If you are suffering from a
autoimmune disorder, read this for a possible END TO
disease. Along with autoimmune disease prevention guidelines.
The Lewis- Sumner syndrome (LSS) is a dysimmune multifocal
demyelinating sensorimotor neuropathy. It should be considered as a
clinical asymmetrical variant of chronic immune demyelinating
polyneuropathy (CIDP). LSS is five times less frequent than CIDP
whose prevalence is between 2 and 7/ 100,000.
Patients with LSS
usually present with an asymmetrical involvement of the upper limb
with distal sensorimotor deficit in median or ulnar territories. A
purely sensory onset with numbness and paresthesia or pain in median
or ulnar territory is observed 30% of cases. A lower limb onset is
present in 30% of patients with a distal and asymmetrical
sensorimotor deficit. Amyotrophy and cranial nerve involvement may
be observed in 50% and 20% of patients, respectively. LSS could
mimick a nerve entrapment or a vasculitis. The course is progressive
or remitting. Electrophysiological pattern associates a multifocal
motor demyelination with conduction blocks mostly situated in the
forearm. Contrarily to CIDP, other conduction anomalies (reduction
of truncal motor nerve velocities, prolonged distal latencies or
prolonged F waves) occur rarely outside the blocked nerve territory.
Sensory conduction shows a multifocal sensory involvement. Sural
nerve biopsy in LSS show elements consistent with a primary
demyelination, indistinguishable from that seen in typical CIDP.
However nervous biopsy is not necessary to establish the diagnosis.
Serum anti-GM1 antibodies are negative and CSF protein content is
usually normal or mildly elevated with a mean value of 0.7 g/l. LSS
is characterized by a responsiveness to IVIg and steroids. For LSS
patients, a treatment similar to that of CIDP, with a first line
treatment with intravenous Ig (IVIg) (2g/kg/course), is recommended.
Patients who do not respond after 2 or 3 courses should be switched
to prednisone; a dose of 1mg/kg/day should be maintained for 4-6
weeks, then slowly tapered. Plasma exchanges are not recommended in
LSS. *Author: Dr K. Viala (August 2003)*.
Update : 07/08/2005
Rinsho Shinkeigaku. 2000 Nov;40(11):1126-9.
[Lewis-Sumner syndrome presenting unilateral
quadriceps amyotrophy as an initial symptom]
Second Department of Internal Medicine, Fukui Medical University.
We report a 55-year-old man with a chief complaint of wasting and
weakness of the left quadriceps muscle. At age 54, he noticed
difficulty in running and weakness in the left thigh, which
gradually progressed. On the first admission to our hospital, based
on the nerve conduction studies (NCS), the muscle biopsy findings
showing neurologenic changes, and no abnormality of spinal MRI, we
diagnosed as unilateral quadriceps amyotrophy, which resulted from
an atypical form of spinal progressive muscular atrophy. One year
later, he showed the bilateral hand weakness, conduction blocks on
the right median and ulnar nerves by NCS, and the presence of serum
anti-GM 1 antibody. From these findings, Lewis-Sumner syndrome was
diagnosed. The therapy of high-dose intravenous immunoglobulin
moderately improved his symptoms. The clinical symptoms of
quadriceps amyotrophy is produced by various disorders including
spinal progressive muscular atrophy, spinal extradural arachnoid
cyst, rimmed vacuole myopathy, Becker dystrophy, limb-girdle
dystrophy, and focal myositis. However, there have been no reports
of a case of Lewis-Sumner syndrome. It is important to consider
Lewis-Sumner syndrome in the differential diagnosis of quadriceps
PMID: 11332195 [PubMed - indexed for MEDLINE]
Rinsho Shinkeigaku. 1999 Jan;39(1):107-9.
[Diagnosis and treatment of multifocal motor
Department of Neurology, Kyoto University Hospital.
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
2004 Sep;127(Pt 9):2010-7. Epub 2004 Aug 2.
Follow-up study and response to treatment in
23 patients with Lewis-Sumner syndrome.
Federation de Neurophysiologie Clinique, Hopital de la
Salpetriere, 47 Boulevard de l'Hopital, 75651 Paris cedex 13,
Lewis-Sumner syndrome (LSS) is a dysimmune peripheral nerve
disorder, characterized by a predominantly distal, asymmetric
weakness mostly affecting the upper limbs with sensory
impairment, and by the presence of multifocal persistent
conduction blocks. The nosological position of this neuropathy
in relation to multifocal motor neuropathy (MMN) and chronic
inflammatory demyelinating polyradiculoneuropathy (CIDP) is
still debated. We report the clinical, biological and
electrophysiological features, the course and the response to
treatment in 23 LSS patients. The initial symptoms started in
the distal part of an upper limb in 70% of patients. They were
sensorimotor in 65% and purely sensory in 35% of patients. A
cranial nerve involvement was observed in 26% of patients and a
distal limb amyotrophy in 52%. The CSF protein level was normal
in 67% of patients and mildly elevated in the remainder. None
had serum anti-GM1 antibodies. There were multiple motor
conduction blocks (average of 2.87/patient), predominantly
located in the forearm, whereas demyelinating features outside
the blocked nerves were rare. Abnormal distal sensory potentials
were found in 87% of patients. The electrophysiological pattern
suggests a very focal motor fibre demyelination sparing the
nerve endings, whereas sensory fibre involvement was widespread.
The course was chronic progressive in 71% of patients and
relapsing-remitting in the others. During the follow-up study
(median duration of 4 years), half of the patients progressed
with a multifocal pattern and the distribution of the motor
deficit remained similar to the initial presentation. The other
patients showed a progression to the other limbs, suggesting a
more diffuse process. Fifty-four percent of the patients treated
with intravenous immunoglobulin showed an improvement, compared
with 33% of the patients treated with oral steroids. Overall,
73% of patients had a positive response to immune-mediated
therapy. LSS may be distinguished from MMN by the presence of
sensory involvement, the absence of serum anti-GM1 antibodies
and, in some cases, a positive response to steroids. In some of
the patients in our study, LSS evolved into a more diffuse
neuropathy sharing similarities with CIDP. Others had a clinical
course characterized by a striking multifocal neuropathy, which
suggests underlying mechanisms different from CIDP. Overall,
whatever the clinical course, LSS responded to immune-mediated
treatment in a manner similar to CIDP.
PMID: 15289267 [PubMed - indexed for MEDLINE]
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