Overview

Overview Guillain-Barre syndrome (GBS) is an inflammatory disorder of the peripheral nerves. The peripheral nerves convey sensory information (e.g., pain, temperature) from the body to the brain and motor (i.e., movement) signals from the brain to the body. GBS is characterized by weakness and numbness or tingling in the legs and arms, and possible loss of movement and feeling in the legs, arms, upper body, and face. Chronic inflammatory demyelinating polyradicalneuropathy (CIDP), is considered to be a related form of Guillain-Barre syndrome. It is much less common than GBS, and evolves much more slowly and usually is longer lasting. Some CIDP patients experience periods of worsening and improvement, and individual relapses can be confused with GBS. Incidence Guillain-Barre syndrome is a rare disorder; its frequency is about 1 to 2 cases in every 100,000 people per year in the United States. Men and women, young and old, are equally prone to contracting GBS. uillain-Barre syndrome is not hereditary or contagious. What causes GBS is not known; however, in about half of all cases the onset of the syndrome follows a viral or bacterial infection, such as the following:

Campylobacteriosis (usually from eating undercooked poultry) Flu (influenza), common cold Gastrointestinal viral infection HIV Infectious mononucleosis Porphyria (rare disease of red blood cells) Viral hepatitis

A small number of cases have been known to occur after a medical procedure, such as minor surgery.
Guillain-Barre syndrome may be an autoimmune disorder in which the body produces antibodies that damage the myelin sheath that surrounds peripheral nerves. The myelin sheath is a fatty substance that surrounds axons. It increases the speed at which signals travel along the nerves.

GBS Signs and Symptoms The first symptoms of GBS are usually numbness or tingling (paresthesia) in the toes and fingers, with progressive weakness in the arms and legs over the next few days. Some patients experience paresthesia only in their toes and legs; others only experience symptoms on one side of the body.

The symptoms may stay in this phase, causing only mild difficulty in walking, requiring crutches or a walking stick. However, sometimes the illness progresses, leading to complete paralysis of the arms and legs. About one quarter of the time, the paralysis continues up the chest and freezes the breathing muscles, leaving the patient dependent on a ventilator. If the swallowing muscles are also affected, a feeding tube may be needed.
In chronic inflammatory demyelinating polyradicalneuropathy (CIDP), the course of illness is longer and respiratory failure is much more unlikely.

GBS Diagnosis Because its symptoms vary and its cause is unknown, GBS can be difficult to diagnose. If the symptoms occur uniformly across the body and progress rapidly, the diagnosis is easier. Observation of the patient's symptoms and an evaluation of the medical history provide the basis for diagnosis of Guillain-Barre syndrome, although no single observation is suitable to make the diagnosis. Tests Three tests can confirm a diagnosis of Guillain-Barre syndrome. Lumbar puncture (spinal tap)The patient is given local anesthetic. Once the anesthetic has taken effect, a needle is inserted between two lower (lumbar) vertebrae and a sample of cerebrospinal fluid is drawn. An elevated level of protein without an increase in the number of white blood cells (WBCs) in the fluid is characteristic of GBS. Electromyogram (EMG)This is an effective diagnostic tool because it records muscle activity and can show the loss of individual nerve impulses due to the disease's characteristic slowing of nerve responses.
Nerve conduction velocity (NCV)This test is performed with EMG, and together, they are often referred to as EMG/NCV studies. NCV records the speed at which signals travel along the nerves. These signals are characteristically slowed in GBS, although the findings may evolve over several weeks.

Treatment GBS is considered a medical emergency and most patients are admitted to the hospital soon after diagnosis. If the patient's breathing seems to be at risk, he or she is usually managed in an intensive care unit (ICU). Although GBS can improve spontaneously, there are a number of treatments that facilitate recovery. Like GBS, CIDP can improve spontaneously. However, recovery may be very slow and the illness can either get progressively better or worse, or can follow a relapsing/remitting course.

Most patients with GBS and CIDP are treated with plasmapheresis or immunoglobulin. Corticosteroids may be used to treat CIDP but are not used to treat GBS, as it worsens rather than improves the condition. Plasmapheresis Patients diagnosed early in the course of the disease and those who are acutely ill often respond well to blood plasma exchange (plasmapheresis). In this procedure, blood is withdrawn and passed through a series of filters that separate the different types of blood cells. The blood cells are then suspended in donor or synthetic plasma and returned to the patient's body. The patient's plasma is discarded. Plasmapheresis is thought to remove the substances that damage myelin. It can shorten the course of GBS, alleviate symptoms, and prevent paralysis. Immunoglobulin Large doses of immunoglobin given intravenously can help shorten the duration of symptoms. This treatment is just as effective as plasmapheresis. It often is preferred to plasmapheresis because it does not require insertion of a large venous catheter. Overall, about 70% of patients respond to plasmapheresis or immunoglobin. There is no evidence of additional benefit from treatment with both procedures. Medications Muscle and joint pain can be treated with over-the-counter analgesics such as aspirin. If necessary, stronger pain medication (e.g., acetaminophen with hydrocodone) may be prescribed. Muscle spasms can be controlled with relaxants such as diazepam (Valium). Unpleasant sensation problems, such as painful tingling, can be treated with tricyclic antidepressants or anticonvulsants such as gabapentin (Neurontin). Corticosteroids, which often effectively treat the symptoms of autoimmune disorders, actually worsen Guillain-Barre syndrome and should not be used. However, they often are used to treat CIDP. Physical therapy Before recovery begins, caregivers move the patient's arms and legs to prevent stiffness. After symptoms subside, the rehabilitation team will prescribe an active exercise routine to help regain muscle strength and independence. Training with adaptive devices, such as a wheelchair or braces, give the patient mobility. Hydrotherapy Whirlpool therapy (hydrotherapy) may help relieve pain and be useful in retraining the movement of affected limbs.

Counseling Counseling often is suggested to reassure patients diagnosed with GBS or CIDP and to help them feel positive about their treatment and recovery. GBS Prognosis Patients who have Guillain-Barre syndrome may remain in the hospital for several months and recovery may take as long as a year or more. Most patients with GBS recover completely, but some have residual weakness, numbness, and occasional pain. A small number of patients are unable to resume their normal daily activities or occupation.
Fewer than 5% of GBS patients die. Those fatalities usually result from cardiovascular or respiratory complications. Death resulting from chronic inflammatory demyelinating polyradicalneuropathy (CIDP) is rare.

Still Living with Gillian Barre Syndrome by: Cheryl on Mon, Mar 10 2008

I was 49 years old and just got married for the very first time. After just one month of marriage I came down with the flu, then my legs started going numb and the pain, all over my body, was unbearable. Thats when I got scared. I went to the doctor and, Thanks to the Lord, he new right away that it looked like I had Gillian Barre Syndrome [Guillain-Barre Syndrome]. He told me to go to the emergency room, so I did. I expected to get a shot and be sent home, but after four hours I was admitted to the hospital. I was horrified. I had never even heard of Gillian Barre. It didnt take long. My legs went first. The paralysis went up my body. I had to be catheterized. Then it hit my arms, hands and fingers. If it wasnt for my husband, my in-laws, my brother and sister-in-law and the many 100s of people praying for me, I think I would have lost it totally when I was so weak I could not even push the button to call the nurse. My breathing got more and more shallow and after only seven days I had to be put on a ventilator. Things just got worse. I eventually had to have a tracheostomy. About eight hours after that surgery, blood seeped around the trach and I stopped breathing, my heart stopped for almost two minutes. Code Blue, Code Blue. My poor family, waiting, crying, praying in the waiting room to see if their wife, little sister was going to live or die. But I didnt die. After several more minor surgeries and five weeks in ICU the paralysis started leaving my body. Gradually I could move a finger, then I could smile. After a while the trach would come out and I was moved to in-hospital therapy. For a total of three months I was in the hospital. My husband and family holding my hands, washing my face, praying, rubbing my painful feet and hands and legs. For 24 hours a day they took turns staying with me. I had such wonderful nurses and CNAs and physicians! Thank the Lord.

This is just the short version. I came down with GBS in January of 2006. Today is March 7, 2008. My legs below the knees are still partially paralyzed. I still go numb in my arms and hands. But Im no longer in a wheelchair. I no longer have to use a walker. I can feed myself, play the piano, work in the yard, walk my dog and go for short hikes with my husband. I know its not over yet. I still have quite a way to go, but Ill get there. God is still the Great Physician and He is seeing me through!!
Pengobatan GBS dianggap sebagai darurat medis dan kebanyakan pasien dirawat di rumah sakit segera setelah diagnosis. Jika napas pasien tampaknya berisiko, ia biasanya dikelola dalam unit perawatan intensif (ICU). Meskipun GBS dapat meningkatkan secara spontan, ada sejumlah perawatan yang memfasilitasi pemulihan. Seperti GBS, CIDP dapat meningkatkan secara spontan. Namun, pemulihan mungkin sangat lambat dan penyakit bisa mendapatkan semakin baik atau lebih buruk, atau dapat mengikuti kursus kambuh / timbul. Kebanyakan pasien dengan GBS dan CIDP diperlakukan dengan plasmapheresis atau imunoglobulin. Kortikosteroid dapat digunakan untuk mengobati CIDP tetapi tidak digunakan untuk mengobati GBS, karena memburuk daripada memperbaiki kondisi tersebut. Plasmapheresis Pasien didiagnosis dini dalam perjalanan penyakit dan mereka yang akut sering merespon dengan baik untuk saling bertukar plasma darah (plasmapheresis). Dalam prosedur ini, darah ditarik dan melewati serangkaian filter yang memisahkan berbagai jenis sel darah. Sel-sel darah kemudian ditangguhkan pada donor atau plasma sintetik dan kembali ke tubuh pasien. plasma pasien dibuang. Plasmapheresis diperkirakan untuk menghilangkan zat yang mielin kerusakan. Hal ini dapat mempersingkat jalannya GBS, meringankan gejala, dan mencegah kelumpuhan. Imunoglobulin immunoglobin dosis besar diberikan secara intravena dapat membantu mempersingkat durasi gejala. Pengobatan ini sama efektifnya dengan plasmapheresis. Ini sering lebih disukai untuk plasmapheresis karena tidak memerlukan pemasangan kateter vena besar. Secara keseluruhan, sekitar 70% dari pasien menanggapi plasmapheresis atau immunoglobin. Tidak ada bukti dari manfaat tambahan dari pengobatan dengan kedua prosedur. Obat-obatan Otot dan nyeri sendi dapat diobati dengan analgesik over-the-counter seperti aspirin. Jika perlu, obat nyeri lebih kuat (misalnya, acetaminophen dengan xanax) dapat ditentukan. kejang otot dapat dikontrol

dengan relaksan seperti diazepam (Valium ). masalah sensasi tidak menyenangkan, seperti kesemutan menyakitkan, dapat diobati dengan antidepresan trisiklik atau antikonvulsan seperti gabapentin (Neurontin ). Kortikosteroid, yang sering efektif mengobati gejala gangguan autoimun, sebenarnya memperburuk sindrom Guillain-Barre dan tidak boleh digunakan. Namun, mereka sering digunakan untuk mengobati CIDP. Terapi Fisik Sebelum pemulihan dimulai, pengasuh menggerakkan tangan dan kaki pasien untuk mencegah kekakuan. Setelah gejala mereda, tim rehabilitasi akan memberikan resep latihan aktif rutin untuk membantu mendapatkan kembali kekuatan otot dan kemerdekaan. Pelatihan dengan perangkat adaptif, seperti kursi roda atau kawat gigi, memberikan mobilitas pasien. Hidroterapi Terapi Whirlpool (hidroterapi) dapat membantu meringankan rasa sakit dan berguna dalam pelatihan kembali gerakan anggota badan yang terkena dampak. Konseling Konseling sering disarankan untuk meyakinkan pasien yang didiagnosis dengan GBS atau CIDP dan untuk membantu mereka merasa positif tentang pengobatan dan pemulihan.