md-medicaldata

Authors

 

Snežana B. Knežević¹ , Bratislav R. Đorđević² , Ivan Z. Gajović<sup>3

1</sup>Dom zdravlja Kraljevo, Srbija
²Opšta bolnica ,,Studenica“ Kraljevo, odeljenje Neurologije, Srbija
³Specijalna hirurška bolnica ,,Sveti Nikola“ Ratina, Kraljevo, Srbija

 

UDK: 616.8-085
615.37

The paper was received / Rad primljen: 09.10.2018.

Accepted / Rad prihvaćen: 12.10.2018

 

Correspondence to:

Dr Snežana Knežević
Dom zdravlja, Kraljevo, Srbija
Ul. Jug Bogdanova 110
tel: +38136301723
e-mail: lesta59@yahoo.com

 

 

Sažetak

 

Uvod. Lewis-Sumner sindrom karakteriše asimetrična multifokalna zahvaćenost distalnog senzornog i motornog neurona, predominantno na gornjim ekstremitetima. Najpre vidimo zahvaćenost jednog nerva, koja progredira i simetrično se razvija. Obično je rame prvo zahvaćeno sa kasnijim širenjem duž ruke.
Prikaz bolesnika. Pacijentkinja, stara 53 godine, javila se na pregled zbog slabosti u potkolenicama, hipestezije i parestezija podlaktica i mišićnom slabošću. Na gornjim ekstremitetima motorna snaga snižena, miotatički refleksi ugašeni, muskulatura hipotrofična, postoje senzorni deficiti. Na donjim ekstremitetima motorna snaga snižena. Detaljnim anamnestičkim, kliničkim, laboratorijskim, ehosonografskim i radiološkim pretragama isključeni su paraneoplastični sindrom, paraproteinemične, imunološke, metaboličke, toksične, nasledne i infektivne polineuropatije. Kompjuterizovana tomografija i magnetna rezonanca glave i vrata nisu ukazali na postojanje demijelinizacionih lezija, tumora ili vaskularnih poremećaja. U likvoru je bila prisutna hipeproteinorahija (0,4 g/L). Elektromiografski pregled ukazao na usporenje brzine provođenja, produžene distalne latence, odsustvo F talasa i blokove provođenja. Biopsija suralisa je potvrdila multifokalnu demijelinizaciju. Idiopatska multifokalna stečena demijelinizaciona senzorna i motorna neuropatija je na terapiju kortikosteroidima bila bez odgovora pa su primenjeni intravenski imunoglobulini, što je dovelo do poboljšanja. Povoljan terapijski odgo­vor je u skladu sa činjenicom da imunoglobulini predstavljaju lek izbora za ovaj sindrom.

 

 

Ključne reči:

Hronična inflamatorna demijelinizaciona poliradikuloneuropatija, trnjenje, demijelinizacione bolesti, imunoglobulini

 

 

 

Abstract

 

Introduction. Lewis-Sumner syndrome is characterized by an asymmetric multifocal coverage of the distal sensory and motor neuron, predominantly on the upper extremities. Primarily we see the coverage of one nerve, which progresses and develops symmetrically. Usually the shoulder is affected at first, with later spreading along the arm.
Case report. The patient, 53 years of age, arrived at the clinic due to weakness of the shins, numbness and annealing, as well as numbness and muscle weakness in the forearms. Motor strength on the upper extremities reduced, myotatic reflexes are off, musculature hypotrophic. On the lower extremities, motor strength is reduced.
Detailed anamnestic, clinical, laboratory, echosonographic and radiological investigations exclude paraneoplastic syndrome, paraproteinemic, immunological, metabolic, toxic, hereditary and infectious polyneuropathies. Computerized tomography and magnetic resonance of the head and neck did not indicate the presence of demyelinating lesions, tumors or vascular disorders. Lumbar puncture shows increased levels of protein (0,4 g/L). Electromyographic examination shows lower speed, extended distal latence, absence of F waves and conduction blocks. Biopsy of the Suralis nerve shows a multifocal demyelination. Idiopathic multifocal acquired demyelinating sensory and motor neuropathy on corticosteroid therapy had no response, so intravenous immunoglobulins were enforced, which led to improvement.

 

 

Key words:

chronic inflammatory demyelinating polyradiculoneuropathy, paresthesia, demyelinating diseases, immunoglobulins.

 

 

 

References:

 

1. Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982;32:958-64.
2. Van den Berg-Vos RM, Van den Berg LH, Franssen H, Vermeulen M, Witkamp TD, Jansen GH, et al. Multifocal inflammatory demyelinating neuropathy: a distinct clinical entity? Neurology 2000;54(1):26-32.
3. Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol 2010;9:402–412.
4. Hughes R. Chronic Inflammatory Demyelinating Polyradiculoneuropathy. J Clin Immunol 2010;30:70–73.
5. Kedra J, Foltz V, Viala K, Tan S, Fautrel B. Lewis-Sumner syndrome in a patient with rheumatoid arthritis: Link between rheumatoid arthritis and demyelinating polyradiculoneuropathies. Joint Bone Spine 2017;84(4):485-487.
6. Laughlin RS, Dyck PJ, Melton LJ 3rd, Leibson C, Ransom J, Dyck PJ. Incidence and prevalence of CIDP and the association of diabetes mellitus. Neurology 2009;73(1):39–45.
7. McCombe PA, Pollard JD, McLeod JG. Chronic inflammatory demyelinating polyradiculoneuropathy. A clinical and electrophysiological study of 92 cases. Brain 1987;110:1617–30.
8. Holzbauer SM, DeVries AS, Sejvar JJ, et al. Epidemiologic investigation of immune-mediated polyradiculoneuropathy among abattoir workers exposed to porcine brain. PLoS One 2010;5(3):e9782.
9. Van den Bergh PY, Hadden RD, Bouche P, Cornblath DR, Hahn A, Illa I, et al. European Federation of Neurological Societies/Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - first revision. Eur J Neurol 2010;17(3):356-63.
10. Kostić V. Neurologija. Beograd:Medicinski fakultet Univerziteta, CIBID; 2016.
11. Damjan I, Cvijanović M, Erak M. Značaj elektromiografskog pregleda u dijagnostikovanju i praćenju hronične inflamatorne demijelinizacione polineuropatije Med Pregl 2010;63(7-8):559-564.
12. Park Y, Yook J , Kim D .Case of Lewis-Sumner Syndrome Showing Dramatic Improvement after Plasma Exchange. J Korean Med Sci 2010;25:1101-1104.
13. Oh SJ, Claussen GC, Kim DS. Motor and sensory demyelinating mononeuropathy multiplex (multifocal motor and sensory demyelinating neuropathy): a separate entity or a variant of chronic inflammatory demyelinating polyneuropathy? J Peripher Nerv Syst 1997;2(4):362-9.
14. Saperstein DS, Amato AA, Wolfe GI, Katz JS, Nations SP, Jackson CE, et al. Multifocal acquired demyelinating sensory and motor neuropathy: the Lewis-Sumner syndrome. Muscle Nerve 1999;22:560–566.
15. Viala K, Renié L, Maisonobe T, Béhin A, Neil J, Léger JM, et al. Follow-up study and response to treatment in 23 patients with Lewis-Sumner syndrome. Brain 2004;127:2010–2017.
16. Dimachkie MM, Barohn RJ, Katz J. Multifocal Motor Neuropathy, Multifocal Acquired Demyelinating Sensory and Motor Neuropathy, and Other Chronic Acquired Demyelinating Polyneuropathy Variants. Neurol Clin 2013;31:533–555.
    

PDF Knežević B.S. et al. • MD-Medical Data 2018;10(4): 201-204