Introduction: NEUROFIBROMATOSIS is a genetic disease characterized by multifocal benign tumors of peripheral nerves, called neurofibromas, and pigmented spots on the skin which inherited as autosomal-dominant. The most common form of the disease is NEUROFIBROMATOSIS type 1, also known as von Recklinghausen's disease of the skin. When an individual has small number of lesions in a limited region of the his body, it could be missed by the patient or not acknowledged by the clinicians as a form of NEUROFIBROMATOSIS. We present here, a case of an 18-year-old male with NEUROFIBROMATOSIS type 1 who referred to Babol Dental School for a routine dental examination.

38-year-old woman presented with sudden weakness of the left upper and lower extremities, severe headache, repeated vomiting, and decreased level of consciousness since five to six hours prior to admission. She had no history of diabetes mellitus, hypertension, or any other remarkable diseases.

Introduction: Since CNS is derived from ectoderm during the fetal period, there are some common congenital or hereditary conditions that affect both the skin and nervous systems. Such conditions are traditionally called neurocutaneous disorders or phakomatosis.In this study, we have reviewed the most common clinical features and paraclinical findings in NEUROFIBROMATOSIS (NF) and tuberous sclerosis.Materials and Methods: In this cross sectional study, 50 patients with tuberous sclerosis or NF were chosen among all the patients who referred to the neurological clinic of Shahrivar 17th Hospital during a ten-month period. They were physically examined to detect any suspicion of neuroectodermal syndromes employing paraclinic exams like CT scan, MRI and EEG. Information about patients’ familial history, age, sex, clinical and paraclinical findings was collected to fill out the questionnaires. Finally, the data were analyzed with SPSS and SAS soft wares.Results: 80% of our patients had NF. The ratio of males to females was calculated as 1.1. The average age for the emergence of the disease was 7.8 years old for tuberous sclerosis, 11.3 years old for NF1 and 59 years old for our only case of NF2. The most common manifestation of these syndromes was Cutaneus Lesions (88.9% in tuberous sclerosis and 100% in NF).Seizure and mental retardation were also common features in tuberous sclerosis with frequencies of 88.9% and 75%, respectively. Paraclinical studies were helpful in diagnosing the 50% of tuberous sclerosis and the8.7% of NEUROFIBROMATOSIS cases.Conclusion: Clinical and paraclinical manifestations of neurocutaneous disorders in this study are similar to other studies in the world.

Breast cancer is highly prevalent amongs t older women but much less common in younger women, especially those under the age of 20 . Here we present a case of an 18-year-old woman with NEUROFIBROMATOSIS 1 diagnosed with breast cancer. Review of literature demons trates a five-fold increased risk of developing breast cancer before age 50 with a mutation in this tumor suppressor gene. Incorporation of earlier screening recommenda tions for at-risk younger women is warranted in more guide lines.

NEUROFIBROMATOSIS is an autosomal dominant progressive disorder with an incidence of approximately 1 in 3000 live births. Its recognized features include hyperpigmented skin lesions (cafe-au-lait spots), neurofibromas, iris hamartomas, macrocephaly, central nervous system tumors, defects of the skull and facial bones, and vascular lesions. Involvement of the external genitalia is extremely unusual. We present a case of a neurofibroma of the dorsal clitoral hood and its management.