Post-operative swallowing in multiple system atrophy.

BACKGROUND: Some patients with multiple system atrophy (MSA) require surgical interventions such as tracheostomy and aspiration prevention. Few studies have investigated the postoperative clinical course of MSA patients. The aim of this study was to determine a management strategy for dysphagia and respiratory disorder in MSA. METHODS: From 2001 to 2014, 18 MSA patients (13 males and 5 females, 52-76 years) underwent tracheostomy (TR, n = 11) or laryngeal closure (LC, n = 12). Five patients underwent LC following TR. Vocal fold impairment, the degree of dysphagia and pre/post-operative oral ingestion, and postoperative survival time were evaluated retrospectively. Swallowing function was assessed using the penetration aspiration scale (PAS). RESULTS: TR was performed due to respiratory disorder in seven patients and due to dysphagia in four patients. PAS scores ranged 1-8 in TR patients and 7-8 in LC patients. Seven of 11 patients who underwent TR displayed worsened PAS scores, and no patients displayed improved PAS scores following TR. All patients who underwent LC regained complete or partial oral intake after surgery. There were no significant differences in postoperative survival time between the two groups. CONCLUSIONS: Considering the impacts of TR and LC on survival time, postoperative feeding and swallowing, LC is a good option for treating MSA patients with dysphagia.

A full digital, high definition video system (1080i) for laryngoscopy and stroboscopy.

OBJECTIVE: This study aimed to estimate the effectiveness of a full digital, high definition video system for laryngeal observations. METHODS: A newly available, full digital, high definition video camera and high definition video monitor were used. With an endoscopic adaptor and rigid telescope, laryngoscopy and stroboscopy were performed on patients with various kinds of laryngeal lesions. RESULTS: All laryngeal lesions were observed and recorded by the full digital, high definition video camera without incident. The image quality for laryngoscopy and stroboscopy was far superior to that of a conventional video system, including video-endoscopy. Even tiny structures or lesions could clearly be visualised on the monitor. The still image obtained from the full digital, high definition video camera was 1920 x 1080 pixels and was comparable to that obtained from a still camera. CONCLUSIONS: Full digital, high definition video cameras are now commonplace products and can easily be applied to patients with laryngeal disorders. They provide superior laryngeal images, compared with conventional video systems. Furthermore, high definition video systems are cheaper than proprietary medical video systems. We consider our system to represent an accessible technique of gaining superior laryngeal observation in otolaryngological clinics.

Head torsion technique for detailed observation of larynx and hypopharynx.

We introduce here an easy but effective method for detailed observation of the larynx and hypopharynx. During the endoscopic observation, the patient's head is turned to one side. Anatomical structures on the same side of the endolarynx, such as the laryngeal ventricle and inferior surface of the vocal fold, are easily observed. In addition, observation of the opposite side of the hypopharynx also becomes easier. Such head turning is also useful in patients with an oblique larynx, in whom the epiglottis obstructs insertion of the endoscope. This is a simple but very effective technique for laryngeal and hypopharyngeal observation.

Use of percutaneous cardiopulmonary support in catastrophic massive pulmonary fat embolism.

We report a patient who sustained massive catastrophic pulmonary fat embolism while undergoing surgery for intramedullary nail of femur. Application of percutaneous cardiopulmonary support, in our view, saved the patient from cardiopulmonary collapse.

A mouse model of pemphigus vulgaris by adoptive transfer of naive splenocytes from desmoglein 3 knockout mice.

BACKGROUND: Pemphigus vulgaris (PV) is an autoimmune blistering disease caused by antidesmoglein3 (anti-Dsg3) IgG autoantibodies. Recently, we developed a PV mouse model by adoptive transfer of splenocytes from recombinant Dsg3-immunized Dsg3(-/-) mice to Rag2(-/-) immunodeficient mice that expressed Dsg3. OBJECTIVES: We determined whether the adoptive transfer of naive splenocytes from nonimmunized Dsg3(-/-) mice induces the anti-Dsg3 IgG production and the PV phenoytpe in recipient mice. METHODS: We adoptively transferred naive Dsg3(-/-) splenocytes into Rag2(-/-) mice and compared their PV phenoytpe with those mice receiving immunized Dsg3(-/-) splenocytes. The numbers of splenocytes and their subpopulations required for anti-Dsg3 IgG production were examined. RESULTS: Mice that received naive Dsg3(-/-) splenocytes produced anti-Dsg3 IgG, which bound to keratinocyte cell surfaces in vivo, and developed the PV phenotype, including oral erosions with suprabasilar acantholysis. Antibody production and the appearance of the PV phenotype were delayed by approximately 2 weeks in mice that received naive splenocytes compared with mice that received immunized splenocytes. However, once the PV phenotypes developed, there were no apparent differences in disease severity between the two models. Interestingly, the anti-Dsg3 IgG titres were significantly lower in mice that received naive splenocytes than in mice that received immunized splenocytes, suggesting that the former antibodies were more potent than the latter. The frequency of anti-Dsg3 IgG production depended on the number of transferred naive splenocytes. Both CD4+ T cells and B220+ B cells from naive Dsg3(-/-) mice were essential for the production of anti-Dsg3 IgG antibodies. CONCLUSIONS: Dsg3-specific naive lymphocytes in Dsg3(-/-) mice can be primed and activated by the endogenous Dsg3 in recipient mice to produce pathogenic anti-Dsg3 IgG without active immunization. This approach using naive lymphocytes provides a unique model to dissect immunological mechanisms of tolerance against peripheral autoimmune targets.

Autologous transplantation of fascia into the vocal fold: long-term results of a new phonosurgical technique for glottal incompetence.

OBJECTIVES: To study the long-term results of autologous transplantation of fascia into the vocal fold, and to evaluate our use of autologous transplantation instead of bovine collagen injection in cases of glottal incompetence. STUDY DESIGN: Retrospective study of the patients who have undergone autologous fascia transplantation using our new technique. METHODS: Follow-up studies were performed for at least 1 year (up to 3 y) on 9 autologous fascia transplant patients (6 cases with type 1 procedures and 3 cases with type 2 procedures). Clinical observations, including laryngeal stroboscopy, and measurement of maximum phonation time (MPT) were carried out. RESULTS: During 3 months after autologous fascia transplantation, MPT gradually increased and stroboscopy showed improved glottal closure. These improvements continued beyond 1 year in all cases of type 1 surgery and 2 of 3 cases of type 2 surgery. CONCLUSIONS: Autologous transplantation of fascia into the vocal folds as a phonosurgical treatment for glottal incompetence yields excellent long-term results. Temporal fascia appears to be a highly suitable tissue for transplantation in Reinke's space. However, the fascia is less suitable for transplantation in the muscle. We speculate that transplantation of temporal fascia leads to regeneration of vocal fold tissue, perhaps using a mechanism similar to stem cell transplantation in other organs.

Nonsense mutation of islet-1 gene (Q310X) found in a type 2 diabetic patient with a strong family history.

Islet-1 (Isl-1) is one of the transcription factors that play an important role for the formation of the islet cells. We scanned the Isl-1 gene in 77 Japanese type 2 diabetic patients with a family history and found a heterozygous nonsense mutation (Q310X) in 1 diabetic patient. The mutation was not found in 180 nondiabetic subjects. This mutation is located in the putative transactivation domain and deletes 40 amino acids of the COOH-terminal lesion. The Q310X mutant exhibited a 50% reduction in activity compared with the wild-type when tested for stimulation of transcription of a human amylin promoter-linked luciferase reporter gene in betaTC3 cells. The patient was a 49-year-old nonobese man who was diagnosed as having type 2 diabetes at 32 years of age and has been treated with sulfonylureas. The mutation was found in his mother, who has type 2 diabetes, and in his 14-year-old daughter, who has normal glucose tolerance but a relatively low insulin response. This is the first reported finding of Isl-1 gene mutation in type 2 diabetes. Although Isl-1 is not a common predisposing gene for Japanese type 2 diabetes, the mutation in this gene may be a rare cause of diabetes in isolated families.

Fentanyl improves analgesia but prolongs the onset of axillary brachial plexus block by peripheral mechanism.

UNLABELLED: We evaluated the effects of fentanyl added to lidocaine for axillary brachial plexus block in 66 adult patients scheduled for elective hand and forearm surgery. In this double-blinded study, all patients received 40 mL of 1.5% lidocaine with 1:200,000 epinephrine, injected into the brachial plexus sheath using the axillary perivascular technique, and they were randomized into three groups. Group 1 was given lidocaine containing 2 mL of normal saline plus 2 mL of normal saline IV. Patients in Group 2 received lidocaine containing 100 microg fentanyl plus 2 mL of normal saline IV. Group 3 patients received lidocaine containing 2 mL of normal saline plus 100 microg fentanyl IV. Sensory and motor blockade were evaluated by using a pinprick technique and by measuring the gripping force, respectively. The success rate of sensory blockade for radial and musculocutaneous nerves and the duration of the sensory blockade significantly increased in Group 2 (323 +/- 96 min) as compared with Group 1 (250 +/- 79 min). However, onset time of analgesia was prolonged in every nerve distribution by adding fentanyl to brachial plexus block. IV fentanyl had no effect on the success rate, onset, or duration of blockade. We conclude that the addition of fentanyl to lidocaine causes an improved success rate of sensory blockade but a delayed onset of analgesia, although this may be accounted for by the decreased pH caused by the fentanyl. IMPLICATIONS: It is still unclear whether the addition of a peripheral opioid is useful for nerve blockade in humans. Peripheral application of fentanyl to lidocaine for axillary brachial plexus blockade in this study provided an improved success rate of sensory blockade and prolonged duration.

Hoarse voice resulting from premature ageing in Werner's syndrome.

Werner's syndrome is characterized by clinical signs of premature ageing. A 42-year-old man presented with three-year history of hoarseness. Also noted were skin atrophy of the face and hands, ulcerations around the ankles, and a history of cataracts. A clinical diagnosis of Werner's syndrome was made. Laryngoscopy revealed bowed vocal folds resulting in a spindle-shaped defect with glottal incompetence during phonation. Examination also revealed decreased maximum phonation time and vocal fatigue. At surgery, atrophy of the vocalis muscle was noted. Furthermore, degeneration of muscle fibres was noted in the temporalis muscle. The atrophic changes in the vocal folds that occur with ageing and result in an increased fundamental frequency were seen in this patient. The characteristic hoarseness of Werner's syndrome appears to be the result of premature ageing of the vocal-folds.

Immunohistochemical expression of Ets-1 transcription factor and the urokinase-type plasminogen activator is correlated with the malignant and invasive potential in meningiomas.

BACKGROUND: The Ets-1 transcription factor has been proposed to play an important role in the invasive process of tumor cells through the induction of urokinase-type plasminogen activator (u-PA). METHODS: Because meningiomas are potentially invasive tumors, irrespective of their malignancy grades, the authors immunohistochemically investigated Ets-1 and u-PA expression in tissues obtained from 50 benign (16 meningotheliomatous, 14 fibrous, 13 transitional, 6 angiomatous, and 1 microcystic), 4 atypical, and 6 anaplastic meningiomas and correlated their results with the malignancy and invasive potential. RESULTS: Ets-1 protein was expressed in 19 of the benign meningiomas (38%)whereas 31 (62%) were u-PA positive. The percentage of positive cells frequently was < 50%. In contrast, Ets-1 and u-PA expression was observed in all 4 atypical (100%) and all 6 anaplastic (100%) cases, respectively. The proportion of cells positive for Ets-1 and u-PA frequently were > or = 50%. A significant difference was observed between Ets-1 and u-PA expression in benign and high grade meningiomas (P < 0.0001). Moreover, Ets-1 expression was found to correlate significantly with u-PA positivity in meningiomas (P < 0.0001). Twenty-one of 60 meningioma cases (35%) showed infiltration either to the brain, dura mater, or bone. Eighteen of these 21 cases (85.7%) were positive for Ets-1 and u-PA. Ets-1 and u-PA positivity was found to correlate well with the invasive phenotype in meningiomas (P < 0.001). CONCLUSIONS: The findings of the current study support the possibility that the Ets-1 transcription factor, through u-PA induction, may be involved in the invasive process in meningiomas.

Sudden onset aphonia caused by a Japanese-style bath.

An 86-year-old man was referred by his family physician to our clinic because of sudden onset aphonia immediately after a Japanese-style bath. On examination, the only abnormality was aphonia, with an otherwise normal physical examination. However, we found vocal fold oedema on laryngeal fibrescopy. For treatment, he was immediately given 30 mg prednisolone intravenously. Twelve hours after injection the oedema had completely disappeared and his aphonia had resolved. The patient was healthy for three months after returning home. However, at the beginning of a cold winter night he again complained of sudden onset hoarseness, after taking a Japanese-style bath. Japanese-style baths are completely different from Western-style baths. There is a temperature difference of almost 30 degrees C between the inside and outside of a bath; the transition may represent a type of physical exercise in elderly and exhausted individuals. This difference could cause a cold or heat-induced allergic reaction. We strongly recommend a laryngeal study in case of sudden onset aphonia.

Proximal row carpectomy versus limited wrist arthrodesis for advanced Kienböck's disease.

The outcomes in 20 patients with advanced Kienböck's disease treated by proximal row carpectomy (seven patients) or limited wrist arthrodesis (13 patients) were reviewed retrospectively. Postoperatively, the results were more satisfactory in terms of wrist pain, the range of wrist flexion-extension, and grip strength following limited wrist arthrodesis than after proximal row carpectomy, although the differences were not statistically significant. We recommend scaphotrapeziotrapezoid arthrodesis in selected patients with advanced Kienböck's disease who have a fragmented lunate.

A rare case of laryngeal myxoma.

We report a rare case of laryngeal myxoma in a 57-year-old Japanese man. Except for a five-year history of gradually progressive hoarseness, he had been in good health. Video-stroboscopic examination revealed a solid mass in the anterior third of the right vocal fold. Phonosurgery performed with a microscope showed that the mass was encapsulated and located between the epithelium and vocal fold ligaments of the right vocal fold. This hard, elastic mass which measured 7 mm in diameter, was diagnosed as a myxoma. Only three cases of myxoma of the larynx have been reported in the English literature, with only one other case involving the vocal fold.

Stroboscopic observation of the larynx after radiation in patients with T1 glottic carcinoma.

We studied laryngeal video stroboscopy (LVS) system for evaluation of patients with glottic carcinoma (T1N0M0) before and after radiotherapy. There were 10 patients with T1 glottic squamous cell carcinoma (9 men and 1 woman) who received radiotherapy at the Hitachi General Hospital. We performed LVS before and after radiotherapy. The presence or absence of mucosal waves (MW) was particularly noted. No MW were present before radiotherapy but at 1-6 months after, MW gradually appeared. One year after radiotherapy all patients showed MW on LVS. In patients with glottic carcinoma MW recovered after radiation therapy. LVS may be useful for the clinical follow-up of post-radiation patients for early detection of recurrence of glottic carcinoma.

Triquetrohamate impaction syndrome: a case report.

A patient who had suffered from ulnar wrist pain for 3 years was diagnosed arthroscopically as having triquetrohamate impaction syndrome. Improvement of symptoms was obtained by arthroscopic treatment. This syndrome and the use of midcarpal arthroscopy are discussed.