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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 199 records · Page 11Linked to original sources
PubMed1995

International Classification of Diseases, tenth revision: neurological adaptation (ICD-10 NA): extrapyramidal and movement disorders.

A classification of diseases is important in epidemiologic studies, indexing of clinical disorders, communication, and education. Since the 10th revision of the International Classification of Diseases (ICD-10) in 1992, a specialty-based neurological adaptation (ICD-10NA) has been developed to reflect the specific needs of individual subspecialties. Although the revision was constrained by the need to maintain compatibility with previous classifications, the new ICD-10NA of Extrapyramidal and Movement Disorders aims to provide an updated and comprehensive categorization of movement disorders.

Basal Ganglia Diseases↗
PubMed1995

Emotional and functional impact of DNA testing on patients with symptoms of Huntington's disease.

The potential impact of DNA testing on asymptomatic subjects at risk for Huntington's disease (HD) has been addressed by numerous studies, but the effect of revealing the genetic results to patients with a clinically established diagnosis of HD has not been previously evaluated. We studied 36 patients, with equal distribution of men and women, mean age 53.9 (SD 12.3) years (range 25-76) and mean duration of symptoms of 11.2 (SD 7.7) years (range 2-33), whose clinical diagnosis of HD was confirmed by expanded CAG repeats (> 40). Coping strategies and depression levels were assessed before the results of DNA testing were imparted. The assessments were repeated two weeks and three months after the results were explained to the patients and their relatives and were compared to the baseline assessments. This group of HD patients was compared with 10 patients who had similar symptoms but the diagnosis of HD was excluded by normal CAG repeats (< 30). Although some patients with HD expressed a subjective reaction to the positive result (four were "surprised", one was "frustrated", and one "devastated"), there were no differences in any psychological scores including Beck Depression Inventory, functional capacity, symptom interference, independence scale, and other measures of mood and behaviour two weeks and three months later. Similarly, no change was noted in any of these measures in the non-HD group. These results suggest that mood and coping strategies are unaffected by DNA confirmation of diagnosis in symptomatic patients with HD.

Adaptation, Psychological↗
PubMed1995

Clozapine for acute and maintenance treatment of psychosis in Parkinson's disease.

In a prospective, open-label study, 12 patients manifesting psychosis associated with Parkinson's disease were treated with clozapine. Cognitive functioning and type of psychotic symptoms were measured prior to treatment, and changes in psychiatric and behavioral symptoms were studied by using the Behave-AD Scale. Significant resolution in psychotic symptoms was found and improvement in global behavioral status observed in all cases, with 10 patients maintaining improvement at follow-up. Careful initiation and titration of the drug resulted in few side effects, and dementia was not found to be a contraindication to such treatment.

Aged↗
PubMed1995

Tremor and longevity in relatives of patients with Parkinson's disease, essential tremor, and control subjects.

To study the relation between essential tremor (ET) and Parkinson's disease (PD), we compared the frequency of familial tremor in relatives of patients with PD (N = 391), ET (N = 140), and the combination of ET and PD (N = 125) with the frequency in patients with progressive supranuclear palsy (PSP) (N = 99) and normal age-matched controls (N = 104). Tremor was present in 96 (5.1%) of 1,874 parents and siblings of patients with PD, 152 of 650 (23.4%) relatives of patients with ET, 91 (20.7%) of 439 relatives of patients with ET-PD, 12 of 462 (2.6%) relatives of patients with PSP, and 10 of 448 (2.2%) relatives of normal controls. The high frequency of familial tremor among relatives of patients with PD, and especially those with the ET-PD combination, compared with relatives of patients with PSP or of normal controls suggests that there is an association of PD and familial tremor. Since the most common form of familial tremor is ET, our study provides support for the notion that ET and PD are pathogenetically related. We also found that parents with tremor lived on the average 9.2 years longer than those without tremor. The association of familial tremor with significantly increased longevity suggests that familial tremor confers some anti-aging influence. Alternatively, tremor may be a simple byproduct of the aging process.

Adult↗
PubMed1995

Response and immunoresistance to botulinum toxin injections.

Botulinum toxin antibodies (ABS) may be a reason why occasionally patients do not have a response to injections with botulinum toxin type A (BTX). We tested 86 patients with cervical or oromandibular dystonia for the presence of BTX ABS; 20 were positive and 66 were negative. All patients who tested positive had no response to BTX injections on at least two consecutive treatment sessions. When compared with 22 randomly selected patients with negative BTX ABS results, the patients with positive BTX ABS tests had an earlier age at onset (mean age: 31.8 +/- 16.7 years versus 43.4 +/- 10.5; p < 0.05), higher mean dose per visit (249.2 +/- 32.5 U versus 180.8 +/- 68.7, p < 0.0005), and higher total cumulative dose (mean dose: 1,709 +/- 638 U versus 1,066 +/- 938; p < 0.01). Four out of five patients with positive ABS tests later had a response to botulinum toxin type F injections. Of 26 patients with negative BTX ABS results who were tested because of poor response on at least one visit, 21 had good response after subsequent injection and five had no effect. Except for young age at onset and higher dosages, there were no other factors that could reliably predict which patients would become immunoresistant to BTX type A injections. Treatment with alternate serotypes may offer clinical benefit to this group of patients. Absence of detectable BTX ABS may occur in patients with poor response to BTX injections because of inadequate dosage, injections of inappropriate muscles, or poor sensitivity of the BTX ABS bioassay.

Adolescent↗
PubMed1995

Outcome after stereotactic thalamotomy for dystonia and hemiballismus.

The outcome after single or staged stereotactic thalamotomies in 17 patients with dystonia and 2 patients with hemiballismus is reviewed. All patients were severely disabled by their movement disorders despite optimal pharmacological therapies. Eight of the patients with dystonia (47%) showed moderate improvement immediately after the procedures. Six of these eight patients maintained their improvement, and two other patients with dystonia improved significantly, during the follow-up period (mean, 37.6 mo). The long-term outcome was better in patients with secondary dystonia (50% moderately or markedly improved at a mean of 41.0 mo) than in patients with primary dystonia (43% moderately or markedly improved at a mean of 32.9 mo). Excellent control was achieved in both of the patients who underwent thalamotomies for hemiballismus.

Adolescent↗
PubMed1995

Outcome after stereotactic thalamotomy for parkinsonian, essential, and other types of tremor.

A better understanding of the mechanisms underlying movement disorders, coupled with refinements in surgical technique, has led to a resurgence of interest in the surgical treatment of patients with tremor. We retrospectively analyzed the outcomes of 60 patients (62 patient sides) with medically intractable tremor who underwent stereotactic thalamotomy. Of these 60 patients, 42 had Parkinson's disease (of whom 2 patients underwent bilateral surgery for a total of 44 patient sides), 6 had essential tremor, 6 had cerebellar tremor, and 6 had post-traumatic tremor. The patients received follow-up for as long as 13 years (mean, 53.4 mo) after their operations. At the most recent follow-up visit, 86% of the patients with Parkinson's disease, 83% of the patients with essential tremor, 67% of the patients with cerebellar tremor, and 50% of the patients with post-traumatic tremor had cessation of or moderate-to-marked improvement in their contralateral tremor, with a concomitant improvement in function. The mean daily dose of levodopa for those patients preoperatively taking levodopa (n = 35) was reduced by approximately 156 mg at a mean of 53.4 months after thalamotomy. Immediate postoperative complications were common, occurring in 58% of patients. The most common complications were contralateral weakness (34%), dysarthria (29%), and confusion (23%). These complications generally resolved rapidly during the postoperative period.

Adult↗
PubMed1994

A comparison of postspace-flight orthostatic intolerance to vasovagal syncope and autonomic failure and the potential use of the alpha agonist midodrine for these conditions.

After space-flights of less than ten days, orthostatic hypotension upon reentry is characterized by plasma volume depletion that may lead to activation of the Bezold-Jarisch reflex which is also considered to be the mechanism of vasovagal (neurocardiogenic) syncope. For space-flight of longer duration, loss of cardiovascular reflex control may take precedence over volume depletion and thus may have similarities to the orthostatic hypotension seen in patients with autonomic failure secondary to basal ganglial disease and peripheral neuropathies. Midodrine is an alpha-one agonist that produces arterial and venous constriction and leads to a decrease in heart rate by baroreceptor reflexes. The efficacy of Midodrine in successfully treating orthostatic hypotension secondary to autonomic failure has been shown in clinical trials. Midodrine's ability to vasoconstrict without increasing heart rate suggests that it might be a useful treatment for vasovagal syncope since stimulation of the Bezold-Jarisch reflex would be less likely. For post-space flight orthostatic hypotension, midodrine may be a useful adjunctive treatment to the measures currently being used.

Adult↗
PubMed1994

A multicenter double-blind placebo-controlled trial of pergolide as an adjunct to Sinemet in Parkinson's disease.

Three hundred and seventy-six subjects with advanced Parkinson's disease participated in a prospective, double-blind placebo-controlled study of the dopamine agonist pergolide mesylate as an adjunct to Sinemet. At 6 months, patients randomized to pergolide had a statistically significant improvement in total Parkinson's score, scores of activities of daily living, motor function, number of "off" hours, Hoehn and Yahr stage, and numerous parameters of parkinsonian function including bradykinesia, rigidity, gait, and dexterity. This benefit was obtained with the addition of a mean dose of 2.94 mg of pergolide, which permitted a 24.7% reduction in dose of levodopa. Adverse reactions were, for the most part, mild, reversible, and not of major clinical significance. No significant cardiac or electrocardiographic abnormalities were detected. This study demonstrates that pergolide mesylate, as an adjunct to levodopa, is an effective antiparkinsonian agent that provides clinical improvement while permitting a reduction in levodopa dose.

Activities of Daily Living↗
PubMed1994

Botulinum toxin in the treatment of dystonic tics.

Botulinum toxin (BTX) injections provide effective treatment for a variety of disorders manifested by inappropriate muscle contractions, but its efficacy in the treatment of tics has not been previously studied. Ten male patients 13-53 years of age who were diagnosed with Tourette's syndrome manifested by disabling focal tics were included in this pilot study. Five patients had frequent blinking and blepharospasm, rendering them "blind," and five patients had severe and painful dystonic tics involving their neck muscles. All 10 patients experienced moderate to marked improvement in the intensity and frequency of tics after BTX injections into the involved muscles. Patients in whom premonitory urges preceded their tics noted marked lessening of these sensory symptoms. The benefit lasted 2-20 weeks after injections. There were no serious complications, except for transient ptosis in two and neck pain, stiffness, or weakness in three patients. BTX injections appear to be safe and effective treatment for patients with focal dystonic tics. The treatment ameliorates not only involuntary movements but also the premonitory sensory component associated with some tics.

Adolescent↗
PubMed1994

Vascular progressive supranuclear palsy.

BACKGROUND: Progressive supranuclear palsy (PSP) is a neurologic syndrome of unknown cause. This idiopathic type of PSP is usually associated with characteristic clinical and pathological features. OBJECTIVE: To assess evidence of cerebrovascular disease in a population of patients with clinically defined PSP, and to compare clinical and neuroimaging features in vascular versus idiopathic PSP. DESIGN AND METHODS: Using predetermined criteria, the records of 128 patients diagnosed with PSP were reviewed for evidence of vascular disease. RESULTS: Thirty patients (23.3%) satisfied criteria for vascular PSP. The vascular group differed from the idiopathic group by asymmetric and predominantly lower body involvement (p < 0.05). Corticospinal signs, pseudobulbar signs, gait difficulties, dementia, and incontinence of bowels and bladder were also more common in the vascular group, but these differences did not reach statistical significance. CONCLUSION: PSP is a syndrome which can be caused by cerebro-vascular disease. In addition to an increased frequency of stroke risk factors and neuroimaging evidence of vascular disease, vascular PSP can be differentiated from idiopathic PSP by a higher degree of asymmetry, lower body involvement, and evidence of corticospinal and pseudobulbar signs.

Aged↗
PubMed1994

Muscle mitochondrial ATP production in progressive supranuclear palsy.

Six patients with progressive supranuclear palsy (PSP) and 12 age-matched disease-free subjects participated in this study designed to compare rates of ATP production by intact mitochondria from biopsied skeletal muscle. When pyruvate and malate were used as metabolic substrates, rates of ATP production were 0.184 +/- 0.025 mumol/min/U of citrate synthase (CS) activity (a mitochondrial marker) in control subjects and 0.131 +/- 0.051 mumol/min/U of CS in PSP patients. In the presence of succinate, rates of ATP formation were 0.137 +/- 0.02 mumol/min/U of CS in controls and 0.109 +/- 0.04 mumol/min/U of CS in patients. With N,N,N',N'-tetramethyl-p-phenylenediamine (TMPD) and ascorbate, rates were 0.034 +/- 0.008 mumol/min/U of CS in controls and 0.022 +/- 0.01 mumol/min/U of CS in PSP subjects. Differences between the control and PSP populations reached statistical significance with pyruvate/malate and TMPD/ascorbate. No differences in either muscle histopathology or histochemistry were found between patient and control subjects. Results of this study suggest that oxidative phosphorylation defects occur in muscle mitochondria from patients with PSP.

Adenosine Triphosphate↗
PubMed1994

Botulinum toxin in movement disorders.

The most potent biologic toxin, botulinum toxin (BTX), has become a powerful therapeutic tool in the treatment of a variety of neurologic, ophthalmic, and other disorders manifested by abnormal, excessive, or inappropriate muscle contractions. This review focuses on the use of BTX in the treatment of dystonia and other movement disorders. The therapeutic application of BTX, however, extends beyond movement disorders; chemodenervation with BTX has been found to ameliorate spasticity, rigidity, spastic bladder, achalasia, and even some cosmetic conditions. In addition to describing its therapeutic effects, this article also reviews recent advances in the understanding of the molecular and cellular mechanisms of BTX. Few therapeutic agents have been better understood in terms of their mechanism of action or have had greater impact on patients' functioning than BTX. BTX-A has been used in nearly all clinical trials. Blocking anti-BTX-A antibodies have been detected in about 5% of patients chronically treated with this type of BTX. Patients who develop immunoresistance to BTX-A may benefit from other serotypes of BTX, such as BTX-B and -F, currently undergoing clinical trials.

Botulinum Toxins↗