Approach to Limb Weakness

Abstract

“Approach to limb weakness” provides an overview of the pathways of the motor system and the type of weakness seen with pathology at each level from the cortex to the muscle. This article provides the clinical pearls needed to identify different patterns of weakness and accurately localize the level of weakness. It offers important pointers that help distinguish among the different etiologies of weakness at each level, as well as various diagnostic approaches and treatments of diseases that lead to limb weakness. The diagnoses discussed are meant to be representative and not exhaustive, as a complete differential for each pattern of weakness is beyond the scope of this article.

Keywords

localization - patterns of weakness - upper motor - lower motor - limb weakness

Publikationsverlauf

Artikel online veröffentlicht:
26. November 2021

• References

• 1 Blumenfeld H. ed. Corticospinal tract and other motor pathways. In: Neuroanatomy through Clinical Cases. 2nd ed.. Sunderland, MA: Sinauer Associates; 2010
• 2 Nogueira RG, Jadhav AP, Haussen DC. et al; DAWN Trial Investigators. Thrombectomy 6 to 24 hours after stroke with a mismatch between deficit and infarct. N Engl J Med 2018; 378 (01) 11-21
• 3 Yang ML, Connolly AM. Other Motor Neuron Diseases of Childhood. Accessed November 20, 2020 at: https://doi.org/10.1016/B978-0-323-37101-8.00140-5
• 4 Boillée S, Vande Velde C, Cleveland DW. ALS: a disease of motor neurons and their nonneuronal neighbors. Neuron 2006; 52 (01) 39-59
• 5 National Institute of Neurological Disorders and Stroke (NINDS). November 2010. Accessed March 24, 2015 at: http://www.ninds.nih.gov
• 6 De Vos KJ, Chapman AL, Tennant ME. et al. Familial amyotrophic lateral sclerosis-linked SOD1 mutants perturb fast axonal transport to reduce axonal mitochondria content. Hum Mol Genet 2007; 16 (22) 2720-2728
• 7 Forsberg K, Andersen PM, Marklund SL, Brännström T. Glial nuclear aggregates of superoxide dismutase-1 are regularly present in patients with amyotrophic lateral sclerosis. Acta Neuropathol 2011; 121 (05) 623-634
• 8 Ravits JM, La Spada AR. ALS motor phenotype heterogeneity, focality, and spread: deconstructing motor neuron degeneration. Neurology 2009; 73 (10) 805-811
• 9 Goutman SA. Diagnosis and clinical management of amyotrophic lateral sclerosis and other motor neuron disorders. Continuum (Minneap Minn) 2017; 23 (5, Peripheral Nerve and Motor Neuron Disorders): 1332-1359
• 10 Swinnen B, Robberecht W. The phenotypic variability of amyotrophic lateral sclerosis. Nat Rev Neurol 2014; 10 (11) 661-670
• 11 Al-Chalabi A, Hardiman O. The epidemiology of ALS: a conspiracy of genes, environment and time. Nat Rev Neurol 2013; 9 (11) 617-628
• 12 Oskarsson B, Gendron TF, Staff NP. Amyotrophic lateral sclerosis: an update for 2018. Mayo Clin Proc 2018; 93 (11) 1617-1628
• 13 https://www.cdc.gov/westnile/healthcareproviders/healthCareProviders-ClinLabEval.html . Accessed November 20, 2020
• 14 Lindsey NP, Staples JE, Lehman JA, Fischer M. Centers for Disease Control and Prevention (CDC). Surveillance for human West Nile virus disease - United States, 1999-2008. MMWR Surveill Summ 2010; 59 (02) 1-17
• 15 Murray K, Baraniuk S, Resnick M. et al. Risk factors for encephalitis and death from West Nile virus infection. Epidemiol Infect 2006; 134 (06) 1325-1332
• 16 Leis AA, Stokic DS. Neuromuscular manifestations of West Nile virus infection. Front Neurol 2012; 3: 37
• 17 Madden K. West Nile virus infection and its neurological manifestations. Clin Med Res 2003; 1 (02) 145-150
• 18 Prior TW, Finanger E. Spinal muscular atrophy. GeneReviews. Accessed March 03, 2017 at: https://www.ncbi.nlm.nih.gov/books/NBK1352/
• 19 Dähnert W. Radiology Review Manual. Philadelphia, PA: Lippincott Williams; 2007
• 20 Buchthal F, Olsen PZ. Electromyography and muscle biopsy in infantile spinal muscular atrophy. Brain 1970; 93 (01) 15-30
• 21 Chiriboga CA, Swoboda KJ, Darras BT. et al. Results from a phase 1 study of nusinersen (ISIS-SMN(Rx)) in children with spinal muscular atrophy. Neurology 2016; 86 (10) 890-897
• 22 Saad AF, Nickell LT, Finn SS, Opatowsky MJ. Spinal cord ependymoma presenting with neurological deficits in the setting of trauma. Proc Bayl Univ Med Cent 2014; 27 (03) 213-214
• 23 Kalayci M, Cağavi F, Gül S, Yenidünya S, Açikgöz B. Intramedullary spinal cord metastases: diagnosis and treatment - an illustrated review. Acta Neurochir (Wien) 2004; 146 (12) 1347-1354 , discussion 1354
• 24 Yang KH, Lee HR, Yi SY, Jung JH, Kang SH, Choi PH. Intramedullary spinal cord metastasis from rectal cancer. Ann Coloproctol 2014; 30 (05) 237-240
• 25 Rao RD, Gourab K, David KS. Operative treatment of cervical spondylotic myelopathy. J Bone Joint Surg Am 2006; 88 (07) 1619-1640
• 26 Schwendimann RN. Metabolic and toxic myelopathies. Continuum (Minneap Minn) 2018; 24 (2, Spinal Cord Disorders): 427-440
• 27 Dietary Supplement Fact Sheet: Vitamin B12 — Health Professional Fact Sheet. National Institutes of Health: Office of Dietary Supplements. 2016-02-11. Archived from the original on 2016-07-27. Retrieved July 15, 2016
• 28 Schwendimann RN. Metabolic and toxic myelopathies. Continuum 2018; 24 (02) 427-440
• 29 Noseworthy JH, Lucchinetti C, Rodriguez M, Weinshenker BG. Multiple sclerosis. (review) N Engl J Med 2000; 343 (13) 938-952
• 30 Lassmann H, Brück W, Lucchinetti CF. The immunopathology of multiple sclerosis: an overview. (review) Brain Pathol 2007; 17 (02) 210-218
• 31 Flanagan EP. Neuromyelitis optica spectrum disorder and other non-multiple sclerosis central nervous system inflammatory diseases. Continuum (Minneap Minn) 2019; 25 (03) 815-844
• 32 Marignier R. Breaking boundaries between demyelinating disorders and autoimmune encephalitis. Lancet Neurol 2020; 19 (03) 199-200
• 33 Armangue T, Olivé-Cirera G, Martínez-Hernandez E. et al; Spanish Pediatric anti-MOG Study Group. Associations of paediatric demyelinating and encephalitic syndromes with myelin oligodendrocyte glycoprotein antibodies: a multicentre observational study. Lancet Neurol 2020; 19 (03) 234-246
• 34 Halperin JJ, Shapiro ED, Logigian E. et al. Practice parameter: treatment of nervous system Lyme disease (an evidence-based review): report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2007; 69 (01) 91102
• 35 AAN Summary of Evidence based Guidelines for CLINICIANS. Treatment of Nervous System Lyme Disease. Accessed November 20, 2020 at: https://www.aan.com/Guidelines/home/GetGuidelineContent/241
• 36 Romi F, Naess H. Spinal cord infarction in clinical neurology: a review of characteristics and long-term prognosis in comparison to cerebral infarction. Eur Neurol 2016; 76 (3-4): 95-98
• 37 Zalewski NL, Rabinstein AA, Krecke KN. et al. Characteristics of spontaneous spinal cord infarction and proposed diagnostic criteria. JAMA Neurol 2019; 76 (01) 56-63
• 38 Ferrante MA. Brachial plexopathies: classification, causes, and consequences. Muscle Nerve 2004; 30 (05) 547-568
• 39 Wilbourn AJ. Brachial plexus lesions. In: Dyck PJ, Thomas PK. eds. Peripheral Neuropathy, 4th ed. Philadelphia, PA: Elsevier Saunders; 2005: 1339-1373
• 40 Narakas AO. The treatment of brachial plexus injuries. Int Orthop 1985; 9 (01) 29-36
• 41 Chuang DC. Adult brachial plexus reconstruction with the level of injury: review and personal experience. Plast Reconstr Surg 2009; 124 (6, Suppl): e359-e369
• 42 Songcharoen P, Shin AY. Brachial plexus injury: acute diagnosis and treatment. In: Berger RA, Weiss AP. eds. Hand Surgery. Philadelphia, PA: Lippincott Williams & Wilkins; 2003: 1005-1025
• 43 Ferrante MA. What We Measure and What It Means. Rochester: American Association of Neuromuscular and Electrodiagnostic Medicine; 2013
• 44 Ferrante MA, Wilbourn AJ. The utility of various sensory nerve conduction responses in assessing brachial plexopathies. Muscle Nerve 1995; 18 (08) 879-889
• 45 Dyck PJ, Thaisetthawatkul P. Lumbosacral plexopathy. Continuum (Minneap Minn) 2014; 20 (5 Peripheral Nervous System Disorders): 1343-1358
• 46 Planner AC, Donaghy M, Moore NR. Causes of lumbosacral plexopathy. Clin Radiol 2006; 61 (12) 987-995
• 47 Stewart JD. Focal Peripheral Neuropathies. Philadelphia, PA: Lippincott Williams & Wilkins; 2000
• 48 Maravilla KR, Bowen BC. Imaging of the peripheral nervous system: evaluation of peripheral neuropathy and plexopathy. AJNR Am J Neuroradiol 1998; 19 (06) 1011-1023
• 49 Dyck PJ, Norell JE, Dyck PJ. Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology 1999; 53 (09) 2113-2121
• 50 Dyck PJ, Windebank AJ. Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment. Muscle Nerve 2002; 25 (04) 477-491
• 51 Krendel DA, Costigan DA, Hopkins LC. Successful treatment of neuropathies in patients with diabetes mellitus. Arch Neurol 1995; 52 (11) 1053-1061
• 52 Thijs RD, Notermans NC, Wokke JH, van der Graaf Y, van Gijn J. Distribution of muscle weakness of central and peripheral origin. J Neurol Neurosurg Psychiatry 1998; 65 (05) 794-796
• 53 Barohn RJ, Amato AA. Pattern-recognition approach to neuropathy and neuronopathy. Neurol Clin 2013; 31 (02) 343-361
• 54 Willison HJ, Winer JB. Clinical evaluation and investigation of neuropathy. J Neurol Neurosurg Psychiatry 2003; 74 (Suppl. 02) ii3-ii8
• 55 Watson JC, Dyck PJ. Peripheral neuropathy: a practical approach to diagnosis and symptom management. Mayo Clin Proc 2015; 90 (07) 940-951
• 56 Finsterer J. Treatment of immune-mediated, dysimmune neuropathies. Acta Neurol Scand 2005; 112 (02) 115-125
• 57 Myotomes, Spinal Nerve Roots, and Dermatomes. https://www.verywellhealth.com/what-is-a-myotome-296992 . Accessed November 20, 2020
• 58 Pasnoor M, Dimachkie MM. Approach to muscle and neuromuscular junction disorders. Continuum (Minneap Minn) 2019; 25 (06) 1536-1563
• 59 Jowkar A. Which maneuvers should be included in the physical exam of suspected myasthenia gravis (MG)?. Accessed November 20, 2020 at: https://www.medscape.com/answers/1171206-92637/which-maneuvers-should-be-included-in-the-physical-exam-of-suspected-myasthenia-gravis-mg
• 60 Ciafaloni E. Myasthenia gravis and congenital myasthenic syndromes. Continuum (Minneap Minn) 2019; 25 (06) 1767-1784
• 61 Guidon AC. Lambert-Eaton myasthenic syndrome, botulism, and immune checkpoint inhibitor-related myasthenia gravis. Continuum (Minneap Minn) 2019; 25 (06) 1785-1806
• 62 Bleck TP. Clostridium botulinum (botulism). In: Mandel GL, Bennett JE, Dolin R. eds. Principles and Practice of Infectious Diseases, 6th ed. Philadelphia, PA: Churchill Livingstone; 2005: 2822
• 63 Dowell Jr VR. Botulism and tetanus: selected epidemiologic and microbiologic aspects. Rev Infect Dis 1984; 6 (Suppl. 01) S202-S207
• 64 Pasnoor M, Dimachkie MM. Approach to muscle and neuromuscular junction disorders. Continuum (Minneap Minn) 2019; 25 (06) 1536-1563
• 65 Goyal NA. Immune-mediated myopathies. Continuum (Minneap Minn) 2019; 25 (06) 1564-1585
• 66 Weihl CC. Sporadic inclusion body myositis and other rimmed vacuolar myopathies. Continuum (Minneap Minn) 2019; 25 (06) 1586-1598
• 67 Doughty CT, Amato AA. Toxic myopathies. Continuum (Minneap Minn) 2019; 25 (06) 1712-1731
• 68 American College of Cardiology American College of Cardiology. Statin intolerance. Updated December 2016. Accessed November 20, 2020 at: tools.acc.org/statinintolerance/#!/
• 69 Thangarajh M. The dystrophinopathies. Continuum (Minneap Minn) 2019; 25 (06) 1619-1639
• 70 Miceli MC, Nelson SF. The case for eteplirsen: paving the way for precision medicine. Mol Genet Metab 2016; 118 (02) 70-71
• 71 Guiraud S, Davies KE. Pharmacological advances for treatment in Duchenne muscular dystrophy. Curr Opin Pharmacol 2017; 34: 36-48