on Lewis-Sumner syndrome
alternatives treatment of autoimmune
disease read our
in this the hands are
very weak you can see below the person has difficulty
opening the hands and making a fist with the left hand.
They usually get diagnosed as carpal tunnel syndrome and
are mistreated with surgery. The best treatment is IVIg
high dose 2g/kg every 4 weeks.
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
Lewis-Sumner syndrome presenting unilateral quadriceps
amyotrophy as an initial symptomFujiyama J,
Shinkeigaku. 2000 Nov;40(11):1126-9.
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. It is important to consider
Lewis-Sumner syndrome in the differential diagnosis of quadriceps
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 (LSS) is a dysimmune peripheral nerve
disorder, characterized by a predominantly , asymmetric weakness
mostly affecting the hands with sensory impairment, and by the
presence of multifocal persistent conduction blocks. . 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 hands 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 atrophy 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.