Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027066 (myoclonus)
 4,275 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Two patients are described, in whom MRI detected unilateral signal abnormalities in the region of the inferior olivary nuclei, suggesting an early stage of olivary hypertrophy. MRI was performed 4 and 7 weeks respectively after traumatic brain-stem injury. Palatal myoclonus was concomitantly observed in one patient, while the other showed no evidence of segmental myoclonus at the time of examination. The authors conclude that MRI is highly sensitive in the detection of olivary hypertrophy and of traumatic lesions of the dentato-rubro-olivary pathway.
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PMID:Early detection of post-traumatic olivary hypertrophy by MRI. 841 80

Palatal myoclonus is a rare syndrome characterized by involuntary rhythmical movements of the soft palate giving rise to clicking objective tinnitus. The intrusive nature of the tinnitus prompts patients to seek medical advice but to date no single treatment modality has been shown to be consistently effective. We present three cases in whom various management regimens were unsuccessful and in whom botulinum toxin injection to the palatal muscles was undertaken. All three cases were rendered free of their tinnitus with complete abolition of the myoclonus. The questions of optimum dosage as well as frequency of injection will be answered as greater numbers are treated by this method.
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PMID:The use of clostridium botulinum toxin in palatal myoclonus. A preliminary report. 850 97

A 45-year-old woman developed a rare case of palatal myoclonus with no other neurological signs after undergoing extirpation of a small cerebellar low-grade astrocytoma that was located in the right dentate nucleus. The palatal myoclonus has persisted for 4 years after the operation. Magnetic resonance T2-weighted imaging revealed a high-intensity lesion in the left inferior olive. Palatal myoclonus associated with the removal of cerebellar tumors is unusual but may easily be overlooked.
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PMID:Palatal myoclonus induced by extirpation of a cerebellar astrocytoma. Case report. 960 9

Palatal myoclonus is usually due to a brainstem or cerebellar lesion disrupting the dentato-rubro-olivary pathway. Rarely it may be caused by a cortical lesion. The precipitating factor in 70% of all cases is an infarct. We describe an unusual case of a patient with palatal myoclonus who had an old ipsilateral cerebellar infarct and a new contralateral subcortical (corona radiata) infarct. We postulate that the new infarct caused disinhibition of the old cerebellar infarct, resulting in palatal myoclonus. Magnetic resonance imaging (MRI) of the brain did not show any hypertrophy of the inferior olivary nucleus. Her myoclonus proved refractory to clonazepam, valproate and phenytoin.
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PMID:Palatal myoclonus--a case report. 1056 80

Palatal myoclonus is a rare neurological disorder of the soft palate and other oropharyngeal muscles, which causes clicking tinnitus. The latter is audible both to the patient and to an observer. The aetiology may be a brain stem lesion, and it is only rarely that a cause cannot be identified. The condition has been described in adults, but seldom in children. We present here a case of palatal myoclonus with distressing tinnitus in a 12-year-old girl and its successful treatment with electromyography (EMG)-guided injections of Clostridium botulinum toxin.
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PMID:Palatal myoclonus and clicking tinnitus in a 12-year-old girl--case report. 1090 79

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.
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PMID:Symptomatic palatal myoclonus with ear click after tick-borne meningoencephalitis. 1670 86

Palatal myoclonus is an uncommon, rhythmic, "shock-like" involuntary movement of the muscles of the soft palate, throat, and other structures derived from the branchial arcs. Objective tinnitus is frequently neglected in review articles about childhood tinnitus. Our aim was to present the case of a 7-year-old girl with bilateral objective tinnitus due to palatal myoclonus without hearing impairment (normal hearing thresholds between 250 Hz and 8 kHz) but with otherwise normal hearing thresholds (250 Hz-8 kHz) and no evidence of intracerebral or systemic disorders. No treatment was useful.
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PMID:Objective tinnitus associated with essential palatal myoclonus: report in a child. 1822 97

An 8-year-old boy was seen by his primary care pediatrician with a chief complaint of "intermittent rapid vibrations of the epiglottis" that began several weeks prior. Intraoral examination revealed rapid, symmetrical bilateral contractions of the soft palate muscles (velum), accompanied by clicking sounds audible to physician (objective tinnitus) and patient. The patient was able to volitionally control the initiation and cessation of the palatal movements. The child's mother stated that there had been no clicking noises heard while the boy was sound asleep. Palatal "clonus" was tentatively diagnosed as the cause of the problem. A normal magnetic resonance imaging study with contrast enhancement confirmed that there was no anatomical basis for the localized movement disorder.Palatal myoclonus is an uncommon localized intraoral movement disorder. There are 2 distinct types, and our patient was diagnosed with the essential palatal myoclonus type. This type is characteristically associated with clicking tinnitus, heard by the affected person as well as those in close proximity. The clicking noise is not continuous, ceases during sleep, and is not lifelong.
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PMID:Loud clicking sounds associated with rapid soft palate muscle contractions. 2230 83

Palatal myoclonus is a rare condition in which there are rhythmic jerky movements of the soft palate and sometimes of the other muscles innervated by the brainstem A particularly annoying symptom is a rhythmic clicking sound in the ear due to the opening and closing of the Eustachian tube. Orofacial buccal dystonia is a focal dystonia with sustained spasms of the masticatory, facial or lingual muscles. The frequent symptoms of this disease have mainly been reported to be involuntary and possibly painful jaw opening, closing, deflecting and retruding, or a combination of the above. However, the subtle and unnoticeable involuntary movement of multiple facial muscles, which might be an infrequent symptom of orofacial buccal dystonia, makes this disease hard to diagnose. Understanding the functional orofacial anatomy that is responsible for the clinical signs and symptoms is necessary for making a proper diagnosis. Here we report on a rare case of palatal myoclonus that was associated with orofacial buccal dystonia, and such a case has not been previously reported. We describe the diagnostic approach and excellent treatment results after Botulinum toxin A (Dysport) injection and proper counseling.
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PMID:Palatal myoclonus associated with orofacial buccal dystonia. 2246 2

In 2010, we reported the successful clinical outcome related to a 20-month course of intravenous, cyclical ceftriaxone, in a patient with adult-onset Alexander's disease. We now provide evidence that the progression of the patient's signs/symptoms was halted and reversed with a 4-year-long extension of the trial.The patient's clinical signs/symptoms were evaluated before the start and every 6 months for 6 years. For the early 2 years, without therapy, and for the following 4 years, after intravenous ceftriaxone 2 g daily, for 3 weeks monthly during the initial 4 months, then for 15 days monthly.Gait ataxia and dysarthria were assessed clinically on a 0 to 4 scale. Palatal myoclonus and nystagmus/oscillopsia were monitored by videotape and a self-evaluation scale. The degree of disability, measured by a modified Rankin scale, and the brain MRI were periodically evaluated.Before ceftriaxone therapy, in a 2-year period, gait ataxia and dysarthria worsened from mild to marked, palatal myoclonus spread from the soft palate to lower facial muscles, and the patient complained of oscillopsia. After 4 years of ceftriaxone therapy, gait ataxia and dysarthria improved, from marked to mild at clinical rating scales. The palatal myoclonus was undetectable; the patient did not complained of oscillopsia and declared a progressively better quality of life. Ceftriaxone was safe.This case report provides Class IV evidence that intravenous cycles of ceftriaxone may halt and/or reverse the progression of neurodegeneration in patients with adult-onset Alexander's disease and may significantly improve their quality of life.
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PMID:Ceftriaxone for Alexander's Disease: A Four-Year Follow-Up. 2343 May 49


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