I was diagnosed with GBS in January of 2003 and after my 2nd relapse they changed the diagnosis to CIDP in April of 2003. I was never hospitalized however because my symptoms were slower at progressing than most GBS patients. (this is very common with CIDP) Please go to the website GBS/CIDP Foundation International and read the forums there. You will find a lot of information that could be very useful for you and your relative. Good luck!

Hi Amal. I'm glad your uncle's doing better. Agree with all the responses. It's good he got IVIg. It hastens recovery. If the symptoms progress, you'd need to consider CIDP rather than GBS, in which case treatment would still be IVIg. CIDP would be responsive to steroids. GBS is not. Hope that helps! Take care, Amal!!!

Guillain-Barre´ syndrome (GBS) is an acute onset, immune-mediated disorder of the peripheral nervous system. The term GBS is often considered to be synonymous with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), but with the increasing recognition over the past few decades of variants, the number of diseases that include axonal variants and more restricted variants such as Miller Fisher syndrome (MFS). GBS is equally common in men and women and can occur at any age.

An otherwise unremarkable infection, such as an upper respiratory infection, often predates the onset of GBS by 10 to 14 days. Many antecedent infections have been identified, including Campylobacter jejuni, cytomegalovirus (CMV), Mycoplasma pneumonia, Epstein-Barr virus, and influenza virus.

GBS usually begins abruptly with distal, relatively symmetrical onset of paresthesias. Sensory disturbances are accompanied by or quickly followed by progressive limb weakness. Patients are able to identify a definite date of onset of sensory and motor disturbances. Progression is rapid, with about 50% of patients reaching clinical nadir by 2 weeks and more than 90% by 4 weeks. Current diagnostic criteria include <4 weeks of progression to clinical nadir. Approximately 80 to 90% of patients with GBS become nonambulatory during the illness.

Neurological examination will demonstrate distal and often proximal, relatively symmetrical, weakness. Sensory examination is often normal in the early phase of disease. Widespread areflexia or hyporeflexia is the rule. GBS patients often develop cranial nerve weakness, usually in the form of facial or pharyngeal weakness. Diaphragmatic weakness due to phrenic nerve involvement is also common. Approximately one third of hospitalized GBS patients require mechanical ventilation because of respiratory muscle or oropharyngeal weakness. Autonomic disturbance is seen in more than 50%. The autonomic disturbance usually manifests as tachycardia but more serious autonomic nervous system dysfunction may occur, including life-threatening arrhythmias, hypotension, hypertension, and gastrointestinal dysmotility.

Supportive ancillary testing for GBS includes CSF analysis and electrodiagnostic testing, both of which may be normal in the early phase of GBS. An elevated CSF protein concentration (with normal cell count) is only found on initial CSF analysis in about 50% of patients; elevated CSF protein concentration occurs in more than 90% of patients at clinical nadir.

Electrodiagnostic testing of GBS patients often also demonstrates features of demyelination, such as temporal dispersion, significantly slow conduction velocities, and prolonged distal and F-wave latencies. Electrodiagnostic testing features of acquired demyelination (e.g., conduction block, temporal dispersion, nonuniform slowing of conduction velocities) are particularly helpful because these findings are characteristic of immune-mediated demyelinating neuropathies.

Some patients have clinical features and disease course similar to GBS except for a slower progression (i.e., progression that lasts longer than 4 weeks); this disease is sometimes referred to as subacute inflammatory demyelinating polyradiculoneuropathy (SIDP).

The most recognizable and distinct regional variantof GBS is MFS. MFS patients classically present with external ophthalmoparesis, areflexia, and ataxia.

Management
Plasma exchange (PE) and IVIg are effective immunotherapies for adult and pediatric patients with GBS if given during the first few weeks of disease. Although IVIg and PE are probably of equal efficacy, nonsignificant trends toward faster recovery have been observed with IVIg in trials designed to compare it to PE. No compelling evidence favors one treatment over the other.
Immunoabsorption therapy is an alternative technique to PE that does not require using a human blood product as a replacement fluid, thereby reducing risk of infection or allergic reaction. Immunoadsorption therapy removes Ig from the circulation without need for replacement with albumin or fresh-frozen plasma because of the lower loss of albumin.
Corticosteroid treatment is ineffective for treating GBS.

Early relapses occur in ~10% of patients with GBS following PE or IVIg therapy. Rate of relapse is similar for PE and IVIg.

For instance, some patients initially diagnosed with GBS— with preceding infectious episode, abrupt onset of neuropathic symptoms, and progression to clinical nadir within 4 weeks—are later rediagnosed as having CIDP because of the persistence of neuropathy deficits, based on ongoing demyelination caused by an active autoimmune process.

It is occasionally challenging to determine whether persistent deficits are caused by ongoing autoimmunity and demyelination or the secondary, residual axonal damage of previously active GBS.
In such instances, repeat electrodiagnostic testing can be helpful, and if testing suggests ongoing demyelination, CIDP and treatment for CIDP (e.g., corticosteroids) should be considered.


Management for Respiratory Failure and Airway Compromise

Neuromuscular respiratory failure requiring mechanical ventilation occurs in 20 to 30% of GBS patients. A vital capacity below 20 mL/kg, maximal inspiratory pressure (PImax) less than 30 cm H2O, or maximal expiratory pressure (PEmax) less than 40 cm H2O predicts imminent respiratory arrest. The mean duration of mechanical ventilation for GBS is 2 to 6 weeks. Some patients require tracheostomies to facilitate management.

Inpatient Rehabilitation
Approximately 40% of patients hospitalized with GBS will need inpatient rehabilitation. Muscle weakness may be associated with muscle shortening and joint contractures, complications that may be prevented by daily range-of-motion exercises. Appropriate exercise regimens are used during rehabilitation to improve strength.
Severe fatigue is a sequela of GBS in approximately two thirds of adult patients.

Thanks a lot for the feedback. By the way, it is not my uncle but my cousin who is only 4 years old.

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my mistake...glad to hear there's no sequelae

The treatment in the early stages of Guillain-Barre Syndrome are designed to shorten the course of the disease. There are two ways to treat GBS. One can use plasmaphresis. This is a process of removing the antibodies from a patient’s blood in a manner very similar to dialysis. Most patients receive five courses of plasmaphresis and then no additional therapy. The other treatment involves receiving intravenous gammaglobulin. Most studies indicate that these two treatments have essentially the same efficacy.

GBS improves spontaneously. However, certain factors can assist recovery:

• good nursing and medical/intensive care;
• hysiotherapy and hydrotherapy, therapies that relieve discomfort and prevent stiffness;
• immunoglobulin — the infusion of immunoglobulin proves successful with similar results to plasmapheresis;
• lasmapheresis — the exchange of blood plasma generally reduces the duration of the disease in severe cases if carried out in the first few days; and
• counselling to reassure the patient and encourage the patient towards recovery.

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Thanks a lot. My cousin is doing great now. He is walking and playing around normally now.

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That's an "urban legend"
Early detection rapid therapy and vigourous physio is the only established form of therapy.