bluuebunny/crossref_metadata_2025 huggingface dataset:
Effect of surfactants on the rate of diffusion‐controlled Cementation of copper from dilute solutions on a stationary zinc disc in stirred vessels
Authors: Zarraa Mahmoud A.
Published: December 2004

The effect of surfactants on the rate of diffusion‐controlled cementation of copper from dilute copper sulphate solutions on a stationary zinc disc in a stirred vessel was studied. Variables investigated were rotational speed of the impeller, concentration and type of surfactants and concentration of copper sulphate solution. These variables were studied for their effect on the mass transfer coefficient of copper cementation. The mass transfer coefficient was found to increase with increasing rotational speed of the impeller and concentration of copper sulphate solution. It was found that surfactants decrease the rate of mass transfer during cementation by an amount ranging from 9.5 to 25° depending on the rotational speed of the impeller and type of surfactant added. Increasing surfactant concentration was found to decrease the mass transfer coefficient. The ability of different surfactants to decrease the mass transfer coefficient increases in the order: CTMAB < SABS < NPPGE. Mass transfer data were correlated, in absence and in presence of surfactants, by the following equations respectively:

Where Sh is Sherwood number, Re is Reynolds number, and Sc is Schmidt number.
Zur Aussagefähigkeit der Almenintensität als Kennwert bei der gezielten Eigenspannungserzeugung durch Kugelstrahlen
Authors: Herzog R. , Zinn W. , Scholtes B. , Wohlfahrt H.
Published: December 2004

In der technischen Praxis werden Kugelstrahlbehandlungen üblicherweise durch die Angabe von Almenintensität, Überdeckung sowie von Art und Härte des Strahlmittels und Strahlgutes beschrieben. Dies ist jedoch im Hinblick auf die erzeugten randnahen Werkstoffeigenschaften nicht in allen Fällen eindeutig und ausreichend. Am Beispiel experimenteller Untersuchungen zum Einfluß der Almenintensität und des Strahlmitteldurchmessers auf die Eigenspannungsausbildung wird diese Problematik dargestellt und anhand energetischer Betrachtungen des Strahlprozesses erläutert und diskutiert.
Gilles de la tourette's syndrome in twins: Clinical and neurochemical data
Authors: Goetz Christopher G. , Tanner Caroline M.
Published: December 2004

A pair of identical twins (probability of monozygosity by genetic marking = 99.99%) with Gilles de la Tourette's syndrome (GTS) were studied clinically and neurochemically. The two boys were distinguished within the family by dramatic differences in their behavior and tics. Twin 1 had severe motor tics, loud vocalizations, coprolalia, and school behavior problems, whereas twin 2 was only mildly affected. Neither boy had ever been treated with medication for his tic disorder. Lumbar puncture was performed on each after a night of bed rest. In spite of the marked clinical differences between the two boys, the major indices of dopamine and serotonin turnover in the cerebrospinal fluid were equivalent. Homovanillic acid levels were 65 and 60 ng/ml, respectively, and 5‐hydroxyindoleacetic acid levels were 36 and 39 ng/ml. Observation of these twins suggests that the phenotypic expression of GTS relates to more factors than inheritance. Neurochemical metabolites of dopamine and serotonin did not reflect the comparative clinical severity of GTS in these boys.
Twin studies and the genetics of Parkinson's disease—a reappraisal
Authors: Johnson William G. , Hodge Susan E. , Duvoisin Roger
Published: December 2004

Parkinson's disease (PD) has long been regarded as having a hereditary component. However, three recent twin studies have been interpreted as excluding any significant genetic component in the etiology of PD. In this article, we reexamine these twin studies and argue that such a conclusion is premature. We review statistical tests of twin concordance rates, including calculation of G, the coefficient of genetic determination. When variation in a trait is due entirely to genetic factors G = 1, and when variation in a trait is due entirely to nongenetic factors G = 0. We conclude that: (a) low monozygotic concordance rates can be compatible with substantial genetic contribution to etiology; (b) the PD twin study data give substantial optimal values of G (up to 0.78) but the very broad 95% confidence limits for G make it impossible for the twin study data to prove or disprove a substantial genetic component to the etiology of PD; and (c) changing clinical concepts of PD have undermined the assumptions underlying the methodology of the PD twin studies. We review three genetic models that are biologically plausible for PD and are compatible with the low twin concordance rates. Finally, we suggest that further family studies, including linkage studies, are needed to resolve this question.
The metabolic anatomy of Parkinson's disease: Complementary [18F]fluorodeoxyglucose and [18F]fluorodopa positron emission tomographic studies
Authors: Eidelberg D. , Moeller J. R. , Dhawan V. , Sidtis J. J. , Ginos J. Z. , Strother S. C. , Cedarbaum J. , Greene P. , Fahn S. , Rottenberg D. A.
Published: December 2004

We studied the metabolic anatomy of typical Parkinson's disease (PD) using [18F]fluorodeoxyglucose (FDG) and [18F]fluorodopa (FDOPA) and positron emission tomography (PET). Fourteen PD patients (mean age 49 years) had FDG/PET scans, of which 11 were scanned with both FDOPA and FDG. After the injection of FDOPA, brain uptake and arterial plasma radioactivity were monitored for 2 h. Striatal FDOPA uptake was analyzed with regard to a two‐compartment model, and target‐to‐background ratios (TBRs) and TBR‐versus‐time slopes were also calculated. Regional patterns of metabolic covariation were extracted from FDG/PET data using the Scaled Subprofile Model (SSM). SSM pattern weights, FDOPA uptake constants (Ki), TBRs, and TBR slopes were correlated with clinical measures for bradykinesia, rigidity, tremor, gait disturbance, left‐right asymmetry, dementia, and overall disease severity. In PD patients, rate constants for FDOPA uptake correlated with individual measures of bradykinesia (p = 0.001) and gait disability (p < 0.05). SSM analysis revealed a distinct pattern of regional metabolic asymmetries, which correlated with motor asymmetries (p < 0.001) and left‐right differences in Ki (p < 0.01). Our data suggest that in PD patients, FDG/PET and FDOPA/PET may provide unique and complementary information about underlying disease processes.
Central motor conduction to hand and leg muscles in Huntington's disease
Authors: Hömberg V. , Lange H. W.
Published: December 2004

Using electromagnetic stimulation of motor cortex and cervical or lumbar roots, central conduction times to the thenar and abductor hallucis muscles bilaterally were determined in a population of 32 patients with definite Huntington's disease (HD) and 14 subjects at risk. The HD patients showed a wide variety of different severity of choreatic movements, disease duration, and total disability. None of the stimulation parameters (latency after cortical stimulation, amplitude, threshold, or central conduction time) revealed statistically significant abnormalities compared with a normal control group as well as between patient subgroups. The data indicate that central motor conduction to upper and lower extremity muscles remains normal in Huntington's disease irrespective of the severity of the disorder.
A controlled trial of piracetam in intellectually impaired patients with Parkinson's disease
Authors: Sano Mary , Stern Yaakov , Marder Karen , Mayeux Richard
Published: December 2004

Twenty patients with Parkinson's disease and marked intellectual impairment or dementia participated in a double‐blind placebo controlled trial of the nootropic, piracetam. A standardized neurological examination, a neuropsychological test battery, and a functional scale, The Sickness Impact Profile, were completed for all patients. They were then assigned by blind randomization to drug or placebo conditions receiving 3.2 g of piracetam or an identical amount of placebo for 12 weeks. The dose was increased to 4.8 g for an additional 12 weeks. Neurological, psychological, and functional measures were rated as improved, unchanged, or worsened in comparison to baseline performance. Twenty‐five percent of the patients did not complete the trial for reasons unrelated to the medication. Although there was a significant improvement on one subtest of the functional scale, no significant effects were demonstrated in cognitive or neurological measures.
Evaluation of a modified inverted walking stick as a treatment for Parkinsonian freezing episodes
Authors: Dietz Mark A. , Goetz Christopher G. , Stebbins Glenn T.
Published: December 2004

We evaluated a modified inverted walking stick [visual cue (VC) stick] in Parkinson's disease patients with freezing episodes. Patients underwent baseline trials on a 60‐ft track with four walking conditions: unassisted, with a straight walking stick, with the VC stick, and on the track with parallel lines spaced every 12 in. Patients completed three trials of each condition in a randomized order, with total course time and number of freezes recorded. Patients were given the VC stick to use at home until subjective maximum functional benefit was obtained and were then brought back for a follow‐up objective evaluation. Eight patients completed the study. The straight stick and VC stick worsened patient performance as a group, while the parallel lines significantly improved group performance. However, two of the eight patients showed benefit from the VC stick. Improvement with parallel lines did not predict improvement with the VC stick. On follow‐up trials, the two who initially showed improvement with the VC stick continued to show improvement, while none of the other patients showed additional benefit from a learning effect. We conclude that the VC stick will help a small subpopulation of Parkinson's disease patients with freezing, and this subpopulation can be identified with the initial office visit trial.
Adrenal to caudate transplantation—postmortem study
Authors: Waters C. H. , Itabashi H. H. , Apuzzo M. L. J. , Weiner L. P.
Published: December 2004

A patient with Parkinson's disease who had undergone bilateral adrenal to caudate implantation by stereotactic technique came to autopsy. The implantation was shown to have produced minimal trauma and to have been well placed. Minimal surviving adrenal medullary tissue remained in the implanted coil, and no outgrowth of tissue into the striatum occurred.
Sleep disorders and sleep effect in Parkinson's disease
Authors: Factor Stewart A. , McAlarney Terence , Sanchez‐Ramos Juan R. , Weiner William J.
Published: December 2004

It has been suggested that sleep may have a positive effect on morning motor symptoms in Parkinson's disease (PD). We examined this possibility and also looked at common sleep disorders in PD. Seventy‐eight PD patients and 43 normal elderly subjects answered a questionnaire. Of the PD patients, 43.6% reported improved motor symptoms in the morning, 37.2% worse, and 19.2% unchanged compared to the rest of the day. No difference was found between morning‐better and ‐worse groups with respect to age, duration or stage of PD; antiparkinsonian medications utilized, and predominant motor symptoms. However, the morning‐same group had a shorter duration of PD and less severe disease and required fewer dopaminergic medications. Sleep disorders were seen with equal frequency in the morning‐better and ‐worse groups. Our results suggest that sleep does not have a direct effect on morning motor function. Alterations in morning motor symptomatology probably represent a manifestation of motor fluctuations. Sleep fragmentation and spontaneous daytime dozing occurred much more frequently in PD patients than controls. In addition, nocturnal vocalizations and daytime hallucinations occurred only in the PD group.
Mitochondrial DNA analysis in Parkinson's disease
Authors: Schapira A. H. V. , Holt I. J. , Sweeney M. , Harding A. E. , Jenner P. , Marsden C. D.
Published: December 2004

The reduced form of nicotinamide adenine dinucleotide coenzyme Q reductase (complex I) activity has recently been shown to be deficient in the substantia nigra of patients dying with Parkinson's disease. This biochemical defect is identical to that produced by the neurotoxin 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP), which also produces parkinsonism in humans. Complex I comprises 25 polypeptides, seven of which are encoded by mitochondrial DNA. Restriction fragment analysis of substantia nigra DNA from six patients with Parkinson's disease did not show any major deletion. In two cases, there were different novel polymorphisms that were not observed in control brain (n = 6) or blood (n = 34) samples.
Spontaneous orofacial dyskinesias in a captive cynomolgus monkey: Implications for tardive dyskinesia
Authors: Rupniak N. M. J. , Tye S. J. , Steventon M. J. , Boyce S. , Iversen S. D.
Published: December 2004

We describe a syndrome of spontaneous orofacial dyskinesias and cage stereotypies in a singly housed adult cynomolgus monkey never previously exposed to neuroleptic drugs. Abnormal movements were readily suppressed by acute treatment with haloperidol (0.03–0.24 mg/kg i.m.) or SCH23390 (0.05–0.2 mg/kg i.m.) but not by physostigmine (0.005–0.04 mg/kg i.m.) or scopolamine (0.0025–0.04 mg/kg i.m.). The symptomatology and response to pharmacological manipulations was indistinguishable from that previously attributed to chronic neuroleptic treatment in primates. Our findings indicate that neuroleptic‐induced tardive dyskinesias in most primate studies have not been clearly demonstrated.
Prevalence of dystonia in veterans on chronic antipsychotic therapy
Authors: Sethi Kapil D. , Hess David C. , Harp Rollie J.
Published: December 2004

The prevalence of dystonia was studied in 125 veterans on chronic antipsychotic therapy using a detailed and systematic examination. Twenty‐seven out of 125 had dystonic manifestations. The most common areas involved were hands and jaw. There was no relation between the presence or absence of dystonia, and duration of neuroleptic therapy. There was a tendency for tardive akathisia to occur more frequently in patients with dystonia than in those without it (Fisher's exact probability test, p = 0.0656). Tardive dystonia in its milder forms may be more common than currently believed.
Involvement of corticospinal tract in Wilson's disease. A study of three cases with transcranial stimulation
Authors: Berardelli A , Inghilleri M. , Priori A. , Thompson P. D. , Fabri S. , Fieschi C. , Manfredi M.
Published: December 2004

Muscle responses evoked by transcranial stimulation were studied in three patients with Wilson's disease. Abnormalities indicating involvement of corticospinal tract were demonstrated in one patient. In keeping with recent evoked potential studies, this finding suggests that lesions in Wilson's disease may affect structures other than the extrapyramidal system.
Neuronal inclusions of Parkinson's disease
Authors: Gibb W. R. G. , Scott T. , Lees A. J.
Published: December 2004

Two distinct neuronal inclusions occur in Parkinson's disease. The Lewy body is the diagnostic hallmark and is recognized by its eosinophilic body and unstained halo. It can be found in specific regions of the nervous system, where its frequency, size, shape, and structure differ. Large neurons of the dorsal vagal nucleus and sympathetic ganglia often contain particularly large quantities of Lewy‐body‐like matter. It consists of filament in the outer part and electron dense material in the core, the outer part staining with silver and with antibodies to neurofilament and tubulin. The pale body is restricted to the substantia nigra and locus ceruleus. It does not react with conventional stains, silver, or neurofilament antibodies, and has a homogeneous structure with a granular and vesicular surface texture. It contains sparse granular matter, vacuoles, and filaments, surrounded by melanin. The Lewy body and pale body may be juxtaposed or contiguous in some cells, but their distinct appearances and structures indicate that they are separate inclusions.
Early‐onset Parkinson's disease
Authors: Giovannini P. , Piccolo I. , Genitrini S. , Soliveri P. , Girotti F. , Geminiani G. , Scigliano G. , Caraceni T.
Published: December 2004

The study was conducted on 120 patients (76 men and 44 women) affected by idiopathic Parkinson's disease (IPD) responsive to L‐dopa and observed for many years. Sixty had clinical onset between the ages of 20–40, representing 10.2% of our PD population; in the others the symptoms began after the 40th birthday. The two groups were matched for sex and length of illness. In all patients a diagnosis of IPD depended on history and clinical and neuroradiological findings. Clinical, pharmacological, evolutive, and epidemiological data were collected on all patients. Thirty‐six patients from each group performed motor dexterity tests (reaction time to expected and unexpected stimuli) and cognitive tests (Wechsler Adult Intelligence Scale, Benton, Short tale, and Zazzo's speed and accuracy test). To assess the prevalence of dementia and the severity of psychiatric side effects of L‐dopa administration, the 60 patients with early‐onset PD were compared with 134 consecutive unselected PD patients. Five percent of early‐onset PD patients had a family history of the disorder. Our study showed that early‐onset PD does not differ fundamentally from the late‐onset form except that the former is characterized by a more rapid establishment of the full‐blown parkinsonian clinical picture and deterioration of the therapeutic efficacy of L‐dopa, with an earlier appearance of side effects. The results of our neuropsychological investigation suggest that early‐onset PD may be a “pure” form of extrapyramidal compromise with exclusively motor manifestations.
Paroxysmal axial spasms of spinal origin
Authors: Brown P. , Thompson P. D. , Rothwell J. C. , Day B. L. , Marsden C. D.
Published: December 2004

A 55‐year‐old man with a 4½‐year history of paroxysmal bouts of involuntary large‐amplitude flexion jerks of the trunk and a 1‐year history of persistent rhythmic small‐amplitude movements of the anterior abdominal wall is described. Clinical and electrophysiological evidence suggested that the paroxysmal myoclonus arose in propriospinal systems intrinsic to the spinal cord.
Motor and sensory evoked potentials in progressive supranuclear palsy
Authors: Abbruzzese G. , Tabaton M. , Morena M. , Dall'Agata D. , Favale E.
Published: December 2004

Motor potentials evoked in the biceps, thenar, and tibialis anterior muscles by electrical stimulation of the scalp and of the spinal regions were recorded in 12 patients with progressive supranuclear palsy (PSP) and in a control group. Abnormalities of central motor conduction for at least one muscle were present in five patients (41.7%), characterized by a long illness duration. The central sensory conduction time of the median nerve was normal, but five patients showed a depressed frontal N30 wave. These findings support the possible occurrence of functional damage to the corticospinal tracts and to the supplementary motor area in PSP.
Writer's cramp: Treatment with botulinum toxin injections
Authors: Rivest J. , Lees A. J. , Marsden C. D.
Published: December 2004

Twelve patients with writer's cramp were treated with injections of botulinum toxin. The overactive muscles were identified by clinical observation of the subjects while they were writing. Repeated injections were given at 2‐week intervals until the optimal response was obtained. Eleven patients reported some benefit, which was considered by seven to be significant. Eight of the 10 patients who had pain reported moderate to significant relief. Five patients had local complications, consisting of disabling weakness of target or neighbouring muscles. These preliminary results suggest that this treatment can be successfully applied to many patients with writer's cramp without performing complex electromyographic recordings while the patients are writing.
Paroxysmal dystonic head tremor
Authors: Hughes Andrew J. , Lees Andrew J. , Marsden C. David
Published: December 2004

Two patients with paroxysmal attacks of “no–no” direction head tremor and mild torticollis are described. One of them had the characteristic features of paroxysmal dystonic choreoathetosis and responded well to clonazepam.
Idiopathic cervical dystonia: Clinical characteristics
Authors: Chan Jane , Brin Mitchell F. , Fahn Stanley
Published: December 2004

We reviewed detailed clinical features of 266 patients with idiopathic cervical dystonia, commonly called spasmodic torticollis. Mean age at onset (41 years), female‐to‐male ratio (1.9:1), clustering of onset between ages 30 and 59 (70%), familial history of dystonia (12%), and remissions (9.8%) were similar to those found in previous studies. In contrast to the single prior large clinical study of this disorder, no predominance of right‐handers or significant thyroid disease was found. Pain, which occurred in 75% of patients and contributed to disability score (p <0.01), distinguishes this syndrome from all other focal dystonias. Pain was also strongly associated with constant (vs. intermittent) head turning, severity of head turning, and presence of spasm. Eighty‐three percent of patients had deviation of the head of >75% of the time when sitting with the head unsupported (constant head deviation at rest). Of the 97% who had head turning, 81% also had head tilting in various combinations. The 23% with hand tremor had an older age at onset (mean, 46 vs. 41 years; p <0.05). An earlier age at onset (p <0.05) was seen in patients with a family history of dystonia (mean, 36 years), with trauma shortly preceding symptoms (mean, 36 years), with a change in the direction of head turning (mean, 30 years), and with remissions (mean, 33 years). Jerky movements or forced transient spasms of the head occurred in 62% of the patients, and these patients would be the ones for whom the designation “spasmodic torticollis” could logically apply. Therefore, because the cervical dystonia in 38% of patients is not spasmodic, we propose that the term “spasmodic torticollis” is not a completely appropriate designation for this condition. We suggest labeling the condition either idiopathic cervical dystonia or idiopathic dystonic torticollis, preferably the former for reasons explained in the text.
Isolated lingual/palatal dystonia
Authors: Robertson‐Hoffman Doreen E. , Mark Margery H. , Sage Jacob I.
Published: December 2004

We report a 45‐year‐old woman with a 3‐year history of continuous dystonic movements of tongue and palate with intermittent episodes of noticeable worsening lasting 6 to 8 h. The movements began immediately after a viral illness. The only contributory history is that the patient received high doses of prochlorperazine 22 years earlier for hyperemesis gravidarum. The patient appears to have an unusual focal lower cranial dystonia. Proposed etiologies may be idiopathic or related to prior use of a phenothiazine with a viral trigger.
Hereditary paroxysmal ataxia with neuromyotonia
Authors: Vaamonde J. , Artieda J. , Obeso J. A.
Published: December 2004

The clinical manifestations of a patient with hereditary paroxysmal ataxia and neuromyotonia are described. Generalized tremor, triggered by sudden movemnts, and spasms of hand and foot muscles were the main clinical findings. Electromyogram (EMG) and nerve blocking studies led to the diagnosis of neuromyotonia. Treatment with acetozolamide was of no therapeutic value, confirming previous observations about the difference in response of paroxysmal ataxia with and without neuromyotonia.
Lesion of the subthalamic nucleus for the alleviation of 1‐methyl‐4‐phenyl‐1,2,3,6‐tetrahydropyridine (MPTP)‐induced parkinsonism in the primate
Authors: Aziz T. Z. , Peggs D. , Sambrook M. A. , Crossman A. R.
Published: December 2004

Central to the neural mechanism of MPTP‐induced parkinsonism is overactivity of the subthalamic nucleus. Lesion of the subthalamic nucleus has therefore been performed in two primates with this condition and found to produce a remarkable amelioration of the disorder.
L‐dopa does not affect electron transfer chain enzymes and respiration of rat muscle mitochondria
Authors: Dagani Fiorenzo , Ferrari Rosaria , Anderson Jeffrey J. , Chase Thomas N.
Published: December 2004

Alterations in mitochondrial respiratory chain enzymes have been found in skeletal muscle of parkinsonian patients. Most of these patients had received treatment with L‐dopa in combination with an inhibitor of peripheral decarboxylase for several years. In order to determine whether these effects are only dependent on the disease or are partially due to its therapy, the effects of L‐dopa methyl ester and benserazide, a peripheral dopa decarboxylase inhibitor, were studied on various parameters related to energy metabolism in rat skeletal muscle mitochondria. The maximum activities related to complexes of the respiratory chain: rotenone‐sensitive NADH‐cytochrome c reductase, succinate‐cytochrome c reductase, cytochrome c oxidase, state 3, state 4, uncoupled state, and respiratory control ratio were measured after 17‐19 days of treatment. The results indicate that L‐dopa treatment does not interfere with any of the parameters investigated and suggest that changes in muscle mitochondrial function found in parkinsonian patients are the result of the disease process and not its treatment.
Idiopathic Parkinson's disease combined with multiple system atrophy. A clinicopathological report
Authors: Hughes Andrew J. , Daniel Susan E. , Lees Andrew J.
Published: December 2004

The clinical and postmortem findings of a patient with Lewy body pathology combined with multiple‐system atrophy are described. When alive the patient was diagnosed as having Parkinson's disease. Pathological examination found evidence of striatonigral and olivopontocerebellar degeneration, together with Lewy bodies at a number of sites characteristic for idiopathic Parkinson's disease. Taken together, the clinical history and histological findings support the coexistence of two disease processes.
Segmental ballistic movement overflow myoclonus: A clinical, electromyographic, and pharmacologic evaluation
Authors: Stübgen J.‐P. , Lotz B. P.
Published: December 2004

A 27‐year‐old man presented with a sporadic, nonprogressive, right‐sided, segmental movement disorder that had started at the age of 2 years. Ballistic movement overflow myoclonus was diagnosed clinically and by surface electromyography. Special investigations did not detect anything except atrophy of the left temporal lobe on CT scan. The movement intensity was attenuated by alcohol but was minimally affected by high doses of oral propranolol (320 mg daily), clonazepam (36 mg daily), or trihexyphenidyl (32 mg daily). High‐dose intravenous biperidine (10 mg) resulted in dramatic improvement, but with unacceptable side effects. The possible role of a cholinergic disturbance in generating myoclonus is discussed.
Neurotoxicity of levodopa on catecholamine‐rich neurons
Authors: Mena María Angeles , Pardo Beatriz , Casarejos María José , Fahn Stanley , de Yébenes Justo García
Published: December 2004

The human neuroblastoma cells NB69 are a catecholamine‐rich cell line with pharmacological properties similar to dopamine neurons. This cell line was used to study the neurotoxicity of levodopa on catecholamine neurons. Levodopa, at 50 × 10−6 M or higher concentrations, produced a doseand time‐dependent reduction in the number of live cells, [3H]thymidine uptake, levels of protein and DNA, and an enhancement of the quinone formation. This is a specific effect of levodopa since it did not happen in NB69 cells incubated with equimolar concentrations of leucine and tryptophan. Treatment with deprenyl, an inhibitor of monoamine oxidase type B, partially prevented levodopa neurotoxicity, suggesting that the mechanism of toxicity was, at least in part, related to an increase in the metabolism of dopamine catalyzed by monoamine oxidase.
Persistent dystonia possibly induced by flecainide
Authors: Miller Lucinda G. , Jankovic Joseph
Published: December 2004

A 55‐year‐old man developed clicking of his teeth, involuntary biting, chewing and grimacing within three days of initiation of flecainide. We suggest flecainide should be added to the list of benzamide derivatives associated with drug‐induced movement disorders.
Progressive dystonia symptomatic of juvenile GM2 gangliosidosis
Authors: Nardocci Nardo , Bertagnolio Barbara , Rumi Viviana , Angelini Lucia
Published: December 2004

A 9‐year‐old boy showed a progressive generalized dystonia, with onset at the age of 4 years, combined with mental deterioration and behavioral disturbances. The values of β‐hexosaminidase activities studied in plasma, leukocytes, and fibroblasts obtained using two different substrates (MUG‐NAc and MUG‐NAc‐6‐S) were significantly reduced but higher than in Tay–Sachs disease and similar to those found in the juvenile chronic form of GM2 gangliosidosis. With anticholinergic therapy, for 1.5 years, the dystonic symptoms did not progress and the boy can still care for himself and attend school. The description of another case of the disease. clinically expressed as dystonia, corroborates the existence of dystonic phenotype of GM2 gangliosidosis.
Permanent bruxism as a manifestation of the oculo‐facial syndrome related to systemic whipple's disease
Authors: Tison François , Louvet‐Giendaj Catherine , Henry Patrick , Lagueny Alain , Gaujard Eric
Published: December 2004

We report here a new case of oculomasticatory syndrome related to systemic Whipple's disease. The patient presented typical ophthalmoparesis associated with ocular myorhythmia consisting of 1 Hz convergent oscillations of both eyes. The masticatory involvement was remarkable and consisted of a permanent bruxism leading to severe tooth abrasions. Possible pathophysiology of such a disorder is discussed.
The undulating toe flexion sign in brain death
Authors: McNair N. L. , Meador K. J.
Published: December 2004

Brain‐dead patients may exhibit gross spontaneous and reflex movements (e.g., Babinski sign, sterotypic flexion of one or more limbs, and Lazarus sign). We report three brain‐dead patients who had unusual complex sequential movements of the toes. Undulating toe flexion was elicited by noxious stimuli to the lower extremities, and consisted of initial plantar flexion of the great toe, followed by sequential brief plantar flexion of the second, third, fourth, and fifth toes. The undulating toe flexion sign differs from previously described responses characterized by plantar flexion of the toes (e.g., Rosselimo's sign and the Mendel‐Bechterew sign) in that it consists of complex patterned sequential movements of the digits rather than brief simultaneous flexion and/or fanning of the toes. Neurologists should be aware of this unusual finding, which should not preclude the diagnosis of brain death.
Diffuse lewy body disease presenting with supranuclear gaze palsy, parkinsonism, and dementia: A case report
Authors: de Bruin V. M. S. , Lees A. J. , Daniel S. E.
Published: December 2004

A 67‐year‐old man with a family history of parkinsonism had visual complaints due to difficulty in convergence, which was followed 2 years later by development of bradykinesia and rigidity. The diagnosis of Steele Richardson‐Olszewski syndrome was made on the basis of a supranuclear gaze palsy, bradykinesia, rigidity, and poor response to levodopa. However, subsequent neuropathological examination revealed diffuse Lewy body disease with no evidence of neurofibrillary tangles involving either subcortical or brain stem structures.
Recumbent tic
Authors: Davies L. , King P. J. L. , Leicester J. , Morris J. G. L.
Published: December 2004

A 59‐year‐old man with a 30‐year history of an unusual movement disorder characterised by involuntary axial spasms that occur only in recumbency is described. Clinical and electrophysiological evidence suggest that this disorder is best characterised as a simple tic of unusual form. We have coined the term “recumbent tic” to describe this disorder.
Stimulus‐sensitive paroxysmal dyskinesias associated with a thalamic infarct
Authors: Nijssen P. C. G. , Tijssen C. C.
Published: December 2004

We describe a patient with the Dejerine‐Roussy syndrome (hemiparesis, hemiataxia, hemidysesthesia, and pain), due to posterolateral thalamic infarction, who also exhibits paroxysmal dyskinesias triggered by action and by sensory stimulation.
Phenytoin and dyskinesias: A report of two cases and review of the literature
Authors: Harrison M. B. , Lyons G. R. , Landow E. R.
Published: December 2004

Dyskinesia is a recognized but uncommon side‐ effect of treatment with phenytoin. Two additional cases of dyskinesia during treatment with phenytoin are described; both had radiographically documented thalamic infarctions. The reported experience to date with movement disorders induced by phenytoin is reviewed and the clinical features summarized. The available experimental evidence addressing the mechanism underlying this side effect is discussed.
Quantitative measurement of cervical range of motion in patients with torticollis treated with botulinum A toxin
Authors: Dykstra Dennis , Ellingham Corine , Belfie Albert , Baxter Tanya , Lee Myoung , Voelker Ann
Published: December 2004

Improvement in cervical range of motion in patients with spasmodic torticollis by botulinum A toxin injection is difficult to objectively measure. Recently, a three‐dimensional cervical range of motion system (EMROM) that measures primary as well as secondary cervical angles has been developed. This system uses an electromagnetic tracking system for data collection and a personal computer for analysis and graphic display of the data. We have tested the EMROM system and, from our results, believe that it can be used clinically to objectively and accurately measure cervical range of motion in patients who have spasmodic torticollis and who receive botulinum toxin injections.
Single‐dose L‐dopa response in early parkinson's disease: Measurements with optoelectronic recording technique
Authors: Johnels Bo , Ingvarsson Páll E. , Holmberg Björn , Matousek Michael , Steg Göran
Published: December 2004

Twenty‐five patients with suspected Parkinson's disease were submitted to optoelectronic movement analysis with the Posturo‐ Locomotor‐Manual (PLM) test before and 60 min after a single dose of L‐Dopa. They were then examined clinically for diagnosis. Two patients were excluded due to L‐Dopa intolerance. Seventeen of the remaining patients were classified as having Parkinson's disease. The movement time (MT) in the PLM test was increased for all these patients, and they improved their performance after L‐Dopa. The degree of improvement was roughly proportional to the pretreatment augmentation of MT in comparison to healthy subjects of the same age. The PLM phase analysis showed a specific disability profile for each individual. Six patients were given diagnoses other than PD. Some improvement was found in one patient with suspected olivopontocerebellar atrophy and one patient with multiple brain injury. Two patients with progressive supranclear palsy, one with suspected striatonigral degeneration, and one with functional disturbance deteriorated after L‐Dopa. In conclusion, truly objective and fully reproducible evaluation of the motor performance before and after a single L‐Dopa dose is easily accomplished with computer‐assisted modern optoelectronic recording equipment. The technique is a valuable tool for the quantitative measurement of treatment effects and contributes to the differential diagnosis.
Comparison of somatosensory evoked potentials with striatal glucose consumption measured by positron emission tomography in the early diagnosis of huntington's disease
Authors: Kuwert T. , Noth J. , Scholz D. , Schwarz M. , Lange H. W. , Töpper R. , Herzog H. , Aulich A. , Feinendegen L. E.
Published: December 2004

Both somatosensory evoked potentials (SEP) and striatal glucose consumption (rCMRGlc) measured by positron emission tomography (PET) have been reported to be abnormal early in the course of Huntington's disease (HD). To compare their diagnostic value, SEP and rCMRGlc were measured in a group of 18 first degree off‐ spring of HD families: 6 had manifest HD with chorea and the remaining 12 individuals were chorea‐ free subjects at risk for HD. In five patients with manifest disease, both SEP and striatal rCMRGlc were significantly abnormal, defined in SEP as having either a bilaterally absent frontal N30 amplitude or a reduction of the parietal N20/P25 amplitude below 1 μV on at least one side; in PET as exhibiting a reduction of the cerebellar ratio (CR) of both caudate and lentiform rCMRGlc below the 99%confidence limits of these variables determined in 20 normal volunteers. The remaining patient with manifest HD had questionably abnormal SEP and significantly reduced indices of striatal rCMRGlc. The five persons at risk for HD who had normal SEP also had normal striatal rCMRGlc; those three at‐risk patients with abnormal SEP also had a reduction of the CR of both caudate and lentiform rCMRGlc. Of the remaining four individuals at risk for HD who had questionably abnormal SEP, three had CR values of striatal rCMRGlc in the normal range and one a reduction of the CR of lentiform rCMRGlc. In at‐risk patients, the SEP diagnosis correlated significantly with caudate (r =−0.8; p < 0.002) and lentiform (r = −0.76; p < 0.005) rCMRGlc. These data indicate a parallel deterioration of SEP and striatal rCMRGlc early in the course of HD even before the development of chorea.
Does tolerance develop to levodopa? Comparison of 2‐and 21‐h levodopa infusions
Authors: Nutt John G. , Carter Julie H. , Woodward William , Hammerstad John P. , Gancher Stephen T.
Published: December 2004

To determine if acute tolerance to levodopa develops, we compared the response to 2‐ and 21‐h levodopa infusions with the rate adjusted during the long infusion to yield the same plasma concentration as at the end of the 2‐h infusion. The duration of response after discontinuing the long infusions was briefer than after discontinuing the short infusion, suggesting the development of tolerance. Furthermore, dyskinesia severity was greater during long infusions. We conclude that continuous dopaminergic stimulation with levodopa may not offer optimal control of parkinsonism in patients with response fluctuations.
Tremor due to stroke
Authors: Ferbert A. , Gerwig M.
Published: December 2004

We report on four patients with unilateral tremor stemming from cerebrovascular accidents. In two patients with proven lesions of the thalamus, the tremor was irregular and, in addition, there was dystonic posturing of the affected arm. Tremor and dystonic posturing had appeared after the stroke. In the other two patients tremor had occurred immediately at the onset of the stroke and lasted only a few days. The tremors were of small amplitude and high frequency, and lesions could not be found on CT or MRI in these two patients. None of our patients showed signs of the so‐called rubral tremor.
Postneuroleptic laryngeal dyskinesias: A cause of upper airway obstructive syndrome improved by local injections of botulinum toxin
Authors: Fève A. , Angelard B. , Fé;nelon G. , Logak M. , Guillard A. , Saint‐Guily J. Lacau
Published: December 2004

We report a case of laryngeal dyskinesia resulting in severe rest and exercise dyspnea. A 51‐year‐old man treated for 2 years with flupentixol, an incisive neuroleptic, developed severe dyspnea due to intermittent, rhythmic, and dystonic movements of the vocal cords and upper airway. Local injections of botulinum toxin resulted in spectacular regression of laryngeal spasms and major improvement in breathing. This case emphasizes the risk of upper respiratory dyskinesias associated with neuroleptic treatment and shows the feasibility of a new local treatment in this life‐threatening disorder.
Familial idiopathic strio‐pallido‐dentate calcifications with late onset extrapyramidal syndrome
Authors: Martinelli P. , Giuliani S. , Ippoliti M. , Martinelli A. , Sforza A. , Ferrari St.
Published: December 2004

A family with autosomal dominant inheritance of idiopathic strio‐pallidodentate calcifications and late onset of extrapyramidal symptoms is reported. Clinical features consisted of parkinsonism in one member and postural tremor in two. Depression and dysarthria were present in all cases. All symptomatic members showed a peculiar biochemical abnormality consisting of reduced 25‐OH vitamin D3 with normal levels of 1,25(OH)2 vitamin D3, suggesting an inborn error of Vitamin D metabolism. The biochemical, clinical, and genetic pattern of this family distinguishes this syndrome from the larger group of secondary familial basal ganglia calcifications.
Ataxia without telangiectasia
Authors: Friedman Joseph H. , Weitberg Alan
Published: December 2004

Ataxia telangiectasia (AT) is an autosomal recessive hereditary disorder characterized by onset in infancy or childhood of a cerebellar and later extrapyramidal disorder associated with telangiectasias and an immune deficit. Only a handful of cases have been described in which the features were not stereotypic. This report describes a case that is classic except for the absence of telangiectasias through age 17. This and other cases suggest that a new, more inclusive term be used to describe the syndrome of ataxia with immune deficit until the genetic abnormalities in these disorders become identifiable.
Dopamine receptors in the basal ganglia: Relevance to parkinson's disease
Authors: Strange Philip G.
Published: December 2004

At least five receptors for dopamine (D1–D5) have been recognised from molecular biological studies, and their pharmacological properties and brain localisations have been determined. The D1 and D2 subtypes are the principal subtypes in brain, and their cellular localisations in the caudate nucleus and putamen have been determined. With recent advances in the understanding of basal ganglia neuronal function, these localisation data enable insights into the mode of action of drugs used at present and in the futue to treat Parkinson's disease.
The detection of preclinical Parkinson's disease: What is the role of positron emission tomography?
Authors: Sawle Guy V.
Published: December 2004

On clinical criteria alone, the diagnosis of early Parkinson's disease can be difficult and, by definition, the prospective recognition of preclinical Parkinson's disease is impossible. Positron emission tomography (PET) using [18F]dopa as tracer has been proposed as a means of identifying patients with preclinical disease. The number of subjects detected to date has been few; most have been identified by serendipity or during the course of family studies. This review examines the significance of a single abnormal scan in an apparently healthty subject in terms of the relationship between normal and abnormal values and the time course of the disease.
Ascorbic acid protects against levodopa‐induced neurotoxicity on a catecholamine‐rich human neuroblastoma cell line
Authors: Pardo Beatriz , Mena María Angeles , Fahn Stanley , de Yébenes Justo García
Published: December 2004

Levodopa, at concentrations of 0.25 × 10−4 M or larger, is toxic for the human neuroblastoma cell NB69. Toxicity is associated with high levels of quinones, increased activity of complex II‐III, and lack of changes of complex I of the mitochondrial respiratory chain. Deprenyl, which does not alter the production of quinones, has a partial protective effect. Tocopherol, 23 or 115 × 10−6 M, lacks significant preventive effect on levodopa toxicity, but ascorbic acid, 10−3 M, prevents levodopa toxicity and quinone formation. Deprenyl, 10−4 M, provides additional protection in cultures treated with levodopa and ascorbic acid. Our results indicate that ascorbic acid and deprenyl prevent levodopa neurotoxicity by unrelated mechanisms. Both compounds should be considered as complementary drugs to test for slowing the progression of Parkinson's disease.
The syndrome of gait ignition failure: A report of six cases
Authors: Atchison P. R. , Thompson P. D. , Frackowiak R. S. J. , Marsden C. D.
Published: December 2004

The syndrome of gait ignition failure is described in six patients in whom difficulty initiating walking was the major symptom. The gait had elements of parkinsonism with start and turn hesitation, shuffling, and freezing. Unlike parkinsonism, however, the gait was relatively normal once entrained; the posture was upright, and good arm swing, a normal stride length, and no festination were seen. Equilibrium was normal or near normal, and when seated or lying, rhythmic leg movements were generated normally. Facial expression, upper limb mobility, and whole body movements were wellpreserved. This gait disorder differed from that seen in Parkinson's disease and the so‐called “frontal” or “senile” disorders of gait and gait “apraxia”. The causes of this gait syndrome are not clear but it may be due to frontal lobe vascular disease and/or focal degeneration of the frontal lobes.
Orofacial dystonia and rest tremor in a patient with normal brain pathology
Authors: Bhatia K. , Daniel S. E. , Marsden C. D.
Published: December 2004

We report a patient with cranial dystonia who also had rest tremor of one arm and who developed drug‐induced parkinsonism on treatment. The patient's brain was normal on autopsy. We also review the findings in the few reported cases of cranial dystonia with pathology.
Linkage analysis with chromosome 9 markers in hereditary essential tremor
Authors: Conway D. , Bain P. G. , Warner T. T. , Davis M. B. , Findley L. J. , Thompson P. D. , Marsden C. D. , Harding A. E.
Published: December 2004

Hereditary essential tremor (ET) is an autosomal dominant disorder with variable expression and reduced penetrance. A tremor indistinguishable from ET may be observed in patients with autosomal dominant idiopathic torsion dystonia (ITD), in which the disease locus has been mapped to 9q32–34 in some kindreds, tightly linked to the argininosuccinate synthetase (ASS) locus. We performed linkage analysis in 15 families with ET containing 60 definitely affected individuals, using dinucleotide repeat polymorphisms at the ASS locus and the Abelson locus (ABL). Cumulative lod scores were − 19.5 for ASS and − 10.8 for ABL at a recombination fraction of 0.01, and tight linkage to ASS was excluded individually in 11 of the families. These data indicate that the ET gene is not allelic to that causing ITD.
Interactive video conferencing: A means of providing interim care to parkinson's disease patients
Authors: Hubble Jean P. , Pahwa Rajesh , Michalek Debra K. , Thomas Calvina , Koller William C.
Published: December 2004

Inequity in health care delivery is attributable to a variety of factors including geographic isolation. Patients living far from major urban centers have limited access to medical specialists. In the instace of Parkinson's disease (PD), optimal assessment and care may depend upon availability of specialty health care providers. In order to broaden health care access, interactive video conference (IVC) units are being developed for medical use. IVC allows a patient at a distant site to be “seen and heard” by a hospitalbased physician; simultaneously, the patient can “see and hear” the doctor. To establish the validity of this technology in the evaluation of PD, nine patients were independently examined and scored (UPDRS) by two movement disorder specialists. One examination was performed in‐person by the usual physician. The other examination was performed on the same day via IVC over a distance of 350 miles by an examiner previously unfamiliar with the patients. Individual patient scores did not differ based on examiner (Spearman Rho Correlation Coefficients: UPDRS total scores r = 0.933, p < 0.0002; Hoehn and Yahr Scale r = 0.883, p < 0.001). A standardized exit interview was conducted to assess patients' perceptions of this application of video technology. Responses were favorable and virtually all patients viewed this as a means of accessing better health care. We conclude that valid motor assessments of PD patients can be made via IVC.