Guillian-Barre causes progressive weakness

Keith Roach

Dear Dr. Roach: Three months ago, my daughter, age 50 and a healthy person, was diagnosed with Guillain-Barre syndrome. The onset was gradual, beginning with numbness in her hands and feet, and severe pain in her back. After an MRI didn’t reveal any nerve problems in her spine, she was referred to a neurologist and began an IV of immunoglobulin. Her condition improved dramatically, but two weeks ago the symptoms returned and she began a series of IVs again. Since she wasn’t totally paralyzed, we assumed her condition wasn’t as severe as some of the cases we had heard of, and thought she had recovered. Should she should continue with this treatment, or try to find something else?

K.S.

Dear K.S.: Guillain-Barre syndrome is an acute autoimmune neurologic illness that affects the peripheral nerves of the body. It often occurs after a viral or bacterial infection, is more common in males and is increasingly prevalent with ad­vancing age. Although Guillain-Barre syndrome comprises several related diseases, the most common (about 90 percent) is acute inflammatory demyelinating polyneuropathy. Myelin is the “sheath” that nerves are wr­apped in, and it acts as an insu­lator. It is the myelin protein th­at is the target of the antibody attack. With no myelin, the ner­ve impulses can’t get through, and weakness is the result.

In AIDP, the patient notices progressive weakness, often beginning in the legs. The weakness can vary from mild difficulty walking to complete paralysis requiring ventilator support in about 20 percent of cases. Symptoms progress over weeks, and by four weeks after the onset of symptoms, most people have gotten as sick as they will and are starting to get better. Treatment consists of IV immune globulin or plasma exchange.

In your daughter’s case, it’s not clear to me whether she has a relapse of AIDP, or whether she has chronic inflammatory demyelinating polyneuropathy. This can be difficult to tell from AIDP. Only time will tell the difference. CIDP continues to progress or has relapses for greater than eight weeks. However, CIDP also is treated with IV immune globulin. If her neurologist is concerned about CIDP, he or she also might recommend plasma exchange or steroids. I can’t emphasize enough the need for experienced clinical judgment in these related conditions. In both AIDP and CIDP, many people recover completely, but some degree of residual weakness is common.

Dear Dr. Roach: Several months ago, I developed a hernia on my right side, but with no pain. My doctor said I don’t need an operation, because there is no pain. Do you agree? I am 74 and in exceptionally good health.

T.I.

Dear T.I.: A hernia is a wea­kness or defect in the abdominal wall, through which abdominal structures can pass. Watchful w­aiting is a reasonable choice for an asymptomatic hernia — one that causes no sy­mpt­oms. The biggest risk is part of the in­testine coming through the her­nia and becoming stuck, and is a surgical emergency. Surge­ons so­metimes recommend sur­gery on hernias with no symptoms to prevent this complication.

How YOU feel about it is the critical issue. If you feel more comfortable getting it fixed now to prevent the chance of future problems, tell your doctor and ask to see a surgeon.

Email questions to ToYourGoodHealth@med.cornell.edu.