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DOI: 10.1055/s-0041-1733794
Inpatient Diagnosis and Management of Neuromuscular Disorders
Funding No financial support or recognition of personal assistance was received for the work being published.Abstract
Although many neuromuscular conditions are evaluated on an outpatient basis owing to their chronic or progressive nature, more urgent evaluation and management is often required for the inpatient presenting with acute to subacute focal or generalized numbness or weakness. This review focuses on clinical pattern recognition and basic anatomic localization principles to aid in the identification of common, as well as some less frequently encountered, neuromuscular disorders in hospitalized patients. The characteristic clinical and diagnostic features, associated complications, and recommended treatments of key neuromuscular conditions with acute and subacute manifestations are discussed. These conditions can be life-threatening in some cases, such as in Guillain–Barré syndrome, owing to associated oropharyngeal weakness, respiratory failure, or marked dysautonomia. Prompt recognition of the clinical and pathologic features is therefore necessary to reduce associated morbidity and mortality.
Keywords
neuromuscular - acute weakness - Guillain–Barré syndrome - myasthenia gravis - inflammatory myopathyPublication History
Article published online:
07 October 2021
© 2021. Thieme. All rights reserved.
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References
- 1 Chaudhry V. Peripheral neuropathy. In: Fauci AS, Braunwald E, Kasper DL. et al, eds. Harrison's Principles of Internal Medicine. 17th ed.. New York: McGraw-Hill Professional; 2008: 2651-2667
- 2 Hobson-Webb LD, Juel VC. Common entrapment neuropathies. Continuum (Minneap Minn) 2017; 23 ( 2, Selected Topics in Outpatient Neurology): 487-511
- 3 Swash M, Schwartz MS. Classification of neuromuscular diseases. In: Swash M, Schwartz MS. eds. Neuromuscular Diseases: A Practical Approach to Diagnosis and Management. London: Springer; 1997: 85-87
- 4 Bennett DLH, Groves M, Blake J. et al. The use of nerve and muscle biopsy in the diagnosis of vasculitis: a 5 year retrospective study. J Neurol Neurosurg Psychiatry 2008; 79 (12) 1376-1381
- 5 Collins MP, Dyck PJB, Gronseth GS. et al; Peripheral Nerve Society. Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst 2010; 15 (03) 176-184
- 6 Amato A, Russell J. Neuromuscular Disorders. 2nd ed.. McGraw-Hill Education; 2015
- 7 Gwathmey KG, Tracy JA, Dyck PJB. Peripheral nerve vasculitis: classification and disease associations. Neurol Clin 2019; 37 (02) 303-333
- 8 Ferrante MA. Brachial plexopathies: classification, causes, and consequences. Muscle Nerve 2004; 30 (05) 547-568
- 9 van Alfen N, van Engelen BGM. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain 2006; 129 (Pt 2): 438-450
- 10 Van Eijk JJJ, Groothuis JT, Van Alfen N. Neuralgic amyotrophy: an update on diagnosis, pathophysiology, and treatment. Muscle Nerve 2016; 53 (03) 337-350
- 11 van Eijk JJJ, Dalton HR, Ripellino P. et al. Clinical phenotype and outcome of hepatitis E virus-associated neuralgic amyotrophy. Neurology 2017; 89 (09) 909-917
- 12 van Alfen N, van Engelen BGM, Hughes RAC. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev 2009; 8 (03) CD006976
- 13 van Eijk JJJ, van Alfen N, Berrevoets M, van der Wilt GJ, Pillen S, van Engelen BGM. Evaluation of prednisolone treatment in the acute phase of neuralgic amyotrophy: an observational study. J Neurol Neurosurg Psychiatry 2009; 80 (10) 1120-1124
- 14 Smith CC, Bevelaqua AC. Challenging pain syndromes: Parsonage-Turner syndrome. Phys Med Rehabil Clin N Am 2014; 25 (02) 265-277
- 15 Llewelyn D, Llewelyn JG. Diabetic amyotrophy: a painful radiculoplexus neuropathy. Pract Neurol 2019; 19 (02) 164-167
- 16 Russell JA. General approach to peripheral nerve disorders. Continuum (Minneap Minn) 2017; 23 (5, Peripheral Nerve and Motor Neuron Disorders): 1241-1262
- 17 Morrison B, Chaudhry V. Medication, toxic, and vitamin-related neuropathies. Continuum (Minneap Minn) 2012; 18 (01) 139-160
- 18 Gwathmey KG. Sensory neuronopathies. Muscle Nerve 2016; 53 (01) 8-19
- 19 Crowell A, Gwathmey KG. Sensory neuronopathies. Curr Neurol Neurosci Rep 2017; 17 (10) 79
- 20 Sopacua M, Hoeijmakers JGJ, Merkies ISJ, Lauria G, Waxman SG, Faber CG. Small-fiber neuropathy: expanding the clinical pain universe. J Peripher Nerv Syst 2019; 24 (01) 19-33
- 21 Hsu CH, Kader M, Mahamud A. et al. Progress toward poliomyelitis eradication - Pakistan, January 2018-September 2019. MMWR Morb Mortal Wkly Rep 2019; 68 (45) 1029-1033
- 22 Marx A, Glass JD, Sutter RW. Differential diagnosis of acute flaccid paralysis and its role in poliomyelitis surveillance. Epidemiol Rev 2000; 22 (02) 298-316
- 23 Boonyapisit K. Poliomyelitis and post-poliomyelitis syndrome. In: Katirji B, Kaminski HJ, Ruff RL. eds. Neuromuscular Disorders in Clinical Practice. New York: Springer New York; 2014: 383-393
- 24 Murphy OC, Pardo CA. Acute flaccid myelitis: a clinical review. Semin Neurol 2020; 40 (02) 211-218
- 25 Sejvar JJ, Marfin AA. Manifestations of West Nile neuroinvasive disease. Rev Med Virol 2006; 16 (04) 209-224
- 26 O'Malley R, Rao G, Stein P, Bandmann O. Porphyria: often discussed but too often missed. Pract Neurol 2018; 18 (05) 352-358
- 27 Ali F, Kumar N, Dyck PJB, Berini S, Klaas J. Porphyria: a rare differential diagnosis of polyradiculoneuropathy. J Neurol Sci 2019; 402: 153-155
- 28 Pasnoor M, Dimachkie MM. Approach to muscle and neuromuscular junction disorders. Continuum (Minneap Minn) 2019; 25 (06) 1536-1563
- 29 Vincent A, Palace J, Hilton-Jones D. Myasthenia gravis. Lancet 2001; 357 (9274): 2122-2128
- 30 Grob D, Brunner N, Namba T, Pagala M. Lifetime course of myasthenia gravis. Muscle Nerve 2008; 37 (02) 141-149
- 31 Hehir MK, Silvestri NJ. Generalized myasthenia gravis: classification, clinical presentation, natural history, and epidemiology. Neurol Clin 2018; 36 (02) 253-260
- 32 Lazaridis K, Tzartos SJ. Autoantibody specificities in myasthenia gravis; implications for improved diagnostics and therapeutics. Front Immunol 2020; 11: 212
- 33 Limburg PC, The TH, Hummel-Tappel E, Oosterhuis HJ. Anti-acetylcholine receptor antibodies in myasthenia gravis. Part 1. Relation to clinical parameters in 250 patients. J Neurol Sci 1983; 58 (03) 357-370
- 34 Lanska DJ. Diagnosis of thymoma in myasthenics using anti-striated muscle antibodies: predictive value and gain in diagnostic certainty. Neurology 1991; 41 (04) 520-524
- 35 Rivner MH, Swift TR. Thymoma: diagnosis and management. Semin Neurol 1990; 10 (01) 83-88
- 36 Witoonpanich R, Dejthevaporn C, Sriphrapradang A, Pulkes T. Electrophysiological and immunological study in myasthenia gravis: diagnostic sensitivity and correlation. Clin Neurophysiol 2011; 122 (09) 1873-1877
- 37 Cavalcante P, Le Panse R, Berrih-Aknin S. et al. The thymus in myasthenia gravis: site of “innate autoimmunity”?. Muscle Nerve 2011; 44 (04) 467-484
- 38 Drachman DB. Myasthenia gravis. N Engl J Med 1994; 330 (25) 1797-1810
- 39 Paik JJ, Corse AM, Mammen AL. The co-existence of myasthenia gravis in patients with myositis: a case series. Semin Arthritis Rheum 2014; 43 (06) 792-796
- 40 Edmundson C, Bird SJ. Acute manifestations of neuromuscular disease. Semin Neurol 2019; 39 (01) 115-124
- 41 Barth D, Nabavi Nouri M, Ng E, Nwe P, Bril V. Comparison of IVIg and PLEX in patients with myasthenia gravis. Neurology 2011; 76 (23) 2017-2023
- 42 Guptill JT, Sanders DB, Evoli A. Anti-MuSK antibody myasthenia gravis: clinical findings and response to treatment in two large cohorts. Muscle Nerve 2011; 44 (01) 36-40
- 43 Sanders DB, Wolfe GI, Benatar M. et al. International consensus guidance for management of myasthenia gravis: executive summary. Neurology 2016; 87 (04) 419-425
- 44 Dhillon S. Eculizumab: a review in generalized myasthenia gravis. Drugs 2018; 78 (03) 367-376
- 45 Wolfe GI, Kaminski HJ, Aban IB. et al; MGTX Study Group. Randomized trial of thymectomy in myasthenia gravis. N Engl J Med 2016; 375 (06) 511-522 . Doi: 10.1056/NEJMoa1602489
- 46 Kolb NA, Trevino CR, Waheed W. et al. Neuromuscular complications of immune checkpoint inhibitor therapy. Muscle Nerve 2018; DOI: 10.1002/mus.26070.
- 47 Haanen JBAG, Carbonnel F, Robert C. et al; ESMO Guidelines Committee. Management of toxicities from immunotherapy: ESMO Clinical Practice Guidelines for diagnosis, treatment and follow-up. Ann Oncol 2017; 28 (Suppl. 04) iv119-iv142
- 48 Titulaer MJ, Lang B, Verschuuren JJ. Lambert-Eaton myasthenic syndrome: from clinical characteristics to therapeutic strategies. Lancet Neurol 2011; 10 (12) 1098-1107
- 49 Titulaer MJ, Soffietti R, Dalmau J. et al; European Federation of Neurological Societies. Screening for tumours in paraneoplastic syndromes: report of an EFNS task force. Eur J Neurol 2011; 18 (01) 19-e3
- 50 Anwar A, Saleem S, Ahmed MF, Ashraf S, Ashraf S. Recent advances and therapeutic options in Lambert-Eaton myasthenic syndrome. Cureus 2019; 11 (08) e5450
- 51 Bain PG, Motomura M, Newsom-Davis J. et al. Effects of intravenous immunoglobulin on muscle weakness and calcium-channel autoantibodies in the Lambert-Eaton myasthenic syndrome. Neurology 1996; 47 (03) 678-683
- 52 Shapiro RL, Hatheway C, Swerdlow DL. Botulism in the United States: a clinical and epidemiologic review. Ann Intern Med 1998; ;1; 129 (03) 221-228
- 53 Guidon AC. Lambert-Eaton myasthenic syndrome, botulism, and immune checkpoint inhibitor-related myasthenia gravis. Continuum (Minneap Minn) 2019; 25 (06) 1785-1806
- 54 Sobel J, Rao AK. Making the best of the evidence: toward national clinical guidelines for botulism. Clin Infect Dis 2017; 66 (Suppl. 01) S1-S3
- 55 Warren JD, Blumbergs PC, Thompson PD. Rhabdomyolysis: a review. Muscle Nerve 2002; 25 (03) 332-347
- 56 Horn J, Hermans G. Intensive care unit-acquired weakness. Handb Clin Neurol 2017; 141: 531-543
- 57 Hermans G, De Jonghe B, Bruyninckx F, Van den Berghe G. Interventions for preventing critical illness polyneuropathy and critical illness myopathy. Cochrane Database Syst Rev 2014; (01) CD006832
- 58 Stevens RD, Marshall SA, Cornblath DR. et al. A framework for diagnosing and classifying intensive care unit-acquired weakness. Crit Care Med 2009; 37 (10, Suppl): S299-S308
- 59 Rich MM, Bird SJ, Raps EC, McCluskey LF, Teener JW. Direct muscle stimulation in acute quadriplegic myopathy. Muscle Nerve 1997; 20 (06) 665-673
- 60 Latronico N, Bolton CF. Critical illness polyneuropathy and myopathy: a major cause of muscle weakness and paralysis. Lancet Neurol 2011; 10 (10) 931-941
- 61 Hermans G, Wilmer A, Meersseman W. et al. Impact of intensive insulin therapy on neuromuscular complications and ventilator dependency in the medical intensive care unit. Am J Respir Crit Care Med 2007; 175 (05) 480-489
- 62 Selva-O'Callaghan A, Pinal-Fernandez I, Trallero-Araguás E, Milisenda JC, Grau-Junyent JM, Mammen AL. Classification and management of adult inflammatory myopathies. Lancet Neurol 2018; 17 (09) 816-828
- 63 Lundberg IE, Miller FW, Tjärnlund A, Bottai M. Diagnosis and classification of idiopathic inflammatory myopathies. J Intern Med 2016; 280 (01) 39-51
- 64 Greenberg SA. Inflammatory myopathies: evaluation and management. Semin Neurol 2008; 28 (02) 241-249
- 65 Dalakas MC. Muscle biopsy findings in inflammatory myopathies. Rheum Dis Clin North Am 2002; 28 (04) 779-798 , vi
- 66 Kubínová K, Dejthevaporn R, Mann H, Machado PM, Vencovský J. The role of imaging in evaluating patients with idiopathic inflammatory myopathies. Clin Exp Rheumatol 2018; 36 (Suppl 114, 5): 74-81
- 67 Okogbaa J, Batiste L. Dermatomyositis: an acute flare and current treatments. Clin Med Insights Case Rep 2019; 12: 1179547619855370
- 68 Noguchi E, Uruha A, Suzuki S. et al. Skeletal muscle involvement in antisynthetase syndrome. JAMA Neurol 2017; 74 (08) 992-999
- 69 De Lorenzo R, Pinal-Fernandez I, Huang W. et al. Muscular and extramuscular clinical features of patients with anti-PM/Scl autoantibodies. Neurology 2018; 90 (23) e2068-e2076
- 70 Allenbach Y, Mammen AL, Benveniste O, Stenzel W. Immune-Mediated Necrotizing Myopathies Working Group. 224th ENMC International Workshop: clinico-sero-pathological classification of immune-mediated necrotizing myopathies Zandvoort, the Netherlands, 14-16 October 2016. Neuromuscul Disord 2018; 28 (01) 87-99
- 71 Pinal-Fernandez I, Parks C, Werner JL. et al. Longitudinal course of disease in a large cohort of myositis patients with autoantibodies recognizing the signal recognition particle. Arthritis Care Res (Hoboken) 2017; 69 (02) 263-270
- 72 Needham M, Fabian V, Knezevic W, Panegyres P, Zilko P, Mastaglia FL. Progressive myopathy with up-regulation of MHC-I associated with statin therapy. Neuromuscul Disord 2007; 17 (02) 194-200
- 73 Mammen AL. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol 2011; 7 (06) 343-354
- 74 Pinal-Fernandez I, Casal-Dominguez M, Mammen AL. Immune-mediated necrotizing myopathy. Curr Rheumatol Rep 2018; 20 (04) 21
- 75 Siao P, Kaku M. A clinician's approach to peripheral neuropathy. Semin Neurol 2019; 39 (05) 519-530
- 76 Vucic S, Kiernan MC, Cornblath DR. Guillain-Barré syndrome: an update. J Clin Neurosci 2009; 16 (06) 733-741
- 77 Hadden RDM, Cornblath DR, Hughes RAC. et al; Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Electrophysiological classification of Guillain-Barré syndrome: clinical associations and outcome. Ann Neurol 1998; 44 (05) 780-788
- 78 Ropper AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326 (17) 1130-1136
- 79 Hughes RAC, Rees JH. Clinical and epidemiologic features of Guillain-Barré syndrome. J Infect Dis 1997; 176 (Suppl. 02) S92-S98
- 80 Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol 1990; 27 (Suppl): S21-S24
- 81 Harms M. Inpatient management of Guillain-Barré syndrome. Neurohospitalist 2011; 1 (02) 78-84
- 82 Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255 (02) 57-65
- 83 Ito M, Kuwabara S, Odaka M. et al. Bickerstaff's brainstem encephalitis and Fisher syndrome form a continuous spectrum: clinical analysis of 581 cases. J Neurol 2008; 255 (05) 674-682
- 84 Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group. Neurology 1985; 35 (08) 1096-1104
- 85 Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2017; 2: CD001798
- 86 Hughes RAC, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev 2014; (09) CD002063
- 87 Korinthenberg R, Schessl J, Kirschner J, Mönting JS. Intravenously administered immunoglobulin in the treatment of childhood Guillain-Barré syndrome: a randomized trial. Pediatrics 2005; 116 (01) 8-14
- 88 Verboon C, van den Berg B, Cornblath DR. et al; IGOS Consortium. Original research: second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study. J Neurol Neurosurg Psychiatry 2020; 91 (02) 113-121
- 89 Verboon C, Doets AY, Galassi G. et al; IGOS Consortium. Current treatment practice of Guillain-Barré syndrome. Neurology 2019; 93 (01) e59-e76
- 90 Raphaël JC, Chevret S, Chastang C, Jars-Guincestre MC. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Appropriate number of plasma exchanges in Guillain-Barré syndrome. Ann Neurol 1997; 41 (03) 298-306
- 91 Mori M, Kuwabara S, Fukutake T, Hattori T. Intravenous immunoglobulin therapy for Miller Fisher syndrome. Neurology 2007; 68 (14) 1144-1146
- 92 Donofrio PD. Guillain-Barré syndrome. Continuum (Minneap Minn) 2017; 23 (5, Peripheral Nerve and Motor Neuron Disorders): 1295-1309