Recurrent stroke and aseptic meningitis in patient with Fabry disease: A case report

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Fabry Disease (FD) is a rare, potentially curable, hereditary disease. Despite of enzyme replacement therapy (ERT) recurrent stroke and aseptic meningitidis are developed. The 26 year-old male was diagnosed FD after the first ischemic stroke. In a 6 months of receiving ERT the second ischemic stroke in the vertebrobasilar system was developed. In a month severe headache with nausea, vomiting and paresis of the right oculomotor nerve developed, without lesions on MRI. The cerebrospinal fluid (CSF) analysis showed lymphocytic pleocytosis of 24/μL. Aseptic meningitis was diagnosed. Prednisolone 50 mg a day was prescribed. After treatment with glucocorticoids, oculomotor disturbances and cerebral symptoms regressed over a 3 day. In a month the third ischemic stroke developed in the patient in the vertebrobasilar system with headache and dysarthria. He had stopped to take prednisolone by this time. After this stroke headache increased, and the patient started taking nonsteroid anti-inflammatory drugs without permanent effect. The cognitive impairment progressed. Despite of normal CSF analysis prednisolone 10 mg once a day was administered. Headache regressed next day after starting this treatment. During the treatment with prednisolone the patient demonstrated cognitive improvement. When we tried to decrease the dose of prednisolone the patent had a deterioration with his oculomotor nerves without lesion on the MRI Activation of immune system in some patients with FD could lead to autoinflammation in the central nervous system, as a result – aseptic meningitidis. Prednisolone administration in spite of recurrent stroke could improve outcome in such patients.