Neurocutaneous melanosis concurrent with a posterior cranial fossa cyst (Dandy–Walker complex) and an intradural arachnoid cyst of the spinal canal

Abstract

The paper describes a female patient with neurocutaneous melanosis (NCM) concurrent with Dandy–Walker malformation and a spinal arachnoid cyst, who has been treated in the A.Ya. Kozhevnikov Clinic of Nervous System Diseases. Over the course of one year, the patient had progressive neurological symptoms as central lower paraparesis and coordination disorders; her medical treatment was ineffective. Neuroimaging revealed Dandy–Walker malformation and a spinal arachnoid cyst. Surgical intervention (fenestration of the upper and lower poles of the arachnoid cyst at the C VI-VII and T IX-X level) was performed. After one month, reoperation (commissure resection in the arachnoid cyst cavity at the T V-VI level; implantation of a cystoperitoneal shunt) was made because of the absence of a clinical result. Reversal of neurological disorders was noted postoperatively. The described clinical case is unique, since NCM is an extremely rare abnormality, especially when concurrent with Dandy–Walker malformation and an arachnoid cyst. In addition, it is of interest that the manifestation of neurological symptoms and verification of the diagnosis occurred just in adulthood, although NCM is most commonly diagnosed in children. The present case shows that an operation aimed at improving cerebrospinal fluid dynamics in patients with arachnoid cysts and Dandy–Walker complex in the presence of NCM can lead to a significant reversal of neurological symptoms and improvement in the quality of life despite gross brain structural changes detected by magnetic resonance imaging.