Lisa R Sun1, Thierry A G M Huisman2, Anusha K Yeshokumar3, Michael V Johnston4. 1. Division of Pediatric Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland. Electronic address: lsun20@jhmi.edu. 2. Section of Pediatric Neuroradiology, Division of Pediatric Radiology, The Johns Hopkins University School of Medicine, Baltimore, Maryland. 3. Division of Pediatric Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland. 4. Division of Pediatric Neurology, The Johns Hopkins University School of Medicine, Baltimore, Maryland; Kennedy Krieger Institute, Baltimore, Maryland.
Abstract
BACKGROUND: Rocky Mountain spotted fever is a tickborne infection that produces a systemic small-vessel vasculitis; its prognosis is excellent if appropriate treatment is initiated early. Because the advent of effective antirickettsial therapies predates the widespread use of brain magnetic resonance imaging, there are limited data on the effect of untreated Rocky Mountain spotted fever infection on neuroimaging studies. PATIENT DESCRIPTION: We describe a 7-year-old girl with delayed treatment of Rocky Mountain spotted fever who suffered severe neurological impairment. Serial brain magnetic resonance images revealed a progressive "starry sky appearance," which is proposed to result from the same small vessel vasculitis that causes the characteristic skin rash of this infection. CONCLUSION: Neurological injury can continue to occur despite specific antirickettsial therapy in Rocky Mountain spotted fever. This child's clinical features raise questions about the optimal management of this infection, particularly the utility of immune modulating therapies in cases of delayed treatment and neurological involvement.
BACKGROUND: Rocky Mountain spotted fever is a tickborne infection that produces a systemic small-vessel vasculitis; its prognosis is excellent if appropriate treatment is initiated early. Because the advent of effective antirickettsial therapies predates the widespread use of brain magnetic resonance imaging, there are limited data on the effect of untreated Rocky Mountain spotted fever infection on neuroimaging studies. PATIENT DESCRIPTION: We describe a 7-year-old girl with delayed treatment of Rocky Mountain spotted fever who suffered severe neurological impairment. Serial brain magnetic resonance images revealed a progressive "starry sky appearance," which is proposed to result from the same small vessel vasculitis that causes the characteristic skin rash of this infection. CONCLUSION:Neurological injury can continue to occur despite specific antirickettsial therapy in Rocky Mountain spotted fever. This child's clinical features raise questions about the optimal management of this infection, particularly the utility of immune modulating therapies in cases of delayed treatment and neurological involvement.