Search the Health Library
Get the facts on diseases, conditions, tests and procedures.
I Want To...
I Want To...
Find Research Faculty
Enter the last name, specialty or keyword for your search below.
School of Medicine
I Want to...
NeuroLogic - Recognizing excellence for GBS and CIDP
NeuroLogic Winter 2012
Recognizing excellence for GBS and CIDP
Date: January 3, 2012
David Cornblath is one of several GBS/CIPD experts at Hopkins, a Center of Excellence for these conditions.
A previously well patient gets a nonspecific illness, such as the flu or diarrhea. When back pain, numbness and tingling, then extraordinary weakness set in, the patient may be alarmed enough to head to the emergency room, only to be sent home with a vague diagnosis. By the time the patient returns, hospitalization may be inevitable.
Neuromuscular disease expert David Cornblath fully understands why most physicians have trouble recognizing Guillain-Barré syndrome (or the even more insidious chronic inflammatory demyelinating polyneuropathy, or CIDP, which behaves like a chronic form of GBS, creeping up more slowly, and typically without an inciting event). With an annual U.S. incidence of only two cases per 100,000 people for each condition, these diseases are so rare that few physicians ever encounter them. Adding to the challenge, these diseases must be treated quickly. The paralysis that GBS causes, for example, can lead to problems with breathing and regulating blood pressure and heart rate.
As is true with other rare medical conditions, Cornblath says, the more cases of GBS and CIDP that a single medical center sees, the better they tend to be at diagnosis and treatment. That’s one reason why Johns Hopkins has been named a Center of Excellence by the GBS/CIDP Foundation International, joining nine other centers around the world that can improve care for GBS and CIDP patients who may not have ready access to experts on these syndromes.
Hopkins not only sees an unusually large number of patients with these conditions, they also train other experts and add to the body of knowledge. For example, Cornblath says, Hopkins’ experience suggests that CIDP patients may not need to be treated long-term with intravenous immunoglobulin (IVIG), a regimen that’s long been considered mandatory for patients with this condition.
“Most physicians believe that CIDP patients will need this treatment forever, but in fact up to 60 percent may not,” Cornblath explains. “We have a program to determine whether individual patients need ongoing IVIG or can successfully discontinue it.”
With IVIG costing several thousand dollars per treatment and sometimes producing uncomfortable side effects, Cornblath says, being able to stop treatment is a win-win for patients and the health care system in general.
He and his Hopkins colleagues are also working to further improve diagnosis and treatment of GBS and CIDP. They’re about to start a clinical trial to treat the most severe and resistant CIDP cases with a pharmaceutical that’s already FDA approved for other conditions. They’re also working with colleagues in the Netherlands to do natural history studies in both conditions, looking for genetic components and biomarkers associated with GBS and CIDP.
“In the future,” Cornblath says, “we expect to be able to extend our expertise and our excellence in treating these rare conditions even further.”
For information: 410-955-2229