Notes from talk of Dr Jacobs, Dayton OH chapter meeting

Dr Alan Jacobs was the speaker at the Dayton, Ohio GBS/CIDP Regional Meeting on 10/4/08 and he gave an excellent talk on Landry-Guillain-Barre Syndrome. He was very informative in understandable language, answered lots of questions from the audience, and treated everyone with respect and kindness—acknowledging that we know and want to know a lot about this illness that bothers us. I thought I would summarize my notes from the talk for this forum. His slides were more technical than how he explained things, so some of what I wrote down from the slides, I retranslated back to “regular speak”. I put things in double parentheses if I could not remember how he explained it because these are more paraphrased. Any errors are mine. He also talked about differences in the variants and about the immune attack which I do not include here because pictures really are important for these areas. This was the second meeting of this Dayton Chapter. There were maybe 40-50 people there (people with GBS and CIDP and families/friends/support people). A lot of us talked about life issues with each other before and after—which was really nice as well—sharing of ways that have helped us and things we have learned along the way. WithHope

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[B]Alan Jacobs, MD
Dayton, OH
10/4/08
Talk on GBS[/B]

[B]Features of GBS[/B]
Progressive over days to 4 weeks
Relatively symmetric one side to other
Mild sensory signs and symptoms
Cranial nerve involvement especially bilateral facial nerves affected
Recovery starts 2-4 weeks after progression ceases
Absence of fever at onset
Typical CSF and EMG/NCV features (higher protein in spinal fluid with few cells = albuminocytologic dissociation and nerve conduction slowing absent F waves, and decrease in amplitude).

[B]Precipitating factors (factors that are associated with the start of GBS)[/B]
Often 2-4 weeks after an illness or immunization
CMV, Campylobactera, Mycoplasma pneumonia, EBV, HIV, ?hepatitis A
immunizations–influenza, +/- oral (live) polio

[B]GBS also has been associated with [/B]
Hodgkin’s lymphoma, hyperthyroidism, sarcoidosis, collagen vascular disease, and renal disease
It can occur after surgery, (?), pregnacy, envenomization ((bite by snake or spider)), bone marrow transplant, drug ingestion

[B]GBS can be thought of as a form of “friendly fire”[/B]
in which ones immune system, designed to protect you from external threats, instead turns against itself. It can happen because of confusion due to molecular mimicry where a new antigen looks like it belongs to your body or, alternatively or additionally, due to excessive cytokine stimulation of the immune response.

[B]Important monitoring and supportive care issues[/B]
Watch for respiratory failure, about 33% are intubated at average of one week after onset of first symptoms
Chest physiotherapy/timing of tracheostomy (ways to help prevent infection in the lungs and transition off the respiratory)
DVT prophylaxis (prevention of blood clots)
GI prophylaxis (help with risk of ulceration/gastritis with stomach bleeding, slowing of the intestinal system/constipation, etc)
Infection control and skin breakdown on pressure areas
Autonomic instability (watch heart rate and blood pressure as these can vary rapidly and not be as well controlled as typical)
Pain control and adjustment/communication issues
Prevention of joint constriction and loss of mobility (PT= physical therapy, positioning, splints–keep joints in neutral position).

[B]Plasmapheresis[/B]
Each “run” removes about 60% of the body protein.
Also removes complement, immunoglobulins, immune complexes, cytokines, and interleukins ((the latter two proteins important for regulation of the immune response))
It is typically done every other day for 3-5 total times.

[B]IV IgG[/B]
It is not completely clear how this works or why. Theories include anti-id ((stopping the immune response by binding to the specific part of the abnormal antibody and preventing it binding)), inhibition of the cytokines that overstimulate the immune response, sponging up all the complement, and interfering with antigen presentation by binding up all the Fc receptors on macrophages.

[B]Arguments for being in an acute care hospital initially[/B]
Risk of deep venous thrombosis, blood clots that can damage limbs or break off and go to the lung and cause serious immediate breathing problems
Risk of compressive neuropathy ((where pressure worsens nerve damage))
Risk of breathing failure due to weak breathing muscles including the diaphragm
Dysautonomia ((wild fluctuations in heart rate and blood pressure that can lead to stroke, fainting, heart trouble))
Bladder dysfunction (cannot empty bladder to get rid of urine waste)
Weight loss/nutrition concers ((need nutrition to heal))
Psychosocial ((it is common to be scared, saddened, and feel helpless with this much sudden change in health))
Rehabilitation plan ((need a group plan for recovery since the effects of GBS alter so many areas so much))

[B]5 phases of recovery[/B]
1. Experiencing dependency
2. Encountering helplessness
3. Wanting to know more about it
4. Discovering inner strength
5. Regaining independence

Bill Werling (the Dayton, OH regional liason) added a very relevant 6th–Learning to appreciate more what one can do in life.

[B]Standard quotes for recovery[/B]
85% full and functional recovery in 6-12 months. Maximum by 18 months.
7-15% permanent sequelae including foot drop, unsteadiness, or numbness/paresthesias
3-8% die
Later he said that newer information is sometimes slower recovery (see below) and sometimes less return to full function.

[B]Recovery-Newer information[/B]
Serious long term complications and problems occur and recovery can take up to 3-6 years
Usually the gains back are greatest in the first year–at least half of what will gain back.
20% still report noticeable improvement 2 1/2 to 6 1/2 years after onset.

[B]Exercise[/B]
Early on, if one overdoes exercise, it can cause a paradoxical (unexpected) weakness and slow down recovery
It is very important to work with therapists that understand GBS and how to help form a slow and steady program for stregthening.
It is also important not to do to little movement or for too limited a time.

[B]Fatigue[/B]
Present in about 2/3–often the most disabling symptom, resulting in a decrease in quality of life
Degree of fatigue is not associated with the severity of the disease at worse point, event preceding disease (illness or vaccination, etc).
Amantidine does not help.
Steady exercise regiment can help. Report of a paper on bicycling for 12 weeks with improvement in fatigue and quality of life. Regiment was three times a week for 30 minutes and included 5 minutes warm-up, 30 minutes of stationary bike cycling, and 10 minutes of cool down. He emphasized the importance of the warm-up stretching and cool down phases to prevent injury or lead to more discomfort/aching from the activity.
He talked about activities that use arms and legs both.

[B]Sleep[/B]
He stated that some people think there is a “circadian rhythm” of activity such that people move the same amount totally in a day. If more is done in the day, there will be less movement at night and vice versa. This is an argument that exercise/mobility in the day can help get restorative sleep at night and so reduce fatigue. He talked about studies that if one gets only 5 hours of good sleep a night, one can drive as well as someone who is at the legal limit of alcohol. If one gets only 3 hours of restful sleep at night, this is comparable to someone at twice the legal limit of alcohol. Lack of sleep affects motor (muscle) function and judgment. Getting better sleep is very important to help fatigue. He said no medicines work well to help sleep—they do not give the quality of sleep ((REM)) a person really needs and that one is better exercising naturally so that the quality of the sleep is more restorative.

[B]Features of CIDP[/B]
Symptoms progress over more than a month
Symptoms fluctuate
Less problems with respiratory failure, dysautonomia, facial weakness/ophthalmoplegia
CSF protein may be very high
Marked slowing in nerve conduction
Steroids may benefit (not so in GBS)

[B]Goals for additional learning by neurologists [/B]
1. Better immunotherapies–especially combinations to affect the immune system
2. Better classification into risk groups and response to different kinds of treatment ((what would work best for any subset of people with GBS))
3. Better supportive care–autonomic instability (help with the blood pressure and heart rate changes), pneumonia, fatigue, rehabilitation

[B]New treatments being investigated[/B]
1. CSF filtration which is supposed to “drain the roots coming off the spinal cord” of antibody. ((This would be by spinal tap to remove and “clean” the spinal fluid to remove antibody. ))
2. Immunoabsorption–removal of antibody ((this is typically a way to remove antibody/immunoglobulin without removing the other “good” things in the solution))
3. Interferon beta
4. Brain derived neurotrophic factors and other cytokines/neurotrophins (proteins) that promote healing of damaged nerves