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Movement Disorders Clinic (movement + disorders_clinic)
Selected AbstractsShort-term effects of tetrabenazine on chorea associated with Huntington's diseaseMOVEMENT DISORDERS, Issue 1 2007Christopher Kenney MD Abstract We sought to assess the short-term clinical effects of tetrabenazine (TBZ) on choreic movements in Huntington's disease patients. A total of 10 patients on stable doses of TBZ were enrolled in this observational study. Patients took their evening dose of TBZ and presented the next day to the Baylor College of Medicine Movement Disorders Clinic without taking the usual morning dose. They were assessed using the Unified Huntington's Disease Rating Scale (UHDRS) motor assessment and Beck Depression Inventory. The usual morning dose of TBZ was then administered and patients were followed with serial UHDRS motor examinations approximately every 2 hours until choreic movements subsided and then returned. TBZ decreased the UHDRS chorea score on average 42.4% ± 17.8%. The duration of effect varied from a minimum of 3.2 hours to a maximum of 8.1 hours (mean = 5.4 ± 1.3). No patient experienced an adverse event related to TBZ or its withdrawal. During short-term follow-up after a single dose, TBZ improves chorea for approximately 5 hours. © 2006 Movement Disorder Society [source] Association between amantadine and the onset of dementia in Parkinson's diseaseMOVEMENT DISORDERS, Issue 9 2006Rivka Inzelberg MD Abstract The objective of this study is to compare the occurrence of dementia among Parkinson's disease (PD) patients treated with amantadine (AM group) with those never exposed to it (NoAM group). PD dementia shares neuroanatomical and biochemical similarities with Alzheimer's disease (AD). Memantine, an N -methyl- D -aspartate (NMDA) receptor antagonist has been shown to be beneficial in AD. Memantine is a dimethyl derivative of amantadine, which also possesses NMDA receptor blocking properties. We hypothesized that amantadine could have a beneficial effect on the occurrence of PD dementia. PD patients attending the Movement Disorders Clinics in Hillel Yaffe, Asaf Harofe Medical Centers (Israel) and Pisa (Italy) were included. Taking the onset of dementia as the endpoint, survival curves for AM and NoAM patients were estimated by the Kaplan,Meier method. The study population consisted of 593 patients (age, 69.5 ± 9.9 years; PD duration, 9.2 ± 6.0 years; 263 patients (44%) amantadine treated). The endpoint of dementia was reached by 116 patients (20%). PD duration until dementia was significantly longer for AM patients (9.1 ± 5.7 years) than for NoAM patients (5.9 ± 4.6 years, P = 0.006). The duration of amantadine exposure positively correlated with PD duration until dementia (P = 0.0001). Survival analysis, taking dementia onset as endpoint, showed slower mental decline in AM patients (Log rank P = 0.0049, Wilcoxon P = 0.0024). Mini-Mental State Examination scores were significantly higher for AM patients than for the NoAM group (P = 0.01). Age of PD onset also significantly influenced the duration of PD until dementia. Amantadine use may delay the onset of dementia in PD patients and may attenuate its severity. © 2006 Movement Disorder Society [source] Postdural Puncture Headache in Complex Regional Pain Syndrome: A Retrospective Observational StudyPAIN MEDICINE, Issue 8 2009Alexander G. Munts MD ABSTRACT Objective., To describe the unusual course of postdural puncture headache (PDPH) after pump implantation for intrathecal baclofen (ITB) administration in patients with complex regional pain syndrome (CRPS)-related dystonia. Design., Case series based on data collected from 1996 to 2005. Setting., Movement disorders clinic, university hospital. Patients., A total of 54 patients with CRPS-related dystonia who were treated with ITB. Results., A high incidence (76%) and prolonged course (median 18 days, range 2 days to 36 months) of PDPH was found. Radionuclide studies performed in two patients with long-lasting symptoms (12,16 months) did not reveal cerebrospinal fluid (CSF) leakage. In patients without signs of CSF leakage (N = 38), epidural blood patches administered in 24 patients were effective in 54%, while ketamine infusions administered in six patients were effective in 67%. Conclusions., Our observations may suggest that other mechanisms besides intracranial hypotension play a role in the initiation and maintenance of PDPH in CRPS and stimulate new directions of research on this topic. [source] A 9-year review of dystonia from a movement disorders clinic in SingaporeEUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2006R. D. G. Jamora The clinical features of dystonia have not been evaluated in Southeast Asia. We therefore investigated the clinical spectrum and characteristics of dystonia in Singapore, a multi-ethnic Southeast Asian country comprising 77% Chinese, 14% Malays, and 8% Indians. We identified all dystonia patients from the Movement Disorders database and Botulinum Toxin clinic between 1995 and November 2004. Their medical records were reviewed to verify the diagnosis of dystonia and obtain demographic and clinical data using a standardized data collection form. A total of 119 (73%) patients had primary dystonia whilst 45 (27%) had secondary dystonia. There were 77% Chinese, 9% Malays, and 8% Indians. The most common focal dystonia were cervical dystonia (47%), writer's cramp (32%), and blepharospasm (11%). There was no significant difference in the distribution of dystonia between the different races. Males were noted to have earlier onset of dystonia overall. There was a significant male predominance in primary dystonia overall (M:F 1.6:1, P = 0.008) and in the subgroup of focal dystonia (M:F 1.6:1, P = 0.037). This contrasts with previous studies that found a female predominance. The role of genetic, hormonal, and environmental factors and their interactions need to be investigated to better understand the gender differences in the occurrence of dystonia. [source] Movement disorders and pregnancy: A review of the literature,,MOVEMENT DISORDERS, Issue 6 2010Sarah M. Kranick MD Abstract Pregnant patients are rarely encountered in the movement disorders clinic, but they present significant dilemmas regarding treatment and counseling for neurologists. While movement disorders in pregnancy once described those disorders arising de novo during pregnancy, such as chorea gravidarum or restless leg syndrome, advancing maternal age in Western countries will likely increase the number of women in whom pregnancy complicates a pre-existing movement disorder. Physicians treating these women must be aware of the impact of the movement disorder and its treatment on fertility, pregnancy, fetal development, lactation, and infant care. This review summarizes retrospective series and case reports to both guide clinicians and to stimulate and direct the design of prospective studies. © 2010 Movement Disorder Society [source] Treatment of cervical dystonia with botulinum toxinMOVEMENT DISORDERS, Issue S8 2004Joseph Jankovic MD Abstract Cervical dystonia (CD) is the most common form of dystonia encountered in a movement disorders clinic. The treatment of this focal dystonia has improved markedly with the advent on botulinum toxin (BTX) injections, which has now become the treatment of choice. Initial studies, even double-blind controlled trials, failed to show robust effect, largely as a result of poor design, often using fixed dosage and site of administration. When the BTX treatment is customized to the needs of the individual patients and the most involved muscles are targeted, the effects can be quite dramatic and the improvement usually lasts 3 to 4 months. Experience and improved skills can largely prevent the adverse effects such as dysphagia and neck weakness. Although there is no evidence that BTX slows the progression of the disease, as a result of early intervention with BTX, many of the long-term complications of CD, such as contractures and radiculopathy, have been largely eliminated. © 2004 Movement Disorder Society [source] Long-term efficacy of botulinum toxin A in treatment of various movement disorders over a 10-year periodMOVEMENT DISORDERS, Issue 6 2002G-Y.R. Hsiung MD Abstract Although botulinum toxin A (BTX) has been licensed in Canada for treatment of various movement disorders since 1990, few clinical studies regarding its long-term efficacy and side effects have been reported. We conducted a retrospective analysis of 235 patients who received BTX from our movement disorders clinic over a 10-year period (January 1990 to December 1999). A total of 2,616 treatment cycles (multiple injections) were administered to 235 patients with cervical dystonia (CD), hemifacial spasm (HS), blepharospasm (BP), and other movement disorders. Substantial benefit at 5 years was seen in most patients (90% in BP, 88% in HS, 63% in CD, 100% in jaw closing and lower limb dystonia, and 56% in writer's cramp). Benefit was maintained for up to 10 years in CD, HS, and BP data, with a 75.8% benefit reported. Twenty-eight percent of patients discontinued treatment during the follow-up period due to a variety of reasons. Of these, 9.1% of patients developed primary resistance, and 7.5% of patients secondary resistance. Adverse effects, mostly minor, developed in 27% of patients at any one time, occurring over 4.5% of treatment cycles. These were most frequently reported in blepharospasm (22 of 36 patients in 40 cycles), followed by hemifacial spasm (21 of 70 patients in 46 cycles), and cervical dystonia (17 of 106 in 28 cycles). Only 1.3% of patients discontinued therapy due intolerable adverse effects. The results show that BTX is a safe and effective treatment of various types of movement disorders, and most side effects are well tolerated. Discontinuation for any reason was also low after 5 years. Efficacy was maintained after long periods of treatment with high degree of patient satisfaction. © 2002 Movement Disorder Society [source] Phasic muscle activity in sleep and clinical features of Parkinson diseaseANNALS OF NEUROLOGY, Issue 3 2010Donald L. Bliwise PhD Objective: The absence of atonia during rapid eye movement (REM) sleep and dream-enactment behavior (REM sleep behavior disorder [RBD]) are common features of sleep in the alpha-synucleinopathies. This study examined this phenomenon quantitatively, using the phasic electromyographic metric (PEM), in relation to clinical features of idiopathic Parkinson disease (PD). Based on previous studies suggesting that RBD may be prognostic for the development of later parkinsonism, we hypothesized that clinical indicators of disease severity and more rapid progression would be related to PEM. Methods: A cross-sectional convenience sample of 55 idiopathic PD patients from a movement disorders clinic in a tertiary care medical center underwent overnight polysomnography. PEM, the percentage of 2.5-second intervals containing phasic muscle activity, was quantified separately for REM and non-REM (NREM) sleep from 5 different electrode sites. Results: Higher PEM rates were seen in patients with symmetric disease, as well as in akinetic-rigid versus tremor-predominant patients. Men had higher PEM relative to women. Results occurred in all muscle groups in both REM and NREM sleep. Interpretation: Although our data were cross-sectional, phasic muscle activity during sleep suggests disinhibition of descending motor projections in PD broadly reflective of more advanced and/or progressive disease. Elevated PEM during sleep may represent a functional window into brainstem modulation of spinal cord activity and is broadly consistent with the early pathologic involvement of non-nigral brainstem regions in PD, as described by Braak. ANN NEUROL 2010 [source] Fragile X-associated Tremor/Ataxia Syndrome (FXTAS)DEVELOPMENTAL DISABILITIES RESEARCH REVIEW, Issue 1 2004Paul J. Hagerman Abstract Carriers of fragile X mental retardation 1 (FMR1) premutation alleles (55 to 200 CGG repeats) are generally spared the more serious neurodevelopmental problems associated with the full-mutation carriers (>200 repeats) of fragile X syndrome. However, some adult male premutation carriers (55,200 repeats) develop a neurological syndrome involving intention tremor, ataxia, dementia, parkinsonism, and autonomic dysfunction. In excess of one-third of male premutation carriers over 50 years of age develop the fragile X- associated tremor/ataxia syndrome (FXTAS). FXTAS also represents a new form of inclusion disease, with eosinophilic intranuclear inclusions found throughout the brain in both neurons and astrocytes. Because FXTAS appears to be relatively specific to male premutation carriers, who are known to possess elevated levels of FMR1 mRNA, the neuropathology may arise as a consequence of a toxic gain-of-function of the mRNA itself, although this proposal requires additional direct testing. One of the critical needs at present is a better estimate for the prevalence of this disorder, because FXTAS is likely to be underdiagnosed in the adult movement disorders clinics. MRDD Research Reviews 2004;10:25,30. © 2004 Wiley-Liss, Inc. [source] | ||||||||||