Abstract

Neurofibromatosis Type 1 (NF-1 or Von Recklinghausen disease) is an autosomal dominant genetic disease, characterized by an extreme variability of its clinical expression which is also found in the same family. Our work focuses on the exploitation of four cases of patients with NF-1 who were enrolled in the paediatric neurology consultation at Rabat Children’s Hospital. They are two infants and two children. Otherwise the diagnosis was made in front of the existence of cafe au lait and lentiginous spots in two boys, also the existence of cafe au lait spots and abnormalities in brain imaging in two girls. Thus an evolution was marked by a favorable outcome for three patients and neurological sequelae in one patient.

Highlights

  • The NF-1 is a condition characterized by changes in skin coloring and the growth of tumors along nerves in the skin, brain, and other parts of the body

  • Neurofibromatosis Type 1 (NF-1 or Von Recklinghausen disease) is an autosomal dominant genetic disease, characterized by an extreme variability of its clinical expression which is found in the same family

  • Our work focuses on the exploitation of four cases of patients with NF-1 who were enrolled in the paediatric neurology consultation at Rabat Children’s Hospital

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Summary

Introduction

The NF-1 is a condition characterized by changes in skin coloring (pigmentation) and the growth of tumors along nerves in the skin, brain, and other parts of the body. Almost all people with NF-1 have multiple café-au-lait spots, which are flat patches on the skin that are darker than the surrounding area. The signs and symptoms of this condition vary widely among affected people It accounts for 95% of all neurofibromatosis; its global distribution is homogeneous without ethnic predilection and its penetrance is almost complete at the age of 8 years [2]

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