Neurocutaneous syndrome (NCS), a newly discovered toxicity disorder, is described in light of our new understanding of its relationships with the causative agents included in the dental liners used in afflicted patients. NCS is characterized by neurological sensations, pain, depleted energy and memory loss as well as itchy cutaneous lesions that may invite various opportunistic infections. Comp...

Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, was reported by Schirmer, Sturge and Weber in succession in mid-19th century. And it is a neurocutaneous disease classically presenting with a facial portwine stain in the ophthalmic distribution of the trigeminal nerve, glaucoma and vascular eye abnormalities, and an ipsilateral occipital leptomeningeal angioma. It is...

ncontinentia pigmenti (IP) is a rare X-linked dominant neurocutaneous syndrome chiefly involving the ectodermal tissues, such as the skin, teeth, hair, eyes, and central nervous system (CNS).1 The diagnosis is usually made when the pathognomonic skin lesions are present; these typically appear in 4 stages: erythematous vesicular rash, verrucous patches, swirling hyperpigmentation, and atrophic ...

white dots in the ocular fundus which appear after several years of age,3 and their occurrence strongly suggests SLS.4 Macrocephaly is not a characteristic or common finding in SLS. Mutation analysis of the ALDH3A2 gene is a highly sensitive method of confirming the diagnosis of SLS.4 More than 90 pathogenic variants of ALDH3A2 have been identified to date.1 The diagnosis of SLS can be confirme...

Two cases of neurocutaneous melanosis are presented. MR showed hyperintense areas in the brain on short-repetition-time/short-echo-time sequences, compatible with intraparenchymal melanin deposits. No leptomeningeal abnormality was seen.

To the Editor: Dr Jean Latrelle, an eminent Parisian neurologist at the time of a meeting in Harriman, N.Y., started his discussion by saying, “Everything has been written, but not everything has been read”. I found the description of a patient with neurocutaneous melanosis by Oliveira de Andrade et al. (Epileptic Disord, 2004; 6: 145-52) interesting. For the past 30 years we have been followin...

A patient with multiple basal cell carcinoma syndrome, a symptom complex characteristized by nevoid basal cell carcinomas of the skin, jaw cysts, skeletal abnormalities, and hyporesponsiveness to parathormone is presented. In addition, the patient had a retroperitoneal lymphagiomyoma, a hamartomatous lesion, causing ureteral obstruction. The association of neuroectodermic syndromes and retroper...

PURPOSE The presence of two or more Lisch nodules (melanocytic hamartomas of the iris) is one of seven diagnostic criteria for neurofibromatosis type 1 (NF1), a common monogenic disorder of dysregulated neurocutaneous growth. The hypothesis that Lisch nodules arise secondary to exposure to ultraviolet (UV) radiation from sunlight was investigated. METHODS Lisch nodule burden was mapped and qu...