Portuguese Version

Year:  2003  Vol. 69   Ed. 4 - (20º)

Relato de Caso

Pages: 566 to 569

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Guillain-Barre syndrome as a complication of acute tonsillitis

Author(s): Suzana B. Cecatto1,
Roberta I. D. Garcia1,
Kátia S. Costa1,
Roberta A B. Novais1,
Ricardo Yoshimura1,
Priscila B. Rapoport2

Keywords: Guillain-Barre Syndrome, acute tonsillitis, complication

Abstract:
The Guillain-Barre Syndrome is an acute or subacute polyradiculoneuropathy that sometimes follows infective illness or surgical procedures. The precise mechanism is unclear, but the disorder probably has an immunological basis. The authors report a case in which acute tonsillitis was complicated with Guillain-Barre Syndrome. We emphasize the simultaneousness of the acute disease and the syndrome, with fast progression to respiratory arrest occuring only in 20% of cases. The treatment was the immunotherapy with prompt recovering of the patient.

Introduction

Acute tonsillitis frequently has viral etiology and in most cases it occurs as part of a cold episode, being normally self-limited. Bacterial acute tonsillitis, in turn, is normally caused by streptococcus Beta hemolytic, being difficult to determine its real incidence by similarity of the clinical picture with viral tonsillitis and by the fact that the swab of oropharynx does not help in the diagnosis.

Serious complications can be associated with bacterial tonsillitis, but in most of the cases they present good evolution, be it by spontaneous resolution or cure after antibiotic therapy 1, 2.

Neurological impairment after acute tonsillitis without formation of an abscess of the parapharyngeal space is extremely rare. In the studied literature, we found one case of Guillain-Barre syndrome in a young patient with quick evolution to respiratory failure.

Guillain-Barre syndrome is defined as an acute inflammatory dimyelinizing polyneuropathy characterized by paresis or paralysis that affects more than one limb, normally symmetrical, associated with loss of tendon reflexes. In only 20% of the cases it can quickly take to respiratory failure. It is the most frequent cause of acute flaccid paralysis after the advent of poliomyelitis vaccine 1, 2, 3.

Literature Review

Complications of acute tonsillitis are normally associated with bacterial etiology and include peritonsillary and parapharyngeal abscesses, suppurative cervical adenitis, acute rheumatic fever and glomerulonephritis. However, after the advance in antibiotic therapy, such complications are getting less frequent. It has more rarely been described as myocarditis, disseminated intravascular coagulation and pyothorax as complications of tonsillitis. There are reports of cases of cerebral abscess and cavernous sinus thrombosis secondary to peritonsillary abscesses 1. Lemierre's syndrome, also known as post-angina sepsis, is characterized by fusobacterial sepsis with multiple systemic metastatic foci after acute tonsillitis in adolescents and young adults. It occurs basically after the formation of internal jugular vein thrombosis and there are many reports in the literature 4-6.

In the study conducted by us, we found only one report of Guillain-Barre syndrome after acute tonsillitis. Morgan and Brookes1 reported three cases of acute or subacute tonsillitis that presented neurological affections without formation of parapharyngeal space abscess. Facial paralysis and hemiplegia, upper sagittal sinus thrombosis and Guillain-Barre syndrome with facial paralysis were the events detected in the patients.

Guillain-Barre syndrome is defined as acute or subacute polyneuropathy and it affects 60% of the cases after an infectious disorder, being that 50% of them have viral etiology. Normally, the viral infection precedes the motor deficiency in 2 to 3 weeks.

In general, it is characterized by being an immune-mediated pathology whose incidence corresponds to 0.38-1.7 cases in 100,000, reaching both genders with male predominance. The age range of top incidence is among young adults (20 to 30 years).

However, in 20% of the cases it can affect children below the age of 10 years and also subjects aged 40 to 70 years 3, 7-9. The main clinical finding is muscle weakness initially in the lower limbs that extends to the upper limbs, face and trunk. Respiratory muscles and swallowing can be affected and autonomous nervous system disorders can also be detected.

Cerebrospinal fluid (CSF) can show protein increase and normal cell count, but only 2 to 3 weeks after disease evolution. The differential diagnosis can be made with neuropathy (diphtheria, intoxication), poliomyelitis, botulism and acute degenerative muscle pathologies 7.

Treatment with corticoids (prednisone) is ineffective and can prolong the period of recovery. Ventilation support in the ICU, plasmapheresis, and intravenous immunoglobulin (400 mg/Kg/day) for 5 days are the advocated treatment despite the current controversy 1-3, 7, 8, 10-12. Most patients present good evolution, which can take months to complete recovery and in 10 to 20% of the patients, there is persistence of some deficiency 7.

CASE REPORT

P.V.S.N., 15-year-old male patient, came to the ER in October 2001 complaining of odynophagia and 39o C fever for one day. The physical examination revealed hypoactivity and at oroscopy there was the presence of white-yellowish exsudate in both tonsils. He was treatment with penicillin benzatin with no significant improvement.

After 48 hours, he still had fever and dysphagia and started to show generalized muscle weakness and paresthesis of the lower limbs. He presented discharge of hemoptoic sputum, claudication and progressive dyspnea, coming to the service of ENT.

The patient was immediately hospitalized and he quickly developed tetraparesis and acute respiratory distress requiring orotracheal intubation with ventilation support in the ICU.

Upon admission at the ICU, he presented Glasgow score 3T, midriatic pupils, 130/90 mmHg blood pressure, heart rate of 92bpm. Oroscopy presented right tonsil with points of necrosis and bleeding, left tonsil with purulent exsudate without the presence of peritonsillary abscess. We started treatment with antibiotic therapy (oxacillin and metronidazol) showing improvement of infectious manifestation 48 hours later, when he regained consciousness. However, the patient still presented tetraparesis and respiratory muscle paralysis. During hospitalization, the analysis of CSF was within the normal range, with negative culture; ASLO = 800 (normal up to 200); CPK = 1,179 (high); inflammatory activity tests were increased; complete blood count with leukocytosis, deviation to the left and platelet reduction; serology for toxoplasmosis and HIV were negative.

After diagnosis of Guillain-Barre syndrome, we started treatment with intravenous immunoglobulin at the dose of 400mg/Kg/day (total of 20g/day) for seven days. In addition, we made a muscle biopsy of the right calf whose result was normal.

Two days after the onset of immunoglobulin administration, he presented complete remission of the respiratory affection. On the seventh day, he was discharged from the ICU after complete recovery of the motor impairment, maintaining only physical therapy exercises for two months.

After hospital discharge, he presented two episodes of non-complicated acute tonsillitis, treated as outpatient care with amoxicillin.

Tonsillectomy was performed in April 2002 and the clinical pathology evidenced nonspecific chronic tonsillectomy with negative immunohistochemistry for Epstein-Barr.

Discussion

The involvement of the central nervous system in acute tonsillitis is normally detected after formation of parapharyngeal space abscess. Normally, the probable mechanism is embolization of the infectious focus via venous and/or lymphatic access or through the internal carotid artery with impairment of cranial nerves (9th and 12th) and the sympathetic chain.

Morgan et al.1 reported difficulty to explain the pathogenesis of neurological complications detected (facial paralysis and hemiplegia, upper sagittal sinus thrombosis with hydrocephalus and papilledema; Guillain-Barre syndrome and facial paralysis) together with absence of abscess. In case of Guillain-Barre syndrome with facial paralysis post-acute tonsillitis, the authors questioned the occurrence of polyneuropathy of possible vital etiology, not confirmed.

Our case showed an infectious process (bacterial acute tonsillitis without abscess) of poor evolution with triggering of Guillain-Barre syndrome in a male adolescent.

As to age (15 years), our patient was not within the age range of highest incidence of the syndrome (20 to 30 years). Moreover, we found only one case described of Guillain-Barre syndrome associated with acute tonsillitis and therefore, we could not defined its real incidence 1.

In the clinical evolution, there is normally paresis of the lower limbs within 3 to 4 weeks after infection and in the case presented here, the period was only of 48 hours. In addition, the patient presented impairment of the respiratory muscles within a short period of time requiring orotracheal intubation and ventilation support.

According to Cornock10, 5% of the cases of Guillain-Barre syndrome are fatal and among them 15 to 30% corresponds to those that require mechanical ventilation. In our case, despite the quick affection of the respiratory muscles, the patient recovered completely within 2 days after using immunoglobulin.
The diagnosis is essentially clinical. However, the analysis of CSF can help. Normally, there is protein elevation (greater than 50mg/100ml) and the number of leukocytes is normal, characterizing protein/cytology dissociation 7, 9. But the exam may be normal in the first week, such as in our patient. The hypothesis of acute myopathy was made for the case and ruled out after negative muscle biopsy.

There is no consensus concerning the best treatment for the syndrome in the literature. It is known that recovery is quicker when immunoglobulin or plasmapheresis are used 8, 10-12. The use of isolate corticoid is not beneficial. However, in the case described here, we decided to use immunoglobulin owing to fewer side effects and easy use, when compared to plasmapheresis. The advocated dose was 400mg/Kg/day for 7 days. Gürses8 conducted a comparative study between patients treated with intravenous immunoglobulin and the control group. He concluded that those that received the medication recovered quickly, were hospitalized shorter and required ventilation support for less time.

According to Hund et al.11, in 15% of the cases there is still some degree of motor or sensorial deficit and in 5% of the cases it is severe.

In most cases, as in our patient, recovery is complete, without sequelae. It is also known that in 3% of the cases there is recurrence of the syndrome and the mechanism is not exactly known yet. Normally, in new episodes the treatment is the same as in the first episode, but the risk of resulting sequelae is increased.

We should bear in mind that physical therapy is necessary to prevent muscle atrophy in prolonged cases.
CLOSING REMARKS

Acute tonsillitis can have unfavorable progression with significant systemic complications. The case described is of scientific interest owing to its rarity and it aims at drawing attention to commonplace diseases that can have unpredictable evolution.

REFERENCES

1. Morgan N, Brokes GB. Central nervous system complications of acute tonsillitis. J Laryngol Otol 1997;111(3):274-6.
2. Yuki N, Koga M, Tai T, Hirata K. Miller Fisher syndrome and Haemophilus influenzae infection. Neurology 2001;57:686-690.
3. Bennett JC, Plum F. Cecil Tratado de Medicina Interna. 20ª Ed. Rio de Janeiro: Guanabara-Koogan; 1997. p. 2372-3.
4. Agarwal R, Arunachalam PS, Bosman DA. Lemierre´s syndrome: a complication of acute oropharyngitis. J Laryngol Otol 2000;114:545-7.
5. Koay CB, Heyworth T, Burden P. Lemierre syndrome - a forgotten complication of acute tonsillitis J Laryngol Otol 1995;109:657-661.
6. Williams A, Nagy M, Wingate J, Bailey L, Wax M. Lemierre syndrome: a complication of acute pharyngitis Int J Pediatr Otorhinolaryngol 1998;45:51-57.
7. Aminoff MJ. In: Tierney LMJ, Mcphee SJ, Papadakis MA. Current Medical Diagnosis e Treatment. 39th Ed. United States: McGraw Hill; 2000. p.959-1018.
8. Gürses N, Vysal S, Çetinkaya F, Islek I, Kalayci AG. Intravenous Immunoglobulin Treatment in children with Guillain-Barre syndrome. Scand J Infect Dis 1995;27:241-43.
9. Marcondes E. Pediatria básica. 8ª ed. São Paulo: Editora Sarvier; 1999. p.1134-5.
10. Cornock MA, Mcmahon-Parkes K. Guillain-Barré syndrome: biological basis, treatment and care. Intensive Crit Care Nurs 1997;13(1):42-8.
11. Hund E, Oborel C, Cornblath D, Hanley D, Mckhann G. ICU management of Guillain-Barre. Critical Care Medicine 1993;21(3):433-46.
12. McFarland HR. What treatment for the Guillain-Barre syndrome? Arch Neurol 1993;50(7):687-8.

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1 Resident physician, Discipline of Otorhinolaryngology, Medical School, ABC.
2 Faculty Professor, Discipline of Otorhinolaryngology, Medical School, ABC.
Affiliation: Discipline of Otorhinolaryngology, Medical School, ABC - Hospital Estadual Santo André - HESA - SP.
Address correspondence to: Suzana Boltes Cecatto - Rua Sao Paulo, 2484 Bairro Barcelona - Sao Caetano do Sul 09541-100
E-mail: suzanacecatto@yahoo.com.br
Study presented at 36º Congresso Brasileiro de Otorrinolaringologia.
Article submitted on December 10, 2002. Article accepted on March 27, 2003.

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