The Orange County Neurological Society For Education
and Service to our
Community President: Mike Kreidie, MD, Coordinator: Elizabeth Pearson
Presented
Jonathan
S.Katz, MD
Department of Neurology
Stanford University
“Chronic Immune
Neuropathies: IVIG as treatment
and diagnostic test”
Here is a review of what Dr. Katz had to
say. He told the neurologist attending this meeting that we should
stop worrying about the cost of the treatments; there is always the
H.M.O. to take care of this issue. So let the HMO worry who can get
IVIg .
Following tests for neuropathy are not needed,
(antibody screens, neuropathy panels) the cost of these screens
varies from 1500 to 2000 dollars and then one does not know what to
do with the result. Sedimentation rate should be done along with a
B12 level as you can diagnose vasculitis or vitamin deficiency based
upon the results.
No nerve biopsy should be attempted;
this will leave the patient with a life long sensory deficit (Do Not
Cause Any Harm) Hippocraties. Biopsy is useless in patients with
CIDP as they have skip lesions, which cannot be seen on a routine
biopsy. Some patients have motor involvement and the sural biopsy
will not show this. Because sural nerve is a pure sensory nerve. The
diagnois should be based upon clinical examination.
Finally do not follow any guidelines to
make a diagnosis. As only 20-30% of the patients will fit the AAN
guideline. Patients will come in with different presentations.
EMG/NCV can be normal if only small fibers
are involved, or show axonal or demyelinating pattern. Thus you
cannot depend upon the EMG/NCV to diagnose CIDP.
So the best test to prove that patient
has a immune neuropathy is to treat them with IVIG.
Those that report improvement should be
considered as suffering from immune neuropathies.
Usually you will not see a improvement in their
examination after they have been treated with a course of IVIg. If
the patient states that they have been helped then its best to
document this.
Steroids will not improve axonal conditions
like M.M.N. They may help CIDP patients but will take 2-3 months to
see a response.
Dr Katz has published several papers on CIDP
and he wrote the Medicare guidelines for CIDP in California.
Read about
axonal EMG findings in CIDP
Axonal multifocal motor neuropathy without conduction block or
other features of demyelination
J.S. Katz, MD;, R.J. Barohn, MD;,
S. Kojan, MD;, G.I. Wolfe, MD;, S.P.
Nations, MD;, D.S. Saperstein, MD; and A.A.
Amato, MD
From the Departments of Neurology, Palo Alto VA
Medical Center and Stanford University (Dr. Katz), Palo Alto, CA;
University of Kansas Medical Center (Dr. Barohn), Kansas City;
University of Texas Southwestern Medical Center (Drs. Kojan, Wolfe,
Nations, and Saperstein), Dallas; and Brigham and Women’s Hospital
(Dr. Amato), Harvard Medical School, Boston, MA.
Address correspondence and reprint requests to
Dr. Jonathan Katz, Department of Neurology (127), Department of
Veterans Affairs, 3801 Miranda Ave., Palo Alto, CA 94304; e-mail:
jskatz@stanford.edu
Background: Conduction block is considered an
essential finding for the distinction between motor
neuropathies and lower motor neuron disorders. Only a
small number of reports describe patients with multifocal
motor neuropathies who lack overt conduction block,
although in these cases other features of demyelination
still suggest the presence of a demyelinating disorder. In contrast,
a purely axonal multifocal motor neuropathy has not been
described.
Methods: This report describes nine patients with
slowly or nonprogressive multifocal motor neuropathies
who had purely axonal electrodiagnostic features.
Results: GM1 antibodies titers were normal in all
nine cases. Six patients were treated with either
prednisone or IV immunoglobulin and three showed
convincing improvement.
Conclusions: These findings suggest an
immune-mediated motor neuropathy with axonal
electrophysiologic features that appears to be distinct
from both multifocal motor neuropathy and established
motor neuron disorders.
