PROPERTIES Allow user to leave interaction: Show ‘Next Slide’ Button: Completion Button Label: Anytime Show always View Presentation Muscular System Suresh Agarwal, M.D. Muscular System • Neuromuscular Physiology • Neuromuscular Disorders • Compartment Syndrome • Rhabdomyolysis www.health-res.com/EX/08-05-01/als1.jpg Page 3 Neuromuscular Physiology: The Motor Unit • Lower Motor Neurons = Alpha Motor Neurons • Alpha Motor Neuron Cell Bodies – Cranial Musculature: In the Brainstem – Somatic Cells: In the Anterior Horn of the Spinal Cord • Nerve Roots • Plexus • Peripheral Nerves • Terminal Ramifications palrehab.net/images/spin20.jpg • Motor Neuron Synapse Page 4 The Motor Unit • Neuromuscular Junction • Presynaptic Acetylcholine Release • Postsynaptic Acetylcholine Binding education.vetmed.vt.edu/Curriculum/VM8054/Labs/ Lab10/IMAGES/MOTOR%20END%20PLATES%20SMAL L%201.jpg – Increases Muscle End-Plate Potential – Threshold Level > Depolarizes • Calcium Ions Released from Sarcoplasmic Reticulum • Excitation-Contraction Coupling > Muscle Contraction • Acetylcholine Degraded by Cholinesterase bp3.blogger.com/_v2GFIISzHOU/SAjilu3b8kI/A AAAAAAAASk/3BRF9vWKgYY/s400/NeuroMuscular+Junction.jpg Page 5 Neuromuscular Disorders • Neuromuscular diseases leading to critical illness – Guillain-Barre Syndrome – West Nile Virus Acute Flaccid Paralysis Syndrome – Myasthenia Gravis • Neuromuscular diseases caused by critical illness – Critical Illness Polyneuropathy & Myopathy www.factmonster.com/images/ES CI342MUSSYS002.gif Page 6 Neuromuscular Disorders • Acute Inflammatory Demyelinating Polyradiculoneuropathy (a.k.a. Guillain-Barre Syndrome) • Motor >>Sensory Peripheral Neuropathy • Monophasic • Nadir at 4 weeks • Immune mediated • Exact etiology unknown • Demyelinating Neuropathy www.infiniteunknown.net/wpcontent/uploads/2009/11/guillain-barresyndrome.jpg • Primary Axonopathy Page 7 Guillain-Barre Syndrome • ? Preceding disease or condition • Gangliosides • Campylobacter jejuni upload.wikimedia.org/wikipedia/commons/thumb/b/ ba/Campylobacter.jpg/450px-Campylobacter.jpg Page 8 Guillain-Barre Syndrome • Clinical Findings Myelin – Subacute – Progressive weakness – Starts in legs – Sensory complaints – No objective sensory deficits upload.wikimedia.org/wikipedia/commons/c/c1/Myeli nated_neuron.jpg absent – Diminished or deep tendon reflexes drdavis.typepad.com/.a/6a00d834525ed16 9e201156f86664c970c-320pi Page 9 Guillain-Barre Syndrome • CSF findings, around 2nd week – Elevated protein – No pleocytosis neuromuscular.wustl.edu/pics/diagrams/emg/gb srecov.gif Page 10 Guillain-Barre Syndrome Electrodiagnostic Studies • Motor and Sensory Nerve Conduction Studies • Needle Electromyography • Findings: – Segmental nerve demyelination – Multifocal conduction blocks graphics8.nytimes.com/images/200 7/08/01/health/adam/9238.jpg – Slow Conduction Velocity – Consistent with a Peripheral Neuropathy Page 11 Guillain-Barre Syndrome • Management • Vent support • Autonomic Dysfunction • Immunotherapy – Plasma exchange – High dose IVIg • Rehabilitation repairstemcell.files.wordpress.com/2009/03/mspic.jpg Page 12 West Nile Virus • West Nile Virus Acute Flaccid Paralysis Syndrome – Flavivirus – Birds and mosquitoes (Culex) – Late summer or Fall media.publicbroadcasting.net/kera/ne wsroom/images/3197830.jpg www.nature.com/nrmicro/journal/v4/n1/i mages/nrmicro1326-f2.jpg Page 13 West Nile Virus 3 Different Clinical Manifestations 1. Asymptomatic infection 2. Mild febrile syndrome West Nile Fever • approx. 20% • 3 – 6 days duration 3. Neuroinvasive disease West Nile t2.gstatic.com/images?q=tbn:vSeOC3WZ2 meningitis or encephalitis • approx. 1 in 150 Wee0M:http://news.bbc.co.uk/nol/shared/s pl/hi/health/03/travel_health/diseases/img/ westnile.jpg Page 14 West Nile Virus • Acute Flaccid Paralysis Syndrome – “poliomyelitis-like” – Ventral Horns and Ventral Roots – Acute – Asymmetrical – Flaccid – No Sensory Deficits – No diffuse reflex deficits – No bowel or bladder www.tmin.ac.jp/english/dept/07/neuro logy2.jpg dysfunction Page 15 West Nile Virus – Acute Flaccid Paralysis Syndrome • Electrodiagnostic testing – Normal sensory potentials – No findings of segmental demyelination (unlike Guillain-Barre) – Low amplitude muscle action potentials I n affected areas – Significant denervation changes in affected areas • MRI • CSF – Mild pleocytosis (lymphocytic) – Mild to Moderate increase in protein www.brown.edu/Courses/Bio_160/Projects – No change in glucose 2000/Polio/Reflexcopy.jpg Page 16 West Nile Virus – Acute Flaccid Paralysis Syndrome • Diagnosis • Treatment – Reverse-transcriptase PCR (insensitive) – Supportive – Antibody-capture ELISA (IgM) – ?Antiretroviral medications – ?IVIg • Prognosis for recovery of strength is poor www.co.klamath.or.us/healthDept/ images/mosquito.jpg Page 17 Myasthenia Gravis • Autoimmune attack on acetylcholine receptor • Fluctuating weakness • Progressive with sustained exertion • Incidence: – Early adulthood: – Women > Men – Later adulthood: – Women = Men www.hakeemsy.com/main/files/images/MyastheniaGravis.JPG Page 18 Myasthenia Gravis • Clinical Presentation • Muscle fatigue – Worst with prolonged exertion • Ocular muscles – Ptosis – Diplopia • Bulbar muscles – Dysphagia – Dysarthria • Respiratory Failure Page 19 Myasthenia Gravis • Diagnosis • Clinical presentation • Edrophonium testing • Electrophysiologic studies – Repetitive nerve stimulation • Antibody Testing – Acetylcholine receptor www.mda.org/publications/images/q 10-3_ach.jpg – Muscle specific receptor tyrosine kinase (MuSK) Page 20 Myasthenia Gravis • Myasthenic Crisis • – 20% of patients with MG – Sensitive to Nondepolarizing agents – Respiratory failure – Resistant to Depolarizing agents – Precipitating factors • Bronchopulmonary processes • Aspiration • Sepsis • Surgical procedures • Immune modulation tapering Neuromuscular blocking agents • Thymomas – More fulminate disease – 30% of patients with myasthenic crisis • Corticosteroids • Pregnancy • Certain Drugs Page 21 Myasthenia Gravis • Treatment – Immunomodulating Methods • Plasma exchange (short-term) – Myasthenic crisis – Surgical preparation – Increased strength after 2 to 3 exchanges • IVIg (short-term) – Alternative to plasma exchange – Possible longer period until onset of effect • Corticosteroids – Occasionally used – Prolonged crises – Transient increase in weakness Page 22 Myasthenia Gravis • Treatment Acetylcholine Receptor – Cholinesterase inhibitors • Cholinergic Crisis – Possible increase in weakness – Muscle fasciculations – Muscarinic symptoms • Avoid repeated/escalating doses • Discontinue after intubation upload.wikimedia.org/wikipedia/commons/6/6e/Nicoti nic_Acetylcholine_receptor.png Page 23 Myasthenia Gravis • Thymus – Abnormal in 75% – Thymoma in 25% • Benign • Malignant • Thymectomy – Necessary for thymoma – Controversial for patients without know thymic abnormalities www.aurorahealthcare.org/health – Disease course often abates Page 24 gate/images/si2141.jpg Critical Illness Polyneuropathy & Myopathy • Generalized weakness • Axonal • Predisposing Factors – Critical Illness – Sepsis – Multiple system organ failure • Prolonged mechanical ventilation www.pathologyoutlines.com/images/softtissue/06_13.jpg Page 25 Critical Illness Polyneuropathy & Myopathy • Common Antecedents – Sepsis – Multiple System Organ Failure • Pathophysiology – ICU days – Number of invasive procedures – Hyperglycemia – Hypoalbuminemia – Severity of MSOF – Neuromuscular Blocking Agents – Corticosteroids Page 26 Critical Illness Polyneuropathy & Myopathy • Clinical Features – Muscle weakness and wasting • Deep Tendon Reflexes – Diminished or absent – Parasthesias – Distal Sensory Loss vasculitis.med.jhu.edu/typesof/images/Muscle _waste_MPA.jpg Page 27 Critical Illness Polyneuropathy & Myopathy • Nerve Conduction – Normal nerve conduction speed – Decreased muscle action potential amplitude – Decreased sensory nerve action potential amplitude • Needle Electrode – Denervation www.nature.com/nrneurol/journal/v5/n7/i mages/nrneurol.2009.75-f1.jpg • Histopathology – Primary axonal degeneration Page 28 Critical Illness Polyneuropathy & Myopathy • Prognosis – Underlying critical illness – Increased ventilator dependence – Functional recovery in several months Ulnar Nerve Compression • Padding and Positioning to prevent compression neuropathies meddb.eznetpublish.ihealthspot.com/portal s/2/MedicalLibraryAssets/Medical/CubitalTu nnel_small.jpg Page 29 Compartment Syndrome • Open or Closed Fractures • Fixed Compartment • Tissue edema and bleeding • Blood flow impeded – Capillaries – Arterioles • Factors effecting tissue necrosis – Amount of Pressure – Duration of increased pressure – Sensitivity of the tissue to ischemia Right Buttock Compartment Syndrome casesjournal.com/content/figures/1 757-1626-2-190-3.gif Page 30 Compartment Syndrome • Tissue Ischemia Necrotic Muscle – Nervous tissue • Functional abnormalities after 30 minutes • Irreversible damage after 12 to 24 hours – Muscle • Functional abnormalities after 2 to 4 hours • Irreversible damage after 4 to 12 hours www.operationgivingback.facs.org/stuff/con tentmgr/files/a384bb3c7b77e154ad25c613 6d7be344/miscdocs/lab_manual_extremity _chapter_4__2_.pdf • Increased capillary permeability -> Edema Page 31 Compartment Syndrome • Risk factors – Severity of fracture – Extent of soft tissue injury – Compressive devices • Anti-shock trousers • Tourniquets – Systemic hypotension www.nexternal.com/medtech/images/MastP ants.jpg Page 32 Compartment Syndrome • Most common location = Anterior Compartment of the Lower Leg Usually from closed tibia fracture • Other sites – Thigh – Arm – Buttock – Foot orthoinfo.aaos.org/figures/A00204F01. Page 33 Compartment Syndrome • Diagnosis • Clinical – Tense compartment to palpation – Severe pain with passive range of motion – Severe compartment tenderness – Impaired sensory exam – Decreased distal perfusion – Pulseless = Too Late • Extensive tissue necrosis present – Serial Exams are Critical Page 34 www.hopkins-arthritis.org/physiciancorner/cme/rheumatologyrounds/images/rounds11/slide22.jpg Compartment Syndrome • Measurement of Compartment Pressures – Unresponsive patients – Pressure > 30 to 45 = Indication for Fasciotomies – Diastolic BP – Compartment Pressure < 30 = indication for Fasciotomies www.hopkins-arthritis.org/physiciancorner/cme/rheumatologyrounds/images/rounds11/slide22.jpg Page 35 Compartment Syndrome • Treatment – Surgical Fasciotomies – Fasciotomy within 12 hours = 68% normal functional result – Hydration – Monitor electrolytes – Monitor for infection of fasciotomy sites upload.wikimedia.org/wikipedia/commons/d/da/ Fasciotomy_leg.jpg Page 36 Lower Leg Fasciotomies • 2 incisions • 4 compartments Anterolateral Incision • Anterolateral Incision Anterolateral Incision img.medscape.com/pi/emed/ckb/orthopedic_surg ery/1230552-1270542-199.jpg Page 37 Lower Leg Fasciotomies • Medial Incision • Incision 1 fingerbreadth posterior to medial edge of the tibia Posteriomedial Incision • Liberal Length Posteriomedial Incision • Avoid saphenous vein • Divide fibers of soleus from tibia • Neurovascular bundle img.medscape.com/pi/emed/ckb/orthopedic_surger y/1230552-1270542-169.jpg Page 38 Upper Leg Fasciotomies • 3 Compartments – Anterior – Posterior – Medial • Compartment syndrome rare • 3 compartments blend with the hip • Lateral incision usually sufficient • Occasionally requires medial incision www.operationgivingback.facs.org/stuff/contentmgr/ files/a384bb3c7b77e154ad25c6136d7be344/miscd ocs/lab_manual_extremity_chapter_4__2_.pdf Page 39 Foot Compartment Syndrome 4 Compartments • Interosseus or Intrinsic Compartment – 4 intrinsic muscles between 1st and 4th metatarsals • Medial Compartment – Abductor hallicus and flexor hallicus brevis • Central or Calcaneal Compartment – Flexor digitorum brevis, quadratus plantae, and the adductor hallicus • Lateral Compartment – Flexor digiti minimi brevis, www.operationgivingback.facs.org/stuff/contentmg abductor digiti minimi r/files/a384bb3c7b77e154ad25c6136d7be344/mis cdocs/lab_manual_extremity_chapter_4__2_.pdf Page 40 Foot Compartment Syndrome - • Up to 10% of calcaneal fractures • 41% of crush injuries to the foot • No classic sign of CS • Most reliable sign: tense bulging tissue www.operationgivingback.facs.org/stuff/contentmgr/fil es/a384bb3c7b77e154ad25c6136d7be344/miscdocs/ lab_manual_extremity_chapter_4__2_.pdf Page 41 Forearm and Hand Fasciotomies • Compartment syndromes are less common than in the leg • Supracondylar humerus fx > antebrachial compartment syndrome • Anterior compartment realeased with volar incision • Dorsal incision if necessary img.medscape.com/pi/emed/ckb/orthopedic_surgery/12 30552-1268992-1269081-126919.jpg Page 42 img.medscape.com/pi/emed/ckb/orthopedic_surgery/1230552-1268992-1269081-1269193.jpg Hand Fasciotomies Thenar and Hypothenar Compartment Fasciotomies • Compartment syndrome of the hands is rare – ? From Trauma – More often iatrogenic (A-line or IV infiltrate) • 10 Osseofascial Compartments jmedicalcasereports.com/content/figur es/1752-1947-1-6-2.gif Dorsal Interosseus Compartment Fasciotomies – Carpal tunnel release – 1 or 2 dorsal incisions • No sensory nerve symptoms • Pressure > 20mmHg = CS jmedicalcasereports.com/content/figur es/1752-1947-1-6-1.jpg Page 43 Rhabdomyolysis • Damage to skeletal muscle – Crush • Injures cells • Decreases perfusion – Metabolic – Cell lysis due to edema • Calcium in sarcoplasmic reticulum – Muscle contractions • Depletes ATP • Neutrophils migrate – Increased inflammatory response • Muscle compresses local structures > Compartment Syndrome > Decreased Perfusion • Muscle cells release potassium, phosphate, myoglobin, creatine kinase and uric acid • Damage to mitochondrion – Reactive oxygen species Page 44 Rhabdomyolysis • Myoglobin – Nephrotoxic • Muscle swelling – Intravascular volume deficit – Renal hypoperfusion • Uric acid – Precipitates in renal tubules • Myoglobin – Accumulates in renal tubules Page 45 Rhabdomyolysis • Myoglobinuria – Plasma myoglobin > 1.5 mg/dL – Myoglobin casts cause nephron obstruction – Urine Acidification • Tea-colored urine • Urine dipstick + for blood • Urine – for red blood cells on microscopy lifeinthefastlane.com/wpcontent/uploads/2009/12/image_34.jpg Page 46 Rhabdomyolysis • Management – Replete Volume – Mannitol • Increases flushing of myoglobin from renal tubules • Effective radical scavenger – Sodium bicarbonate • Alkalization of Urine bioephemera.com/wpcontent/uploads/2007/06/jimstanisg1.jpg • Decreases cast formation • Decreases direct toxic effect of myoglobin on the renal tubules Page 47 Myositis Ossificans • Severe blunt trauma • Intra-muscular hematoma • Delayed ossification of the soft tissue • Suspected to be due to premature return to strenuous activity • Most common sites: - arms - quadriceps • Treatment - Conservative - Rarely, surgical debridement www.radiologyassistant.nl/images/thmb _4acef1936b33836.jpg Page 48 Image Sources • bp2.blogger.com/_OY2fM522a9Y/SBZ0DYml2xI/AAAAAAAABA4/K XVlqSv6hA8/s400/icu.gif • bp3.blogger.com/_v2GFIISzHOU/SAjilu3b8kI/AAAAAAAAASk/3BRF 9vWKgYY/s400/Neuro-Muscular+Junction.jpg • bioephemera.com/wp-content/uploads/2007/06/jimstanisg1.jpg • casesjournal.com/content/figures/1757-1626-2-190-3.gif • ccforum.com/content/figures/cc2978-1.jpg • dericbownds.net/uploaded_images/myoglobin.jpg • download.thelancet.com/images/journalimages/01406736/PIIS0140673609600398.fx1.lrg.jpg • drdavis.typepad.com/.a/6a00d834525ed169e201156f86664c970c320pi Page 49 Image Sources • education.vetmed.vt.edu/Curriculum/VM8054/Labs/Lab10/IMAGES/ MOTOR%20END%20PLATES%20SMALL%201.jpg • graphics8.nytimes.com/images/2007/08/01/health/adam/9238.jpg • img.medscape.com/pi/emed/ckb/orthopedic_surgery/12305521268992-1269081-1269193.jpg • img.medscape.com/pi/emed/ckb/orthopedic_surgery/12305521268992-1269081-1269194.jpg • img.medscape.com/pi/emed/ckb/orthopedic_surgery/12305521270542-169.jpg • img.medscape.com/pi/emed/ckb/orthopedic_surgery/12305521270542-199.jpg • img.medscape.com/pi/emed/ckb/pediatrics_general/13313411331932-982711-1723745.png • jmedicalcasereports.com/content/figures/1752-1947-1-6-1.jpg Page 50 Image Sources • jmedicalcasereports.com/content/figures/1752-1947-1-6-2.gif • lifeinthefastlane.com/wp-content/uploads/2009/12/image_34.jpg • Luis and Ng Journal of Medical Case Reports 2007 1:6 doi:10.1186/1752-1947-1-6 • meddb.eznetpublish.ihealthspot.com/portals/2/MedicalLibraryAssets /Medical/CubitalTunnel_small.jpg • media.publicbroadcasting.net/kera/newsroom/images/3197830.jpg • media-2.web.britannica.com/eb-media/75/2975-050-0D5D3A36.jpg • neuromuscular.wustl.edu/pics/diagrams/emg/gbsrecov.gif • news.bbc.co.uk/nol/shared/spl/hi/health/03/travel_health/diseases/i mg/westnile.jpg • news.stanford.edu/news/2007/january10/gifs/botulismsr_art.jpg Page 51 Image Sources • orthoinfo.aaos.org/figures/A00204F01.jpg • palrehab.net/images/spin20.jpg • pds13.egloos.com/pds/200907/27/61/e0077661_4a6dba 08992cc.jpg • pubs.ext.vt.edu/2911/2911-7041/L_IMG_fig11.jpg • repairstemcell.files.wordpress.com/2009/03/ms-pic.jpg • stevens.scripps.edu/images/toxin1.jpg • Textbook of Critical Care. 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