Neurocutaneous melanosis concurrent with a posterior cranial fossa cyst (Dandy–Walker complex) and an intradural arachnoid cyst of the spinal canal
https://doi.org/10.14412/2074-2711-2020-2-79-85
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 CVI-VII and TIX-X level) was performed. After one month, reoperation (commissure resection in the arachnoid cyst cavity at the TV-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.
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.
About the Authors
G. Yu. Evzikov
Department of Nervous System Diseases and Neurosurgery;
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
M. G. Bashlachev
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
E. V. Shashkova
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
K. A. Belozerskikh
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
F. V. Grebenev
Clinic for Nervous System Diseases, Institute of Clinical Medicine, I.M. Sechenov First Moscow State Medical University (Sechenov University), Ministry of Health of Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
Russian Federation
11, Rossolimo St, Build. 1, Moscow 119021, Russia
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Review
For citations:
Evzikov G.Yu., Bashlachev M.G., Shashkova E.V., Belozerskikh K.A., Grebenev F.V. Neurocutaneous melanosis concurrent with a posterior cranial fossa cyst (Dandy–Walker complex) and an intradural arachnoid cyst of the spinal canal. Neurology, Neuropsychiatry, Psychosomatics. 2020;12(2):79-85. (In Russ.) https://doi.org/10.14412/2074-2711-2020-2-79-85
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