Naotaka Aizawa

Floppy epiglottis as a contraindication of CPAP in patients with multiple system atrophy

Patients with multiple system atrophy (MSA) frequently develop sleep-disordered breathing (SDB), which is effectively treated using continuous positive airway pressure (CPAP). We previously demonstrated that patients with MSA developed upper airway obstructions not only at the vocal cords but also at the base of the tongue, the soft palate, and the laryngeal inlet.1 Obstruction of the laryngeal inlet by the epiglottis leads to a condition known as floppy epiglottis (FE), in which the epiglottis is sucked into the laryngeal inlet during inspiration. Although FE in infants is caused by a congenital abnormality in the laryngeal cartilage,2 the mechanism of FE in MSA remains unknown. A recent study has demonstrated that CPAP does not improve the airway patency in patients with congenital FE; instead, it may exacerbate upper airway obstruction by further promoting the downward displacement of the epiglottis into the laryngeal inlet.2 This finding raises the possibility that CPAP could exacerbate SDB in a subset of patients with MSA. To investigate this possibility, we directly observed the effect of CPAP on MSA-associated FE. ### Methods. This study was approved by the institutional ethics committee. Written informed consent …

Maintaining glottic opening in multiple system atrophy: efficacy of serotonergic therapy.

Involuntary muscle contractions began in the right deltoid muscle 2 weeks after onset of pain. Contractions were rhythmic, with sinusoidal and undulating/peristaltic qualities. Both the pain and the deltoid contractions were continuous. The movements continued during sleep, and the patient could not voluntarily stop them. Therapeutic interventions including medications, physical therapy, and surgery did not mitigate the pain or affect the movements. Oral analgesics including oxycodone and antiepileptics including phenytoin and gabapentin all proved ineffective. The patient was treated by chiropractors and physical therapists with no benefit. In 2001, a shoulder spur was removed, and later that year an anterior cervical discectomy with fusion at 3 levels (C4–C5, C5–C6, and C6–C7) was performed. Neither surgery produced benefit. On exam, the patient had no focal motor or sensory deficits. Tendon reflexes were normal. Involuntary movements in the right deltoid were constant, although the amplitude, frequency, and extent of contractions varied. Contraction ‘‘waves’’ were rhythmic and began in the posterior deltoid, swept anteriorly, and gave an ‘‘undulating’’ or peristalsislike appearance. Contraction progression involved more muscle fascicles in the posterior middle-deltoid and was more visible with large amplitude contractions (see Video Supplement). His multiple spine magnetic resonance images revealed progressively worsening cervical spondylosis. Multichannel needle electromyographic (EMG) recording was performed with concentric needle electrodes. Deltoid EMG did not show myokymia or myoclonus. EMG activity underlying the contractions were normal-appearing interference patterns with bursts lasting 400–800 ms. During voluntary shoulder abduction, the bursting temporarily disappeared and was replaced by normal-looking tonic EMG activity. There were no other EMG abnormalities in the upper extremities. The pathophysiology of PLMT is poorly understood and likely heterogeneous. In some cases there is evidence of a lesion in the spinal cord, the cauda equina, the lumbar roots, or the peripheral nerves; the syndrome may also occur after minor limb trauma or without any antecedents. In at least 1 publication on PLMT, it was observed that movements could be suppressed at the patient’s volition. Peripheral nervous injury may produce a spectrum of movement disorders including PLMT, yet such etiology is not always confirmed by routine EMG, similar to our case. In the absence of confirmed peripheral nerve injury, the pathophysiology of these symptoms remains uncertain but likely arises from aberrant spinal cord plasticity. Painful shoulder—moving deltoid syndrome represents a spinal segmental movement disorder possibly analogous to PLMT.

Laryngeal stridor in multiple system atrophy: Clinicopathological features and causal hypotheses

Laryngeal stridor is recognized as a characteristic clinical manifestation in patients with multiple system atrophy (MSA). However, the pathogenic mechanisms underlying this symptom are controversial. Neurogenic atrophy of the posterior cricoarytenoid muscle has been identified in cases of MSA, suggesting that laryngeal abductor weakness contributes to laryngeal stridor. However, dystonia in the laryngeal adductor muscles has also been reported to cause laryngeal stridor. Depletion of serotonergic neurons in the medullary raphe nuclei, which exert tonic drive to activate the posterior cricoarytenoid muscle, has recently been identified in MSA cases. This adds weight to the possibility that laryngeal abductor weakness underlies laryngeal stridor in MSA. Continuous positive airway pressure therapy is currently used in the treatment of laryngeal stridor, but should be used with caution in patients showing contraindications. Current knowledge of the clinical and neuropathological features of laryngeal stridor is summarized in this paper, and the hypothesized causes and possible therapeutic options for this symptom are discussed.

Discontinuation of continuous positive airway pressure treatment in multiple system atrophy.

OBJECTIVE Continuous positive airway pressure (CPAP) is used for the management of sleep-disordered breathing in patients with multiple system atrophy (MSA). However, the long-term outcome after CPAP treatment is yet to be ascertained. METHODS A retrospective study was performed to investigate the frequency and causes of CPAP treatment discontinuation, and to ascertain the determinations of CPAP treatment duration in Japanese patients diagnosed with probable MSA based upon the consensus diagnostic criteria, who were admitted to our hospital from 2001 to 2012. RESULTS Twenty-nine consecutive patients treated with CPAP were analyzed. During the observation period, 19 patients (66%) discontinued CPAP treatment. The median CPAP treatment duration was 13.0 months (range, 1-53 months). The major causes for discontinuation were pulmonary infection, respiratory insufficiency of undetermined origin, and CPAP intolerance. On comparing the clinical characteristics of the groups subjected to short- and long-term CPAP treatment, floppy epiglottis was more frequently observed in the short-term group than in the long-term group (64% vs. 15%; P = 0.015). CONCLUSION The CPAP treatment duration in MSA patients was not long, and floppy epiglottis may be a determinant of the duration of CPAP treatment.

Comprehensive microbiome analysis of tonsillar crypts in IgA nephropathy

Background Immunoglobulin A nephropathy (IgAN) is the most prevalent primary chronic glomerular disease, in which the mucosal immune response elicited particularly in the tonsils or intestine has been estimated to be involved in the development of the disease. To explore the relationship between IgAN and bacterial flora in the tonsils, we conducted a comprehensive microbiome analysis. Methods We enrolled 48 IgAN patients, 21 recurrent tonsillitis (RT) patients without urine abnormalities and 30 children with tonsillar hyperplasia (TH) who had undergone tonsillectomy previously. Genomic DNA from tonsillar crypts of each patient was extracted, and V4 regions of the 16S ribosomal RNA gene were amplified and analysed using a high-throughput multiplexed sequencing approach. Differences in genus composition among the three study groups were statistically analysed by permutational multivariate analysis of variance and visualized by principal component analysis (PCA). Results Substantial diversity in bacterial composition was detected in each sample. Prevotella spp., Fusobacterium spp., Sphingomonas spp. and Treponema spp. were predominant in IgAN patients. The percentage of abundance of Prevotella spp., Haemophilus spp., Porphyromonas spp. and Treponema spp. in IgAN patients was significantly different from that in TH patients. However, there was no significant difference in the percentage of abundance of any bacterial genus between IgAN and RT patients. PCA did not distinguish IgAN from RT, although it discriminated TH. No significant differences in microbiome composition among the groups of IgAN patients according to clinicopathological parameters were observed. Conclusions Similar patterns of bacteria are present in tonsillar crypts of both IgAN and RT patients, suggesting that the host response to these bacteria might be important in the development of IgAN.

Mechanisms and prevention of sudden death in multiple system atrophy.

BACKGROUND Sudden death in multiple system atrophy (MSA) usually occurs during sleep and was therefore attributed to suffocation resulting from vocal cord abductor paralysis, a characteristic laryngeal finding of MSA. This led to the use of tracheostomy and noninvasive positive pressure ventilation (NPPV) for the prevention of sudden death. However, neither method has been able to prevent sudden death, and both have occasionally precipitated treatment-related complications, including central sleep apneas and exacerbation of floppy epiglottis. Therefore, it is important to determine the mechanisms and prevention of sudden death in MSA. METHODS We reviewed the literature on the mechanisms and prevention of sudden death in patients with MSA. RESULTS Sudden death in MSA is hypothesized to be a consequence of disordered central respiration, suffocation caused by sputum and food, upper airway obstruction from NPPV acting on a floppy epiglottis, cardiac autonomic disturbance, or a combination of these factors. CONCLUSION Various factors may be involved in the mechanism of sudden death in MSA. A multidisciplinary approach is needed to prevent sudden death, and this requires an organized system of several medical specialties. Neurologists require a cooperative network that includes experts in otorhinolaryngology, sleep medicine, dysphagia rehabilitation, and cardiology.

Pharyngolaryngeal edema in mumps virus infection: Rare complication

A 12-year-old boy was admitted to hospital with a 2 day history of progressive symptoms, including swelling and tenderness in on the left side of the neck, pain and difficulty in swallowing, and decreased oral intake. On admission, temperature was 37.6°C, respiratory rate was 12 breaths/min, and oxygen saturation was 97% in room air. The left parotid and submandibular glands were swollen significantly, obscuring the angle of the left mandible; therefore, he was clinically diagnosed with mumps. Laryngoscopy showed edematous change of the left pyriform sinus (Fig. 1a). Contrast-enhanced computed tomography showed diffuse edematous changes and fluid accumulation on the left side of the pharyngolaryngeal area (Fig. 1b), and enlargement of the left-sided submandibular glands (Fig. 1c). Although laboratory data were normal except for elevated serum amylase of 196 IU/L (normal range, 44–132 IU/L), the patient was started on i.v. corticosteroid and empirical antibiotics for possible progressive upper airway obstruction caused by bacterial infection. On the third day of admission, swallowing pain was gone and the edematous change of the left pyriform sinus had disappeared (Fig. 1d); therefore, i.v. corticosteroid was discontinued. On the fourth day, he developed mild swelling of the contralateral parotid and submandibular glands in addition to the ipsilateral side. The sequence of symptoms gradually subsided and the patient was discharged without any complications on the sixth day of hospitalization. Mumps infection was confirmed on enzyme immunoassay; positive mumps IgM (3.32) on admission and a 14-fold increase in mumps IgG between acute and convalescent titers, 3 weeks after discharge (4.5 and 64.3, respectively). Pharyngolaryngeal edema is a rare complication previously described in several case reports, including in two adults who required tracheotomy and in a child intubated for progressive symptoms. Several terms have been used to describe this phenomenon, including supraglottic edema, subglottic edema, laryngeal edema, and pharyngolaryngeal edema. The majority of cases were initially suspected as bacterial abscess requiring empiric antimicrobial treatment, which may be continued until the patient stabilizes. In some cases, corticosteroids were effective at reducing the swelling and preventing airway obstruction. It is speculated that infection occurs in adults rather than children given that the previously reported cases of mumps-related pharyngolaryngeal edema were all in adults, except for one case that occurred in a child. The cause of this condition might be lymphatic obstruction, which is considered a cause of mumps-induced presternal swelling, a known rare complication of mumps virus infection. After mumps vaccines became widely available, the incidence of mumps was reduced in many countries. Mumps is highly endemic in Japan, however, because the previous measles, mumps and rubella vaccine was withdrawn from the

Successful transoral surgical closure of a hypopharyngeal pyriform sinus fistula: a case report

Abstract We describe a case of recurrent hypopharyngeal pyriform sinus fistula after an open neck surgery, which was successfully treated with transoral surgical closure. The patient was a 7-year-old boy who had previously undergone neck surgery for removal of a fistula. The fistula recurred one month later, and transoral closure was selected as a treatment. Under general anesthesia, the fistula was identified in the left pyriform sinus, and cotton balls soaked with trichloroacetic acid were inserted in the fistula opening, after which an electrocautery scalpel was used to close the fistula. At 18 months postoperatively, there has been no recurrence or complications. This is the first report to describe a transoral surgical closure of a recurrent fistula. Transoral surgical closure was successful and resulted in no complications; it thus appears to be an effective treatment option, even for patients who have previously undergone open neck surgery.

A Case of Fungal Infection of the Larynx Requiring Differential Diagnosis from Laryngeal Carcinoma

A case of fungal infection of the larynx requiring differential diagnosis from laryngeal carcinoma is reported. A 75-year-old male was referred to our clinic with a complaint of hoarseness, and a mass lesion was detected on the left vocal cord. Biopsy under laryngeal fiberscopy was performed on suspicion of laryngeal carcinoma. Resection and vaporization of the lesion was carried out using a KTP laser, because the intraoperative histopathological diagnosis was dysplasia. The final histopathological diagnosis was revised to dysplasia with an infection of Aspergillus. Additional therapy for the fungal infection was not performed, but no reccurence was seen for 3 years after surgery. The post-vaporization lesion on the vocal cord was covered with intact mucosa. KTP laser was found to be effective in this case.