Case ReportIsolated sixth nerve palsy as an initial presentation of primary angiitis of the central nervous system
Introduction
Primary angiitis of the central nervous system (PACNS) is an increasingly recognized cause of pediatric inflammatory brain diseases. The diagnosis of childhood PACNS is often based on the Calabrese criteria for adults [1]: 1) newly acquired focal or diffuse neurologic deficits or psychiatric symptoms, 2) angiographic and/or histopathologic features in the central nervous system, and 3) in the absence of an underlying systemic inflammatory process.
Neuroimaging often provides conclusive evidence for PACNS. The most typical angiographic finding of childhood PACNS is unilateral involvement of the terminal segment of the carotid artery and the proximal segments of the anterior and middle cerebral arteries [2]. Whether or not PACNS can be detected by angiography depends on the size of the affected vessel. Such angiography-positive PACNS can result in profound and permanent neurological deficits; therefore, timely identification and immunosuppressive treatment are considered crucial [3]. On the other hand, childhood PACNS can present with a wide variety of neurologic deficits and psychiatric symptoms [4]. Owing to its rarity, the initial decision of neuroimaging can be challenging.
Herein, we describe a girl who initially presented with symptoms of isolated sixth nerve palsy. The swift decision to conduct magnetic resonance angiography (MRA) led to the rapid identification and treatment of PACNS, and thus, the successful resolution of her symptoms.
Section snippets
Case report
A previously healthy 13-year-old girl presented to our hospital with a three-day history of persistent double vision. Her diplopia progressively worsened over the three days, but she did not report any other symptoms. She also did not recall any preceding infection or vaccination within the past few weeks. Her vital signs were as follows: temperature, 36.8 °C; pulse rate, 78 bpm; respiratory rate, 17 bmp; and blood pressure, 94/56 mm Hg. Neuroophthalmological inspection revealed a limitation in
Discussion
We describe a patient with sixth nerve palsy as the initial presentation of PACNS. The presence of vasculitis was identified by MRA and confirmed by subsequent CTA and vessel wall enhancement on DANTE-prepared contrast-enhanced magnetic resonance. Rapid diagnosis and initiation of steroid pulse therapy completely resolved her symptoms, and the dose of steroids could be tapered over a relatively short period.
Childhood PACNS is classified according to the affected vessel size into two types:
Declaration of Competing Interest
The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper.
Acknowledgements
The authors would like to thank the patient and families for their cooperation in this study. The authors would also like to thank Editage (www.editage.com) for English language editing.
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