A Case of Guillain-Barre Syndrome in a Primary Care Setting
Transcription
A Case of Guillain-Barre Syndrome in a Primary Care Setting
A Case of Guillain-Barre Syndrome in a Primary Care Setting Sherly Sebastian, DNP, RN, NP-C ABSTRACT Guillain-Barre Syndrome (GBS) is an immune-mediated peripheral neuropathy characterized by rapidly progressive symmetrical ascending weakness and sensoiy loss. The disease can progress rapidly and be fatal i f diagnosis and treatment interventions are delayed. I n most patients, the neuropathic symptoms are preceded by an antecedent infection. The patient may present w i t h initial symptoms o f upper respiratory tract infection or gastroenteritis. This article presents a case report o f GBS, followed by a detailed discussion o f the pathophysiology, diagnostic studies, differential diagnosis, types, prognosis, and management o f patients w i t h this condition. Keywords: ascending weakness, immune response, peripheral neuropathy, respiratory failure, sensory loss © 2012 American College of Nurse Practitioners G uillain-Barre Syndrome (GBS) is an i m m u n e - ness, tingling, and weakness in her legs. The patient was mediated peripheral neuropathy characterized initially seen in the clinic 3 weeks ago w i t h upper respi- by rapidly progressive symmetrical ascending ratory tract infection and was treated w i t h a course o f weakness and sensory loss. The disease is named after the amoxicillin. Her upper respiratory symptoms improved, physicians Guillain, Barre, and Strohl, w h o described the but a week later she noticed numbness and tingling in clinical presentation in 2 French soldiers during the First her feet, progressively ascending to her legs and thighs. World War about 100 years ago.' According to the She was evaluated at the local hospital, and her diagnostic National Institute o f Neurological Disorders and Stroke work-up included blood cultures and spinal tap.The figures,' the annual incidence rate for GBS is about 1-2 emergency r o o m (ER) physician consulted the neurolo- per population o f 100,000. I n the acute phase o f GBS, gist, but the patient left E R against medical advice. approximately 3% o f patients may die from acute c o m p l i - ^ T h e numbness became progressively worse, and she cations and up to 20% have residual, permanent, severe started having bilateral lower extremity weakness and gait disabilit)' w i t h ambulation deficits or require ventilator issues. She fell 2 days ago, and her family forced her to assistance up to 12 months later,-' seek medical help. Her past medical history was unre- The annual financial burden from GBS is estimated at $1.7 bilhon, including 10.2 bilhon (14%) in direct med- markable except for the upper respiratory infection ( U R I ) 3 weeks ago. The patient's review o f systems was ical costs and $1.5 billion (86%) in indirect costs from negative for shortness o f breath, fever, chilis, cough, and lost productivity or premature death.** Even though inci- swallowing difficulties. H e r vital signs were temperature dence is low, the economic cost o f GBS is substantial as a 3 7 ° C , pulse 112/minute, blood pressure 108/62, respira- result o f high mortahty and morbidity. tion 28/minute, weight 162 lbs, height 5'2". CASE REPORT oriented X 3, w i t h intact speech, comprehension, and mem- This patient is a 36-year-old Hispanic woman w h o pre- 017; pupils equal round reactive to light. Dysmetria was sented to the outpatient clinic w i t h complaints o f numb- noted for finger-to-nose and rapid alternating movements O n neurological examination, the patient was alert and www.npjournal.org The Journal for Nurse Practitioners - J N P 643 Table 1. Guillain-Barre Syndrome Variants Acute Inflammatoty Demyelinating Polyneuropathy (AIDP)^' Most prevalent in United States and Europe Immune response at myelin sheath and Schwann cells Cranial and sensory nerves more affected than motor nerves Acute Motor Axonal Neuropathy (AMAN) More prevalent in pediatric group Immune response is against motor axon membranes Motor involvement without sensory findings Acute Motor and Sensor Axonal Neuropathy (AMSAN) More rapid progression and paralysis with sensory and motor deficits Axonal degeneration of ventral and dorsal nerve roots Prolonged recovery with painful sensory component Miller-Fisher Syndrome (MFS) Rare subtype, classic findings of areflexia, ataxia, and ophthalmoplegia Acute Panautonomic Neuropathy Very rare subtype affecting both sympathetic and parasympathetic systems Recovery is slow and incomplete, with high mortality and morbidity Table 2. Clinical Manifestations^" A. Clinical symptoms definitive of GBS Bilateral symmetrical ascending motor weakness, areflexia B. Clinical symptoms supportive of GBS Ataxia, cranial nerve palsies Respiratory muscle weakness and paralysis Numbness/paresthesia on extremities Decreased proprioception Pain in the lower extremities and back, fatigue Bilateral facial weakness, difficulty with eye movements Chewing and swallowing difficulties Autonomic dysfunction C. Clinical symptoms not supportive of GBS Unilateral weakness, hyperreflexia Sudden onset of weakness Changes in level of consciousness Asymmetry of weakness and paresthesia Severe paresthesia without motor weakness Severe respiratory symptoms with limited motor weakness Bladder or bowel dysfunction at the onset of symptoms bilaterally. Her cranial nerve exam was intact. Her motor peripheral myelin sheath. The commonly recognized strength in triceps, biceps, and supinator were 5/5; deltoids variants are summarized i n Table 1. and pronator were 4/5; wrist extensors and flexors were The patient in the case study provides many o f the 4/5 bilaterally; hip flexors, quadriceps, and hamstrings classic characteristics o f GBS, such as progressive were 4/5 bilaterally; dorsiflexors and plantar flexors were development o f ascending symmetrical paresthesia, 3/5 bilaterally. Her sensation was intact on upper extremities pain, bilateral m o t o r weakness, areflexia, and ataxia. but diminished to touch and pin prick on lower extremities Accurate diagnosis requires clues from the clinical his- and abdomen. Her gait was ataxic, deep tendon reflex tory, such as onset, severity', and rate o f progression o f ( D T R ) 1 + and symmetrical on deltoid, biceps, brachio- the symptoms over hours to days. A careful n e u r o l o g i - radialis and triceps; D T R was absent on knees and ankles. cal exammation m c l u d i n g the m o t o r strength, cranial The patient's laboratory studies did not reveal any infectious process. H e r cerebrospinal fluid (CSF) studies nerves, and reflexes is essential. Clinical manifestations are summarized i n Table 2. from the hospital revealed elevated protein count o f 200 m g / d L , w i t h normal cell count, w h i c h confirmed Antecedent Infections the diagnosis o f GBS. The disease can progress rapidly The history o f a prodromal respiratory infection should and can be potentially fatal i f treatment interventions are raise suspicion for GBS, because in majority o f GBS delayed. The patient was transported expeditiously to the patients, neuropathic symptoms are preceded w i t h a U R l nearest hospital for admission to the intensive care unit. or enteric infection. History o f cough, fever, sore throat, diarrhea, or any other types o f infections 2-3 weeks DIAGNOSIS before the onset o f weakness should alert the clinician to GBS is a heterogeneous syndrome w i t h several variant suspect GBS. Campylobacter jejuni, a bacteria commonly forms affecting the peripheral nervous system as a result associated w i t h gastroenteritis, have been identified as the o f an immune-mediated disturbance involving the most frequent antecedent pathogen o f GBS.' 644 The Journal for Nurse Practitioners - JNP Volume 8, Issue 8, September 2012 Vaccinations The evolution o f GBS after vaccination has been studied Table 3. Differential Diagnoses^^'^" Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) extensively, as concerns about vaccine-induced GBS were S y m p t o m s similar t o GBS but progressing over a longer initially raised after the 1976-77 influenza vaccination period ( > 8 weeks) period.The landmark study done by Lasky et al ' reported marginally increased risk o f GBS after influenza vaccination during the first 6 weeks o f immunization. Subsequently, researchers investigated the relationship between GBS and the influenza vaccinations and reported that low relative risks were not statistically significant.'"Extensive research is reported after the H l N l mass vaccination i n 2009 and found no evidence o f an increased risk o f GBS after the seasonal influenza vaccine or the H l N l vaccination." Myasthenia Gravis (MG) Weakness in t h e v o l u n t a r y muscles c o n t r o l l i n g t h e eyelids, face, s w a l l o w i n g Weakness and fatigue p r o n o u n c e d w i t h effort and relieved w i t h rest Diplopia and/or ptosis, n o r m a l sensation and reflexes Anterior Horn Cell Abnormalities S o m e similar features, but weakness pattern is different Clinical signs of infection, i n c l u d i n g high cell count in cerebrospinal fluid Spinal Cord Disorders Radiculopathy, unilateral m o t o r and sensory deficits Diagnostic Studies Hyperreflexia, sharp sensory levels CSF analysis w i t h evidence o f high protein and normal Pain aggravated w i t h activities cell count support the diagnosis o f GBS. The value o f lumbar puncture is limited i n the early phase o f the disease. CSF may remain normal in up to 50% o f patients Intracranial Abnormalities Change In level o f consciousness, severe headaches Unilateral m o t o r and sensory deficits early i n the disease, but elevated protein w i l l be present i n more than 90% o f the patients by the time they reach clinical nadir.'* However, increased CSF ceD count steers to make the diagnosis on clinical grounds and promptly cUnician to consider other diagnoses and investigate pos- refer to hospital/specialist care.*^''-* sibility o f infectious process, such as leptomeningeal malignancy. West N i l e virus infection, HIV, or Lyme dis- Differential Diagnosis ease.'- I f the CSF results are normal, repeat testing is not Ascending symmetrical weakness progressing over hours done typically. to days is the cardinal symptom o f GBS. I f the symptoms Electrodiagnostic testing is done frequently to iden- are prolonged over 8 weeks, other diagnoses should be tify the acute motor weakness caused by a peripheral considered. I f the weakness is asymmetric, clinicians neuropathy. T h e test is helpful i n confirming the diagno- should consider a spinal or intracranial diagnosis instead sis and differentiating the variants o f GBS. However, o f GBS. I f there is weakness w i t h intact reflexes, another early in the disease process, the testing may be normal. diagnosis should be investigated. The predominant fea- The features o f deniyelination i n GBS include slow con- tures o f Miller Fisher Syndrome, such as ophthalmo- duction, temporal dispersion, and prolonged or absent plegia, areflexia, and ataxia, often mistakenly suggest F-responses.^ Abnormal median and ulnar sensory brain stem infarction. A wide range o f neurological disor- potentials w i t h spared sural potentials are seen i n the ders may mimic the symptoms o f GBS; most comnion initial stages o f GBS.'•'The nerve conduction study is differentials are hsted i n Table 3. considered a useful confirmatory test, w h i c h typically demonstrates the finding o f reduced muscle action PATHOPHYSIOLOGY potentials in chnically weak m u s c l e s . M a g n e t i c reso- The pathogenesis o f GBS has been widely studied but is nance imaging ( M R I ) is typically performed to rule out still not completely understood.The proposed mecha- infiltrative or structural causes o f weakness. Moreover, nism involves an antecedent infection leading to an auto- M R I may reveal enhancement o f the affected nerve immune response reacting w i t h peripheral nerve roots supportive o f GBS diagnosis. The hmitations o f components (Figure 1). Most o f the pathogens gains these tests i n the early phase o f the disease, combined entry to the body through mucosal or gut epithehum w i t h the urgency for early treatment, require clinicians and induce antibody production against specific ganglio- www.npjournal.org The Journal for Nurse Practitioners - JNP 645 JNP r Figure 1. Guillain-Barre Syndrome Pathogenesis. Disease-modifying factors Pathogenesis and recovery Bacterial factors • LOS biosynthesis cluster • Ganglioside mimicry of LOS Campylobacterjejuni infection Tcell Host factors • Genetic polymorphisms • Immune status Immune response to LOS Antigen presenting cell Plasma cell Cross-reactive antibodies to nerve gangliosides ^ k h Macrophage Extent of nerve damage • Ganglioside distribution in nerves • Specificity/affinity antibodies • Complement activation Nerve dysfunction Complement Conduction dysfunction/block I X Clinical prognostic factors -Age • Severity at onset ' Diarrhoea Outcome van Doom PA, Ruts L, Jacobs 2008;7:939-950. 646 BC. Clinical features, patfiogenesis, Reprinted with permission from The Journal for Nurse Practitioners - JNP and treatment of Guillain-Barre Syndrome. Lancet Neurol. Elsevier. Volume 8, Issue 8, September 2012 sides i n myelin/' The immune response depends on bac- Table 4. Autonomic Dysfunction^'^^ terial factors, such as the specificity o f lipo-ohgosaccha- Tachycardia, bradycardia ride (LOS), and on host factors, such as genetic Hypotension, hypertension polymorphism and immune status.'"The presence o f antibodies leads to activation o f T cells and complements, leading to a cascade o f inflammation and demyelination. Facial flushing, anhidrosis, hyperhidrosis Constipation, paralytic ileus The demyelination decreases the velocity o f nerve conduction and slows the impulse transmission along the exchange o f about 5 plasma volumes. The Cochrane axons. The extent o f nerve damage varies, w i t h more Review'^ demonstrated that I V I g and PE are beneficial damage seen in the intensely myelinated peripheral for accelerating the recovery o f GBS, i f given during the nerves, causing motor and sensory weakness. first 4 weeks o f the disease, w i t h most benefit seen i f The self-limiting nature o f the disease process given w i t h i n the first 2 weeks o f symptoms. should be taken into account, symptoms improve once the autoimmune inflammatory process is terminated, CORTICOSTEROIDS and the Schwann cells reverse the process to rebuild the The role o f steroids i n GBS treatment has been widely myelination o f the nerves. I n severe cases, the inflamma- studied, and researchers concluded that oral corticos- tory process can lead to axonal disruption and teroids were not beneficial in GBS treatment and did not permanent damage. recommend it as the first-line therapy."'This finding is in contrast to the standard treatment for other demyelinat- SUPPORTIVE CARE ing diseases for which favorable response is noted w i t h Prevention o f life-threatening complications remains the steroid therapy. I n 1. o f the studies, the use o f intravenous cornerstone o f supportive care. Progressive demyehnation corticosteroids in combination w i t h I V I g demonstrated a o f the phrenic nerve, innervating the diaphragm, may lead short-term improvement i n clinical symptoms when to acute respiratory muscle paralysis. Early detection o f res- compared to I V I g treatment a l o n e . L o n g - t e r m use o f piratory failure and anticipation o f mechanical ventilation corticosteroids causes numerous side effects and may are crucial to avoid emergency intubation and cardiopul- inhibit macrophage repair process, but short-term monary arrest. Life-threatening episodes o f hemodynamic treatment did not result i n serious side effects.'^ instability related to autonomic dysfunction may occur in GBS patients (Table 4).They should be admitted to the PAIN MANAGEMENT hospital for close monitoring o f respiratory status, hemo- Neuropathic pain occurs i n large number o f patients dynamic instability, and autonomic dysfunction. w i t h GBS and often requires recognition and treatment. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be IMMUNOTHERAPY tried initially, but they often do not provide adequate Immunotherapy should be initiated as soon as possible pain relief, as the origin o f pain is usually multifactorial. w i t h high dose intravenous immunoglobuhn (IVIg) or Pain in the acute phase might be nociceptive related to plasma exchange (PE).The mechanism o f action o f I V I g inflammation, whereas later in the course, pain may be involves modulating the humoral response by suppressing related to degeneration o f sensory nerve fibers.'^ Early autoantibody production.^ I V I g also blocks the binding recognition and treatment w i t h gabapentin or carba- o f receptors on macrophages, suppressing the various mazepine are suggested to be effective i n treating neuro- inflammatory mediators. The typical dose o f I V I g is pathic pain. Narcotic analgesics should be used w i t h 0.4g/kg per day for 5 days, started as a lower dose and caution as they precipitate ileus in the setting o f increased to the m a x i m u m dosage based on patient toler- autonomic dysfunction. ance. The goal o f PE is to remove circulating i m m u n e reactive antibodies, complements, and biological response PROGNOSIS modifiers. The factors indicating poor prognosis include older age, The treatment regimen depends on the disease severity; typically, PE is given 5 times i n 2 weeks, for a total www.npjournal.org infection, prolonged hospital stay, need for mechanical ventilation and intensive care, and poor upper extremity The Journal for Nurse Practitioners - J N P 647 Table 5. Complications^' Respiratory failure, cardiac arrhythmias Swallowing difficulties, aspiration pneumonia Deep vein thrombosis, foot drop and joint contractures Bladder and bowel dysfunction, paralytic ileus weakness." G B S patients may also exhibit pain, fatigability', and functional impairments and may have permanent neurologic impairments, such as muscle wasting, ataxia, foot drop, and dysesthesia."^ R e c o v e r y from GBS varies; most patients recover within 6-12 months, but some may take longer. T h r o m b o e m b o l i c complications such as deep vein thrombosis and pulmonary embolus may be prevented with use o f fractionated or unfractionated heparin and compression stockings.** Early initiation o f an individualized program for muscle strengthening and physical therapy is essential to prevent complications (Table 5). EDUCATION AND COUNSELING Clearly, GBS management includes applying an improved understanding of the pathophysiology to individual patients and the effect that it has on the vital organs and tissues. Most patients develop secondary complications, and tailoring o f supportive care and education should be based on their unique needs. T h e care of a G B S patient is challenging for the health care team and the caregivers because some symptoms can be devastating. G B S is a life event with a potentially long-lasting influence on physical and psychosocial well-being, transforming a healthy, independent person into a critically ill and physically helpless p e r s o n . ' ' T h e acute progression of motor weakness and fatigue makes a profound effect on the patient's quahty of hfe. Several neuromuscular disease organizations provide resources to assist w i t h community support networks. T h e Guillain-Barre S y n d r o m e / C h r o n i c Inflammatory Demyelinating Polyneuropathy Foundation (http://www.gbs-cidp.org) is an excellent place to get support, find up-to-date information, and seek opportunities to network. CONCLUSION Even though significant advances have been made regarding the i m m u n o l o g y and pathophysiology,' o f GBS, It is still continuing as a challenging neurological diagnosis associated with high morbiditx' and mortality. GBS 648 The Journal for Nurse Practitioners - J N P should be considered in the differential diagnosis for patients presenting to primary care setting with lower extremity numbness with antecedent history of upper respiratory infection or gastroenteritis.The long-term prognosis is dependent on early diagnosis and treatment and knowledge o f prognostic factors can substantially improve patient care. Biti 1. Winer JB. Guillain Barre syndrome. Mol Path. 2001;54(6l:381-385. 2. National Institute of Neurological Disorders and Stroke. Guillain-Barre syndrome fact sheet. http://wvtfW.ninds.nih.gov/disorders/gbs/detail_gbs.htm. Accessed July 16, 2012. 3. Khan F, Amatya B, Ng L. Use of the international classification of functioning, disability and health to describe patient-reported disability: a comparison of Guillain Barre syndrome with multiple sclerosis in a community cohort. J Rehabil Med. 2010;42(B):708-714. 4. Frenzen PD. Economic cost of Guillain-Barre syndrome in the United States. Neurology. 2008;71{l):21-27. 5. Newswanger DL, Warren CR. Guillain-Barre syndrome. Am Fam Physician. 2004;69(1):2405-2410. 6. Pithadia A, Kakadia N. Guillain-Barre syndrome. Pharmacol Rep. 2010;62(l):220-232. 7. Davids HR, Oleszek JL, Cha-Kim A. Guillain-Barre syndrome. http://emedicine.medscape.com/article/315632. Accessed August 9, 2011. 8. Burns TM. Guillain-Barre Syndrome. Sem Neurol. 2008;28(2):1S2-167. 9. Lasky T, Terracciano GJ, Magder L, et al. The Guillain -Barre syndrome and the 1992-1993 and 1993-1994 influenza vaccines. N Engl J Med. 1998;339(25):1797-1802. 10. Kuitwaard K, Bos-Eyssen ME, Blomkwist-Markens PH, van Doom PA. Recurrences, vaccinations and long-term symptoms in GBS and CIDP. J Peripher Nerv Syst. 2009;14(4):3ia-315. 11. Lehmann HC, Hartung HP Kieseier BC, Hughes RA. Guillain-Barre syndrome after exposure to influenza virus. Lancet Infect Dis. 2010:10(9):643-65. 12. Van Doom PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barre syndrome. Lancet Neurol. 2008;7(10):939-950. 13. Winer JB. Guillain-Barre syndrome. Br Med J. 2008;337:227-231. 14. Spalice A, Parisi P Papetti L, et al. Clinical and pharmacological aspects of inflammatory demyelinating diseases in childhood: an update. Curr NeuropharmacoL 2010;8(2):135-148. 15. Hughes RA, Raphael JC, Swan AV, van Doom PA. Intravenous immunoglobulin for Guillain-Barre syndrome. Cochrane Database Syst Rev. 2006;1:CD002063. 16. Bromberg MB, Carter O. Corticosteroid use in the treatment of neuromuscular disorders: empirical and evidence-based data. Muscle Nerve. 2004;30:20-37. 17. Khan F, Pallant JF Ng L, Bhasker A. Factors associated with long-term functional outcomes and psychological sequelae in Guillain-Barre syndrome. J Neurol. 2010;257(12):2024-203. 18. Rudolph T, Larsen JP Farbu E. The long-term functional status in patients with Guillain-Barre syndrome. Europ J Neurol. 2008;15(12):1332-1337. 19. Bernsen RA, de Jager AE, van der Meche FG, Suurmeijer TP How GuillainBarre patients experience their functioning after 1 year. ,4cta Neurol Scand. 2005;112(l):51-56. Sheiiy Sebastian, DNP, R.N, NP-C, is a luirsc practitioner in the neurosurgery department at Baylor College of Medicine in Houston, TX, and can be reached at sherlysebastian@sbcglobal.net. In compliance with national ethical guidelines, the author reports no relationships with business or industry that would pose a conflict of interest. 1565-4156/121/S see front matter © 2012 American College of Nurse Practitioners http://dx.doi.Org/10.1016/j.nurpra.2012.04.015 Volume 8, Issue 8, September 2012