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Background: Hypertrophic olivary degeneration (HOD), a rare form of transsynaptic degeneration, is secondary to dentato-rubro-olivary pathway injuries in some cases. We describe a unique case of an HOD patient who presented with palatal myoclonus secondary to Wernekinck commissure syndrome caused by a rare bilateral "heart-shaped" infarct lesion in the midbrain. Case presentation: A 49-year-old man presented with progressive gait instability in the past 7 months. The patient had a history of posterior circulation ischemic stroke presenting with diplopia, slurred speech, and difficulty in swallowing and walking 3 years prior to admission. The symptoms improved after treatment. The feeling of imbalance appeared and was aggravated gradually in the past 7 months. Neurological examination demonstrated dysarthria, horizontal nystagmus, bilateral cerebellar ataxia, and 2–3 Hz rhythmic contractions of the soft palate and upper larynx. Magnetic resonance imaging (MRI) of the brain performed 3 years prior to this admission showed an acute midline lesion in the midbrain exhibiting a remarkable "heart appearance" on diffusion weighted imaging. MRI after this admission revealed T2 and FLAIR hyperintensity with hypertrophy of the bilateral inferior olivary nucleus. We considered a diagnosis of HOD resulting from a midbrain heart-shaped infarction, which caused Wernekinck commissure syndrome 3 years prior to admission and later HOD. Adamantanamine and B vitamins were administered for neurotrophic treatment. Rehabilitation training was also performed. One year later, the symptoms of this patient were neither improved nor aggravated. Conclusion: This case report suggests that patients with a history of midbrain injury, especially Wernekinck commissure injury, should be alert to the possibility of delayed bilateral HOD when new symptoms occur or original symptoms are aggravated. [ABSTRACT FROM AUTHOR]

1 Video This video depicts ~2 Hz myoclonus of the patient's face and neck, including the lip, chin, and throat. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion in the right superomedial cerebellum consistent with an acute infarct (Fig. Keywords: palatal tremor; palatal myoclonus; hypertrophic olivary degeneration EN palatal tremor palatal myoclonus hypertrophic olivary degeneration 104 106 3 01/05/22 20220101 NES 220101 Palatal myoclonus, also described as palatal tremor, is characterized by rhythmic continuous 0.5 to 5 Hz movements of the soft palate and pharynx.1 There are two categories described: symptomatic palatal tremor, because of a brainstem or cerebellar lesion, and essential palatal tremor, with no associated structural lesion.2,3 Palatal myoclonus can rarely be associated with synchronous myoclonus of other branchial arch muscles, the diaphragm, facial muscles, or even the extremities.2 One specific variant is termed palato-pharyngo-laryngeal myoclonus, which can result in dysphagia, dysphonia, and dysarthria.4 A 56-year-old woman awoke from sleep with new right-sided weakness and dizziness. [Extracted from the article]

Palatal myoclonus (PM) is a rare disease that may induce dysphagia. Since dysphagia related to PM is unique and is characterized by myoclonic movements of the involved muscles, specific treatments are needed for rehabilitation. However, no study has investigated the treatment effectiveness for this condition. Therefore, the aim of this case report was to describe the benefit of combining behavioral treatment with valproic acid administration in patients with dysphagia triggered by PM. The two cases were treated with combined treatment. The outcomes evaluated by videofluoroscopic swallowing studies before and after the treatment showed significant decreases in myoclonic movements and improved swallowing function. We conclude that the combined treatment was effective against dysphagia related to PM. [ABSTRACT FROM AUTHOR]

Palatal myoclonus is an extremely rare neurotological disorder presenting to an otolaryngologist. It presents as objective tinnitus which is due to rhythmic, involuntary movements of the soft palate causing distress to the patients. Different medical and surgical remedies have been attempted with variable success. Botulinum toxin has been reportedly used in few cases and has reproduced good results lasting for a few months with minimum morbidity. We report a case suffering from palatal myoclonus for about 15 years and injection of botulinum toxin into his tensor veli palatini muscle resolved his objectionable unilateral tinnitus. Optimum results obtained initially, waned off over the years, for which the dose had to be gradually increased to achieve the desired clinical results. [ABSTRACT FROM AUTHOR]

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. © 2012 Movement Disorder Society [ABSTRACT FROM AUTHOR]

Symptomatic palatal tremor is caused by a lesion in the triangle of Guillain and Mollaret and is associated with hypertrophic olivary degeneration that has multiple causes. Essential palatal tremor has no currently demonstrable cause and no accompanying physical or radiological signs. But it is probable that an organic genesis will become apparent. I suggest that some examples of palatal tremor may depend on an ‘upper motor neurone type’, i.e. supranuclear, lesion in the striatum or rostral brainstem releasing medullary activation with denervation hypersensitivity of olivary neurones. Copyright © 2008 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Palatal myoclonus can be primary or secondary. In primary palatal myoclonus, no obvious structural brain lesions can be found within the triangle of Guillain and Mollaret. Common causes of secondary myoclonus include stroke, demyelination, infections, trauma, and neurodegeneration. [ABSTRACT FROM AUTHOR]

Palatal tremor (formerly palatal myoclonus) is an extremely rare, but potentially treatable cause, of objective tinnitus. The tinnitus is thought to be secondary to rhythmic involuntary movements of the soft palate. Its aetiology is variable and it remains difficult to treat. Many different medical and surgical remedies have been tried but none have demonstrated reproducible success. Botulinum toxin has been used in sporadic cases and seems to produce good results. Ten patients with palatal tremor have presented to this department over the last three years. After discussion with the patients with regard to the management of this condition and possible complications, five opted for botulinum toxin therapy and five declined further intervention. Clinical diagnosis was made on the confirmation of soft palate movements synchronous with an audible clicking noise. Five patients underwent botulinum toxin injection into the insertion of the levator and tensor veli palatini muscles. Of the five that were treated with toxin, four showed complete resolution of symptoms after a course of treatment. Only one patient reported transient side effects. This would suggest that botulinum toxin is a safe and effective first line treatment for palatal tremor. [ABSTRACT FROM AUTHOR]

Introduction. – Post brainstem lesion dysphagia is frequently associated with palatal myoclonus (PM) but the correlation between these two symptoms is still unclear.Objective. – The aim of this study was to verify the relationship between PM and dysphagia, and if PM could itself induce dysphagia.Patients and methods. – Twelve patients suffering from post brainstem lesion PM and dysphagia; 10 male and two female, mean aged of 50.5 years, were assessed using clinical examination, radiological and endoscopic examination of deglutition.Results. – In three cases, PM were associated with pharyngeal area decreasing, laryngeal aspiration, and dysphagia.Discussion. – This study confirms the hypothesis of close relationship between PM and dysphagia. In these cases, specific dysphagia therapy should be recommended.Conclusion. – Post brainstem lesion dysphagia is sometimes associated with PM and in some cases, there is probably a relationship between PM and swallowing disorders of these patients. [Copyright &y& Elsevier]

Heterozygous point mutations in the coding region of the human glial fibrillary acidic protein (GFAP) gene have been reported in patients with various forms of Alexander disease (AD). We report a case of genetically confirmed adult-onset AD with palatal myoclonus, pyramidal tract signs, cerebellar signs, and marked atrophy of the medulla oblongata and spinal cord, autonomic dysfunction and heterozygous R416W GFAP mutation. Interestingly, this R416W mutation has also been reported in both infantile and juvenile forms of Alexander disease. The fact that a R416W mutation causes various types of AD suggests that clinical severities of AD are due not only to the different sites and nature of mutations in GFAP, but also to other modifying factor(s). [Copyright &y& Elsevier]

Palatal myoclonus is a syndrome characterized by involuntary, continuous or nearly continuous, rhytmical movements in soft plate, pharynx, larynx, diaphragma and rarely facial muscles. Mostly a variety of causes like vascular diseases, multiple sclerosis, encephalities, neoplasms and infections are blamed in the etiology. A 35 year-old female patient who had ischemic infarct due to left vertebral artery dissection suffered from obvious involuntary movements on the left side of her face. Palatal Myoclonus was considered after neurological and electrophysiological examinations. Botulinum toxin was applied as there was no response to medical therapies and good response was obtained. Palatal myoclonus rarely expands to facial muscles and is more responsive to Botulinum toxin than medical therapies. For this reason this case is reported to review the etiology, symptoms and therapy. [ABSTRACT FROM AUTHOR]

Objective: Hashimoto encephalopathy (HE) is known as a steroid-responsive encephalopathy associated with autoimmune thyroiditis or nonvascular inflammation-related autoimmune meningoencephalitis. The average age of onset of HE is approximately 50 years; and it is more common in women. The onset of HE may be acute or subacute. The course of most HE cases is relapsing and remitting, which is similar to that of vasculitis and stroke. Methods: In this article, we present a previously healthy 32 years old; veterinarian male with palatal myoclonus, as a rare presentation of this disorder, and review the neurologic aspects of hashimoto encephalitis . Results: The clinical presentation of HE is characterized by progressive cognitive decline tremor, transient aphasia, seizures, abnormal gait, sleep disorder and stroke-like episodes . Myoclonus, either generalized or multifocal, and tremor, often of the bilateral upper extremities, is the most frequently observed involuntary movements in HE. Conclusion: The rapidly progressive cognitive dysfunction and encephalopathies observed [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Objectives: We tested the effect of radiofrequency ablation in the treatment of palatal myoclonus refractory to other therapeutic regimens. Methods: A 20-year-old patient who presented with palatal myoclonus and annoying rhythmic objective clicking noise was treated with a radiofrequency generator with topical and local anesthesia in an outpatient setting. Results: The clicking noise was abolished within 3 to 4 days of the radiofrequency ablation. The rhythmic, jerky movements of the soft palate were confirmed to be absent 2 weeks later with electromyographic studies. The patient has been symptom-free for 6 months, and no side effects have occurred. Conclusions: As a new indication, radiofrequency ablation may effectively abolish abnormal movements of the soft palate and relieve associated tinnitus. [ABSTRACT FROM AUTHOR]

We present a case of a 39-year-old patient, who was diagnosed and treated for a tick-borne meningoencephalitis. Three months after the treatment he started to complain of annoying, cracks-resembling, rhythmical sounds, coming from the inside of his head to both his ears. Physical examination revealed rhythmical oscillations of the soft palate with a frequency of 100–120 per minute and a clock ticking noise synchronic with the palate tremor. Electromyography revealed continuous motor unit activity at rest in the tensor veli palatini muscle. Palatal myoclonus (PM) as a result of tick-borne meningoencephalitis was diagnosed. Treatment with several medications was started with no effect, then botulinum toxin was administered under EMG guidance to both sides of the patient’s soft palate with great improvement. A 5-year follow-up and continuation of botulinum toxin injections with only minor and reversible side effects proved the treatment efficacy and safety. In the article we present a case of symptomatic palatal myoclonus with ear click and shortly discuss its aetiology, types and treatment options. We also stress the efficacy and safety of PM treatment with repetitive injections of botulinum toxin. [ABSTRACT FROM AUTHOR]

Background: Wernekink commissure syndrome causes a peculiar combination of internuclear ophthalmoplegia, dysarthria, and delayed-onset palatal myoclonus. Palatal myoclonus is thought to be secondary to delayed hypertrophic degeneration of the bilateral inferior olivary nuclei secondary to involvement of bilateral dentatoolivary tract. We describe a case of a patient with early-onset palatal myoclonus.Case Presentation: A 53-year-old Caucasian man with several vascular risk factors presented to our emergency room with slurred speech, double vision, difficulty with swallowing and walking, and rhythmic contractions of the soft palate. Brain magnetic resonance imaging showed an acute infarct of the right caudal midbrain and an old infarct of the right medulla. We hypothesize that the cause of early palatal myoclonus in our patient was a two-hit mechanism with degeneration of the right olivary nucleus resulting from prior right medullary lacunar stroke with the new infarct affecting the dentato-rubro-olivary tract on the left side, causing bilateral dysfunction initiating palatal myoclonus.Conclusions: Wernekink commissure syndrome with palatal myoclonus at onset suggests the presence of a prior ischemic insult in the medulla. Careful examination is important to identification of this presentation. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented in response to the article about Adult-onset Alexander disease with palatal myoclonus and intraventricular tumour.