A RARE CASE OF POSTINFECTIOUS MOYAMOYA SYNDROME: CASE REPORT AND REVIEW OF THE LITERATURE

Abstract Background Postinfectious Moyamoya syndrome (MMS) is a rare vasculopathy that can follow a meningitis. Only 9 cases of MMS following a meningitis have been reported in literature. We present a unique case of MMS after a meningitis caused by A. fumigatus and E. coli and review all cases of MMS post-meningitis in literature. Case Description A 41 years-old man was admitted to our Emergency department for sudden hypoesthesia in left arm and an intense headache not responsive to drugs. The CT scan and the MRI showed acute ischemic lesions in the right centrum semiovale associated to bilateral chronic watershed cerebral ischemic lesions. The cerebral DSA documented a typical Moyamoya pattern. In anamnesis, the patient suffered from meningitis due to A. fumigatus and E. coli infection after neurosurgery for subependymoma of the fourth ventricle two years before. Lab tests, clinical investigation and imaging ruled out any other cause of vasculopathy and led to the final diagnosis of postinfectious MMS. The patient started a medical therapy with oral acetyl salicylic acid, verapamile and prednisone while surgical approach was excluded in the first instance. Conclusions Physicians should be aware of an uncommon but severe complication of meningitis such as MMS, even several years after the infection. Neuroimaging is essential for the diagnosis and to exclude other causes of neurological symptoms in these patients.