity,Hyperactive deep tendon reflexes,Babinski sign |
| LMN damage signs | weakness, Decreased muscle toneMuscle atrophyFasciculationsReduced or absent reflexes |
| Hoffman's sign | Flick the finger and it will continue to twitch = hyperactive muscles |
| _____are a sign of active degeration within the motor nerves | Fasiculations |
| Examples of UMN only diseases | primary lateral sclerosis, pseudobulbar palsy, hereditary spastic paraplegia, adrenomyeloneuropathy, HTLV-1 |
| Examples of LMN only diseases | Progressive muscular atrophy, Progressive Bulbar palsy, hereditary infantile, juvenile and adult onset SMA, Poliomyelitis |
| Polio affects | the anterior horn cells of the lower motor neurons |
| ALS epidemiology | Highest incidence among ages 65-74; M:F=1.5:1. Mean age of onset 56-63. Rarely occurs before age 20. Familial ALS<10% of all cases. Autosomal dominant inheritance. |
| What population does ALS have higher incidence in? | Veterans; athletic people |
| ALS symptoms | Weakness starting in thumb and progressing upward. No pain, no sensory symptoms, soley motor problem. Head drops, foot drop, Cramps, Fasiculations, frontotemporal dementia |
| 25% of ALS patients complain of | bulbar weakness: slurred speech, difficulty swallowing/choking. These people have lower rates of survival b/c of inability to maintain airway |
| ALS diagnosis | UMN and LMN signs in 3 regions (bulbar, cervical, thoracic, lumbosacral), UMN signs by PE, LMN signs by Electromyography: denervations, fasiculations, large complex motor units, SrCrkinase normal or elevated |
| HIV can also cause | Motor neuron disease |
| Pharmaceutical Treatment of ALS | Riluzole. Reduces presynaptic release of glutamate. $9K/year |
| SymptomatiC Treatment of ALS | Respiratory assistance, PEG tube prevents complications of aspiration and maintains weight, Dysarthria communication devices, Spasticity and Pseudobulbar drugs |
| Common AE of Spasticity meds | fatigue |
| ____% of ALS patients die within 5 years of symptom onset | 60%, people are usually diagnosed 9-12 months after symptom onset |
| Primary Lateral Sclerosis (PLS) Epidemiology | Very rare, onset typically middle age, mean age 45-53 years, Slight male predominance |
| PLS symptoms and signs | Leg stiffness/weakness, spasticity, legs usually affected before the arms, ultimately spreads to arms and bulbar muscles. Urinary urgency, Dysphagia, Dysarthria, hyperreflxia |
| PLS with LMN signs | have progressed to ALS |
| Recommended lab evaluation of PLS | EMG, Vit B12, Lyme, RPR, HTLV-1, HIV, lumbar puncture, MRI of brain and cervical spine, serum long-chain fatty acids, evoked potentials |
| Treatment for PLS | 100% supportive. No evidence for Riluzole |
| Primary Muscular Atrophy | Diagnosis of exclusion, >3 years from onset. focal and distal extremity weakness, atrophy, fasiculations, hyporeflexia. Often spares bulbar musculature. CK elevated up to 10X normal. Slower progression than ALS |
| Kennedy's disease | X-linked spinal bulbar muscular atrophy, mutation in androgen receptor, Symptom onset from adolescence to mid-80's. Facial fasiculations, weakness of mouth and tongue, dysphagia, gynecomastia, DM, oligospermia, limb weakness |
| Myasthenia Gravis pathology | Autoantibodies produced against the ACH receptor on the post-synaptic receptors/sarcolemma |
| Inhibiting the activity of ACH esterase is a treatment option for | Myasthenia Gravis |
| Myasthenia Gravis Signs and symptoms | Fluctuating weakness (worsens with activity), Ocular - drooping of eyelids, double vision, Bulbar/facial - difficulty chewing or swallowing "tired" facial appearance, ptosis. Generalized - asymmetric extremity weakness, SOB, difficulty holding up head |
| Myasthenia Gravis PE | Ocular, ptosis, EOMs, Muscle power testing: weakness worsens with repitition. Choose the muscle that they are complaining of weakness in. Do it at the beginning and end of the exam. Improves with rest. Ice pack test, upgaze test |
| Myasthenia Gravis Diagnosis | Tensilon Test (edrophonium), ACH receptor antibodies, MuSK antibodies, repetitive nerve stimulation, single fiber EMG (most sensitive), CT chest to exclude thymoma. |
| Treatment of Myasthenia Gravis | Symptomatic: cholinesterase inhibitors, Surgical: thymectomy - best response in young patients, Medical: Immunosuppressants - prednisone works well and fast. Steroids are first-line |
| MG: exacerbation/crisis causes | often occurs in association with infection, pregnancy, medication noncompliance, steroid use, meds that affect neuromuscular transmission. Treated with plasmaphoresis, IV Ig, supportive respiratory care |
| what does plasmaphoresis do? | Filters blood like dialysis and removes antibodies |
| MG: Medications to avoid | Neuromuscular blocking agents, Quinine, Quinidine, Procainamide, Abx (CIPRO is a big offender), BB, CCB, Magnesium, IV contrast. |
| Lambert-Eaton myasthenic Syndrome (LEMS) etiology | Usually paraneoplastic (screen patients for lung cancer), autoimmune, Voltage gated calcium channel antibodies |
| Lambert-Eaton myasthenic Syndrome (LEMS) signs and symptoms | Affect autonomic symptoms (sluggish pupils, blurry vision, dry mouth-eating hard candy more often), Proximal wekness, hypo/absent reflexes. RNS: Facilitation |
| Lambert-Eaton myasthenic Syndrome (LEMS) treatment | if cancer, treat. 3,4-diaminopyridine blocks K efflux and therefore increases Ca influx. ACH esterase inhibitors, Immunosuppression may be helpful |
| Botulism toxin | Clostridium botulinum causes flaccid paralysis |
| Botulism pathophysiology | Irreversible blockade of acetylcholine release |
| Causes of Botulism | Ingestion of contaminated home-canned foods, infants ingesting honey with spores, Iatrogenic Botox injections, wound botulism, airborne: terrorism |
| Is ACH released in Botulism? | No, there is an irreversible blockade of Acetylcholine release. |
| Symptoms of Botulism | Symptoms within 24 hrs of ingestion, diplopia, ptosis, dilated pupils, facial and respiratory weakness, descending paralysis, autonomic dysfunction, |
| Treatment of Botulism | Supportive care, horse serum antitoxin (available from CDC), Patients take months to fully recover |
| Myopathy | Stable, does not progress over time |
| Dystrophy | Progressively worsens over time |
| Muscular dystrophies are characterized by | muscle weakness and wasting, muscle biopsy reveals necrosis of muscle fibers |
| Duchenne/Becker | X-linked (males affected, females carriers). Dystrophin muscle membrane protein needed for stabilization (absent in Duchenne, Reduced in Becker). |
| Duchenne/Becker signs | exaggerated lordosis, shoulder thrown back, pelvis tilted forward. |
| Duchenne MD epidemiology | onset at 3-5 years, calf pseudohypertrophy, loss of ambulation in early teens, cardiomyopathy, death in early 20's |
| Gower's sign is seen in | Duchenne/Becker Muscular Dystrophy. Can't stand up normally. |
| Myopathy | Stable, does not progress over time |
| Dystrophy | Progressively worsens over time |
| Muscular dystrophies are characterized by | muscle weakness and wasting, muscle biopsy reveals necrosis of muscle fibers |
| Duchenne/Becker | X-linked (males affected, females carriers). Dystrophin muscle membrane protein needed for stabilization (absent in Duchenne, Reduced in Becker). |
| Duchenne/Becker signs | exaggerated lordosis, shoulder thrown back, pelvis tilted forward. |
| Duchenne MD epidemiology | onset at 3-5 years, calf pseudohypertrophy, loss of ambulation in early teens, cardiomyopathy, death in early 20's |
| Gower's sign is seen in | Duchenne/Becker Muscular Dystrophy. Can't stand up normally. |
| Becker MD Epidemiolgy | Milder course than Duchenne, onset 5-10 years, remain ambulatory until late teens, cardiomyopathy may be more disabling than weakness |
| Duchenne Muscular Dystrophy Pathophysiology | Fibrous tissue moved in where muscle fibers have died. |
| Diagnosing Duchenne Muscular Dystrophy | muscle biopsy, sequence entire gene, CK sometimes>10,000, Family hx |
| Treatment of Duchenne Musclar Dystrophy | Prednisone starting at age 5(delays transition from ambulation to non-ambulation), supportive care, cardiac transplants, gene therapy |
| Which is worse, Duchenne's or Becker's? | Duchennes |
| Facioscapulohumeral: FSHD signs | small pectoralis muscles, winging of scapula, weakness of dorsiflexor, weak eye closure |
| Facioscapulohumeral Epidemiology | Autosomal Dominant, Onset 1st-5th decade, can be asymptomatic |
| What is the 3rd most common muscular dystrophy? | Facioscapulohumeral Dystrophy |
| FSHD signs | Scapular winging and sleeping with eyes open are often the first symptoms. No EFFECT ON LIFE EXPECTANCY. Supportive care and genetic counseling. |
| Emery-Dreifuss Muscular Dystrophy | Humeroperoneal or scapuloperoneal weakness with early contractures. Typical onset in teens. Cardiac conduction block and arrhythmias can be lethal |
| Emery-Dreifuss MD signs | Contractures of ankles, neck and elbow, scapular winging, walking on balls of feet, deltoid sparing |
| Signs of Myotonic Dystrophy | frontal balding, ptosis, gynecomastia, early cataracts, weakness of SCM, DM, Facial weakness (tented upper lip), weakness in distal muscles |
| What is the second most common muscular dystrophy? | Myotonic Dystrophy |
| Definition of myotonia | inability to relax the muscle after contraction. Can check by tapping on thenar eminance or grip test. Myonic discharges on EMG |
| If you think someone has myasthenia, send off an ______ panel | antibody |
| Myasthenia gravis EMG results | Single fiber will be abnormal, repetitive nerve stimulation |
| Imaging for evaluation of Myasthenia | CT for thymoma |
Created by:
ltm12