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Biomedical subjects

J Jankovic

Publications and source records attributed to J Jankovic.

At least 145 records · Page 8Linked to original sources
PubMed1998

Peripherally induced oromandibular dystonia.

OBJECTIVES: Oromandibular dystonia (OMD) is a focal dystonia manifested by involuntary muscle contractions producing repetitive, patterned mouth, jaw, and tongue movements. Dystonia is usually idiopathic (primary), but in some cases it follows peripheral injury. Peripherally induced cervical and limb dystonia is well recognised, and the aim of this study was to characterise peripherally induced OMD. METHODS: The following inclusion criteria were used for peripherally induced OMD: (1) the onset of the dystonia was within a few days or months (up to 1 year) after the injury; (2) the trauma was well documented by the patient's history or a review of their medical and dental records; and (3) the onset of dystonia was anatomically related to the site of injury (facial and oral). RESULTS: Twenty seven patients were identified in the database with OMD, temporally and anatomically related to prior injury or surgery. No additional precipitant other than trauma could be detected. None of the patients had any litigation pending. The mean age at onset was 50.11 (SD 14.15) (range 23-74) years and there was a 2:1 female preponderance. Mean latency between the initial trauma and the onset of OMD was 65 days (range 1 day-1 year). Ten (37%) patients had some evidence of predisposing factors such as family history of movement disorders, prior exposure to neuroleptic drugs, and associated dystonia affecting other regions or essential tremor. When compared with 21 patients with primary OMD, there was no difference for age at onset, female preponderance, and phenomenology. The frequency of dystonic writer's cramp, spasmodic dysphonia, bruxism, essential tremor, and family history of movement disorder, however, was lower in the post-traumatic group (p<0.05). In both groups the response to botulinum toxin treatment was superior to medical therapy (p<0.005). Surgical intervention for temporomandibular disorders was more frequent in the post-traumatic group and was associated with worsening of dystonia. CONCLUSION: The study indicates that oromandibular-facial trauma, including dental procedures, may precipitate the onset of OMD, especially in predisposed people. Prompt recognition and treatment may prevent further complications.

Adolescent↗
PubMed1998

Assessment of motor function after stereotactic pallidotomy.

Despite a paucity of controlled data, stereotactic pallidotomy is increasingly used for the treatment of advanced Parkinson's disease (PD). To study the efficacy of the procedure on the cardinal PD features of rigidity, tremor, bradykinesia, and postural instability, we blindly rated randomized videos of 34 patients recorded in the "off' state immediately before and 3 months after unilateral stereotactic lesioning of the globus pallidus internus. Total "off' time Unified Parkinson's Disease Rating Scale motor scores improved 13.6% from 28.9 +/- 7.5 to 25.0 +/- 7.0 (p < 0.001). Particularly robust improvement was seen in contralateral tremor, gait, and arising from a chair (p < 0.001). Significant improvement was also seen in ipsilateral tremor, contralateral and some ipsilateral dexterity measures, and body bradykinesia. Most other features tended toward improvement but did not reach statistical significance. We conclude that pallidotomy is a safe and effective treatment of parkinsonian symptoms, many of which improve bilaterally.

Adult↗
PubMed1998

Mouse bioassay versus Western blot assay for botulinum toxin antibodies: correlation with clinical response.

OBJECTIVE: To compare the mouse protection bioassay (MPB) to the Western blot assay (WBA) in detecting antibodies against botulinum toxin A (BTX-A) and to correlate the assay results with clinical responses to BTX-A injections. METHODS: MPB and WBA assay results were compared in 51 patients (34 nonresponders and 17 responders) who received BTX-A injections, most commonly for cervical dystonia. A subset of patients received a "test" injection into either the right eyebrow (14) or right frontalis (12). RESULTS: Twelve patients with antibodies against BTX-A (Ab+) detected by WBA did not demonstrate antibodies (Ab-) by MPB. Conversely, five patients were Ab+ by MPB but Ab- by WBA. Specificity of the MPB was 100% on all three parameters (clinical, eyebrow, and frontalis injections), whereas WBA specificity was only 71% for clinical response but 100% for both eyebrow and frontalis responses. Sensitivities for both assays were low (33 to 53%). Of the 16 patients previously Ab+ by MPB, seven became negative on retesting after a mean interval of 33 months (range, 6 to 93 months). CONCLUSIONS: The lower specificity of the WBA compared to the MPB suggests that the WBA detects nonblocking antibodies. Eyebrow and frontalis "test" injections correlated well with MPB and WBA results and with clinical responses and may be useful in the evaluation of BTX nonresponders.

Adult↗
PubMed1998

Unilateral thalamic deep brain stimulation for refractory essential tremor and Parkinson's disease tremor.

OBJECTIVE: To determine the efficacy and tolerability of unilateral thalamic deep brain stimulation (DBS) for patients with medically refractory essential tremor (ET) and the tremor associated with Parkinson's disease (PD). BACKGROUND: The tremor of ET and PD may produce functional disability despite optimal medical therapy. Several reports have demonstrated efficacy of thalamic DBS in this scenario. METHODS: Preoperative and 3-month postoperative tremor ratings were compared in 33 patients (14 ET and 19 PD) with severe tremor. Evaluations included Unified Parkinson's Disease Rating Scale (UPDRS) scores for PD patients and a modified Unified Tremor Rating Scale in ET patients. Open-label and blinded data (unknown activation status) were obtained. RESULTS: ET patients demonstrated an 83% reduction (p < 0.0001) in observed contralateral arm tremor. All measures of tremor including writing samples, pouring tests, subjective functional surveys, and disability scores improved significantly. PD patients demonstrated an 82% reduction (p < 0.0001) in contralateral tremor and significant improvement in disability and global impressions. There was, however, no meaningful improvement in other motor aspects of the disease, and the total UPDRS part II (activities of daily living) score did not change. Adverse events, more common in ET patients, were generally mild and were usually eliminated by adjustment of the device parameters. CONCLUSIONS: Thalamic DBS is a safe and effective treatment of ET and the tremor of PD. In PD, its use should be limited to patients in whom high-amplitude tremor results directly in significant functional disability.

Aged↗
PubMed1998

Posteroventral medial pallidotomy for treatment of Parkinson's disease: preoperative magnetic resonance imaging features and clinical outcome.

OBJECT: The goal of this study was to investigate the impact of mild or moderate degrees of degenerative or ischemic encephalopathy on predicting clinical outcome following unilateral posteroventral medial pallidotomy for treatment of advanced Parkinson's disease (PD). METHODS: Thirty-five patients with PD were studied prospectively. The presence and degree of cortical atrophy, ventriculomegaly, deep white matter lesions (DWML), periventricular lucencies (PVL), and the presence of lacunes and status cribriformis (multiple and bilateral enlarged Virchow-Robin spaces) were determined by magnetic resonance (MR) imaging before the patients underwent stereotactic pallidotomy performed according to a standard protocol. Clinical outcome was measured using a standard battery of tests including application of the Unified Parkinson's Disease Rating Scale (UPDRS). The preoperative MR imaging features were correlated with UPDRS subscores such as motor "off' score, the activities of daily living (ADL) off score, the off subscore for bradykinesia, the percentage of "on" time dyskinesias, and a global outcome rating. The MR findings were also correlated with the occurrence of side effects. Global outcome was rated as markedly improved in 22 patients (63%) and as moderately improved in 12 patients (34%) 6 months postoperatively. At the 1-year follow-up examination, global outcome in 31 patients was rated as markedly improved in 14 patients (45%), as moderately improved in another 14 (45%), as slightly improved in two (6%), and as worse in one patient (3%). The mean UPDRS motor off score changed from 58.7 preoperatively to 33.2 at 6 months and 33.4 at 1 year (p < 0.0001), the ADL off score from 31.8 to 18.2 at 6 months and 18.6 at 1 year (p < 0.0001), the off score from contralateral bradykinesia from 11.6 to 5.6 at 6 months and 4.1 at 1 year (p < 0.0001), and the percentage of awake time with dyskinesias from 37.4 to 17.4% at 6 months and 21.1% at 1 year (p < 0.0001). The presence of mild or moderate degrees of cortical atrophy, PVL, and DWML had no effect on clinical outcome. Patients with status cribriformis and those with lacunes tended to show comparatively less improvement in the UPDRS ADL off score (p = 0.014 and p = 0.016, respectively) at 6 months. This tendency was also present in patients with status cribriformis 1 year postoperatively (p = 0.046). Patients with both status cribriformis and lacunes had a higher incidence of transient altered mental status immediately postoperatively (p = 0.05). CONCLUSIONS: Mild-to-moderate degrees of cortical atrophy, ventriculomegaly, and ischemic encephalopathy do not predispose patients to less favorable outcomes following unilateral pallidotomy. Patients with both status cribriformis and lacunes have a higher risk of transient side effects; however, with regard to clinical outcome, these patients should not be denied surgical treatment.

Activities of Daily Living↗
PubMed1998

Post-traumatic movement disorders: effect of the legal system on outcome.

Since patients with post-traumatic, neurologic, movement disorders often seek legal counsel and become involved in litigation, we used this group of disorders as a model for testing the hypothesis that an interaction with the legal system may influence its outcome. We reviewed 100 consecutive medical records of patients with post-traumatic movement disorders from the Movement Disorders Clinic at the Baylor College of Medicine. Additionally, 40 patients completed a detailed questionnaire and a health survey. Nineteen of the 40 respondents had obtained legal representation in regards to compensation for their medical problems. The group of patients with attorneys differed from those without legal representation in that they were significantly younger and a significantly higher percentage of these patients were disabled as compared to the group without attorneys. While most were dissatisfied with the legal system, 68% were satisfied with services provided by their attorneys. Although the patients with legal aspects of their movement disorders seemed to have more severe and persistent disability, we could not definitely conclude that the legal system had adversely affected the outcome of the post-traumatic movement disorders.

Adolescent↗
PubMed1997

[Tics and Tourette's syndrome].

This article is reviewing current knowledge of Tourette's syndrome (TS) including its clinical picture, pathophysiology, genetics and treatment. The semiology of TS is characterized with various types of motor and vocal tics and associated pathological behaviors as attention deficit hyperactivity disorder and obsessive-compulsive disorder. Recent pathophysiological and genetic data confirm organic character of the condition. Treatment strategies are proposed with regard to prevailing type of symptoms.

Diagnosis, Differential↗
PubMed1997

(-)-Deprenyl protection of 1-methyl-4 phenylpyridium ion (MPP+)-induced apoptosis independent of MAO-B inhibition.

Selective loss of central dopaminergic neurons in vitro and in vivo can be initiated by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) through its metabolite phenylpyridium ion (MPP+). Such MPTP-mediated cytotoxicity can be blocked by (-)-Deprenyl, a monoamine oxidase (MAO)-B inhibitor, but the exact mechanisms of MPP(+)-induced cytotoxicity and (-)-Deprenyl's protection against such neurotoxicity are not fully understood. Using a hybrid clone MES 23.5, a dopaminergic cell line that does not contain MAO-B, we document that MPP+ induces apoptotic cell death. Application of (-)-Deprenyl at concentrations of 0.1-10 microM significantly reduces MPP(+)-induced apoptosis in MES 23.5 cells; (-)-Deprenyl at higher concentrations (> 100 microM) that completely inhibit MAO-B activity, however, induces apoptosis. Pretreatment with N-(2-aminoethyl)-p-chlorobenzamide (Ro 16-6491), a selective MAO-B inhibitor, does not protect MES 23.5 cells against MPP(+)-induced cell death. These results suggest that the protective action of (-)-Deprenyl against MPP+ neuro-toxicity in dopaminergic cell line may be independent of the inhibition of MAO-B.

1-Methyl-4-phenylpyridinium↗
PubMed1997

Decreased [18F]spiperone binding in putamen in idiopathic focal dystonia.

In this study we have investigated the pathophysiology of two idiopathic focal dystonias: hand cramp with excessive cocontractions of agonist and antagonist hand or forearm muscles during specific tasks, such as writing, and facial dystonia manifested by involuntary eyelid spasms (blepharospasm) and lower facial and jaw spasms (oromandibular dystonia). We used positron emission tomography (PET) to measure the in vivo binding of the dopaminergic radioligand [18F]spiperone in putamen in 21 patients with these two focal dystonias and compared the findings with those from 13 normals. We measured regional cerebral blood flow and blood volume in each subject as well as the radiolabeled metabolites of [18F]spiperone in arterial blood. A stereotactic method of localization, independent of the appearance of the images, was used to identify the putamen in all of the PET images. We analyzed the PET and arterial blood data with a validated nonsteady-state tracer kinetic model representing the in vivo behavior of the radioligand. An index of binding called the combined forward rate constant was decreased by 29% in dystonics, as compared with normals (p < 0.05). There were no significant differences between dystonics and normals in regional blood flow, blood volume, nonspecific binding, permeability-surface area product of [18F]spiperone or the dissociation rate constant. These findings are consistent with a decrease of dopamine D2-like binding in putamen and are the first demonstration of a receptor abnormality in idiopathic dystonia. These results have important implications for the pathophysiology of dystonia as well as for function of the basal ganglia.

Adult↗
PubMed1997

Familial essential tremor in 4 kindreds. Prospects for genetic mapping.

OBJECTIVES: To describe 4 large families with essential tremor (ET) to draw attention to the marked clinical heterogeneity of ET. To use computer simulation analysis to provide information about the power of the family material for future linkage studies. SUBJECTS: We examined a total of 251 members from 4 kindreds with ET. The mean (+/-SD) age at onset of ET varied among the 4 kindreds between 19.0 +/- 11.4 years and 45.6 +/- 7.4 years. Three of the kindreds had a total of 41 members with the combination of ET and dystonia, typically manifested as torticollis or dystonic writers' cramp. In 1 of the kindreds, ET seemed to be associated with malignant hyperthermia. One kindred represented "pure" ET without any associated disorders. METHODS: In addition to detailed clinical assessments, we conducted computer simulations on the families' pedigrees using a model that presumed an autosomal dominant inheritance pattern with high penetrance. RESULTS: Although there was evidence of clinical heterogeneity between the families, the duration of symptoms directly correlated with the severity of disease. The computer simulations indicated that 3 of the 4 pedigrees had enough power to generate a significant linkage result in a total genome search with highly polymorphic markers. CONCLUSIONS: This study confirms the frequent coexistence of ET and dystonia in individual families. Computer simulations can be used to determine the power of the family to detect a linked marker. Identification of the defective gene(s) will enable a better understanding and classification of these common movement disorders.

Aged↗
PubMed1997

What is the accuracy of the clinical diagnosis of multiple system atrophy? A clinicopathologic study.

BACKGROUND: The presentation of symptoms for multiple system atrophy (MSA) varies. Because there are no specific markers for its clinical diagnosis, the diagnosis rests on the results of the neuropathologic examination. Despite several clinicopathologic studies, the diagnostic accuracy for MSA is unknown. OBJECTIVES: To determine the accuracy for the clinical diagnosis of MSA and to identify, as early as possible, those features that would best predict MSA. DESIGN: One hundred five autopsy-confirmed cases of MSA and related disorders (MSA [n=16], non-MSA [n=89]) were presented as clinical vignettes to 6 neurologists (raters) who were unaware of the study design. Raters identified the main clinical features and provided a diagnosis based on descriptions of the patients' first and last clinic visits. METHODS: Interrater reliability was evaluated with the use of kappa statistics. Raters' diagnoses and those of the primary neurologists (who followed up the patients) were compared with the autopsy-confirmed diagnoses to estimate the sensitivity and positive predictive values at the patients' first and last visits. Logistic regression analysis was used to determine the best predictors to diagnose MSA. RESULTS: For the first visit (median, 42 months after the onset of symptoms), the raters' sensitivity (median, 56%; range, 50%-69%) and positive predictive values (median, 76%; range, 61%-91%) for the clinical diagnosis of MSA were not optimal. For the last visit (74 months after the onset of symptoms), the raters' sensitivity (median, 69%; range, 56%-94%) and positive predictive values (median, 80%; range, 77%-92%) improved. Primary neurologists correctly identified 25% and 50% of the patients with MSA at the first and last visits, respectively. False-negative and -positive misdiagnoses frequently occurred in patients with Parkinson disease and progressive supranuclear palsy. Early severe autonomic failure, absence of cognitive impairment, early cerebellar symptoms, and early gait disturbances were identified as the best predictive features to diagnose MSA. CONCLUSIONS: The low sensitivity for the clinical diagnosis of MSA, particularly among neurologists who followed up these patients in the tertiary centers, suggests that this disorder is underdiagnosed. The misdiagnosis of MSA is usually due to its confusion with Parkinson disease or progressive supranuclear palsy, thus compromising the research on all 3 disorders.

Aged↗
PubMed1997

Posteroventral medial pallidotomy in levodopa-unresponsive parkinsonism.

BACKGROUND: Parkinsonism in a 42-year-old patient, which was presumably related to peripheral trauma, did not respond to levodopa therapy. OBSERVATION: We treated the patient with microelectrode-guided unilateral posteroventral medial pallidotomy and followed up with magnetic resonance imaging and prospective clinical evaluation. Pallidotomy resulted in marked improvement of right-sided parkinsonian symptoms and functional disability at 4.5 months after surgery. Microelectrode recording during pallidotomy revealed discharge patterns that were similar to those seen in patients with Parkinson disease. Postoperative magnetic resonance imaging confirmed the location of the lesion in the posteroventral medial pallidum. CONCLUSIONS: Posteroventral pallidotomy usually has limited benefit in patients with degenerative atypical parkinsonism who do not respond to levodopa therapy. Nevertheless, pallidotomy can be an effective treatment for other levodopa-unresponsive parkinsonian disorders.

Adult↗
PubMed1997

Cervical dystonia associated with tumors of the posterior fossa.

Cervical dystonia was associated with posterior fossa tumors in three patients. The onset of dystonia paralleled the appearance of other focal neurologic signs. All patients had extraaxial tumors located in the cerebellopontine angle that were removed via suboccipital approaches. The tumors were identified as schwannomas arising from the glossopharyngeal nerve and from the vagus/accessory nerves; and a meningioma. Postoperatively, the cervical dystonia improved markedly during a period of 8 years in one patient, and it remitted completely within 1 year in another patient. In the third patient, cervical dystonia persisted. The combination of the clinical findings and the temporal relationship of their appearance suggest a causal association between the posterior fossa tumors and cervical dystonia in three cases. Possible pathogenic mechanisms are reviewed.

Adolescent↗
PubMed1997

Tics secondary to craniocerebral trauma.

We describe three adult patients who presented with multifocal motor and vocal tics secondary to craniocerebral trauma. In one case, the tics were accompanied by marked obsessive-compulsive behavior. All patients were involved in motor vehicle accidents resulting in closed craniocerebral trauma. The latency of onset between head trauma and the movement disorder varied between 1 day and a few months. Magnetic resonance imaging, which was performed in all three patients, did not detect any structural lesions of the basal ganglia or the brainstem. Extensive bifrontal leukoencephalopathy was found in one patient who suffered severe head trauma.

Accidents, Traffic↗