Palatal Tremor Research Articles

Palatal Tremor, Periocular and Perioral Myokymia, and Pseudoathetosis in a Patient with Whipple's Disease.

Palatal Tremor, Periocular and Perioral Myokymia, and Pseudoathetosis in a Patient with Whipple's Disease.

A Rare Midbrain Syndrome with Bilateral Cerebellar Ataxia and Isolated Superior Rectus Palsy: Wernekinck Commissure Syndrome

Wernekinck commissure syndrome is a rare midbrain syndrome, usually resulting in selective destruction of the decussation of the superior cerebellar peduncle. This syndrome can present with symptoms such as bilateral cerebellar ataxia, ophthalmoplegia, and palatal tremor. Here, we report a 61-year-old female with acute onset of bilateral cerebellar ataxia and isolated superior rectus palsy diagnosed on Wernekinck commissure syndrome confirmed by magnetic resonance imaging.

Clinical and Imaging Profile of Patients with Cerebrotendinous Xanthomatosis - a Video Case Series from India.

Cerebrotendinous xanthomatosis (CTX) is a rare autosomal recessive disorder caused by bi-allelic pathogenic variants in CYP27A1 gene that results in the deposition of cholestanol in the eyes, tendons, soft tissues and nervous system leading to cataracts, xanthomas, and various neuropsychiatric manifestations. The aim of our study is to describe the clinical, radiological and genetic profile of patients with CTX. This is a retrospective chart review of patients with CTX diagnosed based on classical clinical and radiological findings. The available clinical details, and investigations, including imaging, electrophysiological, pathological and genetic data, were documented. Five patients (4 males) were recruited in the study. The median age at presentation was 32 years (range: 21-66 years). Walking difficulty was the most common symptom at presentation. All patients had cataracts, tendon xanthomas, eye movement abnormalities, dysarthria, pyramidal signs, ataxia and gait abnormality. Dystonia was noted in three patients. Palatal tremor and parkinsonism were noted in one patient each. In MRI brain, dentate, and corticospinal tract involvement were the most frequent imaging findings. Bilateral hypertrophic olivary degeneration was noted in one patient and hot cross bun sign in two. Three patients underwent genetic testing and all had pathogenic variants confirming the diagnosis. CTX is a rare treatable disorder. Apart from the usual neurological presentation with spastic-ataxia, it can present at a later age with parkinsonism. Typical patterns of imaging findings are helpful in early diagnosis which aids in the treatment to prevent the neurological sequelae of the disease.

Electrophysiology in Functional Movement Disorders: An Update.

Functional movement disorders (FMD) are a diagnostic and therapeutic challenge, both to the neurologist and psychiatrists. The phenomenology is varied and can present as tremors, dystonia, jerks/myoclonus, gait disorder, other abnormal movements or a combination. There has been an increase in the use of electrophysiological studies that are an important tool in the evaluation of FMDs. We searched the database platforms of MEDLINE, Google scholar, Web of Sciences, Scopus using the Medical Subject Heading terms (MeSH) for all the articles from 1st January 1970 till November 2022. A total of 658 articles were obtained by the search mechanism. A total of 79 relevant articles were reviewed thoroughly, of which 26 articles that had electrophysiological data were included in the present review. Variability, distractibility and entertainability can be demonstrated in functional tremors by using multichannel surface electromyography. Voluntary ballistic movements tend to decrease the tremor, while loading the tremulous limb with weight causes the tremor amplitude to increase in functional tremor. Presence of Bereitschaftspotential demonstrates the functional nature of palatal tremor and myoclonus. Co-contraction testing may be helpful in differentiating functional from organic dystonia. The R2 blink reflex recovery cycle has been found to be abnormally enhanced in organic blepharospasm, whereas it is normal in presumed functional blepharospasm. Plasticity is found to be abnormally high in organic dystonia and normal in functional dystonia, in addition to enhanced facilitation in patients with organic dystonia. Electrophysiological tests supplement clinical examination and helps in differentiating FMD from organic movement disorders.

Four New Cases of Progressive Ataxia and Palatal Tremor (PAPT) and a Literature Review

PAPT syndrome is a rare neurologic disorder characterized by progressive ataxia and palatal tremor (rhythmic movements of the soft palate). The first large study of PAPT patients was published in 2004, included a total of 28 sporadic PAPT cases, and suggested a neurodegenerative origin. In the last several years, case reports and small case series followed, underlining the heterogeneity of the clinical picture and underlying aetiology (including neurodegenerative, vascular, infectious/autoimmune, and genetic). As a contribution to the literature, we report on four new patients with PAPT syndrome from Bern. Our study highlights the diverse clinical presentation (pyramidal, extrapyramidal, bulbar, cognitive, psychiatric symptoms, and autonomic features), summarizes the known literature, and extends it by findings on sleep studies (obstructive/central sleep apnoea, sleep disturbance). Possible aetiologies and management aspects are discussed in light of the current literature.

Tongue Myorhythmia and Palatal Tremor as the Main Clinical Manifestation in Anti-IgLON5 Disease.

Tongue Myorhythmia and Palatal Tremor as the Main Clinical Manifestation in Anti-IgLON5 Disease.

Palatal Tremor and Central Sleep Apnea Accompanied by Bilateral Hypertrophic Olivary Degeneration after Brainstem Hemorrhage

Palatal Tremor and Central Sleep Apnea Accompanied by Bilateral Hypertrophic Olivary Degeneration after Brainstem Hemorrhage

Palato-ocular synchrony: Seeing what is behind closed eyelids in palatal tremor (P5-11.010)

<h3>Objective:</h3> To call attention to palato-ocular synchrony, a little-known manifestation of palatal tremor. <h3>Background:</h3> Palatal tremor due to a lesion of the dentato-rubro-olivary pathway (Guillain-Mollaret triangle) may spread to contiguous or remote muscles. When it involves the eyes it is known as oculopalatal tremor (OPT) manifesting as pendular nystagmus, typically vertical and/or torsional, causing oscillopsia. Nystagmus may be minimal or absent with eyes open and appear only under closed eyelids, a phenomenon described in 1976 by Jacobs and Bender, and called palato-ocular synchrony (Arch Neurol;33:289). These authors observed that palato-ocular synchrony was caused by eyelid closure rather than simply the removal of vision. <h3>Design/Methods:</h3> Two illustrative video case reports. <h3>Results:</h3> Following brainstem hemorrhage (patient 1 – midbrain; patient 2 – pons) involving the Guillain-Mollaret triangle, two patients developed hypertrophic olivary degeneration and asymptomatic palatal tremor. In patient 1, there was no nystagmus with eyes open, but under closed eyelids, vertical oscillations were readily apparent. In patient 2, there was very subtle torsional pendular nystagmus without oscillopsia, but large vertical oscillations were apparent with eyelid closure that were synchronous with PT. The mechanism of palato-ocular synchrony is unknown but may be related to the observation that palatal tremor, due to a lesion of the Guillain-Mollaret triangle, is one of the few movement disorders that persist during sleep, and this may simply reflect eyelid closure. <h3>Conclusions:</h3> A lesion, typically stroke, involving the Guillain-Mollaret triangle often leads to delayed development of palatal tremor. Such patients should undergo a careful ocular motor examination looking for oculopalatal tremor; if absent, then the globes should be observed during active eye closure. A visible or palpable ocular oscillation under closed eyelids is known as palato-ocular synchrony, an underappreciated phenomenon that extends the spectrum of manifestations of palatal tremor. <b>Disclosure:</b> Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Best Doctors. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving as a Consultant for UpToDate. Dr. Reich has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Enterin. Dr. Reich has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for NIH. Dr. Reich has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Various law firms. The institution of Dr. Reich has received research support from ninds. Dr. Reich has received publishing royalties from a publication relating to health care. Dr. Reich has received publishing royalties from a publication relating to health care. Dr. Gold has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Springer .

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.

Wernekink Commissure Syndrome With Bilateral Cerebellar Signs and Holmes Tremor in a Patient With a Preexisting Movement Disorder

Wernekink commissure syndrome (WCS) is an extremely rare midbrain syndrome, in which there is the selective destruction of the decussation of the superior cerebellar peduncle, which commonly presents with bilateral cerebellar signs. We describe a case of WCS with Holmes tremorin a patient having an undiagnosed involuntary movement disorder since childhood following an undocumented case of meningitis. The patient presented with sudden onset gait instability with bilateral cerebellar signs (more prominent on the left side), Holmes tremorin bilateral limbs, slurred speech, and marked dysarthria. No ophthalmoplegia or palatal tremors were noted. The patient was conservatively managed along the lines of a stroke, and there was a marked improvement in cerebellar signs and Holmes tremorwith time but no evolution (improvement or worsening) was observed in the involuntary movements of limbs and face that were present before the onset of WCS.

PALATAL MYOCLONUS-A CASE STUDY

Palatal myoclonus is a repetitive, involuntary jerking movement of the pterygopalatine arch and soft palate, frequently involving the diaphragm and vocal muscles. Palatal tremor is classified into essential palatal myoclonus (EPM) and symptomatic palatal myoclonus (SPM). In EPM, the only clinical signs are palatal tremor and ear clicks, whereas, in SPM, tremor may encompass facial, ocular, and extremities muscles. In our study, a healthy 26 years old man presented a persistent auditory clicking on the temporomandibular joint bilaterally 18 months ago. The clicking was not related to mandibular movement. The final clinical examination showed that the patient suffered from palatal myoclonus with EPM. EPM is a rare condition characterized by clicking noises and muscle contraction at the back of the throat. Due to the conditions rarity, there have yet to be any randomized controlled trials of treatment options for EPM. However, our patient showed positive results after one week of treatment using Carbendazim 200mg.

Deep Brain Stimulation for Holmes Tremors and Literature Review.

Deep Brain Stimulation for Holmes Tremors and Literature Review.

PO055 / #573 DEEP BRAIN STIMULATION AS PALLIATIVE TREATMENT FOR MYORHYTHMIA. A CASE REPORT: E-POSTER VIEWING

Myorhythmia is a hyperkinetic movement disorder that derives from a disruption of the Guillain–Mollaret triangle, due to an identifiable structural lesion. It is often disabling and with disappointing medical control. Herein, we report a case of a 67-year-old man with late onset cranial, cervical and diaphragm myorhythmias and palatal tremor, developed six months after a pontomesencephalic haematoma. Pharmacological treatment failed to improve the symptoms and the condition was devastating to the patient’s quality of life.

A-2 Neuropsychological and Neuropsychiatric Symptoms Following Hypertrophic Olivary Degeneration

Abstract Objective: Hypertrophic Olivary Degeneration (HOD) is a rare neurological condition causing hypertrophy and eventual degeneration of the inferior olivary nucleus via transneuronal degeneration in the dentato-rubro-olivary pathway (DROP), or Guillain–Mollaret triangle. HOD is considered unique because the olive initially becomes hypertrophic rather than atrophic. Etiology is often cerebral vascular insult, cavernous malformation, surgical resection, and neurological disease, albeit cause may be unclear. While the neurologic and imaging findings have been well delineated in the literature, few studies investigate the neuropsychological impact. Methods: With consent, a review of the medical chart was conducted of a 39-year-old female with hypertension referred for assessment due to progressive cognitive, motor, and psychiatric symptoms. Results: Physical symptoms included progressive limb and trunk ataxia, palatal tremor, and opsoclonus myoclonus that plateaued within 4 years. Initial Magnetic Resonance Imaging (MRI) revealed bilateral, nonenhancing hyperintensity of the pontomedullary junction and enlargement of the inferior olive. Subsequent MRI displayed cerebellar atrophy. Cognitive assessment documented significant executive, language, spatial and fine motor dexterity deficits, alongside depression, anxiety, emotional blunting, and passive suicidality. She reported believing her neighbors were listening and following her in public. She remained semi-independent in daily living. Conclusions: This is the first case study that captures the neurocognitive profile associated with HOD and associated degeneration of DROP. This case highlights the potential for Cognitive Cerebellar Affective Syndrome (CCAS) to arise from damage to adjacent cerebellar pathways via transneuronal degeneration of brainstem circuits. It also provides evidence for the presence of delusions within CCAS as dysmetria of thought.

A 6-month trial of memantine for nystagmus and associated phenomena in oculopalatal tremor.

IntroductionOculopalatal tremor (OPT) is a late manifestation of a Guillain-Mollaret triangle lesion. Memantine has been shown to improve nystagmus in OPT, but its long-term efficacy and putative distinct effects on each plane of nystagmus and on associated phenomena (e.g., gravity perception) are largely unknown.MethodsWe conducted a 6-month open-label study to evaluate the effect of memantine in OPT patients. Baseline (visit 1), 2 (visit 2), and 6 months (visit 3) assessments included video-oculography, best corrected visual acuity (BCVA), visual function questionnaire (VFQ25), palatal tremor frequency, and subjective visual vertical (SVV). Memantine was titrated to 20 mg per day and stopped after 6 months.ResultsWe included six patients (5 females; mean age 68.5+/-9.7). At visit 2, nystagmus improved >50% only along the horizontal plane in two patients, while worsening >50% along the vertical and horizontal planes in 4 and 1 patients, respectively. At visit 3, previous improvement of nystagmus along the horizontal plane in two patients was not sustained, and it further worsened >50% along the vertical plane in 4. The mean vertical velocity and amplitude of nystagmus in the left eye significantly worsened from visit 2 to visit 3 (p = 0.028). Throughout the study, nystagmus frequency remained unchanged (p = 0.074), BCVA improved in both eyes (p = 0.047, p = 0.017), SVV progression was unpredictable (p = 0.513), and the mean VFQ-25 score (p = 0.223) and mean palatal frequency remained unchanged.ConclusionThe long-term use of memantine 20 mg per day in OPT produced a modest and only transient improvement in nystagmus, predominantly along the horizontal plane. Visual acuity improved, albeit without relevant changes in vision-related quality of life.

Palatal Tic Disorder Causing Objective Clicking Tinnitus in an 8 Years Old

Palatal tremors including myoclonus are well-known causes of objective tinnitus. Essential palatal tremors and tic disorders are recognized but rarer causes of objective pulsatile tinnitus. An 8-year-old boy presented with a chief complaint of clicking sounds in both the ears for 1 month, intermittent, occurs frequently in episodes, wherein intraoral examination revealed bilateral rhythmic, low frequency, symmetrical contractions of the soft palate muscles accompanied by clicking sounds audible to physician as well (objective tinnitus). The child's mother stated that the clicking sounds were not present when he was asleep. When the child was distracted by tuning fork sound, the palatal movements stopped. CNS examination, developmental history, birth, and growth history were all normal. Magnetic resonance imaging study with contrast enhancement was normal. The child was started on clonazepam and clonidine and follow-up was done after 1st and 2nd months. The tinnitus reduced but did not resolve completely. Clinical features of a patient with palatal tremor should be carefully assessed with prior knowledge of possible etiopathologies to guide the investigations and management. We highlight a case of palatal tic disorder to bring the possibilities quickly to the reader's mind.

Teaching Video NeuroImage: Tongue Tremor as a Presenting Feature of Progressive Supranuclear Palsy.

A 76-year-old man was referred to neurology by speech and language therapy after video fluoroscopy showed oropharyngeal dysphagia, aspiration, and tongue tremor (Video 1). He had presented with 6 months of progressive dysphagia, dysarthria, weight loss, and falls. Examination revealed akinetic rigidity, vertical supranuclear gaze palsy, slowed saccades, dysarthrophonia, and tongue tremor without palatal, jaw, or limb tremor. We diagnosed probable progressive supranuclear palsy (PSP). He did not respond to levodopa (400 mg/d). On a follow-up assessment 27 months later, the tremor persisted (Video 1). Tremor, usually upper limb, occurs in 42% of PSP cases.1 Isolated tongue tremor is a rare feature of PSP.1,2

Teaching Video NeuroImage: Unilateral Myorhythmia in a Patient With a Midbrain-Diencephalic Junction Cavernous Malformation.

A 60-year-old woman with a cavernous malformation at the left midbrain-diencephalic junction presented 9 months after hemorrhage and resection. She developed a palatal tremor and rhythmic, 2–3 Hz movements of her right leg (myorhythmia) 3 months earlier. Trihexyphenidyl and amantadine provided benefit (Video 1). MRI of the brain demonstrated bilateral olivary pseudohypertrophy (Figure).

A Report of Two Rare Cases of Palatal Tremors

Palatal tremor (PT), also known as palatal myoclonus (PM) is a very rare neurological phenomenon, manifesting as rhythmic movements of the soft palate which is hypothesized to occur either due to a lesion in the inferior olivary nucleus, or in an idiopathic manner. Prevalence data of the disease are lacking. Only a few hundred cases have been reported so far. Here we present two cases of palatal tremor, who presented to our tertiary care centre for unrelated symptoms. The first case was a 56 year old gentleman, who was incidentally found to have palatal tremor. Further investigation revealed a posterior circulation stroke involving the left cerebellar hemisphere and cerebellar peduncle. The second case, a 35 year old female, presented with an upper respiratory tract infection, and was discovered to have palatal tremor on routine examination. The cause was attributed to her history of surgical removal of a left cerebellar cyst five years ago.

A syndrome of progressive ataxia and palatal tremor in a patient with mild bilateral idiopathic hypertrophic olivary degeneration

A syndrome of progressive ataxia and palatal tremor in a patient with mild bilateral idiopathic hypertrophic olivary degeneration