Abstract
Introduction. Cutis verticis gyrata is an uncommon benign dermatological disorder characterised by hypertrophic scalp folds resembling cerebral gyri. The primary form is subdivided into essential and non-essential; the latter associated with neurological abnormalities. Its relationship with epilepsy and neurodevelopmental impairment remains insufficiently recognised. We report a case of primary non-essential cutis verticis gyrata in a patient with drug-resistant epilepsy.
Presentation of the case. A 29-year-old male, born preterm with perinatal hypoxia, developed seizures from the second day of life. He subsequently presented impaired awareness seizures with automatisms and focal to bilateral tonic–clonic seizures, evolving into drug-resistant epilepsy despite multiple anti-seizure therapies. He exhibited global developmental delay, microcephaly, intellectual disability, and right hemiplegia. Brain magnetic resonance imaging demonstrated generalized atrophy and bilateral parieto-occipital encephalomalacia. During early adulthood, progressively deep longitudinal folds became clinically evident over the parietal and occipital scalp. After exclusion of secondary causes, primary non-essential cutis verticis gyrata was diagnosed.
Discussion and conclusions. The delayed dermatological manifestation relative to longstanding neurological disease underscores limited understanding of its natural history. Increased clinical awareness and further investigation of shared pathogenic mechanisms are warranted.
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