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)

Palatal myoclonus with its concomittant objective tinnitus is often a missed diagnosis and poses a problem to the clinician in separating it from other middle ear anomalies. The etiology and symptomatology is outlined, but as suggested by our case presentations are not always diagnostic. The anatomical pathways of palatal myoclonus are traced, and its etiological complexities are realized when any locus or pathology along these tracks may create the characteristic chronic rhythmic contractions. We have experienced excellent results in determining a differential diagnosis of palatal myoclonus from other middle ear problems by the correlations of impedance audiometry with our clinical findings. Although the etiology is not defined the target structures can be separated from this test method and, therefore, enable more specific therapy.
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PMID:The otological aspects of palatal myoclonus. 93 88

Palatal myoclonus is thought to occur after damage to certain brain-stem structures, and with a delay following the causative lesion. A case of palatal myoclonus, probably of epileptic nature, is described.
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PMID:Palatal myoclonus occurring during complex partial status epilepticus. 159 90

A seven-year-old girl with Krabbe disease presenting palatal myoclonus only when awake is reported. The patient was diagnosed as having Krabbe disease enzymatically at the age of eleven months. She developed rhythmical contractions of the soft palate, pharynx, larynx, lips and tongue at two years. The surface electromyography showed rhythmical 2 Hz electrical activities. The MRI disclosed markedly attenuated intensity in the midbrain, pons and medulla oblongata on T2-weighted images. Palatal myoclonus was not controlled by carbamazepine in therapeutic doses, but disappeared when the patient was asleep. This is the first reported case of Krabbe disease with palatal myoclonus.
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PMID:Palatal myoclonus in Krabbe disease. 178 60

Palatal myoclonus is a movement disorder consisting of rhythmic myoclonus of the soft palate, pharynx, larynx, and other muscles derived from the embryonal branchial arches. These movements are continuous and involuntary, and the patients are, in general, unaware of them. In the majority of patients, palatal myoclonus persists for life. In oculopalatal myoclonus, the eyes can be involved in the form of a nystagmus. Often a clicking noise in one or both ears is the initial symptom which can be heard by the examiner. A variety of etiologies have been linked to palatal myoclonus. The most common defined cause is a stroke. The variable delay between the proposed cause and the appearance of the disorder causes difficulties in determining the exact etiology. Pathologic findings show a transsynaptic hypertrophic degeneration of the inferior olivary nucleus which is due to a lesion of a specific, inhibitory, anatomic pathway. This somatotopic pathway leaves the contralateral dentate nucleus, passes through the superior cerebellar peduncle, and crosses the posterior commissure before joining the central tegmental tract and descending to the ipsilateral inferior olive. Treatment of palatal myoclonus is only occasionally effective. Some patients have responded to tryptophan, carbamazepine, and trihexyphenidyl. Surgical attempts have not been successful. - In the present paper the authors report on a case of an oculopalatal myoclonus following Leber's optic atrophy which involved the brain stem.
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PMID:[Etiology and clinical aspects of palatal myoclonus]. 224 51

Palatal myoclonus associated with extremity movements such as myoclonus or tremor is uncommon and reports are rare. Five patients with palatal myoclonus and a rest tremor are presented. In four patients, a slow rest tremor (3 Hz or less) was present. The tremor persisted on sustained posture and finger-to-nose maneuvers and was usually not synchronous with the palatal movements. It was not associated with clinical manifestations of Parkinson's disease and occurred in conjunction with brain-stem infarction in three patients.
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PMID:Palatal myoclonus associated with extremity tremor. 261 93

Palatal myoclonus and acquired pendular nystagmus result from lesions in dentatorubroolivary pathways. We have investigated the effect of high doses of the anticholinergic drug trihexyphenidyl in four patients with palatal myoclonus and in four patients with acquired pendular nystagmus. The movements of each patient were videotaped three times: before administration of trihexyphenidyl, at the time of maximum or effective dosage, and after withdrawal from trihexyphenidyl. In five patients the movements were also electrographically recorded. A neurologist not familiar with the patients reviewed the tapes and rated the changes. In seven of eight patients, administration of trihexyphenidyl resulted in marked improvement of both movements and complaints by patients. This observation indicates that disturbance of cholinergic mechanisms plays an important role in the pathophysiology of these two movement disorders.
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PMID:Effectiveness of trihexyphenidyl against pendular nystagmus and palatal myoclonus: evidence of cholinergic dysfunction. 350 67

We describe a 74 year old man who showed the jaw opening phenomenon by painful stimuli from two months after the onset of basilar artery thrombosis. He was admitted to our hospital because of consciousness disturbance and paralysis of all extremities. Soon after admission, he was in a state of impending herniation but with conservative therapy he recovered slightly, and then fell into an akinetic and mute state. Two months after the onset of the stroke, he began to open his mouth in response to painful stimuli, and five months after the stroke palatal myoclonus also appeared. Neurological signs and symptoms five months after admission were as follows; he was akinetic, mute and always kept his eyes closed because of complete blepharoptosis due to oculomotor nerve palsy. Pupils were dilated and adducted. Bilateral light reflexes were absent and the oculocepharic reflex could not adduct the eyes inwardly. Bilateral corneal reflexes were present, facial reflexes were exaggerated and jaw reflexes were also active. All limbs were spastic and paralyzed, and no voluntary movement was observed. Deep tendon reflexes were active in all extremities, and bilateral plantar responses were extensor. Palatal myoclonus was recognized in his soft palate, lips, sternocleidomastoid and diaphragm. Its frequency was about 150 cycles per minute. CT scan revealed severe low density areas in the midbrain and bilateral posterior lobes. In cerebral angiography, the upper part of the basilar artery was completely occluded.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of jaw opening phenomenon associated with basilar artery thrombosis]. 400 70

Palatal myoclonus is defined as a continuous, rhythmic contraction of the palatal musculature. Reverberant neuronal activity in a region of the brain stem known as the Guillain-Mollaret triangle is believed to underlie this condition. We present a case of palatal myoclonus which could be abolished by anterior neck flexion. The pathology and management of this condition is briefly discussed.
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PMID:Palatal myoclonus affected by neck position. 872 17

Olivary pseudohypertrophy (OH) and its chronological change was examined by MR images in two patients with brainstem vascular disease. Patient 1 was a 63-year-old woman who developed an infarction in the red nuclei associated with "top of the basilar" syndrome. Two months later, she showed 2-4 c/s rhythmic myoclonus (rubral tremor) involving four extremities. Palatal myoclonus was absent. MR images of the inferior olives did not demonstrate a significant lesion in 10 days after the onset, but showed OH in 6 months, and then their size attained a maximum in 10 months after. On T2-weighted (T2) images and proton-density-weighted (PD) images obtained at 20 and 24 months, OH gradually became irregular but discrete in their intensity, and the intensity had also decreased to some extent. Rhythmic myoclonus had subsided to some extent after 20 to 24 months. Patient 2 was a 62-year-old woman who had a small hemorrhage in the pontine tegmentum. She developed 2.5 c/s vertical ocular myoclonus without palatal myoclonus two months after the onset. MR images showed OH in 6 and 8 months after the onset. On T2 and PD images obtained at 20 months, the image of OH gradually developed to become irregular in intensity and slightly atrophic in size. The ocular myoclonus somewhat reduced in their intensity 12 months after the onset. These serial changes in MR images were considered to correspond to the chronological changes of the pathology of OH. Appearance and subsidence of the myoclonic movement was also considered to correlate to the sequential changes of MR images of OH.
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PMID:[Chronological changes in MR imaging of inferior olivary pseudohypertrophy--report of two cases]. 789 37

The authors analyzed rhythmical involuntary movements at rest, which appeared as complications in 12 (sporadic 11, hereditary 1) out of a total of 139 cases (sporadic 99, hereditary 40) of olivo-ponto-cerebellar atrophy. These movements tended to be seen in patients with sporadic OPCA of longer illness duration and at more advanced stages. They were distributed over the face, neck and extremities. Palatal myoclonus was observed in only one case. The movements were exacerbated by maintenance of a fixed posture, motion and mental stress, and stopped during sleep. In some cases, clonazepam, trihexyphenidyl or 1-Dopa was effective. In the surface electromyogram, rhythmical grouped discharges of 2-4 Hz were recorded only on agonist muscles or on both agonist and antagonist muscles synchronously, which is characteristic of skeletal myoclonus. However, pathological study of 3 cases with involuntary movements revealed marked putaminal degenerations as compared with 3 uncomplicated cases. This suggests that these rhythmical movements might be related to parkinsonian tremor.
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PMID:[Rhythmical involuntary movement at rest associated with olivo-ponto-cerebellar atrophy (OPCA)]. 833 81


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