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1.? incidence 1-2/100,000 per year

2.? the most common cause of acute weakness with areflexia after cord compression and tick
paralysis are ruled out
3.? Peak ages: teens and adults > 55 yo
4.? Male:Female = 1.25:1

0 0

 c

GBS is an autoimmune demyelinating process whereby viral infection predisposes a body to

make autoAbs that attack various components of peripheral myelin sheath (gangliosides)

0


There are multifocal areas of demylelination and inflammatory changes in peripheral nerves

 


1.? Antecedent illness within previous 4weeks

a. URI

b. GI infection especially Campylobacter jejuni (more severe disease)

c. EBV

d. CMV

2.? Weaknessmost often in the legs, symmetric, proximal and distal, progressive in
severity, most patients reach a nadir by 3 weeks; about 1/3 patients become bed-bound,
1/3 require ventilatory support secondary to respiratory muscle weakness.
3.? Cranial nerve weakness- symmetric, tongue most common
4.? Tendon reflex losscommon early symptom progressive loss in 1st week, starts with
ankles, progresses proximally
5.? Pain and paresthesiaspain usually in back, hips, legs
6.? Autonomic dysfunctiontransient hypertension or hypotension, sinus tachycardia,
urinary retention, ileus.
7.? Mild sensory loss including vibratory and propioception

8. Miller-Fisher variant of GBS-Severe form of GBS

a. Clinical features:
i. Acute onset of areflexia, ataxia, ophthalmoplegia,




1.? CSF: Albumino-cytologic dissociation( i.e. high protein but normal cells)
2.? Nerve conduction test- slowing

 

1. Progressive motor weakness involving multiple limbs

2. Areflexia

3. Onset with pain in limb

4. Cranial nerve involvement

5. Autonomic dysfunction

6. Elevated CSF protein

7. Abnormal electrodiagnosis

  


1. Supportive care

2. Plasma exchange

3. IVIG

0

1. 3 % mortality secondary to autonomic disturbances and respiratory failure

2. 80% complete recovery in 12 months

3. Some patients have residual neurologic deficits, but few unable to perform normal daily tasks



1.? Asbury, AK New concepts in Guillain-Barre syndrome. Journal of Child Neurology.

2000 Mar; 15(3):183-91.
2.? Evans, OB, Vedanarayanan V. Guillain-Barre syndrome. Pediatrics in Review. 1997
Jan;18(1):10-6.
3.? Fenichel, G. Clinical Pediatric Neurology, 4th ed. WB Saunders Co., 2001.
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c ! 


  

Chun-Liang Pan1, To-Jung Tseng2, Yea-Huey Lin1, Ming-Chang Chiang1, Whei-Min Lin2 and
Sung-Tsang Hsieh1,2
1
Department of Neurology, National Taiwan University Hospital and 2 Department of Anatomy and Cell Biology,
National Taiwan University College of Medicine, Taipei, Taiwan

Correspondence to: Dr Sung-Tsang Hsieh, Department of Anatomy and Cell Biology, National Taiwan University
College of Medicine, 1 Jen-Ai Rd, Sec. 1, Taipei 10018, Taiwan E-mail: sthsieh@ha.mc.ntu.edu.tw

Guillain±Barré syndrome (GBS) is traditionally considered to be a large-fibre neuropathy.

However, the presence of hypo-aesthesia, dysaesthesia and dysautonomia in GBS patients raises
the possibility that small-diameter sensory and autonomic nerves may also be affected. To
investigate small-fibre neuropathy in GBS, we performed a skin biopsy from the distal leg of 20
patients with the demyelinating form of GBS. Skin sections were immunohistochemically stained
with antiserum against protein gene product 9.5 (PGP 9.5), a ubiquitin C-terminal hydrolase.
Cutaneous innervation was evaluated by measuring epidermal nerve density (END), and END
was further correlated with various clinical and electrophysiological parameters. In GBS patients,
END values were much lower than in age- and gender-matched control subjects (5.03 ± 1.18
versus 10.16 ± 0.87 fibres/mm, 0 < 0.001). Eleven patients (55%) had reduced epidermal
innervation with pathological evidence of active nerve degeneration in the dermis: fragmentation
of subepidermal nerve plexuses and a beaded appearance of dermal nerves. GBS patients had
significantly elevated thermal thresholds with higher warm threshold temperatures (44.54 ± 1.04
versus 39.00 ± 0.35°C, 0 < 0.001) and lower cold threshold temperatures (25.57 ± 1.11 versus
29.05 ± 0.21°C, 0 = 0.032). Reduced END values were associated with an elevated warm
threshold (0 = 0.027), ventilatory distress (0 = 0.037) and dysautonomia (0 = 0.001). END
values were negatively correlated with disability grade on a scale of 1±6 (slope ±0.134 ± 0.038, 0
= 0.0018). Patients with reduced END values tended to have a slower recovery than those with
normal END values (0 = 0.013, median time 12 versus 2 weeks). Patho logically, sudomotor
innervation of the skin was reduced in five of 17 (29.4%) GBS patients in whom sweat glands
could be recognized. These findings suggest that small-fibre sensory and autonomic neuropathies
exist in a significant proportion of GBS patients, and that END values are correlated with
functional disabilities. In summary, GBS should be considered a global neuropathy instead of a
pure large-fibre neuropathy.

?
INTRODUCTION

The acute immune-mediated polyneuropathies are classified under the eponym Guillain-Barré
syndrome (GBS), after the authors of early descriptions of the disease. GBS is an acute
monophasic paralyzing illness, usually provoked by a preceding infection. GBS occurs world-
wide with an incidence of 1 to 2 per 100,000 per year. All age groups are affected, with peaks in
young adults and the elderly [1].

Historically, the Guillain-Barré syndrome (GBS) was considered a single disorder. It now is
recognized as a heterogeneous syndrome with several variant forms. The major forms are acute
inflammatory demyelinating polyradiculoneuropathy (AIDP), the Miller Fisher syndrome
(MFS), acute motor axonal neuropathy (AMAN), and acute sensorimotor axonal neuropathy
(AMSAN). Each form of GBS has distinguishing clinical, pathophysiologic, and pathologic
features. (See "Clinical features and diagnosis of Guillain-Barré syndrome in adults".)

The pathogenesis of GBS will be reviewed here. Other aspects of GBS are discussed separately.
(See "Clinical features and diagnosis of Guillain-Barré syndrome in adults" and "Treatment and
prognosis of Guillain-Barré syndrome in adults".)

MECHANISMS

The proposed mechanism for Guillain-Barré syndrome (GBS) is that an antecedent infection
evokes an immune response, which in turn cross-reacts with peripheral nerve components
because of the sharing of cross-reactive epitopes (molecular mimicry) [2]. The end result is an
acute polyneuropathy. This immune response can be directed towards the myelin or the axon of
peripheral nerve.

Immune reactions directed against epitopes in Schwann cell surface membrane or myelin can
cause acute inflammatory demyelinating neuropathy (AIDP) [2]. The pathology is that of
multifocal inflammatory demyelination starting at the level of the nerve roots. The earliest
changes are frequently seen at the nodes of Ranvier. Both the cellular and humoral immune
responses participate in the process. Invasion by activated T-cells is followed by macrophage-
mediated demyelination with evidence of complement and immunoglobulin deposition on
myelin and Schwann cells [3-5]. No specific myelin antigen(s) have been identified.

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