bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clozapine treatment of psychosis in patients with tardive dystonia: Report of three cases
Authors: Friedman Joseph H. 
Published: December 2004

AbstractAmong the tardive dyskinesia syndromes, dystonia can be the most difficult to treat. It may be severe to the point of being disabling, yet the patients may require antipsychotic medications for an even more disabling psychosis. Clozapine, an atypical neuroleptic drug that lacks extrapyramidal effects, may be the drug of choice for such patients. This report describes three patients with significant dystonia, previously disabled by their psychoses, who have been successfully manged with clozapine plus other agents for >3 years.
Spinal myoclonus resembling belly dance
Authors: Kono Ichiyo , Ueda Yoshihiro , Araki Kuniharu , Nakajima Kenji , Shibasaki Hiroshi 
Published: December 2004

AbstractA 63‐year‐old man presented with an 11‐month history of progressive myoclonus in the right abdominal wall. Administration of clonazepam reduced the frequency and amplitude. When the therapy was discontinued, the frequency and amplitude of the myoclonus increased, and synchronous and weak myoclonus also was observed in the left abdomen. The trunk was twisted just after the appearance of the abdominal myoclonus associated with myoclonic jerks spreading from the rostral to caudal paraspinal muscles. Later in the clinical course, the myoclonus became stimulus sensitive and was induced by tendon tap given anywhere on the body, with the latency ranging from 50 to 150 ms irrespective of the sites of tapping. Myoclonus seen in the abdominal wall was segmental and considered to be of spinal origin. The reflex myoclonus had a 150‐ms refractory period. It can be postulated that increased excitability of anterior horn cells at a certain segment might make a spino‐bulbo‐spinal reflex manifest at the corresponding segment. This myoclonus is considered to be a new form of spinal reflex myoclonus, because the abdominal myoclonic jerk seems to trigger another myoclonic jerk involving the paraspinal muscles.
Familial hemifacial spasm
Authors: Micheli Federico , Scorticati Maria Clara , Gatto Emilia , Cersosimo Graciela , Adi Javier 
Published: December 2004

AbstractTwo brothers developed hemifacial spasm at 63 and 70 years of age. Spasms occurred on the left and right sides of the face, respectively. Computed tomography scan and magnetic resonance imaging failed to show any abnormality. In addition, a third sibling reported a history of a peripheral facial palsy, which remitted spontaneously without sequelae. This is the fourth description of familial hemifacial spasms. This family is unique in that hemifacial spasm presented on different sides in the two brothers, and involvement was limited to one generation. Age at onset was later than for other familial cases and similar to sporadic cases.
Tyrosine hydroxylase polymorphism in familial and sporadic Parkinson's disease
Authors: Planté‐Bordeneuve V. , Davis M. B. , Maraganore D. M. , Marsden C. D. , Harding A. E. 
Published: December 2004

AbstractTyrosine hydroxylase (TH) is a theoretical candidate gene determining susceptibility to Parkinson's disease (PD), and an association between one allele of a polymorphism at the TH locus and sporadic PD has been reported. We investigated TH polymorphism in 44 patients with sporadic PD, 48 patients with familial PD and 89 of their unaffected relatives, and 50 control subjects. No evidence of allelic association was detected in either familial or sporadic PD, and linkage analysis excluded the TH locus, or a closely linked gene, as a major determinant of familial PD.
Botulinum toxin in the treatment of dystonic tics
Authors: Jankovic Joseph 
Published: December 2004

AbstractBotulinum toxin (BTX) injections provide effective treatment for a variety of disorders manifested by inappropriate muscle contractions, but its efficacy in the treatment of tics has not been previously studied. Ten male patients 13–53 years of age who were diagnosed with Tourette's syndrome manifested by disabling focal tics were included in this pilot study. Five patients had frequent blinking and blepharospasm, rendering them “blind,” and five patients had severe and painful dystonic tics involving their neck muscles. All 10 patients experienced moderate to marked improvement in the intensity and frequency of tics after BTX injections into the involved muscles. Patients in whom premonitory urges preceded their tics noted marked lessening of these sensory symptoms. The benefit lasted 2–20 weeks after injections. There were no serious complications, except for transient ptosis in two and neck pain, stiffness, or weakness in three patients. BTX injections appear to be safe and effective treatment for patients with focal dystonic tics. The treatment ameliorates not only involuntary movements but also the premonitory sensory component associated with some tics.
Leuprolide acetate for exhibitionism in huntington's disease
Authors: Rich Stuart S. , Ovsiew Fred 
Published: December 2004

AbstractWe treated a patient with Huntington's disease and exhibitionism using leuprolide acetate (Lupron Depot, TAP Pharmaceuticals), a gonadotropin‐releasing hormone agonist. The result was elimination of his deviant sexual behavior with few side effects. This regimen may be a safe and effective pharmacological treatment of the paraphilias. The occurrence of paraphilias in basal ganglia disorders is reviewed.
Subcortical neurofibrillary degeneration presenting as steele‐richardson‐olszewski and other related syndromes: A review of 90 pathologically verified cases
Authors: de Bruin V. M. S. , Lees A. J. 
Published: December 2004

AbstractWe have reviewed 90 patients from the literature with histopathological features compatible with currently accepted criteria for the diagnosis of the Steele‐Richardson‐Olszewski syndrome (SROS). Only 62 patients (69%) had clinically definite SROS based on the criteria of Maher and Lees. Neurofibillary degeneration of subcortical structures with involvement of the internal pallidum, the subthalamic nucleus, and substantia nigra was considered essential for the pathological diagnosis. Thirty‐six cases (40%) had neocortical neurofibrillary change that bore no clear relationship to the degree of cognitive impairment (χ2 = 9.293; p < 0.4107). Grumose degeneration of the dentate nucleus was present in 25 cases (28%), and occasionally there were other, less usual, findings such as Pick bodies and Lewy bodies.
Utility of an objective dyskinesia rating scale for Parkinson's disease: Inter‐ and intrarater reliability assessment
Authors: Goetz C. G. , Stebbins G. T. , Shale H. M. , Lang A. E. , Chernik D. A. , Chmura T. A. , Ahlskog J. E. , Dorflinger E. E. 
Published: December 2004

AbstractAlthough dyskinesia is a frequent and important problem in Parkinson's disease (PD), a reliable assessment measure has not been thoroughly developed and tested. We modified the Obeso dyskinesia scale to creat an objective rating scale for dyskinesia assessment during activities of daily living. Thirteen physicians and 15 study coordinators involved in a clinical trial independently reviewed videotape segments of PD patients performing three tasks: walking, putting on a coat, and lifting a cup to the lips for drinking. Raters evaluated the severity of worst dyskinesia seen, the types of all dyskinesias seen, and the type of dyskinesia most associated with motoric disability. For all assessments, the total group showed statistically significant inter‐ and intrarater reliability. Physicians had a higher consistency than did coordinators, but for most measures the difference was not statistically significant. Physicians and coordinators found the scale easy to use and especially practical for rating dyskinesia severity and for identifying the most disabling dyskinesia. Dyskinesias can be assessed in clinical trials and warrant regular documentation.
Clinical and electromyographic features of levator palpebrae superioris muscle dysfunction in involuntary eyelid closure
Authors: Aramideh M. , de Visser B. W. Ongerboer , Koelman J. H. T. M. , Bour L. J. , Devriese P. P. , Speelman J. D. 
Published: December 2004

AbstractWe report on five patients with involuntary eyelid closure, diagnosed as blepharospasm and referred to us for treatment with botulinum A toxin. Synchronous electromyographic (EMG) recording was performed from the levator palpebrae superioris (LP) and the orbicularis oculi (OO) muscles. In the first two cases, EMG registration showed alternating, semirhythmic dystonic activities in both the LP and OO, clinically perceptible as “flickering” of the eyelids. While the eyelids were lowered, one of them alos showed involuntary upper eyelid tractions due to dystonic activities of LP. In the third patient, EMG patterns were characterized by a gradual decrease in the level of LP activity, followed by the contraction of OO, which facilitated the return of LP to its tonic activity, termed “postinhibition potentiation” of LP. In the fourth patient, EMG recording showed involuntary inhibition of LP in combination with blepharospasm. Involuntary closure of the eyelids in the fifth patient was caused by short or prolonged periods of involuntary LP inhibition, whereas OO activity remained normal. Our results provide further evidence that LP muscle activities are regulated by burst‐tonic motoneurons, and we suggest that these motoneurons, and/or the input signals regulating their activities, can be involved independently in a pathological process. Clinical symptoms are discussed that may be helpful to recognize those cases with LP motor dysfunction, whether or not accompanied by OO activity disorders. Because the term blepharospasm indicates an abnormal motor function of OO, we propose “blepharospasm‐plus” to designate those cases with a combined motor dysfunction of LP and OO muscles.
Suppression of dyskinesias in advanced Parkinson's disease: Moderate daily clozapine doses provide long‐term dyskinesia reduction
Authors: Bennett James P. , Landow Elke R. , Dietrich Susan , Schuh Lori A. 
Published: December 2004

AbstractDyskinesias commonly appear during L‐dihydroxyphenylalanine (L‐DOPA) therapy of advanced Parkinson's disease (PD) and can occur in both dose‐related and dose‐independent patterns. Clozapine exerts a dose‐related suppression of L‐DOPA‐induced dyskinesias by shifting the i.v. L‐DOPA dose‐response curve for production of dyskinesias without altering relief of parkinsonism. We report our outpatient experience with 13 patients on daily clozapine therapy (maximum dose 400 mg/day), followed for 3–21 months (median 10). Beneficial effects of clozapine, determined from twice‐weekly diaries, included increased “on time” and decreased “off time” and time “on with dyskinesia.” Improvements were statistically apparent by 75 mg/day and remained so through 200 mg/day. Sedation was a common problem, reflected by increased time “asleep” which was significant by 50 mg/day. Sedation was dose limiting in most patients. Orthostatic hypotension and sialorrhea were variably present. No patients had seizures, bone marrow toxicity, or detectable loss of efficacy of clozapine with chronic use. We conclude that clozapine is an effective agent for suppression of dyskinesias in PD with an effective daily dose for most patients of 100–200 mg/day.
Pergolide compared with bromocriptine in Parkinson's disease: A multicenter, crossover, controlled study
Authors: Pezzoli G. , Martignoni E. , Pacchetti C. , Angeleri V. A. , Lamberti P. , Muratorio A. , Bonuccelli U. , De Mari M. , Foschi N. , Cossutta E. , Nicoletti F. , Giammona F. , Canesi M. , Scarlato G. , Caraceni T. , Moscarelli E. 
Published: December 2004

AbstractWe compared the efficacy and safety of pergolide and bromocriptine in 57 patients with Parkinson's disease (PD) with a declining response to levodopa therapy in a single‐blind, crossover study. Patients were placed randomly on the sequence bromocriptine‐pergolide (12 + 12 weeks) or vice versa. Regular evaluations using the New York University Parkinson's Disease Scale were performed by a clinician blinded to treatment assignment. Patients' and clinicians' impressions also were recorded. The average daily dose of pergolide was 2.3 ± 0.8 mg, and that of bromocriptine was 24.2 ± 8.4 mg. Significantly greater efficacy was demonstrated by both drugs as adjunctive therapy to levodopa compared with previous treatment of levodopa alone (pergolide, p = 0.0001; bromocriptine, p = 0.0005; Wilcoxon t test). Pergolide was more effective than bromocriptine in daily living scores (p = 0.020) and motor scores (p = 0.038). No difference in dyskinesias, dystonias, and psychosis was observed. Adverse events were more frequent in bromocriptine‐treated patients. Most patients and physicians preferred pergolide to bromocriptine. Pergolide as adjunctive therapy to levodopa was more effective than bromocriptine in this short‐term trial.
Treatment of severe axial tardive dystonia with clozapine: Case report and hypothesis
Authors: Trugman Joel M. , Leadbetter Robert , Zalis Michael E. , Burgdorf Rose O. , Wooten G. Frederick 
Published: December 2004

AbstractWe report a patient with severe axial tardive dystonia who has had dramatic improvement for 4 years after treatment with the atypical antipsychotic drug clozapine (625 mg/day). Clozapine differs from conventional neuroleptics in that it has higher affinity for D1 and lower affinity for D2 dopamine receptors than do conventional antipsychotics, which are relatively selective D2 antagonists. We propose that repetitive stimulation of the D1 receptor by endogenous dopamine, resulting in sensitization of the D1‐mediated striatal output in the presence of D2 receptor blockade, is a fundamental mechanism mediating tardive dyskinesia, including the dystonic type. According to this hypothesis, it is primarily the D1 antagonist action of clozapine that accounts for its inability to cause tardive dyskinesia as well as its therapeutic effect in tardive dystonia. Regardless of its mechanism of action, the sustained improvement observed in this case suggests that clozapine should be tried in cases of severe refractory tardive dystonia.
Unusual synkinetic movements between facial muscles and respiration in hemifacial spasm
Authors: Pavesi G. , Medici D. , Macaluso G. M. , Ventura P. , Allegri I. , Gemignani F. 
Published: December 2004

AbstractWe describe two cases of hemifacial spasm (HFS) with unusual synkinetic movements between facial muscles and respiratory activity. Patient 1 developed an idiopathic HFS. She underwent microvascular decompression surgery, followed by transitory facial nerve palsy; she recovered but later developed a facial synkinesia characterized by involuntary and forced eyelid closure on spontaneous and deep breathing; the orbicularis oculi muscle was active also when her mouth was open (the so‐called Marin‐Amat syndrome). Patient 2, 1 year after a peripheral facial nerve palsy (Bell's palsy), developed an HFS together with synkinetic movements between the orbicularis oculi muscle and respiration. In both cases electrophy siological studies showed pathological synkinetic electromyographic activity. An enhanced hyperexcitability of brain stem interneurons and facial motoneurons could be suggested to explain the phenomenon.
Dystonis in ataxia telangiectasia: Report of a case with putaminal lesions and decreased striatal [123I]Iodobenzamide Binding
Authors: Koepp M. , Schelosky L. , Cordes I. , Cordes M. , Poewe W. 
Published: December 2004

AbstractA 6‐year‐old girl with ataxia telangiectasia and severe progressive dystonic posturing is presented. Magnetic resonance imaging showed cerebellar atrophy and a right‐sided putaminal lesion. A single‐photon emission computed tomography study of cerebral dopamine‐(D2)‐receptor binding with [123I]iodobenzamide showed a decreased tracer uptake in the striatum bilaterally. Dystonia deteriorated with levodopa treatment, whereas trihexyphenidyl led to significant improvement. Although dystonic symptoms have been repeatedly described in ataxia telangiectasia, this is the first report demonstrating structural and functional basal ganglia abnormalities in this disorder.
Late‐onset ataxia telangiectasia in two brothers presenting with juvenile resting tremor
Authors: Hiel J. A. P. , Weemaes C. M. R. , Smeets D. F. C. M. , van de Vlasakker C. J. W. , Horstink M. W. I. M. 
Published: December 2004

AbstractTwo young adult brothers presented with a 5‐ to 6‐Hz resting tremor of the upper limbs. Although ataxia was not unequivocally present and ocular telangiectasia was minimal, typical rearrangements of chromosomes 7 and 14, and increased α‐fetoprotein levels indicated the presence of ataxia telangiectasia (AT). Resting tremor as a predominating symptom in AT is uncommon and to our knowledge has not been described previously.
Fluctuations in plasma levodopa and motor responses with liquid and tablet levodopa/carbidopa
Authors: Metman L. Verhagen , Hoff J. , Mouradian M. M. , Chase T. N. 
Published: December 2004

AbstractTo evaluate the ability of orally administered liquid levodopa/carbidopa (LD/CD) to stabilize plasma levodopa levels and reduce motor reponse fluctuations in Parkinson's disease, five patients received LD/CD hourly, as standard tablets or as an aqueous solution on two separate days in a double‐blind, placebo‐controlled, cross‐over design. Except for a slightly earlier peak plasma levodopa level, no significant advantage of the liquid formulation over tablet therapy was found. However, liquid LD/CD could be helpful in quickly resolving “off” states and by facilitating small dose adjustments that are not possible with tablets.
Unusual focal dyskinesias: The ears, the shoulders, the back, and the abdomen
Authors: Caviness J. N. , Gabellini A. , Kneebone C. S. , Thompson P. D. , Lees A. J. , Marsden C. D. 
Published: December 2004

AbstractFourteen patients with focal or segmental involuntary movements affecting the ears, back, shoulder girdle, and upper extremity, as well as the abdomen and pelvic girdle, are presented. The unusual locations and appearance of these dyskinesias distinguishes them from recognized movement disorder syndromes. It is argued that the slow, sinuous, and semirhythmic character of the movements and the variable long‐duration bursts of motor unit activity responsible for them most closely fit into the spectrum of dystonia. A history of pain in the affected region and/or peripheral trauma in some cases also suggests that peripheral factors may play a role in their pathogenesis.
Neurological sequelae following carbon monoxide poisoning clinical course and outcome according to the clinical types and brain computed tomography scan findings
Authors: Lee M. S. , Marsden C. D. 
Published: December 2004

AbstractThe prognosis for patients who survive carbon monoxide (CO) poisoning is uncertain, particularly in those who develop persistent neurological complications after recovery from the initial coma. Thirty‐one patients with the sequelae of CO poisoning, followed for a year, are described. Eight had a progressive course, and 23 had a delayed relapse after an initial recovery period of ∼20 days (range, 1–36 days). Those with a progressive course developed a persistent akinetic‐mute state, and four of the eight died. Those with the delayed relapsing course either developed a parkinsonian state with behavioral and cognitive impairment but could walk (nine cases); or progressed further to an akinetic‐mute state, and were bed‐bound (14 cases); the deterioration to either condition occured rapidly over a few days to a week. Fourteen of the patients with the delayed relapses (61%) subsequently improved, but three (13%) died. Those with a progressive course without initial recovery were younger (mean age, 37.0 years) than those with a delayed relapsing course (55.2 years; p < 0.01). The mean duration of their initial coma (9.8 days) was longer than that in delayed relapsing cases (2.0 days; p < 0.01). The mean initial CO hemoglobin level was not different in the two groups. Brain computed tomography (CT) scans were obtained at the onset of sequelae in both groups. Ten patients had a normal CT scan, 13 had white matter low‐density lesions, and four had globus pallidus low‐density lesions. These findings did not accurately predict the outcome, although all four patients who had both globus pallidus and white matter low‐density lesions at initial CT scan had a bad prognosis. Follow‐up brain CT scans were performed in eight patients; all showed progressive changes and/or new lesions, despite which three patients improved; four of the five patients with globus pallidus and white matter lowdensity lesions at follow‐up CT scan had a bad prognosis.
Superficial hemosiderosis of the central nervous system
Authors: River Y. , Honigman S. , Gomori J. M. , Reches A. 
Published: December 2004

AbstractSuperficial hemosiderosis (SH) of the CNS is a rare disease caused by repeated subarachnoid hemorrhage, with progressive superficial siderosis of the CNS. We report a patient with SH whose clinical picture was marked by progressive gait ataxia, hearing loss, dysarthria, and recurrent episodes of hemifacial spasm. Iron and ferritin levels in the CSF were significantly higher than in a control group of patients. Six month's treatment with the iron‐chelating agent trientine dihydrochloride led to clinical improvement, with a concomitant reduction of CSF iron level. We suggest that, in addition to magnetic resonance imaging findings, CSF levels of iron and ferritin should be used as diagnostic criteria for SH, as well as to estimate the efficacy of iron chelation treatment.
Propriospinal myoclonus: Evidence for spinal “pattern” generators in humans
Authors: Brown P. , Rothwell J. C. , Thompson P. D. , Marsden C. D. 
Published: December 2004

AbstractThe clinical and electrophysiological characteristics of eight patients with propriospinal myoclonus are described. Myoclonus developed within days or weeks of cervical trauma in half the patients. Seven cases had axial flexion jerks, and one axial extension jerks. Myoclonic EMG activity consisted of repetitive bursts with a frequency of 1–7 Hz. The jerks in three of the cases were comprised of alternating and rhythmic bursts of EMG activity in rectus abdominis and the paraspinal muscles. From these new observations, it is proposed that cervical trauma can lead to the partial release of a spinal pattern generator. The latter is capable of recruiting muscles through long propriospinal pathways into complex rhythmic activity.
Factors influencing the amplitude and frequency of essential tremor
Authors: Elble Rodger J. , Higgins Constance , Leffler Keith , Hughes Larry 
Published: December 2004

AbstractQuantitative electromyography (EMG) and triaxial accelerometry were used to measure hand tremor, finger tremor, and forearm motor unit activity in 36 men and 23 women with essential tremor. Hand tremor was studied with and without 300‐g mass loading. Tremor and EMG were recorded twice, with a 3‐min rest interval, to assess the spontaneous intertrial variability in tremor amplitude, frequency, and motor unit entrainment. In general, the minimum detectable differences in tremor amplitude, frequency, and motor unit entrainment were roughly 30%, 5%, and 10% of the initial sample means, respectively, assuming a sample size of 30 patients, a statistical power of 0.9, and a significance level of 0.05. Linear regression analyses were performed to test the hypotheses that (a) there is an age‐associated decrease in tremor frequency that is independent of symptom duration and (b) tremor frequency and motor unit entrainment make independent contributions to tremor amplitude. Our data supported both hypotheses.