2019 Gullian Barre Syndrome
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2019 Gullian Barre Syndrome
- 2. CASE PRESENTATION CASE #1 • DISPATCHED TO A PEDIATRIC FALL PATIENT AT A PRIVATE RESIDENCE • 7 Y/O FEMALE PATIENT. MOTHER STATES PATIENT FELL WHILE STUMBLING DOWN HALLWAY • PATIENT HAS NO KNOWN SIGNIFICANT HX, HAD THE FLU A FEW WEEKS AGO. NO KNOWN TRAUMA. • NOW BOTH HER LEGS FEEL TINGLY AND NUMB. STILL HAS COURSE MOVEMENT. SOME “FOOT DROP” IN BOTH LEGS. CASE #2 • DISPATCHED TO AN ADULT PATIENT WITH BACK PAIN AND LOWER EXTREMITY NUMBNESS. • 34 Y/O MALE WENT TO PICK SOMETHING UP AND HEARD A “POP” WITH ONSET OF PAIN. • SEEN DAY #2 WITH BACK PAIN AT ED, GIVEN NSAIDS AND D/C • PATIENT SMOKES, OCCASIONAL DRINKING, AND HAD THE FLU A FEW WEEKS AGO. • CALLS FOR EMS ON DAY #3 WITH DIFFICULTY AMBULATING, AND NUMB/TINGLY INBOTH LOWER LEGS. WHAT DO THESE CASES HAVE IN COMMON
- 3. OBJECTIVE • DEFINE GUILLAIN-BARRE’ SYNDROME (GBS) • UNDERSTAND PATHOPHYSIOLOGY OF GBS • IDENTIFY THE CHARACTERISTICS OF GBS AS AN ACUTE PRESENTATION • UNDERSTAND CHALLENGES IN THE IDENTIFICATION MANAGEMENT OF GBS • DISCUSS PREHOSPITAL CONSIDERATIONS IN THE ACUTE MANAGEMENT OF GBS.
- 4. WHAT IS GUILLAIN-BARRE’ SYNDROME? • CONSIDERED AN AUTO-IMMUNE DISORDER AFFECTING THE NEUROLOGICAL SYSTEM RATHER THAN A PRIMARY NEUROLOGICAL DISORDER. • RARE BUT POTENTIALLY LIFE-THREATENING CONDITION CHARACTERIZED BY RAPID ASCENDING SYMMETRICAL PARALYSIS/WEAKNESS. • MORTALITY APPROX. 3-10% DUE TO CARDIO-VASCULAR AND RESPIRATORY COMPLICATIONS. • LIFELONG MORBIDITY 10-15% • 1/3 WILL REQUIRE INTUBATION
- 5. BRIEF HISTORY • DESCRIBED IN 1859 BY OCTAVE LANDRY. 10 PATIENTS WITH 3 DISTINCT PRESENTATIONS, MOST WITH “ASCENDING PARALYSIS” • THIS IS WHY IT IS SOMETIMES CALLED LANDRY’S PARALYSIS. • ONE CURED WITH LEECHES, DON’T ASK WHERE HE PUT THEM. • DESCRIBED AGAIN BY FRENCH DOCTORS GUILLAIN, BARRE’ AND STROHL IN 1916. • WHAT HAPPENED TO STROHL? POOR DUDE. (TOO YOUNG, TOO BRIGHT, TOO GERMAN?) • MULTIPLE PAPERS AND STUDIES IN THE EARLY TO MID 20TH CENTURY • IDENTIFIED THE “PATHOLOGY” OF DEMYELINATION • BECAME A “THING” AFTER A SPIKE IN CASES IN 1978 ASSOCIATED WITH THE FLU (OR THE FLU VACCINE ACCORDING TO SOME) • SWINE VACCINE AND OLD RABIES VACCINE FROM MAMMALS ASSOSCIATED • I BECAME INTERESTED IN IT WHEN…….
- 6. WHY IS THIS IMPORTANT TO EMS PROVIDERS • CAN STRIKE ANY AGE, THOUGH MOST COMMON IN ELDERLY. • IS ASSOCIATED WITH INFECTIOUS OUTBREAKS. • WEAKNESS AND ABNORMAL SENSATION ARE COMMON PRESENTATION TO ED VISITS, AND BY EXTENSION TO EMS. • INCIDENCE IS ABOUT 2-4:100,000 • IS THE MOST COMMON OF THE DEMYELINATING CONDITIONS. • 80 TO 160 CASES OF GBS EACH WEEK • WHILE RARE, MISDIAGNOSIS OR MARGINALIZATION CAN LEAD TO DELAY IN CARE. MISMANAGEMENT CAN LEAD TO POOR OUTCOMES. • EMS PROVIDERS MAY ALSO BE REQUIRED TO CARE FOR THESE PATIENTS AFTER DIAGNOSIS FOR INTERFACILITY TRANSPORT. • TREATMENTS MAY ALSO CAUSE SEVERE COMPLICATIONS AND IMPLICATIONS FOR EMS PERSONNEL.
- 7. GBS PATHOPHYSIOLOGY • GBS IS A AUTOIMMUNE DISORDER CAUSING DEMYELINATION OF THE NERVOUS SYSTEM. • 3 MAIN TYPES WITH MULTIPLE VARIANTS AND OVERLAPS AND RELATED CONDITIONS. • WESTERN- ACUTE INFLAMMATORY DEMYELINATING POLYNEUROPATHY (AIDP) • ASIAN- ACUTE MOTOR AXONAL NEUROPATHY (AMAN) • MILLER-FISHER SYNDROME
- 9. CLASSIC PRESENTATION • PRECEDED BY AN “INFECTIOUS ASSAULT” (2/3 OF PATIENTS) 1-3 WEEKS PRIOR. RESPIRATORY OR LOWER GI MOST COMMON. • CAMPYLOBACTER JEJUNI ENTERITIS (25-50%) (VERY COMMON LOWER GI BACTERIAL CAUSING DIARRHEA) • VIRAL (E.G. CMV, EBV, HEPATITIS A-E VIRUSES, HIV, INFLUENZA, ARBOVIRUSES (E.G. ZIKA, CHIKUNGUNYA)) • MYCOPLASMA INFECTION • VACCINATION (E.G. INFLUENZA??, RABIES (CONFIRMED) • “ INFECTIOUS ASSAULT”: IMMUNE ACTIVATION EVENT LEADS TO AUTOANTIBODY PRODUCTION • AFFECTS PERIPHERAL NERVES, PRIMARILY SPINAL AND CRANIAL NERVE ROOTS. • ATTACK ON AUTONOMIC SYSTEM CAN CAUSE INFLAMMATION OF CRANIAL NERVE X AND BRADYCARDIA REQUIRING PACING. • PROGRESSIVE SYMPTOMS: PEAK S/S IN 4 WEEKS. RARELY OUT TO 6 WEEKS.
- 10. NEURO • ASCENDING SYMMETRICAL PARALYSIS • ASCENDING SYMMETRICAL PARESTHESIA AND WEAKNESS. • VISUAL DISTURBANCES (DOUBLE VISION) • ATAXIA • FACIAL AND OCULOMOTOR WEAKNESS (THIS IS WHY REMEMBERING THOSE CRANIAL NERVES ARE IMPORTANT) • EOMS • PUPIL RESPONSE • POOR EYE LID CLOSURE • NYSTAGMUS / INABILITY TO HOLD GAZE • WILL PROGRESS ALONG THE “DERMATOMES’ • NOTE: 15% OF GBS WILL ONLY HAVE MOTOR, BUT NO SENSORY SYMPTOMS.
- 11. NEURO • PARESTHESIA VS. DYSESTHESIAS • DYSESTHESIAS ARE BURNING OR SHOCKING SYMPTOMS • PRESENT IN 50% OF CASES, MOST COMMON IN LOWER EXT. • WILL PERSIST IN 10% OF PATIENTS AFTER RECOVERY. • 50 % WILL PRESENT WITH SIGNIFICANT PAIN INITIALLY • BACK PAIN MOST COMMON. • SHOULDER GIRDLE, BACK, BUTTOCKS, AND THIGHS PAIN ALSO. • MAY OCCUR WITH EVEN THE SLIGHTEST MOVEMENTS. THE PAIN IS OFTEN DESCRIBED AS ACHING OR THROBBING IN NATURE. • THE INTENSITY OF PAIN ON ADMISSION CORRELATES POORLY WITH NEUROLOGIC DISABILITY ON ADMISSION AND WITH THE END OUTCOME.
- 12. AIRWAY, RESPIRATORY • EXERTIONAL DYSPNEA • SOB • DIFFICULTY SWALLOWING • SLURRED SPEECH
- 13. AUTONOMIC INSTABILITY • DIARRHEA VS CONSTIPATION AND URINE RETENTION. • TACHYCARDIA VS BRADYCARDIA • ANHIDROSIS VS. DIAPHORESIS • PAROXYSMAL HYPERTENSION VS. ORTHOSTATIC HYPOTENSION AND SYNCOPE • FACIAL FLUSHING
- 15. ASCENDING? KNOW YOUR DERMATOMES! • SHARP • DULL • PRESSURE • MOVEMENT • DIFFERENTIATION
- 16. EOM ASSESSMENT WHY IS AN EYE ASSESSMENT IMPORTANT?
- 17. PROGNOSIS • 25-30% WILL REQUIRE MECHANICAL VENTILATION WITHIN ACUTE OR SUBACUTE PHASE • 10 % WILL GET BETTER AND THEN DETERIORATE ABOUT WEEK 6-10. • 20% WILL BE UNABLE TO WALK AFTER 6 MONTHS • 3-10% WILL DIE • LIFELONG MORBIDITY 10-15% • FASTER ONSET/PROGRESSION = WORSE PROGNOSIS
- 18. TREATMENT PREHOSPITAL • ASSESSMENT AND RECOGNITION • AIRWAY SUPPORT- EARLY AIRWAY MANAGEMENT? • RARELY – PACEMAKER FOR AUTONOMIC DYSREGULATION OF HEARTRATE (AV BLOCKADE) • FLUIDS AND SUPINE POSITION FOR HYPOTENSION • SHORT ACTING BETABLOCKERS FOR HYPERTENSION – EX. ESMOLOL 50 MG IVP • ANALGESICS SELDOM EFFECTIVE IN HOSPITAL • PLASMAPHERESIS • IMMUNOGLOBIN • SHORT ACTING VASOPRESSORS /ANTI-HYPERTENSIVES INTERFACILITY • REASSESSMENT • TRACH CARE (IF APPLICABLE) • PSYCH CARE • OBSERVE FOR COMPLICATIONS OF IG THERAPY • PREVENTION OF THROMBOEMBOLI
- 19. ADDITIONAL WATCHING • AS YOU WATCH THESE VIDEOS OBSERVE FOR THE TALE TALE PRESENTATIONS OF GBS. • ANDREW, A FIREFIGHTER WITH SUDDEN ONSET OF GBS. THERE ARE GREAT EXAMPLES OF THE ATAXIC GAIT AND OTHER PRESENTATIONS. • HTTPS://WWW.YOUTUBE.COM/WATCH?V=VWVRHHS2JPS • CASE OF DELILAH BOGGS • HTTPS://WWW.YOUTUBE.COM/WATCH?V=6VYWVPQ77J4
- 20. QUESTIONS?
Notas do Editor
- Guillain-Barré syndrome (GBS) is a rare and potentially catastrophic paralyzing disorder of the peripheral nerves.1 It is also known as Landry’s ascending paralysis, postinfectious polyneuropathy, and acute inflammatory demyelinating polyneuropathy. The emergency physician is often the first to see GBS patients as the illness evolves. Early diagnosis, or at least suspicion of GBS, is important to provide a better outcome as delayed treatment can lead to unrecognized failing respiration and life-threatening dysrhythmias.2–4 Given that GBS is rare, presents with the common chief complaint of weakness, and has no simple diagnostic lab test for its confirmation, it is an easy diagnosis to miss.
- https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3437179/ Octave Landry2 is credited with the first reported case of what later came to be known as the GBS. In 1859, Landry described a neurologic condition characterized by ascending motor paralysis with poor prognosis that he referred to as “ascending paralysis”. Although literature reports emphasize the ascending type of motor paralysis, Landry originally described three types of presentations in ten patients. The first part of his historic article was devoted to a description of one patient with classical ascending type of motor paralysis without sensory signs or symptoms. In the second part of his article, Landry discussed nine other patients with three types of clinical picture: 1) ascending paralysis without sensory signs and symptoms, 2) ascending paralysis with concomitant ascending anesthesia and analgesia, and 3) progressive generalized polyradiculoneuritis. Landry searched for antecedent and associated conditions in exploring the etiology of the new condition he reported. He noted that “the paucity of facts makes it impossible to study the etiology of this process, but the circumstances in which it developed can be noted”.2 The circumstances he observed included convalescence from acute illness, background of menstrual difficulties, exposure to cold, and convalescence from childbirth. In one case with menstrual difficulties, leeches applied to the vulva led to recovery. In another case, menstrual difficulties during a “moral crisis” were followed by acute ascending paralysis. Landry's contribution to medical literature ceased shortly after his 1859 report. He left academic pursuits4 to manage an institute of hydrotherapy for the treatment of the nervous disorders. Fifty seven years after Landry's article was published, Guillain, Barré and Strohl,3 in 1916, described a condition similar to that of Landry, with two distinctive features: I) better prognosis, 2) unique cerebrospinal fluid findings of increased protein with no increase in cells. Their paper described two French soldiers whose clinical picture was similar and consisted of (1) motor difficulties, (2) areflexia, (3) preservation of cutaneous reflexes, (4) paresthesias with slight impairment of objective sensations, (5) muscle tenderness, (6) slight alteration in nerve conduction, (7) remarkable increase albumin in the absence of cellular (albuminocytologic dissociation). Their paper was published in the Bulletin of the Society of Medicine of the Hospital of Paris. The motivation for performing a spinal tap in these two patients is uncertain. It is believed, however, that the authors performed a spinal tap either to rule out an infectious process, or because spinal taps were fashionable5 having been described by Quincke 25 years earlier, in 1891.
- affects peripheral nerves, primarily spinal and cranial nerve roots autonomic nerves sometimes effected two major distinct pathologies of immune injury: the myelin sheath and related Schwann-cell components are targetted in acute inflammatory demyelinating polyneuropathy membranes on the nerve axon (the axolemma) are targetted in acute motor axonal neuropathy mainly a humorally-mediated, rather than T-cell-mediated disorder, at least in the progressive phase of nerve injury recovery involves dampening of the immune response and endogenous repair of nerves Demyelination (de-myelin-ation), is an 'attack' by the immune system on the myelin covering the nerves in the brain, optic nerves, or spine. Our brain contains thousands of cells called neurons. Neurons send signals from our brain to different parts of our body, just like an electrical wire. For example, when you want to walk, your brain sends a message to your legs. Demyelination occurs when the immune cells attack and injure myelin. When this happens neurons are not able to send signals as before. For example, if the part of the brain that sends signals to your legs is affected by demyelination, the message may not reach your legs and it will be hard to walk. Demyelination can occur as a one-time illness, or it may be a relapsing, chronic disease. MS is one type of chronic demyelinating disease. What causes demyelination? Demyelination is thought to be an abnormal immune system reaction, causing the patient’s own immune cells to attack the myelin coating in the CNS. But the reason that the immune system reacts this way is still unknown
- Motor-sensory features bilateral, symmetrical limb weakness, typically ascending may initially only affect lower limbs usually starts distally, but can start more proximally may have facial, oculomotor, or bulbar weakness, which might then extend to involve the limbs (Miller-Fisher syndrome) areflexia may initially be normal or even hyper-reflexic paraesthesia sensory symptoms and signs are common usually milder than motor 15% of GBS patients have no sensory symptoms (pure motor) muscular or radicular pain commonly back pain, but not always may precede weakness in 1/3 cases Other features autonomic dysfunction diarrhoea, vomiting, dizziness, abdominal pain, ileus, orthostatic hypotension, urinary retention, bilateral tonic pupils, fluctuating heart rate and dysrhythmias, decreased sweating, salivation and lacrimation respiratory failure (affects 20-30% of cases) corneal ulceration (poor lid closure)
- Cranial nerve involvement is observed in 45-75% of patients with GBS. Cranial nerves III-VII and IX-XII may be affected. Common complaints include the following: Facial droop (may mimic Bell palsy) Diplopias Dysarthria Dysphagia Ophthalmoplegia Pupillary disturbances Guillian-Barré Syndrome (GBS) by Chris Nickson, Last updated September 10, 2017 Reviewed and revised [last-modified] OVERVIEW Guillian-Barré Syndrome (GBS) is the most common and most severe acute paralytic neuropathy, consisting of multiple variants with distinct clinical and pathological features two main pathological types: acute motor axonal neuropathy acute inflammatory demyelinating polyneuropathy can progress to a chronic form affects about 1 in 100,000 people each year (higher rates in elderly than young) may be associated with infectious outbreaks (e.g. Campylobacter infection) CAUSE GBS is an autoimmune response to an immune activating event, such as: Campylobacter jejuni enteritis (25-50%) viral (e.g. CMV, EBV, hepatitis A-E viruses, HIV, influenza, arboviruses (e.g. Zika, Chikungunya)) Mycoplasma infection vaccination (e.g. influenza, rabies) PATHOPHYSIOLOGY Precise interplay of host and infectious factors leading to autoreactivity remains uncertain immune activation event leads to autoantibody production in the case of GBS-associated with Campylobacter jejuni infection, auto-antibodies result from molecular mimicry (e.g. glycans expressed on bacterial lipooligosaccharides (LOS)) however, this occurs in <1% of people with the infection other genetic and environmental factors are presumably important but poorly understood affects peripheral nerves, primarily spinal and cranial nerve roots autonomic nerves sometimes effected two major distinct pathologies of immune injury: the myelin sheath and related Schwann-cell components are targetted in acute inflammatory demyelinating polyneuropathy membranes on the nerve axon (the axolemma) are targetted in acute motor axonal neuropathy mainly a humorally-mediated, rather than T-cell-mediated disorder, at least in the progressive phase of nerve injury recovery involves dampening of the immune response and endogenous repair of nerves The immune activating event affects the clinical phenotype and prognosis e.g. C jejuni infections are usually associated with a pure motor axonal form of GBS, more severe limb weakness, and a serological antibody response directed against GM1 and GD1a gangliosides CLINICAL FEATURES Course immune activation, usually due to infection (e.g. Campylobacter, viral or Mycoplasma) preceding respiratory or gastrointestinal infection reported by 2/3 of patients progression sensory and/or cranial nerve involvement over 1-2 weeks peak clinical deficits at 2-4 weeks usually 2 weeks subacute GBS may progress up to 6 weeks recovery period in survivors lasts months to years Severity and duration of disease is highly diverse can range from mild weakness with spontaneous recovery to quadriplegic and ventilator-dependence without signs of recovery for several months resulting in severe, permanent disability though all surviving patients will show some signs of improvement Motor-sensory features bilateral, symmetrical limb weakness, typically ascending may initially only affect lower limbs usually starts distally, but can start more proximally may have facial, oculomotor, or bulbar weakness, which might then extend to involve the limbs (Miller-Fisher syndrome) areflexia may initially be normal or even hyper-reflexic paraesthesia sensory symptoms and signs are common usually milder than motor 15% of GBS patients have no sensory symptoms (pure motor) muscular or radicular pain commonly back pain, but not always may precede weakness in 1/3 cases Other features autonomic dysfunction diarrhoea, vomiting, dizziness, abdominal pain, ileus, orthostatic hypotension, urinary retention, bilateral tonic pupils, fluctuating heart rate and dysrhythmias, decreased sweating, salivation and lacrimation respiratory failure (affects 20-30% of cases) corneal ulceration (poor lid closure)
- ost patients complain of paresthesias, numbness, or similar sensory changes. Sensory symptoms often precede the weakness. Paresthesias generally begin in the toes and fingertips, progressing upward but generally not extending beyond the wrists or ankles. Loss of vibration, proprioception, touch, and pain distally may be present. Sensory symptoms are usually mild. In most cases, objective findings of sensory loss tend to be minimal and variable.
- Normally, the eyes move in concert (e.g. when the left eye moves left, the right eye moves left to a similar degree). The brain takes the input from each eye and puts it together to form a single image. This coordinated movement depends on 6 extraocular muscles that insert around the eye balls, allowing them to move in all directions. Each muscle is innervated by one of 3 Cranial Nerves (CNs): CNs 3 (Oculomotor), 4 (Trochlear) and 6 (Abducens). Movements are described as: elevation (pupil directed upwards), depression (pupil directed downwards), adbduction (pupil directed laterally), adduction (pupil directed medially), extorsion (top of eye rotating away from the nose), and intorsion (top of eye rotating towards the nose). We'll first review the individual Extraocular Muscles (EOMs), then the CNs which innervate them, and lastly functional testing and pathology.
- https://www.hopkinsmedicine.org/transplant/programs/kidney/incompatible/plasmapheresis.html Plasmapheresis is similar to dialysis; however, it removes the plasma portion of the blood where the antibodies are located. Only plasma exchange (PE) therapy and intravenous immune globulin (IVIG) have proven effective for Guillain-Barré syndrome (GBS). They may decrease autoantibody production and increase solubilization and removal of immune complexes. Both have been shown to shorten recovery time by as much as 50%. IVIG is easier to administer and has fewer complications than PE.[12, 13] The cost and efficacy of each are comparable. Randomized trials in severe disease show that IVIG started within 4 weeks from onset hastens recovery as much as plasma exchange.[14, 12, 15, 16, 17] Combining PE and IVIG neither improved outcomes nor shortened illness duration.[18] IVIG has also been found safe and effective in the treatment of pediatric GBS.[18, 19] Additionally, IVIG is the preferential treatment in hemodynamically unstable patients and in those unable to ambulate independently.[20, 18] Some evidence suggests that in select patients who do not respond initially to IVIG, a second dose may be beneficial.[21] However, this is not currently standard therapy and warrants further investigation.