Pneumococcal-meningitis associated acute disseminated encephalomyelitis (ADEM) – case report of effective early immunotherapy

SpringerPlus, Aug 2014

Introduction Unvaccinated patients with history of splenectomy are prone to fulminant courses of Streptococcus pneumoniae-associated bacterial meningitis. Besides direct brain damage those patients may additionally suffer from parainfectious syndromes, notably vasculitis and acute disseminated encephalomyelitis (ADEM). Differentiation and treatment of these immunological reactions is challenging. Methods Case report. Results A 61 year-old woman with history of splenectomy without vaccination for S. pneumoniae presented with progressive headache and meningism. CSF-analysis revealed pleocytosis with microbiological evidence for pneumococcal meningitis. After unsuspicious initial cranial CT imaging and initiation of appropriate antibiotic therapy, MRI two days later showed widespread FLAIR- and T2-hyperintense white matter lesions that further progressed upon follow-up MRI and that fulfilled imaging criteria of ADEM. Meanwhile the patient deteriorated and required mechanical ventilation. Cranial angiography showed no signs of vasculitis or vasospasms. Screening for autoimmune diseases remained negative, however oligoclonal bands turned positive. Brain biopsy mainly revealed perivascular CD4+ T-cells and demyelinated areas. Despite ongoing acute meningitis, a 10-day corticosteroid-pulse was initiated followed by steroid-tapering. Within 4 weeks, clinical and MRI findings ameliorated. In an one-year follow-up visit, the patient significantly recovered, MRI lesions were markedly reduced and no further relapses occurred. Conclusion Acute pneumococcal meningitis in unvaccinated splenectomized patients may be complicated by a monophasic course of parainfectious ADEM that can be controlled with high-dose corticosteroids. Parainfectious vasculitis or cerebritis are important differential diagnoses and exact differentiation of these entities is important to initiate early appropriate immunotherapy.

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Pneumococcal-meningitis associated acute disseminated encephalomyelitis (ADEM) – case report of effective early immunotherapy

SpringerPlus Pneumococcal-meningitis associated acute disseminated encephalomyelitis (ADEM) - case report of effective early immunotherapy Konstantin Huhn 0 De-Hyung Lee 0 Ralf A Linker 0 Stephan Kloska 1 Hagen B Huttner 0 0 Department of Neurology, University Hospital Erlangen , Schwabachanlage 6, 91054 Erlangen , Germany 1 Department of Neuroradiology, University Hospital Erlangen , Erlangen , Germany Introduction: Unvaccinated patients with history of splenectomy are prone to fulminant courses of Streptococcus pneumoniae-associated bacterial meningitis. Besides direct brain damage those patients may additionally suffer from parainfectious syndromes, notably vasculitis and acute disseminated encephalomyelitis (ADEM). Differentiation and treatment of these immunological reactions is challenging. Results: A 61 year-old woman with history of splenectomy without vaccination for S. pneumoniae presented with progressive headache and meningism. CSF-analysis revealed pleocytosis with microbiological evidence for pneumococcal meningitis. After unsuspicious initial cranial CT imaging and initiation of appropriate antibiotic therapy, MRI two days later showed widespread FLAIR- and T2-hyperintense white matter lesions that further progressed upon follow-up MRI and that fulfilled imaging criteria of ADEM. Meanwhile the patient deteriorated and required mechanical ventilation. Cranial angiography showed no signs of vasculitis or vasospasms. Screening for autoimmune diseases remained negative, however oligoclonal bands turned positive. Brain biopsy mainly revealed perivascular CD4+ T-cells and demyelinated areas. Despite ongoing acute meningitis, a 10-day corticosteroid-pulse was initiated followed by steroid-tapering. Within 4 weeks, clinical and MRI findings ameliorated. In an one-year follow-up visit, the patient significantly recovered, MRI lesions were markedly reduced and no further relapses occurred. Conclusion: Acute pneumococcal meningitis in unvaccinated splenectomized patients may be complicated by a monophasic course of parainfectious ADEM that can be controlled with high-dose corticosteroids. Parainfectious vasculitis or cerebritis are important differential diagnoses and exact differentiation of these entities is important to initiate early appropriate immunotherapy. Acute disseminated encephalomyelitis; ADEM; Bacterial meningitis; Streptococcus pneumonia; Parainfectious disease Introduction Bacterial meningitis in adults is most commonly caused by Streptococcus pneumoniae and often shows a fulminant clinical course (Weisfelt et al. 2006) . Especially patients with a history of splenectomy – without vaccination – are at risk of an accompanying “overwhelming post-splenectomy infection” (OPSI) (Morgan and Tomich 2012) . Besides direct brain damage due to cerebral infection, there is some evidence that patients with bacterial meningitis may additionally suffer from parainfectious inflammatory syndromes such as vasculitis or acute disseminated encephalomyelitis (ADEM) (Ohnishi et al. 2007) , (Beleza et al. 2008; Okada and Yoshioka 2010) which is usually more common in younger patients (Tenembaum et al. 2007) . However, an exact differentiation of these immunological reactions is often challenging. Case report A 61 year-old woman with a history of splenectomy in childhood - without any vaccination for Streptococcus pneumoniae - presented in our emergency room with reduced general condition, subfebrile body temperature, progressive headache, bilateral hypacusis and signs of meningism. Laboratory values for systemic inflammation were markedly elevated (16.900 leukocytes/μl, CRP 420 mg/dl, procalcitonin 7.9 μg/l) and CSF-analysis revealed neutrophil dominated pleocytosis of 98 leukocytes/ μl (2 days later: 5.200 cells/μl) as well as highly elevated lactate (22.9 mmol/l) and protein (5.8 g/l), whereas glucose was barely measurable. Initial cranial CT imaging was normal and calculated initial therapy consisting of ceftriaxon, ampicillin, aciclovir and low dose dexamethasone was started immediately (de Gans and van de Beek 2002) . After microbiological confirmation of Streptococcus pneumonia in CSF and blood cultures antibiotic treatment was switched to penicillin G according to resistance screening. Two days after symptom onset the patient clinically deteriorated, showed a progressive tetraparesis and decreased vigilance requiring mechanical ventilation. MR imaging of the neuroaxis revealed FLAIR- and T2-hyperintense white matter lesions without gadolinium enhancement and no restrictions in diffusion-weighted sequences (Figure 1), whereas spinal cord was unaffected. Despite multidisciplinary investigation no additional source of infection was found. Serological screening for autoimmune-mediated and paraneoplastic diseases including ANA, ANCA, AntidsDNA, IL-2 receptor, ACE, CCP, Anti-Cardiolipin, beta2microglobulin, AMA, Anti-histone, Anti-nucleosome, Anti-PCNA, Anti-centromer B, Anti-Jo/-Hu/-Ri, (...truncated)


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Konstantin Huhn, De-Hyung Lee, Ralf A Linker, Stephan Kloska, Hagen B Huttner. Pneumococcal-meningitis associated acute disseminated encephalomyelitis (ADEM) – case report of effective early immunotherapy, SpringerPlus, 2014, pp. 415, Volume 3, Issue 1, DOI: 10.1186/2193-1801-3-415