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.

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.

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.

Does drug-induced sleep endoscopy predict surgical success of limited palatal muscle resection in patients with obstructive sleep apnea?

OBJECTIVE: The aims of this study were to determine the associated factors affecting the success rate of limited palatal muscle resection (LPMR), and to investigate whether drug-induced sleep endoscopy (DISE) could predict the therapeutic response to LPMR in patients with obstructive sleep apnea obstructive sleep apnea (OSA). METHODS: Twenty-one consecutive OSA patients underwent LPMR were enrolled. All patients received routine ENT examination, preoperative DISE, and polysomnography (PSG). Clinical, polysomnographic, cephalometric variables, and DISE findings were evaluated. The measurements were related to the success or failure of LPMR based on the results of preoperative and postoperative PSG. RESULTS: The overall success rate of LPMR was 66.6%. Postoperative AHI and minimal oxygen saturation were significantly decreased after LPMR (p<0.001). Comparison between success and failure groups revealed no significant differences in BMI, Friedman stage, preoperative AHI, minimal oxygen saturation, and all cephalometric parameters. However, the success of LPMR was significantly correlated with site, degree, and configuration of obstruction in DISE. In the velopharynx, complete obstruction (p=0.006) with anterolateral or concentric pattern (p=0.044) had significantly better success rate than partial obstruction with lateral pattern. CONCLUSION: DISE was only predictive method for identifying the success in OSA patients undergoing LPMR. Patients with anteroposterior or concentric total obstruction in the velopharynx might be suitable candidate for LPMR.

Palatoglossus coupling in selective upper airway stimulation.

OBJECTIVES/HYPOTHESIS: Selective upper airway stimulation (sUAS) of the hypoglossal nerve is a useful therapy to treat patients with obstructive sleep apnea. Is it known that multiple obstructions can be solved by this stimulation technique, even at the retropalatal region. The aim of this study was to verify the palatoglossus coupling at the soft palate during stimulation. STUDY DESIGN: Single-center, prospective clinical trail. METHODS: Twenty patients who received an sUAS implant from April 2015 to April 2016 were included. A drug-induced sedated endoscopy (DISE) was performed before surgery. Six to 12 months after activation of the system, patients' tongue motions were recorded, an awake transnasal endoscopy was performed with stimulation turned on, and a DISE with stimulation off and on was done. RESULTS: Patients with a bilateral protrusion of the tongue base showed a significantly increased opening at the retropalatal level compared to ipsilateral protrusions. Furthermore, patients with a clear activation of the geniohyoid muscle showed a better reduction in apnea-hypopnea index. CONCLUSIONS: A bilateral protrusion of the tongue base during sUAS seems to be accompanied with a better opening of the soft palate. This effect can be explained by the palatoglossal coupling, due to its linkage of the muscles within the soft palate to those of the lateral tongue body. LEVEL OF EVIDENCE: 4 Laryngoscope, 127:E378-E383, 2017.

Palatal and Oromandibular Tremor Secondary to Degenerative Olivary Hypertrophy After Ependymoma Surgery.

Palatal tremor (PT) is a rare movement disorder that involves pharynx, tongue, and other facial muscles. Symptomatic PT is due to lesions on the dentate-rubro-olivary pathways. We present an illustrative case of PT due to degenerative olivary hypertrophy after ependymoma surgery.

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.

Evaluation of hearing deficit in patients with oral submucous fibrosis.

Oral submucous fibrosis (OSMF) is a well recognized premalignant condition predominantly affecting the oral cavity, but sometimes extending to the pharynx, esophagus or even the larynx. OSMF may lead to stiffness of the oral cavity, resulting in trismus and inability to eat, difficulty with speech or swallowing, pain in the throat and ears, and a relative loss of auditory acuity. To evaluate the hearing deficit in patients with OSMF, we examined a study group comprising 40 patients, and also 10 age- and sex-matched healthy subjects as controls. All of the subjects were evaluated for hearing loss by pure tone audiometry. The OSMF group showed a significant degree of hearing loss relative to the control group. OSMF in advanced stages was significantly associated with mild conductive hearing loss (P < 0.01). The present study revealed a significant association between OSMF and hearing deficit. Involvement of the palatal muscles with OSMF may decrease the patency of the Eustachian tube, leading to conductive hearing loss. Therefore, all patients with OSMF should be evaluated for hearing deficit and advised about appropriate treatment.

Electrophysiological change in the tensor veli palatini muscle after radiotherapy.

CONCLUSION: Electromyography of the tensor veli palatine (TVP) was abnormal and showed mainly myogenic impairment in patients with nasopharyngeal carcinoma (NPC) with secretory otitis media (SOM) after radiotherapy. The diseased ears showed impairment in opening functions of the eustachian tubes (ETs). OBJECTIVES: To characterize electrophysiology of the TVP muscle using electromyography (EMG) in patients with SOM after radiotherapy of NPC. METHODS: Twenty healthy volunteers and 20 patients with NPC and SOM after radiotherapy were chosen for assessment of EMG of the TVP during swallowing. RESULTS: The measurements of average duration and amplitude of action potential, swallowing contraction duration, and peak voltage in NPC patients with both SOM (n = 25) and healthy ears (n = 6) were significantly lower than those of ears (n = 38) in healthy controls (p < 0.01). In patients with NPC, the average action potential duration and swallowing contraction duration in ears with SOM were lower than those of subjects with healthy ears (p < 0.05), whereas no significant difference was found in average amplitude of action potential and peak voltage between them.

Oculopalatal tremor, facial myokymia and truncal ataxia in a patient with neurosarcoidosis.

Symptomatic palatal tremor (SPT) is the result of a structural lesion, in the form of stroke, trauma or demyelinating disease. SPT is due to contractions of the levator veli palatini and can be accompanied by simultaneous movements of the facial and ocular muscles. Facial myokymia (FM) is a persistent quivering of the facial muscles. FM is usually encountered with conditions involving the pontine tegmentum. We report, to our knowledge, the first patient with neurosarcoidosis with simultaneous SPT and FM. A 49-year-old African American woman, with non-caseating granulomas in a paratracheal lymph node biopsy, presented with progressive gait disturbances for the last 3 years. Neurological examination revealed ataxic speech, bilateral rotatory nystagmus, myokymia of the chin and perioral muscles, palatal tremor without ear click and marked truncal ataxia. MRI demonstrated a lesion involving the facial nucleus and the right middle cerebellar peduncle. Based on exclusion of alternative etiologies, a diagnosis of neurosarcoidosis was made and the patient was started on methotrexate for 9 months, with minimal improvement. She was then switched to intravenous infliximab without major adverse events. The patient's speech and gait ataxia improved and follow up MRI demonstrated resolution of the enhancing lesions. To our knowledge, this is the first reported case of the combination of palatal tremor and FM due to neurosarcoidosis. Methotrexate may fail to produce clinical or radiographic response in up to 39% of patients. Tumor necrosis factor-α inhibitors, such as infliximab, should be considered in refractory cases.

Strategies to improve regeneration of the soft palate muscles after cleft palate repair.

Children with a cleft in the soft palate have difficulties with speech, swallowing, and sucking. These patients are unable to separate the nasal from the oral cavity leading to air loss during speech. Although surgical repair ameliorates soft palate function by joining the clefted muscles of the soft palate, optimal function is often not achieved. The regeneration of muscles in the soft palate after surgery is hampered because of (1) their low intrinsic regenerative capacity, (2) the muscle properties related to clefting, and (3) the development of fibrosis. Adjuvant strategies based on tissue engineering may improve the outcome after surgery by approaching these specific issues. Therefore, this review will discuss myogenesis in the noncleft and cleft palate, the characteristics of soft palate muscles, and the process of muscle regeneration. Finally, novel therapeutic strategies based on tissue engineering to improve soft palate function after surgical repair are presented.

A review of the evaluation and management of velopharyngeal insufficiency in children.

This article highlights the most common causes of velopharyngeal insufficiency (VPI), and discusses routine evaluation and treatment algorithms for the management of VPI in children. VPI is a multifactorial condition that occurs commonly in syndromic and non-syndromic children. The most common features of VPI are audible hypernasal speech, facial grimacing, decreased speech intelligibility, nasal regurgitation, and nasal emission from failure to produce oronasal separation. Work-up of VPI typically involves radiologic and endoscopic testing performed with the assistance of a speech-language pathologist. Management of VPI involves initial speech therapy followed by operative repair with sphincter or pharyngeal flap pharyngoplasty, if needed.

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.

Correction of incomplete cleft palate by u-shaped flap palatoplasty.

OBJECTIVES: During cleft palate repair, velopharyngeal sphincter reconstruction is still a challenge to plastic surgeons. To improve results of surgical treatment of cleft palate and secondary velopharyngeal incompetence, a carefully designed modified procedure for palatoplasty is presented. MATERIALS AND METHODS: Thirty patients with incomplete cleft palate corrected by this procedure from April 2003 to October 2007 were included. A u-shaped incision was made in the anterior palate to separate bipedicle flap based on the greater palatine arteries of both sides. After complete dissection of the nasal and palatal mucosa, palatal muscles were carefully dissected from the posterior edge of the bones of the hard palate, wherein the well-mobilised flap receded backward spontaneously and elongated the soft palate. After suturing the nasal mucosa in the midline, the dissected palatal muscles of both sides were sutured together in the anterior third of the soft palate. On suturing the nasal mucosa and the palatal muscles, the soft palate became elongated and the oral mucosa was seen receding backward spontaneously to cover the anterior part of the soft palate. RESULTS: All corrected patients showed good results, with no recurrence, no post-operative fistulae, with accepted speech pattern and no need for further pharyngoplasty. The line of the sutured nasal mucosa and the palatal muscles became covered by the healthy non-sutured oral mucosa and the soft palate became elongated, with narrowing of the vellopharyngeal isthmus after this technique. CONCLUSION: The designed flap allows covering of sutured nasal mucosa and palatal muscles with a healthy unsutured oral mucosa and elongates the soft palate. Thus, there was no incidence of post-operative fistula and no need for further pharyngoplasties.