bluuebunny/crossref_metadata_2025 huggingface dataset:
Patterns of regional cerebral blood flow in corticobasal degeneration studied using HMPAO SPECT; comparison with Parkinson's disease and normal controls
Authors: Markus H. S. , Lees A. J. , Lennox G. , Marsden C. D. , Costa D. C.
Published: May 2005

Corticobasal degeneration (CBD) is a rare syndrome characterised by an asymmetrical rigidity with localised cortical signs, particularly apraxia. Using positron emission tomography, abnormal patterns of cortical metabolism have recently been shown. We have studied patterns of regional cerebral blood flow (rCBF) using single photon emission tomography, with the tracer 99Technetium hexamethylpropylenamine (HMPAO), in subjects with CBD. In subjects with CBD, compared with 12 age‐matched normal controls, in the clinically more affected hemisphere a characteristic pattern was found with significant reductions in HMPAO uptake in the posterior frontal cortex (by 11.5%), and in the superior, inferior, anterior, and posterior parietal cortex (by 12.2, 12.9, 12.9, and 9.7, respectively). Reduced uptake was also found in the caudate (9.3%), putamen (9.7%), and thalamus (8.6%). In contrast, HMPAO uptake in the temporal and occipital cortex was normal. In comparison with 12 Parkinson's disease (PD) controls, significant reduced uptake was seen in the thalamus (9.0%), posterior frontal (8.9%), and inferior (9.9%), and anterior parietal (9.5%) cortex. A similar pattern of impaired uptake was seen in the clinically les/unaffected cerebral hemisphere in patients with CBD compared with normal controls, with a significant reduction in HMPAO uptake in the thalamus (6.8%), superior parietal (9.6%) and anterior parietal (7.6%), and posterior parietal (7.3%) cortex. This implies that the disease process is bilateral even in those cases with clinically unilateral disease. This widely available technique may be useful in the early diagnosis of CBD and in differentiation from other extrapyramidal disorders.
Oculofacial‐skeletal myorhythmia in central nervous system Whipple's disease: Additional case and review of the literature
Authors: Simpson David A. , Wishnow Robin , Gargulinski Richard B. , Pawlak Anne M.
Published: May 2005

A case of oculofacial‐skeletal myorhythmia associated with cerebral Whipple's disease is presented. This peculiar abnormal movement disorder consists of a convergent‐divergent pendular nystagmus associated with a synchronous, rhythmic movement of the mouth, jaw, and extremities. The movements in previous cases have responded inconsistently to a variety of broad‐spectrum antibiotic drugs, and antiepileptic, muscle relaxant, antispastic, and psychotropic drugs. The current patient's mental status improved after starting intravenous ceftriaxone but improvement of the abnormal movements occurred only after the addition of oral valproate. Current treatment consists of intravenous trimethoprim‐sulfamethoxazole for 2 weeks followed by oral trimethoprim‐sulfamethoxazole twice daily for 1 year. Presented here is an alternative treatment of intravenous ceftriazone followed by oral trimethoprim‐sulfamethoxazole in combination with valproate for abnormal movements not responsive to antibiotic drugs alone.
Bismuth subsalicylate toxicity as a cause of prolonged encephalopathy with myoclonus
Authors: Gordon Mark Forrest , Abrams Russell Ira , Rubin Daniel B. , Barr William B. , Correa Denise D.
Published: May 2005

Bismuth subsalicylate preparations are over‐the‐counter products for gastrointestinal complaints. Bismuth toxicity causes delirium, psychosis, ataxia, myoclonus, and seizures and is reversible over several weeks or months, when bismuth intake is stopped. We report a 54‐year‐old man with a 6‐week history of progressive confusion and memory difficulty and a 2–3‐week history of involuntary movements and gait impairment. His encephalopathy was further characterized by marked multifocal myoclonic jerks, coarse postural tremors, postural instability, and gait ataxia. He gradually improved. Extensive toxic, metabolic, and infectious workup demonstrated bismuth toxicity. Spinal tap and brain magnetic resonance scan were normal. Electroencephalography showed bihemispheric slowing. As his encephalopathy cleared, he reported using bismuth subsalicylate long term (daily intake of 8 oz). Bismuth levels 5 weeks after cessation of bismuth were elevated and normalized after 12 weeks. He followed a typical course for bismuth toxicity with subacute progressive encephalopathy and gradual recovery. Creutzfeldt‐Jakob was strongly considered due to his rapidly progressive encephalopathy, multifocal myoclonus, and ataxia. Due to its rarity, bismuth toxicity is often overlooked. We hope this presentation will increase recognition of bismuth toxicity. We believe more detailed labeling of bismuth products is needed to avoid similar toxicity from this readily available product.
Propriospinal myoclonus upon relaxation and drowsiness: A cause of severe insomnia
Authors: Montagna Pasquale , Provini Federica , Plazzi Giuseppe , Liguori Rocco , Lugaresi Elio
Published: May 2005

Three patients, aged 71, 50, and 41 years, presented with myoclonic activity arising in the relaxation period preceding sleep onset and causing severe insomnia. Polygraphic studies showed that the myoclonic activity began in spinally innervated muscles, propagating at low speed to rostral and caudal muscular segments. Myoclonus arose whenever patients relaxed mentally and showed diffuse electroencephalographic alpha activity and was independent of posture. The jerks disappeared during sleep. Clonazepam afforded symptomatic improvement. Propriospinal myoclonus arising from a spinal generator may be facilitated by changes in supraspinal control related to vigilance levels. When arising during relaxation and drowsiness, it may cause severe insomnia.
A long‐term follow‐up study of cinnarizine‐ and flunarizine‐induced parkinsonism
Authors: Negrotti A. , Calzetti S.
Published: May 2005

The natural course of calcium‐entry blocker–induced parkinsonism was evaluated in 13 elderly patients previously exposed to cinnarizine or flunarizine or both for a median period of 7 months. Clinical assessments were carried out before drug discontinuation and twice thereafter over a period lasting ≤7 years. None of the patients showed a full recovery of extrapyramidal signs, indicating that the long‐term prognosis of the parkinsonism is less benign than previously reported. Two main patterns of clinical outcome were recognized (i.e., “remittent” and “persistent and not progressive”parkinsonism), whereas the development of a progressive disorder was observed only in one patient. No significant correlation was found between the patterns of outcome and some clinical variables, such as total duration of exposure to cinnarizine and flunarizine, cumulative drug dosages, and age at onset of parkinsonism. There was no significant difference in terms of family history of essential tremor or parkinsonism or both among the patients with the two main patterns of clinical course.
Dietary antioxidants and other dietary factors in the etiology of Parkinson's disease
Authors: Scheider W. L. , Hershey L. A. , Vena J. E. , Holmlund T. , Marshall J. R. , Freudenheim J. L.
Published: May 2005

It has been suggested that dietary antioxidants reduce Parkinson's disease (PD) risk by neutralizing free radicals, thus preventing injury to neurons in the substantia nigra. This case‐control study examined the possible role of long‐term dietary antioxidant intake in PD etiology. Cases (n = 57) were males 45–79 years old with at least two cardinal signs of PD and no evidence of other forms of parkinsonism or dementia. Age‐matched friend controls (n = 50) were chosen from lists provided by the cases. Usual dietary intake 20 years ago, including vitamins E and C and carotenoids, was assessed by a 102‐item food frequency questionnaire. Odds ratios and 95% confidence intervals were calculated using conditional logistic regression. Antioxidant intake, adjusted for age, education, smoking, rural living, and total energy intake, was not associated with reduced PD risk. Trends toward greater PD risk were associated with higher intakes of vitamin C and carotenoids, especially xanthophylls, reflecting higher intakes by PD cases of fruit and certain vegetables. Intakes of sweet foods, including fruit, were associated with higher PD risk, suggesting that the observed trends may be due to a preference for sweet foods. This study does not provide support for a protective effect of long‐term dietary antioxidant intake on PD risk.
A shortened version of the motor section of the unified Huntington's disease rating scale
Authors: Siesling S. , Zwinderman A. H. , van Vugt J. P. P. , Kieburtz K. , Roos R. A. C.
Published: May 2005

Objective assessment of clinical findings of patients with Huntington's disease (HD) is necessary for an evaluation of the individual progression of the disease and the effect of therapy, and it requires specific assessment scales. The Unified Huntington's Disease Rating Scale (UHDRS) is an overall scale to assess clinical performance and functional capacity. In the course of carrying out studies in HD, several items in the motor function section were found to be difficult to score, had high cognitive loading, and appeared to be redundant. The objective of this study was to shorten the motor section of the UHDRS to the smallest number of items, without loss of internal consistency, while still assessing the important clinical features of HD. Shortening the total motor score of the UHDRS was carried out on the UHDRS data set of the Leiden University Hospital using four methods. The first two methods employed principal‐component analysis with Varimax rotation. Strongly interrelated test items were uncovered, resulting in a reduction of test items to a smaller set. The third method reduced items so that the internal consistency (Cronbach's alpha) was maximal. The fourth method omitted items subjectively. The results of the Dutch data set were validated on follow‐up data and on a data set from the University of Rochester Medical Center, New York. This study determined that the number of items in the motor function section could be reduced from 31 to 15. The reduced set maintains the relationships between the motor and other sections of the UHDRS and still assesses the major clinical features of HD.
Freezing phenomenon in patients with parkinsonian syndromes
Authors: Giladi Nir , Kao Richard , Fahn Stanley
Published: May 2005

Freezing is a common symptom in parkinsonian syndromes, but its association with different causes of parkinsonism as well as with other symptoms has never been investigated. We conducted a database survey of the occurrence of freezing in parkinsonism. Of 347 patients with a clinical diagnosis of parkinsonism other than idiopathic Parkinson's disease and with specific data regarding freezing, 158 patients had freezing (46%). Freezing was significantly associated with progression of the disease as rated on the Hoehn & Yahr scale [odds ratio (OR), 1.69; p < 0.004]. Gender was not a risk factor for the development of freezing. Patients with drug‐induced parkinsonism were at a very low risk for developing freezing (p < 0.00001; OR, 0.1). Freezing was found in a high frequency in patients with vascular parkinsonism (57%), normal‐pressure hydrocephalus (56%), and generally in teh group of patients who had parkinsonism resulting from neurodegenerative disease (progressive supranuclear palsy, multiple system atrophy, and corticobasal ganglionic degeneration; 45%). Freezing was significantly associated with the presence of dementia, incontinence, and tachyphemia (OR, 2.01, 1.7, and 5.09, respectively).
Short‐term effects of behavioral treatment on movement initiation and postural control in Parkinson's disease: A controlled clinical study
Authors: Müller Viktor , Mohr Bettina , Rosin Regina , Pulvermüller Friedemann , Müller Friedemann , Birbaumer Niels
Published: May 2005

In a controlled clinical study, we investigated the effects of behavioral treatment on postural and gait initiation problems in idiopathic Parkinson's disease (PD). Comparable groups of patients received behavioral therapy (experimental group, n = 15) and nonspecific psychological treatment (control group, n = 14) for 10 weeks. We monitored various variables reflecting properties of posture and gait initiation by using an optoelectronic motion analyzer (electronic movement analysis system, ELITE). A clinical blind to group membership of the patients assessed PD severity with the Unified Parkinson's Disease Rating Scale (UPDRS) before and after the treatment period. ELITE measures of postural stability and movement initiation revealed treatment‐specific effects. In addition, UPDRS motor scores showed significant improvement only after behavioral treatment. We conclude that behavioral treatment in Parkinson's disease may improve motor disabilities in moderately advanced PD patients.
Torticollis due to disinhibition of the vestibulo‐collic reflex in a patient with Steele‐Richardson‐Olszewski syndrome
Authors: Bisdorff A. R. , Bronstein A. M. , Wolsley C. , Lees A. J.
Published: May 2005

A patient with the clinical picture of Steele‐Richardson‐Olszewski syndrome and an unusual intermittent neck twisting is reported. He had virtually no voluntary ocular movements and only very slow, low‐amplitude voluntary head movements. However, in response to optokinetic or vestibular stimulation, he developed full eye deviations in the direction of the slow phase of the expected nystagmus. No quick phases were observed, and the deviation outlasted the duration of the vestibular stimuli because of defective saccades. The head also turned fully during these stimuli, quicker than on attempted voluntary movements, and remained derivated similarly to the eyes. This suggests that the neck deviations in this patient were due to a disinhibited vestibulo‐collic reflex and a disturbed head position resetting mechanism. Neck electromyographic responses in response to whole‐body rotation indicated that the vestibulocollic reflex responsible for the torticollis in this patient had a short latency of ∼ 30 ms.
Effects of thalamic deep brain stimulation based on tremor type and diagnosis
Authors: Hubble J. P. , Busenbark K. L. , Wilkinson S. , Pahwa Rajesh , Paulson George W. , Lyons K. , Koller W. C.
Published: May 2005

It has been suggested that deep brain stimulation (DBS) is less effective in alleviating proximal than distal postural arm tremor and tremor reduction is said to be less in essential tremor (ET) than in Parkinson's disease (PD). We analyzed blinded rater's tremor scores and subjects' disability ratings at 3‐month follow‐up to examine the effects of DBS based on tremor type (rest, kinetic, distal postural, proximal postural) and diagnosis (ET, PD). An independent examiner provided tremor scores using randomized videotaped footage of 19 ET and 10 PD subjects at baseline and at follow‐up with DBS “on.” Subjects provided self‐ratings of disability at baseline and at follow‐up. Comparisons of baseline and follow‐up tremor scores and disability ratings were made using the Mann–Whitney U and Wilcoxon rank sum W test; correlation analyses were performed using Spearman rank order correlation test. There were significant and essentially equal improvements in tremor scores for rest, kinetic, distal postural, and proximal postural tremor at follow‐up. Only one subject had no improvement in tremor. Tremor improved significantly and to the same extent in ET and PD subjects in each position except “at rest,” which was most improved in PD (p = 0.0003). ET and PD subjects did not differ in the extent of disability improvement. Improved disability correlated only with improved postural tremor scores: proximal postural and distal postural (r = 0.41, p = 0.03; r = 0.47, p = 0.01). DBS is effective in alleviating tremor and disability in both ET and PD. Resting, kinetic, distal postural, and proximal postural tremor can be reduced to an equal degree. However, DBS produces the greates improvement in disability in association with improved postural tremor in both ET and PD.
Reflex myoclonus in cortical‐basal ganglionic degeneration involves a transcortical pathway
Authors: Strafella A. , Ashby P. , Lang A. E.
Published: May 2005

The short‐latency reflex myoclonus that appears to be characteristic of cortical‐basal ganglionic degeneration (CBGD) was investigated in two patients. Stimulating the digital nerves of the middle finger caused exaggerated reflex activity in the first dorsal interosseus (FDI) muscle of that hand with a latency of 46–51 ms. Magnetic stimulation over the contralateral cortex, delivered 25 ms after the digital nerve stimulus, resulted in greater than expected facilitation of FDI, implying spatial summation. Poststimulus time histograms (PSTH) of individual FDI motor units indicated that this spatial summation was occurring “upstream” from the motoneurons. It is argued that this occurs at the motor cortex. Magnetic stimulation over the cortex in normal subjects results in short‐latency facilitation of the contralateral motoneurons followed by inhibition. This inhibition was less in the patients with CBGD. It is argued that this results from the loss of an intrinsic cortical or corticothalamic inhibitory mechanism.
Familial cortical myoclonic tremor as a unique form of cortical reflex myoclonus
Authors: Terada Kiyohito , Ikeda Akio , Mima Tatsuya , Kimura Kaku , Nagahama Yasuhiro , Kamioka Yu , Murone Lkuo , Kimura Jun , Shibasaki Hiroshi
Published: May 2005

Previously it was reported that some patients with tremor had the same electrophysiological findings as those seen in patients with cortical reflex myoclonus, and consequently the tremor was named “cortical tremor”. In the present study, we examined six patients from three families with cortical tremor of relatively late onset. The inheritance pattern of cortical tremor was compatible with autosomal dominant trait. Those patients had relatively rhythmic involuntary movements (tremor) in the distal upper and lower limbs, especially during posture and/or fine movements. There was no cerebellar ataxia or dementia, and fits of loss of consciousness occurred only infrequently. Electrophysiologically, they had generalized spikes on electroencephalogram (EEG), giant cortical components of somatosensory evoked potential, an enhanced long‐loop reflex (C‐reflex), and cortical spikes preceding the rhythmic jerk demonstrable by the jerk‐locked back averaging method, thus fulfilling the criteria of cortical reflex myoclonus. Furthermore, they had normal slow negative EEG shift starting 1–2 s prior to voluntary movements, suggesting that, as opposed to the conventional form of progressive myoclonus epilepsy (PME), the cerebellar efferent input to the motor cortices was normal. These clinical and electrophysiological pictures are distinct from those of familial essential tremor, familial essential myoclonus, or the conventional form of PME, and the term “familial cortical myoclonic tremor” will represent the clinical and electrophysiological features of this unique entity most appropriately.
Clonic perseveration following thalamofrontal disconnection: A distinctive movement disorder
Authors: Fung Victor S. C. , Morris John G. L. , Leicester Jonathan , Soo Y. S. , Davies Llewelyn
Published: May 2005

We describe four patients who developed asymmetrical, rhythmic, stereotyped, and repetitive movements of the upper and lower limbs hours to days after infarction that involved the thalamus and/or basal ganglia. The movements appeared to occur spontaneously and were initially labeled as focal motor seizures, ballism, or tremor; they could, however, be induced by passive movement of the limbs. The movements most commonly observed were scratching or rubbing movements of the hands that were of such persistence as to cause trauma to the skin; in the lower limbs, the heel was run up and down the bed sheet, often until it bled. The movements were part of a syndrome characterised initially by a reduced level of consciousness and followed by aspontaneity, usually with mutism and frontal release signs. One patient who had relatively preserved cognition and language repeated words or phrases again and again when encouraged to speak, but had no difficulty changing responses appropriately to different cues. In drawing, he overwrote each figure but could change the figure on command. The distinctive movement disorder in these patients was due to clonic perseveration. We suggest that clonic perseveration results from disconnection of prefrontal cortico–basal ganglia–thalamo–cortical loops that are important for the termination of motor plans. Clonic perseveration should be recognised as a movement disorder following thalamic lesions.
Dopamine agonist treatment of tourette disorder in children: Results of an open‐label trial of pergolide
Authors: Lipinski Joseph F. , Sallee Floyd R. , Jackson Cherry , Sethuraman Gopalan
Published: May 2005

This exploratory study was meant to determine the effect of the dopamine (DA) agonist pergolide on Gilles de la Tourette syndrome (GTS) in children and adolescents and to ascertain correlates of pergolide response. Thirty‐two outpatients, aged 7–19 years, were systematically assessed in a neuropsychiatric clinic for the presence of GTS and comorbid disorders. After a 6‐week open‐label, fixed‐flexible dosing schedule, response to pergolide on standard GTS severity outcome measures was assessed. Overall, 75% of patients (24/32) had a >50% drop in their tic severity rating from baseline with a mean treatment dosage of 177 ± 61 μg/day. Highly significant (p = 0.0001) baseline to week 6 differences were demonstrated in all tic symptom measures. The presence of restless legs syndrome (RLS) comorbidity (59%) was highly associated with a positive response. These results suggest DA agonism as a strategy, and pergolide in particular, may be a practical form of therapy for GTS. Response predictors of patient comorbid RLS argue for its further study with regard to GTS.
G/A1947 polymorphism in catechol‐O‐methyltransferase (COMT) gene in Parkinson's disease
Authors: Xie T. , Ho S. L. , Li L. S. W. , Ma O. C. K.
Published: May 2005

High and low catechol‐O‐methyltransferase (COMT) activity is significantly determined by thermostability, which is caused by a valine/methionine108 polymorphism associated with polymorphic G/A1947 bases, in exon 4 of the COMT gene. Our allelic association study on this polymorphism did not find any statistically significant difference between our Chinese Parkinson's disease and that of control subjects. These results show that this polymorphism and hence the thermostability of COMT enzyme are not related to a risk of developing Parkinson's disease.
The emergin role of clozapine in the treatment of movement disorders
Authors: Factor Stewart A. , Friedman Joseph H.
Published: May 2005

Clozapine, the only commercially available atypical neuroleptic, is approved for the treatment of schizophrenic patients who are unresponsive to or intolerant of typical neuroleptics. It has an unusual pharmacologic profile compared with standard neuroleptics, and it follows that clinical response to this drug is also different. It has shattered the notion that a drug must be capable of inducing or worsening parkinsonism to be a potent antipsychotic. Based on these findings, it is being used increasingly by neurologists for psychiatric and nonpsychiatric problems in patients with movement disorders. The most common use for clozapine among neurologists is in the management of druginduced psychosis in Parkinson's disease (PD). This problem has been a source of increased morbidity and mortality in PD because of a lack of adequate therapeutic intervention. At this time, because of success in numerous open trials, with improvement of >80% of patients, clozapine therapy for psychosis in PD is becoming the standard of care. It also appears to be of value in the management of some motor features of PD, including tremors and dyskinesia and possibly even sensory symptoms such as akathisia and pain. The literature also suggests that clozapine may be of potential benefit in hyperkinetic movement disorders including essential tremor, Huntington's disease, and tardive dyskinesia. We review the current data concerning the use of clozapine in patients with these movement disorders and others.
Simultaneous MAO‐B and COMT inhibition in L‐dopa‐treated patients with parkinson's disease
Authors: Lyytinen Jukka , Kaakkola Seppo , Ahtila Sirpa , Tuomainen Päivi , Teräväinen Heikki
Published: May 2005

The effect of selegiline (L‐deprenyl) on plasma catecholamines, clinical response, and drug tolerability was studied in 13 patients with Parkinson's disease (PD) treated with L‐Dopa/benserazide and entacapone, a peripheral catechol‐O‐methyltransferase (COMT) inhibitor, in a placebo‐controlled double‐blind study. An L‐Dopa test was performed on 3 study days. The first study day was with L‐Dopa/benserazide only (control), the second after 14 days of treatment with 200 mg entacapone taken concomitantly with L‐Dopa/benserazide in combination with either selegiline (10 mg daily) or placebo. After a 2‐week washout period, selegiline and placebo treatments were switched, and the third study day was after 14 days of treatment. During the study days, clinical response was evaluated at 30‐min intervals for 6 h, by using the motor score of the Unified Parkinson's Disease Rating Scale (UPDRS). In addition, repeated blood pressure measurements were made, and plasma samples were taken for analysis of L‐Dopa, 3‐O‐methyldopa (3‐OMD), dihydroxyphenyl acetic acid (DOPAC), homovanillic acid (HVA), dopamine, nor‐adrenaline, and 3‐methoxy‐4‐hydroxyphenylethylene glycol (MHPG). Monoamine oxidase B (MAO‐B) and COMT enzyme activities were measured from platelets and erythrocytes, respectively. Entacapone improved the clinical response to L‐Dopa during both selegiline and placebo (p < 0.001) treatments. The improvement was more marked during combined selegiline and entacapone treatment than with entacapone alone (p < 0.01). Entacapone significantly increased plasma L‐Dopa and DOPAC levels and decreased plasma 3‐OMD and MHPG levels both with selegiline and placebo. Selegiline partially inhibited the entacapone‐induced increase of plasma DOPAC. Plasma dopamine and noradrenaline levels did not change. Entacapone decreased erythrocyte COMT activity by >35% (p < 0.001), and platelet MAO‐B activity was almost completely inhibited by selegiline (p < 0.001). One patient withdrew because of diarrhea, dizziness, and loss of sleep when receiving selegiline treatment. Otherwise no differences in adverse events, mean daily blood pressures, or other safety parameters were observed between selegiline and placebo treatments. Our results suggest that entacapone can be safely administered together with L‐Dopa and selegiline in patients with PD, although further studies with larger number of patients and longer treatment periods are necessary to confirm this finding.
The “cold hands sign” in multiple system atrophy
Authors: Klein Christine , Brown Richard , Wenning Gregor , Quinn Niall
Published: May 2005

Patients with multiple system atrophy (MSA) often have cold, dusky, violaceous hands, with poor circulatory return after blanching by pressure. We therefore compared hand skin temperature in nine age‐matched subjects with probable MSA, 10 with idiopathic Parkinson's disease (PD), and 10 healthy controls both at rest and following a standard cooling procedure.

Baseline skin temperature in the MSA group was significantly lower than in both the PD and control groups, and four of the nine MSA patients were colder at baseline than the coldest control value. After cooling, the MSA group also showed a significantly greater reduction in skin temperature than the PD group. The rate of skin temperature recovery did not differ between the three groups, although the MSA group required more time to return completely to their baseline temperature.

Changes in skin colour or temperature are easily detected, and suggest a defect in neurovascular control of distal extremities. The “cold hands sign” is another clinical “red flag” that helps to raise the suspicion of MSA.
Vomiting and retching in gilles de la tourette syndrome: A report of ten cases and a review of the literature
Authors: Rickards Hugh , Robertson Mary M.
Published: May 2005

Retching and vomiting are common symptoms in childhood. We describe the cases of 10 patients with Gilles de la Tourette syndrome (GTS) for whom vomiting or retching tics were part of the clinical picture, and discuss other instances where retching and vomiting occur with neuropsychiatric or movement disorders.
Saccades in gilles de la tourette's syndrome
Authors: Straube Andreas , Mennicken Jan‐Baldem , Riedel Michael , Eggert Thomas , Mülller Norbert
Published: May 2005

Gilles de la Tourette's syndrome (GTS) is presumed to be an inherited disorder with an unclear pathophysiology. An involvement of the basal ganglia is suspected. Besides vocal tics, one of the main symptoms is the presence of motor tics. As eye movements are a specialized part of the motor system, we investigated whether they differed in some typical way in GTS patients. To study the control of saccades in GTS, different paradigms were used to elicit saccades, which were either externally triggered and visually guided or internally triggered and without visual target. GTS patients (n = 10) showed a significant increase of the latency of antisaccades, a highly impaired performance of sequences of memory‐guided saccades, and an isolated reduction of the peak velocity in the antisaccades. Overall the results were similar to those found with similar paradigms in patients with Huntington's disease (HD). In analogy to the known pathology of HD, these findings can be attributed to ascending loops from the basal ganglia that inappropriately activate the frontal cortex, especially some of the eye movement‐related areas there. This impairment seems to be a rather specific effect, since some oculomotor features that are preserved by structures of the frontal cortex, such as the effect of a fixation target on saccadic latency (“gap effect”), were normal.
Hand tremor in patients with spasmodic torticollis
Authors: Deuschl Günther , Heinen Florian , Guschlbauer Brigitte , Schneider Sybilla , Glocker Franz X. , Lücking Carl H.
Published: May 2005

The occurrence of hand tremors in patients with spasmodic torticollis (ST) was investigated in 55 patients by clinical and quantitative measurements. Ten patients had first‐order or second‐order relatives affected with postural tremor. Although 40% of the patients had a medical history and clinical findings for mild postural and action tremor of the hands, only four had moderate tremor amplitudes. One patient had a severe tremor, including resting tremor, and two had mild voice tremor. A positive correlation was found between hand and head tremor. Patients with hand tremor were younger at the onset of ST than were those without. The mean amplitudes of postural tremor were only slightly higher than for the controls and much smaller than those found in classic essential tremor. The tremor caused only mild disabilities. The tremor frequencies were indistinguishable from physiologic tremor. Further analysis of the tremor records showed evidence for physiologic tremor mechanisms only. We conclude that slightly enhanced postural hand tremors are common in ST but have a low amplitude and are only rarely clinically relevant for ST patients. Although the present data support the notion of an enhanced risk of postural tremor in families of patients affected with ST, none of the criteria allowed the separation of the hand tremor of ST from other postural/action tremors and especially from enhanced physiologic tremor. Thus, the present data do not support the classification of hand tremor in ST as either “dystonic” or essential tremor.
Lateralization of the p22/n30 component of somatosensory evoked potentials of the median nerve in patients with cervical dystonia
Authors: Kǎovský Petr , Streitová Hana , Dufek Jaroslav , Rektor Ivan
Published: May 2005

Somatosensory evoked potentials (SEPs) of the median nerve were recorded in 40 patients suffering from cervical dystonia and in 40 healthy volunteers as a control. Before recording the median nerve SEPs, polymyographic recordings were performed in all patients with cervical dystonia. The activity of cervical muscles was recorded, and the leading muscle of cervical dystonia was determined. Patients were divided into two groups according to the results of polymyography. The leading muscle was sternocleidomastoid in the first group and the splenius capitis in the second group. Patient SEPs were recorded during abnormal head rotation. SEPs in 20 healthy volunteers were recorded with the head in the middle position. SEPs of another 20 healthy volunteers were recorded with the head rotated 60° to the right. The mean peak‐to‐peak amplitude values of the precentral P22/N30 complex and the mean ratio of the P22/N30 amplitudes between both hemispheres were calculated in the F3 (F4) and C3′ (C4′) electrode positions in all four groups. No significant lateralization of the precentral P22/N30 component was found in either group of healthy volunteers. In dystonic patients in whom the sternocleidomastoid was determined as the leading muscle of dystonia, a statistically significant lateralization of the P22/N30 component toward the ipsilateral side of the leading muscle was found. In the group with the splenius capitis determined as the leading muscle of dystonia, a statistically significant lateralization of the P22/N30 component toward the contralateral side of the leading muscle was found. The possibility that the precentral cortex is activated differently in cervical dystonia patients who have different muscle patterns of dystonia is discussed.
Neurophysiological investigations in patients with head tremor
Authors: Valls‐Solé Josep , Tolosa Eduardo S. , Nobbe Fritz , Diéguez Elena , Mun̄oz Esteban , Sanz Pilar , Valldeoriola Francesc
Published: May 2005

We studied 30 patients whose primary complaint was head tremor in an attempt to characterize neurophysiological aspects of their abnormal movement. Based on family medical history and physical examination, 23 patients had definite or probable essential tremor (essential head tremor, EHT). The remaining seven had mild dystonic signs accompanying their head tremor (head tremor plus dystonic signs, HT + DS). We recorded head movement and the electromyographic (EMG) activity of the sternomastoid and splenius capitis muscles, determined the spontaneous blinking rate, and measured the excitability recovery curve of the blink reflex and of the masseteric inhibitory reflex. All patients had tremor bursts at a frequency ranging between 3 and 9 Hz in at least one of the muscles examined. The predominant pattern seen when patients were sitting relaxed and facing forward was that of synchronized EMG bursts in both splenius capitis muscles. Maintenance of extreme head postures demonstrated two types of additional abnormalities: type 1 (enhancement of tremor), which was observed in 11 patients (47.8%) with EHT and in two (28.5%) with HT + DS; and type 2 (activation of neck muscles not required for maintenance of the posture), which was observed in two patients (8.7%) with EHT and in five (71.5%) with HT + DS (X2 = 26.4; p < 0.001). Mean blinking rate per minute was 24.9 ± 14.6 in patients with EHT and 42.3 ± 10.5 in patients with HT + DS (paired t test, p = 0.001). The blink reflex and masseteric inhibitory reflex excitability recovery curves showed an abnormal interneuronal excitability enhancement in seven (30.4%) of the 23 patients with EHT and in two (28.5%) of the seven with HT + DS (X2 = 3.1; p > 0.05). Abnormal patterns of EMG activity of the neck muscles correlated well with the presence of mild dystonic signs. However, the analysis of brainstem interneuronal excitability did not enable recognition of those patients with head tremor who could potentially develop cervical dystonia. The enhancement of brainstem interneuronal excitability found in −30% of patients with head tremor could be related to plastic changes triggered by increased activity of the cranial muscles.
Performance of sequential arm movements with and without advance knowledge of motor pathways in Parkinson's disease
Authors: Currà Antonio , Berardelli Alfredo , Agostino Rocco , Modugno Nicola , Puorger Cristiana Conti , Accornero Neri , Manfredi Mario
Published: May 2005

Patients with Parkinson's disease are slower than normal subjects in executing sequential arm movements, and their bradykinesia worsens as the execution of motor sequences progresses. In parkinsonian and normal subjects, we studied the execution of two types of fast sequential arm movements. The subjects had to perform a motor sequence following with their arm a path marked by six targets on a screen In one experimental condition, they performed the motor sequence without advance knowledge of its path and executed each submovement in response to the consecutive appearance of the targets on the screen (unknown motor sequences). In the other condition, all the targets appeared simultaneously on the screen before the subject moved, and the subject internally determined when to execute each submovement (known motor sequences). Patients were slower than normal subjects in executing both sequences. Patients and normal subjects were faster in executing known than unknown sequences, but the patients' percentage of total movement time diminished less. During the unknown condition, both groups tended to lengthen submovement duration whereas, during the known condition, both groups tended to correct this trend. Patients performed submovements during both sequences with longer acceleration phases. The submovement symmetry ratio of the velocity profile changed according to the direction of movement (up or down). We conclude that these findings depend mainly on the mode of movement execution. They also suggest that patients with Parkinson's disease have more difficulty in executing internally determined than externally triggered sequential movements.
Chorea in fifty consecutive patients with rheumatic fever
Authors: Cardoso Francisco , Eduardo Carlos , Silva A. P. , Mota Cleonice C. C.
Published: May 2005

Rheumatic fever (RF) remains a significant public health problem in developing countries. Although its incidence has been declining in developed areas, several outbreaks of RF have been identified in the United States since 1985. To study the prevalence and clinical features of Sydenham's chorea (SC), a well‐recognized expression of RF, we examined 50 consecutive patients with RF seen at our Pediatric Cardiology Clinic. Chorea was rated on a scale of 0‐‐4(0 = no chorea; and 4 = severe chorea, patient unable to walk). Chorea, active or in remission, occurred in 13 patients (26%). The male‐to‐female ratio was 1.08 among the total population and 0.86 among the SC group. The mean age of the 50 patients at onset of RF was 8.4 years and the standard deviation (SD) 3.1 years. Carditis was diagnosed in 84% of patients with and without chorea, but arthritis was more common in the latter group (83.8%) than in the SC group (30.8%). The mean age at onset of SC was 9.2 years (SD 2.7). Involuntary movement, noticed by nine patients, was the most common initial symptom. Chorea went into remission in eight patients after 9.9 months (SD 13.4). Chorea was rated 3 in ten patients, 1 in two patients, and 4 in one patient. Among the five patients with active SC, three had hemichorea and two had generalized chorea. We recognized abnormal saccades in 80% of patients with active chorea, whereas just 13.5% of patients without chorea displayed this abnormality. SC remains an important cause of chorea in countries plagued by RF.
Perception of spatial orientation in spasmodic torticollis. Part 2: The visual vertical
Authors: Anastasopoulos D. , Bhatia K. , Bronstein A. M. , Marsden C. D. , Gresty M. A.
Published: May 2005

Twenty‐nine patients with idiopathic spasmodic torticollis (ST) and matched normal control subjects were asked to align a target line to perceived earth vertical [visual vertical (VV)]. Settings were made against a whole‐field random‐dot background that was either stationary or rotating around the line of sight, and subjects performed the task upright and lying horizontally on their sides. Normal subjects were tested both head upright and after assuming a voluntary head tilt. Patients with ST set the VV close to true upright with a minimal deviation toward tilt of the head in contrast to normal subjects assuming a head tilt who set the VV in the opposite direction to the head tilt (Müller ‘E’ effect). Settings against the spinning disk were biased in the direction of rotation similarly for both subject groups. Settings made against static or spinning disk performed when subjects lay horizontally were tilted in the direction of recumbence (Aubert ‘A’ effect) similarly for both subject groups. When attempting to set the target line parallel to the long axis of the face, patients with head tilt set the line to upright, whereas normal subjects correctly estimated their tilts. One hypothesis offered to explain these results is that the patients referenced only their upright trunk for vertical and did not make use of neck proprioceptive or vestibular signals of head tilt so that all settings were made as if the trunk and head were upright. Alternatively, patients may have used only otolith signals as the reference for upright, and these are recalibrated in ST patients with head tilt. The pathological deviation becomes accepted as upright posture, and VVs and facial orientation are estimated as if the head were upright. In either case the findings indicate abnormal processing of the perception of visual verticality in ST patients.
Severe constipation in parkinson's disease relieved by botulinum toxin
Authors: Albanese Alberto , Maria Giorgio , Bentivoglio Anna Rita , Brisinda Giuseppe , Cassetta Emanuele , Tonali Pietro
Published: May 2005

A parkinsonian patient with severe outlet‐type constipation was treated with injection of botulinum toxin into the puborectalis muscle. A total of 30 units (Botox) was injected in two sites. Resting anal pressure, maximum voluntary contraction, and pressure on straining were evaluated before treatment and 4, 8, 12, and 16 weeks afterward. Pressure values declined following treatment, the decline of pressure on straining ending by week 12. Proctography performed 8 weeks after treatment showed improvement in the anorectal angle and evacuation of barium paste. The clinical benefit lasted for ˜12 weeks. The present data show that botulinum toxin is a promising tool for treating outlet‐type constipation in Parkinson's disease.
BotB (botulinum Toxin type B): Evaluation of safety and tolerability in botulinum toxin type A‐Resistant cervical dystonia patients (preliminary study)
Authors: Truong Daniel D. , Cullis Paul A. , O'Brien Christopher F. , Koller Martin , Villegas Timothy P. , Wallace Jan D.
Published: May 2005

Botulinum toxin (BTX) injection is considered the treatment of choice for patients with cervical dystonia (torticollis). We conducted a pilot, open‐label, dose‐escalation study with BTX type B in 12 patients who no longer responded clinically to injections with BTX type A. At the doses tested, BTX type B was safe and well tolerated without evidence of dose‐limiting toxicity in this patient population. Mild‐to‐moderate adverse events generally resolved quickly and included asthenia, pain, nausea, dysphagia, hypertonia, and tremor. No serious adverse events or antibodies to type‐B treatment were reported. Low‐dosing‐session (100‐‐899 units) and high‐dosing‐session (900–1,500 units) groups were defined based on units administered per dosing session. Toronto Western Spasmodic Torticollis Rating Scale‐Severity Scale (TWSTRS‐Severity), Patient Analogue Pain Scale, and Physician and Patient Global Assessment Scales were measured during this study. The TWSTRS‐Severity mean maximum percent improvement from baseline demonstrated a 9.9% versus 28.8% difference between the low‐dose and high‐dose groups, respectively. Effectiveness was noted for the high‐dose group on the Patient Analogue Pain Scale but not on the Global Assessment Scales.
Cervical and facial myoclonus associated with dolichoectasia of the left vertebral artery
Authors: Munñoz Esteban J. , Vila Nicholas , Valls‐Solé Joseph , Tolosa Eduardo
Published: May 2005

A 52‐year‐old man presented with rapid jerking movements of his head to the right and clonic involuntary twitches involving his left eyelid and cheek. Electromyographic (EMG) recordings were consistent with left sternocleidomastoid and facial asynchronous myoclonus. Magnetic resonance imaging showed the presence of a dolichoectatic left vertebral artery displacing the medulla. Facial involvement and the asynchronism between cervical and facial myoclonus did not support a medullary origin for the movements. A direct compression of the eleventh and seventh cranial nerves by the dolichoectatic left vertebral artery may be the mechanism responsible for myoclonus in our patient.
Tremor in latin texts of dutch physicians: 16th‐18th centuries
Authors: Koehler Peter J. , Keyser Antoine
Published: May 2005

In his monographAn essay on the shaking palsy(1817), James Parkinson mentioned tremor and propulsion to be the most important signs of the disease that he was describing. In this article, we study aspects of the history of one of the signs that he mentioned, that is,tremorand see how the meaning of this term evolved since its description by Galen, particularly in the period from the 16th to the 18th centuries. We'll pay attention to the development of a distinction made between action tremor and rest tremor. Work by the following authors is covered: Pratensis and Forestus (16th century); Tulp, Van Beverwijck, and Sylvius (17th century); and Boerhaave and Van Swieten (18th century). Not all authors made the distinction, originally noticed by Galen, between action tremor and rest tremor. Parkinson tremor probably was observed but was classified among the tremors of the elderly. The meaning ofpalpitationchanged through the ages and finally was applied only to pathologic heart and artery pulsations. Sylvius and Van Swieten were the only authors in this study who clearly distinguished between action tremor and rest tremor. They are discussed in Parkinson's monograph.
Postprandial hypotension and parkinsonian state in parkinson's disease
Authors: Chaudhuri K. Ray , Ellis Catherine , Love‐Jones Sarah , Thomaides Thomas , Clift Susan , Mathias Christopher J. , Parkes J. David
Published: May 2005

Abnormal postprandial cardiovascular responses such as postprandial hypotension (PPH) occur in primary autonomic failure and contribute significantly to morbidity. The extent and frequency of PPH and its relationship to the parkinsonian state in idiopathic Parkinson's disease (IPD) is unknown. By studying 20 patients with IPD (without autonomic failure) and 16 age‐matched controls after both groups ingested a standard isocaloric balanced liquid meal we have shown that supine PPH complicates IPD and is reduced to marked worsening of the parkinsonian state as measured by a cumulative score of tremor, rigidity, bradykinesia, posture, and gait, Furthermore, significant postural hypotension is unmasked that results in posturnal intolerance due to presyncopal symptoms. Our study in posturnal intolerance due to presyncopal symptoms. Our study indicates that, in patients with IPD, ingestion of a meal may lead to abnormal postprandial cardiovascular responsea and aggravation of the parkinsonian state. The underlying mechanisms are unclear, although vasodilatory gut peptides released in response to food ingestion may be contributory.