Ghosh, RitwikLeón Ruiz, MoisésPurkait, SikthaRoy, DipayanGhosh, TapasBenito León, Julián2024-04-222024-04-222023-07-16Ghosh R, León-Ruiz M, Purkait S, Roy D, Ghosh T, Benito-León J. Headache as the presenting manifestation of Gorlin-Goltz syndrome with diastematomyelia: A case report. Neurol Clin Neurosci. 2023 Nov;11(6):328-331. doi: 10.1111/ncn3.12767. Epub 2023 Jul 16. PMID: 381306662049-41732049-417310.1111/ncn3.12767https://hdl.handle.net/20.500.14352/103315Gorlin-Goltz syndrome (GGS) is an autosomal dominant multisystemic disease with high penetrance. Headache heralding GGS has been previously reported but without discussing potential sources. We report a patient with headache and a novel association (diastematomyelia), which helped with the diagnosis. A 46-year-old woman presented with persistent holocranial headache. On examination, countless hyperpigmented basal cell nevi over the face, pits over the palmar/plantar surface, and palmar and plantar keratosis were observed. A magnetic resonance imaging (MRI) of the spinal cord revealed diastematomyelia. Diagnosis of GGS was finally made. Headache and diastematomyelia should be included in the clinical picture of GGS.engAttribution 4.0 Internationaljournal articlehttps://doi.org/10.1111/ncn3.1276738130666https://onlinelibrary.wiley.com/doi/full/10.1111/ncn3.12767open access616.857Gorlin-Goltz syndromeDiastematomyeliaHeadacheMalformationPresenting manifestationMedicina interna3205.07 Neurología