Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.

We report a case of epidermal nevus syndrome involving the brain in which there is chronic occlusion of the left distal internal carotid artery resulting in ipsilateral atrophy. Orbital and cerebellopontine angle cistern lipomas and a wide cortical developmental malformation are associated with the condition. We present MR imaging findings of a patient and discuss features in the context of oth...

Sturge Weber Disease is one of the important neurocutaneous syndrome characterized by abnormities of both the integument and central nervous system. We present here a classical case of Sturge Weber Disease. This 16 month male child had facial angioma and seizures. CT Scan head was consistent with the diagnosis.

OBJECTIVE: Neurofibromatosis type I (NF1), Tuberous sclerosis complex (TSC) and Sturge-Weber syndrome (SWS) are the most common neurocutaneous syndromes. The purpose of this study is to evaluate clinical neuroradiological manifestations patients diagnosed with NF1, TSC, SWS.MATERIAL AND METHODS: In our clinic, records 15 SWS were retrospectively reviewed between December 2017 May 2019. Clinical...

Case presentation: A 2-year-old boy with Dandy-Walker syndrome diagnosed by obstetric ultrasound, presented diffuse and large nevi at birth. He was submitted to endoscopic third ventriculostomy 15 days old due obstructive hydrocephalus. Spinal fluid then sent for analysis but showed no melanocytes. MRI of the brain additional findings mutation could not be performed. After 1 month he needed a v...

BACKGROUND In China, the basic insurance system consists of three schemes: the UEBMI (Urban Employee Basic Medical Insurance), URBMI (Urban Resident Basic Medical Insurance), and NCMS (New Cooperative Medical Scheme), across which significant differences have been observed. Since 2009, the central government has been experimenting with consolidating these schemes in selected areas. This study e...

Neurocutaneous melanosis (NCM) is a rare congenital syndrome characterized by the presence of large or multiple congenital melanocytic nevi with either benign or malignant pigmented cell tumors of the leptomeninges. We report a case of NCM in a patient with previously robust health. No neurological deficit was noted in the patient. At the age of 21 years old, blurred vision initially appeared. ...

Sjögren-Larsson syndrome is a rare neurocutaneous disorder characterized by ichthyosis, spastic diplegia or tetraplegia, and intellectual disability. Herein, we describe a case of a Greek patient with ichthyosis and spasticity of the legs but with normal intelligence (IQ 95). This syndrome should be suspected when a child presents with ichthyosis and spastic diplegia or tetraplegia, even if int...