bluuebunny/crossref_metadata_2025_split huggingface dataset:
Olivopontocerebellar pathology in multiple system atrophy
Authors: Wenning G. K. , Tison F. , Elliott L. , Quinn N. P. , Daniel S. E. 
Published: December 2004

AbstractOlivopontocerebellar atrophy (OPCA) is widely accepted as part of the neuropathological spectrum of multiple system atrophy (MSA). The distribution of affected sites in the olivopontocerebellar (OPC) system and their interrelationship remain poorly understood due to lack of quantitative studies. To further investigate the OPC pathology in MSA, we performed a morphometric analysis of 20 MSA cases and eight healthy controls. In the MSA cases, mean neuronal cell densities were significantly reduced in (medial and dorsal) accessory and principal inferior olives, pontine nuclei, cerebellar vermis (except nodulus), and hemispheres. Inferior olives and pontine nuclei were more severely affected than cerebellar Purkinje cells in most cases. Cerebellar Purkinje cells were more severely depleted in vermis rather than in hemisphere. There was a poor topographic correlation between neuronal cell loss in inferior olives and cerebellar cortex. These results suggest a primary degeneration of olivopontine nuclei and cerebellar Purkinje cells in OPCA. Inferior olives, pontine nuclei and cerebellar cortex were all significantly more severely affected in cases with a pure or predominating cerebellar syndrome (OPCA type, n = 4) compared to those with pure or predominating parkinsonism (SND type, n = 14). However, although cerebellar signs had been noted in life in only six cases, morphometry revealed OPCA in 17 of the 20 MSA brains.
Response to botulinum toxin F in seronegative botulinum toxin A—resistant patients
Authors: Greene Paul E. , Fahn Stanley 
Published: December 2004

AbstractBotulinum toxin type A (btx A) injections are the most effective treatment for most patients with focal dystonia. Some patients who improve after btx A injections and later lose response have serologic evidence of antibodies to btx A with the mouse neutralization assay (seropositive patients). Another group of patients who lose response to btx A do not have detectable antibodies (seronegative patients). Seropositive patients may improve after injections of botulinum toxin type F (btx F), an alternative serotype of botulinum toxin. We treated nine seronegative resistant patients with btx F. None of these patients had muscle atrophy after injection with btx A. Five of the nine had improvement after btx F injection that was sustained for at least three consecutive btx F injections. This observation is consistent with the hypothesis that btx resistance in seronegative patients is caused by undetected antibodies to btx A. If this be the case, then there may be techniques for preventing or reversing btx resistance, as in the case of resistance to factor VIII in the treatment of hemophilia A.
Reliability of patient completion of the historical section of the unified Parkinson's disease rating scale
Authors: Louis Elan D. , Lynch Timothy , Marder Karen , Fahn Stanley 
Published: December 2004

AbstractEvaluating the reliability of self‐assessed disability in patients with Parkinson's disease (PD) is important for therapeutic trials and epidemiologic surveys. This is the first study to examine the interrater reliability between physician and patient in the historical section of the Unified Parkinson's Disease Rating Scale (UPDRS). Thirty consecutive subjects with idiopathic PD self‐administered the historical section of a modified UPDRS. This instrument was then readministered to these 30 subjects by a neurologist. Interobserver reliability was assessed with the weighted kappa statistic (kw). The kw for each of the 17 items in the historical section of the UPDRS ranged from 0.63 to 1.0 (moderate to excellent agreement). Total kw = 0.83 (95% confidence interval = 0.79‐0.87). There were no correlations between kw and age, disease duration, dose of levodopa, Hoehn and Yahr score, Schwab and England score, or modified Mini‐Mental State score. Nondemented subjects with PD may reliably assess their level of disability by self‐administering the historical section of the UPDRS. This has important implications for the reliable use of self‐administered disability instruments for clinical research.
Rhythmic auditory stimulation in gait training for Parkinson's disease patients
Authors: Thaut M. H. , McIntosh G. C. , Rice R. R. , Miller R. A. , Rathbun J. , Brault J. M. 
Published: December 2004

AbstractRhythmic auditory stimulation (RAS) was used as a pacemaker during a 3‐week home‐based gaittraining program for Parkinson's disease (PD) patients (n = 15). Electromyogram (EMG) patterns and stride parameters were assessed before and after the test without RAS to evaluate changes in gait patterns. Data were compared with those of two control groups (n = 11), who either did not participate in any gait training or who participated in an internally self‐paced training program. RAS consisted of audiotapes with metronome‐pulse patterns embedded into the on/off beat structure of rhythmically accentuated instrumental music. Patients who trained with RAS significantly (p < 0.05) improved their gait velocity by 25%, stride length by 12%, and step cadence by 10% more than self‐paced subjects who improved their velocity decreased by 7% and no‐training subjects whose velocity decreased by 7%. In the RAS‐group, timing of EMG patterns changed significantly (p < 0.05) in the anterior tibialis and vastus lateralis muscles. Evidence for rhythmic entrainment of gait patterns was shown by the ability of the RAS group to reproduce the speed of the last training tape within a 2% margin of error without RAS.
Occupation, education, and Parkinson's disease: A case‐control study in an Italian population
Authors: Rocca Walter A. , Anderson Dallas W. , Meneghini Francesca , Grigoletto Francesco , Morgante Letterio , Reggio Arturo , Savettieri Giovanni , Di Perri Raoul 
Published: December 2004

AbstractCurrent epidemiologic data on the association between occupational exposures and Parkinson's disease (PD) are inconsistent. In a case‐control study, we investigated the associations between occupation and PD and between education and PD. The cases (n = 62) were those identified in a prevalence survey (door‐to‐door, two‐phase) of three Sicilian municipalities, as of November 1, 1987. We then randomly selected from the general population two controls for each case, matched for age (±1 year), sex, and municipality (n = 124). Information on current and past occupations and education for cases and controls was obtained during the survey. Subjects who worked for most of their lives as farmers were not at increased risk of PD (OR = 0.6; 95% CI = 0.3–1.3). Neither were subjects who worked for most of their lives in other occupations (e.g., housewives, fishermen, factory workers, salesmen, craftsmen, clerks). PD was not associated with low education. Our findings suggest that farming, as a broad occupational category, does not play a major role in the causation of PD.
DC60: An example of one of the 450 cases that compose the Denny‐Brown collection of primate lesion material
Authors: Vilensky Joel A. , Gilman Sid , Morecraft Robert J. 
Published: December 2004

AbstractThe Denny‐Brown collection consists of films depicting the behavioral responses of ∼450 monkeys after central nervous system lesions; operative, behavioral, and neuropathologic descriptions; and histologic slides. This collection is available for use by interested investigators. This report describes one case, DC60, to provide an example of the types of material that are available and how these materials might be used better to understand the nervous system. To demonstrate how precisely the actual lesions can be defined and compared with the planned ablations, this report also includes a detailed evaluation of the extent of the lesions made in this case, based on the histologic slides and on photographs of the brain taken before histologic processing.
Rapid eye movement sleep behavior disorder preceding Parkinson's disease with therapeutic response to levodopa
Authors: Tan Adrian , Salgado Miran , Fahn Stanley 
Published: December 2004

AbstractRapid eye movement (REM) sleep behavior disorder (RBD) involves complex behavior and a loss of muscle atonia occuring during REM sleep. Half of these patients with RBD have an underlying neurologic disorder including dementia, olivopontocerebellar atrophy, subarachnoid hemorrhage, and cerebrovascular disease. Clonazepam is the drug of choice for RBD. RBD has been rarely reported to precede the onset of Parkinson's disease (PD). Three patients are presented here whose RBD preceded the onset of PD by several years, and both the symptoms of PD and RBD improved with levodopa treatment. It is postulated that levodopa ameliorates RBD by suppressing REM sleep, and it remains to be seen whether levodopa can be an alternative to clonazepam in idiopathic RBD without PD.
Ataxia without telangiectasia masquerading as benign hereditary chorea
Authors: Klein C. , Wenning G. K. , Quinn N. P. , Marsden C. D. 
Published: December 2004

AbstractWe report a nonconsanguineous family in whom two (of three) sons developed isolated chorea in early childhood, suggesting a diagnosis of benign hereditary chorea (BHC). However, cerebellar ataxia and oculomotor apraxia, without telangiectasia, subsequently developed. Chromosome analysis showed increased radiosensitivity in both brothers and translocations in the younger one. We conclude that ataxia with chromosomal instability may masquerade as BHC in some patients.
Effect of the putative dopamine D1 agonist and D2 antagonist FCE 23884 on Parkinson's disease
Authors: Metman L. Verhagen , Blanchet P. J. , de Jong D. , Mouradian M. M. , Chase T. N. 
Published: December 2004

AbstractThe ergoline derivative FCE 23884 acts as a dopamine D1 agonist in untreated parkinsonian animals and as a D2 antagonist in animals whose dopamine system is intact or levodopa treated. To evaluate whether this dual action might benefit patients with Parkinson's disease (PD) who have developed levodopa‐induced dyskinesias, the motor effects of FCE 23884 were examined in seven such individuals using a double‐blind, placebo‐controlled design. At doses up to the maximum tolerated dose (3.5 ± 0.5 mg), FCE 23884 monotherapy did not affect parkinsonian severity. On the other hand, coadministration of FCE 23884 with a mildly dyskinetic dose of levodopa, infused intravenously under steady‐state conditions, reduced the antiparkinson response by 54 ± 19% and tended to diminish dyskinesia severity. The results thus fail to suggest any useful role for FCE 23884 in the symptomatic treatment of PD. Although D2 receptor blockade provided by FCE 23884 antgonizes both the antiparkinson and dyskinesigenic responses to levodopa, the degree of D1 receptor stimulation appears insufficient to ameliorate parkinsonian symptomatology.
Neutrophil function, nitric oxide, and blood oxidative stress in Parkinson's disease
Authors: Gatto Emilia M. , Carreras María C. , Pargament Griselda A. , Riobo Natalia A. , Reides Claudia , Repetto Marisa , Pardal Manuel M. Fernandez , Llesuy Susana , Poderoso Juan J. 
Published: December 2004

AbstractWe studied nitrogen radical nitric oxide (.NO) release and reactive oxygen species (ROS) production by isolated neurophils after phorbol myristate acetate (PMA) stimulation in 12 newly diagnosed and nine treated Parkinson's disease (PD) patients and 10 age‐matched healthy controls. Neutrophils of both groups of PD patients had an elecated PMA‐activated release of ·NO [61 and 57%, respectively, higher than that of controls (p < 0.05)]. In contrast, H2O2 release was only significantly increased by 56% in chronically treated patients. In agreement, the maximum rate of luminoldependent chmiluminescence, which partly represents O2‐H2O2‐.NO interactions, was increased only in the treated group. When other blood markers of oxidative stress were compared, only erythrocyte catalase activity was decreased in both PD patient series by 33 and 39%, respectively (p < 0.05), whereas plasma antioxidant capacity and erythrocyte superoxide dismutase activity levels were decreased only in treated PD patients. This study suggests that neurophils express a primary alteration of ·NO release in PD patients, whereas H2O2 and oxidativestress parameters are more probably related to the evolution of PD or to effects of treatment with L‐dopa.
Sympathetic skin response and R‐R interval variability in multipele system atrophy and idiopathic Parkinson's disease
Authors: Bordet R. , Benhadjali J. , Destee A. , Hurtevent J. F. , Bourriez J. L. , Guieu J. D. 
Published: December 2004

AbstractWe compared autonomic function in patients with multiple system atrophy (MSA) or with idiopathic Parkinson's disease (IPD) by measuring sympathetic skin response (SSR) and R‐R interval variability (RRIV). SSR was investigated in 26 patients (13 with MSA and 13 patients with IPD). RRIV during deep breathing, Valsalva maneuver, and on standing was investigated in 20 patients (nine with MSA and 11 with IPD). MSA and IPD patients had similar age, illness duration, and therapy. Abnormal SSR was more frequent in MSA (69%) than in IPD (7.7%; X2, 10.4; p < 0.002). RRIV during deep breathing and the Valsalva maneuver was lower in MSA than in IPD (p < 0.02). RRIV during standing up was not significantly different in IPD and MSA, These differences between MSA and IPD may be due to more severe and widespread autonomic disturbance in MSA, related to more severe neuropathologic involvement of the autonomic nervous system. SSR and RRIV may aid in the differntial diagnosis of parkinsonism and help to exclude from clinical trials MSA patients clinically misdiagnosed as having IPD.
A controlled trial of remacemide hydrochloride in Huntington's disease
Authors: Kieburtz Karl , Feigin Andrew , McDermott Michael , Como Peter , Abwender David , Zimmerman Carol , Hickey Charlyne , Orme Constance , Claude Kathy , Sotack Jennie , Greenamyre J. Timothy , Dunn Cynthia , Shoulson Ira 
Published: December 2004

AbstractWe conducted a randomized, double‐blind, placebo‐controlled tolerability study of a N‐methyl‐D‐aspartate (NMDA) glutamate receptor ion‐channel blocker, remacemide hydrochloride, in 31 independently ambulatory patients (18 men, 13 women) with Huntington's disease (HD). Subjects were randomized to receive either placebo or active remacemide at dosages of 200 mg/day or 600 mg/day. The primary outcome measure was the proportion of subjects able to compleate the study with the assigned treatment. Remacemide was generally well tolerated, and no significant differences between the treatment arms were found in the primary outcome measure. A trend toward impovement in chorea was observed among subjects administered remacemide 200 mg/day. Based on the tolerability and safety demonstrated during this short‐trem trial, remacemide warrants more extended controlled investigation in patients with HD.
Stuttering may be a type of action dystonia
Authors: Kiziltan G. , Akalin M. A. 
Published: December 2004

AbstractWe observed abnormal involuntary movements, involving principally the facial and neck muscles, in 23 patients with stuttering. These movements were similar to involuntary movements seen in distinct dystinic syndromes. There was a history of stuttering in the first degree relatives of six patients. The association of stuttering with degenerative neurologic disorders and focal brain lesions, cerebral blood flow changes in patients with developmental stuttering, its occurrence as a side effect of centrally acting drugs, induction and alleviation of stuttering by mechanical perturbation, or by electrical stimulation of the thalamus, a strong genetic predisposition with male preponderance, and the statistically significant occurrence of stuttering in the family history of patients with idiopathic torsion dystonia suggest an organic basis for developmental stuttering. These findings and the reported similarities between the involuntary movements associated with stuttering and dystonic involuntary movements support the hypothesis that stuttering is a form of segmental or focal action dystonia.
The electroencephalogram in acetazolamide‐responsive periodic ataxia
Authors: Neufeld M. Y. , Nisipeanu P. , Chistik V. , Korczyn A. D. 
Published: December 2004

AbstractAcetazolamide‐responsive periodic ataxia (ARPA) is a rare movement disorder, characterized by recurrent episodes of vertigo, cerebellar ataxia, and nystagmus, which has recently been characterized genetically. The pathophysiology is unknown, but it is probably not epileptic. By definition, acetazolamide produces an impressive symptomatic relief. Because of the paroxysmal nature of the disorder, EEG tracings were often obtained. We report four new cases (two familial and two sporadic) with typical ARPA (none of whom had metabolic abnormalities or continuous electrical muscle activity) and review the EEG findings associated with this disorder. EEG findings were reported in 18 kindreds and nine sporadic cases (including ours). EEG was described in 54 of the 140 affected cases and was abnormal in 52% (28/54). Most commonly seen was intermittent generalized slow activity, observed in 35% (19/54), frequently intermingled with spikes (10 cases). Other abnormalities included nonspecific mild generalized or focal slowing in seven (13%) and focal epileptic activity in two (4%) patients. The paroxysmal EEG activity frequently seen in ARPA should not establish a diagnosis of epilepsy. Although not specific, it may suggest the correct diagnosis and indicate treatment with acetazolamide.
Computational analysis of open loop handwriting movements in Parkinson's disease: A rapid method to detect dopamimetic effects
Authors: Eichhorn T. E. , Gasser T. , Mai N. , Marquardt C. , Arnold G. , Schwarz J. , Oertel W. H. 
Published: December 2004

AbstractWe used a computational analysis of open loop handwriting movements and a clinical rating scale for monitoring the effect of apomorphine in 16 patients with early untreated parkinsonism [subsequently L‐DOPA responsive, probable Parkinson's disease (PD)], six patients with long‐standing PD with L‐DOPA associated motor fluctuations, and seven patients with known L‐DOPA unresponsive parkinsonism. Subjects were instructed to write fluently concentric circles of ∼ 12 mm in diameter. Movements were recorded for two periods of 3 a each, using a digitizing tablet. Mean Peak velocity (Vmax) and mean peak acceleration (Amax) were determined. In addition, two sensitive indices describing the degree of automation of handwriting were derived: (a) NCV, calculated as the mean Number of Changes in direction of Velocity per half circle, and (b) NCA, the mean Number of Changes in the direction of Acceleration. Clinical rating was performed according to the Unified Parkinson's disease Rating Scale part III (UPDRS III). After apomorphine injection, the patients with early untreated probable PD showed significant improvement of Vmax' Amax' NCV, NCA, and UPDRS III scores. Likewise, the patients with long‐standing PD improved significantly in all kinematic parameters and UPDRS III scores. Patients with L‐DOPA unresponsive parkinsonism failed to change significantly in any of the parameters tested. These observations suggest that the computer‐assisted analysis of automated handwriting movements can be used as an objective quick method for quantifying dopamimetic effects on the kinematics of handwriting movements in parkinsonian patients.
Familial paroxysmal dystonic choreoathetosis revisited
Authors: Schloesser David T. , Ward Thomas N. , Williamson Peter D. 
Published: December 2004

AbstractA case of familial paroxysmal dystonic choreoathetosis (PDC) documented by video/EEG monitoring is described. The father of the proband is affected by exertional cramping but not PDC, lending support to the previous hypothesis that exertional cramping may represent a “forme fruste” or the incomplete expression of PDC. Other family members affected by PDC are women, with exercise‐induced cramping alone found in two men. Two of the women report prolonged exertion as a precipitant of lengthy spells consistent with typical PDC rather than the previously described “intermediate,” exercise‐induced form of PDC. Exertional cramping in families affected by PDC may represent the variable expression of the “dystonia gene” in male members. Conversely, exercise‐induced PDC, both of the intermediate and longer form described here, may have a predilection to manifest in women.
Assessment of coenzyme q10 tolerability in huntington's disease
Authors: Feigin A. , Kieburtz K. , Como P. , Hickey C. , Abwender D. , Zimmerman C. , Steinberg K. , Shoulson I. 
Published: December 2004

AbstractWe performed a 6‐month open‐label trial to evaluate the tolerability and efficacy of coenzyme Q10 (CoQ) in 10 patients with Huntington's disease (HD), Subjects were evaluated at baseline, 3 months, and 6 months using the HD Rating Scale (HDRS), the HD Functional Capacity Scale (HDFCS), and standardized neuropsychological measures. Adverse events (AEs) were assessed by telephone interview every month. CoQ doses ranged from 600 to 1,200 mg per day. All subjects completed the study, although four subjects reported mild AEs, including headache, heartburn, fatigue, and increased involuntary movements. There was no singnificant effect of the treatment on the clinical ratings. The good tolerability of CoQ suggests that it is a good candidate for evaluation in liong–term clinical trials designed to slow the progression of HD.
Complex movement disorders induced by fluoxetine
Authors: Bharucha Kersi J. , Sethi Kapil D. 
Published: December 2004

AbstractWe report two cases of complex movement disorders induced by fluoxetine. A 72‐year‐old woman developed rhythmic palatal movements, myoclonus, chorea, and possibly dystonia after 2 years of therapy with fluoxetine. On withdrawal of fluoxetine, the movements abated after 5 days and did not recur. The second patient, a 58‐year‐old man, developed myoclonic jerking and rapid, stereotypic movements of his toes after a year of fluoxetine therapy. These complex movements have not been reported previously as an adverse effect of fluoxetine.
Improvement of severe postural cerebellar tremor in multiple sclerosis by chronic thalamic stimulation
Authors: Geny C. , Nguyen J.‐P. , Pollin B. , Feve A. , Ricolfi F. , Cesaro P. , Degos J.‐D. 
Published: December 2004

AbstractTremor can be particularly disabling in patients with multiple sclerosis (MS) and is mildly improved by drug treatment. The efficiency of stereotactic thalamotomy has been reported in a small number of patients but was counterbalanced by severe postoperative complications. Stimulation of the thalamic ventral intermediate nucleus, which is a less aggressive surgical method, is efficient in essential and in parkinsonian tremors. We report here the results of thalamic stimulation in 13 patients with MS with tremor. All patients were subjected to clinical examination, videorecording, and quantification of the functional disability before surgery and 3 months postoperatively. The surgical intervention was well tolerated in all cases. A clear improvement of the tremor was observed in 69.2% of the patients. Functional improvement was more varied and depended on the severity of tremor and coexistence of other neurological symptoms. Of the eight most severely affected patients, seven recovered the possibility to easily catch an object and use it. The results indicate that thalamic stimulation may be useful in the treatment of severe postural cerebellar tremor in MS.