Certified Neuroscience Registered Nurse (CNRN) Practice Exam

What characterizes Von-Hippel Lindau disease?

1. Multiple benign hemangiomas of the CNS

2. Malignant tumors of the CNS

3. Spinal bifida

4. Hydrocephalus

The correct answer is: Multiple benign hemangiomas of the CNS

Von-Hippel Lindau disease is primarily characterized by the presence of multiple benign hemangiomas, particularly in the central nervous system (CNS), as well as in other areas such as the retina and various organs. These hemangiomas, also known as hemangioblastomas, can manifest in the cerebellum and spinal cord, contributing to neurological symptoms. The notion of benign hemangiomas being a hallmark feature of this genetic disorder is key to its identification and understanding. While malignant tumors can be a concern in general oncology, they are not the primary characteristic of Von-Hippel Lindau disease, which is more associated with benign lesions. Similarly, spinal bifida and hydrocephalus may arise from other neurological conditions but are not typical or definitive features of Von-Hippel Lindau disease. Recognizing the distinct nature of hemangiomas in this context reinforces the importance of understanding specific diseases' clinical presentations in neuroscience nursing.