Early-onset palatal myoclonus in Wernekinck commissure syndrome secondary to caudal paramedian midbrain infarction: A case report and a mini review of the literature.

INTRODUCTION: Wernekinck commissure syndrome (WCS) is an extremely rare midbrain syndrome, which selectively destroys the decussation of the superior cerebellar peduncle and the central tegmental tract, which commonly presents with bilateral cerebellar ataxia, dysarthria, and internuclear ophthalmoplegia. Palatal myoclonus in Wernekinck commissure syndrome is uncommon and often occurs as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei. MATERIAL AND METHOD: A patient with WCS, admitted to our hospital from December 2023, was chosen for this study, and the syndrome's clinical manifestations, imaging features, and etiology were retrospectively analyzed based on the literature. A 68-year-old right-handed East Asian man presented with dizziness, slurred speech, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. He had several risk factors for ischemic cerebrovascular diseases (age, sex, dyslipidemia, hypertension and smoking history). Brain magnetic resonance imaging showed hyperintensity of DWI and hypointensity of ADC at the caudal midbrain which was around the paramedian mesencephalic tegmentum anterior to the aqueduct of midbrain. RESULTS: He was diagnosed with Wernekinck commissure syndrome (WCS) secondary to caudal paramedian midbrain infarction. He was started on dual antiplatelet therapy (aspirin and clopidogrel) and intensive statin therapy. Blood pressure and glucose were also adjusted. His symptoms improved rapidly, and he walked steadily and speak clearly after 7 days of treatment. CONCLUSIONS: Palatal myoclonus is known to occur as a late phenomenon due to hypertrophic degeneration of bilateral inferior olivary nuclei. However, Our case suggests that palatal myoclonus can occur in the early stages in WCS.

Differences in the Tensor Veli Palatini Between Adults With and Without Cleft Palate Using High-Resolution 3-Dimensional Magnetic Resonance Imaging.

OBJECTIVE: To investigate the dimensions of the tensor veli palatini (TVP) muscle in adults with and without cleft palate. DESIGN: Prospective study. PARTICIPANTS: There were a total of 14 adult participants, 8 noncleft and 6 with cleft palate. METHODS: Analysis and comparison of the TVP muscle and surrounding structures was completed using 3D MRI data and Amira 5.5 Visualization Modeling software. TVP muscle volume, hamular process distance, mucosal thickness, TVP muscle length, and TVP muscle diameter were used for comparison between participant groups based upon previous research methods. RESULTS: Mann-Whitney U tests revealed a significantly smaller ( U < .001, P = .002) TVP muscle volume in the cleft palate group (median = 536.22 mm3) compared to individuals in the non-cleft palate group (median = 895.19 mm3). The TVP muscle was also significantly shorter ( U = 1.00, P = .003) in the cleft palate group (median = 18.04 mm) versus the non-cleft palate (median = 21.18 mm). No significant differences were noted for the other measured parameters. CONCLUSION: Significant differences in the TVP muscle volume and length among the noncleft participants found in this study may insights into the reported increased incidence of otitis media with effusion (OME) seen in the cleft population. Results from this study contribute to our understanding of the underlying anatomic differences among individuals with cleft palate.

Fibrosis impairs the formation of new myofibers in the soft palate after injury.

Muscle repair is a crucial component of palatoplasty but little is known about muscle regeneration after cleft palate repair. We hypothesized that the formation of new myofibers is hampered by collagen accumulation after experimental injury of the soft palate of rats. One-millimeter excisional defects were made in the soft palates of 32 rats. The wound area was evaluated after 3, 7, 28, and 56 days using azocarmine G and aniline blue to stain for collagen and immunohistochemistry to identify myofibroblasts and to monitor skeletal muscle differentiation. To evaluate age effects, 16 unwounded animals were evaluated at 3 and 56 days. Staining was quantified by image analysis, and one-way ANOVA was used for the statistical analysis. At day 56, the area percentage of collagen-rich tissue was higher in the injured palatal muscles (46.7 ± 6.9%) than in nonwounded controls (15.9 ± 1.0%, p < 0.05). Myofibroblasts were present in the injured muscles at days 3 and 7 only. The numbers of proliferating and differentiating myoblasts within the wound area were greater at day 7 (p < 0.05), but only a few new myofibers had formed by 56 days. No age effects were found. The results indicate that surgical wounding of the soft palate results in muscle fibrosis. Although activated satellite cells migrated into the wound area, no new myofibers formed. Thus, regeneration and function of the soft palate muscles after injury may be improved by regenerative medicine approaches.

Vim thalamotomy in a patient with Holmes' tremor and palatal tremor - Pathophysiological considerations.

BACKGROUND: We peformed a ventral intermediate nucleus (Vim) thalamotomy in a patient with Holmes' tremor and palatal tremor. The frequencies of these movement disorders were 4 Hz and 3 Hz, respectively. Vim thalamotomy stopped the Holmes' tremor but not the palatal tremor. Our observations suggest different mechanisms for these two involuntary movements. CASE PRESENTATION: A 57-arm 11 months after a pontine hemorrhage. Transoral carotid ultrasonography revealed periodic motion of her posterior pharyngeal wall with a frequency of 3 Hz. Recording of neuronal activities in the thalamus revealed a 4Hz rhythmic discharge time that was associated with her tremor in the contralateral arm. A left Vim thalamotomy was performed. The resting tremor of the upper limb stopped, but the kinetic tremor recurred 6 months after the thalamotomy. No effect was observed on her palatal tremor. CONCLUSIONS: The different effects of Vim thalamotomy on the Holmes' tremor and palatal tremor suggest different oscillation sources for these two involuntary movements.

The upper airway in sleep-disordered breathing: UA in SDB.

Sleep disordered breathing (SDB) is a common condition and could be a risk factor for cardiovascular morbidity and mortality. However, the pathogenesis of SDB remains to be elucidated. In general, SDB is divided into two forms, obstructive and central sleep apnea (OSA and CSA, respectively). OSA results from the sleep-related collapse of the upper airway (UA) in association with multiple factors like race, gender, obesity and UA dimensions. CSA primarily results from a fall in PaCO2 to a level below the apnea threshold during sleep through the reflex inhibition of central respiratory drive. It has been reported that UA alterations (i.e., collapse or dilation) can be observed in CSA. This review highlights the roles of the UA in the pathogenesis and pathophysiology of SDB.

Psychogenic palatal tremor may be underrecognized: reappraisal of a large series of cases.

BACKGROUND: Palatal tremor is characterized by rhythmic movements of the soft palate and can be essential or symptomatic. Some patients can have palatal movements as a special skill or due to palatal tics. Psychogenic palatal tremor is recognized but rarely reported in the literature. METHODS: We retrospectively evaluated all patients with palatal tremor seen in our center over a period of 10 years. RESULTS: Of 17 patients with palatal tremor, we identified 10 patients with isolated palatal tremor. In 70% of those the diagnosis of psychogenic palatal tremor could be made. Of the remainder, 2 had palatal tics and 1 essential palatal tremor. CONCLUSIONS: We suggest that psychogenic palatal tremor may be underrecognized and propose that targeted clinical examination of positive signs for psychogenic movement disorders in these patients is essential. The correct identification of such patients has important clinical and scientific implications.

A clockwork ear.

Objective tinnitus refers to a tinnitus that can also be heard by the examiner. It is a relatively rare condition, and can be misdiagnosed or neglected. Some causes of objective tinnitus are head and neck vascular malformations, or muscular myoclonus of the tensor tympani, stapedial, or palatal muscles. The case of an 11-year-old girl with an objective tinnitus lasting from 1 year is herein presented, and the diagnostic workup performed in this unusual case is described.

New insights into mechanism of Eustachian tube ventilation based on cine computed tomography images.

There is debate concerning the mechanism of Eustachian tube (ET) ventilation. While a mechanism of complete opening has been advocated previously, sequential contraction of the levator veli palatini and medial pterygoid muscles followed by the tensor veli palatini and lateral pterygoid muscles may produce a transient sequential opening mechanism, allowing an air bolus to traverse the ET. This may explain confusion surrounding sonotubometry reports that not every swallow leads to sound passage in normal subjects. We hypothesize that the ET may not need to open completely when ventilating the middle ear; rather, a discrete air bolus can pass through it. Five normal and five disordered subjects underwent low-radiation dose cine computed tomography (CT) scans of the ET. Sixteen contiguous 2.5 mm slice locations were chosen through a 4 cm area in the nasopharynx that were parallel to and encompassed the entire ET. Twelve images were acquired at each slice over 4.8 s during swallowing and other tasks. Serial images were analyzed. An air bolus was observed passing through the ET in the normal subjects, but not the subject with ET dysfunction. Medial and lateral pterygoid contractions were also observed. A new hypothetical mechanism of transient sequential ET ventilation is presented. This is not a definitive conclusion, as the number of scans taken and maneuvers used was limited. Improved understanding of ET ventilation may facilitate management of middle ear disease as treatment evolves from ventilatory tube placement to ET manipulation.

Functional aspects of the Eustachian tube by means of 3D-modeling.

The extent of dysfunction of the Eustachian tube (ET) is relevant in understanding the pathogenesis of secondary otological diseases such as acute or chronic otitis media. The underlying mechanism of ET dysfunction remains poorly understood except for an apparent genesis such as a nasopharyngeal tumor or cleft palate. To better describe the ET, its functional anatomy, and the biomechanical valve mechanism and subsequent development of diagnostic and interventional tools, a three-dimensional model based on thin-layer histology was created from an ET in this study. Blackface sheep was chosen as a donor. The 3-D model was generated by the coherent alignment of the sections. It was then compared with the cone-beam computed tomography dataset of the complete embedded specimen taken before slicing. The model shows the topographic relation of the individual components, such as the bone and cartilage, the muscles and connective tissue, as well as the lining epithelium with the lumen. It indicates a limited spiraling rotation of the cartilaginous tube over its length and relevant positional relationships of the tensor and levator veli palatine muscles.

Methods for increasing upper airway muscle tonus in treating obstructive sleep apnea: systematic review.

OBJECTIVE: Treatment of obstructive sleep apnea (OSA) using methods for increasing upper airway muscle tonus has been controversial and poorly reported. Thus, a review of the evidence is needed to evaluate the effectiveness of these methods. DESIGN: The design used was a systematic review of randomized controlled trials. DATA SOURCES: Data sources are from the Cochrane Library, Medline, Embase and Scielo, registries of ongoing trials, theses indexed at Biblioteca Regional de Medicina/Pan-American Health Organization of the World Health Organization and the reference lists of all the trials retrieved. REVIEW METHODS: This was a review of randomized or quasi-randomized double-blind trials on OSA. Two reviewers independently applied eligibility criteria. One reviewer assessed study quality and extracted data, and these processes were checked by a second reviewer. The primary outcome was a decrease in the apnea/hypopnea index (AHI) of below five episodes per hour. Other outcomes were subjective sleep quality, sleep quality measured by night polysomnography, quality of life measured subjectively and adverse events associated with the treatments. DATA SYNTHESIS: Three eligible trials were included. Two studies showed improvements through the objective and subjective analyses, and one study showed improvement of snoring, but not of AHI while the subjective analyses showed no improvement. The adverse events were reported and they were not significant. CONCLUSIONS: There is no accepted scientific evidence that methods aiming to increase muscle tonus of the stomatognathic system are effective in reducing AHI to below five events per hour. Well-designed randomized controlled trials are needed to assess the efficacy of such methods.

Effects of velopharyngeal dysfunction on middle ear of repaired cleft palate patients.

OBJECTIVE: Cleft palates are strongly associated with the development of otitis media due to the anatomic and functional defect of the soft palate musculature and the associated alterations of velopharyngeal muscle insertion on tubal cartilage, or even intrinsic alterations of the cartilage, which affects eustachian tube function. This study will assess velopharyngeal muscle adequacy after palatoplasty through videonasoendoscopy and verify if there is a correlation with otologic status. DESIGN: Transversal study. SETTING: Otorhinolaryngology and cleft palate outpatient service of the Hospital de Clínicas de Porto Alegre (HCPA), Porto Alegre, Brazil. PATIENTS: Seventy-three patients with cleft palate or cleft lip and palate between the ages of 6 and 12 years who had already undergone palatoplasty. INTERVENTIONS: Videonasoendoscopy for evaluation of velopharyngeal function and videotoscopy to assess middle ear status. MAIN OUTCOME MEASURES: Severity scale for videonasoendoscopic and videotoscopic findings. RESULTS: There was no significant correlation between the videonasoendoscopic and the videotoscopic scores in the population studied. DISCUSSION AND CONCLUSIONS: Intrinsic defects of the eustachian tube cartilage and of the insertion of the velopharyngeal muscles seem to contribute to the evolution of otitis media in patients with cleft palate, in addition to the actual defect of the soft palate. There was no correlation between the severity of the otoscopic findings and the degree of velopharyngeal dysfunction.

Functional anatomy of the soft palate applied to wind playing.

Wind players must be able to sustain high intraoral pressures in order to play their instruments. Prolonged exposure to these high pressures may lead to the performance-related disorder velopharyngeal insufficiency (VPI). This disorder occurs when the soft palate fails to completely close the air passage between the oral and nasal cavities in the upper respiratory cavity during blowing tasks, this closure being necessary for optimum performance on a wind instrument. VPI is potentially career threatening. Improving music teachers' and students' knowledge of the mechanism of velopharyngeal closure may assist in avoiding potentially catastrophic performance-related disorders arising from dysfunction of the soft palate. In the functional anatomy of the soft palate as applied to wind playing, seven muscles of the soft palate involved in the velopharyngeal closure mechanism are reviewed. These are the tensor veli palatini, levator veli palatini, palatopharyngeus, palatoglossus, musculus uvulae, superior pharyngeal constrictor, and salpingopharyngeus. These muscles contribute to either a palatal or a pharyngeal component of velopharyngeal closure. This information should guide further research into targeted methods of assessment, management, and treatment of VPI in wind musicians.

Electrophysiological Evidence for Functional (Psychogenic) Essential Palatal Tremor.

Background: There is little published work describing the electrophysiological characteristics of essential palatal tremor, a condition now believed by many to be a functional (psychogenic) movement disorder. Case Report: Here we combine electroencephalography and electromyography with time-locked video recordings to document two cases of essential palatal tremor in which a definitive diagnosis is achieved using these electrophysiological tools. Discussion: We believe that sharing how these objective tools can be used to diagnose a functional movement disorder, as well as providing more published evidence to support the functional origin of essential palatal myoclonus, will help to diagnose this condition in the future.

Isolated Ear Clicks with Partial Voluntary Control.

BACKGROUND: Ear click is a rare type of objective tinnitus, classically described with associated palatal tremor/myoclonus (PT). CASE REPORT: A 15-year-old boy reported a constant bilateral ear clicking for 4 years, that could be stopped at will for a few seconds. Clinically, the ear clicks were audible without visible eardrum or palatal movement, and could be entrained by the examiner. Brain MRI was normal. DISCUSSION: We propose to classify this as isolated ear clicks with partial voluntary control, putting it into context with other subcategories of "essential" or "isolated" PT.

Functional characterization of extrinsic tongue muscles in the Pink1-/- rat model of Parkinson disease.

Parkinson disease (PD) is associated with speech and swallowing difficulties likely due to pathology in widespread brain and nervous system regions. In post-mortem studies of PD, pathology has been reported in pharyngeal and laryngeal nerves and muscles. However, it is unknown whether PD is associated with neuromuscular changes in the tongue. Prior work in a rat model of PD (Pink1-/-) showed oromotor and swallowing deficits in the premanifest stage which suggested sensorimotor impairments of these functions. The present study tested the hypothesis that Pink1-/- rats show altered tongue function coinciding with neuromuscular differences within tongue muscles compared to wildtype (WT). Male Pink1-/- and WT rats underwent behavioral tongue function assays at 4 and 6 months of age (n = 7-8 rats per group), which are time points early in the disease. At 6 months, genioglossus (GG) and styloglossus (SG) muscles were analyzed for myosin heavy chain isoforms (MyHC), α-synuclein levels, myofiber size, centrally nucleated myofibers, and neuromuscular junction (NMJ) innervation. Pink1-/- showed greater tongue press force variability, and greater tongue press forces and rates as compared to WT. Additionally, Pink1-/- showed relative increases of MyHC 2a in SG, but typical MyHC profiles in GG. Western blots revealed Pink1-/- had more α-synuclein protein than WT in GG, but not in SG. There were no differences between Pink1-/- and WT in myofiber size, centrally-nucleated myofibers, or NMJ innervation. α-synuclein protein was observed in nerves, NMJ, and vessels in both genotypes. Findings at these early disease stages suggest small changes or no changes in several peripheral biological measures, and intact motor innervation of tongue muscles. Future work should evaluate these measures at later disease stages to determine when robust pathological peripheral change contributes to functional change, and what CNS deficits cause behavioral changes. Understanding how PD affects central and peripheral mechanisms will help determine therapy targets for speech and swallowing disorders.

Management of palatal myoclonic tinnitus based on clinical characteristics: a large case series study.

Background: Palatal myoclonic tinnitus (PMT) is a rare otological condition caused by rhythmic contractions of soft palate muscles. Due to its rarity, only a few case series studies have been reported in the literature at the present time.Aims: This large case series study reviews treatment outcomes of PMT patients over the past 15 years based on clinical characteristics.Materials and methods: Between the year 2003 and 2018, 54 patients with a diagnosis of PMT were assessed. Clinical characteristics, audiological findings, psychological status, and other relevant medical history were thoroughly reviewed. Response to various treatment modalities were retrospectively analyzed.Results: The mean age of enrolled patients was 29.0 ± 16.4 years, with female gender predominance. All of the patients complained of 'clicky' sounding tinnitus. Twenty-nine patients had comorbid tinnitus of other type. Reassurance and behavior therapy was sufficient for young patients. Medication was effective in 44.4% of the patients. Botulinum toxin injection in the palate led to complete resolution of symptoms in a majority of intractable PMT patients.Conclusions and significance: Management of PMT should be customized according to the individual clinical characteristics of the patients. This study may provide insightful information to establish optimal treatment modalities for PMT.

Palatal Tremor - Pathophysiology, Clinical Features, Investigations, Management and Future Challenges.

Background: Palatal tremor is involuntary, rhythmic and oscillatory movement of the soft palate. Palatal tremor can be classified into three subtypes; essential, symptomatic and palatal tremor associated with progressive ataxia. Methods: A thorough Pubmed search was conducted to look for the original articles, reviews, letters to editor, case reports, and teaching neuroimages, with the keywords "essential", "symptomatic palatal tremor", "myoclonus", "ataxia", "hypertrophic", "olivary" and "degeneration". Results: Essential palatal tremor is due to contraction of the tensor veli palatini muscle, supplied by the 5th cranial nerve. Symptomatic palatal tremor occurs due to the contraction of the levator veli palatini muscle, supplied by the 9%th and 10%th cranial nerves. Essential palatal tremor is idiopathic, while symptomatic palatal tremor occurs due to infarction, bleed or tumor within the Guillain-Mollaret triangle. Progressive ataxia and palatal tremor can be familial or idiopathic. Symptomatic palatal tremor and sporadic progressive ataxia with palatal tremor show signal changes in inferior olive of medulla in magnetic resonance imaging. The treatment options available for essential palatal tremor are clonazepam, lamotrigine, sodium valproate, flunarizine and botulinum toxin. The treatment of symptomatic palatal tremor involves the treatment of the underlying cause. Discussion: Further studies are required to understand the cause and pathophysiology of Essential palatal tremor and progressive ataxia and palatal tremor. Similarly, the link between tauopathy and palatal tremor associated progressive ataxia needs to be explored further. Oscillopsia and progressive ataxia are more debilitating than palatal tremor and needs new treatment approaches.

A Case of Paraneoplastic Myoclonus Attributed to Non-Small Cell Lung Cancer.

Background: It is well known that myoclonus can be a paraneoplastic manifestation of underlying malignancy. Case Report: A 78-year-old male diagnosed with papillary variant non-small cell lung cancer (NSCLC) presented with tremulousness that rapidly evolved into severe, diffuse myoclonus with prominent palatal involvement requiring intubation. The generalized myoclonus resolved with on levetiracetam, chemotherapy and immune modulation. While low titer positive P/Q type calcium channel autoantibodies were detected, it's etiologic relevance is unclear. Discussion: This case highlights a rare neurologic paraneoplastic presentation of papillary NSCLC. It also illustrates the importance of monitoring airway safety when myoclonus is generalized. Highlights: A new, rare paraneoplastic presentation of papillary variant non-small cell lung adenocarcinoma is described. The patient presented with severe diffuse myoclonus with prominent palatal involvement without encephalitis that responded to a combination of chemotherapy, immune modulation, and levetiracetam. No clear causal antibody was found.

[Objective tinnitus and essential palatal tremor in children: report of a case]. / Acouphènes objectifs et myoclonies vélaires: à propos d'un cas chez l'enfant.

Palatal tremor is a rare neurotological disorder responsible for objective tinnitus in children. Palatal tremor may be symptomatic of an underlying neurological disease or essential when a cause cannot be identified. We report a case of an essential palatal tremor in a 10-year-old girl complaining of clicking tinnitus. No treatment was undergone as she was not obviously bothered by the ear-clicking sound. Different treatment modalities have been used for distressing tinnitus related to palatal myoclonus. Recently several publications reported satisfactory results with botulinum toxin injection, which seems to be the treatment of choice.