bluuebunny/crossref_metadata_2025 huggingface dataset:
Benign neonatal sleep myoclonus: Clinical features and video‐polygraphic recordings
Authors: Di Capua Matteo , Fusco Lucia , Ricci Stefano , Vigevano Federico
Published: December 2004

Benign neonatal sleep myoclonus is a syndrome characterized by the occurrence of repetitive myoclonic jerks of the extremities exclusively during non–rapid eye movement sleep in the early life of healthy newborns. No etiological factors are present. The onset is within the first 15 days of life with spontaneous disappearance within 3–4 months. These myoclonic events are commonly diagnosed as epileptic seizures. We observed 12 newborns with this clinical pattern; the follow‐up ranges from 12 to 60 months. Long‐term videopolygraphic electroencephalographic (EEG) monitoring demonstrated normal EEG activity, thus confirming that these unusual events were nonepileptic.
Adult onset myoclonic Huntington's disease
Authors: Carella F. , Scaioli V. , Ciano C. , Binelli S. , Oliva D. , Girotti F.
Published: December 2004

A patient with adult onset Huntington's disease (HD) and prominent action myoclonus is described. Neither epileptiform activity nor electroencephalography (EEG) correlates of the movements was found. Unlike the case with most (nonmyoclonic) HD patients, centro‐parietal components of somatosensory evoked potentials (SEPs) were well defined and a clear V2 response was found. Treatment with valproic acid greatly reduced myoclonus suggesting that the σ‐aminobutyric acid (GABA) system might be involved in the pathophysiology of myoclonus in HD.
A case of posttraumatic tremor treated by chronic stimulation of the thalamus
Authors: Broggi G. , Brock S. , Franzini A. , Geminiani G.
Published: December 2004

We describe a case in which tremor associated with unilateral bradykinesia due to craniocerebral trauma was successfully treated by chronic stimulation of the thalamic ventralis intermedius nucleus.
MR imaging findings of tremors associated with lesions in cerebellar outflow tracts: Report of two cases
Authors: Nakamura Ryohji , Kamakura Keiko , Tadano Yutaka , Hosoda Yoshito , Nagata Naokazu , Tsuchiya Kazuhiro , Iwata Makoto , Shibasaki Hiroshi
Published: December 2004

Two cases with severe tremors were studied by means of electromyograms using surface electrodes and also by magnetic resonance (MR) imaging. The first case was associated with multiple sclerosis and demonstrated a severe postural cerebellar tremor and an alternate activation of antagonist muscles in the right arm. The second case, with hemorrhage in the brainstem, demonstrated a severe tremor at rest and mixed synchronous and alternating activation of antagonist muscles in the left forearm. MR imaging studies localized lesions possibly responsible for these tremors. In the first case a lesion was located in the superior cerebellar peduncle just under the decussation, and in the second case a lesion was found between the red nucleus and the thalamus, with possible involvement of both the cerebellothalmic and nigrostriatal pathways. The first case accords with the theory that a lesion located in the dentate nucleus and its projection can cause severe postural cerebellar tremor. The lesion demonstrated in the second case may be responsible for “cerebellar tremor at rest” or “static (resting) cerebellar tremor.”
Function of dopamine receptors in young‐onset parkinson's disease: Prolactin response
Authors: Kostic Vladimir S. , Marinkovic Zorica , Filipovic Sasa , Momcilovic Dragana
Published: December 2004

The basal levels of prolactin (PRL) and their changes after i. v. thyrotropin releasing hormone (TRH) administration after bromocriptine (BCT) pretreatment (BCT/TRH test) were monitored in 12 patients with young‐onset Parkinson's disease (PD) (before 40 years of age) and 10 patients with older‐onset PD (after 40 years of age), as well as in two groups of healthy subjects (10 persons in each), age‐matched with older‐onset (control A) and young‐onset (controlB) parkinsonians. The basal PRL levels were normal in both groups of patients. When given after BCT, TRH induced a significantly lower PRL increase in older‐onset parkinsonians than in controls. This response was even more blunted in young‐onset patients, being significantly more attenuated than in older‐onset PD patients.
Early combination therapy with bromocriptine and levodopa in parkinson's disease
Authors: Factor Stewart A. , Weiner William J.
Published: December 2004

The use of early combination therapy with bromocriptine (Br) and levodopa (LD) in Parkinson's disease is controversial. It has been suggested that treatment with this regimen would prevent or delay the onset of motor fluctuations and dyskinesia. Thus, some have recommended it as a standard of care. This recommendation is based on the theory that LD may accelerate the progression of PD and clinical experience using Br monotherapy in early Parkinson's disease, which suggested that Br causes fewer late complications. This article reviews these arguments and shows that the theories are uproven. A single, uncontrolled trial is often referred to as evidence for efficacy of early combination therapy. We critically review this and five other studies which have evaluated the treatment strategy. We show that the literature is often misleading and that these trials do not support the efficacy of early combination therapy. We conclude that there is no justifiable reason to use a combination of Br and LD in early parkinsonian patients.
Lewy bodies in the lateral hypothalamus: Do they imply neuronal loss?
Authors: Kremer H. P. H. , Bots G. Th. A. M.
Published: December 2004

Lewy bodies have been found in the hypothalamic lateral tuberal nucleus (NTL) and the adjoining tuberomammillary nucleus (TM) in Parkinson's disease (PD). The NTL is severely atrophic in Huntington's disease; the TM seems unaffected. In this study, we examined we examined the NTL and the TM of seven PD patients and one patient with presumed PD to assess whether the presence of Lewy bodies indicated neuronal loss. Most Lewy bodies were found in the TM, but they were also present in the NTL of seven of the eight patients. The number of NTL neurons in the PD patients was similar to a group of 14 nonneurological controls, seven Alzheimer's disease (AD) patients, and two AIDS patients with dementia. This challenges the hypothesis that Lewy bodies are a sign of significant cell death. The TM, whose cells could not be counted, did not seem depleted in neuronal numbers, although occasional neuronophagia was observed.
Axial motor disturbances after hypoxic lesions of the globus pallidus
Authors: Fève A. P. , Fénelon G. , Wallays C. , Rémy P. , Guillard A.
Published: December 2004

Four subjects aged between 29 and 60 years were examined because of axial motor impairment after hypoxic brain injury. Magnetic resonance imaging revealed circumscribed lesions of the globus pallidus in every case. The association of freezing of the gait, speech disorders, axial bradykinesia, and postural disturbances, with no rigidity or tremor and little or no distal akinesia, suggests a role of the globus pallidus in controlling axial motion.
Laterality of onset in idiopathic torsion dystonia
Authors: Inzelberg Rivka , Ziber Nelly , Kahana Esther , Korczyn Amos D.
Published: December 2004

Idiopathic torsion dystonia (ITD) is a dominantly inherited disorder with incomplete penetrance. It is important to identify factors that may cause dystonia or prevent its occurrence in a genetically predisposed individual. Because dystonia may be precipitated by peripheral triggers, we have investigated whether the preferential use of a limb affects the development of dystonia. Analysis of the correlation between the side of motor dominance and the limbs in which dystonic symptoms first appeared was performed in 49 patients with ITD ascertained in a country‐wide survey in Israel. The dominant motor side was determined in 45 cases (92%). Among 29 patients with lateralized limb onset, 24 showed right‐side motor dominance, of whom 21 had dystonia onset in a right limb. The first sign was in a left limb for all five cases with left‐side motor dominance (90% coincidence). The pattern of limb involvement was studied. Detection bias could be ruled out. The highly significant relationship between the motor dominance and the laterality of limb onset in ITD patients suggests that the preferred use of a limb may trigger the onset of dystonia.
Geniospasm: Hereditary chin trembling
Authors: Danek Adrian
Published: December 2004

Geniospasm is a hereditary disorder characterized by episodic, usually stress‐induced involuntary trembling of the chin. Nineteen families from Europe and the U.S.A. suffering from this disorder have been described since 1894. We present three cases from two newly detected families. There was no evidence of any other nervous system abnormality, although abnormal EEG, sleep disorders, and involvement of other facial muscles have been described in rare cases. Geniospasm is transmitted as an autosomal‐dominant trait with high penetrance. It must be differentiated from facial myokymia, palatal tremor, and essential tremor affecting facial muscles. Neurophysiological and molecular mechanisms of this peculiar disorder are unknown.
Cisapride treatment of constipation in parkinson's disease
Authors: Jost W. H. , Schimrigk K.
Published: December 2004

Constipation, a frequent symptom in Parkinson's disease (PD), is probably caused by degeneration of the autonomic nervous system, particularly the myenteric plexus. Cisapride is a drug that causes increased release of acetylcholine in the myenteric plexus. In a pilot study, cisapride therapy was investigated in 20 PD patients, 10 women and 10 men, who suffered from delayed intestinal transit. In all cases, cisapride therapy was associated with a significant acceleration of colonic transit, as measured by radioopaque pellets viewed on radiographs. Pellet count fell from a mean of 53.8 pretreatment to 30.4 after cisapride treatment. No adverse reaction and no “overshoot effects,” such as diarrhea, were seen. Our findings suggest that cisapride may alleviate the constipation associated with Parkinson's disease.
Visual and auditory evoked potentials in early onset parkinson's disease and their relationship to cerebrospinal fluid monoamine metabolites
Authors: Muthane Uday B. , Satishchandra P. , Subhash M. N.
Published: December 2004

We studied visual (VEP) and brainstem auditory (BAEP) evoked potential changes in 23 patients with early onset Parkinson's disease (EOPD) to establish the nature of the changes as well as their relationship to dopaminergic (DA) and serotonergic (5‐HT) disturbances, as determined by cerebrospinal fuid levels of homovanillic acid (HVA) and 5‐hydroxyindoleacetic acid (5‐HIAA). We also compared these parameters between the young onset (YOPD) and juvenile Parkinsonism (JP), the two subgroups of EOPD, to look for any possible differences between the two. In EOPD, the mean P100 latency of the VEP was significantly prolonged compared to controls (p < 0.001). However, within EOPD the evoked potential parameters were not significantly different between YOPD and the JP subgroups. P100 latency was abnormal in six patients (YOPD: 5, JP:1) (26%). Six patients (YOPD: 3, JP:3) (26%) had abnormal BAEP. A significant negative correlation (r: −0.89, p <1%) was observed between the P100 latency and CSF HVA levels. No correlation was observed between the BAEP interpeak latencies and either CSF HVA or 5‐HIAA levels. This study suggests that VEP and BAEP abnormalities do occur in EOPD (in both YOPD and JP), and that the prolongation of P100 latency is secondary to DA deficiency as in PD. The cause of BAEP abnormalities in EOPD. There was no correlation between the VEP or BAEP changes to either the age at onset or duration of EOPD.
Modulatory effect of clozapine on levodopa response in parkinson's disease: A preliminary study
Authors: Arevalo Gonzalo J. Gomez , Gershanik Oscar S.
Published: December 2004

Clozapine has been shown not only to be effective in ameliorating dopaminomimetic psychosis but to improve parkinsonian symptomatology. Six parkinsonian patients with motor fluctuations under levodopa treatment and severe interdose “off” periods (believed to be mediated by an inhibitory effect of subthreshold levels of levodopa) underwent a trial of clozapine. The effects of this drug on levodopa response were measured by means of an acute levodopa test both before and after receiving clozapine. After 1 month of treatment, clozapine 25 mg/day reduced parkinsonian scores at all stages of the evaluation (pre‐levodopa “off,” “on,” and interdose “off”). The effect was consistently more significant for the interdose “off.” Clozapine could be exerting its beneficial effects through the inhibition of an inhibitory effect mediated by low‐level dopaminergic stimulation, thus behaving as an apparent antiparkinsonian drug.
Inverse masticatory muscle activity due to syringobulbia
Authors: Merello Marcelo , Lees Andrew J. , Leiguarda Ramon , Guevara Juan , Nogués Martin
Published: December 2004

The clinical, radiological, and electrophysiological data of a 25‐year‐old woman with “inverse masticatory muscle activity” due to syringobulbia is presented. This uncommon brain stem syndrome may be due to disturbance in central programming of mastication.
Significant improvement of stiff‐person syndrome after paraspinal injection of botulinum toxin A
Authors: Davis Dave , Jabbari Bahman
Published: December 2004

Following several months of low back pain, a 36‐year‐old developed progressive stiffness of the abdominal, low back, and thigh muscles. On examination, these muscles demonstrated marked hypertonia consistent with the clinical diagnosis of stiff‐person syndrome. The patient demonstrated increased lumbar lordosis and had focal hyperhidrosis at different sites. Electromyography showed continuous activity of the paraspinal and thigh muscles, and serum and cerebrospinal fluid antibodies to glutamic acid decarboxylase (GAD) were markedly elevated. Diazepam and Lioresal offered partial pain relief. Paraspinal muscle administration of botulinum toxin A reduced the tone of paraspinal and thigh muscles significantly and resulted in marked improvement of ambulation and cessation of pain.
Painless legs and moving toes: A syndrome related to painful legs and moving toes?
Authors: Walters Arthur S. , Hening Wayne A. , Shah Shashi K. , Chokroverty Sudhansu
Published: December 2004

The syndrome of painful legs and moving toes consists of continuous or semicontinuous involuntary writhing movements of the toes associated with pain in the affected extremity. We report a 57‐year‐old man with a 33‐year history of painless and semicontinuous involuntary movements of the toes of the left foot similar to those seen in painful legs and moving toes. There was no family history of movement disorder. The history and physical examination were negative for significant trauma, radiculopathy, or peripheral neuropathy. There were no other neurological findings or involuntary movements. It is unlikely that the involuntary movements were precipitated by neuroleptics or psychosis. CT scan of the head; EEG, CT, and MRI scans of the lumbosacral spine; and EMG and nerve conduction studies of the legs showed no significant abnormalities except for a predominant cocontraction of the left foot flexors and extensors at 0.6–1.2 Hz in a pattern sometimes seen in painful legs and moving toes. We conclude that there is a condition clinically and electrophysiologically similar to painful legs and moving toes that we call painless legs and moving toes, the etiology of which remains undetermined.
Brain glucose metabolism and dopamine D2 receptor analysis in a patient with hemiparkinsonism‐hemiatrophy syndrome
Authors: Przedborski S. , Goldman S. , Levivier M. , Giladi N. , Bidaut L. M. , Hildebrand J. , Stanus E. , Labar D. , Luxen A.
Published: December 2004

We report findings on brain glucose metabolism and dopamine D2 receptors generated by positron emission tomography (PET) in a 67‐year‐old woman with right hemiparkinsonism‐hemiatrophy syndrome (HP‐HA). PET with [18F]‐fluorodeoxy‐glucose (FDG) showed marked glucose metabolism asymmetry. There were significant reductions in glucose uptake at the level of the basal ganglia and, to lesser extent, in the fronto‐parietal cortex contralateral to the clinically involved side. These changes were different from those found in a patient with hemi‐Parkinson's disease who was scanned under similar conditions. Because the patient with HP‐HA had only minimal response to levodopa therapy, we evaluated post‐synaptic dopaminergic structures using PET with [18F]‐fluoroethylspiperone (FESP). No striatal binding asymmetry was found in FESP/PET, which suggests a sparing of striatal dopamine D2 receptors. The changes in FDG uptake which we found were in brain areas relevant to the clinical features of HP‐HA syndrome. In addition, our study provides evidence that FDG/PET may help to differentiate HP‐HA syndrome from hemi‐Parkinson's disease. In most instances, since HP‐HA is associated with a more benign clinical course than Parkinson's disease, this distinction is of clinical important.
The subthalamic nucleus: A possible target for stereotaxic surgery in parkinson's disease
Authors: Guridi J. , Luquin M. R. , Herrero M. T. , Obeso J. A.
Published: December 2004

Hyperactivity in the subthalamic nucleus (STN) projections to the globus pallidus medialis (GPM) has been established as a crucial feature of parkinsonism in animal models of Parkinson's disease (PD). Recent experiments blocking the STN glutamatergic output to GPM or lesioning the STN support this concept by showing a dramatic reversal of parkinsonism. We review the role of stereotaxic surgery in the past and the possible future application of subthalamotomy for PD.
Pre‐ and postcentral cortical somatosensory evoked potentials in hemiparkinsonism
Authors: Huttunen J. , Teräväinen H.
Published: December 2004

We recorded cortical frontal, central, and parietal somatosensory evoked potentials (SEPs) in 9 patients with hemiparkinsonism and in a group of 25 healthy volunteers. No differences were observed in the SEPs recorded after stimulation of the asymptomatic and symptomatic sides in the patients. Likewise, comparison with the healthy controls did not reveal significant group differences or abnormal waveforms in the patients. Even frontal N30 deflection, which has been reported to be diminished in Parkinson's disease (PD), was normal and symmetric in the patients. Therefore, no evidence was found for altered sensory input to the motor or premotor and supplementary motor cortices in PD.
Increased striatal glucose consumption in sydenham's chorea
Authors: Weindl A. , Kuwert T. , Leenders K. L. , Poremba M. , GräFin von Einsiedel H. , Antonini A. , Herzog H. , Scholz D. , Feinendegen L. E. , Conrad B.
Published: December 2004

Positron emission tomography and 18F‐fluorodeoxyglucose were used to measure the regional cerebral glucose consumption in a 15‐year‐old choreatic girl with classical Sydenham's chorea shortly after the onset of hyperkinetic movements and 5 months later after chorea had resolved and in a 74‐year‐old hemichoreatic woman with long‐standing hyperkinesia as a residuum of Sydenham's chorea in adolescence. Whereas cerebellar, thalamic, and cortical glucose consumption was within normal limits in both patients, lentiform and caudate glucose consumption was significantly increased in both hemispheres of the 15‐year‐old patient and in the hemisphere contralateral to the chorea in the 74‐year‐old patient. In the younger patient, striatal glucose consumption returned to normal after her hyperkinesia had disappeared with antibiotic therapy. The observation of an increase in striatal glucose consumption in Sydenham's chorea, in contrast to the decrease of this variable encountered in the vast majority of other choreatic disorders, leads to questioning the pathophysiology of chorea in humans and suggests the use of emission tomographic measurement of variables related to cerebral energy metabolism for differential diagnosis in choreatic disorders.
Correlation of clinical response in apomorphine test with D2‐receptor status as demonstrated by 123I IBZM‐SPECT
Authors: Schelosky L. , Hierholzer J. , Wissel J. , Cordes M. , Poewe W.
Published: December 2004

The knowledge of functional capacities of postsynaptic dopaminergic receptors in parkinsonian syndromes is important for differential diagnosis and for planning therapeutic approaches. Subcutaneous apomorphine challenges serve as a pharmacological tool in testing dopaminergic responsiveness, but discrepancies between results of the apomorphine test and long‐term levodopa treatment remain. 123I IBZM (I‐123 labeled iodobenzamide) as a dopaminergic receptor ligand allows depiction of D2‐receptors by means of SPECT methods. The correlation between dopaminergic responsiveness and D2‐receptor status as demonstrated by 123I IBZM‐SPECT imaging was assessed by applying an apomorphine test to 41 patients with parkinsonian syndromes. All subsequently underwent an 123I IBZM‐SPECT. Apomorphine responders showed a significantly higher binding of 123I IBZM than nonresponders, and patients with idiopathic Parkinson's disease (IPD) had higher D2‐receptor density as visualized by SPECT than patients with other parkinsonian syndromes. The marked overlap between the groups allowed a reliable prediction only in patients with an abnormally low basal ganglia/frontal cortex ratio of 123I IBZM binding.
Relief of akinesia by apomorphine and cerebral metabolic changes in parkinson's disease
Authors: Broussolle E. , Cinotti L. , Pollak P. , Landais P. , Le Bars D. , Galy G. , Lavenne F. , Khalfallah Y. , Chazot G. , Mauguière F.
Published: December 2004

The cerebral metabolic rate of glucose was measured in 14 Parkinson's disease patients with severe on‐off fluctuations. Two positron emission tomography (PET) scans with [18F]fluorodeoxyglucose were performed, one after a challenge of subcutaneous apomorphine at a dose able to relieve akinesia within 15 min and the other with the vehicle. Apomorphine reduced glucose utilization by 4‐6% in the lenticular nuclei and the occipital cortex and by 6‐9% in the thalamic nuclei, but this effect was not statistically significant. Thus, central stimulation of dopamine receptors by apomorphine in advanced Parkinson's disease is not associated with cerebral methabolic changes as assessed by PET. Despite a dramatic improvement of the motor state, the global neuronal activity in the striatum and its downstream projections remains stable, suggesting an equilibrium between excitatory and inhibitory dopaminergic activities.
Terguride in fluctuating parkinsonian patients: A double‐blind study versus placebo
Authors: Pacchetti C. , Martignoni E. , Bruggi P. , Godi L. , Aufdembrinke B. , Miltenburger C. , Voet B. , Nappi G.
Published: December 2004

Terguride (TER), a semisynthetic derivative of lisuride, has been found to display dopamine (DA) agonist and DA antagonist effects in animals, depending on the experimental model used. TER (2 mg/day) was compared to placebo in 41 fluctuating Parkinson's disease patients to test its effect on akinesia and dyskinesia. Mean hours “off” decreased at weeks 6 and 12 (p < 0.05) in the TER group but the overall difference from the placebo group was not significant. Only the TER group displayed a decrease over time in mean Columbia University Rating Scale total score “on” and “off” (p = 0.001 and p = 0.03, respectively). Duration of involuntary movements and resulting disability were not significantly different between patients on TER and those on placebo administration. In the overall evaluation, patients preferred TER (p = 0.01). Tolerance of TER was very good in all but one patient whose wearing‐off increased; no one dropped out because of side effects. This 3‐month doubleblind study showed that TER, added to stable doses of L‐dopa, may have slight antiparkinsonian efficacy.
Reliability of the columbia scale for assessing signs of parkinson's disease
Authors: Hely M. A. , Chey T. , Wilson A. , Williamson P. M. , O'Sullivan D. J. , Rail D. , Morris J. G. L.
Published: December 2004

Inter‐ and intrarater reliability in scoring the signs of Parkinson's disease using the original Columbia scale and a modified version of this, the Sydney scale, were assessed in five neurologists participating in a long‐term study of Parkinson's disease. Scoring was done on video recordings of 41 patients whose disability ranged from mild to severe. Although all the neurologists were familiar with the scales and had received training designed to produce uniformity of scoring, interrater reliability was poor. The mean score for the Columbia scale varied from 18.6 to 30 and for the Sydney scale from 15.2 to 23.2. By contrast, intrarater reliability was good. This study highlights the limitations of clinical rating scales in Parkinson's disease when more than one rater is used. In designing clinical trials, every effort should be made to ensure that the same patient is always assessed by the same rater.
Reversal of reserpine‐induced catalepsy by selective D1 and D2 dopamine agonists
Authors: Hubbard C. Anthony , Trugman Joel M.
Published: December 2004

To gain insight into the antiparkinsonian effects of selective D1 and D2 dopamine receptor stimulation, we examined the ability of D1 (SKF 38393) and D2 (quinpirole) agonists to reverse catalepsy induced by the combined administration of reserpine and α‐methyl‐p‐tyrosine (AMPT) in rats. Catalepsy, the failure to correct an externally imposed posture, is a measure of akinesia and was assessed using the bar test. Rats injected with reserpine alone (2.5 mg/ kg i.p.) developed akinesia and ptosis within 60‐90 min. The D1 agonist SKF 38393 (30 mg/ kg i.v.) rapidly reversed ptosis and restored near‐normal mobility when administered 24 h after reserpine and AMPT; catalepsy was reversed for 90 min, after which the drug effect wore off. Quinpirole (1 mg/ kg i.v.) reversed catalepsy for the duration of the test period (4 h) but did not consistently reverse ptosis or promote normal mobility; the rats continued to exhibit kyphotic postures with little spontaneous locomotion. These results indicate that selective D1 stimulation is sufficient to reverse reserpine‐induced akinesia and highlight the need for the development of potent selective D1 agonists for clincal traial in Parkinson's disease. In serve dopamine depletion, D2 stimulation alone appears to be insufficient to restore normal movement. Quinpirole, but not SKF 38393, elicited paroxysmal limb/ body jerking in reserpine‐AMPT‐treated rats, providing further evidence that atypical jerking can be elicited by D2 stimulation in the complete absence of D1 stimulation. This laboratory observation suggests that some jerking dyskinesias seen in treated parkinsonian patients may be mediated by an imbalance in D1‐D2 receptor stimulation.
Anxiety and motor performance in parkinson's disease
Authors: Siemers Eric R. , Shekhar Anantha , Quaid Kimberly , Dickson Hal
Published: December 2004

Previous work has suggested that anxiety disorders are common in patients with Parkinson's disease. To study the relationship between anxiety and response fluctuations in Parkinson's patients further, we compared change in Spielberger anxiety state scores with changes in parkinsonian disability as determined by the Parkinson's symptom diary (PSD). Anxiety state and PSD scores were obtained in 19 patients with idiopathic Parkinson's disease during on and off periods. Spielberger anxiety state scores were higher during off periods than during on periods (38.8 ± 12.4 vs. 45.6 ± 12.4, p < 0.03); further, the magnitude of the change in anxiety state scores was correlated with the change in PSD scores (rs= 0.616, p = 0.006). Spielberger anxiety trait scores were also correlated with disease duration. Our findings support existing data suggesting that anxiety can contribute significantly to morbidity in Parkinson's disease and suggest that anxiety varies with fluctuations in motor performance.
Relationship between osteopenia and clinical characteristics of parkinson's disease
Authors: Ishizaki Fumiko , Harada Toshihide , Katayama Sadao , Abe Haruo , Nakamura Shigenobu
Published: December 2004

Pathological bone changes affect locomotor activity and may influence the outcome and prognosis of Parkinson's disease (PD). In this study, we determined the relationship between bone changes and PD. Bone study was performed by multiple scanning x‐ray photodensitometry (MD/MS) in 64 patients with PD and 42 age‐matched controls. We then compared the results with the clinical characteristics of PD. Osteopenia was detected in 22 (53.6%) of the 41 female and 6 (26%) of the 23 male patients, and in 6 (26%) of the 23 female and 2 (10.5%) of the 19 male controls. The frequency of osteopenia was significantly greater in the female patients than in the male patients or the female controls. Osteopenia was related to the duration of PD in the men, but not in the women. Twenty of 40 PD patients' hands showed side‐related differences in the analysis of both hands. In 19 of the 20 patients, the side of more severe osteopenia coincided with that of parkinsonian symptoms, suggesting that osteopenia is related to the pathophysiology of PD.
Effects of thalamic stimulation on tremor, balance, and step initiation: A single subject study
Authors: Burleigh A. L. , Horak F. B. , Burchiel K. J. , Nutt J. G.
Published: December 2004

This study was conducted to evaluate the clinically apparent balance improvements in a patient with Parkinson's disease who had stimulating electrodes surgically implanted to the VIM nucleus of the right thalamus for control of left‐upperextremity tremor. Experiments were conducted to determine if balance improved simply because the large‐amplitude upper‐extremity tremor was reduced or if the neural control of the thalamic stimulation on the contralateral upper‐extremity tremor and on the lowerextremity postural muscle activatios for quiet stance, step initiation, and equilibrium responses to surface displacements. The results demonstrated that, beside reducing the amplitude and destabilizing effects of the upper‐extremity tremor, the thalamic stimulation was also effective in reducing tremor activity of the trunk and contralateral lowerextremity muscles. In addition, the contralateral lower‐extremity muscle activation patterns, strengths, and durations for the balance tasks were enhaced during stimulation. These results suggest that thalamic stimulation improved this patient's balance by reducing tremor in the contralateral extremities and by increasing burst duration and magnitude of the tibialis anterior, which functions as the postural prime mover for the step initiation and balance tasks.
Histologic assessment of dose‐related diffusion and muscle fiber response after therapeutic botulinum a toxin injections
Authors: Borodic Gary E. , Ferrante Robert , Pearce L. Bruce , Smith Karen
Published: December 2004

Fiber diameter variability, acetylcholinesterase staining properties, and average fiber diameter were determined 5 weeks after varying doses of botulinum A toxin were administered into albino rabbit longissimus dorsi muscle. The average fiber diameter within the muscle appeared to be a function of the dose of botulinum toxin injected. Fiber diameter variability correlated with the dose of botulinum toxin administered. Both fiber diameter variability and acetylcholinesterase spread characteristics showed a distinct diffusion gradient over a defined field within a muscle. At lower doses (1 IU), collapse of the diffusion gradient occurred over a 15–30‐mm segment of muscle. At higher doses (5–10 IU), diffusion of botulinum A toxin effect occurred throughout the entire muscle with no apparent end point. This study demonstrated that botulinum A toxin produces a gradient of denervation in a given muscle and that both the magnitude of denervation and the extent of the gradient are dose dependent. Furthermore, both muscle fiber diameter variability and acetylcholinesterase staining were useful as measures of chemodenervation.
A case‐control study of Parkinson's disease in a horticultural region of British Columbia
Authors: Hertzman Clyde , Wiens Michele , Snow Barry , Kelly Shona , Calne Donald
Published: December 2004

We compared personal histories of 127 cases and 245 controls to identify possible environmental risk factors for idiopathic parkinsonism (IP). Of our controls, 121 had cardiac disease (CD) and 124 were randomly selected from electoral lists (voters). Using logistic regression and adjusting for sex and age, we ran separate analyses: IP versus CD and IP versus voters. A full occupational history was collected, as was known contact with all pesticides associated with the tree fruit sector of the agricultural industry. We found a significant association between IP and having had an occupation in which exposure through handling or directly contacting pesticides was probable, but no specific chemicals were associated with IP. We conclude that although occupations involving the use of agricultural chemicals may predispose to the development of IP, it seems likely that the pathogenesis is multifactorial rather than related to a specific agent.
Unified Parkinson's disease rating scale characteristics and structure
Authors: Martínez‐Martín P. , Gil‐Nagel A. , Gracia L. Morlán , Gómez J. Balseiro , Martínez‐Sarriés J. , Bermejo F. ,   The Cooperative Multicentric Group
Published: December 2004

Our purpose was to verify some basic aspects of validation of the Unified Parkinson's Disease Rating Scale (UPDRS). One hundred and sixtyseven Parkinson's disease (PD) patients were included. Group A (n=40) was simultaneously assessed by five raters who applied the UPDRS and other PD rating scales (PDRS). A set of timed tests, the Mini‐Mental State Examination (MMSE), and the Hamilton Scale for Depression (HSD) were administered by an independent examiner. Group B (n = 127) was individually assessed through the UPDRS and the other PDRSs by one neurologist in four different hospitals. The UPDRS was administered in 16.95 ± 7.98 min. The internal consistency was high (Cronbach's alpha = 0.96). Nevertheless, the items related to depression, motivation/initiative, and tremor were scarcely consistent. The Interrater reliability was satisfactory (all the items had k > 0.40). There was a high correlation of the UPDRS with the Hoehn and Yahr staging (rs = 0.71; p < 0.001) and some timed tests (finger tapping; arising from chair), but also with the MMSE and HSD (rs = 0.53; rs = 0.64; p < 0.001). The convergent validity with the other PDRS (Intermediate Scale and Schwab and England Scale) was very high (rs = 0.76−0.96; p < 0.001). The factor analysis identified six factors that explained 59.6% of the variance. The dimension “tremor” showed a remarkable independence. The UPDRS is a multidimensional, reliable, and valid scale, with some inconveniences derived from its internal consistency, discriminant validity, and pragmatic application.
Posttraumatic paroxysmal nocturnal hemidystonia
Authors: Biary N. , Singh B. , Bahou Y. , Al Deeb S. M. , Sharif H.
Published: December 2004

The term “nocturnal paroxysmal dystonia” has been used to describe patients who display paroxysmal episodes of dystonic‐dyskinetic movements arising during nornrapid eye movement sleep, in particular stages 2–3 (Lugaresi E, Cirignotta F. Hypnogenic paroxysmal dystonia: epileptic seizure or a new syndrome. Sleep 1981; 4:129–138). The pathogenesis of these attacks has remained controversial. We describe a patient with posttraumatic paroxysmal nocturnal hemidystonia. Acetazolamide led to improvement.
Late presentation of congenital muscular torticollis: Use of MR imaging and CT scan in diagnosis
Authors: Singer Carlos , Green Barth A. , Bruce Jocelyn H. , Bowen Brain C. , Weiner William J.
Published: December 2004

A 17‐year old boy presented with a 10‐year history of progressive head tilt to the right. Bilateral posterolateral cervical pain was mild and he was fully functional. The right sternocleidomastoid (SCM) muscle was prominent without ratation of the head to the left. The SCM had a cord‐like consistency on palpation. Magnetic resonance (MR) and computed tomography (CT) scan imaging of the neck musculature suggested fibrous tissue within the substance of the muscle. This was histopathologically confirmed when the right SCM was surgically explored and resected. Congenital muscular torticollis is usually seen in newborns, infants, and children but may also present in adolescence and young adulthood. It should be included in the differential diagnosis of cervical dystonia as one of the nondystonic causes of abnormal head posture. Combined use of MR and CT scan of neck muscles may be of help in the diagnosis.
Masticatory muscle spasm in a non‐japanese patient with satoyoshi syndrome successfully treated with botulinum toxin
Authors: Merello Marcelo , García Horacio , Nogués Martín , Leiguarda Ramón
Published: December 2004

A non‐Japanese patient with Satoyoshi syndrome is presented. Severe masticatory muscle spasms interfered with feeding, but were successfully treated with botulinum toxin.
The initiation of normal walking
Authors: Elble Rodger J. , Moody Charles , Leffler Keith , Sinha Raj
Published: December 2004

Lower extremity electromyograms (EMGs), ground reaction forces, and body motion were measured during the brisk initiation of forward walking performed by 12 healthy adults, aged 20 to 82 years. Gait was initiated 20 times in response to a visual cue. During gait initiation, the body rotated about the ankles like a flexible inverted pendulum. The muscles of the lower extremities were activated stereotypically so as to create moments of force about the ankles that propelled the body toward the stance foot and into forward motion. All volunteers exhibited similar patterns of gait initiation, which were so reproducible that computer averaging of multiple steps by each person was possible. Gait initiation is a stereotyped sequence of postural shifts that culminates in forward step. Disturbances of gait initiation could result from abnormalities in postural control, movement, or their integration.
Cerebellar ataxia, dystonia, and tremor within a family: Variable phenotypes of a single genetic disorder?
Authors: Adler Charles H. , Wrabetz Lawrence , Brin Mitchell F. , Hurtig Howard I.
Published: December 2004

We report a non‐Jewish, Anglo‐Saxon, American family, in which one sibling has dystonia, a second has cerebellar ataxia, and a third has a combination of dystonia and ataxia. All three siblings have pyramidal signs. Their mother and maternal uncle have tremor, and their maternal grandmother may have had a neurodegenerative disorder. Although the inheritance pattern is uncertain, this may represent phenotypic variability resulting from a single gene mutation. The multiple phenotypes within this family do not fit any known inherited neurodegenerative or metabolic disorder.
Chronic administration does not alter the accumulation of L‐dopa into muscle
Authors: Rose S. , Jenner P. , Marsden C. D.
Published: December 2004

Rats were treated for 12 months with L‐dopa (191.4–210.4 mg/kg/day) plus carbidopa (23.9–26.2 mg/kg/day), or carbidopa (24.4–26.3 mg/kg/day) alone. Four days after drug withdrawal, animals received an acute challenge with either L‐dopa (50 mg/kg p.o.) alone or following acute carbidopa (25 mg/kg i.p.) pretreatment, and the uptake and metabolism of L‐dopa in muscle was studied. Following the acute bolus challenge, plasma levels of L‐dopa peaked between 0.5 and 3 h after L‐dopa alone and between 1.5 and 2 h after L‐dopa plus carbidopa. Peak levels in muscle were observed between 1.5 and 4 h after L‐dopa administration, and this accumulation was enhanced by the acute pretreatment with carbidopa. Chronic administration of L‐dopa plus carbidopa or carbidopa alone for 12 months had no effect on the accumulation of L‐dopa into muscle following the acute challenge with L‐dopa alone or after carbidopa pretreatment. 3‐O‐Methyldopa, dopamine, DOPAC, and HVA levels were elevated in both plasma and muscle following acute oral challenge with L‐dopa or L‐dopa plus carbidopa. Levels of these metabolites were unaffected by chronic administration of L‐dopa plus carbidopa or carbidopa alone. In conclusion, chronic administration of L‐dopa plus carbidopa did not alter the accumulation of L‐dopa into muscle following an acute oral challenge with the drug, with or without carbidopa pretreament.
Electromyographic silent period after transcranial brain stimulation in huntington's disease
Authors: Priori Alberto , Berardelli Alfredo , Inghilleri Maurizio , Polidori Luigi , Manfredi Mario
Published: December 2004

The silent period evoked by transcranial (TCS) and nerve stimulation was studied in the hand muscles in 13 patients with Huntington's disease and in 11 normal subjects. The duration of the silent period after TCS was longer in patients and correlated significantly with the severity of chorea; in contrast, the duration of the silent period after nerve stimulation was similar in patients and controls. The prolongation of the cortical silent period suggests that the duration of the silent period is a functional correlate reflecting basal ganglia influence over the motor cortex.
Novel rat cardiac arrest model of posthypoxic myoclonus
Authors: Truong Daniel D. , Matsumoto Rae R. , Schwartz Philip H. , Hussong Matthew J. , Wasterlain Claude G.
Published: December 2004

We describe the time course of and pharmacology associated with auditory‐induced muscle jerks following cardiac arrest in rats. The data indicate that several key features of this model mimic those of human posthypoxic myoclonus. Similar to the human form, the muscle jerks appear in the rats following an acute hypoxic episode (cardiac arrest). Initially, it is known that both spontaneous and auditory‐induced myoclonus are present in these animals; some cardiac‐arrested rats also exhibit seizures. Over the first few days after the arrest, episodes of both the seizure activity and spontaneous myoclonus disappear. The auditory‐induced myoclonus continues to worsen, reaches a peak about 2 weeks after the arrest, then declines over time to subnormal levels. The auditory‐induced muscle jerks exhibited by the cardiac arrested animals are attenuated by the typical antimyoclonic drugs 5‐hydroxytryptophan, valproic acid, and clonazepam. In addition, the novel anticonvulsant felbamate was found to have antimyoclonic properties. The data suggest that this rat cardiac arrest model may be a valuable tool for investigating the pathophysiologic mechanisms of posthypoxic myoclonus and for developing new therapeutic strategies for treating the disorder.
Smoking and Parkinson's disease
Authors: Mayeux Richard , Tang Ming‐Xin , Marder Karen , Cǒté Lucien J. , Stern Yaakov
Published: December 2004

Smoking was examined in relation to Parkinson's disease (PD) in a population‐based study in northern Manhattan (New York City) because of its putative “protective effect.” Using a case‐control design, information on smoking and associated behaviors was obtained in structured interviews after standard diagnostic evaluations in both cases and controls. The overall prevalence of smoking in the population was 43.7%, decreasing to 37% after age 85. Smoking was most frequent in men, Blacks, and in both cases and controls using alcohol once per week or more. Cases had quit smoking more often than controls (87 vs. 64%), and had smoked for significantly fewer years (31 vs. 41 yrs; p < 0.05 for both). The age‐at‐onset for smokers with PD was similar to age‐at‐onset for nonsmokers with PD. The odds ratio (OR) for a history of smoking associated with PD was 1.1 (95% CI 0.7–1.8). No protective gradient was associated with heavier smoking patterns. However, the odds that patients with PD were still smoking at the time of the interview were significantly less than those for controls (OR = 0.2; 95% CI 0.1–0.5). These results do not support the hypothesis that smoking protects against PD; rather they strongly imply the converse, that PD reduces smoking.
Motor program memory storage in Parkinson's disease patients tested with a delayed response task
Authors: Labutta Robert J. , Miles Rosalyn B. , Sanes Jerome N. , Hallett Mark
Published: December 2004

We used a delayed response paradigm to test the hypothesis that the prolonged reaction time in patients with Parkinson's disease (PD) is related to a deficiency in their ability to store a motor program in memory while waiting to move. PD patients, both on and off medication, were compared with age‐matched normal subjects during arm movements directed toward a target light. The target light was displayed either during a 3‐ to 9‐s delay or for only 1 s followed by a 2‐ to 8‐s delay before the go signal. At the end of the delay, subjects were required to begin movement rapidly. The reaction time of PD patients was longer than normal and increased slightly when the patients were off medication. The patients had no excessive increase in reaction time with delay in either task compared with the control subjects. We conclude that patients with PD can hold a motor program in memory storage for at least 8 s.
Facial action myoclonus in patients with olivopontocerebellar atrophy
Authors: Lou Jau‐Shin , Valls‐Solé Josep , Toro Camilo , Hallett Mark
Published: December 2004

We studied four patients with familial olivopontocerebellar atrophy (OPCA) who had abnormal twitching of the cheeks and perioral muscles induced by facial movements. With the muscles at rest, electromyographic (EMG) recordings of the orbicularis oris and risorius muscles revealed myokymic discharges in the absence of visible movements. With voluntary contraction, the EMG showed synchronous discharges in the orbicularis oris and risorius muscles ipsilaterally associated with visible twitching. The duration of the EMG bursts was 10 to 75 ms with a frequency of 8 to 25 Hz, which suggested that the abnormal twitching was most consistent with a myoclonic disorder. Because it was induced by activation of the facial muscles, this movement disorder represents a form of action myoclonus.
Infantile hypotonia and paroxysmal dystonia: A variant of alternating hemiplegia of childhood?
Authors: Andermann Frederick , Ohtahara Shunsuke , Andermann Eva , Camfield Peter , Kobayashi Katsuhiro
Published: December 2004

We report 2 children with early onset of hypotonia and frequent episodes of paroxysmal dystonia. The episodes were abolished even by brief naps. One of the children developed alternating hemiplegia in the second decade. These children seem to have a variant of the now well‐recognized syndrome of alternating hemiplegia of childhood. In that disorder, episodes of alternating hemiplegia develop before the age of 18 months. This syndrome must be considered in the differential diagnosis of paroxysmal dystonia in childhood.
Effectiveness of botulinum toxin type A against painful limb myoclonus of spinal cord origin
Authors: Polo Kathleen B. , Jabbari Bahman
Published: December 2004

Botulinum toxin is now an established treatment for blepharospasm, hemifacial spasm, spasmodic torticollis, and spastic dysphonia. We report the effectiveness of botulinum toxin against painful limb myoclonus of spinal cord origin. The patient, a 16‐year‐old girl with a pulmonary vascular anomaly, Scimitar syndrome, suffered from an acute spinal cord infarct at age 11. She was left with paralysis of the right leg and bladder dysfunction. Four years after the original insult, she developed “painful cramping” and involuntary movements of the left thigh, which were unresponsive to a wide range of therapeutic trials. The movements were continuous, rhythmic, and confined to the left quadriceps muscles. Electromyographic examination revealed continuous myoclonic discharges. Treatment with botulium toxin in the left quadriceps muscles resulted in complete cessation of pain and marked reduction in amplitude of the movements, both clinically and electromyographically. This observation indicates the efficacy of botulinum toxin in the treatment of painful spinal myoclonus.