Neurofibromatosis-I Presentating with Multiple Spinal and Intracranial Neurofibromas.
نویسندگان
چکیده
Von Recklinghausen's neurofibromatosis (NF-1) is a phacomatosis characterised by widespread nervous system tumours with cutaneous manifestations and variably associated anomalies. We report here a case, who, in addition to classical features of NF-1 (café-au-lait spots, cutaneous and subcutaneous neurofibromas) demonstrated radiological evidence of both spinal and intracranial neurofibromas and an incidentally discovered horse-shoe kidney. The unique constellation of spinal and intracranial neurofibromas, with associated horse-shoe kidney.
منابع مشابه
Unilateral and multiple nodular plexiform Neurofibromas: An uncommon presentation of Neurofibromatosis type 1
Plexiform neurofibroma is a rare variant but pathognomonic of von Recklinghausen disease. We report an uncommon presentation of neurofibromatosis type I in a girl. This 11-year old girl had multiple plexiform neurofibromas on her left hand, and also several Lisch’s nodules. Classification and clinical features are discussed briefly with emphasis on the possibility this condition may not be give...
متن کاملEvaluation of Phenotypic Characteristics of 53 Cases of Neurofibroma in Hazrat Fatemeh Hospital during the Years 1994-2005
Background and Objective: Neurofibromas occur as solitary and multiple forms. The solitary ones are extraneural and more common, histologically exhibiting spindle cells with wavy nuclei, scattered among collagen fibers. Multiple neurofibromas are seen in neurofibromatosis (types I and II), including intr...
متن کاملA Complete Constellation of Nervous System Lesions of NF2: Imaging Evaluation
The radiological findings fulfilling the criteria of neurofibromatosis type 2 (NF2) were reviewed. NF2 is a rare disease with few cutaneous but frequent, typical radiological findings in the central nervous system. The presenting symptom is most commonly hearing loss due to acoustic schwannomas, although symptoms emanating from other intracranial or tumors are not uncommon. The discovery of mul...
متن کاملHereditary spinal neurofibromatosis: a rare form of NF1?
We describe a family in which seven members in three generations were affected with a rare spinal neurofibromatosis. The affected adults showed, at the ages of 32, 37, 38, and 61, respectively, multiple spinal neurofibromas symmetrically affecting all spinal roots. Two patients were operated on for histopathologically proven cervical spinal neurofibromas. All patients had café au lait spots, on...
متن کاملSegmental Neurofibromatosis Type 1, a Rare Variant of Neurofibromatosis: Report of Two Cases
Segmental neurofibromatosis type I (SNF-I) is a rare variant of neurofibromatosis (NF). It is classified as NF type V and defined as cafe'-au-lait macules and/or neurofibromas in a single ,unilateral segment of the body .We report two cases with SNF-I with striking similar manifestations.
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
عنوان ژورنال:
- The Journal of the Association of Physicians of India
دوره 64 2 شماره
صفحات -
تاریخ انتشار 2016