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netherlands journal of critical care Copyright ©2006, Nederlandse Vereniging voor Intensive Care. All Rights Reserved. Received June 2006; accepted in revised form September 2006 c a s e r e p o r t Syphilis-associated Guillain- Barré Syndrome M. Hijmering 1 , C. Hoedemaekers 1 , A. Oude Lashof 2 and J. van der Hoeven 1 1 Department of Intensive Care, 2 Department of Internal Medicine Radboud University Hospital Nijmegen, the <strong>Netherlands</strong> Abstract. Guillain-Barré Syndrome (GBS) is the most common cause of acute flaccid paralysis in previously healthy adults. It is strongly associated with several bacterial and viral infections, with C. jejuni, CMV, EBV, HIV, Mycoplasma pneumoniae and H. influenzae being the most common infecting micro-organisms. We present a case in which a previously healthy male developed GBS syndrome that did not respond to the standard treatment with immunoglobulins and plasmapheresis. An infection with treponema pallidum was proven both by serological testing and histological investigations . After treatment for syphilis, the patient made a remarkable recovery. Case Report A 47 year-old, previously healthy Dutch male, was admitted with a three day history of progressive symmetrical weakness in his arms and legs, accompanied by abnormal sensations in his hands and feet. He reported no fever, chills, diarrhoea, or other signs of infection in the weeks prior to admission. He denied IV drug abuse. On examination, the patient had moderate quadriparesis, no deep tendon reflexes, and distal paraesthesia/hypoesthesia in all extremities. A non-tender solitary lymph node was palpated in the supraclavicular region. No other abnormalities were found on general clinical examination. Laboratory investigations revealed a slightly raised sedimentation rate of 49 mm/h and a C-reactive protein concentration of 39 mg/l. The cerebrospinal fluid had normal protein levels but an increased IgG concentration of 54 mg/dl (normal 10-29 mg/dl), without pleiocytosis. Nerve conduction studies and electromyography (EMG) findings identified clear evidence of demyelination with secondary axonal damage compatible with Guillain-Barré Syndrome (GBS). The patient was treated with intravenous immunoglobulin (0.4 g/kg/day) for seven days. Forced vital capacity was lowered and showed a downward trend. On the third day respiratory failure occurred and the patient required intubation and mechanical ventilation. After completion of immunoglobulin treatment the patient underwent plasmapheresis, but neither treatment had any apparent clinical effect. Serological examination revealed no recent infection with Campylobacter, Mycoplasma, Cytomegalovirus, Epstein-Barr virus, hepatitis C virus, influenza virus, para-influenza virus, or Borrelia. Serological testing and PCR for HIV was negative, but VDRL [1:8], TPPA and FTA-ABS testing was positive for syphilis. Histological examination revealed Treponema pallidum in the supraclavicular lymph node (photo). Treponema pallidum antigen was not detected in the cerebrospinal fluid by PCR, VDRL, TPPA and FTA-ABS. Clinical signs of primary or secondary syphilis were not detected. The patient was treated with benzylpenicillin for two weeks, which led to a dramatic clinical improvement, with a return of muscle strength and normal sensibility. The patient was discharged from the intensive care unit five weeks after admission. Correspondence: M. Hijmering E-mail: m.l.hijmering@isala.nl Discussion GBS is the most common cause of acute flaccid paralysis in previously healthy adults, with an average incidence of approximately 1.5 per 100,000 (1). Several subtypes can be defined depending on the clinical and electrophysiological characteristics, with acute demyelinating neuropathy (AIDP), being the most frequent subtype. Although our patient showed clear signs of axonal involvement of both motor and sensory fibres, the phenotype is still compatible with AIDP, because a variable degree of axonal degeneration is universal in severe cases of GBS. The exact cause of the autoimmune reaction that leads to inflammatory demyelination is unknown. The association with numerous infectious diseases suggests that the autoimmune response in GBS may be triggered by molecular mimicry between the infectious organism and peripheral nerve antigens. GBS is strongly associated with several bacterial and viral infections, with C. jejuni, CMV, EBV, HIV, Mycoplasma pneumoniae and H. influenzae being the most common infecting micro-organisms. Although review of the literature Table 1. Results of a Pubmed search based on the search terms “Guillain Barré and infection”. Micro-organisms that were reported in at least 20 patients were qualified as frequent. Frequent reports Anecdotal reports C. jejuni Mycoplasma pneumoniae H. influenzae Helicobacter pylori Herpes virusses HIV West-Nile virus Hepatitis A, B, C virusses M. neisseria Y. enterocolitica S. typhy Chl. pneumoniae Hanta virus Parvo virus B19 Para-infuenza virus Barmah Forest virus Enterovirus 71 Coxsackie B-5 Rocky Mountain spotted fever Japanese encephalitis virus Treponema pallidum Rickettsia conorii Plasmodium falciparum, vivax Toxoplasma Leptospira interrogans Cyclospora Borrelia burgdorferi 534 neth j crit care • volume 10 • no 5 • october 2006
netherlands journal of critical care Figure 1. Spirochetes in a supraclavicular lymph node. (Pubmed search of literature published in English language using Guillain-Barré and infection as search terms) identified numerous case reports of other infections (Table 1), it is uncertain whether these infections had a causative role or represented chance associations. Our patient was diagnosed with syphilis on the basis of serological tests and the detection of Treponema pallidum in a lymph node. We found one case report of syphilis associated with GBS in a 24yearold African-American female diagnosed by positive serological testing [2]. In that case, plasmapheresis alone resulted in clinical and electrophysiological improvement. In contrast, both clinical signs and electrophysiological parameters showed our patient to have deteriorated despite immunoglobulin therapy and plasmapheresis. There was, however, a dramatic improvement on administration of penicillin. This suggests that the immune response in our patient was actively triggered by living micro-organisms. The lymphatic system is the primary reservoir of facultative anaerobic treponemes. Generalized inflammatory lymph node hyperplasia is a characteristic feature of early syphilis, including latent syphilis. In the absence of dermatological and mucosal changes, the lymph nodes may be the only infected tissues accessible for detection of T pallidum .[3]. The incidence of primary and secondary syphilis in Europe and North America is increasing, especially among people with high-risk sexual behaviour [4-6]. In this population there is a high rate of HIV co-infection [7]. Clinicians examining patients who have an infectious process underlying GBS should be aware of the possibility of syphilis infection. In our case, penicillin treatment resulted in rapid clinical improvement and restoration of electrophysiological variables. References 1. Govoni V, Granieri E. Epidemiology of the Guillain-Barre syndrome. Curr Opin Neurol 2001; 14(5):605-613. 2. Weisenberg E, Baron BW. Syphilis-associated Guillain- Barre syndrome: response to plasmapheresis. J Clin Apher 1994; 9(3):200-201. 3. Kouznetsov AV, Prinz JC. Molecular diagnosis of syphilis: the Schaudinn-Hoffmann lymph-node biopsy. Lancet 2002; 360(9330):388-389. 4. Ciesielski CA. Sexually Transmitted Diseases in Men Who Have Sex with Men: An Epidemiologic Review. Curr Infect Dis Rep 2003; 5(2):145-152. 5. Doherty L, Fenton KA, Jones J, Paine TC, Higgins SP, Williams D et al. Syphilis: old problem, new strategy. BMJ 2002; 325(7356):153-156. 6. Golden MR, Marra CM, Holmes KK. Update on syphilis: resurgence of an old problem. JAMA 2003; 290(11):1510- 1514. 7. Lynn WA, Lightman S. Syphilis and HIV: a dangerous combination. Lancet Infect Dis 2004; 4(7):456-466. neth j crit care • volume 10 • no 5 • october 2006 535
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