Neurocutaneous Diseases

Neurocutaneous Diseases is a systematic presentation limited to diseases that affect both of the nervous system and skin of humans. Neurologists and dermatologist will find knowledge of these diseases of real clinical value. Many of the diseases described in these pages do not affect the skin and nervous system simultaneously. Many of these diseases, mostly not well understood, may stimulate new lines of scientific inquiry, for understanding of a pathologic change in easily accessible dermal cells that promises to clarify a more recondite brain disease. This book is organized into five main parts. The chapters describe different types of diseases including those with autosomal dominant inheritance, those with autosomal recessive inheritance, those with x-linked inheritance, those with unknown or multiple inheritance and congenital and vascular anomalies. These diseases include neurofibromatosis, Cockayne's Syndrome, adrenoleukodystrophy, albinism and neurocutaneous melanosis. This book will be of interest to dermatologists and neurologists.

Contributing AuthorsForeword Preface 1 Genetic PrinciplePart One / Diseases with Autosomal Dominant Inheritance 2 Neurofibromatosis 3 Tuberous Sclerosis 4 Von Hippel-Lindau Disease 5 Nevoid Basal Cell Carcinoma Syndrome 6 Lentiginosis-Deafness-Cardiopathy Syndrome 7 Hypomelanosis 8 Other Autosomal Dominant Diseases Part Two / Diseases with Autosomal Recessive Inheritance 9 Ataxia-Telangiectasia 10 Xeroderma Pigmentosum 11 Cockayne's Syndrome 12 Rothmund-Thomson Syndrome 13 Fucosidosis 14 Phenylketonuria and Hyperphenylalaninemia 15 Homocystinuria due to Cystathionine Synthase Deficiency 16 Citrullinemia and Arginosuccinicaciduria 17 Biotin-Responsive Multiple Carboxylase Deficiency 18 Cerebrotendinous Xanthomatosis 19 Familial Dysautonomia 20 Chediak-Higashi Syndrome 21 Neuroichthyosis 22 Sjögren-Larsson Syndrome 23 Refsum's Disease 24 Giant Axonal Neuropathy 25 Werner's Syndrome 26 Progeria 27 Neuroectodermal Melanolysosomal Disease 28 Ruvalcaba-Myhre Syndrome Part Three / Diseases with X-Linked Inheritance 29 Fabry-Anderson Disease 30 Adrenoleukodystrophy 31 Kinky Hair Disease 32 Incontinentia Pigmenti 33 A Syndrome of Anosmia, Ichthyosis, and Hypogonadism with Steroid Sulfatase and Arylsulfatase C Deficiencies Part Four / Diseases with Unknown or Multiple Inheritance 34 Coffin-Siris Syndrome 35 Albinism Part Five / Congenital and Vascular Anomalies 36 Neurocutaneous Melanosis 37 Linear Sebaceous Nevus 38 Cerebello-Trigemino-Dermal Dysplasia 39 Encephalo-Cranio-Cutaneous Lipomatosis 40 Sturge-Weber Syndrome 41 Klippel-Trenaunay Syndrome 42 Wyburn-Mason Syndrome 43 Maffucci's Syndrome 44 Rare and Questionable Vascular Neurocutaneous Diseases Index