Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Infectious Diseases
Volume 2011, Article ID 972096, 4 pages
http://dx.doi.org/10.1155/2011/972096
Case Report

Guillain-Barré Syndrome with Fatal Outcome during HIV-1-Seroconversion: A Case Report

Department of Infectious Diseases, Galliera Hospital, Via Volta, 16128 Genoa, Italy

Received 7 May 2011; Accepted 21 June 2011

Academic Editors: M. Murhekar and W. I. van der Meijden

Copyright © 2011 Emanuele Pontali et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Guillain-Barré syndrome (GBS) is an acute or subacute peripheral polyneuropathy characterized by symmetrical muscle weakness. Its occurrence has been reported during acute HIV seroconversion since 1985. Among HIV-infected subjects, GBS has generally a favourable outcome. We report a case of GBS with fatal outcome during HIV seroconversion.

1. Introduction

Guillain-Barré syndrome (GBS) is an acute or subacute peripheral polyneuropathy characterized by symmetrical muscle weakness occurring in the absence of identifiable causes of genetic, metabolic, or toxic origin. The characteristic presentation of the syndrome involves symmetrical paresis or paresthesia of an ascending nature, with reduction or loss of deep reflexes and variable autonomic dysfunction. Signs and symptoms observed in the course of GBS are extremely variable. The observed dysfunctions can be motor, sensory, or autonomic (Table 1) [1, 2]. Diagnostic approach to GBS is based on clinical, laboratory, and electrophysiological criteria (Table 1). Progressive motor weakness and areflexia are prime requirements for diagnosis. Cerebrospinal fluid analysis is the only laboratory criterion. Guillain-Barré syndrome onset is associated with a history of infection, mainly of viral origin. Many patients with GBS describe a febrile illness followed by an ascending paralysis occurring days to weeks later. Actually, symptoms are preceded by an antecedent event in about two thirds of cases. Respiratory infections are the commonest, reported in about 40% of cases within one month before the onset of the disease. About 20% of patients experience gastroenteritis as the antecedent cause [1]. Since 1985, several cases of the disease among HIV-infected patients have been reported, with GBS occurring concomitantly with HIV seroconversion or during the initial phases of infection [310]. In fact, HIV infection is one of the most common causes of this syndrome in African countries, India, and other developing countries. In addition, cases of GBS associated with the immunological reconstitution induced by the use of antiretroviral therapy have also been reported [11, 12]. GBS has frequently a favourable outcome in HIV-infected subjects [312].

tab1
Table 1: Characteristics clinical features of Guillain-Barré syndrome and recommended diagnostic investigations.

To our knowledge, we report the first case of GBS with fatal outcome during HIV seroconversion.

2. Case Presentation

A 56-year-old man who had already been observed at our department for acute B (1988) and A (2000) hepatitis was evaluated at our Outpatient Unit (OPD). His medical history included viral encephalitis at age 6, chronic inflammatory bowel disease (treated with mesalazine), and past surgery for discal hernia. Homosexual intercourse with risk for HIV transmission was reported. He had tested HIV-negative about six-months before.

When presenting at our OPD, the patient reported the occurrence of fever (up to 38°C) and diffused maculo-papular rash about one month earlier. At that time, headache and intense pulsatory pain at shoulders had also been present for a few days. Blood tests showing evidence of probable acute viral infection had also been performed. During the three days before presentation at our OPD, he had been showing progressive asthenia, with increasing weakness at all limbs, dysarthria, and dysphagia. He was immediately hospitalised.

At entry he was confused, with amimic face, bilateral facial palsy (prevalent on the right), all limbs weakness (right side more than left), generalized hyporeflexia or areflexia at all limbs, anhysocoria (left > right), and heart rate 90 bpm, with blood pressure (BP) ascending in a couple of hours from 110/70 to 170/100. Skin rash was not anymore visible.

Among blood tests performed there was a mild increase in liver enzymes (complete results in Table 2). Antibodies for HIV-1 were detected, and confirmation test was positive. CD4+ lymphocyte count was 710/mm3 (45%), and viral load was 4,400 copies of HIV-RNA/mL.

tab2
Table 2: Results of diagnostic investigations during hospitalization.

One day after hospitalisation MRI scan of brain and truncus showed no specific signs (Table 2). The neurologist consultant then recommended lumbar puncture in the suspect of GBS. Lumbar puncture was performed on the third day. CSF examination showed 3 cells/mm3 and increased protein level (291 mg/dL), a pattern compatible with GBS [1]. Electroencephalogram was normal. BP was irregular, but gradually under control with antihypertensive drugs. Electrophysiological study was scheduled.

On the fifth day, the patient presented a worsening of limb weakness (both proximal and distal); anhysocoria was not anymore visible. He was started on intravenous immunoglobulins (IVIG) (400 mg/kg/day). Three days later he suddenly died because of cardiac arrest; prolonged resuscitation procedures were ineffective.

3. Discussion

The mechanisms proposed for GBS in HIV-1-infected patients include a direct action of HIV-1 on the nerves by neurotropic strains, or autoimmune mechanisms, with the formation of antibodies against myelin secondary to the abnormal immunoregulation determined by HIV infection [13]. The onset of GBS in this case coincided with the acute retroviral syndrome of HIV, during the phase of initial immune reconstitution following serologic conversion, as observed in other cases reported in the literature [6, 7]. Although GBS in HIV-infected subjects has generally a favourable outcome, rapid worsening of clinical conditions such as respiratory failure has been reported [7].

Both plasmapheresis and IVIG have been used to treat GBS in HIV-infected individuals [1416]. The reported results have ranged from ineffective and mild benefit to good response [6, 14]. Nevertheless, IVIG currently remains the preferred choice in treating GBS. IVIG is preferred to plasmapheresis because of greater convenience, with overall comparable cost and equal efficacy [1, 17].

Twenty-five percent of all GBS patients require ventilator support of some type, and many of them require access to ICU [1]. Nevertheless, outcomes after ICU care were reported to be similar in HIV-positive and HIV-negative patients with GBS [18].

Unfortunately, in our case the rapid fatal evolution did not allow for ICU support. Cases with autonomic disturbances have been reported with similar evolution. Many deaths due to GBS are in fact attributed to autonomic disturbances; cardiac arrest being the most common cause, responsible for 20–30% of deaths [2]. Approximately 25% of deaths occur within the first week of onset of GBS.

Some conclusions can be drawn from this case. First, it is necessary to consider the possibility of GBS when patients are diagnosed with a symptomatic acute HIV syndrome. Patients could be advised to promptly report any neurological symptom or sign suggestive of GBS. This would allow for early diagnosis and proper timely management, including treatment and support. Second, the opposite is also true: in cases of GBS, HIV infection should be considered and searched. Last, but not less important, HIV-infected patients with GBS should be accurately monitored, because serious complications and fatal outcome of the disease are possible as in the HIV-uninfected.

References

  1. U. Seneviratne, “Guillain-Barré syndrome,” Postgraduate Medical Journal, vol. 76, no. 902, pp. 774–782, 2000. View at Publisher · View at Google Scholar · View at Scopus
  2. K. McClellan, E. Armeau, and T. Parish, “Recognizing Guillain-Barré syndrome in the primary care setting,” The Internet Journal of Allied Health Sciences and Practice, vol. 5, no. 1, 2007. View at Google Scholar
  3. L. Hagberg, B. E. Malmvall, L. Svennerholm, K. Alestig, and G. Norkrans, “Guillain-Barré syndrome as an early manifestation of HIV central nervous system infection,” Scandinavian Journal of Infectious Diseases, vol. 18, no. 6, pp. 591–592, 1986. View at Google Scholar · View at Scopus
  4. C. A. Thornton, A. S. Latif, and J. C. Emmanuel, “Guillain-Barré syndrome associated with human immunodeficiency virus infection in Zimbabwe,” Neurology, vol. 41, no. 6, pp. 812–815, 1991. View at Google Scholar · View at Scopus
  5. T. H. Brannagan III and Y. Zhou, “HIV-associated Guillain-Barré syndrome,” Journal of the Neurological Sciences, vol. 208, no. 1-2, pp. 39–42, 2003. View at Publisher · View at Google Scholar · View at Scopus
  6. S. Kumar, M. Alexander, V. Markandeyulu, and C. Gnanamuthu, “Guillain-Barré syndrome presenting in the anti-HIV seroconversion period,” Neurology India, vol. 51, no. 4, p. 559, 2003. View at Google Scholar · View at Scopus
  7. S. H. Ting, J. H. Yeh, and H. C. Chiu, “Guillain-Barré syndrome with HIV infection: a case report,” Acta Neurologica Taiwanica, vol. 12, no. 3, pp. 139–142, 2003. View at Google Scholar · View at Scopus
  8. A. Millogo, A. Sawadogo, D. Lankoande, and A. B. Sawadogo, “Guillain-Barré syndrome in HIV-infected patients at Bobo-Dioulasso Hospital (Burkina Faso),” Revue Neurologique, vol. 160, no. 5, pp. 559–562, 2004. View at Google Scholar
  9. H. K. Aggarwal, D. Chakrabarti, N. Nand, Sonia, K. Bharti, and R. P. Verma, “HIV infection presenting as Guillain-Barré syndrome,” Journal, Indian Academy of Clinical Medicine, vol. 6, no. 4, pp. 341–342, 2005. View at Google Scholar · View at Scopus
  10. G. de Castro, P. G. Bastos, R. Martinez, and J. F. de Castro Figueiredo, “Episodes of Guillain-Barré syndrome associated with the acute phase of HIV-1 infection and with recurrence of viremia,” Arquivos de Neuro-Psiquiatria, vol. 64, no. 3, pp. 606–608, 2006. View at Google Scholar · View at Scopus
  11. P. Makela, L. Howe, S. Glover, I. Ferguson, A. Pinto, and M. Gompels, “Recurrent Guillain-Barré syndrome as a complication of immune reconstitution in HIV,” Journal of Infection, vol. 44, no. 1, pp. 47–49, 2002. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  12. P. J. Piliero, D. G. Fish, S. Preston et al., “Guillain-Barré syndrome associated with immune reconstitution,” Clinical Infectious Diseases, vol. 36, no. 9, pp. e111–e114, 2003. View at Google Scholar · View at Scopus
  13. M. C. Dalakas and G. H. Pezeshkpour, “Neuromuscular diseases associated with human immunodeficiency virus infection,” Annals of Neurology, vol. 23, supplement 1, pp. S38–S48, 1988. View at Google Scholar · View at Scopus
  14. J. R. Berger, J. A. Difini, M. A. Swerdloff, and D. R. Ayyar, “HIV seropositivity in Guillain-Barré syndrome,” Annals of Neurology, vol. 22, no. 3, pp. 393–394, 1987. View at Google Scholar · View at Scopus
  15. R. P. Kleyweg, F. G. A. van der Meche, and J. Meulstee, “Treatment of Guillain-Barré syndrome with high-dose gammaglobulin,” Neurology, vol. 38, no. 10, pp. 1639–1641, 1988. View at Google Scholar · View at Scopus
  16. K. M. Hassan and I. Mathew, “Guillain-Barré syndrome in an HIV seropositive subject,” The Journal of the Association of Physicians of India, vol. 48, no. 12, p. 1214, 2000. View at Google Scholar · View at Scopus
  17. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group, “Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome,” The Lancet, vol. 349, no. 9047, pp. 225–230, 1997. View at Publisher · View at Google Scholar · View at Scopus
  18. G. K. Schleicher, A. Black, A. Mochan, and G. A. Richards, “Effect of human immunodeficiency virus on intensive care unit outcome of patients with Guillain-Barré syndrome,” Critical Care Medicine, vol. 31, no. 6, pp. 1848–1850, 2003. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus