Oculopharyngeal muscular dystrophy (OPMD) is a late-onset myopathy caused by (GCN) expansions in the polyalanine binding protein nuclear 1 gene (PABPN1) located on chromosome 14q11. This study reports a case of an incomplete clinical characteristics of OPMD with heterozygous (GCN)11 expansion. A fifty-nine-year-old Korean woman was suffering from non-progressive dysarthria, dysphagia for five years. Neurologic findings were unremarkable except for tongue atrophy and mild ptosis. A genetic screening confirmed heterozygous (GCN)11 expansion in the PABPN1 gene.

Oculopharyngeal muscular dystrophy (OPMD) is a late-onset myopathy caused by (GCN) expansions in the polyalanine binding protein nuclear 1 gene (PABPN1) located on chromosome 14q11. This study reports a case of an incomplete clinical characteristics of OPMD with heterozygous (GCN)11 expansion. A fifty-nine-year-old Korean woman was suffering from non-progressive dysarthria, dysphagia for five years. Neurologic findings were unremarkable except for tongue atrophy and mild ptosis. A genetic screening confirmed heterozygous (GCN)11 expansion in the PABPN1 gene.

To learn the developmental changes in intrinsic electrophysiological properties of the second order taste neurons, whole cell recordings from the developing nucleus of the solitary tract neurons were done in brainstem slices of postnatal rats. Rats aged from postnatal 0 to 21 days (P0-P21) were used, being divided into 3 age groups: postnatal first week (P0-P7 days), second week (P8-P14 days), and third week P15-P21 days). Slices containing gustatory NTS were cut horizontally in the thickness of 300 micrometer. Whole cell recordings were obtained from neurons in response to a series of hyperpolarizing and depolarizing current pulses. The intrinsic electrophysiological properties of the rostral NTS (rNTS) neurons were compared among the age groups. Depolarizing current pulses evoked a train of action potentials in all neurons of all age groups. The resting membrane potential and input resistance of the neurons did not show any significant differences during the ostnatal 3 weeks. The time constant, however, decreased during the development. Duration of action potential measured at half maximum amplitude was longer in younger age groups. Both the maximum rate of rise and the maximum rate of fall in the action potential increased during the first 3 weeks postnatal. Electrophysiologically more than half neurons were type III. In summary, it is suggested that developmental changes in electrophysiological properties in rNTS occur during the first three weeks in rats.
Action Potentials ; Animals ; Brain Stem ; Humans ; Membrane Potentials ; Neurons* ; Patch-Clamp Techniques ; Rats* ; Solitary Nucleus

The present study was designed to investigate whether endogenous nitric oxide (EDNO) is involved in submandibular vasodilation and salivation induced by parasympathetic nerve stimulation. Effects of Nw-nitro-L-arginine-methyl ester (L-NAME) which blocks the synthesis of EDNO from L-arginine on the submandibular vasodilation and salivation induced by chorda stimulation or administration of various vasodilators were examined in anesthetized cats. Effect of L-NAME on K+ efflux induced by carbachol was also examined using the excised submandibular slice in vitro. In the submandibular slices, acetylcholine (10(-5) mol/L) or vasoactive intestinal polypeptide (VIP, 10(-5) mol/L) increased NO2 contents, which was prevented by pretreatment with L-NAME. Salivary secretion in response to the chorda stimulation (3 V, 1 msec, 10 ~ 20 Hz) was completely blocked by treatment with atropine (1 mg/kg). Increased blood flow response to the low frequency (1, 2, 5 Hz) stimulation was significantly reduced, whereas the blood flow induced by the higher frequency (10, 20 Hz) stimulation was not affected. Lingual-arterial infusion of L-NAME (100 mg/kg) significantly diminished the vasodilatory and salivary responses to the chorda stimulation at all stimuli frequencies used. Intra-arterial infusion of L-NAME (100 mg/kg) markedly diminished the vasodilatory responses to acetylcholine (5 mug/kg), VIP (5 mug/kg) or bradykinin (5 mug/kg). In the excised submandibular slice, K+ efflux in response to carbachol (10(-5) mol/L) was significantly decrease by pretreatment with L-NAME (10(-5) mol/L). In the isolated submandibular artery precontracted with phenylephrine (10(-5) mol/L), the vasorelaxation induced by ACh (10-7 mol/L) was reversed into a contraction by methylene blue (10(-4) mol/L). These results suggest that EDNO may play an important role in vasodilation and secretion of the submandibular gland.
Acetylcholine ; Animals ; Arginine ; Arteries ; Atropine ; Bradykinin ; Carbachol ; Cats ; Infusions, Intra-Arterial ; Methylene Blue ; NG-Nitroarginine Methyl Ester ; Nitric Oxide* ; Phenylephrine ; Salivary Glands ; Salivation ; Submandibular Gland ; Vasoactive Intestinal Peptide ; Vasodilation ; Vasodilator Agents

OBJECTIVES: To identify diagnostically meaningful differences between Warthin's tumor and malignant masses in the parotid gland by dynamic susceptibility contrast (DSC) MR imaging. METHODS: Eleven malignant parotid tumors and 9 Warthin's tumors were included. MR imaging was performed on all patients. Signal intensity time curves of tumors were obtained by DSC MR imaging and dynamic susceptibility contrast percentages (DSC%) were calculated. RESULTS: No significant difference was observed between malignant tumors and Warthin's tumors (P = 0.437), although DSC% values tended to be higher for Warthin's tumors. CONCLUSIONS: Warthin's tumor tended to have higher DSC% values than malignant parotid tumors, but this difference was not significantly different.
Humans ; Magnetic Resonance Imaging ; Parotid Gland ; Perfusion

Injection laryngoplasty (IL), commonly used to treat unilateral vocal cord paralysis, aims to improve glottic insufficiency, while preventing aspiration and enhancing voice quality by injecting material into the vocal cords. Moreover, recent advancements in endoscopic technology and biomaterials facilitated this procedure in the office setting under local anesthesia. IL is generally considered a safe procedure due to its minimally invasive nature. However, as clinical cases increase, reports of various complications associated with IL are also on the rise. These complications are typically not serious and they improve spontaneously with follow-up. However, they can often lead to serious issues affecting the patient’s safety and function, requiring active treatment or management. In this article, we will review possible complications that may arise during and after IL.