1/3. Congenital sensory neuropathy. Ophthalmological implications.The authors examined a patient presenting with congenital sensory neuropathy with selective loss of small myelinated nerve fibres. The appearance of (bilaterial) keratitis or corneal ulceration in early childhood is strongly suggestive of congenital corneal anaesthesia. Concomitant symptoms such as anisocoria, abnormal pupillary reaction, diminished tear production and disturbed sensibility to pain and temperature point to a generalized disease: one of the hereditary sensory and autonomic neuropathies. In order to establish a definite diagnosis, elaborate neurological examination, including ultrastructural study of a muscle-nerve biopsy, is required. Tarsorrhaphy, therapeutic flushfitting PMMA scleral lenses and hydrophilic HEMA contact lenses are advocated, in order to protect the cornea. The results with high-water-content hydrophilic contact lenses are promising, those of keratoplasty limited.- - - - - - - - - - ranking = 1keywords = anaesthesia (Clic here for more details about this article) |
2/3. Hereditary neuropathy with liability to pressure palsies and anaesthesia: peri-operative nerve injury.A 43-year-old female with carcinoma of the left breast underwent wide local excision of the tumour and sentinel lymph node biopsy under general anaesthesia. Three lymph nodes were removed uneventfully during the operation. Postoperatively, the patient complained of weakness and decreased sensation of her left arm. A diagnosis of peri-operative neuropraxia was made. This resolved completely over the following 4 weeks. genetic testing confirmed a diagnosis of hereditary neuropathy with liability to pressure palsies.- - - - - - - - - - ranking = 5keywords = anaesthesia (Clic here for more details about this article) |
3/3. Proposal for a standardized protocol for the systematic orofacial examination of patients with Hereditary Sensory Radicular Neuropathy.AIM: To apply a standardized protocol for the orofacial evaluation of two adult siblings (one male and one female) with Hereditary Sensory Radicular Neuropathy (HSRN) that presented with dental problems. SUMMARY: The systematic evaluation consisted of (a) clinical questionnaire; (b) radiographs [orthopantomography and computarized tomography (CT)]; (c) orofacial psychophysical tests (pain, thermal, mechanical and electrical sensation); and (d) histology of gingiva and pulp (optical and transmission electronic microscopy). The female patient had complete insensitivity to orofacial pain and partial facial heat sensitivity, and received dental treatment without anaesthesia or pain. She had a severe and painless jaw infection due to pulp necrosis in tooth 37. The male patient had partial insensitivity to orofacial pain and required anaesthesia for dental treatment. Histological examination of gingivae and pulpal tissue revealed an altered proportion of unmyelinated and myelinated sensory nerve fibres. KEY learning POINTS: * patients with HSRN may present with significant, silent dental disease. * A standard protocol is helpful when evaluating such patients. * If the opportunity arises, evaluation of pulp tissue may reveal an altered proportion of myelinated and unmyelinated nerve fibres. This may avoid the more estabilished sural nerve biopsy.- - - - - - - - - - ranking = 2keywords = anaesthesia (Clic here for more details about this article) |