Search PubMed⌕ Search

Biomedical subjects

B Ford

Publications and source records attributed to B Ford.

At least 55 records · Page 3Linked to original sources
PubMed1998

Reliability between two observers using a protocol for diagnosing essential tremor.

Protocols with demonstrated reliability have been established for the diagnosis of numerous movement disorders. whereas in the essential tremor (ET) literature, there is no discussion about the reliability of diagnostic protocols. Lack of knowledge of the reliability of diagnostic protocols in ET limits the use of these protocols because reliability is an essential requirement for scientific quality in data management. The objective of this study was to determine the reliability of a protocol for diagnosing ET. The protocol consists of a Tremor Interview, a videotaped Tremor Examination, and a diagnostic algorithm. Eighty-three subjects with ET, identified in a community-based health study in Washington Heights-Inwood, New York, were matched with 83 control subjects from the same community. These subjects and their relatives are being recruited to participate in the Washington Heights-Inwood Genetic Study of ET. Two hundred twenty-six subjects have been evaluated to date (35 ET cases, 40 controls, 151 relatives). All 226 underwent an 84-item Tremor Interview and 26-item videotaped Tremor Examination. Diagnoses (normal, possible ET, probable ET, definite ET) were independently assigned by two blinded neurologists specializing in movement disorders. The kappa statistic, k, was used to determine diagnostic agreement between these two neurologists. The concordance rate between two raters using diagnostic categories definite ET, probable ET. possible ET, and normal was 80%; kw = 0.84 (near perfect to perfect agreement). The concordance rate between two raters using two diagnostic categories (definite ET and normal) was 100%; k = 1.00 (perfect agreement). There was high correlation between the two raters' total tremor scores (r = 0.89, p < 0.00001). This diagnostic protocol is highly reliable. Research in ET would greatly benefit from diagnostic protocols with demonstrated reliability.

Adolescent↗
PubMed1998

Clinical characteristics of essential tremor: data from a community-based study.

BACKGROUND: 99.5% of individuals with essential tremor (ET) who live in the community have mild tremor and do not attend clinics. Clinic-based studies of ET have not allowed investigators to characterize the full clinical spectrum of this disorder. In community-based studies of ET, the primary focus has been the prevalence rather than the clinical characteristics of ET. OBJECTIVE: To describe the clinical characteristics of ET as seen in a community-based study. METHODS: 73 subjects with ET, identified in a community-based study of ET in Washington Heights-Inwood, New York, underwent a standardized 84-item physician-administered tremor interview and a 26-item videotaped tremor examination which included 12 bedside tests for ET. Two neurologists who specialized in movement disorders and who demonstrated excellent interrater agreement rated the severity of tremor using a 0 to +3 clinical rating scale and assigned a total tremor score (range, 0-36) and a diagnosis of ET. RESULTS: Diagnoses in the 73 cases were: definite ET (18, 24.7%), probable ET (32, 43.8%), and possible ET (23, 31.5%). The mean total tremor score was 17.8 of 36. Thirty-six of 73 (49.3%) were asymptomatic, answering "no" to the question "do you often have shaking or tremor that you can't control?" Sixty-seven of 73 (91.8%) had not been prescribed medication for tremor. On average, subjects received tremor ratings of > or =+2 on only 5.4 of the 12 bedside tests for ET. Kinetic tremor was rated as more severe than postural tremor in 72 (98.6%) of 73 cases. CONCLUSIONS: We present the clinical findings of a group of largely untreated, unselected cases of ET that would not otherwise have come to neurologic attention. The tremor was mild, often asymptomatic, and not uniformly present throughout the examination. It was rarely treated. The kinetic component of the tremor was more severe than the postural component. These clinical data further our understanding of the clinical spectrum of ET.

Adolescent↗
PubMed1998

Outcome of selective ramisectomy for botulinum toxin resistant torticollis.

OBJECTIVE: To investigate the long term outcome of selective ramisectomy denervation in patients with botulinum toxin resistant spasmodic torticollis. BACKGROUND: The published surgical series of ramisectomy treatment for torticollis do not provide systematic information on patients who develop resistance to the current standard of treatment-botulium toxin injections. Moreover, there is little information on surgical outcome using rating scale measurements of torticollis, or assessments of functional and occupational capacity. METHODS: Using a structured interview format and videotape assessments of severity of dystonia in a retrospective fashion, detailed follow up information was obtained on 16 patients who underwent open label selective denervation for severe, disabling torticollis, refractory to injections of botulinum toxin. RESULTS: Of 16 patients with disabling torticollis followed up postoperatively for a mean of 5 years, six (37.5%) had a moderate or complete return of normal neck function, as determined using functional capacity scales, whereas 10 had only minimal relief of dystonia or gain in function. Six of the 16 patients (37.5%) underwent a second peripheral denervation operation, and one required a third. Of 11 patients working outside the home before surgery, nine were disabled by dystonia, and only one continued to work after surgery. Dystonia rating scale scores of videotaped examinations using a modification of the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS) improved in 12 of 14 patients (85.7%) who underwent selective ramisectomy. When patients with primary botulinum toxin resistance were excluded, the magnitude of benefit for this subgroup was 31.9% of the baseline dystonia score (p<0.0002), comparable with the degree of improvement in a group of control patients receiving botulinum toxin treatment for torticollis. CONCLUSION: About one third of patients with torticollis resistant to injections of botulinum toxin may derive modest long term functional improvement from selective denervation, with a reduction in dystonia by about 30%, but remain unable to work.

Adult↗
PubMed1998

Does a screening questionnaire for essential tremor agree with the physician's examination?

In research studies of essential tremor (ET), monetary and geographic factors often necessitate diagnosis by interview rather than by examination. Few attempts have been made to determine the validity of a screening instrument for ET. A total of 242 subjects (33 definite or probable ET, 54 possible ET, and 155 normal) were part of a community-based family study of ET in northern Manhattan. Subjects underwent a tremor interview and videotaped tremor examination. The interview included 12 screening questions for ET. Two neurologists rated the severity of tremor on videotape and assigned diagnoses. The subjects' responses to the 12 screening questions were compared with the neurologic examination and the neurologists' diagnoses. There was an association between the number of affirmative responses on the 12-item screen and the severity of tremor during the videotaped tremor examination (r = 0.66, p < 0.01). Including those with probable and definite ET and defining a positive screen result as an affirmative response to at least 1 of 12 screening questions, the sensitivity of the screening questionnaire was 73%, the specificity was 96%, and the positive predictive value was 80%. The responses to the questionnaire closely correlated with the severity of tremor on neurologic examination. The questionnaire had moderate validity for subjects with probable or definite ET; subjects with mild ET often screened negative. Hence, in population-based studies and family studies of ET, it is important to examine all subjects for the presence of mild asymptomatic ET.

Adolescent↗
PubMed1998

Post-traumatic shoulder 'dystonia': persistent abnormal postures of the shoulder after minor trauma.

The authors describe two patients with fixed shoulder elevation and prominent regional muscle hypertrophy that developed within days after local minor injury. The condition lacked several typical features of dystonia, such as the presence of torsional movements, task specificity, or relief by antagonistic gestures. These patient reports add to the growing literature indicating that persistent post-traumatic abnormal postures and muscle hypertrophy in different body parts may be a distinct response of the nervous system to injury.

Accidents, Traffic↗
PubMed1998

Pain in Parkinson's disease.

Pain, defined as an unpleasant or distressing sensory experience, has been recognized as feature of Parkinson's disease (PD) since the first descriptions of the disorder. Pain is estimated to occur in approximately 40% of patients with PD, and in a minority of individuals becomes severe enough to overshadow the motor symptoms of the disorder. Recent studies based on patients' descriptions of pain have enabled a classification of painful sensations into 1 or more of 5 categories: musculoskeletal pain, neuritic or radicular pain, dystonia-associated pain, primary or central pain, and akathitic discomfort. The existence of a central pain syndrome, intrinsic to PD, finds support in a collection of case reports, but the precise mechanism is unknown, and a correlation with pathology has not been made. This review describes the clinical features of the pain syndromes in PD, and provides a framework for evaluating, classifying, and treating painful symptoms in PD.

Depression↗
PubMed1997

Prevalence of asymptomatic tremor in relatives of patients with essential tremor.

BACKGROUND: The extent to which essential tremor (ET) clusters within families (ie, the familial aggregation of ET) is not precisely known. In part, this is because studies assign disease status in relatives of patients with ET based solely on interviews without conducting physical examinations. This may lead to underascertainment of affected relatives with mild asymptomatic ET. OBJECTIVE: To determine the prevalence of asymptomatic ET among relatives of patients with ET. METHODS: Interview and examination of 25 patients with ET and 58 of their relatives. The interview included 12 questions that screened the patients for ET. Two neurologists who specialize in movement disorders reviewed the videotaped examinations. Based on standardized criteria, diagnoses included ET (definite, probable, or possible) on normal. RESULTS: Of the 8 relatives who received diagnoses of ET, 5 (62.5%) had asymptomatic ET. Hence, 5 (8.6%) of the 58 relatives (95% confidence interval 1.4%-15.8%) had asymptomatic ET. In those with asymptomatic ET, there was a preponderance of young individuals with mild tremor in the nondominant hand. CONCLUSIONS: The prevalence of asymptomatic ET in relatives of patients with ET was similar to that of symptomatic ET. Family studies that do not perform both an interview and a physical examination will underascertain the number of affected relatives. Therefore, future family studies should evaluate relatives of patients with ET with an interview supplemented by a physical examination.

Aged↗
PubMed1997

Idiopathic torsion dystonia linked to chromosome 8 in two Mennonite families.

The DYT1 locus on chromosome 9q34 is responsible for most childhood limb-onset idiopathic torsion dystonia (ITD). Linkage to DYT1 has been excluded in families with adult-onset, and predominantly cranial-cervical, ITD. We mapped a locus (DYT6) associated with prominent cranial-cervical ITD in two large Mennonite families to chromosome 8. An identical haplotype spanning 40-cM segregates with ITD in these families, suggesting a shared mutation from the recent past.

Adolescent↗
PubMed1997

Spinal myoclonus induced by an intrathecal catheter.

We report a case of spinal myoclonus induced by the tip of an intrathecal catheter in a 35-year-old patient with severe, adult-onset, generalized dystonia of unknown cause, treated for 2 years using intrathecal baclofen. One month after a falling episode, the patient developed focal myoclonus of the right proximal leg whenever she stood up from a seated position. The electrophysiologic recordings were compatible with spinal segmental myoclonus, originating at a focus corresponding to the L2-S2 segments. At this site, the tip of the intrathecal catheter was demonstrated by myelography to be in close proximity to the nerve roots and conus medullaris. The myoclonus resolved promptly once the catheter tip was withdrawn. We review the literature on spinal myoclonus and discuss the possible mechanisms of spinal myoclonus pertaining to the present case. This report represents an unusual complication of intrathecal catheter systems that, if recognized, can lead to prompt therapeutic intervention.

Adult↗
PubMed1997

The Washington Heights-Inwood Genetic Study of Essential Tremor: methodologic issues in essential-tremor research.

Essential tremor (ET) is the most prevalent movement disorder. It is unknown to what extent ET clusters within families, and the role of genetic susceptibility in etiology of ET has not been adequately investigated at the population level. The problem is largely methodological, with few well-designed studies. The Washington Height-Inwood Genetic Study of ET, begun in 1955, is designed to investigate the genetics of ET using a methodology that has not been applied to ET research to date. Part of the design includes a new set of clinical and electrophysiological diagnostic criteria for ET; the present paper describes this novel study design.

Case-Control Studies↗
PubMed1997

Chemoprevention of cancer: review of the literature.

PURPOSE/OBJECTIVES: To explain the concept of cancer chemoprevention, describe chemoprevention trials, and discuss the agents currently under study to lower the incidence of oral premalignancy and head and neck, lung, breast, prostate, and colon cancers. DATA SOURCES: Published journal articles, books, bulletins, monographs, protocols, and professional experience. DATA SYNTHESIS: Chemoprevention is a promising approach to cancer control, and several key agents have been identified that may be able to block carcinogenesis. Phase III chemoprevention trials are being conducted to identify the role of agents (e.g., retinoids, tamoxifen, finasteride, aspirin) in cancer prevention. CONCLUSION: Knowledge of the genetic and hormonal changes that occur during carcinogenesis continues to increase. Agents are being identified that block the development of cancer by arresting carcinogenesis. As more agents are identified and investigated, their importance in chemoprevention is increasing. IMPLICATION FOR NURSING PRACTICE: The goal of nursing care for participants in prevention trials is to promote compliance with the study protocol while supporting optimum quality of life. Through proactive education of participants, family members, and the public and skillful management of side effects, nurses can play an integral part in the success of prevention trials.

Adult↗
PubMed1996

Delayed-onset cerebellar syndrome.

BACKGROUND: Delayed-onset involuntary movements, including dystonia and myoclonus, have been reported after stroke or head trauma. Moreover, there have been reports of delayed-onset isolated intention tremor and, in several of these cases, gait ataxia. OBJECTIVE: To further define the clinical features of a delayed-onset cerebellar syndrome. DESIGN: Subjects with cerebellar tremor and either head trauma or stroke were identified using a computerized database, providing detailed demographic and clinical information of 4002 patients with involuntary movements other than Parkinson's disease seen at our center between 1983 and 1995. Medical records and videotaped neurological examinations were retrospectively reviewed. SETTING: The Center for Parkinson's Disease and Other Movement Disorders at Columbia-Presbyterian Medical Center, New York, NY. PATIENTS: Five patients with delayed-onset cerebellar syndromes. RESULTS: Five patients with stroke or head trauma developed a cerebellar syndrome 3 weeks to 2 years after the initial insult. The syndrome, characterized by intention tremor, ataxic dysarthria, nystagmus, dysmetria, dysdiadochokinesis, and gait ataxia, was progressive in at least one patient. In four patients, lesions were present on neuroimaging in the thalamus or brain stem (especially in the midbrain). CONCLUSIONS: A delayed-onset cerebellar syndrome may follow head trauma or stroke. The syndrome is sometimes progressive and often disabling. The delayed onset implies that the syndrome is not caused by the initial lesion itself but may be caused by development of post-synaptic supersensitivity or secondary reorganization of involved pathways.

Adolescent↗
PubMed1996

Use of intrathecal baclofen in the treatment of patients with dystonia.

BACKGROUND: Continuous infusion of intrathecal (IT) baclofen is a highly effective standard therapy for severe spasticity of spinal origin. By contrast, there is limited clinical experience regarding the use of IT baclofen in treating patients with dystonia, and little is known regarding the indications for treatment, efficacy, and safety of IT baclofen in this disorder. OBJECTIVE: To study retrospectively the effects of IT baclofen in treating 25 patients with severe segmental or generalized dystonia. SETTING: Neurological Institute, Columbia-Presbyterian Medical Center, New York, NY. PATIENTS: Twenty-five patients with severe segmental or generalized dystonia that was refractory to oral medications underwent IT baclofen test dosing. In addition to dystonia, 17 patients had spasticity or painful spasms. Thirteen of 25 patients responded to the test doses of IT baclofen, according to unblinded neurological assessments that included the patient's subjective report; all 13 underwent implantation of a pump for continuous IT baclofen infusion. RESULTS: In contrast to reports of patients with spasticity of spinal origin, those with dystonia in the present series had a lower response rate to bolus IT baclofen doses and a smaller degree of clinical improvement. For 10 of the 13 responders to the test doses of IT baclofen, dystonia rating scale scores of videotaped examinations by blinded observers detected no significant change (P < .07) in severity of dystonia. Retrospective data from 11 of 13 patients with implantable pumps, followed up for a mean interval of 21 months after pump insertion, showed continuing efficacy in 6 individuals (55%), based on a determination of patient satisfaction; however, only 3 patients (27%) reported a sustained improvement in functional capacity. Five (38%) of the 13 patients with implantable pumps experienced severe complications that required hospitalization. CONCLUSIONS: Despite recent reports that have described the benefit in small numbers of patients with dystonia, we concluded that the role of IT baclofen in treating severe dystonia remains uncertain. Intrathecal baclofen may be more effective when dystonia is associated with spasticity or pain. In the present series, we detected no significant difference in the response to IT baclofen in patients with or without spasticity or pain, perhaps owing to the small sample size.

Baclofen↗
PubMed1996

Exclusion of the DYT1 locus in familial torticollis.

Clinical-genetic studies of idiopathic torsion dystonia (ITD) indicate that the DYT1 gene on chromosome 9q34 is responsible for most childhood limb-onset disease. The genetic basis of adult-onset ITD is less well studied. In most multiplex adult-onset ITD families, dystonia is limited to the cervical, cranial, or brachial muscles; in a few rare families, dystonia also involves the legs and trunk. Previous linkage studies have excluded the DYT1 locus in these atypical families. We studied two large non-Jewish families with adult-onset ITD limited to the cervical and brachial muscles and excluded the DYT1-containing region. This study further restricts the role of DYT1 to childhood limb-onset ITD and suggests that other genes are responsible for focal adult-onset ITD.

Adolescent↗
PubMed1996

Oral and genital pain syndromes in Parkinson's disease.

Painful sensory complaints are known to occur in Parkinson's disease, but painful oral or genital syndromes have not been described. We report seven individuals with presumed idiopathic Parkinson's disease and one with atypical parkinsonism who experienced chronic severe oral or genital pains that appeared to be examples of a primary sensory disturbance related to the underlying parkinsonism. In each case, the pain syndrome overshadowed the other features of the parkinsonism. Five patients experienced oral pain and three patients, all women, had genital pain. No other definable organic cause was detected to explain the symptoms in any case. The genital pain tended to fluctuate in severity with the motor manifestations of the parkinsonism and could be abolished or reduced by levodopa. The recognition of painful oral or genital sensations in patients with parkinsonism should lead to further study regarding the prevalence, neurochemical basis, and treatment for these disabling symptoms.

Aged↗