Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 38-year-old woman had an episode of headache, fever and convulsion in October, 1993. She became alert in two weeks, though weakness and atrophy remained in the upper limb muscles. She was diagnosed as Russian spring summer encephalitis (RSSE) based on several serological studies. Three kinds of involuntary movements were noted after recovery. These were spontaneous muscle jerks in the left arm, action tremor in the right arm and pathological associated-movement in the right leg. The movements were studied physiologically by electroencephalogram (EEG)-electromyogram (EMG) polygraphic recordings, jerk-locked averaging (JLA), magnetencephalography (MEG), and sensory evoked potentials (SEPs). The jerky movements in her left arm were observed at rest and aggravated by emotional stress. EEG-EMG polygraph showed that the jerks were sometimes associated with small spikes thus were considered to be due to epilepsia partialis continua (EPC). JLA analysis revealed a pre-myoclonus spike on the right hemisphere which preceded the onset of the jerk in the left first dorsal interosseous muscle by 25.2ms, which was equal to the magnetic cortical latency of that muscle. Jerk-locked magnetic field, obtained by averaging neuromagnetic activities with respect to the onset of myoclonus, showed that the spike preceding the jerk, originated from the right motor cortex. The spontaneous spikes were localized mainly on the right motor cortex. Sensory evoked potentials (SEPs) were normal in both size and latency. No hyperexcitability of the sensory cortex was demonstrated even by using paired stimulation SEPs. Based on these results, we conclude that the jerky movements in this patient were produced by abnormal spontaneous discharges in the motor cortex. The action tremor had a frequency of 4-5 Hz and its clinical features were compatible with cerebellar tremor. This is thought to result from a lesion in the left thalamus, affecting the cerebellothalamic pathways. Her right leg unintentionally moved whenever she began to move the hands. This was considered to be a pathological associated-movement due to dysfunction of the pyramidal tracts.
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PMID:[Involuntary movements observed in a patient with Russian spring summer encephalitis]. 881 Aug 52

We reported a sporadic case of DRPLA that had an elderly age of onset. The patient was a 71-year-old woman. Her parents, sisters and a brother didn't have neurologic diseases. She had been well until the age of 68 years, when she noticed her unstable gait. On neurologic examination cerebellar ataxia and a tremor at the neck were noticed, but we were unable to differentiate her disease from the other types of spinocerebellar degeneration. An MRI of the cranium showed atrophy of the cerebellum, pons, brain stem and cerebrum, and a diffuse lesion of the cerebral white matter. These findings made us suspect her disease of DRPLA. When we analyzed the CAG repeat in the DRPLA gene, we found it expanded to 57. We thought that the elderly onset related to a relatively mild expansion.
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PMID:[A sporadic case of dentatorubral-pallidoluysian atrophy (DRPLA) having an elderly age of onset]. 881 Aug 54

We report a 63-year-old woman who presented myotonia and parkinsonism. The patient was well until 15 years of the age when she noted that the ring finger of her left hand at times flexed when she did not intend to do so. She noted weakness in her left upper extremity at the age of 40, and difficulty in relaxing her hand grip at 45. She had an onset of tremor in her right foot at age 50, which was followed by difficulty in gait and hand writing. She was admitted to Juntendo University Urayasu Hospital when she was 63-year-old. Her mother, two sisters, and a son were affected with similar muscle weakness and myotonia. Although some of them developed stooped posture in the late stage of the disease, none of them had overt parkinsonism. General physical examination was unremarkable. Neurologic examination revealed an alert and oriented woman with some recent memory loss. She had bilateral ptosis, facial weakness, and a masked face. Myerson's sign was present. Her speech was small and monotonous. The sternocleidomastoid muscles were markedly atrophic and weak. The remaining of the cranial nerves were intact. She walked in small steps with freezing with support. She showed bradykinesia, retropulsion, and resting tremor in her right leg. Slight distal dominant weakness was noted in both upper and lower extremities more on the left. No cerebellar signs were noted. Muscle stretch reflexes were within normal limits in the upper extremities and diminished in the lower limbs. Sensation was intact. Routine laboratory findings were unremarkable. Cranial CT scan and MRI revealed slight cortical atrophy and leukoaraiosis. She responded to levodopa and she became able to walk by herself. She was transferred to another hospital one month after her admission. She had several bouts of airway obstruction with one episode of respiratory arrest. She expired six month after the transfer. The patient was discussed in a neurological CPC, and the chief discussant arrived at the conclusion that this patient suffered from myotonic dystrophy and Parkinson's disease which set in later years. Postmortem examination on the iliopsoas muscle revealed uneven muscle fiber diameters, central nuclei, and type 1 fiber predominance; the pathologic finding was consistent with myotonic dystrophy. The substantia nigra showed marked cell loss and Lewy bodies in the remaining neurons. The finding was consistent with Parkinson's disease. In myelin stain, diffuse myelin pallor was noted in the cerebral white matter which was the pathologic substrate of leukoaraiosis in this patient. Combination of these two disorders have never been reported in the literature to our knowledge. It appears to be that the coincidence is just a by-chance phenomenon, but it seems interesting to note that accelerated aging process appears to be present in both myotonic dystrophy and Parkinson's disease.
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PMID:[A 63-year-old woman with muscle weakness, myotonia, and parkinsonism]. 886 42

We report a 63-year-old woman with a progressive illness which began as a parkinsonian syndrome with bilateral rest tremor, limb rigidity and a gait disorder followed by cognitive decline, visuomotor apraxia and visual agnosia. She died 10 years after the onset of the illness and at autopsy the brain showed characteristic changes of progressive multifocal leukoencephalopathy (PML) with the presence of the JC virus confirmed by in situ hybridisation. Neuropathology also showed some unusual features in the form of atypical linear lesions at the cortico-white matter junction. Some of these lesions were active while others were inactive and similar to the rarely described "burnt out" lesions of PML. PML can in rare cases occur without an underlying immune disorder or malignancy (primary PML) and a parkinsonian syndrome can be produced by a predominantly white matter disorder.
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PMID:Primary progressive multifocal leukoencephalopathy presenting as an extrapyramidal syndrome. 886 94

We report a 43-year-old woman who died after 18 years history of parkinsonism. She was well until 25 years of the age (1976) when she noted a difficulty in stepping her feet. In the next year, she started to drag her feet. She was treated with levodopa with good response, however, she developed dyskinesia when she was 33 years of the age. She was evaluated in another hospital in 1984. She showed normal intelligence, normal ocular movement, masked face, small voice, small step gait, stooped posture, freezing of the gait, retropulsion, and cogwheel rigidity in limbs. No tremor or ataxia was noted. She received left ventrolateral thalamotomy at that time. Rigidity on the right side markedly reduced, however, she continued to show bradykinesia and motor fluctuations. On August 1 of 1994, she developed fever of 40 degrees C and dyspnea. On the next day, she expired from acute respiratory distress. She was able to walk unsupported until just before her last admission. The patient was discussed in a neurological CPC. The chief discussant arrived at the conclusion that this patient had Lewy body-positive young onset Parkinson's disease. Opinions were divided into two groups, i.e., young onset Lewy-body positive Parkinson's disease and Lewy-body negative young onset parkinsonism. Post-mortem examination revealed moderate loss of pigmented neurons in the substantia nigra more in the ventro-lateral part. Lewy bodies were found in the remaining neurons. Lewy bodies were more frequently seen in the locus coeruleus, although neuronal loss was less prominent in the locus coeruleus. The dorsal vagal motor nucleus showed moderate loss of neurons. Otherwise, the central nervous system was unremarkable. To our knowledge, this patient had the second youngest age of the onset so far reported in the literature for Lewy-body positive typical Parkinson's disease.
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PMID:[A 43-year-old woman with 18 years history of parkinsonism]. 892 38

A 21-year-old woman presented to the emergency department complaining of head, neck, and abdominal pain after being assaulted. She denied a previous history or significant symptoms of hyperthyroidism. The physical examination was remarkable for lethargy, low-grade fever, tachycardia, facial abrasions, swelling of the anterior neck, and a tremor. A diagnosis of thyroid storm was made. An extensive work up excluded other causes of the patient's tachycardia, altered mental status, and neck swelling. A review of the clinical features and management of thyroid storm is presented. Relatively minor trauma can be a precipitating event for thyroid storm.
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PMID:Thyroid storm precipitated by trauma. 896 88

Whipple's disease (WD) is a rare disorder that is more common in males than in females. Progressive supranuclear ophthalmoplegia (SNO) in conjunction with oculomasticatory myorhythmia (OMM) or oculofacioskeletal myorhythmia are characteristic movement abnormalities when WD involves the nervous system. Limb myorhythmia without facial or ocular myorhythmia has not been reported in WD. We report such a case who had SNO and leg myorhythmia but no facial or ocular myorhythmia. She had onset of WD at age 28 and 16 years later developed SNO and leg myorhythmia. The neurological manifestations did not respond to antimicrobial agents or to the drugs used for parkinsonism or essential tremor. Valproate produced a remarkable improvement in leg myorhythmia, but the efficacy declined after 3 months. Because WD may infest as a neurological disorder without gastrointestinal symptoms, all SNO cases, with or without OMM, and those with skeletal myorhythmia should be suspected of WD. These patients should be treated vigorously and followed carefully since neurological involvement is the most disabling feature and it has a propensity to relapse.
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PMID:Ophthalmoplegia and leg myorhythmia in Whipple's disease: report of a case. 899 64

Reported is a case of fetal death associated with a misoprostol overdose. The 25-year-old US woman, gravida 3 para 0 abortion 2, at 36 weeks' gestation presented to the hospital 3 hours after self-administering 6000 mcg of misoprostol intravaginally and 600 mcg orally, reportedly to induce and shorten the duration of labor. Prior to this regimen, the woman was experiencing mild contractions every 15-20 minutes and was aware of fetal movement. She presented with agitation and confusion, shaking chills, abdominal and extremity cramping, emesis, tachycardia, hypotension, and hyperthermia. Pelvic ultrasonography performed 3.5 hours after misoprostol administration showed no fetal movement or heart motion. A nonviable fetus was delivered by emergency cesarean section. Hypotension has not been previously reported in an overdose case and may reflect a direct vasodilatory effect of misoprostol on systemic vascular tone. Marginal placental abruption also may have contributed to the hypotension. The patient's clinical course resolved over 15 hours with supportive care, including intravaginal saline irrigation and endotracheal intubation with neuroparalytic therapy to control agitation and hyperthermia. Divided intravaginal doses of 50 mcg of misoprostol every 4 hours, for a total dose of 50-600 mcg, have effectively induced labor with no significant neonatal or maternal adverse outcomes. In developing countries, doses of 800-1600 mcg of misoprostol administered intravaginally have successfully induced first-trimester abortion.
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PMID:Acute intravaginal misoprostol toxicity with fetal demise. 901 89

We report a 83 year-old woman with dementia. She was apparently well until December of 1993 when she was 81-year-old. At that time, she was operated or her cataract. Her post operative course was uneventful, however, shortly after her operation, she had an onset of memory loss and abnormal behavior. She showed a fluctuating course in her mental disturbance. In 1995, her dementia worsened with nocturnal agitation. She was admitted to our service on June 12, 1995. She was alert and her blood pressure was 140/100 mmHg. She showed recent memory loss and disorientation to time. Motor wise, she was unable to stand unsupported. Her gait with support showed small steps and a wide base. She was bradykinetic and ataxic in her finger-to-nose and heel-to-knee test, however, no rigidity or tremor was noted. Her MRI showed T2-high signal lesions in both medial thalamic areas, in the right occipital lobe, and in the bilateral cerebral white matters as well as in the basal ganglia. She was discharged for out-patient follow up on July 3, 1995. Four days after the discharge, she showed declining responses to stimuli and she developed dyspnea on July 14, 1995. She was admitted again on the same day. Her body temperature was 38.5 degrees C and moist rales were heard in the left lung field. She appeared drowsy and no verbal response was obtained; no apparent motor palsy was noted. Blood count showed leukocytosis (14,300/ml). Blood gas analysis under 61 of oxygen inhalation through a mask was as follows: pH 7.460, PCO2 39.6 mmHg, PO2 67 mmHg, and HCO3-28.5 mEq/l. Two days after admission, she developed a convulsion in her left arm and she became unconscious. Her EEG showed periodically recurring lateralized epileptic discharges on the right fronto-central areas. Her subsequent course was complicated by status epilepticus and respiratory distress. She died on July 26, 1995. She was discussed in a neurological CPC. The chief discussant arrived at a conclusion that she suffered from multi-infarct dementia. Bilateral thalamic infarctions were considered to have played a significant role in her dementia. Post-mortem examination revealed subcortical leukoencephalopathy of Binswanger's type and cerebral infarctions in the thalamic and basal ganglia regions and in the right occipital lobe. In addition, she showed isolated angitis of the central nervous system involving mainly in the small arteries located in the superficial areas of the brain and the spinal cord. This patient was interesting in that despite relatively mild leukoaraiosis in MRI, post-mortem examination revealed profound pathologic changes in the subcortical white matters. In addition, she showed the isolated angitis of the CNS. The cause and the clinical correlates of her angitis were unclear.
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PMID:[A 83 year-old woman with dementia, gait disturbance, and convulsion]. 904 33

An autopsy case of pure akinesia (PA) is reported. The patient manifested L-dopa-unresponsive akinesia without accompanying rigidity, tremor, eye movement disorder or dementia from the age of 58 years. Brain magnetic resonance T2-weighted imaging at the age of 63 showed high intensity areas in the subthalamic regions, but brain atrophy was not observed. She received amantadine-HCl and L-threo-3,4-dihydroxyphenylserine (L-DOPS) for 5 years. At the age of 66, she died of the severe illness accompanied by consciousness disturbances, hyperthermia, muscle rigidity, abnormal blood pressure and elevated serum enzymes which were derived from the muscle. We considered her condition to be neuroleptic malignant syndrome (NMS). Pathologically the brain revealed degeneration in the subthalamic nucleus, globus pallidus and substantia nigra. Neurofibrillary tangles were detected in the temporal cortex, hippocampus, amygdaloid body and spinal cord, as well as in the basal ganglia, thalamus and brain stem. These findings were consistent with that of progressive supranuclear palsy (PSP); the change in the ventral pons was insignificant, suggesting that PA may have minimum involvement in the ventral pons. The skeletal muscle showed scattered necrosis that was compatible with NMS. As far as we know, this is the first report of NMS accompanied with PA.
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PMID:Pure akinesia manifested neuroleptic malignant syndrome: a clinical variant of progressive supranuclear palsy. 908 64


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