Authors: Zhao, Hong¹; Yuan, Yun²; Li, Yue³; Si, Chong-Wen⁴; Tian, Geng-Shan⁴; Wang, Gui-Qiang⁴; Yang, Xue-Dong⁵

Source: Rheumatology International, Volume 28, Number 12, October 2008 , pp. 1261-1264(4)

Publisher: Springer

Abstract:

Adult-onset Still's disease (AOSD) is a rare, systemic inflammatory disorder, characterized by spiking high fever, fever-associated evanescent rash, arthritis, myalgia, serositis and hepatosplenomegaly. White blood cell count, neutrophilic cell count, and serum ferritin level are markedly elevated in the active stage of the disease. Neurological complications of AOSD commonly were cranial nerve palsies, seizures, aseptic meningoencephalitis, peripheral neuropathy and Miller-Fisher syndrome. We report a previously healthy 60-year-old Chinese man who fulfilled the criteria for AOSD and had a combination of focal and peripheral neurological symptoms. Magnetic resonance angiography (MRA) and transcranial Doppler ultrasonography (TCD) showed narrowness of cerebral blood vessel. Peripheral neuropathy was confirmed by electromyography and sural nerve biopsy. His generalized neuropathy and other symptoms were rapidly improved by receiving glucocorticoid therapy. We do a literature review about neurological manifestations observed in AOSD patients.

Keywords: Adult-onset Still's disease; Narrowness; Neurological manifestation

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s00296-008-0599-3

Affiliations: 1: Department of Infectious Diseases, Peking University First Hospital, 8, Xishiku Street, West District, 100034, Beijing, People's Republic of China, Email: minmin2001@gmail.com 2: Department of Neurology, Peking University First Hospital, 100034, Beijing, China 3: Beijing Di Tan Hospital, Beijing, China 4: Department of Infectious Diseases, Peking University First Hospital, 8, Xishiku Street, West District, 100034, Beijing, People's Republic of China 5: Department of Radiology, Peking University First Hospital, 100034, Beijing, China

Publication date: 2008-10-01

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