Ear click in palatal tremor: its origin and treatment with botulinum toxin

We report the successful treatment of a rhythmic, continuing ear click in a patient with palatal tremor with local injections of botulinum toxin into the tensor veli palatini muscle. We could demonstrate that the ear click occurred during contraction of the tensor veli palatini, which opens the eustachian tube. Therefore, we believe that the clicking noise is due to the sudden breakdown of the surface tension within the eustachian tube. Our observations suggest that the ear click is due to rhythmic discharges of the trigeminal nucleus rather than the ambiguous nucleus.     

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.     

Unusual position-sensitive jaw tremor responsive to botulinum toxin.

We report on a woman with a focal position-sensitive jaw tremor present with the jaw held slightly open or while drinking from a cup. Electromyography demonstrated a rhythmic 5 Hz alternating tremor involving masseter and digastric muscles with normal reciprocal inhibition. The tremor was markedly reduced with botulinum toxin injections.     

Botulinum toxin is effective and safe for palatal tremor

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.     

Isolated high-frequency jaw tremor relieved by botulinum toxin injections.

Jaw tremor can be seen as a component of various neurological disorders such as essential tremor, Parkinson's disease, dystonia, branchial myoclonus, hereditary geniospasm, task-specific tremor, and Whipple's disease, as well as in normal situations such as shivering, and subclinical physiological jaw tremor. In most of these conditions, the jaw tremor is usually associated with tremor or other abnormal involuntary movements affecting additional body parts, and its frequency is lower than 12 Hz. Schrag and colleagues reported a patient with a high-frequency idiopathic jaw tremor, and they speculated it could be related to orthostatic tremor affecting the masseter muscles. We encountered a similar patient with intermittent rapid focal jaw tremor that was successfully treated with botulinum toxin injections to the masseters.     

Voluntary control and a wider clinical spectrum of essential palatal tremor.

Essential palatal tremor (EPT) may not be a uniform or single entity. We present two patients who had some "voluntary control" over EPT, including entrainment. We review the English language literature on EPT to describe a wider clinical spectrum of this syndrome.     

Safety and efficacy of botulinum toxin in primary orthostatic tremor

Primary orthostatic tremor (POT) is a rapid 13–18 Hertz tremor that produces a subjective feeling of unsteadiness when standing, and is absent when seated or supine. It predominantly affects the legs during isometric contraction though a similar tremor can be seen in the arms and jaw. When present in the jaw this rapid tremor has been successfully treated with botulinum toxin. We sought to test whether symptoms of POT improved following injection of abobotulinumtoxinA to muscles in the legs. This randomised, double blind, placebo controlled cross-over design study enrolled eight patients with electrophysiologically confirmed POT. Each patient received injections of either 200 mU abobotulinumtoxinA or 0.9% saline in the tibialis anterior bilaterally, with cross-over after 20 weeks. Electrophysiological and clinical assessments were performed before and 6 weeks after each injection. Seven patients completed the study. No significant differences were seen in the primary outcome measures of time from standing to unsteadiness or symptom diary scores. Electrophysiological characteristics of POT remained remarkably constant throughout the study in all patients with variability of less than 1 Hertz in the frequency recorded. Falls were common, with one patient experiencing a fall with upper limb fracture whilst on the placebo. The frequency of falls correlated with both the severity of the self-rated symptoms and a shorter time to feeling unsteady with eyes closed. In conclusion, treatment with 200 mU of abobotulinumtoxinA in the tibialis anterior does not alter subjective experience of unsteadiness in POT. Postural instability and falls are common.     

Case of a palatal tic resembling palatal tremor in a patient with Tourette syndrome.

We describe a case of a palatal tic resembling palatal tremor (PT) in a young female patient with a previously unrecognized mild Tourette syndrome. At the time of her visit, the patient complained about ear clicks that were audible to others. We discuss the differential diagnoses of hyperkinetic palatal movements emphasizing the ongoing discussion about essential PT representing a more heterogeneous disorder than previously thought.     

特发性震颤的临床和电生理学特点

目的 探讨特发性震颤(EI)的临床和电生理学特点.方法 回顾性分析并比较33例ET患者(ET组)和30例生理性震颤患者(对照组)的震颤类型、程度、幅度和负重对其的影响,以总结ET的临床和电生理学特点.结果 ET组患者动作性震颤(KT)的震颤程度明显高于对照组,震颤幅度明显大于对照组(均P＜0.01);而姿势性震颤(PT...     

A randomized, double masked, controlled trial of botulinum toxin type A in essential hand tremor

OBJECTIVE: To evaluate the safety and efficacy of botulinum toxin type A injection in essential tremor of the hand. BACKGROUND: Botulinum toxin type A is an effective treatment for dystonia, spasticity, and other movement disorders and has been found to be useful in open-label studies and one double-masked study of essential hand tremor. METHODS: One hundred thirty-three patients with essential tremor were randomized to low-dose (50 U) or high-dose (100 U) botulinum toxin type A (Botox) or vehicle placebo treatment. Injections were made into the wrist flexors and extensors. Patients were followed for 16 weeks. The effect of treatment was assessed by clinical rating scales, measures of motor tasks and functional disability, and global assessment of treatment. Hand strength was evaluated by clinical rating and by a dynamometer. RESULTS: Both doses of botulinum toxin type A significantly reduced postural tremor on the clinical rating scales after 4 to 16 weeks. However, kinetic tremor was significantly reduced only at the 6-week examination. Measures of motor tasks and functional disability were not consistently improved with botulinum toxin type A treatment. Grip strength was reduced for the low- and high-dose botulinum toxin type A groups as compared with the placebo group. Adverse reactions consisted mainly of dose-dependent hand weakness. CONCLUSION: Botulinum toxin type A injections for essential tremor of the hands resulted in significant improvement of postural, but not kinetic, hand tremors and resulted in limited functional efficacy. Hand weakness is a dose-dependent significant side effect of treatment at the doses used in this study.     

Self-reported benefit and weakness after botulinum toxin in dystonia

With this retrospective, single center, chart review study, we investigate the self-reported benefit and weakness after botulinum toxin injections in three different types of dystonia: focal hand dystonia (FHD), blepharospasm and cervical dystonia. We found that the benefit lasts significantly longer in FHD compared to the other two groups.     

Electrophysiologic transition from physiologic tremor to essential tremor.

We electrophysiologically examined the transition from physiologic tremor to essential tremor in people at risk for familial essential tremor. Two healthy people from different families with hereditary essential tremor were studied on multiple occasions. A 23-year-old man was studied in 1995, 1997, and 2004, and a 44-year-old woman was studied in 1993, 1995, 1997, and 2003. Hand acceleration and forearm electromyographic readings were measured with and without 300-g loading to determine the characteristic frequency-invariant motor unit entrainment of essential tremor. Clinically and electrophysiologically, the man and woman had normal tremor until the last examination, when both exhibited a fine tremulousness in the outstretched hands and frequency-invariant motor unit entrainment at 7.5 and 6.5 Hz, respectively. At no time did either patient exhibit a prominent 8-12 Hz component of physiologic tremor. Essential tremor in young adults may begin at frequencies less than 8-12 Hz, and this electrophysiologic abnormality is detectable when clinical examinations reveal only questionably abnormal tremor. More young adults at risk for essential tremor must be studied to determine whether initial frequencies less than 8 Hz are the rule or the exception. Nevertheless, the data from our 2 patients demonstrate that a prominent 8-12 Hz component of physiologic tremor does not always precede the development of essential tremor; therefore, the origins of essential tremor and the 8-12 Hz component of physiologic tremor may be different.     

Clinical features of childhood onset essential tremor

Childhood onset essential tremor (ET) is uncommon. It is not clear as to whether ethnicity-specific differences may influence the phenotypic features. To determine the frequency and clinical characteristics of childhood ET in a tertiary referral center. In a prospective evaluation of 120 consecutive ET patients in a movement disorders clinic, we found a 15.5% (19) frequency of childhood onset ET patients. The mean age of onset and mean age was 10.8 ± 4.1 (6–16) years and 25.7 ± 15.0 (16–73) years consisting of 73.6% (14/19) men and 26.4% (5/19) women. A positive family history of ET was present in 11 of 19 (52.6%). Presence of a head tremor was observed in 2/19 (10.5%). We highlighted a relatively high frequency (15,5%) of childhood ET in our Asian cohort. In addition, we drew attention to the male preponderance and the low frequency of head tremor in childhood ET corroborating study findings in white ET patients. These observations appear to transcend ethnic and cultural differences and lend further support that gender difference may play a role in the pathogenesis and expression of ET.     

Distant effects of local injection of botulinum toxin

We studied five patients who received local injections of botulinum toxin for dystonic disorders to determine if there had been any distant effects on neuromuscular transmission. No patient developed weakness or abnormalities on routine electrophysiologic testing. In all patients who received more than 245 U of toxin, SFEMG in the extensor digitorum communis muscle, a muscle distant to all those injected, was abnormal. Fiber density was increased in the two patients who received the largest cumulative dose. Jitter was maximally increased at slow firing rates, confirming its presynaptic basis. Our results reveal that there is an effect on neuromuscular transmission in muscles distant to those injected with botulinum toxin for dystonia.     

Severe botulism after focal injection of botulinum toxin

We report a 34-year-old woman who developed clinical botulism after the cosmetic use of an unapproved botulinum toxin type A. Electrophysiologic findings demonstrated complete denervation with complete electrical silence. She had a lengthy recovery but was able to ambulate by discharge.