Guillain-Barré syndrome
BMJ 2008; 337 doi: https://doi.org/10.1136/bmj.a671 (Published 17 July 2008) Cite this as: BMJ 2008;337:a671
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The review article on Guillain-Barré syndrome (1) (July 26), the comprehensive review of the condition on the web-site http://neuromuscular.wustl.edu, and the
rapid responses on bmj.com do not make comment on the
established association with epidemics of enterovirus infections, most
often EV71 and an association with human hand foot and mouth in some
epidemics (2). Our interest in this developed when a family member, aged
30, developed neurological symptoms; initially a unilateral facial palsy
progressing to Guillain-Barré syndrome syndrome with CSF and EMG
confirmation. Ten days before the facial palsy there had been contact
with a nephew (aged 1) at the time of an attack of hand, foot and mouth
disease when other infants in his crèche had similar mouth and skin
symptoms. One other adult member of the family had concurrent oral
symptoms.
An internet search located well documented “epidemic” outbreaks of
neurological illness reviewed by Huang and colleaugues (2), including
patients with features of Guillain-Barré syndrome, in association with
epidemics of human hand, foot and mouth disease. Small epidemics were
reported from Victoria (3) and from Western Australia(4) and large
outbreaks in South-East Asia (2) were associated with mortality and
permanent neurological disability.
Neither enterovirus 71 or other known pathogens were identified from our
family member. Her illness did follow shortly after discharge from an
admission with gastro-intestinal symptoms, including diarrhea, from a
hospital that had other wards closed due to an epidemic of gastro-
intestinal infection. It is also of interest that conversations with
physiotherapists involved with rehabilitation indicated an unusually large
number of patients reporting for rehabilitation to Victorian units at that
time. There is no reporting of neither Guillain-Barré syndrome nor of
hand, foot and mouth disease in our state so no formal epidemiological
data is available.
The association of Guillain-Barré syndrome with entero-virus
infections and with hand, foot and mouth disease merited inclusion in
Winer’s review. Clinicians and microbiologists should be aware of the
association, especially those working in SE Asia and Australia.
Martin Knapp FRCP Consultant Physician
Merilyn Mackenzie Dip Res. Methods (Latrobe) Physiotherapist
1. John B Winer Guillain-Barré syndrome
BMJ 2008;337 227-231
2. Huang CC, Liu CC, Chang YC et al. Neurologic complications of children
with enterovirus infection N Eng J Med 1999, 341: 936-942
3. Gilbert GL, Dickson KE Waters M, et al. Outbreak of enterovirus
infection in Victoria with a high incidence of neurologic involvement.
1988 Paediatr Infect Dis J; 7: 484-488
4. McMinn, P Stratov I ,Nagarajan L, Davis S, Neurological
Manifestations of enterovirus 71 infection in children during an outbreak
of hand, foot and mouth disease in Western Australia. Clinical Infectious
Diseases , 2001;15: 236-242
Competing interests:
None declared
Editorial note
The person whose case is described has given her signed, informed consent to publication.
Competing interests: No competing interests
John Winer's clinical review of Guillain-Barre syndrome (GBS)
provides an excellent summary of our current understanding of the
neuropathology, associated infections and best practice management of this
perplexing neurological condition.1 Surprisingly poliomyelitis was not
mentioned in the differential diagnosis of GBS presented. This probably
reflects the current exceedingly rare occurrence of poliovirus
presentation in most countries due to the remarkable efforts of the polio
eradication campaign.
Sustained political commitment, health service energy and non-
governmental organisation investment have driven confirmed poliovirus
cases down to levels unthinkable just a few decades ago . Unfortunately
as the end-game draws near, it is easy to become complacent, lose momentum
and even neglect to consider poliomyelitis in GBS presentations. It is
perilous to drop our surveillance guard now.2 While wild poliovirus
continues to circulate in a small number of countries, all countries
remain vulnerable to possible importation and a possible upswing in the
number of cases (U-shaped curve) as demonstrated in 2005 and 2006 with 21
previously polio-free countries importing wild poliovirus.3
Although the acute motor weakness associated with poliomyelitis is
more commonly asymmetrical in nature, and sensory and autonomic features
are uncommon, it is hazardous to definitively exclude poliovirus infection
on clinical grounds alone.
Now is not the time to declare a premature victory against polio. It
is essential that all countries, both developing and developed, maintain a
high level of alertness and ensure that appropriate stool specimens are
submitted from all cases of acute flaccid paralysis (~GBS), carefully
maintaining the reverse cold chain of these specimens in transit to
approved virological laboratories that can actively exclude poliovirus as
the cause of the syndrome.4
References
1. Winer JB. Guillain-Barr Syndrome. BMJ 2008; 337: 227-231.
2. Durrheim D, Massey P, Kelly H. Re-emerging poliomyelitis – is
Australia’s surveillance adequate? Commun Dis Intell 2006; 30: 275–277.
3. Centres for Disease Control and Prevention. Resurgence of wild
poliovirus type 1 transmission and consequences of importation, 21
countries, 2002–2005. MMWR Morb Mort Wkly Rep 2006; 55: 145–150.
4. World Health Organization. Acute flaccid paralysis (AFP) surveillance:
the surveillance strategy for poliomyelitis eradication. Wkly Epidemiol
Rec 1998; 16: 113–117.
Competing interests:
None declared
Competing interests: No competing interests
Editor
The clinical review on Guillain-Barre Syndrome by Winer1 whilst
mostly comprehensive leaves out a vital management component. The author
omits to mention therapy support and rehabilitation as an essential
adjuvant intervention to pharmaceutical and intensive measures.
Rehabilitation must form an essential component of management plan for
patients with the diagnosis of Guillain-Barre Syndrome2. We regularly see
patients with Guillain-Barre Syndrome and do so at an early stage.
Involvement of neurorehabilitation team must start as early as acute
medical unit or Intensive Care stage. The role of the team at this stage
is to prevent complications such as tissue shortening and pressure ulcers.
Early swallow assessment by speech and language therapist is necessary to
reduce the risk of chest infections. Maintenance of joint position and
joint range of motion is essential for subsequent optimized functional
outcome. Early commencement of sitting regime helps orthostatic
retraining. Effective continence management regime must also be
established. A well integrated multi-disciplinary rehabilitation team is
vital for achieving early and thereafter long-term functional goals and
improved out come for patients with Guillain-Barre syndrome.
We understand that review articles are widely utilized by trainees to
update themselves on current trends. It is therefore essential that such
articles are comprehensive and contain all relevant clinical,
pathophysiological, therapeutic and current management strategies.
Rehabilitation forms an essential component of treatment in patients with
Guillain-Barre and it must be included as part of management strategy in
clinical reviews.
1. Winer JB, BMJ 2008;337:a671
2. Carroll A; McDonnell G; Barnes M, International Journal of
Rehabilitation Research. 26(4):297-302, December 2003.
Competing interests:
None declared
Competing interests: No competing interests
The neuro-pathology of "Demyelination" of Guillane-Barre syndrome is
shared with Neuromyelitis Optica, Multiple Sclerosis, Motor Neurone
Disease, Idiopathic Paroxysmal Vertigo and Herpes Zoster, together with
other entitled syndromes.
Vitamin B.12 (cyanocobalamin and hydroxocobalamin) has a known
effectiveness in Herpes Zoster when given early. This has been known since
1944. It is certainly ineffective after three months and perhaps less.
However, in many of these syndromes, by the time the ultimate dread
diagnosis is made since it takes time before a Consultant's opinion is
eventually obtained, it is very possible that Vitamin B.12 would then be
quite ineffective.
Cyanocobalamin is essential for the formation of Myelin: OVESEN, L.
"DRUGS" 27 148 (1984). Martindale's Pharmacopoeia.
A series of cases has been reviewed and there is evident instant
relief from pain when Vitamin B.12 is given early to cases of Zoster and
there is no Post Herpetic Neuralgia.
In the suspected cases of these other demyelination syndromes, why
not then adminster early intramuscular injections of B.12? Perhaps they
have been tried, but perhaps too late to have any beneficial effect?
Competing interests:
None declared
Competing interests: No competing interests
Winer’s clinical review on Guillain-Barré syndrome (GBS) deals with
current views and clinical practice of a rare disorder and rightly
emphasizes on recognition of its rapid progression to avoid high mortality
due to respiratory failure (1). We find the spectrum of GBS interesting
and thought provoking and wonder why an important group of patients,
children were missed in this review. This would merit special mention, for
number of reasons. GBS in children differs in many ways compare to adults.
It is an acute, immune-mediated flaccid paralysis frequently associated
with Campylobacter infection. Of two predominant GBS subtypes (three in
adults), a demyelinating subtype (acute inflammatory demyelinative
polyneuropathy [AIDP]) predominates in the United States and Europe, and
axonal subtype (acute motor axonal neuropathy [AMAN]) is the predominant
form in China. I t is considered relatively benign condition in children
in comparison with adults (2).
Similar to adult pattern, boys appear to be at greater risk for GBS
than girls. The average age is in the range of 4-8 years. Individuals
affected with GBS can be as young as 1 year. Estimates of annual incidence
of GBS range from 0.5-1.5 per 100,000 in individuals younger than 18
years. Full recovery within 3-12 months is experienced by 90-95% of
pediatric patients with GBS. Overall mortality rate in childhood GBS is
much less then adults and is estimated to be less than 5% (2). Myasthenia
Gravis, Lyme disease and HIV are among other differential diagnosis in
children apart from those described by Winer (3). Treatment is generally
same for children and adults. Contact a family, is an excellent resource
for information, for parents and carers (4).
References:
1. Winer JB. Guillain-Barré syndrome. BMJ 2008;337:a671
2. Nachamkin I, Barbosa PA, Ung H, Lobato C, Rivera AG, Rodriguez P.
Patterns of Guillain-Barre syndrome in children: results from a Mexican
population. Neurology. Oct 23 2007;69(17):1665-71.
3. Tseng BS. Guillain-Barre Syndrome in Childhood. Emedicine. Jan 2008.
4. Medical texts in The Contact a Family Directory, can be accesses
through, http://www.cafamily.org.uk.
Imran Mushtaq, Associate Specialist-Child & Adolescent
Psychiatrist, Milton Keynes SP-CAHMS, Eaglestone Centre, Standing Way,
Milton Keynes MK6 5AZ
Nasim Mehmood, Consultant Paediatrician, Royal Liverpool Children’s
NHS Trust Eaton Road Liverpool L12 2AP
Competing interests:
None declared
Competing interests: No competing interests
This was a helpful article. However, (as with all diagnostic tests)
it would be useful to know the sensitivity and specificity of a raised
protein in the CSF. Also, is there evidence (ideally from a Randomised
Controlled Trial) that performing a lumbar puncture in these patients
improves the outcome for patients? I ask this, because Lumbar Puncture can
be a painful procedure for patients and in some patients causes
significant morbidity (e.g. post-lumbar puncture headache).
Competing interests:
None declared
Competing interests: No competing interests
Dear Editor,
Winer overlooks an important differential diagnosis in the clinical
review on Guillain-Barré syndrome1. Cauda equina compression must be
considered in all patients presenting with lower limb areflexic paralysis.
Traditionally, back pain has been used to differentiate between the two
diagnoses. However it is not a reliable discriminator. Back pain is a
recognised feature of Guillain-Barré syndrome2,3. Both share a similar age
distribution. A diagnostic delay can result in significant morbidity in
cases of cauda equina compression. There is a temporal window of 48 hours
within which any surgical intervention should be performed; after which
permanent neurological deficit is likely4.
If any suspicion exists urgent magnetic resonance imaging should be
performed. MRI can have diagnostic utility in Guillain-Barré syndrome.
Enhancement of the cauda equina and conus medullaris with gadolinium
administration on T1 weighted images has been consistently demonstrated in
cases of Guillain-Barré5. MRI should be used to exclude cauda equina
compression and support the diagnosis of Guillain-Barré.
1. Winer JB, Guillain-Barré syndrome. BMJ 2008; 337:671
2. Clague JE, Macmillan RR, Backache and the Guillain-Barré syndrome: a
diagnostic problem. Br Med J 1986; 293:325-326
3. Ropper AH, Shanani BT. Pain in Guillain-Barré syndrome. Arch Neurol
1984; 41:511-515.
4. Ahn UM, Ahn NU, Buchowski JM, Garrett ES, Sieber AN, Kostuik JPCauda
equina syndrome secondary to lumbar disc herniation: meta-analysis of
surgical outcomes. Spine 2000; 25:1515-1522.
5. Garson KC, Ropper AH, Muriello MA, Blair R, Prospective evaluation of
MRI lumbosacral nerve root enhancement in acute Guillain-Barré syndrome.
Neurology 1996; 47: 813-817
Competing interests:
None declared
Competing interests: No competing interests
Guillain Barré Syndrome disease requires an effective and appropriate diagnosis and treatment.
We read your Clinical Review
about Guillain Barré Syndrome and we consider it very important to the
medical knowledge due to its low incidence in the daily life medical
practice, but with a high seriousness which requires an effective and
appropriate diagnosis and treatment.
In some studies done about this disease in the last ten years in our
Trinidad City with a population of 70283 people, the incidence was 15
cases with the predominance of female sex and young people, in the 65% of
these cases their ages were between 6 and 12 years old.
The infection of the respiratory tract preceded the appearance of this
disease mainly by cytomegalovirus.
In our study did not exist recurrence of this disease.
The clinical spectrum was of an acute inflammatory demyelinating
polyradiculoneuropathy with cramp, muscular pains in the limbs, non
reflex, sings of dysfunction of the nervous system such as a persistent
tachycardia, orthostatic hypotension, urinary retention, ataxic walk.
The dissociation albuminocytologic occur in the study of the cerebrospinal
fluid.
There were some diseases such as polymyositis, metabolic and toxic
polineuropathy, which offer confusion in the diagnosis.
The seriousness of the disease was higher in adult patient with
comorbility of disease.
The 100 % of patients were seen in Intensive Care Units where a 45%
received control ventilation, everyone used Immunoglobulin EV (Intaglobin)
regimen of 400 mg/kg body weight each day for 5 consecutive day, plasma
exchange in 5 sessions the first 15 days, vitamin B1-B6.
The subcutaneous Heparin was used in the prophylaxis of the pulmonary
embolism; a patient presented an acute vein thrombosis.
The respiratory infection was a complication in the 34 % of the ventilated
patients, who used antibiotics according to the established protocols.
In the 10 % of the patients there were motor and sensitive squealers
besides there were disorders to walk which have become permanent in two
cases in spite of the rehabilitation.
There was not any a dead man or woman in our study.
Competing interests:
None declared
Competing interests: No competing interests