A Rare Case of Postinfectious Moyamoya Syndrome: Case Report and Review of the Literature

Background: Postinfectious Moyamoya syndrome (MMS) is a rare vasculopathy that can follow meningitis. Only 9 cases of MMS after meningitis have been reported in the literature. We present a unique case of MMS after meningitis caused by Aspergillus fumigatus and Escherichia coli and review all cases of MMS postmeningitis in the literature. Case description: A 41-year-old man was admitted to our emergency department for sudden hypoesthesia in the left arm and an intense headache not responsive to drugs. Computed tomography scan and magnetic resonance imaging showed acute ischemic lesions in the right centrum semiovale associated with bilateral chronic watershed cerebral ischemic lesions. The cerebral digital subtraction angiography documented a typical Moyamoya pattern. In anamnesis, the patient suffered from meningitis caused by A. fumigatus and E. coli infection after neurosurgery for subependymoma of the fourth ventricle 2 years before. Laboratory tests, clinical investigation, and imaging ruled out any other cause of vasculopathy and led to the final diagnosis of postinfectious MMS. The patient started medical therapy with oral acetyl salicylic acid, verapamil, 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 neurologic symptoms in these patients.