A 28-year-old woman was admitted to the Neurovascular Unit for transient right hemiplegia associated with language disorders, without fever. The patient complained of unusual headaches since she had returned from Burkina-Faso, 6 months before, where she had worked for a humanitarian organization for one year. She first had a cerebral Magnetic Resonance Imaging (MRI) as soon as she returned to France, which failed to show any disorder. She reported no illness during her mission or before, but had frequently swum in a small lake near where she lived. A cranial MRI was performed at admission, showing multiple vascular hyperintense signals in the left hemisphere. A day after admission, as hemiplegia persisted, an angio-MRI was performed which showed a left junctional infarct, straight stenosis of the left carotid artery and inflammatory aspect of the right carotid artery (Figure
). No other big trunk was involved on the complete aortic imaging, neither the supra-aortic trunk nor the thoraco-abdominal portion of aorta. Transoesophageal echocardiography did not show any abnormalities. The physical examination showed no hepatomegaly or splenomegaly. The vascular examination found all the pulses. There were no symptoms of arthritis, uveitis or skin manifestation. The white blood cell count was normal, including the eosinophil count (300/mm3; normal, < 500/mm3). C-reactive protein was normal. By contrast, cerebrospinal fluid showed moderate lymphocytic pleocytosis (28 cell/mm3) but without eosinophils. The IgG index was elevated, although the protein concentration remained normal (0.28 gr/l; normal, < 0.50 gr/l). Rheumatoid factor, Cryoglobulinemia, anti-cardiolipid, anti-nuclear and anti-neutrophil cytoplasmic antibodies were negative. Complement titer was elevated. Serologic tests for Human Immunodeficiency Virus, Cytomegalovirus, Epstein-Barr-Virus and viral hepatitis B and C were negative. The serologic test for schistosomiasis was strongly positive in hemagglutination: 1/128 (normal, < 1/8, in-house peparated antigen). Other helminthic serologic tests were negative (trichinellosis, fasciolasis, toxocariasis, anguillulosis). Repeated stool examinations (Kato and Baermann method) failed to detect any Schistosoma ova or other helminth ova. To substantiate the diagnosis of disseminated schistosomiasis, a rectal biopsy was performed, confirming the presence of S. mansoni granuloma in rectal mucosa (Figure
Bilateral carotid vasculitis. Angio-MRI showing straight stenosis of the left carotid artery and inflammatory aspect of the right carotid artery (white arrows).
Rectal mucosa biopsy. Granuloma around a Shcistosoma mansoni egg (black arrow). Magnification X 400.
Treatment with anticoagulant and steroid therapy (prednisone 1 mg/kg/day) was started immediately, with rapid improvement of neurologic symptoms. A few days later, a first-line course of praziquantel was given (40 mg/kg/day for 3 days) and was well tolerated. Ten days after admission, the patient was discharged from hospital with oral anticoagulant therapy and prednisone to be tapered progressively. One month later, a second course of praziquantel was prescribed while she was still taking prednisone at 20 mg/day. The day after she took praziquantel, she complained of a headache and diplopia. The neurologic examination showed right cranial nerve VI paralysis. She was admitted to hospital and a new angio-MRI revealed stenosis of the right carotid siphon. Exacerbation of vasculitis after the praziquantel therapy was suspected and intra-venous methylprednisolone therapy was started (500 mg/day) with resolution of diplopia within 24 h. Oral corticotherapy (1 mg/kg/day) was prolonged for 6 weeks and progressively tapered. The patient did not relapse when prednisone was discontinued. Anticoagulant therapy was maintained for a longer period because of persistent carotid stenosis. A new angio-MRI 6 months later showed minor improvement of both carotid stenoses. The serologic level progressively decreased: 1/32 one year after the first neurologic episode.