Miyazaki Syndrome due to Ventriculoperitoneal Shunt Treatment

Abstract

Background: The signs and pathomechanism of Miyazaki syndrome is presented through the case of a young female patient.

Case description: The 33-year-old patient had undergone placement of a ventriculoperitoneal shunt with a pressure-adjustable valve for communicating hydrocephalus years before presenting to our department with the complaints of constant headache and unsteady gait. On the basis of the clinical picture and her history, plain and contrast-enhanced cranial and whole spine magnetic resonance imaging and magnetic resonance angiography examinations were performed, with the scans revealing signs indicative of cerebrospinal fluid hypotension typical of Miyazaki syndrome.

Conclusion: The article discusses the available literature suggesting the underlying cause in such cases to be the dysfunction of the Starling resistor mechanism due to an improperly adjusted ventriculoperitoneal shunt, which results in excessive cerebrospinal fluid loss accompanied by consequent cerebral venous overflow with vertebral venous engorgement and compressive cervical myelopathy.

Keywords: Cerebrospinal fluid hypotension; Miyazaki syndrome; Overshunting-associated myelopathy; Ventriculomegaly; Ventriculoperitoneal shunt.