Neuroborreliosis-Associated Cerebral Vasculitis

A 9-year-old boy was referred to our pediatric department with symptoms of chronic fatigue, headache, intermittent nausea, and vomiting for 2 months. At admission a weight loss of 2 kg and intermittent subfebril temperature as well as an unspecific tremor of both hands was reported. He had a medical history of erythema migrans 2 years ago treated adequately with amoxicillin for 2 weeks. There was no history of trauma or stay in subtropical countries. Family history revealed a Waldenstrom's macroglobulin-emia of his father and paternal uncle. Clinically, only mild postural tremor of both hands and mild horizontal nystag-mus with no other clinical symptoms was noticed. A cerebral magnetic resonance imaging (MRI) angi-ography was performed, showing 2 small acute cerebel-lar infarctions in the posterior inferior cerebellar artery area and occlusion of the basilar artery, highly suspicious of an underlying vasculitis (Figure 1A and B). Conventional cerebral angiography detected a subtotal occlusion of the basilar and bilateral vertebral arteries with retrograde blood flow to the posterior cerebral circulation (Figure 1C). Extended immunological, hematological, and endo-crinological laboratory evaluation revealed no abnormalities , among others normal blood count; negative C-reactive protein; normal erythrocyte sedimentation rate; normal renal, thyroid, and liver function; negative thrombophilic parameters; and negative immunological tests with no contribution for systemic lupus erythema-tosus, antiphospholipid syndrome, or other rheumato-logic diseases (Table 1). Instead, Borrelia burgdorferi serology was positive for IgM and IgG, with confirmation of 4 positive bands in Western blot. A lumbar puncture was performed, showing lymphocytosis with increased protein and decreased glucose levels along with an intrathecal production of Borrelia burgdorferi IgM antibodies. Therefore, neuroborreliosis was assumed and treatment was started with intravenous ceftriaxone 80 mg/kg/ day for 3 weeks in addition to oral acetylsalicylic acid (ASS) 5 mg/kg/day and prednisolone 1 mg/kg/day. Clinical symptoms subsequently improved and neuro-logical examination returned to normal soon after therapy was started. A follow-up lumbar puncture 3 weeks later showed a reduction of pleocytosis and normalization of liquor glucose and protein. Two months later, MRI angiography showed the absence of cerebellar diffusion restrictions, but an unchanged presentation of the basilar and bilateral vertebral arteries occlusion. The prednisolone therapy was tapered over 3 weeks, whereas ASS therapy was continued. Three months later, a subsequent MRI angiography showed an unvaried occlusion of the basi-lar and bilateral vertebral arteries with no areas of new infarction (Figure 1D). Clinical and radiological follow-up 6 and 12 months after diagnosis showed …