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)

Brain single photon emission computed tomography (SPECT) studies were conducted in three patients with A3243G mutation of the mitochondrial (mt) DNA tRNA. All were born to mothers suffering from chronic progressive external ophthalmoplegia (CPEO) with the same A3243G point mutation of the mtDNA tRNA. The first case manifested clinically with MELAS, the second case manifested with CPEO, and third case was characterized by recurrent migraine-like headache, tremor, and epilepsy. Brain SPECT of all patients, regardless of whether they had or had not suffered from stroke-like episodes, showed multiple areas of asymmetrical decreased perfusion, particularly in the posterior and lateral head regions, especially the temporal lobes. Crossed-cerebellar diaschisis may occur. Conventional brain magnetic resonance images failed to show some of the lesions. Decreased regional cerebral blood flow, rather than previously proposed hyperemia, is likely to be the cause. We conclude that mitochondrial vasculopathy with regional cerebral hypoperfusion may be seen on brain SPECT in patients with mitochondrial disorders and A3243G mutations, regardless of whether they have or have not suffered from stroke-like episodes.
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PMID:Brain single photon emission computed tomography in patients with A3243G mutation in mitochondrial DNA tRNA. 1596 44

We investigated the prevalence of cerebrovascular disease and other comorbidities in Parkinson's disease (PD) patients compared to the general population. Five hundred PD patients were chosen randomly from one author's (A.H.R.) database. Age- and sex-matched controls were derived from 270 patients with essential tremor from the same database and from 490 patients in a general practitioner's database. Age, hypertensive status, smoking status, coronary artery disease, orthostatic hypotension, diabetes mellitus, and symptomatic cerebrovascular disease (stroke or transient ischemic attack) were assessed. Statistical analysis was performed using Pearson chi(2) testing and binary logistic regression analysis. The prevalence of coronary artery disease, hypertension, diabetes mellitus, and orthostatic hypotension was similar among groups. The PD group had more patients who never smoked and less current smokers than the other groups. While there were similar frequencies of symptomatic cerebrovascular disease among groups, the prevalence of stroke was lower in PD patients. This difference disappeared upon stratification into groups based on smoking status and in the addition of smoking as a covariate in the multivariate analysis. Diminished smoking in PD patients likely plays a role in our finding of decreased stroke in patients with PD. Increased access to appropriate neurological care and subsequent prevention of stroke after a warning transient ischemic attack may also play a role, as may diminished levels of excitotoxic neurotransmitters in PD patients.
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PMID:Parkinson's disease, stroke, and related epidemiology. 1603 18

Fragile X-associated tremor/ataxia syndrome (FXTAS) is a newly described disorder that occurs in premutation carriers of the fragile X mental retardation 1 (FMR1) gene. Fifty-six patients with FXTAS were given 98 prior diagnoses: most were in the categories of parkinsonism, tremor, ataxia, dementia, or stroke. Data from this study and others were used to develop guidelines for FMR1 diagnostic testing for FXTAS.
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PMID:Initial diagnoses given to persons with the fragile X associated tremor/ataxia syndrome (FXTAS). 1604 84

This study describes a comprehensive method to assess neurological deficits after brain ischemia produced by sequential common carotid artery sectioning (SCAS) in aged mice, and a scale to determine the degree of functional incapacity of ischemic animals. The method involves an initial phase of undisturbed observation and a later manipulative phase during which each animal is subjected to a sequence of very simple manipulations. Sham-operated animals demonstrated 96% survival throughout the study period (72 h), whereas the 24, 48 and 72 h survival rates of SCAS-mice were 48, 38 and 36%, respectively. In the surviving SCAS-mice, we detected a total of 23 neurological alterations throughout the observation period (72 h); the most frequent alterations were: motor incoordination, abnormal body position, hypomobility, decreased body tone and muscular strength, tremor, hunched back, passivity, forelimb flexion and ataxic gait. Based on these alterations, we used a global scale that comprises 10 progressive grades beyond 0 (normal), extending to status 10 (death due to SCAS), with higher scores indicating greater deficit. The median neurological scores for sham-operated animals were 1.36, 1.48 and 1.32 at 24, 48 and 72 h, respectively, whereas total neurological scores in SCAS-mice of 6.1, 6.8 and 7.4, at 24, 48 and 72 h, respectively, were substantially greater than those observed in sham-operated animals. The simplicity of the procedure, herein described, to measure the functional neurological condition of ischemic animals, and the remarkable level of functional impairment produced by SCAS offer the possiblity to test the efficacy of putative stroke therapies and to monitor progress of deficits over time in groups of animals.
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PMID:A simplified procedure for the quantitative measurement of neurological deficits after forebrain ischemia in mice. 1605 13

Though basal ganglia calcification (BGC) has been recognized as a feature of mitochondriopathy, little is known about its frequency in a larger cohort. The aim of this work was to assess the frequency of BGC, type and frequency of clinical and additional imaging central-nervous-system (CNS) abnormalities and of non-CNS abnormalities in mitochondriopathy patients with BGC. Retrospectively reviewed were the records of all mitochondriopathy patients in whom BGC was found on cerebral CT during 10 years. Among those who underwent cerebral CT, thirty-six, 24 women, 12 men, aged 33-93 years, showed BGC. The most frequent clinical CNS manifestations in these patients were epilepsy (n = 9), Parkinson syndrome (n = 9), dementia (n = 7), dysarthria (n = 5), spasticity (n = 4), tremor (n = 4), or stroke (n = 4). Additional cerebral CT-findings were atrophy (n = 10), lacunas (n = 6), leucaraiosis (n = 6), focal gliosis (n = 4), or stroke (n = 1). MR imaging, carried out in 12 patients, confirmed BGC in one. The 36 patients presented with involvement of the CNS (n = 32), endocrine system (n = 29), peripheral nervous system (n = 28), heart (n = 23), inner ear (n = 16), eyes (n = 15), guts (n = 11), blood (n = 9), kidney (n = 2), or dermis (n = 2). BGC occurs in one sixth of non-selected patients with mitochondriopathy and is associated with clinical and imaging CNS abnormalities and multisystem disease in the majority of them.
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PMID:Basal Ganglia calcification in mitochondrial disorders. 1616 99

Stereotactic targeting strategies differ between thalamotomy and thalamic deep brain stimulation (DBS) for tremor control. In thalamotomy, a minimal radiofrequency lesion created within the lateral portion of the nucleus ventralis intermedius (Vim) often affords the best control of parkinsonian tremor, supporting the assumption that there is a concentrated cluster of cells within this area which is responsible for tremor. However, this assumption may not always be true; such neural elements sometimes appear to spread out across wide areas. Cells with tremor-frequency activity are widely distributed over the areas extending from the Vim to the nuclei ventralis oralis posterior and anterior (Vop and Voa). All of these cells appear to be more or less involved in tremor generation, especially in patients with essential tremor and post-stroke tremor. In contrast to radiofrequency lesions for thalamotomy, electrodes for DBS can be arranged in such a way that wide areas can be stimulated, if necessary. For this purpose, it is critically important to determine optimal placement and orientation of DBS leads for arranging the electrodes to yield maximal benefits in patients with tremor.
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PMID:Difference in surgical strategies between thalamotomy and thalamic deep brain stimulation for tremor control. 1622 33

Laryngeal physiology depends on dynamic neuromuscular forces acting on a basic framework of cartilage and specialized soft tissues, that is, the vocal folds. A working understanding of this organ in health and disease requires knowledge of specific neurological processes that may affect voice, swallowing, and airway regulation. Neuromuscular impairment continues to be a dominant topic in the study of laryngeal disorders. This subcommittee presentation reviews important aspects of the neurolaryngeal history and physical examination. After this foundation, 4 common movement disorders affecting the larynx are covered in separate subsections. These are stroke, Parkinson's disease, laryngeal tremor, and spasmodic dysphonia. State-of-the-art reviews reflecting our understanding of these clinical issues are presented in this summary.
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PMID:Common movement disorders affecting the larynx: a report from the neurolaryngology committee of the AAO-HNS. 1627 88

Hashimoto's encephalopathy (HE) is a severe but treatable condition that rarely complicates Hashimoto's thyroiditis. Clinically it is characterized by progressive or relapsing symptoms, including tremor, myoclonus, stroke-like episodes, seizures, impairment of consciousness, and dementia. We describe a patient presenting with recurrent generalized convulsive status epilepticus (GCSE), despite antiepileptic medications, who was successfully treated with methylprednisolone. Our observation confirms that the clinical spectrum of HE at presentation is heterogeneous and diagnosis is often difficult. This case highlights the crucial importance of antithyroid antibody measurement in patients presenting with otherwise unexplained episodes of GCSE with or without adjunctive signs of encephalopathy or thyroiditis.
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PMID:Recurrent status epilepticus as the main feature of Hashimoto's encephalopathy. 1634 98

We report three patients with anticholinergic poisoning caused by the substitution of hyoscine hydrobromide for hyoscine butylbromide in preparations compounded by two different pharmacists. The patients took the preparations for gastrointes tinal discomfort and presented with altered mental status tachycardia, facial flushing, dilated pupils, and dry skin shortly after the ingestion. In one patient the intoxication was initially not recognized and he was treated as suffering from an acute cerebrovascular accident. Two patients experienced long-lasting effects such as decreased ability to concentrate, memory dis turbances, tremor, and photo- and phonophobia. It was obviously impossible to elucidate the exact nature of the relationship between the intoxication and these long-lasting complaints. Information from the Belgian poison control center revealed that cases of substitution error with hyoscine hydrobromide are not unique The Belgian authorities issued a warning to all pharmacists.
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PMID:Three cases of substitution errors leading to hyoscine hydrobromide overdose. 1644 May 14

Hashimoto's encephalopathy (HE) has been described as an encephalopathy, with acute or subacute onset, accompanied by seizures, tremor, myoclonus, ataxia, psychosis, and stroke-like episodes, with a relapsing/remitting or progressive course. HE patients have positive antithyroid antibodies, are usually in a subclinical hypothyroid state, have elevated cerebral spinal fluid (CSF) protein, and have nonspecific electroencephalogram (EEG) and imaging abnormalities in the absence of CNS infection, tumor, or stroke. The authors present two cases of HE, demonstrating an excellent response to high dose steroids acutely followed by long-term treatment with steroids and other immunomodulatory agents. A review of the literature is also provided.
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PMID:Long-term treatment of Hashimoto's encephalopathy. 1652 66


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