Lewis Sumner Syndrome

[Rich5521]

Hi:

I seem to have a fairly aggressive form of CIDP, but they tell me that it is the Lewis Sumner variant.  My last conduction tests showed 0 sensory results in my left hand, and about 50% in the right, motor was significantly down in the left as well and my left foot has foot-drop along with a general weakness in both legs and absence of reflex.

I have been all over the web trying to find ANYTHING on Lewis Sumner Syndrome.  Can you give me some information about this disorder and what the prognosis is?  My family is driving me crazy for information and I can't find much.

Thanks

[Rich5521]

THANKS!

That first one was textbook as far as my most recent nerve conduction tests, 50% drop in conduction, focalized motor and sensory blockage and slow F waves.  So that one really helps clear up some things I couldn't find elsewhere.

I appreciate everyone's help, you've provided me with lots of print material for the family (although it's still hard for them to grasp why this means I'm losing my ability to walk - it's tough for someone not going through a neuro problem to relate to it.)

Anyway - thanks again :)

[CCF Neuro[P]-M.D.-RPS]

Dear Rich:

Sorry for the slight delay in getting back to you.  I have searched everywhere looking for Lewis Sumner variant of CIDP.  I never had heard of the use of this term for CIDP.  I couldn't find a thing.  A.J. Sumner wrote a nice chapter on CIDP in 1984 in a text on peripheral nerve disorders, but that is all I can dig up.  CIDP is a peripheral neuropathy much akin to Guillen-Barre syndrome.  There can be both sensory and motor nerve involvement, but usually motor is more pronounced.  This can be a primary diagnosis or we have seen CIDP in a setting of a mitochondrial cytopathy.  The usual treament is either steriods (which works well with most patients), or IVIg infusions (we have used this with good success).  Some people have used plasmaphoresis as a treatment.  We have one patient who did well on methotrexate.  Some patients with severe disease can be treated with combination treatment, steriods and cytoxan.  Usually with treatment, the muscle weakness and/or sensory problems will resolve in most cases. The time and frequency of treatment varies between patients.  Some of our patients have been on treatment for many, many years.  I hope this helps.

Sincerely,

CCF Neuro MD

[Rich5521]

Yes, I gathered that some people were using MMN in that other site I was referred to.  It IS multifocal and asymmetrical and often doesn't show up through the normal testing methods used for CIDP so people get diagnosed with "polyneuropathy cause unknown".   That was my diagnosis for a year and a half, so I wasn't receiving any treatment because the doctors didn't know what I had.  It's only recently that my nerve conduction tests confirmed Lewis-Sumner.

Thanks for the help, I'll check out dogpile :)

[nurse12hr]

Here is what I found:


1: Wien Klin Wochenschr 1995;107(10):301-8
[Multifocal-motor neuropathy and motor neuropathy with multifocal
                       conduction block (Lewis-Sumner syndrome)].
                       [Article in German]
                       Finsterer J, Mamoli B
                       Neurologische Abteilung, Neurologisches Krankenhaus Rosenhugel, Wien.
Multifocal motor neuropathy, which mimics lower motor neuron disease, is a rare and curious
                       demyelinating neuropathy characterised by slowly progressive, asymmetric limb weakness within
                       the distribution of individual peripheral nerves, wasting, cramps, fasciculations and rare sensory
                       involvement, but without upper motor neuron signs. The cardinal feature and primary
                       pathophysiological basis for the weakness is the multifocal motor conduction block which remains
                       stable for years at the same site and is confined to motor axons. It is defined as > 50% reduction
                       in both the CMAP and the negative peak area on proximal stimulation, as compared with the distal
                       stimulus response without any change in the negative peak duration. Nerves at the site of the
                       conduction block show demyelination, endoneural edema, rudimentary onion bulbs and
                       lymphocytic inflammation. Sensory nerves may show mild demyelination, axon loss and
                       lymphocytic inflammation. The majority of patients shows elevated titers of anti-glycolipid
                       antibodies, which may block the Na+ channels, produce demyelination or interfere with
                       remyelination. However, their role in the pathogenesis of multifocal motor neuropathy remains
                       uncertain. Multifocal motor neuropathy is regarded as the predominantly motor variant of chronic
                       inflammatory demyelinating polyneuropathy and can be treated best with immunoglobulins and
                       cyclophosphamide.

Rinsho Shinkeigaku 1999 Jan;39(1):107-9
                                                                            Related Articles, Books


                       [Diagnosis and treatment of multifocal motor neuropathy (Lewis-Sumner)].

                       [Article in Japanese]

                       Kaji R

                       Department of Neurology, Kyoto University Hospital.

                       [Record supplied by publisher]

                       We made a retrospective long-term follow-up study of 25 patients with multifocal motor
                       neuropathy (Lewis-Sumner). The diagnosis was based upon criteria modified from those of
                       AAEM (Sumner 1997). The electrophysiological findings indicating conduction block or focal
                       demyelinative lesions were more diagnostic than anti-GM 1 antibody titers, which were elevated in
                       only 40% of these patients. Demonstration of definite conduction block was not always possible in
                       those patients who responded favorably to intravenous immunoglobulins (IVIg), whereas indirect
                       pieces of evidence such as F-wave abnormalities or focal conduction delay or dispersion were
                       equally helpful. IVIg had superior outcome to cyclophosphamide, which sometimes caused serious
                       adverse effects. Three patients with severe axonal involvement showed elevated monospecific
                       antibodies to GalNAc-GD1a.

                       PMID: 10377829

: Muscle Nerve 2000 Oct;23(10):1472-87
                                                                      Related Articles, Books, LinkOut


                       Electrophysiological features of inherited demyelinating neuropathies: A
                       reappraisal in the era of molecular diagnosis.

                       Lewis RA, Sumner AJ, Shy ME

                       Department of Neurology, Wayne State University School of Medicine, UHC 8D, 4201 St.
                       Antoine, Detroit, Michigan, USA.

                       [Medline record in process]

                       The observation that inherited demyelinating neuropathies have uniform conduction slowing and
                       that acquired disorders have nonuniform or multifocal slowing was made prior to the identification
                       of mutations in myelin-specific genes which cause many of the inherited disorders involving
                       peripheral nerve myelin. It is now clear that the electrophysiological aspects of these disorders are
                       more complex than previously realized. Specifically, certain mutations appear to induce
                       nonuniform slowing of conduction which resemble the findings in acquired demyelinating
                       neuropathies. It is clinically important to recognize the different electrodiagnostic patterns of the
                       various inherited demyelinating neuropathies. In addition, an understanding of the relationship
                       between mutations of specific genes and their associated neurophysiological findings is likely to
                       facilitate understanding of the role of these myelin proteins in peripheral nerve function and of how
                       abnormalities in myelin proteins lead to neuropathy. We therefore review the current information
                       on the electrophysiological features of the inherited demyelinating neuropathies in hopes of
                       clarifying their electrodiagnostic features and to shed light on the physiological consequences of
                       the different genetic mutations. Copyright 2000 John Wiley & Sons, Inc.

                       PMID: 11003782


Muscle Nerve 1999 May;22(5):560-6
                                                                      Related Articles, Books, LinkOut


                       Multifocal acquired demyelinating sensory and motor neuropathy: the
                       Lewis-Sumner syndrome.

                       Saperstein DS, Amato AA, Wolfe GI, Katz JS, Nations SP, Jackson CE, Bryan WW, Burns
                       DK, Barohn RJ

                       Department of Neurology, University of Texas Southwestern Medical Center, Dallas, USA.

                       [Record supplied by publisher]

                       We report 11 patients with multifocal acquired demyelinating sensory and motor (MADSAM)
                       neuropathy, defined clinically by a multifocal pattern of motor and sensory loss, with nerve
                       conduction studies showing conduction block and other features of demyelination. The clinical,
                       laboratory, and histological features of these patients were contrasted with those of 16 patients
                       with multifocal motor neuropathy (MMN). Eighty-two percent of MADSAM neuropathy patients
                       had elevated protein concentrations in the cerebrospinal fluid, compared with 9% of the MMN
                       patients (P < 0.001). No MADSAM neuropathy patient had elevated anti-GM1 antibody titers,
                       compared with 56% of MMN patients (P < 0.01). In contrast to the subtle abnormalities described
                       for MMN, MADSAM neuropathy patients had prominent demyelination on sensory nerve biopsies.
                       Response to intravenous immunoglobulin treatment was similar in both groups (P = 1.0).
                       Multifocal motor neuropathy patients typically do not respond to prednisone, but 3 of 6 MADSAM
                       neuropathy patients improved with prednisone. MADSAM neuropathy more closely resembles
                       chronic inflammatory demyelinating polyneuropathy and probably represents an asymmetrical
                       variant. Given their different clinical patterns and responses to treatment, it is important to
                       distinguish between MADSAM neuropathy and MMN.

                       Comment in:
                            Muscle Nerve. 1999 Dec;22(12):1738-9
                            Muscle Nerve. 1999 Dec;22(12):1739-40
                            Muscle Nerve. 1999 May;22(5):557-9

[Rich5521]

THANKS, I will try that.  I kind of figured that's why I hadn't gotten a response when everyone else did.  There's a little information on it on Medline too, but not much.

[links]

Go to the following web site:

http://www.ncbi.nlm.nih.gov/PubMed/

enter lewis sumner in the search field.




Also look on the folowing site:

http://www.neuro.wustl.edu/neuromuscular/antibody/pnimdem.html

Do a local search for sumner




The info there doesn

[TxLefty]

I found some sites while using my favorite search engine, dogpile.com  It's a meta search.  Most of what I found dealt with MMN (multifocal motor neuropathy??) and evidently this is the formal name used by some.