Meningiomas are rare intracranial tumors in pediatric patients. In contrast to meningiomas in adults, childhood ones have a poorer prognosis because of their high growth potential and tendency to recur. Meningiomas are often associated with neurofibromatosis type 2 (NF2) which is an autosomal-dominant disorder. In contrast to adults who primarily present with symptoms due to vestibular tumors, the initial symptoms in children with NF2 are subtle skin tumors, posterior capsular cataracts, or neurological signs secondary to cranial nerve(s) schwannoma excluding vestibular nerve, and/or brainstem or spinal cord compression. Here we report on the clinical, radiological, and histological findings in an 8-year-old boy who was diagnosed with an isolated infratentorial meningioma and a novel splice site mutation in the NF2 gene. The same mutation was detected in the boy's mother who suffered from hearing loss and tinnitus due to a bilateral vestibular schwannoma. Our patient demonstrates the need for molecular testing for NF2 gene mutations even in isolated childhood meningiomas although they do not fulfill the clinical criteria of NF2.
Glycerol is a known agent in the therapy of chronic tic douloureux. It has been used for about 20 years in percutaneous, retrogasserian minimal-invasive rhizotomy, although the pharmacological mechanism of the pain relief involved remains unclear. To investigate glycerol treatment as a possible replacement for invasive approaches in the therapy of chronic cervicogenic headaches, we performed an experimental study on the pathomorphologic action of anhydrous glycerol injection into the second upper cervical dorsal root ganglion (DRG) of rats. Glycerol injections into the second cervical ganglion were investigated light- and electron-microscopically in a series of 40 rats for survival times of up to 30 days. We detected an unspecific overall effect on sensory neurons and satellite cells, as well as on myelinated and unmyelinated axons and Schwann cells. This could be detected after 5 days and sometimes led to degeneration of most of the neurons. Contralateral saline injections as a control showed no morphological effects. The loss of afferent fiber connections to the posterior horn of the myelon could be detected by immunohistochemical labeling of reactive astrocytes. Our results show a glycerol-induced deterioration of the cytoarchitecture of the neurons and their glial satellite cells. The effects on the ganglion cells appear to have been mediated by membrane disturbances and loss of glial integrity. These observations are contrary to previously reported results indicating the specific effect of glycerol on thin myelinated sensory axons.
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