GUILLAIN-BARRÉ SYNDROME AFTER
VARICELLA-ZOSTER INFECTION
CASE REPORT
WALTER OLESCHKO ARRUDA* LUIS R. C. AGUIAR**
PAULO R. MIRANDA SANDOVAL***
In 1892, Sir William Osier noted an a s s o c i a t i o n between an acute febrile infectious illness and the later d e v e l o p m e n t o f facial diplegia and limb w e a k n e s s
in s o m e p a t i e n t s1 0
. Indeed 5 0 to 7 0 % of Guillain-Barre s y n d r o m e ( G B S ) in most series d e s c r i b e antecedent "viral" s y n d r o m e s involving mainly the
res-piratory tract which p r e c e d e s the s y m p t o m s o f polyneuritis b y o n e to three w e e k s , and a w i d e variety o f specific viral entities p r e c e d i n g G B S has been
d e s c r i b e d1 9
. G B S f o l l o w i n g c h i c k e n p o x has been s e l d o m reported in the literature.
T h e authors d e s c r i b e a case o f severe G B S after a p r o d r o m a l varicella infection and make s o m e c o n s i d e r a t i o n s a b o u t this u n c o m m o n c o m p l i c a t i o n in
a relatively benign exanthematic disease.
CASE REPORT
J.B., a 13 years-old young boy was admitted at 30th October 1986 complaining of progressive ascending paraparesis already involving the upper extremities and with dyspnea. Symptoms began for 4 diays, and 11 days after a clinical picture of fever, malaise and vesicular skin eruptions compatible with chickenpox. At the time of admission he also complained of dysarthria and dysphagia, burning paresthesias of the lower members, sometimes painful, and diplopia. On examination the patient was anxious and dyspneic, he could not move his legs neither deglute his own salliva without great difficulty. There was a grade 3 paresis of the upper members, with global arreflexia and hypotonia. It was observed a superficial and profound anesthesia of the lower extremities, without a well-delimitated upper level and the patient refered diffuse pain on his back irradiating bilaterally to the legs when he was passively moved. Painful dysesthesias improved well with the use of carbamaze-pine 600mg/day. It was noted a bilateral abducens paresis, bilateral peripheric facial palsy, with other cranial nerves normal. There were no fundoscopic alterations. On November 3rd his respiration worsened and he was entubated and connected to the respirator. A tracheostomy was performed at November 6th. During the first 3 weeks we observed the occurrence of episodic tachycardia, profuse sweet and sallivation, and hypertension, which was controlled with the use of propranolol. No major arrythmias did develop. An electrocardiogram was normal. A trial with dexamethasone
was made for 4 days after what it was suspended as there was no response. Four days after being ventilated he developed a picture of herpes labialis which subsided in three days. He was mantained on mechanical ventilation until November 25th, when he ressumed voluntary respiration without dyspnea, after a period of weaning off the respirator with intermitent mandatory ventilation (IMV). Hypertensive crisis subsided and antihypertensive drugs were withdrawn. At this time he was already tetraplegic, but could already deglute and talk with little difficulty. Bilateral facial and abducens paresis was still present. He was discharged on December 3rd, when he was bedridden and tetraparetic. He did not return for follow-up. The following laboratory tests in the serum were normal: aspartate and alanine aminotrasferases, total bilirrubin, alkaline phosphatase, creatine phosphokinase, lactate dehydrogenase, sodium, potassium, urea, creatinine, glucose. The hemogram at admission and during
GUILLAIN-BARRÉ SYNDROME 431
the hospitalization disclosed only leukocytosis with bastonetosis. Doehle corpuscles, without atypical lymphocytes nor anemia. A CT scan was normal. A CSF tap showed 27 red cells, 3 white cells (100% mononuclear), protein 92,0 mg/dl, glucose S4 mg/dl, chloride 118,6 mEq./l. Feces diluted to 15% were cultivated in cell cultures (VERO, Hep 2-C and RD) for isolation of enterovirus (polio, Coxsackie, Echo). No virus were isolated (Dr. Miguel Angez Rodriguez, Setor de Vírus, Laboratório de Pesquisas Biológicas, Fundação de Saúde Caetano Munhoz da Rocha, Curitiba, per-formed the virus culture). The serological tests results are depicted in table 1.
T h e Guillain-Barre s y n d r o m e is an acute o r subacute, relatively symmetric
a s c e n d i n g m o t o r paralysis usually b e g i n n i n g in the l o w e r m e m b e r s from w h i c h
greater than 85 p e r c e n t o f patients obtain a full o r functional r e c o v e r y2
^8
.
T h e d i a g n o s i s is descriptive and rests u p o n r e c o g n i t i o n o f the clinical picture plus other features including elevated c e r e b r o s p i n a l fluid ( C S F ) protein level
( w h i c h can b e normal at the b e g i n n i n g o f the d i s e a s e ) plus light C S F p l e o c y t o s i s o r normal C S F white cell count, e l e c t r o p h y s i o l o g i c a l c h a n g e s ( m a r k e d s l o w i n g
of c o n d u c t i o n velocities, p r o l o n g e d distal latencies, c o n d u c t i o n b l o c k ) and p a -t h o l o g i c a l c h a n g e s ( l o w - g r a d e inflamma-tion and demyelina-tion-remyelina-tion of
the peripheral n e r v e s )1 9
. T h e p r e s e n c e o f h i g h - g r a d e fever at the onset o f the neuritic s y m p t o m s , transient b l a d d e r p a r a l y s i s and severe s e n s o r y l o s s with
pain m a y cause s o m e confusion for the d i a g n o s i s o f G B S1 5
.
Table 1 — Case. Serological tests: *, complement fixation; **, immunofluorescence; soroneutralization (paired samples with 20 days interval); ****, radioimmu-noassay; OMV, cytomegalovirus; EBV, Epstein-Barr virus; VDRL, Veneml Disease Reference Laboratory.
In 3 t o 5 per cent o f patients with G B S the p o l y r a d i c u l o n e u r o p a t h y b e c o m e s c h r o n i c o r recurrent. A n increased p r e v a l e n c e o f human l e u k o c y t e antigen ( H L A )
histocompatibility t y p e s A w 3 0 , A w 3 1 , B 8 D w 3 A and g l y o x a l a s e 1 is f o u n d in
these p a t i e n t s2 7
, w h a t is n o t o b s e r v e d in patients with acute m o n o p h a s i c G B S1
'2 7
.
A great variety o f specific infectious agents have b e e n implicated as causative of G B S , either b y direct isolation o r b y rising a n t i b o d y titers. E x a m p l e s o f such
agents include p a r a influenza A 2 , herpesviruses ( h e r p e s simplex, herpes zoster, c y t o m e g a l o v i r u s , Epstein-Barr v i r u s ) 3,10,2^ E c h o virus, C o x s a c k i e virus,
influ-enza A and B , hepatitis B v i r u s5
, m u m p s2 4
, m e a s l e s1 3
-2 3
, falciparum m a l a r i a1 4
-2 6
,
infectious m o n o n u c l e o s i s1 1
, M y c o p l a s m a p n e u m o n i a e1 6
'1 6
, t u l a r e m i a2 2
, and s y
-philis 2 0
.
Deficits in pain and temperature sensation in a s t o c k i n g - a n d - g l o v e
distribu-tion predict greater residual impairment, since they imply severe axonal injury 18. A u t o n o m i c dysfunction is not u n c o m m o n in G B S and c a r d i a c arrythmia is a
frequent cause o f death in these patients. T h e s e c o n d i t i o n s w e r e well o b s e r v e d in our patient. N e u r o l o g i c c o m p l i c a t i o n s at the time o r f o l l o w i n g p r i m a r y
varicella infection in n o n i m m u n o c o m p r o m i s e d patients are u n c o m m o n . T h e m e
-COMMENTS
Anti-CMV (CF *) Anti-CMV IgG (IF**) Anti-CMV IgM (IF **) Anti-EBV IgG Anti-EBV IgM
Anti-poliovirus PI, P2, P3 (SN ***) VDRL,
Australia antigen (****) Toxoplasmosis IgG (IF **) Toxoplasmosis IgM (IF **)
1:4 0 0
+
0 0 1:2
0 1:6
432 ARQ. NEURO-PSIQUIATRIA (SÃO PAULO) VOL. tf, Nv
h DEZEMBRO, 1987
chanism of nervous system disease caused b y herpesviruses is still controversial 28
and include a direct invasion of the neural p a r e n c h y m a b y the virus. H o w e v e r
the evidence o f viral particles in the central o r peripheral nervous system during disease is lacking. T h a t means that an i m m u n o l o g i c a l p r o c e s s , non-dependent
of the p r e s e n c e o f the virus, m a y b e operative. B o t h the cellular and humoral c o m p o n e n t s of the immune system have been implicated in the mechanism of
demyelination in G B S 7
>1 7
. S o m e viral antigenic determinants w o u l d " t r i g g e r " an abnormal host immunological r e s p o n s e genetically determined ( e g . H L A
s y s t e m ) . A p p a r t the acute polyradiculoneuritis other i m m u n o l o g i c a l l y mediated diseases have been d e s c r i b e d after varicella-zoster infection and include cerebellar
ataxia, o p t i c neuropathy, other cranial nerve palsies 4
and e n c e p h a l o m y e l i t i s2 0
'2 8
. A similar p a t h o g e n e s i s theory has been p r o p o s e d for the nervous system p o s t
-infectious c o m p l i c a t i o n s caused b y measles and rubella virus 20. In 302 c a s e s of G B S studied b y Samantray et al. o n l y o n e ( 0 , 3 % ) w a s related to
chicken-p o x . Indeed there are f e w rechicken-ports in the literature, and from 1873 to 1972
only 20 patients w e r e cited in the w o r l d l i t e r a t u r e3 2
. In recent reports the a g e
of the patients varied from 9 to 32 y e a r s o l d 6,8,0,30 in an 0f them there w a s
complete recuperation and t w o c a s e s8
-9
d e v e l o p e d severe arterial hypertension as o b s e r v e d in our case, w h i c h later subsided c o m p l e t e l y .
One c o u l d postulate that the s u p e r i m p o s e d herpes simplex ( h e r p e s labialis) infection in our c a s e c o u l d lead to a m o r e severe and p r o l o n g e d picture o f
polyradiculoneuritis due to antigenic and ultrastructural similarities between v a
-ricella-zoster virus and herpes simplex v i r u s3 1
.
SUMMARY
T h e authors report a case of Guillain-Barrè s y n d r o m e ( G B S ) f o l l o w i n g a
varicella infection in a 13 y e a r - o l d b o y . D u r i n g his admission he d e v e l o p e d respiratory insuficiency and d y s a u t o n o m i c events, as well as a severe sensitive peripheral neuropathy. S o m e aspects related to the e t i o l o g y and p a t h o g e n e s i s
of G B S are discussed.
RESUMO
Síndrome de Guillain-Barrè após varicela: relato de caso.
O s autores relatam c a s o g r a v e de s í n d r o m e de Guillain-Barré a p ó s q u a d r o
inicial de varicela em rapaz de 13 a n o s . C o m o c o m p l i c a ç õ e s este paciente d e -senvolveu q u a d r i p l e g i a flácida c o m insuficiência respiratória, hipertensão arterial, taquicardia e importante neuropatía sensitiva periférica (disestesia d o l o r o s a ) .
C o m e n t a - s e a r e l a ç ã o entre q u a d r o s virais b e n i g n o s e o d e s e n v o l v i m e n t o de polirradiculoneurite a g u d a , assim c o m o o possível m e c a n i s m o i m u n o p a t o g ê n i c o
e n v o l v e n d o fatores humorais e celulares.
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