bluuebunny/crossref_metadata_2025 huggingface dataset:
Profile of patients enrolled in a new movement disorder clinic
Authors: Siemers Eric , Reddy V.
Published: December 2004

To assess the need for a regional expertise in movement disorders, the numbers of patients, clinic visits, and medication changes for a new movement disorder clinic were recorded. During 3½ years, 355 patients were seen, with 1,329 clinic visits. Idiopathic Parkinson's disease was the most common diagnosis, comprising 36% of the population, followed by dystonia (17%), tremor (12%), parkinsonism (i. e., Parkinson's plus syndromes, drug‐induced parkinsonism, etc.) (10%), chorea (10%), Tourette's syndrome (6.5%), and tardive dyskinesia (3.4%). Distribution of follow‐up visits was similar, with Parkinson's disease (52%) being most frequent and Tourette's syndrome (3.1%) least frequent. The relative utilization of medical care by each patient group was assessed by determining the number of medication changes and the number of clinic visits per follow‐up year. No differences in these measures were found using a one‐way analysis of variance. Of the Parkinson's disease patients, 67% had Hoehn and Yahr stages III‐IV and 77% of the clinic visits were made by this subgroup. When considered in light of the prevalence of each of the diseases, these data show a need for an expertise in movement disorders for a population base of the size we have served.
Steroid‐responsive chorea in moyamoya disease
Authors: Pavlakis Steven G. , Schneider Steven , Black Karen , Gould Robert J.
Published: December 2004

We report the case of a 13‐year‐old boy with disabling chorea due to moyamoya disease. His chorea seemed to improve with steroid therapy. We conclude that steroid therapy may ameliorate moyamoya‐associated chorea, and perioperative steroids can confound neurosurgical outcome. We are unable to assess the effect of cerebrovascular bypass procedures on the outcome of chorea in this patient.
Complex stereotypies after right putaminal infarction: A case report
Authors: Maraganore D. M. , Lees A. J. , Marsden C. D.
Published: December 2004

A 17‐year‐old boy is described who experienced circling behavior interrupting his gait and complex stereotyped movements of the hands after a complicated repair of an aortic coarctation. An MRI scan showed a right putaminal infarct. The boy's behavior and stereotypies bear a close resemblance to those found in autism.
The coordination of posture and voluntary movement in patients with cerebellar dysfunction
Authors: Diener H.‐C. , Dichgans J. , Guschlbauer B. , Bacher M. , Rapp H. , Klockgether T.
Published: December 2004

Postural adjustments associated with the task of rising on tiptoes were investigated in a reaction time paradigm in 10 normal subjects and 18 patients with cerebellar disorders. Cerebellar dysfunction was due to either degenerative cerebellar disease, tumor, or ischemia. Displacements of the center of foot pressure (CFP) were recorded. The task, accomplished by the triceps surae muscle (executional activity, mean latency of 411 ms), is mechanically effective only if the center of gravity has been shifted forward in advance. To this effect, a phasic burst of preparatory EMG activity in the tibialis anterior normally occurs at a mean latency of 163 ms, shifting the center of gravity forward. Shortly thereafter, activity of the quadriceps femoris (175 ms) extends the knee and aids the forward shift of the center of gravity. Different aspects of this motor sequence were disturbed in individual patients: Latencies of preparatory and executional activity were uncorrelated in 15 of the 18 patients. Executional (n = 16) or preparatory (n = 13) EMG activity was tonic instead of phasic. Latencies of either preparatory or executional EMG activities or both were prolonged (n = 10). The time interval between motor preparation and execution was increased (n = 9). The trial‐to‐trial variability of biomechanical parameters and EMG latency was increased. Preparatory EMG activity in the quadriceps was entirely missing (n = 9), resulting in knee bending at the unsuccessful attempt to rise on tiptoes. Patients who were most severely affected had no preparatory activity at all (n = 2), and therefore were unable to perform the task. In conclusion, the cerebellum contributes to the scaling of size and duration of preparatory and executional motor activity and controls their temporal relationships.
Is nocturnal paroxysmal dystonia a form of frontal lobe epilepsy?
Authors: Meierkord H. , Fish D. R. , Smith S. J. M. , Scott C. A. , Shorvon S. D. , Marsden C. D.
Published: December 2004

The sex ratio, age at onset, and ictal features of nine patients with nocturnal paroxysmal dystonia were compared with those of eight patients with daytime frontal lobe seizures and eight patients with nocturnal motor attacks of known epileptic origin. All patients underwent video electroencephalography telemetry. There were no clinical features that would allow distinction between the groups and no single phenomenon was uniquely seen in nocturnal paroxysmal dystonia. This study provides no evidence that nocturnal paroxysmal dystonia is a separate diagnostic entity.
Marked reduction of striatal dopamine D2 receptors as detected by 123IBZM‐SPECT in a Wilson's disease patient with generalized dystonia
Authors: Schwarz J. , Tatsch K. , Vogl T. , Kirsch C. M. , Trenkwalder C. , Arnold G. , Gasser T. , Oertel W. H.
Published: December 2004

[123I]iodobenzamide‐single photon emission computed tomography (IBZMSPECT) was employed to study the distribution of dopamine D2 receptors in a patient with biochemically proven Wilson's disease presenting with generalized dystonia. IBZM is a dopamine D2 receptor antagonist with high affinity and specific binding to basal ganglia detectable by SPECT. IBZM‐SPECT in this patient (age, 20 years) displayed a striatum to frontal cortex ratio of 1.2 compared to 1.55 ± 0.05 (mean ± SD) in normal controls (n = 7; mean age, 53.3 years). In parallel with this finding, MRI with heavily T2‐weighted sequences showed atrophy and low signal intensity changes of the basal ganglia. There was no improvement of dystonia after a subcutaneous injection of apomorphine. In contrast, IBZM‐SPECT of a neurologically asymptomatic Wilson's disease patient (age, 21 years) displayed a striatum to frontal cortex ratio of 1.6. The MRI scan of this patient was normal. It is suggested that the observed apomorphine‐unresponsive generalized dystonia in this Wilson's disease patient is related to striatal lesions proven by IBZM‐SPECT and MRI.
A case of bilateral alternating hemifacial spasms
Authors: van de Biezenbos J. B. M. , Horstink M. W. I. M. , van de Vlasakker C. J. W. , van Engelen B. G. M. , van Eikema Hommes O. R. , Barkhof F.
Published: December 2004

We report a 24‐year old woman who developed bilateral hemifacial spasm alternating from one side to the other. The spasms followed a left peripheral facial palsy 2 years previously. This unusual type of bilateral hemifacial spasm was possibly due to lesions of multiple sclerosis in the brainstem.
Hemiballism and chorea in a patient with parkinsonism due to a multisystem degeneration
Authors: Steiger M. J. , Pires M. , Scaravilli F. , Quinn N. P. , Marsden C. D.
Published: December 2004

Dyskinesias associated with dopaminergic treatment in idiopathic Parkinson's disease (PD) can be indistinguishable from those arising spontaneously in other conditions involving degeneration of, or damage to, the basal ganglia. However, those due to levodopa treatment of PD disappear on cessation of therapy. We report a patient with a clinical diagnosis of PD who, on levodopa treatment, developed hemiballism and chorea that were originally thought to be drug induced. However, the dyskinesias persisted despite stopping levodopa. Postmortem analysis showed a multisystem degeneration.
Posttraumatic segmental axial dystonia
Authors: Jabbari Bahman , Paul John , Scherokman Barbara , van Dam Bruce
Published: December 2004

Isolated dystonia of trunk and neck muscles without involvement elsewhere has been termed segmental axial dystonia—a rare disorder. We report a 31‐year‐old man who developed marked dystonia of paraspinal muscles and progressive scoliosis 6 months after a closed head injury. Computed tomography (CT) disclosed three small areas of encephalomalacia, one involving the head of the caudate nucleus. Treatment with trihexyphenidyl resulted in significant improvement of the dystonia and scoliosis.
Pathophysiology of cerebellar ataxia
Authors: Diener H.‐C. , Dichgans J.
Published: December 2004

Human and animal experiments performed recently have resulted in a more detailed understanding of limb movement and body posture disorders associated with cerebellar dysfunction. The delay in movement initiation can be explained by a delay in onset of phasic motor cortex neural discharge owing to decreased input from the cerebellar hemispheres. Disorders of movement termination (dysmetria), which can occur for movements at proximal and distal joints, result from disturbances of the timing and intensity of antagonist electromyographic (EMG) activity necessary to break the movement. Disorders in velocity and acceleration of limb movements result from muscular activity that is smaller in amplitude and more prolonged. The cerebellum is important for control of constant force but not for generation of maximal force. Dysdiadochokinesia is explained by a combination of the abovementioned mechanisms. During complex movements in three‐dimensional space, the cerebellum contributes to timing between single components of a movement, scales the size of muscular action, and coordinates the sequence of agonists and antagonists. The basic structure of motor programs is not generated in the cerebellum. Hypotonia can be observed only in acute cerebellar lesions. Cerebellar tremor appears to result from a central mechanism, but is modulated or provoked through increased long‐loop EMG responses. The common assumption that cerebellar ataxia of stance does not improve with visual feedback is true only of vestibulocerebellar lesions, not for ataxia resulting from atrophy of the anterior lobe of the cerebellum.
Levodopa‐induced dyskinesias in Parkinson's disease: Clinical and pharmacological classification
Authors: Luquin M. R. , Scipioni O. , Vaamonde J. , Gershanik O. , Obeso José A.
Published: December 2004

Levodopa‐induced dyskinesias (LID) in Parkinson's disease (PD) may be classified into three main categories: “On” dyskinesias, diphasic dyskinesias (DD), and “off” periods. The study of 168 parkinsonian patients showed that about half (n = 84) showed one pattern of LID only. A combination of two was present in 68, and 16 had the three presentation patterns. A fairly good correlation between type of dyskinesia and presentation pattern was established. Chorea, myoclonus, and dystonic movements occurred during the “on” period. Dystonic postures, particularly affecting the feet, were mainly present in the “off” period, but a few patients had a diphasic presentation. Repetitive stereotyped movements of the lower limbs always corresponded to DD. Acute pharmacological tests using dopamine agonists 9subcutaneous apomorphine 3–8 mg; intravenous lisuride 0. (1–0. 15 mg) and dopamine antagonists (intravenous sulpiride 200–400 mg and intravenous chlorpromazine 25 mg) were performed in 40 patients. Dopamine agonists enhanced “on” dyskinesias and markedly reduced or abolished “off” period dystonia and DD. Dopamine antagonists reduced all types of LID but usually aggravated parkinsonism. These clinical and pharmacological results indicate that LID in PD are a heterogeneous phenomenon difficult to explain on the basis of a single pathophysiological mechanism.
Effect of ethosuximide on rest tremor in the MPTP monkey model
Authors: Gomez‐Mancilla Baltazar , Latulippe Jean‐François , Boucher René , Bédard Paul J.
Published: December 2004

Based on the hypothesis that low‐threshold calcium conductance in the thalamus might be involved in the pathophysiology of parkinsonian tremor, ethosuximide was given chronically to a monkey previously treated with MPTP and displaying exceptionally a typical rest tremor. After 5 days of daily treatment, the tremor was reduced by 60%. Diltiazem and verapamil which act on different calcium channels had no such effect. Ethosuximide also potentiated the antitremor effect of the dopamine D2 agonist LY‐171555.
Movement disorders as sequelae of central pontine myelinolysis: Report of three cases
Authors: Maraganore Demetrius M. , Folger W. Neath , Swanson Jerry W. , Ahlskog J. Eric
Published: December 2004

In three survivors of central pontine myelinolysis, dystonia (in two patients) and rest tremor (in one) were sequelae. The onset of these movements occurred 3 weeks to 5 months after the initial presentation with central pontine myelinolysis. Magnetic resonance imaging revealed basal ganglia lesions suggestive of extrapontine myelinolysis in all three patients. We propose that the movement disorders seen in our cases are clinical correlates of extrapontine myelinolysis.
Cerebrospinal fluid antibodies to coronavirus in patients with Parkinson's disease
Authors: Fazzini Enrico , Fleming John , Fahn Stanley
Published: December 2004

The etiology of Parkinson's disease remains unknown, and a search for environmental agents continues. In 1985, Fishman (10) induced infection of the basal ganglia by a coronavirus in mice. Although coronavirus is recognized primarily as a respiratory pathogen in humans, its affinity for the basal ganglia led us to investigate its possible role in human Parkinson's disease. The cerebrospinal fluid of normal controls (CTL) (n = 18), and patients with Parkinson's disease (PD(n = 20)) and other neurological disease (OND) (n = 29) was analyzed in a blinded manner by enzyme‐linked immunosorbent assay [measurements in optical density (OD) units] for antibody response to four coronavirus antigens: mouse hepatitis virus JHM (J) and A59 (A), and human coronavirus 229E (E) and OC43 (O). When compared with CTL, PD patients had an elevated (p < 0.05) mean OD response to J (0.0856 vs. 0.0207) and A (0.1722 vs. 0.0636). Response (p > 0.05) to O (0.0839 vs. 0.0071) was greater than that to E (0.1261 vs. 0.0743). When compared to OND, PD patients had an elevated mean OD response to J (0.0856 vs. 0.0267, p < 0.05). Responses (p > 0.05) to A (0.1722 vs. 0.0929) and O (0.0839 vs. 0.0446) were greater than that to E (0.1261 vs. 0.0946). These results suggest that there may be an association between coronavirus and PD.
Severe but temporary injury to rabbit orbicularis oculi muscle using dihematoporphyrin ether and laser photochemomyectomy
Authors: Wirtschafter Jonathan D. , Slovut David Paul , Stordal Leif , Valentino Joseph , McLoon Linda Kirschen
Published: December 2004

The use of local dihematoporphyrin ether (DHE) injections, followed by laser light activation, was investigated as a potential permanent myectomy treatment for muscle spasms, in particular blepharospasm and hemifacial spasm. DHE was injected into the eyelids of rabbits, followed by laser activation, as used in photochemotherapy. Four days after treatment, histological examination indicated that doses of ≥0.5 mg of DHE and laser treatment with an energy density of at least 100 J/cm2 resulted in an almost total destruction of the orbicularis oculi muscle in the treated eyelid. The amount of muscle injury was dependent on both dose of DHE and energy density levels. Histologically, the tarsal glands and conjunctiva were damaged. Glandular tissue was markedly reduced, and the conjuctival epithelium showed hyperplasia and a loss of mucous cells. Six months after DHE and laser treatment, the majority of the muscle tissue had regenerated, although there was evidence of previous injury. While DHE injections combined with laser light activation were lethal to muscle at the site of treatment, this treatment was not permanent. The orbicularis oculi muscle retained its ability to regenerate. However, photochemomyectomy may be studied further as an adjuvant treatment to temporarily injure and debulk large muscles when botulinum toxin is contraindicated due to the large doses involved or as permanent treatment when used togeter with an antimitotic agent such as doxorubicin.
Cellular interactions in the striatum involving neuronal systems using “classical” neurotransmitters: Possible functional implications
Authors: Nieoullon André , Goff Lydia Kerkerian‐Le
Published: December 2004

The neostriatum contains a wide variety of neuroactive substances associated with several well‐defined functional neuronal systems. This structure, which is the seat of numerous neurological pathological disorders, is commonly used as a model for studying the basic mechanisms of neurotransmitter interactions in the brain and their putative involvement in striatal functions. Increasing interest has been focusing lately on the cellular interactions that may occur between the corticostriatal putatively glutamatergic system and the nigrostriatal depaminergic input. Current evidence suggests that the activatory corticostriatal glutamatergic input may play a more crucial role in regulating striatal functions than was formely assumed in comparison with the dopaminergic input. The key role of cholinergic interneurons in the striatum may therefore be attributable to the fact that they modulate the glutamatergic transmission to GABA striatal efferent neurons. Likewise, dopamine may actually act indirectly in the striatum by “tuning down” the cortical excitation of striatal neurons. Consequently, an impairment of the dopaminergic transmission such as that occurring in parkinsonism may lead to an increase in the corticostriatal glutamatergic transmission, which may further contribute towards reinforcing the “imbalance” between subsets of striatal neuronal systems controlling the output of the basal ganglia.
Golfers' cramp: Clinical characteristics and evidence against it being an anxiety disorder
Authors: Sachdev Perminder
Published: December 2004

The clinical characteristics of 20 golfers suffering from golfers' cramp or the “yips” are described. The typical description is that of a middleaged golfer who has played competitive golf since his teens and develops the problem during a tournament in the form of a jerk, spasm, or freezing of movement while putting or chipping, with the rest of the game being relatively unaffected. The problem generally takes a chronic fluctuating course, and a number of trick strategies are partially or fully successful. In this study, the subjects were compared with a matched group of 20 unaffected golfers on a number of indices of psychopathology; no significant differences emerged. The more severely affected golfers also did not differ significantly from the mildly affected ones, except on the subjective report of anxiety. These data support the argument that golfers' cramp is not an anxiety disorder or a neurosis. The important role of anxiety and arousal in its manifestation is, nevertheless, recognized and its pathophysiology speculated upon.
Noise‐induced psychogenic tremor associated with post‐traumatic stress disorder
Authors: Walters Arthur S. , Hening Wayne A.
Published: December 2004

Tremors in post‐traumatic stress disorders have not been previously well characterized. A 67‐year‐old man has a 46‐year history of a noiseinduced exaggerated startle reflex followed by a large amplitude rest, postural and kinetic tremor that may persist for up to 3 days. This tremor is superimposed on a continuous mild organic postural/kinetic tremor whose electrophysiological characteristics are different from those of the overlying tremor. We attribute the exaggerated startle reflex and the noise‐induced tremor to Post‐Traumatic Stress Disorder (PTSD) and postulate a psychogenic origin for the noise‐induced tremor. The patient alos believes the noise‐induced tremor to be psychologically based and to be produced by the fear and anxiety he experiences when he hears loud, unexpected noises. The sudden onset of the noise‐induced tremor, its intermittent character, its temporary disappearance on distraction despite the patient's inability to suppress it, inconsistencies in handwriting and figure drawing, and the fact that the noise‐induced tremor is stimulus specific and persists long after the offending stimulus (noise) is no longer present all suggest a tremor of psychogenic origin.
Premorbid smoking, alcohol consumption, and coffee drinking habits in Parkinson's disease: A case‐control study
Authors: Jiméanez‐Jiméanez Félix Javier , Mateo Dolores , Giméanez‐Roldan Santigo
Published: December 2004

A number of studies have reported lower cigarette consumption in patients with Parkinson's disease (PD) previous to onset of the disease. In an attempt to determine whether there existed a “premorbid attitude” by patients against the use of socially accepted “drugs,” the premorbid tobacco, alcohol, and coffee consumption habits were compared in 128 PD patients and 256 controls. Patients and controls were selected by case control method and were recruited from the same health area and socioeconomic stratum. In males, the habits of smoking more than 10 cigarettes/day (p < 0.001) and drinking more than 50 g/day of alcohol (p < 0.001) were significantly less frequent in the PD patients than in the controls, but the differences in coffee consumption were nonsignificant. In females behavior did not differ significantly between the PD group and the controls for any of the three habits. There was no correlation between the amount of smoking and alcohol drinking and age at onset of PD or current Hoehn and Yahr's staging. Our results suggest the existence of a premorbid personality in males with PD, possibly conditioning a restrictive attitude toward the consumption of such toxic substances as tobacco and alcohol, yet a more tolerant attitude toward habits more widely accepted socially, like coffee consumption.
Corticobasal degeneration: Decreased and asymmetrical glucose consumption as studied with PET
Authors: Blin J. , Vidailhet M.‐J. , Pillon B. , Dubois Bruno , Feve J.‐R. , Agid Y.
Published: December 2004

Cerebral energy metabolism was studied by positron emission tomography and [18F]fluorodeoxyglucose in five patients with clinical diagnoses of probable corticobasal degeneration. A reduction in glucose consumption was observed in most cortical and subcortical structures compared to agematched controls. The reduction was greatest on the side of the brain contralateral to the most affected limbs, as shown by the significantly lower ratios of contralateral to homolateral metabolic rates, in the temporal and sensorimotor cortex of patients compared to controls. A distinct asymmetry between the two hemispheres could be observed in a patient who was examined twice in the course of his illness. Detection of this asymmetrical decrease in brain cortical and subcortical glucose metabolism may prove useful as additional evidence supporting clinical diagnoses of the disease.
Isolated continuous rhythmic lingual myoclonus
Authors: Gobernado José M. , Galarreta Mario , de Blas Genma , Jimenez‐Escrig Adriano , Hernandez Amalia
Published: December 2004

Isolated continuous lingual myoclonus is an exceptional entity, poorly documented and understood. A patient with a nonepileptic continuous rhythmical myoclonus, affecting the anterior portion of the tongue, as an independent involuntary disorder, is reported. Electromyography showed low frequency (2‐4 Hz) bursts of genioglossus muscles activity. The EEG, visual, auditory and somatosensory evoked responses were normal. Imaging techniques like CT and MRI failed to reveal any brainstem or cerebellar lesion. Lingual myoclonus showed a very good response to sodium valproate.
Reaction time responses in parkinsonian and hemiparkinsonian patients
Authors: Mazzucchi A. , Sinforiani E. , Ludovico L. , Turla M. , Pacchetti C. , Brianti R. , Parma M. , Nappi G.
Published: December 2004

Twenty‐ one normal subjects, 32 bilateral parkinsonian patients (BPs) and 29 hemiparkinsonian patients (HP) were submitted to separate or sequentially associated motor tasks that included simple reaction times (RT), choice RTs, directional RTs, and movement RTs. The results showed that simple RTs, directional RTs, and movement times (MT) were slower in BPs as compared to normal subjects; for choice RTs there was no difference. Response patterns were similar in normal controls and BPs. In both groups RTs became more prolonged as sequentially programmed operations increased. If movement occurred at the end of the sequence, they prolonged the RTs of the preceding operations, but MTs per se did not vary. In HPs the same results were observed on the “bad” hand side versus normal controls and versus the healthy side, but a significant statistical level was reached mainly when the “bad” hand was the right one.
The cholinergic system‐dependent myoclonus of the baboon papio is a reticular reflex myoclonus
Authors: Rektor I. , Švejdová M. , Silva‐Barrat C. , Menini C.
Published: December 2004

Neurophysiological studies were performed on four Papio papio baboons presenting with nonepileptic myoclonus (a startle response resembling stimulus‐sensitive jerk). Investigations of the EEG, back‐averaged EEG, and somatosensory evoked potentials revealed the absence of cortical correlates preceeding the jerks, and exclusion of cerebral cortex involvement. No long‐latency reflexes could be recorded in these animals. The jerks were symmetric when evoked by unilateral stimulation in normal baboons as well as in a split‐brain animal. Polymyographic records showed that the first muscle involved during the jerk was the trapezius; other muscles were involved with latencies increasing in both cranial and caudal directions. From these data, nonepileptic myoclonus of baboons can be classified as a reticular reflex myoclonus. The involvement of cranial nerves did not follow the layout of the nuclei in the brainstem, indicating that the jerk is most likely generated as a complete movement. The generating structure is probably under cholinergic control. Finally, the Papio papio baboon, which was already known as a model for cortical myoclonus elicited by intermittent photic stimulation in predisposed animals, can also be considered a model for the study of the reticular reflex myoclonus.
Time course of distant effects of local injections of botulinum toxin
Authors: Garner C. G. , Straube A. , Witt T. N. , Gasser T. , Oertel W. H.
Published: December 2004

Botulinum toxin A (btx) is used to treat focal dystonias. From accidental intoxications it is known that btx can cause generalized pathologic single‐fiber electromyography (SFEMG) findings. We monitored the onset and course of these disturbances in eight patients who received a small dose of btx (2–22 ng) for therapy of focal dystonias in the head/neck region for the first time via repeated SFEMG investigations at days 0, 3, 6, 9, 12, 28, and 56. Recordings were performed in the extensor digitoum brevis muscle, and in two patients additionally in the tibialis anterior muscle. In six of these patients we found an increase of jitter and blocking. The onset of these changes was in the range of 3–13 days after injection. Fiber density showed a tendency to increase. There was no correlation between SFEMG findings and the dose of injected btx. Possible mechanisms for these observations may be either a very efficient local uptake and retrograde axonal transport via the spinal motor neurons or a systemic distribution via the blood circulation.
Inefficacy of propranolol in attenuation of drug‐induced parkinsonian tremor
Authors: Metzer W. Steven , Paige Stephen R. , Newton Joseph E. O.
Published: December 2004

We investigated the tremolytic effect of long‐acting propranolol (propranolol‐LA) in six subjects with drug‐induced parkinsonism (DIP), using a double‐blind, placebo‐controlled, crossover experimental design. Subjects received propranolol‐LA for 2 weeks and placebo for 2 weeks, with no change in neuroleptic treatment. Tremor frequency and amplitude were objectively quantified at the end of each 2‐week period by computerized tremorgram recording. There were no significant differences in attenuation of DIP tremor by propranolol‐ LA and placebo. Previous investigations reported in the literature have found propranolol to attenuate the tremor of idiopathic parkinsonism (IPD). It is expected that DIP and IPD tremor would respond similarly to propranolol if a solely peripheral or spinal cord tremolytic action were operative. A possible differential attenuation of IPD tremor and DIP tremor provides support for the concept of a higher central tremolytic mechanism of β‐adrenergic receptor‐blocking drugs.
Neuropsychological correlates of brain atrophy in Parkinson's disease: A CT‐scan study
Authors: Starkstein Sergio E. , Leiguarda Ramón
Published: December 2004

We examined the presence of cortical or subcortical brain atrophy (as shown by CT scans) in patients with Parkinson's disease (PD) and assessed whether there were significant correlations between CT measurements and the presence of cognitive deficits. There were three main findings. First, patients with bilateral symptoms of PD showed more severe cortical and subcortical atrophy than age‐matched normal controls. Second, the presence of unilateral symptoms of PD was significantly associated with contralateral brain atrophy only in patients with right hemi‐PD. Third, there was a significant correlation between neuropsychological deficits and atrophy in specific brain areas.
Effectiveness of piracetam in cortical myoclonus
Authors: Brown P. , Steiger M. J. , Thompson P. D. , Rothwell J. C. , Day B. L. , Salama M. , Waegemans T. , Marsden C. D.
Published: December 2004

Twenty‐one patients with disabling spontaneous, reflex, or action myoclonus due to various causes, who had shown apparent clinical improvement on introduction of piracetam, entered a placebo‐controlled double‐blind crossover trial of piracetam (2.4– 16.8 g daily). All but one patient had electrophysiological evidence of cortical myoclonus. Patients were randomly allocated to a 14‐ day course of piracetam followed by identical placebo, or placebo followed by piracetam. Nineteen patients received piracetam/placebo in addition to their routine antimyoclonic treatment (carbamazepine, clonazepam, phenytoin, primidone, sodium valproate, or tryptophan plus isocarboxazid, alone or in combination) and two received piracetam/placebo as monotherapy. All patients were rated at the end of each treatment phase using stimulus sensitivity, motor, writing, functional disability, global assessment, and visual analogue scales. Ten of the 21 patients had to be rescued from the placebo phase of the trial because of a severe and intolerable exacerbation of their myoclonus. No patients required rescue from the piracetam phase of the double‐blind trial. When the 21 patients were considered together, there was a significant improvement in motor, writing, functional disability, global assessment, and visual analogue scores during treatment with piracetam compared with placebo. The total rating score also improved significantly with piracetam, by a median of 22%. Piracetam, usually in combination with other antimyoclonic drugs, is a useful treatment for myoclonus of cortical origin.
PCR Analysis of platelet mtDNA: Lack of specific changes in Parkinson's disease
Authors: Sandy Martha S. , Langston J. William , Smith Martyn T. , di Monte Donato A.
Published: December 2004

An alteration within the mitochondrial DNA (mtDNA) has been hypthesized to underlie the deficiencies in mitochondrial complex I activity observed in the platelets, striatal muscle, and brain tissue of individuals with Parkinson's disease. Here we utilized the polymerase chain reaction (PCR) to analyze mtDNA obtained from the platelets of nonmedicated patients with early Parkinson's disease (n =8) and aged‐matched cantrols (n =6) for the presence of deletion(s) or addition(s) equal to or greater than 50–100 base pairs. Initial attention was focused upon detecting a 4.977 kb deletion previously found in the brains of parkinsonian patients and some aged controls. Indeed, a large deletion of approximately 5.0 kb was observed in the platelet mtDNA from all parkinsonian individuals. However, this defect was also found in all age‐matched controls as well as in a group of young healthy subjects (n = 5). In addition, we searched for the presence of smaller changes in platelet mtDNA from parkinsonian patients by PCR analysis of four mtDNA segments that code for seven of the complex I polypeptides. No large deletions or additions were detected within these four regions of mtDNA in any of the disease or age‐matched control samples. We conclude that (a) a 4.977 kb deletion is apparently present in a subpopulation of platelet mtDNA from all individuals, and (b) no macrosequence alteration in mtDNA is likely to underlie the deficiency in complex I activity reported in platelet mitochondria from parkinsonian patients.
Gastrointestinal symptoms in parkinson disease: 18‐month follow‐up study
Authors: Edwards L. , Quigley E. M. M. , Hofman R. , Pfeiffer R. F.
Published: December 2004

In a recent study we identified abnormal salivation, dysphagia, nausea, constipation, and defecatory dysfunction as those gastrointestinal (GI) symptoms associated with Parkinson disease (PD) and characterized their relationship to PD severity and therapy. In this study, we re‐evaluated these symptoms and their relationship to parameters of PD 18 months later. Sixty‐six percent of the original participants responded. Over the 18 months, 68% of originally untreated PD subjects commenced anti‐PD therapy. Abnormal salivation, dysphagia, nausea, constipation, and defecatory dysfunction were again identified as those GI symptoms more common in PD. Constipation increased both in severity and frequency. Comparison of GI symptom scores and parameters of PD dysfunction failed to reveal significant progression of either GI symptomatology or PD dysfunction, or the development of new GI symptoms over the 18‐month period. This study validates our GI dysfunction assessment system and confirms abnormal salivation, dysphagia, nausea, constipation, and defecatory function as those GI symptoms truly associated with PD. A direct relationship between PD and its related GI symptoms is again supported.
Parkinson's disease mortality and the industrial use of heavy metals in Michigan
Authors: Rybicki Benjamin A. , Johnson Christine Cole , Uman Jane , Gorell Jay M.
Published: December 2004

Parkinson's disease (PD) mortality rates in Michigan counties for 1986–1988 were calculated with respect to potential heavey metal exposure (iron, zinc, copper, mercury, magnesium, and manganese) from industry based on recent census data. Individuals were counted as a PD death if the diagnosis was listed as an “underlying” or “related” cause of death on the death certificate. Counties with an industry in the paper, chemical, iron, or copper related‐industrial categories (ICs) had statiscally significantly (p < 0.05) higher PD death rates than counties without these industries. Significant correlations of chemical (rs =0.22;p = 0.05), paper (rs =0.22;p = 0.05) and iron (rs =0.29;p = 0.008) industry densities with PD death rates were also present. Counties were divided into high (>15/100,000 individuals 45 years old and over) and low (< = 15/100.000) PD death rate counties by cluster analysis. Geographically, counties with high PD mortality were located mainly in the southern half of the lower peninsula and eastern half of the upper peninsula; low PD death rate counties formed two distinct clusters in the western edge of the upper peninsula and the north‐central portion of the lower peninsula. Other possible risk factors that may explain the varied distribution of PD death rates in Michigan were examined. Those significantly correlated with PD mortality included population density (rs =0.31;p= 0.005), farming density (rs= 0.25;p =0.02), and well water use (rs =−0.24;p =0.03). These ecologic findings suggest a geographic association between PD mortality and the industrial use of heavy metals.
Tardive and chronically recurrent oculogyric crises
Authors: Sachdev Perminder
Published: December 2004

Six patients with chronically recurrent oculogyric crises (OGC) are reported. Four of these were derived from a study of 100 schizophrenic patients on maintenance neuroleptic medication, thereby giving a prevalence of 4% in such patients. Three of the six had the OGC develop as a tardive sideeffect, and in one patient the episodes persisted for some months after the cessation of the offending neuroleptic drug. The episodes of ocular dystonia were associated with other dystonic movements and a number of psychiatric symptoms, with obsessional thoughts and hallucinations being the outstanding features in one patient each. This paper argues for an increased recognition of chronically recurrent and tardive OGC. It also draws attention to the fact that drug‐induced OGC may be a multifaceted disorder with disturbances of movement, thought, behavior, and emotion, reminiscent of the OGC described in association with epidemic encephalitis lethargica.
Movement disorders with cerebral toxoplasmosis and AIDS
Authors: Nath Avindra , Hobson Douglas E. , Russel Anne
Published: December 2004

Movement disorders occur in some patients with cerebral toxoplasmosis with HIV‐1 infection. Such movement disorders have not been described in patients with cerebral toxoplasmosis without HIV‐1 infection. This report discusses their diagnostic features, aspects of management, and possible mechanisms underlying the pathogenesis of the movement disorders.
Akinetic‐rigid syndrome in a 13‐year‐old girl with HIV‐related progressive multifocal leukoencephalopathy
Authors: Singer Carlos , Berger Joseph R. , Bowen Brian C. , Bruce Jocelyn H. , Weiner William J.
Published: December 2004

A 13‐year‐old HIV‐ infected girl presented with a rapidly progressive akineticrigid syndrome. Cranial magnetic resonance imaging revealed bilateral centrum semiovale lesions extending into the basal ganglia. Detailed histopathological study of a biopsy specimen from the involved regions resulted in a diagnosis of progressive multifocal leukoencephalopathy (PML). This case illustrates that an akinetic‐rigid syndrome may be a rare presentation of PML. HIV‐related PML may have unusual radiological features. Brain biopsy may be necessary to confirm PML in cases associated with atypical clinical and radiological presentations.
Suppressive effect of L‐dopa on dopamine cells remaining in the ventral tegmental area of rats previously exposed to the neurotoxin 6‐hydroxydopamine
Authors: Blunt S. B. , Jenner P. , Marsden C. D.
Published: December 2004

Ever since the introduction of levo‐3, 4‐dihydroxyphenylalanine (L‐dopa) for the treatment of Parkinson's disease, there has been concern that it might accelerate the degeneration of dopamine neurones. Using rats with a unilateral 6‐hydroxydopamine (6‐OHDA) lesion of the medial forebrain bundle (MFB), we have studied the effect of chronic L‐dopa treatment on the survival of dopamine cells which remain in the ventral tegmental area (VTA) ipsilateral to a 6‐OHDA lesion. Following lesion surgery, rats were treated with L‐dopa and carbidopa administered in the drinking water for 27 weeks. At the end of the treatment period, the number of dopamine cells remaining in each of the lesioned and intact substantia nigra (SN) and VTA were assessed, using tyrosine hydroxylase immunohistochemistry. Chronic L‐dopa treatment resulted in an apparent reduction in the number of dopamine neurones remaining in the VTA ipsilateral to the lesion, whereas it had no effect on the number of dopamine cells remaining in the intact SN and VTA. This finding suggests a possible suppressive effect in vivo of L‐dopa on dopamine cells in the midbrain of adult animals that have been previously exposed to 6‐OHDA.
PET study on striatal dopamine D2 receptor changes during the progression of early parkinson's disease
Authors: Rinne J. O. , Laihinen A. , Rinne U. K. , Någren K. , Bergman J. , Ruotsalainen U.
Published: December 2004

[11C] Raclopride uptake to dopamine D2 receptors was investigated with positron emission tomography (PET) in patients with early Parkinson's disease at the time of the diagnosis and after a half‐year interval. During this progressive period of the disease, the patients received no antiparkinsonian medication. The upregulation of striatal D2 receptors, which was seen in all patients already at the time of the diagnosis, persisted. Although the patients initially showed unilateral disease, they had developed bilateral symptoms by the time of the second PET scan, but the disease was still asymmetric. The present results show that the relative increase in [11C]raclopride uptake in the striatum contralateral to the symptoms as compared with the opposite striatum will be preserved even during the progression of the disease, provided that the symptoms show clear‐cut asymmetry.
Effect of adding the D1 agonist CY 208–243 to chronic bromocriptine treatment. I: Evaluation of motor parameters in relation to striatal catecholamine content and dopamine receptors
Authors: Gomez‐Mancilla Baltazar , Boucher René , Gagnon Céline , Di Paolo Thérèse , Markstein Rudolf , Bédard Paul J.
Published: December 2004

A group of four cynomolgus monkeys previously rendered parkinsonian by the toxin 1‐methyl‐4‐phenyl, 1, 2, 3, 6‐tetrahydropyridine (MPTP) were observed in locomotion cages equipped with photocells during four periods of 7 days during which they received saline or two doses of the D1 agonist CY 208–243. The larger dose of 0.5 mg/kg produced a singificant increase in locomotion in three of four animals. A second group of eight monkeys also previously rendered parkinsonian by MPTP and having received no other treatment were given a daily treatment of bromocriptine 1.66 mg/kg orally daily during 4 weeks. In four of the animals, after a week on bromocriptine alone, the D1 agonist CY 208–243 was added in increasing doses of 0.05, 0.1, and 0.5 mg/kg. The motor response as measured by locomotion, hand dexterity, and a disability score improved progressively at least in some of the animals on bromocriptine alone. The addition of CY 208–243 produced a more striking improvement of all three parameters, which appeared to be dose dependent. Biochemical analysis of the brain of these animals plus one control and one MPTP untreated monkey showed a<90% loss of dopamine in the striatum in six of the eight treated monkeys. Both D2 and D1 dopamine receptors were increased in density by denervation, but both treatments abolished this increase for the D2 receptors while increasing the affinity of the D1 receptors.
Sympathetic skin response and R–R interval variation in parkinson's disease
Authors: Wang Shuu Jiun , Fuh Jong Ling , Shan Ding E , Liao Kwong Kum , Lin Kon Ping , Tsai Ching Piao , Wu Zin An
Published: December 2004

We investigated autonomic function in patients with idiopathic Parkinson's disease (PD) by measuring sympathetic skin response (SSR) and R‐R interval variation (RRIV). Sixty‐two PD patients and 62 age‐matched normal subjects were recruited. Abnormal SSR was noted in nine (14.5%) PD patients, including three in Stage II, three in Stage III, and Three in Stage IV, but not in Stage I patients or normal subjects. Four of these nine patients had postural hypotension. Abnormal SSR was correlated with duration of illness and impotence. In PD patients, abnormal SSR may be due to intermediolateral column dysfunction. After logarithmic transformation and age adjustment, 19 (31.6%) of 60 PD patients had abnormal RRIV during rest and deep breathing. Abnormal RRIV was not related to staging or duration of illness. Patients with constipation had significantly lower RRIV, indicationg parasympathetic dysfunction. RRIV was not affected by acute or chronic L‐dopa treatment. The agreement between RRIV and SSR in PD patients was poor (k = −0.07). It appears that abnormal SSR, but not RRIV, may be associated with more autonomic disturbances in PD patients.
Apomorphine test for dopaminergic responsiveness: A dose assessment study
Authors: Bonuccelli U. , Piccini P. , Dotto P. Del , Rossi G. , Corsini G. U. , Muratorio A.
Published: December 2004

The clinical diagnosis of idiopathic Parkinson's disease (IPD) remains difficult and is supported by a favorable response to levodopa, while failure to respond represents an exclusion criterion. Recently, the response to subcutaneous apomorphine has been suggested as a tool in predicting levodopa responsiveness in parkinsonian syndromes. We administered apomorphine at doses of 10, 50, and 100 μg/kg dose. Because of the high frequency of side effects with the dose of 100 μg/kg, 50 μ/kg seems more useful. After an adequate period of levodopa/carbidopa therapy (12‐month follow‐up), 29 patients improved; 25 of these had demonstrated a positive response to the apomorphine test. The final diagnosis of IPD, made on the basis of an exhaustive clincal and neuroradiological evaluation and on the response to chronic levodopa therapy, was in good agreeement with the response to the apomorphine test (predictivity of diagnosis, 86.4%). Our data indicate that subcutaneous apomorphine at the dose of 50 μg/kg is a useful tool in the differential diagnosis of parkinsonian syndromes.
Subcutaneous apomorphine in parkinson's disease: Response to chronic administration for up to five years
Authors: Hughes A. J. , Bishop Susan , Kleedorfer Birgit , Turjanski Nora , Fernandez W. , Lees A. J. , Stern G. M.
Published: December 2004

Subcutaneous apomorphine, administered by continuous waking‐day infusion with boluses, or by repeated intermittent injection, was given to 71 parkinsonian patients with severe refractory levodopa related on‐off flutuations for 1–5 years. A mean reduction in daily off period time of ∼50% was maintained, and the incidence of neuropsychiatric toxicity remained low on long‐term follow‐up. No clinically significant tolerance or loss of therapeutic effect was seen, although increasingly severe on‐phase dyskinesias and postural instability marred the long‐term therapeutic response in many patients. Despite these drawbacks, apomorphine, when combined with the peripheral dopamine receptor agonist domperidone, represents a significant therapeutic advance in the management of late‐stage Parkinson's disease and should certainly be considered before experimental implantation procedures.
Nocturnal akathisia in parkinson's disease: Treatment with clozapine
Authors: Linazasoro G. , Massó J. F. Martí , Suárez J. A.
Published: December 2004

We present the results obtained using low doses of clozapine (mean dose 26.4 mg at bedtime) in the treatment of noctural akathisia in nine patients with Parkinson's disease for a mean period of 12.5 months. The results were excellent in all the patients. Furthermore, three patients experienced a remarkable improvement in rest tremor and in five patients the confusional state that accompanied the akathisia also disappeared. No serious side‐effects were observed. We believe that clozapine is a very useful drug for the relief of nocturnal akathisia in parkinsonian subjects.
Assessment of adaptive functioning in huntington's disease
Authors: Bylsma Frederick W. , Rothlind Johannes , Hall Michelle R. , Folstein Susan E. , Brandt Jason
Published: December 2004

The Huntington's Disease Activities of Daily Living (HD–ADL) scale is a 17‐item informant‐completed instrument for rating adaptive functioning in Huntington's disease (HD) patients. To assess the reliability and clinical correlates of the HD–ADL, it was administered along with the Mini‐Mental State Exam (MMSE) and Quantified Neurological Exam (QNE) to 93 HD patients. The HD‐ADL has high internal consistency (coefficient alpha = 0.91). Principal components factor analysis of the HD‐ADL revealed four factors (General Functioning, Domestic Activities, Home Upkeep, and Family Relationships) accounting for 72% of the total variance. MMSE score, motor impairment score, and duration of disease were the best predictors of HD‐ADL score. Similar results were obtained in an independent replication sample of patients. The HD‐ADL scores correlate with Shoulson and Fahn's total functional capacity (TFC) index (r = −0.89). The HD‐ADL scale is a reliable and valid instrument for assessing adaptive function in HD patients.