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.     

New device to control combined lingual and palatal myoclonus.

Lingual myoclonus is a poorly understood disorder that may occur in isolation or combined with palatal myoclonus. In this report, we present the case history of a 21-year-old patient with a therapy-resistant essential lingual and palatal myoclonus where a simple dental device was able to control symptoms. The use of this device will be highlighted and compared to previously described methods. Cases of previously recorded lingual and palatal myoclonus will be reviewed and compared to the case of our patient.     

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.     

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.     

Palatal tremor, progressive multiple cranial nerve palsies, and cerebellar ataxia: a case report and review of literature of palatal tremors in neurodegenerative disease.

We describe a patient with an unusual clinical presentation of progressive multiple cranial nerve palsies, cerebellar ataxia, and palatal tremor (PT) resulting from an unknown etiology. Magnetic resonance imaging showed evidence of hypertrophy of the inferior olivary nuclei, brain stem atrophy, and marked cerebellar atrophy. This combination of progressive multiple cranial nerve palsies, cerebellar ataxia, and PT has never been reported in the literature. We have also reviewed the literature of PT secondary to neurodegenerative causes. In a total of 23 patients, the common causes are sporadic olivopontocerebellar atrophy (OPCA; 22%), Alexander's disease (22%), unknown etiology (43.4%), and occasionally progressive supranuclear palsy (4.3%) and spinocerebellar degeneration (4.3%). Most patients present with progressive cerebellar ataxia and approximately two thirds of them have rhythmic tremors elsewhere. Ear clicks are observed in 13% and evidence of hypertrophy of the inferior olivary nucleus in 25% of the patients. The common neurodegenerative causes of PT are OPCA/multiple system atrophy, Alexander's disease, and, in most of them, the result of an unknown cause.     

Ciprofloxacin-induced palatal tremor.

We describe an 84-year-old man with an unusual clinical presentation of palatal tremor in association with ciprofloxacin treatment. The patient had rhythmical movements not only of the soft palate but also of the face and trunk. Complete resolution of the symptoms occurred 2 days after discontinuation of ciprofloxacin and administration of sodium valproate. This is the first reported case of palatal tremor secondary to the use of ciprofloxacin.     

Palatal tremor suppressed by mouth opening

We report clinical and neurophysiological findings in two patients with palatal tremor (PT). In both patients a prompt and persistent suppression of palatal movements and clicking sounds is caused by slight passive or active mouth opening. One patient has a typical essential palatal tremor (EPT) according to current classification criteria. The other one has a PT characterized by involuntary activation of levator veli palatini and genioglossus muscles. Objective clicking sounds were observed late during the course of the disease. A symptomatic aetiology was excluded, suggesting a diagnosis of atypical EPT. Our observations further confirm that unusual features of some cases may not fit current PT classification criteria. These two cases have several features that distinguish them from voluntary PT and from psychogenic PT. We emphasize that clear cut modulating phenomena may be relevant features in both typical and atypical involuntary EPT. We finally focus on the significance of jaw posture in determining PT suppression in our patients.     

Efficacy and complication of botulinum toxin injection in palatal myoclonus: experience from a patient.

We report the outcome of botulinum toxin injection for essential palatal myoclonus, given on two occasions over a period of one year, in an eight-year-old boy, the youngest patient treated with botulinum toxin to date. Though there was significant relief of ear clicks each time after the injection, he developed severe palatal palsy following the second injection, which persisted for a month. We suggest that appropriate caution needs to be exercised when repeating botulinum toxin injections for palatal myoclonus in children.     

Idiopathic palatal myoclonus

Two cases with idiopathic palatal myoclonus without other neurological deficits were described. They did not have any other neurological deficits other than myoclonus of branchial muscles. In these cases, the myoclonus disappeared during natural or induced sleep. In Case 1, the myoclonus ceased transiently when the patient was calculating or receiving an injection. In Case 2, the myoclonus disappeared with intravenous injection of saline as a placebo. Detailed examinations, including brain CT, MRI and multiple evoked potentials, showed normal results. The myoclonus in Case 2 disappeared after we had explained that her disease was benign. Since the clinical features and laboratory data in idiopathic palatal myoclonus are quite different from those in palatal myoclonus with other neurological deficits, idiopathic palatal myoclonus is considered to be a separate syndrome. Invasive examinations or excessive medications should be avoided because of its benign prognosis.     

Rhythmic skeletal myoclonus without palatal myoclonus]

We reported a case of 89-year-old woman showing rhythmic skeletal myoclonus mainly on the right upper limb. This myoclonus appeared five days after the cerebral infarction. It was seen constantly both at rest and in posture, and decreased during voluntary movement. When the patient was under emotional stress, it spread to the submandibular, neck and trunks of upper limb. During sleep, this movement completely disappeared. There was no myoclonus in palato-pharyngo-laryngo-oculo-diaphragmatic muscle group. In the examination of the surface electromyography, the movement was not reciprocal between extensor and flexor muscles, and its cycle was about 3.5 Hz. It was different from the intention tremor because it did not increase during the movement phase on the finger nose test. The examination of MRI revealed a small infarction including right dentate nucleus and right superior cerebellar peduncle, and from which an infarction of the superior cerebellar artery territory was considered. Only a few cases of rhythmic skeletal myoclonus without palatal myoclonus have been reported in the literature. All of these cases had small infarction of the same region as the above case. Their myoclonus began 5 to 15 days after the onset of cerebral infarction. These periods were markedly shorter than that of intention tremor and palatal myoclonus. This fact suggest that the rhythmic skeletal myoclonus has a different mechanism from that of the palatal myoclonus.     

Psychogenic palatal tremor

We describe a case of psychogenic palatal tremor. The diagnosis was supported by clinical criteria and neurophysiological testing, including frequency analysis and jerk‐locked back‐averaging. We discuss the differential diagnosis of palatal tremor as well as the role of neurophysiological testing in the diagnosis of psychogenic movement disorders. © 2005 Movement Disorder Society     

Autoantibodies to glutamic acid decarboxylase in palatal myoclonus and epilepsy

We report the presence of serum autoantibodies directed aginst glutamic acid decarboxylase in a patient with epilepsy and palatal myoclonus not associated with brain lesions. Glutamic acid decarboxylase antibody reactivity was dependent on the presence of carboxy-terminal amino acids, similar to that reported in patients with stiff-man syndrome. Marked reduction in the frequency of epileptic attacks and improvement in palatal myoclonus occurred when benzodiazepine was administered and phenytoin was gradually tapered. Testing for antiglutamic acid decarboxylase antibodies may be indicated in patients with palatal myoclonus and with convulsive disorders refractory to therapy.     

Voluntary palatal tremor is associated with hyperactivation of the inferior olive: a functional magnetic resonance imaging study.

Voluntary palatal tremor in a patient with essential palatal tremor induced activation predominantly within regions corresponding to the inferior olive, adjacent brainstem, and dentate nuclei. Finger movements elicited only ipsilateral lobular cerebellar activation, suggesting a dysfunctional nuclear activation by palatal tremor.     

Increased glucose metabolism in the medulla of patients with palatal myoclonus

Hypertrophic degeneration of the inferior olivary nuclei is the pathologic substrate for palatal myoclonus, but the physiologic correlate of this finding is uncertain. Using the 2-[18F]fluoro-2-deoxy-D-glucose and PET method, we determined the local cerebral metabolic rate of glucose utilization in seven patients with palatal myoclonus (following stroke or infection, or idiopathic), one patient with oculopalatal myoclonus (following a stroke affecting the brainstem), and nine normal subjects. The metabolism of glucose in the medulla of the patients with palatal myoclonus was significantly greater than that of the normal subjects. This may well have been due to increased metabolism of the inferior olivary nuclei. Glucose metabolism in the medulla of the patient with oculopalatal myoclonus was normal. These findings suggest that the inferior olivary nuclei, or a region of the brainstem encompassing the inferior olivary nuclei, are hypermetabolic in palatal myoclonus and may be the generators of the involuntary movements in palatal myoclonus.     

Case of essential palatal tremor: atypical features and remarkable benefit from botulinum toxin injection.

We describe a 21-year-old man with essential palatal tremor. The patient had rhythmic contractions not only of tensor veli palatini but also of facial, lingual, temporalis, pharyngeal, and neck muscles. He had some voluntary control of palatal tremor and ear clicks. He was treated with 5 units of botulinum toxin-A (BOTOX) injected into each tensor veli palatini, and had complete resolution of all the symptoms.     

Natural course of combined limb and palatal tremor caused by cerebellar-brain stem infarction.

After infarction of the left superior cerebellar peduncle and dentate nucleus, a patient developed tremor of the left upper limb beginning on the twelfth day followed by palatal tremor appearing 10 months after infarction. Surface electromyogram revealed a difference in the frequency of the tremor in the upper limb and soft palate. When the palatal tremor appeared, brain magnetic resonance T2-weighted images revealed high signal intensity of the contralateral, right inferior olivary nucleus. Subsequently, when the amplitude of palatal tremor became less severe, the high olivary signal intensity subsided whereas the hypertrophy of the nucleus remained. This patient provides useful information on the pathogenesis of skeletal and palatal tremor with brain stem or cerebellar lesions based on the differences in the onset and frequency of tremors and morphologic changes in the inferior olive.     

Psychogenic palatal tremor.

Recent criteria for the classification of palatal tremor use clinical, imaging, and electrophysiological features to differentiate essential and symptomatic forms. A case of probable psychogenic palatal tremor (PPT) is described within the context of these criteria, which lack clear guidelines for diagnosing PPT. The heterogenous nature of essential palatal tremor and its relationship with PPT, voluntary palatal movements, and tics is discussed.     

Clinical and imaging characterization of a patient with idiopathic progressive ataxia and palatal tremor

We describe clinical and imaging features of a patient with sporadic progressive ataxia and palatal tremor (PAPT) of unknown etiology. There was hypertrophy of bilateral inferior olivary nuclei with hyperintense T2-weighted signal and mild cerebellar atrophy at brain magnetic resonance imaging. 18F-fluoro-2-desoxy- d -glucose positron emission tomography scanning (FDG-PET) showed hypometabolism in the red nucleus, external globus pallidus and precuneus while FP-CIT-SPECT imaging revealed mild and progressive loss of striatal dopaminergic terminals. Our findings suggest that in idiopathic PAPT involvement of the dentato-rubro-olivary pathway occurs along with some dopaminergic dysfunction.     

Autosomal dominant palatal myoclonus and spinal cord atrophy

We report a new family with palatal myoclonus, pyramidal tract signs, cerebellar signs, marked atrophy of the medulla oblongata and spinal cord, and autosomal dominant inheritance. These findings were almost identical with those in patients previously reported to have histopathologically confirmed adult-onset Alexander disease. Recently, heterozygous point mutations in the coding region of glial fibrillary acidic protein (GFAP) in patients with an infantile form of Alexander disease have been reported. We found a new heterozygous amino acid substitution, Val87Gly in exon 1 of GFAP, in the affected individuals in this family but not in 100 spinocerebellar ataxia (SCA) patients and 100 controls. Therefore, this family might have new clinical entities related to adult-onset Alexander disease and GFAP mutation.     

Progressive ataxia and palatal tremor: Two autopsy cases of a novel tauopathy

Background: Sporadic progressive ataxia and palatal tremor is a rare syndrome characterized by mid‐ to late‐adult‐onset symptomatic palatal tremor and slowly progressive cerebellar ataxia. To date, there has been only one autopsy report, which described a novel 4‐repeat tauopathy with hypertrophic olivary degeneration and tau‐positive inclusions in olivary neurons and dystrophic neuritic processes termed glomeruloid bodies. We report on 2 additional autopsy cases. Methods: Sections from selected paraffin‐embedded brain regions were stained with hematoxylin and eosin/Luxol fast blue and processed for phosphorylated tau, 3‐repeat tau, 4‐repeat tau, neurofilament, glial fibrillary acid protein, phosphorylated α‐synuclein, phosphorylated TAR DNA‐binding protein 43, beta‐amyloid, and p62 immunohistochemistry. Results: Two male patients were aged 74 and 64 years at onset. Both had clinical findings consistent with progressive ataxia and palatal tremor and T2 hyperintensity in the bilateral olives on MRI. Pathological findings included bilateral hypertrophic olivary degeneration accompanied by glomeruloid bodies, 3‐repeat and 4‐repeat tau‐positive neuronal inclusions in the olive, and additional tauopathy in the midbrain, pons, and thalamus. Cerebellar cortical degeneration was extensive, but involvement of the dentate was minimal. P62‐positive, but tau‐ and TAR DNA‐binding protein 43–negative, inclusions in the cerebellum of 1 case was also a feature. Conclusions: Whereas our findings are largely in keeping with the previously published case report, we found a more extensive and mixed 3/4‐repeat tauopathy and additional cerebellar p62 pathology, highlighting our incomplete understanding of the pathogenesis of this disease. © 2017 International Parkinson and Movement Disorder Society