scholarly journals VON RECKLINGHAUSEN'S DISEASE

2004 ◽  
Vol 43 (154) ◽  
Author(s):  
Salamat Khan ◽  
A Chandra ◽  
N Jain ◽  
A Kumar ◽  
I U Khan

Von Recklinghausen's disease (VRD) is a rare genetic autosomal dominant disorder that affects the ectodermaltissues (nerve and skin). The cardinal features of this disorder are spots of increase pigmentation (cafe-au-lait spot, CAL), peripheral nerve tumours (neurofibromatosis) and iris hamartoma (Lisch nodule). Clinicaldiagnosis of VRD is based on the criteria given by National Institute of Health (NIH) because the mutationanalysis is laborious. Here, we are reporting a case of a family with VRD.Key Words: VonRecklinghausen's disease (VRD), Cafe-au-lait (CAL) spot, Lisch nodule,Neurofibromatosis (NF)

Neurosurgery ◽  
1987 ◽  
Vol 20 (6) ◽  
pp. 946-949 ◽  
Author(s):  
Charles E. Rawlings ◽  
Robert H. Wilkins ◽  
Wesley A. Cook ◽  
Peter C. Burger

Abstract Of the various forms of neurofibromatosis, the best known are peripheral neurofibromatosis (von Recklinghausen's disease) and central neurofibromatosis. There is a rare form called segmental neurofibromatosis, in which the cutaneous and neural changes are limited to one sector of the body and with which there is usually no family history of neurofibromatosis. We report four patients with this disorder and stress the value of repetitive, limited excision of peripheral nerve tumors in their management.


1986 ◽  
Vol 48 (3) ◽  
pp. 491-499
Author(s):  
Shuhei IMAYAMA ◽  
Yutaka YASHIMA ◽  
Atsushi IRIKI ◽  
Emiko SATO ◽  
Harukuni URABE

1987 ◽  
Vol 49 (3) ◽  
pp. 460-464
Author(s):  
Kazumoto KATAGIRI ◽  
Tomohito HONDA ◽  
Satoshi ITAMI ◽  
Etsuji MATSUNAGA ◽  
Hiroshi SHINKAI ◽  
...  

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