New Jefferson Study May Redefine How a Chronic Auto-Immune Disease is Diagnosed
New research from Jefferson Hospital for
Neuroscience (JHN) may redefine how Chronic Inflammatory Demyelinating
Polyneuropathy (CIDP) is diagnosed. Eduardo De Sousa, M.D., assistant professor
of Neurology at Jefferson Medical College of Thomas Jefferson University, and
director of the Electrodiagnostic Neuromuscular Lab at JHN, led the study which
looked at the number of demyelinating features that are needed to differentiate
between CIDP, Amyotrophic lateral sclerosis (ALS, or Lou Gehrig's disease) and
diabetic neuropathy. His research suggests a minimum number of three
demyelinating features can be used to positively identify CIDP in a patient.
CIDP is a neurological disorder characterized by progressive weakness and
impaired sensory function in the legs and arms. It affects about 50,000 people
in the United States. The study, available in the current edition of the
Journal of Clinical Neuromuscular Disease, may help doctors more
effectively diagnose and treat CIDP.
Currently, to make a diagnosis of CIDP,
patients undergo nerve conduction studies to determine the number and severity
of abnormalities on electrodiagnostic tests. Patients with a specific pattern
and number of abnormalities, also know as demyelinating findings, during these
studies are determined to have CIDP.
The study involved 26 CIDP patients and a
control group of 21 patients, nine ALS patients and 12 diabetic neuropathy
patients. The researchers judged the number of demyelinating findings in the
CIDP patients that responded to the treatment. They then analyzed the number of
features to make the screenings more sensitive. Their findings suggest that
with three demyelinating features significantly increased the specificity of the
diagnosis of CIDP, but in exchange, the sensitivity was reduced; two features
produced a less specific pattern making it difficult to distinguish between
CIDP, ALS or diabetic neuropathy, but increased the sensitivity of the test
allowing to diagnose patients earlier on the course of their disease; and one
feature was determined to have low specificity for the diagnosis of CIDP.
“This is a clinically significant finding
that may help doctors screen potential CIDP patients,” said Dr. De Sousa.” In
determining the number of demyelinating findings needed to define CIDP, doctors
may be able to make a diagnosis sooner allowing for a targeted treatment to
begin quicker, preventing further disability.”
CIDP can occur at any age, but is most
common in the elderly and in men. It often presents with symptoms that include
tingling or numbness (beginning in the toes and fingers), weakness of the arms
and legs, loss of deep tendon reflexes, fatigue, and abnormal sensations. CIDP
is closely related to Guillain-Barre syndrome, but instead of having rapid
onset, CIDP has a more protracted chronic course. Treatment for CIDP includes
corticosteroids such as prednisone, plasmapheresis (plasma exchange) and
intravenous immunoglobulin (IVIg). IVIg may be used even as a first-line
therapy. Immunosuppressant drug therapy may be effective in patients who fail
standard therapy. Physiotherapy may improve muscle strength, balance, function
and mobility, and minimize the shrinkage of muscles and tendons and distortions
of the joints.
Media Only Contact:
Ed Federico
Thomas Jefferson University Hospital
Phone: (215) 955-6300
Published: 6/10/2009