open access

Vol 51, No 5 (2017)
Case reports
Submitted: 2017-04-03
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Autoimmune meningitis and encephalitis in adult-onset still disease – Case report

Milena Bożek, Magdalena Konopko, Teresa Wierzba-Bobrowicz, Grzegorz Witkowski, Grzegorz Makowicz, Halina Sienkiewicz-Jarosz
DOI: 10.1016/j.pjnns.2017.06.006
·
Neurol Neurochir Pol 2017;51(5):421-426.

open access

Vol 51, No 5 (2017)
Case reports
Submitted: 2017-04-03

Abstract

Introduction

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs’ involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis – potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis.

Case presentation

We report a case of 31-year-old male patient, who was referred to neurological department for extending diagnostics of frontal lobes lesions with involvement of adjacent meninges. Abnormalities have been revealed in brain MRI, which was performed due to persistent headaches, visual disturbances, fever, fatigue and cognitive decline. Wide differential diagnosis was performed including laboratory findings, contrast enhanced MRI, MR spectroscopy, flow cytometry and finally brain biopsy to exclude neoplastic or infectious origin. Final diagnosis of autoimmune meningoencephalitis in adult-onset Still disease has been made.

Conclusion

Adult-onset Still disease is a rare cause of inflammatory changes in central nervous system, which if diagnosed, may be treated successfully with steroids (commonly available agent), intravenous immunoglobulins or more specific immunomodulating regiments.

Abstract

Introduction

Adult-onset Still disease (AOSD) is a rare systemic inflammatory disease of unknown cause. Its symptoms usually include persistent fever, fugitive salmon-colored rash, arthritis, sore throat (not specific), but it may also lead to internal organs’ involvement, which presents with enlargement of the liver and spleen, swollen lymph nodes, carditis or pleuritis – potentially life-threatening complications. In rare cases, AOSD can cause aseptic meningitis or/and encephalitis.

Case presentation

We report a case of 31-year-old male patient, who was referred to neurological department for extending diagnostics of frontal lobes lesions with involvement of adjacent meninges. Abnormalities have been revealed in brain MRI, which was performed due to persistent headaches, visual disturbances, fever, fatigue and cognitive decline. Wide differential diagnosis was performed including laboratory findings, contrast enhanced MRI, MR spectroscopy, flow cytometry and finally brain biopsy to exclude neoplastic or infectious origin. Final diagnosis of autoimmune meningoencephalitis in adult-onset Still disease has been made.

Conclusion

Adult-onset Still disease is a rare cause of inflammatory changes in central nervous system, which if diagnosed, may be treated successfully with steroids (commonly available agent), intravenous immunoglobulins or more specific immunomodulating regiments.

Get Citation

Keywords

Still disease, Adult-onset, Meningoencephalitis

About this article
Title

Autoimmune meningitis and encephalitis in adult-onset still disease – Case report

Journal

Neurologia i Neurochirurgia Polska

Issue

Vol 51, No 5 (2017)

Pages

421-426

DOI

10.1016/j.pjnns.2017.06.006

Bibliographic record

Neurol Neurochir Pol 2017;51(5):421-426.

Keywords

Still disease
Adult-onset
Meningoencephalitis

Authors

Milena Bożek
Magdalena Konopko
Teresa Wierzba-Bobrowicz
Grzegorz Witkowski
Grzegorz Makowicz
Halina Sienkiewicz-Jarosz

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