1/6. Axonal degeneration of peripheral facial nerve in a patient with progressive hemifacial atrophy.We report a case of a 23-year-old woman with progressive hemifacial atrophy. She showed an atrophic change on the left side of her face for 8 years. A skin biopsy obtained from the lesion revealed the fibrotic changes in the deep dermis and adipose tissue with infiltrations of lymphocytes and plasma cells. She underwent the augmentation using a deepithelialized anteromedial thigh flap with endoscopic assistance. A specimen of the peripheral facial nerve taken from the region adjacent to the skin lesion during the operation showed atrophy of neurofibers with vacuole degeneration. On an electron microscopic examination, a high degree of degeneration of myelinated and unmyelinated axons was observed. These findings may provide direct evidence that atrophic changes of nerve fibers are closely related with the pathology of this disease.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |
2/6. Lesions of the inferior alveolar nerve arising from endodontic treatment.A lesion of the IAN following endodontic treatment of the lower molars and premolars is not a rare event and presents an uncomfortable situation both for the dental surgeon and the patient. Injury can result on the one hand by direct intrusion of the instrument through the apex into the mandibular canal, and on the other by the filling material which becomes forced into the mandibular canal. In the latter case, a nerve lesion will only result when the filling material contains neurotoxic substances such as paraformaldehyde. With a direct lesion or when forcing of resorbable filling material into the mandibular canal is suspected, one should first employ a wait-and-see approach, because usually the only nerve damage is in the form of neuropraxy or axonotmesis for which there is a high rate of spontaneous regeneration. However, if neurotoxic filling material is introduced into the direct vicinity of the nerves, the mandibular canal should be opened and the filling material should be removed as early as possible. If the filling material is forced directly within the endoneurium between the nerve bundles, the damaged nerve sections must be resected and bridged using transplants from the sural or greater auricular nerves.- - - - - - - - - - ranking = 1.6666666666667keywords = nerve (Clic here for more details about this article) |
3/6. The giant axonal neuropathy--clinical and hisotological aspects, differential diagnosis and a new case.The giant axonal neuropathy (GAN) is morphologically characterized by axonal swellings and accumulations of neurofilaments in giant axons and other cell types. Curly hair is not a constant finding. The clinical course is progressive and mostly starts in early childhood. We report the case of a boy aged 6 years at the time of sural nerve and muscle biopsy. Suralis nerve showed a reduced numerical density of myelinated fibres with a consecutive endoneural fibrosis. Morphometric investigation revealed a pronounced reduction of fibres measuring 8-12 microm in diameter. Giant axons were seen in relatively low number and were not very large with a maximum diameter of 18 microm. They had a relatively thin myelin sheet proved also by the high G ratio in the histogram. Many onion bulb formations of schwann cells were present. There are only few reports of giant axons with such low maximum diameter in cases with GAN, the lowest maximum diameters being reported in case reports on Japanese children. Up to now, this is the first report of a non-Japanese patient with a low maximum diameter of giant axons of less than 20 microm in peripheral nerve biopsy. Ultrastructurally, typical accumulations of neurofilaments and osmiophilic aggregates were found in giant axons. Other diagnoses with occurrence of giant axons could be excluded in view of the absence of specific findings. Sporadic or familial cases with giant axons are discussed. Sceletal muscle biopsy (M. quadriceps femoris) showed neurogenic affection with presence of small angulated atrophic muscle fibres.- - - - - - - - - - ranking = 0.5keywords = nerve (Clic here for more details about this article) |
4/6. Hereditary neuropathy with liability to pressure palsy: fulminant development with axonal loss during military training.Hereditary neuropathy with liability to pressure palsy (HNPP) is characterised by recurrent mononeuropathies following minor trauma. We describe a case of fulminant HNPP beginning on the first day of military physical training. Protracted weakness, muscle atrophy, hand contractures, and multifocal sensory loss developed during a further three weeks of basic training. Nerve conduction changes were typical of HNPP, but without segmental slowing. Electromyographically, there was prominent acute denervation in muscles of the hands and right shoulder. sural nerve biopsy demonstrated tomaculae and remyelination. genetic testing revealed PMP-22 gene deletion. This case report demonstrates that HNPP can present with rapidly progressive peripheral nerve dysfunction and electrophysiological evidence of focal axonal loss.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
5/6. Progressive systemic sclerosis associated with multiple mononeuropathy.BACKGROUND: Progressive systemic sclerosis (PSS) is a chronic connective tissue inflammatory disease which commonly attacks the skin and the visceral organs, but rarely the peripheral nervous system. OBJECTIVE: The aim of this study was to investigate PSS accompanied by peripheral neuropathy clinically, electrophysiologically and pathologically from a sural nerve biopsy. methods: Two women suffering from PSS but without any other collagen disease were studied. Both patients developed peripheral neuropathy with multiple mononeuropathy of the limbs, and in one woman, in the trunk as well. RESULTS: A biopsy of the sural nerve revealed axonal and myelin segmental degeneration, loss of large myelinated fibers and an increase of collagen fibers, but there was no evidence of vasculitis. An electron microscopic examination revealed degenerated axons, disrupted myelin sheaths and multilayered basal lamina in the capillaries. CONCLUSION: Mononeuropathy in PSS suggests that ischemic neuropathy may be related to the immune-mediated vasculopathy.- - - - - - - - - - ranking = 0.33333333333333keywords = nerve (Clic here for more details about this article) |
6/6. Anterior chiasmal optic nerve avulsion.In a case of traumatic avulsion of the optic nerve at the anterior chiasm, MR imaging provided highly specific images of the injury site, including the absence of the optic nerve within the optic canal and the point of transection at the anterior portion of the chiasm. This was confirmed clinically and histopathologically. MR imaging should be considered in cases of suspected chiasmal injury.- - - - - - - - - - ranking = 1keywords = nerve (Clic here for more details about this article) |