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Transverse Myelitis and Neuralgic Amyotrophy

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The Pediatric Upper Extremity

Abstract

Neuralgic amyotrophy (NA), a lower motor neuron (LMN) lesion, presents a flaccid monoplegia of the upper extremity in children. Transverse myelitis (TM) may present with either an LMN or an upper motor neuron (UMN) injury, depending on areas affected on the cord. Both entities are inflammatory and autoimmune in nature. Diagnosis is with MRI and serological studies as well as nerve conduction studies (NCS) and electromyography (EMG). Treatment varies slightly, but includes immunosuppression with steroids, replacement of antibodies with plasmapheresis in addition to intravenous immunoglobulin (IVIG), immunosuppressant, and antineoplastic agents. Further management includes rehabilitation measures with stretching, strengthening, range of motion, neuromuscular electrical stimulation, patient and family education, and equipment evaluation. Children with persistent LMN deficits and limited recovery (6–8 months after onset of disease) may warrant surgical considerations for peripheral nerve surgery. This may include the use of nerve transfers. Secondary surgery, including muscle and tendon transfers, can be considered 1–2 years after disease onset, if persistent residual deficits are present. Most children with NA have good outcomes with resolution of symptoms and improvement in function. Children with TM have a less favorable outcome with one third resolution rate.

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References

  • Alper G, Petropoulou KA, Fitz CR, Kin Y. Idiopathic acute transverse myelitis in children: an analysis and discussion of MRI findings. Mult Scler. 2011;17(1):74–80.

    Article  PubMed Central  PubMed  Google Scholar 

  • Bae DS, Ferretti M, Waters PM. Upper extremity size differences in Brachial Plexus Birth Palsy. Hand. 2008;3:297–303.

    Article  PubMed Central  PubMed  Google Scholar 

  • Bertelli JA, Kechele PR, Santos MA, Duarte H, Ghizoni MF. Axillary nerve repair by triceps motor branch transfer through an axillary access: anatomical basis and clinical results. J Neurosurg. 2007;107:370–7.

    Article  PubMed  Google Scholar 

  • Borchers AT, Gershwin ME. Transverse myelitis. Autoimmun Rev. 2012;11(3):231–48.

    Article  CAS  PubMed  Google Scholar 

  • Curtis C, Stephens D, Clarke HM, Andrews D. The active movement scale: an evaluative tool for infants with obstetrical brachial plexus palsy. J Hand Surg Am. 2002;27:470–8.

    Article  PubMed  Google Scholar 

  • Custer JW, Rau RE. The Harriet Lane handbook: formulary. 18th ed. Philadelphia: Elsevier Mosby; 2009.

    Google Scholar 

  • Davids JR, Peace LC, Wagner LV, et al. Validation of the Shriners Hospital For Children Upper Extremity Evaluation (SHUEE) for children with hemiplegic cerebral palsy. J Bone Joint Surg Am. 2006;88:326–33.

    Article  PubMed  Google Scholar 

  • Dorsi MJ, Belzberg AJ. Nerve transfers for restoration of upper extremity motor function in a child with upper extremity motor deficits due to transverse myelitis: case report. Microsurgery. 2012;32(1):64–7.

    Article  PubMed  Google Scholar 

  • Host C, Skov L. Idiopathic neuralgic amyotrophy in children. Case report, 4 year follow up and review of the literature. Eur J Paediatr Neurol. 2010;14:467–73.

    Article  PubMed  Google Scholar 

  • Huh SY, Gordon CM. Fractures in hospitalized children. Metabolism. 2013;62(3):315–25.

    Article  CAS  PubMed  Google Scholar 

  • Jeannet PY, Watts GD, Bird TD, Chance PF. Craniofacial and cutaneous findings expand the phenotype of hereditary neuralgic amyotrophy. Neurology. 2001;57:1963–8.

    Article  CAS  PubMed  Google Scholar 

  • Kaplan AA, Fridey JL. Complications of therapeutic plasma exchange. (place unknown), UpToDate; (date unknown) (updated 2012 December 4; cited 2013 September 10). http://uptodate.com

  • Kliegman RM, Stanton BF, St. Geme JW, Schor NF, Behrman RE. Nelson textbook of pediatrics. 19th ed. Philadelphia: Elsevier; 2011.

    Google Scholar 

  • MacDonald BK, Cockerell OC, Sander JWAS, Shorvon SD. The incidence and lifetime prevalence of neurological disorders in a prospective community-based study in the UK. Brain. 2000;123:665–76.

    Article  PubMed  Google Scholar 

  • Malanga GA, Nadler SF. Musculoskeletal physical examination: an evidence-based approach. Philadelphia: Elsevier; 2006.

    Google Scholar 

  • Moriguchi K, Miyamoto K, Takada K, Kusunoki S. Four cases of anti-ganglioside antibody-positive neuralgic amyotrophy with good response to intravenous immunoglobulin infusion therapy. J Neuroimmunol. 2011;238:107–9.

    Article  CAS  PubMed  Google Scholar 

  • Msall ME, DiGaudio K, Rogers BT, et al. The functional independence measure for children (WeeFIM). Conceptual basis and pilot use in children with developmental disabilities. Clin Pediatr (Phila). 1994;33:421–30.

    Article  CAS  Google Scholar 

  • Mulcahey MJ, Kozin S, Merenda L, et al. Evaluation of the box and blocks test, stereognosis and item banks of activity and upper extremity function in youths with brachial plexus birth palsy. J Pediatr Orthop. 2012;32:114–22.

    Article  Google Scholar 

  • Naito K, Fukushima K, Suzuki S, et al. Intravenous immunoglobulin (IVIg) with methylprednisolone pulse therapy for motor impairment of neuralgic amyotrophy: clinical observations in 10 cases. Intern Med. 2012;51:1493–500.

    Article  CAS  PubMed  Google Scholar 

  • Pidcock FS, Krishnan C, Crawford TO, Salorio CF, Trovato M, Kerr DA. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology. 2007;68:1474–80.

    Article  CAS  PubMed  Google Scholar 

  • Pindrik J, Belzberg AJ. Peripheral nerve surgery: primer for the imagers. Neuroimaging Clin N Am. 2014;24(1):193–210.

    Article  PubMed  Google Scholar 

  • Pindrik J, Chhabra A, Belzberg AJ. Update on peripheral nerve surgery. Neurosurgery. 2013;60 Suppl 1:70–7.

    Article  PubMed  Google Scholar 

  • Preston DC, Shapiro BE. Electromyography and neuromuscular disorders: clinical-electrophysiologic correlations. 2nd ed. Philadelphia: Elsevier; 2005.

    Google Scholar 

  • Ramos LE, Zell JP. Rehabilitation program for children with brachial plexus and peripheral nerve injury. Semin Pediatr Neurol. 2000;7:52–7.

    Article  CAS  PubMed  Google Scholar 

  • Sadowsky CL, Becker D, Bosques G, Dean JM, McDonald JW, Recio A, et al. Rehabilitation in transverse myelitis. Continuum. 2011;17(4):816–30.

    PubMed  Google Scholar 

  • Scalf RE, Wenger DE, Frick MA, Mandrekar JN, Adkins MC. MRI findings of 26 patients with Parsonage-Turner syndrome. AJR Am J Roentgenol. 2007;189:W39–44.

    Article  PubMed  Google Scholar 

  • Scott TF, Frohman EM, De Seze J, Gronseth GS, Weinshenker BG. Evidence-based guideline: clinical evaluation and treatment of transverse myelitis. Neurology. 2011;77:2128–34.

    Article  CAS  PubMed  Google Scholar 

  • Sellner J, Lüthi N, Schüpbach WMM, Gebhardt A, Findling O, Schroth G, et al. Diagnostic workup of patients with acute transverse myelitis: spectrum of clinical presentation, neuroimaging and laboratory findings. Spinal Cord. 2009;47:312–7.

    Article  CAS  PubMed  Google Scholar 

  • Spirtos M, O’Mahony P, Malone J. Interrater reliability of the Melbourne assessment of unilateral upper limb function for children with hemiplegic cerebral palsy. Am J Occup Ther. 2011;65(4):378–83.

    Article  PubMed  Google Scholar 

  • Suarez GA, Giannini C, Bosch EP, et al. Immune brachial plexus neuropathy: suggestive evidence for an inflammatory-immune pathogenesis. Neurology. 1996;46:559–61.

    Article  CAS  PubMed  Google Scholar 

  • Sung IY, Ryu JS, Pyun SB, Yoo SD, Song WH, Park MJ. Efficacy of forced-use therapy in hemiplegic cerebral palsy. Arch Phys Med Rehabil. 2005;86(11):2195–8.

    Article  PubMed  Google Scholar 

  • Teboul F, Kakkar R, Ameur N, Beaulieu JY, Oberlin C. Transfer of fascicles from the ulnar nerve to the nerve to the biceps in the treatment of upper brachial plexus palsy. J Bone Joint Surg Am. 2004;86A:1485–90.

    Google Scholar 

  • Thorley M, Lannin N, Cusick A, Novak I, Boyd R. Reliability of the quality of upper extremity skills test for children with cerebral palsy aged 2 to 12 years. Phys Occup Ther Pediatr. 2012;32(1):4–21.

    Article  PubMed  Google Scholar 

  • Transverse Myelitis Consortium Working Group. Proposed diagnostic criteria and nosology of acute transverse myelitis. Neurology. 2002;59(4):499–505.

    Article  Google Scholar 

  • Tsairis P, Dyck PJ, Mulder DW. Natural history of brachial plexus neuropathy. Report on 99 patients. Arch Neurol. 1972;27:109–17.

    Article  CAS  PubMed  Google Scholar 

  • van Alfen N. The neuralgic amyotrophy consultation. J Neurol. 2007;254:695–704.

    Article  PubMed Central  PubMed  Google Scholar 

  • van Alfen N. Clinical and pathophysiological concepts of neuralgic amyotrophy. Nat Rev Neurol. 2011;7:315–22.

    Article  PubMed  Google Scholar 

  • van Alfen N, van Engelen BGM. The clinical spectrum of neuralgic amyotrophy in 246 cases. Brain. 2006;129:438–50.

    Article  PubMed  Google Scholar 

  • van Alfen N, Schuuring J, van Engelen BGM, Rotteveel JJ, Gabreels FJM. Idiopathic neuralgic amyotrophy in children. A distinct phenotype compared to the adult form. Neuropediatrics. 2000a;31:328–32.

    Article  PubMed  Google Scholar 

  • van Alfen N, van Engelen BGM, Reinders JWC, Kremer H, Gabreels FJM. The natural history of hereditary neuralgic amyotrophy in the Dutch population. Two distinct types? Brain. 2000b;123:718–23.

    Article  PubMed  Google Scholar 

  • van Eijk JJ, van Alfen N, Berrevoets M, et al. Evaluation of prednisolone treatment in the acute phase of neuralgic amyotrophy: an observational study. J Neurol Neurosurg Psychiatry. 2009;80:1120–4.

    Article  PubMed  Google Scholar 

  • Vassallo G, Martland T, Forbes W, Mccullagh G. A valuable non-invasive diagnostic investigation for paediatric idiopathic brachial neuritis. Dev Med Child Neurol. 2010;52:966–8.

    Article  PubMed  Google Scholar 

  • Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse Myelitis overview and differential diagnosis. J Child Neurol. 2012;27(11):1426–36.

    Article  PubMed  Google Scholar 

Further Reading

  • Consortium for Spinal Cord Medicine. Acute management of autonomic dysreflexia: individuals with spinal cord injury presenting to health-care facilities. 2nd ed. Eastern Paralyzed Veterans Association, Washington; 2001.

    Google Scholar 

  • Leis AA, Trapani VC. Atlas of electromyography. New York: Oxford University Press; 2000.

    Google Scholar 

  • Liao H, Chuang DCC, Ulusal AE, Schrag C. Surgical strategies for brachial plexus polio-like paralysis. Plast Reconstr Surg. 2008;121(4):1518.

    Google Scholar 

  • van Alfen N, van Engelen BG, Hughes RA. Treatment for idiopathic and hereditary neuralgic amyotrophy (brachial neuritis). Cochrane Database Syst Rev. 2009;3, CD006976.

    PubMed  Google Scholar 

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Belzberg, A., Bosques, G., Pham, K. (2015). Transverse Myelitis and Neuralgic Amyotrophy . In: Abzug, J., Kozin, S., Zlotolow, D. (eds) The Pediatric Upper Extremity. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-8515-5_32

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  • DOI: https://doi.org/10.1007/978-1-4614-8515-5_32

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  • Print ISBN: 978-1-4614-8513-1

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