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Lewis- Sumner Syndrome –  a Case Report


Authors: I. Okáčová 1;  B. Mičánkováihash2 1,2 1,2
Authors‘ workplace: Neurologická klinika LF MU a FN Brno 1;  CEITEC – Středoevropský technologický institut, MU, Brno 2;  Ústav patologie a patologické anatomie LF MU a FN Brno 3;  Radiologická klinika LF MU a FN Brno 4
Published in: Cesk Slov Neurol N 2012; 75/108(4): 498-502
Category: Case Report

Overview

Lewis-Sumner syndrome, also known as Multifocal Acquired Demyelinating Sensory And Motor neuropathy (MADSAM), is considered to be a focal asymmetric variant of the chronic inflammatory demyelinating polyneuropathy. The aim of this case report is to discuss this rare disease, its differential diagnosis and available treatment modalities.

Case report:
50 years old man experienced subacute onset of impaired sensation and weakness in his left hand. EMG examination revealed focal chronic sensory and motor lesion of median, ulnar and radial nerves of the left hand (primary demyelination with partial motor block), with signs of chronic axonopathy, located between axilla and the Erb’s point. MRI of the left brachial plexus showed spinal root enlargement. Sural nerve biopsy showed signs of demyelination. CSF was normal, except for a marked elevation of protein. Serum anti-GM1 antibody titer was below cut-off. Differential diagnosis included MMN (Multifocal Motor Neuropathy), hereditary neuropathy with liability to pressure palsy and neurofibroma of brachial plexus. The patient did not respond to corticosteroid treatment. Treatment with intravenous immunoglobulins led to clinical and electrophysiological improvement.

Key words:
Lewis-Sumner syndrome – multifocal motor neuropathy – chronic inflammatory demyelinating neuropathy


Sources

1. Lewis RA, Sumner AJ, Brown MJ, Asbury AK. Multifocal demyelinating neuropathy with persistent conduction block. Neurology 1982; 32(9): 958– 964.

2. 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(Pt 9): 2010– 2017.

3. 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– 369.

4. Gorson KC, Ropper AH, Weinberg DH. Upper limb predominant, multifocal chronic inflammatory demyelinating polyneuropathy. Muscle Nerve 1999; 22(6): 758– 765.

5. Saperstein DS, Amato 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(5): 560– 566.

6. Van der 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.

7. Jean AP, Duprez T, Van den Bergh PY. Massive peripheral nerve hypertrophy in patient with multifocal upper limb demyelinating neuropathy (Lewis- Sumner syndrome). Acta Neurol Belg 2001; 101(4): 234– 238.

8. Pouget J, Verschueren A, Azulay JP, Attarian S. Lewis Sumner syndrome. Rev Neurol 2001; 157(12): 1561– 1564.

9. Park YE, Yook JW, Kim DS. A case of Lewis- Sumner syndrome showing dramatic improvement after plasma exchange. J Korean Med Sci 2010; 25(7): 1101– 1104.

10. Thomas PK, Claus D, Jaspert A, Workman JM, King H et al. Focal upper limb demyelinating neuropathy. Brain 1996; 119(Pt 3): 765– 774.

11. Kuwabara S, Nakajima M, Matsuda S, Hattori T. Magnetic resonance imaging at the demyelinative foci chronic inflammatory demyelinating polyneuropathy. Neurology 1997; 48(4): 874– 877.

12. Bouchard C, Lacroix C, Planté V, Adams D, Chedru et al. Clinicopathological findings and prognosis of chronic inflammatory demyelinating polyneuropathy. Neurology 1999; 52(3): 498– 503.

13. Vallat JM, Tabaraud F, Magy L, Torny F, Bernet Bernardy P et al. Diagnostic value of nerve bio­psy for atypical chronic inflammatory demyelinating polyneuropathy, evaluation of eight cases. Muscle Nerve 2003; 27(4): 478– 485.

14. Rajabally YA, Chavada G. Lewis- Sumner syndrome of pure upper limb onset: diagnostic, prognostic, and therapeutic features. Muscle Nerve 2009; 39(2): 206– 220.

15. Maisonobe T, Chassande B, Verin M, Jouni M, Leger JM et al. Chronic dysimmune demyelinating polyneuropathy: a clinical and electrophysiological study of 93 patients. J Neurol Neurosurg Psychiatry 1996; 61(1): 36– 42.

16. Kuwabara S, Ogawara K, Misawa S, Mori M, Hattori T. Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy. J Neurol Neurosurg Psychiatry 2002; 72(1): 37– 42.

17. Nobile– Orazio E. Multifocal motor neuropathy. J Neuroimmunol 2001; 115(1– 2): 4– 18.

18. Verschueren A, Azulay JP, Attarian S, Boucraut J, Pellissier JF, Pouget J. Lewis– Sumner syndrome and multifocal motor neuropathy. Muscle Nerve 2005; 31(1): 88– 94.

19. Parry GJ, Clarke S. Multifocal acquired demyelinating neuropathy masquerading as motor neuron disease. Muscle Nerve 1988; 11(2): 103– 107.

20. van Es HW. MRI of brachial plexus. Eur Radiol 2001; 11(2): 325– 336.

21. Mazanec R, Horáček O, Kobesová A, Smetana P. Hereditární neuropatie. Cesk Slov Neurol N 2009; 72/ 105(1): 5– 17.

22. Ambler Z. Lymfomatózní neuropatie (neurolymfomatóza) –  kazuistika. Cesk Slov Neurol N 2010; 73/ 106(1): 725– 728.

Labels
Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 4

2012 Issue 4

Most read in this issue
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