[Case report of dentatorubral pallidoluysian atrophy in a patient on a ketogenic diet].

We report a 16-year-old woman with dentatorubral pallidoluysian atrophy (DRPLA) on a ketogenic diet. She was scheduled for dental treatment under general anesthesia. She was diagnosed as having DRPLA at the age of 10. Medication included clonazepam, chlordiazepoxide, ethosuximide, sodium valproate and piracetam. She had been placed on a ketogenic diet at the age of 15, and seizures decreased. Preoperative laboratory data were normal except for serum cholesterol level (228 mg x dl(-1)) and blood ketones (1.8 mmol x l(-1)) with the use of Medisense Xtra. General anesthesia was induced using thiamylal, vecuronium, sevoflurane and maintained with sevoflurane and nitrous oxide-oxygen. Fluid infusion employed Solita T1 (glucose content of 2.6%: total 150 ml). Operation and general anesthesia presented no problems. We continued to infuse Solita T1 (total 350ml) for about 4 hours postoperatively. Ketogenic diet and additional medicine were started after 5 hours postoperatively. We measured perioperative and postoperative BS and blood ketones with the use of Medisense Xtra. She recovered from anesthesia without any significant complication.

Immunohistochemical localization of SNARE proteins in dental pulp and periodontal ligament of the rat incisor.

Distribution of three soluble N-ethylmaleimide-sensitive fusion protein attachment protein receptor (SNARE) proteins, syntaxin-1, synaptosomal-associated protein of 25 kDa (SNAP-25), and vesicle-associated membrane protein-2 (VAMP-2), was examined in dental pulp and periodontal ligament of the rat incisor. In the trigeminal ganglion, syntaxin-1 and SNAP-25 immunoreactivity was predominately detected in medium- to large-sized neurons. Most syntaxin-1 immunoreactive neurons expressed SNAP-25. In contrast, VAMP-2 was localized in small- to medium-sized neurons and in slender-shaped cells surrounding SNAP-25-immunopositive neurons. When the inferior alveolar nerve, one of the mandibular nerve branches innervating the dental pulp and periodontal ligament, was ligated, SNARE proteins accumulated at the site proximal to the ligation. In the incisor dental pulp, all nerve fibers displayed immunoreactivity for syntaxin-1, SNAP-25, and VAMP-2. In the periodontal ligament of the incisor, almost all nerve fibers displayed both syntaxin-1 and SNAP-25 immunoreactivity, but lacked VAMP-2 immunoreactivity. SNAP-25 protein expression was localized around the vesicle membranes at the axon terminal of the periodontal mechanoreceptors. These present data suggest that these three SNARE proteins are synthesized at the trigeminal ganglion, transported centrally and peripherally, and expressed in sensory endings where apparent synapses are not present. Because those proteins participate in docking and exocytosis of synapse vesicles in the central nervous system, they might also contribute to vesicle exocytosis at receptive fields where apparent synapses are not present.