The gene for von Recklinghausen neurofibromatosis (NF1) was recently identified by positional cloning and found to code for a large, ubiquitously expressed protein. This protein has both structural and functional similarity to a family of proteins with guanosine triphosphatase-activating properties, involved in the regulation of the protooncogene ras. One of the postulated functions of the NF1 ...

INTRODUCTION Neurogenic tumors of the larynx are extremely rare, accounting for less than 1% of all benign laryngeal tumors. The lesions that have been described in current literature are located either in the supraglottis or subglottis, mainly affecting the pediatric population and associated with von Recklinghausen disease. STUDY DESIGN Descriptive study of an unusual case of an isolated ne...

Manifestations of central von Recklinghausen 's neurofibromatosis are well known and may include bilateral acoustic neuromas in the absence of peripheral neurofibromatosis or associated gliomas, meningiomas, other cranial nerve neuromas, optic nerve gliomas, and ependymomas [1]. A few reports deal specifically with the cranial CT findings in central neurofibromatosis [2-4]. One series [3] inclu...

Von Recklinghausen (1) in 1882 recognized the fact that the presence of "multiple neuromas" could not be considered as a purely acquired disease, but Thomson (2) in 1900 was the first to point out clearly that the condition was hereditary. Since that time the hereditary and familial character of the disease has been fully established and is generally recognized. Cases of multiple neurofibromato...

Attacks of dizziness and syncope have been relatively common features in the recorded cases of primary intracardiac tumour and of intracardiac ball thrombus (Wood, 1814; von Recklinghausen, 1885; Schwarz and Biloon, 1931; Gorlitzer, 1934; Elson, 1934; Fawcett and Ward, 1939; Garvin, 1941; Wright, Flynn, and Druet, 1944; Burnett and Davidson, 1945; Evans and Benson, 1948). Actual epileptiform co...

Neurofibromatosis type-1 (NF1), also known as Von Recklinghausen disease, is an autosomal dominant disorder with incidence of one per 4000. Neurofibromas are benign, heterogeneous, peripheral nerve sheath tumors coming up from the connective tissue of peripheral nerve sheaths, particularly the endoneurium. Visceral involvement in disseminated neurofibromatosis is considered rare. Neurofibroma o...

Descriptions of individuals supposed to have neurofibromatosis have been discovered in manuscripts dating from 1000 AD (Zanca, 1980). However, it was not until 1881 that Von Recklinghausen coined the term ‘‘neurofibroma’’ when he observed that this benign tumour arose from the peripheral nerve sheath. His colleagues honored his contribution by naming the condition Von Recklinghausen’s disease. ...