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Developmental Diseases of the Nervous System
Published in Philip B. Gorelick, Fernando D. Testai, Graeme J. Hankey, Joanna M. Wardlaw, Hankey's Clinical Neurology, 2020
James H. Tonsgard, Nikolas Mata-Machado
Adenoma sebaceum are small, red-pink, flat-topped skin lesions with a smooth, glistening surface, a few millimeters in size, that are distributed in a butterfly-like pattern on the face. They appear in mid to late childhood in 70% of patients. Fibrous plaques are a similar lesion, usually appearing in late childhood or adult years in 19%. Both of these lesions are angiofibromas, characterized by hyperplasia of connective and vascular tissue (Figure 9.53).
Paper 2
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Tuberous sclerosis is an autosomal dominant disease which can present with seizures, development delay and, in around 75% of cases, a skin condition called adenoma sebaceum. Central nervous system features include subependymal hamartomas, cortical tubers, heterotopic grey matter islands and subependymal giant cell astrocytoma. Optic nerve gliomas and focal areas of signal intensity (FASIs) are associated with NF1. Subcortical calcification and prominent leptomeningeal enhancement is associated with Sturge-Weber syndrome.
Neurology and neurosurgery
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
12.26. Which of the following skin lesions is/are associated with central nervous system abnormalities?Linear streaky vesicles in the newborn.Vitiligo.Adenoma sebaceum.Café au lait spots.Telangiectasia.
Genetics and optical coherence tomography features in a child with an achromatic retinal patch
Published in Ophthalmic Genetics, 2022
Noy Ashkenazy, Nicolas A. Yannuzzi, Audina M. Berrocal
Achromatic retinal patch does not always present as an isolated macular lesion. In a retrospective series of tuberous sclerosis patients by Shields et al., there was a median of one lesion per eye (range 1–7), with 28 lesions across 12 eyes. The depigmented lesions were found in the extramacular, superonasal quadrant in a quarter of cases (11). Of possible prognostic value, punched out retinal pigment epithelial (RPE) lesions have been associated with positive seizure history, mental retardation, cutaneous adenoma sebaceum, ash leaf macules, and increasing numbers of retinal astrocytic hamartomas (4,11).