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