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Query: UMLS:C0040822 (tremor)
 18,428 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

X-linked spinal and bulbar muscular atrophy or Kennedy's disease is an adult-onset motor neuronopathy caused by a CAG repeat expansion within the first exon of an androgen receptor gene. We report the case of a 66-year-old man, previously diagnosed with motor neuron disease (MND), who presented acute and reversible left vocal fold (dysphonia) and pharyngeal paresis, followed by a slowly progressive weakness and also bouts of weakness, wasting and fasciculation on tongue, masseter, face, pharyngeal, and some proximal more than distal upper limb muscles, associated to bilateral hand tremor and mild gynecomastia. There were 5 electroneuromyography exams between 1989 and 2003 that revealed chronic reinnervation, some fasciculations (less than clinically observed) and rare fibrillation potentials, and slowly progressive sensory nerve action potentials (SNAP) abnormality, leading to absent/low amplitude potentials. PCR techniques of DNA analysis showed an abnormal number of CAG repeats, found to be 44 (normal 11-34). Our case revealed an acute and asymmetric clinical presentation related to bulbar motoneurons; low amplitude/absent SNAP with mild asymmetry; a sub-clinical or subtle involvement of proximal/distal muscles of both upper and lower limbs; and a probable evolution with bouts of acute dennervation, followed by an efficient reinnervation.
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PMID:X-linked spinal and bulbar muscular atrophy (Kennedy's disease) with long-term electrophysiological evaluation: case report. 1583 83

Spinocerebellar ataxia type 20 (SCA20) was reported in 2004 in a single Australian Anglo-Celtic pedigree. The phenotype is distinctive, with palatal tremor, and hypermetric saccades, and early dentate (but not pallidal) calcification in the absence of abnormalities of calcium metabolism. Dysarthria, rather than gait ataxia, was the initial symptom in most, and was typically conjoined with dysphonia, clinically resembling adductor spasmodic dysphonia. The onset of these speech abnormalities was abrupt in some cases. MRI scanning showed mild to moderate pancerebellar atrophy with dentate calcification, with olivary pseudohypertrophy in some cases, in the absence of other brainstem or cerebral changes. Nerve conduction studies were normal. Progression appeared to be slow. SCA20 is probably rare, as despite the distinctive phenotype, only this one pedigree has been described. The locus mapped to the pericentromeric region of chromosome 11 with a LOD score of 4.47, and its candidate region overlaps that of SCA5. It seems probable that these two SCAs may be separate genetic entities, on the basis of their divergent clinical features, but formal proof awaits discovery of one or both responsible genes.
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PMID:Spinocerebellar ataxia type 20. 1589 61

A 10-year-old boy presented with fever, headache, vomiting, and hypersomnolence. An akinetic-rigid syndrome with tremor, dysphagia, dysphonia, and sialorrhea, as well as pyramidal signs, developed. Slightly elevated protein content was found in the cerebrospinal fluid and serological investigations were suggestive of a primary Epstein-Barr virus infection. Magnetic resonance imaging (MRI) showed massive bilateral hyperintense striatal and punctiform periventricular lesions. After 2-month treatment with steroids and antiparkinsonian drugs, all features resolved without sequelae. Control MRI demonstrated only minimal residual lesions in both putamina. Strongly resembling the encephalitis lethargica-like syndrome, this case is an unusual presentation of parainfectious acute disseminated encephalitis.
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PMID:Acute reversible parkinsonism in Epstein-Barr virus-related encephalitis lethargica-like illness. 1625 20

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

Both posteroventral pallidotomy and pallidal deep brain stimulation (DBS) have a documented effect on Parkinsonian symptoms. DBS is more costly and more laborious than pallidotomy. The aim of this study was to analyse the respective long-term effect of each surgical procedure on contralateral symptoms in the same patients. Five consecutive patients, two women and three men, who at first surgery had a mean age of 64 years and a mean duration of disease of 18 years, received a pallidotomy contralateral to the more symptomatic side of the body. At a mean of 14 months later, the same patients received a pallidal DBS on the side contralateral to the pallidotomy. All patients had on-off phenomena and dyskinesias. There were three left-sided and two right-sided pallidotomies, and, subsequently, two left-sided and three right-sided pallidal DBS. The latest evaluation was performed 37 months (range 22-60) after the pallidotomy and 22 months (range 12-33) after the pallidal DBS. Mean UPDRS motor score pre-operatively was 49 and at last follow-up 33 (32.7% improvement, p<0.05). Appendicular items 20-26 contralateral to pallidotomy remained improved more significantly than contralateral to DBS. Dyskinesia scores were also improved more markedly contralateral to the pallidotomy. Two patients exhibited moderate dysarthria and one patient severe dysphonia following DBS. Symptoms contralateral to the chronologically older pallidotomy, especially dyskinesias, rigidity and tremor, were still more improved than symptoms contralateral to the more recent pallidal DBS, despite numerous post-operative patient visits to optimise stimulation parameters.
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PMID:Pallidotomy versus pallidal stimulation. 1655 82

A patient with tremor associated with spasmodic dysphonia developed a marked exacerbation of her upper limb tremor whilst on co-careldopa (Sinemet CR) that improved when the drug was withdrawn.
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PMID:Levodopa can worsen tremor associated with dystonia. 1685 31

Spasmodic dysphonia is a focal form of laryngeal dystonia that causes unintended contractions of vocal folds with speech interruptions and affecting the voice quality. There are adductor (82%), abductor (36%), mixed (1%) types and reported by Blitzer--respiratory adductor type with paradoxical vocal fold movement and stidor. As an example of diagnostic and therapeutic difficulties in spasmodic dysphonia and its multidisciplinary approach with needed cooperation of many specialists we presented patient with adductor spasmodic dysphonia. In stroboscopic evaluation there were observed glottic overclosure and hyperaduction in supraglotic stuctures (sphincteric). During diagnostic procedures there was made acoustic analysis by digital spectrograph (by KAY Elmetrics Company). IV degree of hoarsness and voice breaks were observed in sonogram. In multidimentional analysis there were deviations of the frequency, amplitude, noise, tremor and voice break parameters. Treatment included regular speech therapy, relaxation- and psychotherapy. The results of treatment were very instable because every stress-related problem released symptoms. Botulinum toxin injections into thyro-arytenoid muscle which had made before our hospitalisation were also not succeeding. Because of lack of etiologic factor and plenty diseases that can mimic spasmodic dysphonia, close communication between many specialists is needed.
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PMID:[Therapeutic difficulties in spasmodic dysphonia--case report]. 1687 62

If an abscess is not able to establish drainage through the skin surface or into the oral cavity, it may spread diffusely through fascial planes of the neck's soft tissue. Once the infection descends into the submandibular space, it may extend to the lateral pharyngeal space, and then to the retro-pharyngeal space. From here, it may reach the thyroid gland. The authors here describe a case of submandibular phlegmon derived from a periapical abscess of inferior premolar, which has reached the thyroid gland. The damage caused to the gland resulted in the release of a conspicuous quantity of thyroid hormones, thus causing a thyrotoxic pattern: temperature, cutaneous pallor, excessive perspiration, tremor, tiredness, weight loss, increased appetite, and tachycardia. Additionally, the gland's edema caused dysphagia and dysphonia commonly seen with thyroid gland enlargement. After dental drainage and appropriate anti-inflammatory and antibiotic therapy, administration of oral beta-blockers and corticosteroid therapy were performed to counteract thyrotoxicosis in order to prevent recurrences. Finally, a root canal was performed once the thyrotoxicosis had been resolved.
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PMID:Acute thyroiditis of odontogenic origin. 1793 25

This paper proposed a support vector machine (SVM) based classification method to identify diversified pathological voices. Sound signals were sampled from the pronunciation of a vowel "a" vocalized by 214 subjects, including 181 patients suffered from various dysphonias (such as polypoid degeneration, adductor spasmodic dysphonia, vocal fatigue, vocal tremor, vocal fold edema, hyperfunction, and erythema), and 33 healthy subjects. 25 acoustic parameters were calculated from the sampled data for each subject. The original acoustic dataset was first transformed via principal component analysis (PCA) method into a new feature space. To learn the identification boundary for healthy and pathological voices, a soft-margin SVM and three kinds of kernels were examined. The results under different combination of parameters and kernels were investigated. The effectiveness of SVM-based approach seems to be promising in the application of pathological voice identification.
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PMID:SVM-based identification of pathological voices. 1800 22

Botulinum toxin has long been known for its paralytic effects on the human voluntary musculature via inhibition of acetylcholine release at neuromuscular junctions. Its original clinical use for the treatment of strabismus has expanded significantly to include neurological conditions related to muscle hyperactivity and/or spasticity (e.g., dystonia, spasticity, tics, tremor, dysphonia). Recently, botulinum toxin has been shown to impact autonomic disorders by acting at acceptors on glands and smooth muscle, and consequently it has been used in the management of a number of other conditions including hypersecretory disorders, pain syndromes, detrusor sphinchter dyssenergia or overactivity and gastointestinal smooth muscle/sphincter spasm; it may also reduce pain in patients for whom it is used to treat these and other primary conditions. This article will review the pharmacology and formulations of botulinum toxins as well as data from clinical trials demonstrating their efficacy for numerous conditions based on their effects on cholinergic synapses outside the motor nervous system.
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PMID:Current clinical applications of botulinum toxin. 1992 19


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