Epidemiology of restless legs syndrome.
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Journal Article↗
Biomedical subjects
W Ondo
Publications and source records attributed to W Ondo.
Alcohol dehydrogenase polymorphism and Parkinson's disease.
A particular alcohol dehydrogenase (ADH) polymorphism (allele A1) in the promoter region of the gene has been recently demonstrated to be associated with increased risk of Parkinson's disease (PD). In a case control study, we examine frequencies of ADH A1 allele in 100 PD patients (i.e. 200 alleles), 100 diseased controls (i.e. 200 alleles), and 194 healthy controls (i.e. 388 alleles). In addition, we study possible association of a combined non-amyloid component of plaque (NACP-Rep 1) allele and ADH A1 allele with risk of PD. There was no statistical significance of the frequencies of ADH A1 allele between PD patients 12/200 (6%), diseased controls 13/200 (6.5%), and healthy controls 20/388 (5.2%). No strong evidence of an association was found between ADH A1 allele and PD susceptibility in our study patients. There was also no suggestion of linkage disequilibrium between NACP-Rep 1 and ADH A1 alleles.
Adult↗
Thalamic deep brain stimulation: comparison between unilateral and bilateral placement.
BACKGROUND: Unilateral thalamic deep brain stimulation (DBS) is accepted as an effective treatment for essential tremor (ET) and the tremor of Parkinson disease (PD). There are, however, relatively little data concerning bilateral thalamic DBS and no thorough comparisons between the 2 methods. METHODS: To assess the relative benefit of a staged second contralateral DBS placement in patients with PD and ET, we compared preoperative baseline assessments with those at 3 months after the initial implantation, and again at 3 months after the second contralateral implantation. The assessments included the Unified Parkinson's Disease Rating Scale for patients with PD (n = 8) and a modified Unified Tremor Rating Assessment for patients with ET (n = 13). The design included open and blinded (unknown activation status) assessments. RESULTS: Overall, after the second implantation, all specific measures assessing tremor contralateral to that side improved in patients with PD and ET, generally without sacrificing those contralateral to the first side implantation. Midline tremors (face and head) improved only after the second side implantation. In patients with ET, functional and subjective scores tended to further improve after the second placement; however, patients with PD had less subjective improvement. Hand tremor scores in patients with ET randomized to "on" stimulation improved from 6.7 +/- 0.9 to 1.3 +/- 1.2 (P<.005). The scores of patients with PD randomized to on stimulation improved from 9.3 +/- 1.0 to 1.0 +/- 0.5. (Data are given as mean +/- SD.) Tremor scores did not change from baseline in those patients randomized to "off" stimulation in either group. Adverse events related to stimulation increased after the second implantation in both groups. CONCLUSIONS: Bilateral thalamic DBS is more effective than unilateral DBS at controlling bilateral appendicular and midline tremors of ET and PD. Despite this, overall functional disability only improved in patients with ET, possibly secondary to more problematic adverse events in patients with PD, especially balance problems. Bilateral DBS should be considered when unilateral DBS does not offer satisfactory benefit, especially in patients with ET.
Aged↗
Intrathecal baclofen for dystonia.
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Adult↗
Thalamic deep brain stimulation: effects on the nontarget limbs.
Unilateral thalamic ventral intermediate (VIM) deep brain stimulation (DBS) is now accepted as an effective treatment for essential tremor (ET) and tremor related to Parkinson's disease (PD). The effects of unilateral placement on the side ipsilateral to the surgical site have not been carefully evaluated. To systematically assess the effects ipsilateral to the surgical side and to determine the effects of device inactivation on the baseline tremor, we evaluated tremor in 73 patients approximately 3 months after their unilateral thalamic placement. Assessment included blinded and unblinded ratings using the Unified Parkinson's Disease Rating Scale for PD patients and a modified Tremor Rating Scale in ET patients. All measures of tremor contralateral to the implantation site improved significantly and robustly in both PD and ET. Implantation did not worsen tremor by any measure on the ipsilateral side. There was mild ipsilateral improvement as measured by lower observed tremor scores in ET (6.0 +/- 1.8 to 5.0 +/- 1.9, P < 0.005), but not PD. There was no rebound augmentation of tremor in either hand after the devices were deactivated in either group. We conclude that VIM DBS may mildly improve ipsilateral ET, and that concerns about meaningful ipsilateral tremor augmentation after device deactivation are not warranted.
Aged↗
Comparison of thalamotomy and pallidotomy for the treatment of dystonia.
OBJECTIVE: Thalamotomy and, more recently, pallidotomy have been used to treat selected patients with intractable dystonia, although few studies have compared the effectiveness of these two surgical procedures. In this study, we compare our results using thalamotomy and pallidotomy to treat patients with different forms of dystonia, and we discuss our results in the context of other published series. METHODS: Thirty-two patients with intractable dystonia underwent thalamotomy (n = 18) or pallidotomy (n = 14). Dystonia was classified according to cause and distribution, and each patient was evaluated postoperatively at two or more time points, using a global outcome scale. RESULTS: Although comparisons are limited by differences between the two surgical groups, including longer follow-up periods for the thalamotomy group, differences in symptom distribution, and more bilateral procedures for the pallidotomy group, patients with primary dystonia who underwent pallidotomies demonstrated significantly better long-term outcomes than did patients who underwent thalamotomies (P = 0.0467). Patients with secondary dystonia experienced more modest improvements after either procedure, with little or no difference in outcomes between the two procedures. CONCLUSION: For patients with primary dystonia, pallidotomy seems to result in better outcomes than does thalamotomy.
Adolescent↗
Computerized posturography analysis of progressive supranuclear palsy: a case-control comparison with Parkinson's disease and healthy controls.
BACKGROUND: Progressive supranuclear palsy (PSP) is a neurodegenerative disorder that is frequently mistaken for Parkinson's disease (PD) in its early stages. OBJECTIVE: To compare balance measures using computerized posturography in patients with early PSP and early PD. METHODS: We performed computerized posturography (SMART Balance Master; NeuroCom International, Inc, Clackamas, Ore) in 20 patients with clinically diagnosed mild to moderate PSP (ambulatory) and compared results with those from 20 patients with PD of similar age and disease duration who were not receiving medications, and from 20 healthy age- and sex-matched controls. Sensory organization testing (SOT), limits of stability (LOS), and toes-up perturbations (4 degrees at 50 degrees per second) were tested while receiving and not receiving a combination of oral carbidopa (25 mg) and levodopa (250 mg) in the PSP group. Clinical assessment included Unified Parkinson's Disease Rating Scale, Performance-oriented assessments, and functional reach. RESULTS: When compared with the PD and control groups, total LOS time (P < .001) and path sway (P < .001) were significantly prolonged in PSP. Total SOT showed significantly worse scores in PSP compared with PD and control groups (F(2,57) = 29.6; P < .001). Univariate follow-up tests comparing PSP and PD showed differences in the following conditions: eyes open and visual sway (P = .003), eyes open and platform sway (P = .003), eyes closed and platform sway (P < .001), and eyes open and platform and visual sway (P < .001). Medium- and long-latency responses to perturbation were similar, but a larger number in the PSP group lacked short-latency responses (chi(2) = 11.3; P = .002). Levodopa administration did not significantly improve any aspect of posturography testing in PSP. In differentiating PSP from PD, LOS time and SOT condition of eyes open and platform and visual sway were nearly 100% sensitive and 100% specific (canonical correlation, 0.91). CONCLUSIONS: Computerized posturography testing reliably differentiated early PSP from early PD and age-matched controls. The PSP group demonstrated severely contracted limits of stability with probable deficits in motor programming. Results of SOT in PSP suggested a vestibular pattern and overreliance on visual cues, even when incorrect. The absence of short-latency responses (monosynaptic reflex arch) suggests an additional disturbance in the spinal cord or peripheral nervous system. Arch Neurol. 2000;57:1464-1469
Aged↗
Clinical characteristics of pramipexole-induced peripheral edema.
BACKGROUND: Pramipexole, a new dopamine agonist, effectively treats early and advanced Parkinson disease and restless legs syndrome. OBJECTIVES: To report the clinical features of and to investigate the predisposing factors and eventual outcomes in patients who developed peripheral edema (PE) following treatment with pramipexole. DESIGN: Retrospective case series in a tertiary referral center. RESULTS: Of the 300 patients who were receiving pramipexole therapy, 17 patients had mild to severe PE, which was attributable to the medication. Fifteen patients had Parkinson disease and 2 patients had restless legs syndrome. The mean (+/-SD) time of onset of PE after pramipexole therapy was started was 2.6 +/- 3.6 months (range, 0.25-11 months). The mean (+/-SD) dose at onset of PE was 1.7 +/- 1.0 mg/d (dose range, 0.75-3 mg/d) and the mean (+/-SD) dose when PE was at its maximum was 2.6 +/- 0.7 mg/d (dose range, 1.5-3 mg/d). In all cases, the PE rapidly abated with discontinuation of therapy, and in all cases that we rechallenged, it rapidly returned. The condition seemed to be dose dependent but also idiosyncratic, as we could not identify any predisposing features. It resulted in extensive medical evaluation in some patients and was only minimally responsive to diuretic therapy. CONCLUSION: Peripheral edema should be included among the potential adverse events associated with pramipexole therapy.
Adult↗
Bruxism in Huntington's disease.
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Adolescent↗
Rheumatologic serologies in secondary restless legs syndrome.
Diagnostic criteria for restless legs syndrome (RLS) have been established; however, the pathophysiology of this common condition remains elusive. Several secondary forms of RLS potentially include renal failure, iron deficiency, pregnancy, and neuropathy. RLS has also been reported in approximately 25% of patients diagnosed with rheumatoid arthritis and Sjögren's syndrome. We performed clinical and serologic evaluations on 68 patients diagnosed with RLS to determine how many may have concurrent rheumatologic disease that could be causing their RLS symptoms. We compared these with other postulated secondary causes of RLS. No patient had clinical evidence of rheumatologic disease, and only four had any positive serologic evaluations (two positive SSA/SSB and two mildly elevated RF titers). Three of these had a positive family history for RLS. Patients without a family history of RLS did have lower ferritin levels, more cases of neuropathy, and an older age at symptom onset. We do not think rheumatologic disease represents a significant secondary cause of RLS and do not recommend serologic investigation unless there are overt clinical signs. In contrast, our study suggests that neuropathy and serum iron deficiency do represent secondary forms of RLS.
Adult↗
Gabapentin for essential tremor: a multiple-dose, double-blind, placebo-controlled trial.
Essential tremor (ET) is a common movement disorder that often becomes refractory to conventional pharmacologic management. Open-label studies suggest that gabapentin is efficacious for ET, but the results of controlled trials have been mixed. To determine the efficacy and tolerability of gabapentin in ET, we conducted a double-blind, placebo-controlled, cross-over trial evaluating two doses (1800 mg per day and 3600 mg per day; N = 25). Patients on other ET medications were maintained on their concurrent medications for 3 months prior to study initiation and throughout the study. Twenty patients (mean age, 69.9 +/- 6.1 yrs) completed the study. Overall, patient global assessments (p <0.05), observed tremor scores (p <0.005), water pouring scores (p <0.05), and activities of daily living scores (p <0.005) significantly improved. Accelerometry scores, spirographs, and investigator global impression scores did not improve. The results were similar for high and low doses. Statistical regression models did not demonstrate any significant predictors for response. Gabapentin may be effective in some cases of ET.
Acetates↗
Restless legs syndrome: clinical features and treatment.
Restless legs syndrome (RLS), widely recognized as a definite clinical entity, has an estimated prevalence of 1 to 15% in different ethnic populations. However, it remains an underdiagnosed condition and its symptoms are frequently ascribed to stress and anxiety. Advancement in modern imaging techniques and clinical drug trials provide evidence of an impaired dopaminergic system in RLS. Management involves investigating and correcting treatable secondary causes, avoidance of aggravating factors, and pharmacologic therapy. Recent controlled trials have demonstrated the effectiveness of dopamine agonists such as pramipexole and pergolide. Additional research is needed to further elucidate the pathophysiology of RLS, through obtaining post-mortem specimens and refinement of neuroimaging and neurophysiologic techniques. Isolation of specific genetic loci in familial cases would enable better characterization of distinct clinical and genetic subsets of RLS and result in better understanding of this disease at the molecular level.
Adrenergic Agonists↗
Ropinirole for restless legs syndrome.
Restless legs syndrome (RLS) is a common and underdiagnosed condition that results in a desire to move the extremities often associated with paresthesia/dysesthesia, motor restlessness, worsening of symptoms at rest with at least temporary relief by activity, and worsening of symptoms in the evening or night. We tested the new dopamine agonist ropinirole in 16 patients with RLS in an open-label trial. The mean daily dose was 2.8+/-2.3 mg (range, 0.5-12.0). The 13 patients who completed the study reported a 58.7% improvement (p = 1.08 x 10(-8)) as judged by the abbreviated International Restless Legs Study Group questionnaire. Three patients discontinued the medication secondary to adverse events (rash and nervousness) and other extenuating circumstances. These encouraging preliminary results justify larger and more controlled trials of ropinirole in patients with RLS.
Adult↗
A novel sublingual apomorphine treatment for patients with fluctuating Parkinson's disease.
We tested a novel preparation of a sublingual apomorphine hydrochloride tablet (APO) in 10 patients with advanced Parkinson's disease complicated by motor fluctuations. After a dose titration, patients took either 40 mg APO three times per day alternating with levodopa doses (eight patients) or six doses of 20 mg APO taken concurrently with levodopa doses (two patients) for 3 months. Assessments included timed tapping and ambulation tests, Unified Parkinson's Disease Rating Scale (UPDRS), and patient diaries. Tapping speed while taking only APO (12 hours after stopping levodopa) was faster than while taking only levodopa (p <0.05). The daily levodopa dose decreased by 32.1% (p <0.01), yet the total "on" time increased from 73.5% +/- 10.2% to 81.5% +/- 7.5% of the day (p <0.01) after starting APO. "On" UPDRS part II scores (p <0.05) and "on" UPDRS part III (motor examination) scores (p <0.05) also improved. Adverse events such as nausea, orthostatic hypotension, and disagreeable taste did not limit the dose of APO in any case. The short-term benefit and tolerability demonstrated in this study warrant further study of this new APO preparation.
Administration, Sublingual↗
The alpha-synuclein gene is not a major risk factor in familial Parkinson disease.
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Adult↗
Re-emergent tremor of Parkinson's disease.
OBJECTIVE: To characterise postural tremors in patients with Parkinson's disease. Rest tremor is a well recognised cardinal symptom of Parkinson's disease, but postural tremors associated with the disease may cause more disability than the more typical rest tremor. Postural tremor of Parkinson's disease has been attributed to enhanced physiological tremor, clonus, or coexistent essential tremor. It is postulated that one type of postural tremor in Parkinson's disease represents a rest tremor that re-emerges after a variable delay while maintaining posture, hence "re-emergent tremor". METHODS: Accelerometry, peak frequency, peak frequency amplitude, root mean square (RMS) amplitude, and latency were determined in 18 patients (mean age: 63.2 (SD 9.8) years) with Parkinson's disease who had clinically evident postural tremor, 20 (mean age: 66.9 (SD 5. 8) years) with typical essential tremor, and seven (mean age: 68.7 (SD 15.3) years) with the combination of pre-existing essential tremor and subsequent Parkinson's disease (essential tremor/Parkinson's disease). Latency, the time interval starting with the assumption of an outstretched posture and ending with the onset of postural tremor, was measured by marking the start time by a pulse produced from interrupting a beam to a photocell when the arm reached a horizontal position. RESULTS: The latency for the re-emergent tremor (9.37 (SD 10.66) s), present in 12 of 18 patients with Parkinson's disease, was significantly (p<0.0005) longer than the latency for postural tremor of essential tremor (1.29 s in one patient, absent in 19 others); five of seven essential tremor/Parkinson's disease patients had an observed latency (6.57 (SD 8.23 s) which was also significantly (p<0.005) longer than that for essential tremor. There was no difference in the mean tremor frequency ( approximately 5.5 Hz) between the re-emergent tremor and the more typical Parkinson's disease rest tremor. The amplitudes were generally higher for the postural tremor associated with Parkinson's disease compared with those of essential tremor. CONCLUSION: These studies suggest that the re-emergent tremor of Parkinson's disease can be differentiated from the postural tremor of essential tremor and that it may share pathophysiological mechanisms with the more typical rest tremor.
Aged↗
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↗
Encouraging initial response of restless legs syndrome to pramipexole.
Restless legs syndrome (RLS) is a common condition that results in uncomfortable sensations and an urge to move the limbs. Two centers tested a new dopamine agonist, pramipexole, in 23 patients with RLS in a time-limited, open-label, clinical trial. After 4 weeks or more, 19 patients reported significant improvement as assessed by the short International Restless Legs Syndrome Study Group questionnaire (p < 0.0001). These encouraging preliminary results justify larger, controlled trials for pramipexole in patients with RLS.
Adult↗