This is what I have If the HNPP is negative.
This is out of the Oxford Journal. By running search on
Mulifocal form of CIDP (lewis, sumner, brown, asbury).
All four of these Neuros are active. 3 of which are on this foundation
advisory board. My Neuro is Mark J. Brown who currently works with asbury
at the Hosp. of the Univ of PA. One of em is a generation older than the rest and is the mentor. the fourth is active in Rochester NY, Brown told me.
These four Gents were all part of the same office for many years. They also are all professors that teach this stuff to college students when they are not busy torturing patients. The have co written in all the big medical journals over the last 30 years.
This form is basically an asymetical version of CIDP with some features of MMN. As rare as CIDP is, I believe this is a variant of few numbers.
This is a match for me if HNPP is ruled out because I have cranial involvement, normal spinal fluid protein, asymetrical symptoms, multfocal blockages etc. See Journal article below if interested.
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.
Key Words: Lewis–Sumner syndrome; multifocal acquired demyelinating sensory and motor neuropathy; multifocal motor neuropathy; chronic inflammatory demyelinating polyradiculoneuropathy