Symptomatic Palatal Tremor Research Articles

Progressive Ataxia with Palatal Tremor: A Case Series (P9-11.006)

<h3>Objective:</h3> This case series adds to the limited literature about progressive ataxia with palatal tremor (PAPT) by describing clinical findings and the approach to diagnostic testing in six cases of PAPT. <h3>Background:</h3> PAPT is a rare clinical syndrome characterized by symptomatic palatal tremor and progressive cerebellar ataxia. Imaging typically shows bilateral hypertrophic olivary degeneration (HOD). Though published work based on autopsy cases suggests a novel tauopathy as a possible etiology of sporadic PAPT, the syndrome can arise from a variety of vascular, traumatic, autoimmune, or genetic causes. Despite its many possible underlying etiologies, the published literature provides little guidance on a systematic approach to diagnostic testing. <h3>Design/Methods:</h3> We retrospectively reviewed the charts of patients evaluated at the Ataxia Center at Massachusetts General Hospital between 2010–with clinical findings consistent with PAPT. Data were collected about disease presentation, disease progression, imaging findings and diagnostic testing. <h3>Results:</h3> Six patients with PAPT were identified. Average age of symptom onset was 61, range 54–65. Presenting symptoms were gait imbalance in three, and dysarthria, dysphagia, and throat tremor, respectively, in the rest. All developed palatal tremor (patient 6 with ear clicks), appendicular ataxia, and ataxic eye movements throughout their course. All patients had evidence of bilateral HOD on brain MRI. PAPT was attributed to cavernous malformations in patient 2 and multicompartmental hemorrhage in patient 4. The causes in the remainder are unknown. Diagnostic testing varied. Four patients (inclusive of patients 2 and 4) underwent whole exome sequencing, two underwent cerebrospinal fluid analysis (both non-inflammatory), four had celiac testing, and one was tested broadly for neuronal autoantibodies. <h3>Conclusions:</h3> PAPT is a clinical syndrome with a variety of etiologies. As in our study, literature suggests that diagnostic evaluation often varies. Systematic evaluation is important to find underlying causes and aid future research to better identify and study patients with idiopathic PAPT. <b>Disclosure:</b> Dr. Wruble has nothing to disclose. Dr. Gupta has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Biogen. The institution of Dr. Gupta has received research support from Biogen. Dr. Schmahmann has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Medavante. Dr. Schmahmann has stock in Cadent. The institution of Dr. Schmahmann has received research support from National Ataxia Foundation. The institution of Dr. Schmahmann has received research support from Biohaven. Dr. Schmahmann has received intellectual property interests from a discovery or technology relating to health care. Dr. Schmahmann has received publishing royalties from a publication relating to health care. Dr. Schmahmann has received publishing royalties from a publication relating to health care. Dr. Schmahmann has received publishing royalties from a publication relating to health care. The institution of Dr. Balaban has received research support from Biogen.

Palato-Pharyngo-Laryngeal Myoclonus with Facial Involvement After an Ischemic Stroke.

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. Magnetic resonance imaging (MRI) of the brain showed restricted diffusion in the right superomedial cerebellum consistent with an acute infarct (Fig. 1). The etiology of stroke was atheroembolism from an aortic arch plaque atheroma, for which she was started on aspirin. The follow-up MRI of the brain 4 months later showed a new fluid-attenuated inversion recovery (FLAIR) signal abnormality in the left medulla involving the left inferior olivary nucleus, reflecting hypertrophic olivary degeneration related to the chronic cerebellar infarct in the proximity of the contralateral dentate nucleus (Fig. 2). Ten months after the stroke, the patient reported a subjective, rhythmic, non-suppressible “whooshing” sound in her right ear that did not correspond to her heart rate and persisted during sleep. A computed tomography (CT) angiogram of the head and neck ruled out any vascular abnormalities. Nasopharyngolaryngoscopy showed spasm of the hypopharyngeal musculature and larynx. The patient returned to clinic to report new involuntary twitching of the right lower face. On examination, she had ~2 Hz rhythmic myoclonus of the right lip, chin, and throat (Video 1) as well as the palate (Video 2). There was no evidence of facial weakness, ocular motility problems, dysarthria, or dysphagia. She was prescribed a trial of carbamazepine 200 mg twice daily for palatal tremor followed by baclofen 5 mg twice daily with mild relief. She declined to try clonazepam or botulinum toxin injections. Palato-pharyngo-laryngeal myoclonus is a rare condition that can be idiopathic or lesional. Symptoms of palatal tremor are usually delayed 6 to 9 months after the initial injury, but can be delayed up to 30 months.1 The etiology of palatal tremor is disruption of the dento-rubro-olivary pathway, known as the Guillain Mollaret triangle. A lesion in this pathway causes denervation of olivary neurons from inhibitory cerebellar inputs, leading to hypertrophy and sustained synchronized oscillations causing tremor.2, 3 We present a unique case of palato-pharyngo-laryngeal and facial myoclonus caused by an ischemic stroke of the cerebellum. The patient also had pulsatile tinnitus, which correlated with direct visualization of spasms of the palate, hypopharyngeal musculature, and larynx via nasopharyngolaryngoscopy. Pulsatile tinnitus is most common in essential palatal tremor because of contraction of the tensor veli palatini on the eustachian tube. Although symptomatic palatal tremor affects the levator veli palatini, it can also rarely cause subjective auditory phenomena, likely because of some fibers inserting on the eustachian tube.1, 5 The patient's mild improvement in symptoms with the combination of baclofen and carbamazepine suggest a potential therapeutic option that warrants further investigation. (1) Research project: A. Conception, B. Organization, C. Execution; (2) Statistical Analysis: A. Design, B. Execution, C. Review and Critique; (3) Manuscript Preparation: A. Writing of the First Draft, B. Review and Critique. C.L.N.: 1B, 1C, 3A S.Q.: 1B, 1C, 3A M.S.: 1A, 3B Per the University of Pennsylvania Institutional Review Board, “a single retrospective case report is a medical/educational activity and does not meet the Federal Policy for the Protection of Human Subjects definition of research which is a systematic investigation, including research development, testing and evaluation, designed to develop or contribute to generalizable knowledge. Therefore, the activity does not require review by the IRB. Under HIPAA, a single case report is an activity to develop information to be shared for medical/educational purposes. Therefore, the use of protected health information to prepare a paper for publication of a single case report does not require IRB review for HIPAA purposes.” The patient provided verbal consent while at a clinic visit. The patient also provided signed/written consent which was mailed to the Neurology office. We confirm that we have read the Journal's position on issues involved in ethical publication and affirm that this work is consistent with those guidelines. No specific funding was received for this work. The authors declare that there are no conflicts of interest relevant to this work. M.S. has received clinical trial support from AbbVie, Abbott, United States (US) World Meds, and Sanofi. C.L.N. and S.Q. have no other financial disclosures to report.

A brainstem hypermetabolism in a patient with essential palatal tremor: A simultaneous 18F-FDG-PET/3 T-MRI study

Essential palatal tremor (EPT), previously referred as palatal myoclonus, is a rare movement disorder characterized by rhythmic and continuous jerks of soft palate [1]. While symptomatic palatal tremor (SPT) is typically caused by brainstem or cerebellar lesions [2], pathogenetic mechanism underlying EPT remains debated [1]. Here we reported a simultaneous brain fluorine-18-fluorodeoxyglucose-positron emission tomography (18F-FDG-PET)/3 T- magnetic resonance imaging (MRI) study in a woman with EPT.

Oculopalatal tremor and hypertrophic olivary degeneration

Background: Oculopalatal tremor is characterized by synchronous occurrence of palatal tremor and pendular nystagmus. Oculopalatal tremor (OPT) follows a destructive lesion in the Guillain- Mollaret triangle (GMT) and is associated with hypertrophic inferior olive degeneration (HOD). Case presentation: We report a case of 45 yearsold patient with palatal tremor and pendular nystagmus without ear click. He has had a brainstem stroke 11 months back. He had right side pyramidal drift, left side lower motor neuron type facial weakness and left abducens nerve palsy and mild gait ataxia in addition to oculopalatal tremor. MRI showed hypertrophic inferior olive degeneration. Conclusion: Ear click is an infrequent finding in symptomatic palatal tremor. Additional clinical features may be present in OPT depending on topography of initial structural lesion. There is a wide variety of time intervals between the occurrence of anatomical lesions and recognition of palatal tremor.

Symptomatic palatal tremor: A descriptive cohort study of 27 cases in a tertiary hospital

Symptomatic palatal tremor: A descriptive cohort study of 27 cases in a tertiary hospital

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.

Palatal Tremor Revisited: Disorder with Nosological Diversity and Etiological Heterogeneity.

This case series aimed to describe clinicoradiological, electromyographic, and etiological spectra in palatal tremor (essential=1; symptomatic=26). Patients with symptomatic palatal tremor had 2 to 10 Hz arrhythmic electromyographic bursts, a spectrum of changes in inferior olivary nucleus, with/without lesions in Guillain Mollaret triangle, and varied etiologies (genetic=9, vascular=6, trauma=3, infections=3). Exome sequencing showed variations in POLG, WDR81, NDUFS8, TENM4, and EEF2. Clinical phenotypes of patients with POLG, WDR81, and NDUFS8 variations were consistent with that described in literature. We highlight salient magnetic resonance imaging features, electrophysiological observations, and diverse etiologies in a large cohort of palatal tremor.

Vietnamese patient with progressive ataxia and palatal tremor syndrome

Background: We present clinical and MRI features of progressive ataxia and palatal tremor (PAPT). Case Report: A 67 year-old gentleman visiting from Vietnam presented with intermittent stroke-like episodes consisting of facial weakness, dysarthria, and osillopsia. He reported gradually worsening ataxia and dysequilibrium over 4 years. Examination revealed small amplitude nystagmus towards the right, impaired VOR to the left, palatal tremor, left-sided dysmetria, and an unsteady gait. MRI of the brain demonstrated increased T2/FLAIR signal within the inferior olive. Contrast enhanced and diffusion sequences were normal. MRA was normal. Electrocardiography, telemetry, and echocardiogram were normal. CSF was normal. Glial fibrillary acidic protein (GFAP) and vitamin E levels were normal. Genetic testing for hereditary forms of ataxia including spinocerebellar ataxia was not completed. Conclusion: Palatal tremor is commonly classified into symptomatic or essential subgroups. Symptomatic palatal tremor is frequently caused by a lesion in the triangle of Guillain and Mollaret leading to hypertrophic olivary degeneration. A subgroup of symptomatic palatal tremor form a syndrome of PAPT. Published details of cases of PAPT are sparse and the disorder appears to be mainly sporadic. Common features include progression of ataxia, olivary degeneration, gaze-evoked nystagmus, and internuclear ophthalmoplegia. There is no known effective treatment for progressive ataxia, which is the most disabling symptom of PAPT.

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 3years. 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 9months, 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.

Palatal tremor as a manifestation of epilepsia partialis continua caused by acute precentral gyral infarction

We describe a patient with palatal tremor (PT) as a manifestation of focal seizure caused by acute cortical infarction. Brain MRI showed acute infarction in the left precentral gyrus without evidence of brainstem lesions or hypertrophy of the inferior olivary nucleus. We discuss the differences between our patient and previous reports of symptomatic PT and the mechanisms involved in the development of PT associated with cortical lesions.

Essential Palatal Tremor Treated With Botulinum Toxin

Palatal tremor is a rare movement disorder characterized by rhythmic movement of the soft palate. There are two subtypes: essential and symptomatic palatal tremor. Essential palatal tremor is characterized by tinnitus and an absence of other neurological deficits. Different treatment options have been used to treat palatal tremor, with varying success rates. Here we describe a patient with essential palatal tremor and who was treated with botulinum toxin injections.

Progressive ataxia and palatal tremor: T1-weighted with magnetization transfer pulse hyperintensity in the inferior olivary nucleus

Progressive ataxia and palatal tremor (PAPT) is a rare distinct clinical entity characterized by symptomatic palatal tremor associated with progressive ataxia1,2. Few cases have been described in literature, and MRI findings show, in most cases, cerebellar atrophy and bilateral olivary hypertrophy with signal change1. We report a case where MRI study at 3T demonstrated marked bilateral olivary complex hyperintensity at T1, with magnetization transfer contrast pulse (MTC) sequence, which was subtle at conventional T2 and FLAIR sequences at 1,5 and [...]

Symptomatic palatal tremor of cortical origin due to stroke

Palatal tremor (PT) is usually considered a movement disorder that presents with recurring rhythmic contractions of the soft palate. The inferior olive shows a characteristic pseudohypertrophy secondary to brainstem lesions in the triangle of Mollaret and Guillain that interrupt dentato-olivary and tegmental pathways. We report a 35-year-old man with a history of uncontrolled hypertension who presented to the emergency department with PT after a left middle cerebral artery ischemic stroke. The diagnostic work-up consisted of brain MRI, which revealed restricted diffusion over the left frontoparietal lobes without involvement of the brainstem. During hospitalization, the patient reported two brief episodes of soft palate and base-of-the-tongue high-frequency, low-amplitude and rhythmic tremor that resolved after intravenous administration of lorazepam. A 2-hour video electroencephalogram showed no abnormalities. After initiation of levetiracetam therapy, no further spells were reported by the patient. At the 2-month follow-up, the patient had had no episodes of stereotypical PT or upper limb tremors since discharge. This report provides further evidence of the central role of the cortex in the generation of PT. The cortical origin of symptomatic palatal tremor (SPT) should be considered in patients presenting after an acute ischemic insult, particularly if there is no evidence of a brainstem lesion. Potential causes of SPT of cortical origin include focal epilepsy and diaschisis.

The Dentato-Rubro-Olivary Tract: Clinical Dimension of This Anatomical Pathway

Symptomatic palatal tremor is potentially the result of a lesion in the triangle of Guillain-Mollaret (1931) and is associated with hypertrophic olivary degeneration (HOD) which has characteristic MR findings. The triangle is defined by dentate efferents ascending through the superior cerebellar peduncle and crossing in the decussation of the brachium conjunctivum inferior to the red nucleus, to finaliy reach the inferior olivary nucleus (ION) via the central tegmental tract. The triangle is completed by ION decussating efferents terminating on the original dentate nucleus via the inferior cerebellar peduncle. We can demonstrate the anatomy of this anatomical triangle using a clinical case of palatal tremor presenting with bilateral subjective pulsatile tinnitus along with the pathognomonic MR findings previously described. The hyperintense T2 signal in these patients may be permanent, but the hypertrophied olive normally regresses after 4 years. The temporal relationship between the evolution of the histopathology and the development of the palatal tremor remains unknown as does the natural history of the tremor. Botox injection at the level of tensor and levator veli palatini insertion have been used to treat patients with disabling tremor synchronous tinnitus. A lesion involving the triangle can have a quite varied clinical expression.

Symptomatic palatal tremor is not limited to the activity of guillain-mollaret triangle: A functional magnetic resonance imaging case study

Symptomatic palatal tremor is not limited to the activity of guillain-mollaret triangle: A functional magnetic resonance imaging case study

Symptomatic palatal tremor is not limited to the activity of guillain-mollaret triangle: A functional magnetic resonance imaging case study

Elliyedi yasinda erkek hasta alti aydir olan konusma ve yutma zorlugu nedeni ile klinigimize basvurdu. Muayenede ritmik palatal tremor ve solda ust ekstremite kaslarinda miyoritmi goruldu. Beyin manyetik rezonans goruntuleme calismasinda oliver cekirdekte hipertrofik dejenerasyon, dentat cekirdek patolojisi, Wallerian dejenerasyon ve sol parietal hemisferde kistik ensefalomalazik alan goruldu. Fonksiyonel manyetik rezonans goruntulemede her iki globus pallidus ve talamusta belirgin aktivasyon tespit edildi. Bu olgu calismasindan elde edilen sonuclar; palatal tremorda gorulen istemsiz hareketlerin olivar cekirdegin disinhibisyonu sonucu bazal cekirdekler tarafindan olusturuldugunu dusundurmustur

Teaching Video Neuro Images : An endoscopic view of symptomatic palatal tremor

A 76-year-old woman presented with symptomatic palatal tremor (SPT) characterized by rhythmic contractions of the levator veli palatini muscle (video on the Neurology ® Web site at www.neurology.org). She did not complain of ear clicks. Physical examination revealed bilateral saccadic pursuit, bilateral impaired suppression of the vestibulo-ocular reflex, and autonomic …

Palatal Myoclonus (syn. Palatal Tremor)

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.

Inferior Olivary Hypertrophy Is Associated with a Lower Functional State after Pontine Hemorrhage

Background: Inferior olivary hypertrophy (IOH) may develop after pontine hemorrhage and may become a pacemaker for symptomatic palatal tremor (SPT). However, there is no information available that elucidates how IOH may affect the functional outcome. The purpose of this study was to investigate how frequently IOH was associated with clinical manifestations of involuntary movements, including ocular myoclonus (OM) and SPT, and whether IOH influenced the functional outcome after pontine hemorrhage. Methods: In 20 consecutive patients undergoing inpatient multidisciplinary rehabilitation after pontine hemorrhage, the location of lesions (tegmental vs. ventral) and the presence of IOH were examined by magnetic resonance imaging, and the functional outcome was assessed by means of Fugl-Meyer scale for neurological impairment and Functional Independence Measure for disability on admission and discharge. Results: In 10 patients, IOH was detected, and the tegmentum was involved in 7 of the 10 patients. OM or SPT was present in 7 of these 10 patients. In the remaining 10 patients, IOH was not detected, and the tegmentum was involved only in 2 of these 10 patients. None of them had OM or SPT. The presence of IOH was associated with a lower functional status on admission and discharge. Conclusion: After pontine hemorrhage, IOH may be associated with tegmental lesions with OM or SPT and may impose a detrimental effect on the functional outcome.

Endonasal approach of salpingopharyngeus muscle for the treatment of ear click related to palatal tremor

Palatal tremor (PT) is a rare disease associated with rhythmic movements of the soft palate. It can be separated into two distinct clinical entities: symptomatic and essential. Most patients with essential PT complain of the rhythmic ear clicks and in some cases tinnitus, but usually have an uneventful medical history. Symptomatic PT patients are often unaware of the palatal movements and have symptoms and signs of brainstem or cerebellar dysfunction. We describe the case of a 25-year-old patient who developed severe essential PT, with very distressing bilateral objective tinnitus, constantly perceived as ear clicks. Several oral medications were prescribed with poor results. No significant improvement was obtained with repetitive injections of botulinum toxin type A (BTX A) distributed in soft palate muscles. Because of the continuous tinnitus and its impact on the patient's quality of life, chemical denervation of the salpingopharyngeus muscles, which is involved in the production of tinnitus, with BTX A was performed endonasally under endoscopic guidance. The result was very satisfactory. Tinnitus due to essential PT may be satisfactorily treated by endonasal injection of BTX into the salpingopharyngeus and palatopharyngeus muscles.