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S A Factor

Publications and source records attributed to S A Factor.

At least 37 records · Page 2Linked to original sources

Clinical presentation and pharmacological therapy in corticobasal degeneration.

BACKGROUND: To date, to our knowledge, there is no systematic presentation of treatment outcome in large series of patients clinically diagnosed as having corticobasal degeneration. OBJECTIVE: To evaluate the clinical presentation and treatment outcome of patients clinically diagnosed as having corticobasal degeneration. SUBJECTS: We gathered case patients seen in 8 major movement disorder clinics during the last 5 years who were diagnosed as having corticobasal ganglionic degeneration. METHODS: Using a chart review method, we recorded the clinical presentation, medications used, response to medications, and adverse effects. RESULTS: A total of 147 case patients were reviewed, 7 were autopsy proven. Parkinsonian features were present in all, other movement disorders in 89%, and higher cortical dysfunction in 93%. The most common parkinsonian sign was rigidity (92%), followed by bradykinesia (80%), gait disorder (80%), and tremor (55%). Other movement disorders were dystonia in 71% and myoclonus in 55%. Higher cortical dysfunction included dyspraxia (82%), alien limb (42%), cortical sensory loss (33%), and dementia (25%). Ninety-two percent of the case patients received dopaminergic drugs, which resulted in a beneficial effect for 24%. Parkinsonian signs were the elements improving the most and levodopa was the most effective drug. Benzodiazepines, primarily clonazepam, were administered to 47 case patients, which resulted in improvement of myoclonus in 23% and dystonia in 9%. The most frequent disabling adverse effects of drug trials in these case patients were somnolence (n = 24), gastrointestinal complaints (n = 23), confusion (n = 16), dizziness (n =12), hallucinations (n = 5), and dry mouth (n = 5). CONCLUSIONS: Pharmacological intervention was largely ineffective in the management of corticobasal degeneration, and new treatments are needed for ameliorating the symptoms of this syndrome.

Antiparkinson Agents↗

Clinical comparison of tardive and idiopathic cervical dystonia.

It has been suggested that tardive cervical dystonia may be clinically indistinguishable from the idiopathic form and that the diagnosis rests solely on documenting an exposure to dopamine antagonist medications. To investigate this, we performed a retrospective evaluation of patient records on 102 patients with idiopathic and 20 patients with tardive cervical dystonia seen in our Movement Disorder Clinic over the past 8 years. Several clinical and demographic variables were compared and a number of differences were observed. The presence of extracervical involvement, retrocollis, and spasmodic head movements were individually found to be predictive of tardive cervical dystonia. Torticollis, laterocollis, and trick maneuvers were predictive of idiopathic cervical dystonia. Head tremor (42.2%) and family history of dystonia (9.8%) were present only in the idiopathic group. Cervical muscle hypertrophy was significantly more common in the idiopathic group (100% versus 75%). No difference was found between the two groups in their response to treatment with botulinum toxin A. These results indicate that clinical differences between idiopathic and tardive cervical dystonia exist. These differences may help to distinguish them in the clinical setting, improve diagnostic accuracy, and support the existence of a causal relationship between exposure to dopamine antagonist medications and chronic dystonia.

Adult↗

Acute delirium after withdrawal of amantadine in Parkinson's disease.

We present three patients who, after long-term therapy with amantadine (4 to 18 years), experienced an acute delirium with confusion, disorientation, agitation, and paranoia on withdrawal. These patients had Parkinson's disease for 5 to 29 years; mean age was 73 years. All had a history of varying degrees of dementia and transient hallucinations in the past. Adjustment of other medications was ineffective in improving their condition and no other cause was found. Only with reinstitution of amantadine did the patients return to baseline status (usually within days).

Acute Disease↗

The emerging role of clozapine in the treatment of movement disorders.

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

Antiparkinson Agents↗

Mosaic pattern of gliosis in the neostriatum of a North American man with craniocervical dystonia and parkinsonism.

We present the case of a 51-year-old patient with a 31-year history of psychiatric symptoms, craniocervical dystonia, bulbar dysfunction, and parkinsonism. His dystonic movements included blepharospasm, jaw opening and lingual dystonia, and spasmodic retrocollis. Psychiatric symptoms included psychosis and depression, with onset years before the movement disorder. After his death by aspiration, examination of his brain revealed abnormalities limited to the neostriatum. Staining of brain sections, including Holzer, glial fibrillary acidic protein, and immunohistochemical stain for calbindin D28k, revealed the presence of a mosaic pattern of gliosis with neuronal loss (sparing large neurons) within this region. The islands of tissue between stands of gliosis had a normal appearance. This patient represents only the fourth case (and first North American born) with a mosaic pattern of gliosis in the neostriatum. The clinical and pathologic features were similar in all four cases except that our patient was the first with prominent psychiatric symptoms and a more stable, less progressive course. Mosaicism has been described in the X-linked Filipino disorder Lubag. Occurrence in non-Filipino patients, such as ours, suggest that either Lubag can develop in non-Filipino families or that mosaicism is a nonspecific pathologic finding in some patients with idiopathic dystonia. Finally, our case reports the notion that craniocervical dystonia may result from neostriatal dysfunction.

Age of Onset↗

Adult-onset tics associated with peripheral injury.

We report the cases of two patients with adult-onset, simple, nonvarying tic disorder that commenced after a peripheral (non-CNS) injury. The first patient is a 38-year-old man who suffered a right facial injury when his car fell off its jack while he was working underneath. Bilateral facial twitching began hours after the trauma and was characterized as a sniffinglike gesture. The movements waxed and waned, were suppressible, and were associated with a premonitory sensation. The tics remitted after 9 months but still recur occasionally under stressful situations. The second patient is a 34-year-old man with a 3-year history of abrupt, rapid head-turning movements that began 12 months after a motor vehicle accident in which he injured his neck. The tics continue to wax and wane, are suppressible, and are associated with an urge. Neither patient suffered a head injury or had a family history of Tourette syndrome. Based on the clinical and historical features of these patients, the temporal relationship between the trauma and onset of tics, and the occurrence of tics only in the traumatized region, a causal relationship is possible. These may represent the first reported cases of tic disorder in association with peripheral injury. The mechanism by which the tic disorder resulted from the peripheral injury is unclear, but these patients might have been susceptible individuals and the trauma acted as a trigger.

Adult↗

Botulinum toxin type B: a double-blind, placebo-controlled, safety and efficacy study in cervical dystonia.

We enrolled and treated 122 patients with idiopathic cervical dystonia in a double-blind, placebo-controlled safety and efficacy study of botulinum toxin type B (BotB). Both A-responsive and A-resistant patients were enrolled. Patients received intramuscular injections of either BotB (2,500 U, 5,000 U, or 10,000 U) or placebo. The primary outcome measure of efficacy was the Toronto Western Spasmodic Torticollis Rating Scale (TWSTRS)-Total score at 4 weeks following study drug administration. Secondary measures of efficacy were TWSTRS-Severity, -Disability, and -Pain subscale scores, and Analog Pain Assessment, Investigator Global Assessment, Patient Global Assessment, and Sickness Impact Profile scores. Duration of effect was estimated with an intent-to-treat analysis of responders. Safety measures included clinical parameters, laboratory tests, and adverse events. The primary and most of the secondary analyses indicated a statistically significant treatment effect and a dose response. BotB is safe, well tolerated, and efficacious in the treatment of cervical dystonia at the doses tested.

Adult↗

Chorea caused by lithium intoxication: a case report and literature review.

We report a patient who developed an acute, reversible, generalized choreiform disorder from lithium (Li) intoxication. This medication was prescribed for manic-depressive disorder, and serum levels became elevated after the addition of a diuretic for the treatment of hypertension. There were no other apparent causes for the movement disorder, and it was associated with other known features of Li intoxication, including ataxia and encephalopathy. There was a delay between the initial symptoms of Li intoxication and the onset of chorea. The chorea improved as serum Li levels diminished, with some lag time. This represents the eleventh case report of Li-induced chorea, but only the sixth in a patient without concomitant neuroleptic therapy, and the first presented with videotape confirmation. A review of these other cases is included, and possible mechanisms are discussed.

Aged↗

Peripheral catecholamine output in Parkinson's disease: effects of drug treatment.

Recent reports have indicated depressed levels of catecholamines in the adrenal medulla of advanced Parkinson's Disease (PD) patients undergoing autologous transplant and at autopsy. Such an adrenal defect might have compromised the efficacy of autologous transplants in PD patients. The question arose whether these findings were the result of a generalized defect in catecholamine metabolism in both central and peripheral nervous systems or simply due to drug treatment with levodopa and carbidopa (L/C), the latter being an inhibitor of peripheral catecholamine synthesis. If indeed there are defects in adrenal catecholamine output in PD it might be possible to screen candidates for autologous adrenal medullary transplants. We investigated these issues by measuring 24-h urinary epinephrine (EPI), norepinephrine (NE), metanephrines (METS), and vanilmandelic acid (VMA) levels in three groups of patients: PD patients on standard treatment with L/C, PD patients not on L/C drug treatment, and control non-PD patients. There were no significant differences in 24-h urinary catecholamines, METS, or VMA among the three groups. However, trends in the present results together with previously published data suggest that parkinsonian drug treatment may lower urinary EPI excretion and increase NE excretion. The former may be related to the depressed adrenal medullary tissue levels of EPI, while the latter may reflect conversion of levodopa and incomplete suppression of sympathetic catecholamine synthesis. If there are depressed levels of tissue catecholamines in the sympathoadrenal system in untreated PD patients this is not reflected in a significantly decreased catecholamine output in 24-h urine samples.

Adrenal Medulla↗

Psychogenic movement disorders: frequency, clinical profile, and characteristics.

Of 842 consecutive patients with movement disorders seen over a 71 month period, 28 (3.3%) were diagnosed as having a documented or clinically established psychogenic movement disorder. Tremor was most common (50%) followed by dystonia, myoclonus, and parkinsonism. Clinical descriptions of various types are reviewed. Clinical characteristics common in these patients included distractability (86%), abrupt onset (54%), and selective disabilities (39%). Distractability seems to be most important in tremor and least important in dystonia. Other diagnostic clues included entrainment of tremor to the frequency of repetitive movements of another limb, fatigue of tremor, stimulus sensitivity, and previous history of psychogenic illness. On examination, 71% had other psychogenic features. Over 60% had a clear history of a precipitating event and secondary gain and 50% had a psychiatric diagnosis (usually depression). Twenty five per cent of patients presented with combined psychogenic movement disorder and organic movement disorder; 35% resolved and this subgroup had a shorter duration of disease than those who are unresolved. Psychogenic movement disorder represents an uncommon diagnosis among patients with movement disorders. The ability to make a diagnosis rests on the presence of a multitude of clinical clues and therapeutic action should be taken as early as possible.

Adolescent↗

Combined clozapine and electroconvulsive therapy for the treatment of drug-induced psychosis in Parkinson's disease.

Drug-induced psychosis is a serious late complication of Parkinson's disease (PD) that requires aggressive treatment. Recent studies have found clozapine a highly effective and ECT a possibly useful intervention. Two cases are presented that illustrate a possible treatment role for ECT. The cases demonstrate that ECT has significant but short-lived antipsychotic effects when used alone. However, patients who do not respond to clozapine monotherapy can be given adjunctive treatment with ECT. The combination therapy resulted in abrupt alleviation of psychotic symptoms in one of the cases, and maintenance with low-dose clozapine allowed for long-term efficacy. On the basis of these findings, a therapeutic approach to patients with drug-induced psychosis in PD is suggested.

Antiparkinson Agents↗

Parkinson's disease: drug-induced psychiatric states.

Drug-induced psychiatric states occur frequently in PD. In the prelevodopa era, depression and other psychiatric disorders were described in PD, but in untreated patients psychosis was rare. Since the development of levodopa and other pharmacological treatments for PD, however, psychotic symptoms have become much more common (10-50%). In some individuals these problems can be more disabling than the motor features of PD and, as a result, pose a serious threat to the patient's ability to maintain independence. The drug-induced psychoses consist of several distinct psychiatric syndromes that can be divided broadly into those occurring on a background of a clear sensorium and those which are accompanied by confusion and clouding of consciousness. Benign organic hallucinosis is the most common of these syndromes (30%). It usually occurs on a background of a clear sensorium and may not be a particularly troublesome problem if the patient is able to retain insight into the nature of these symptoms. More disabling syndromes usually include delusional thinking that is frequently paranoid, confusion and even frank delirium. Although all these psychotic syndromes can occur in isolation, there is a tendency for mild symptoms to progress to more disabling ones if adequate and timely treatment is not instituted. Abnormal dreaming and sleep disruption often precede these difficulties by weeks to months and may provide an important early clue to their onset. The mechanisms responsible for drug-induced psychotic symptoms in PD are unknown, but dopaminergic (especially mesolimbic) and serotoninergic systems are likely to be involved. The treatment of the drug-induced psychoses in PD should be undertaken in a stepwise manner. A detailed discussion of this approach, including the use of anti-PD medication adjustment, clozapine, and other medications (neuroleptic and nonneuroleptic) and ECT is provided (see Fig. 1). Although drug-induced psychoses are the most important of the drug-induced psychiatric states, mania, anxiety, and hypersexuality may also occur. Depression is also common in PD, but it is unlikely to occur as a side effect of antiparkinsonian medications.

Aged↗

The use of pramipexole, a novel dopamine (DA) agonist, in advanced Parkinson's disease.

We evaluated the efficacy, safety and tolerability of a new dopamine D-2 receptor agonist, pramipexole [(S)-2-amino-4,5,6,7-tetrahydro-6-propylamino-benzathiazol-dihydro chloride], as adjunctive therapy in patients with advanced Parkinson's disease (PD). Twenty-four PD patients with motor fluctuations were treated in an 11 week prospective, single-blind parallel-group, placebo-controlled trial. The pramipexole treated group experienced a significant improvement in "off" time functioning as measured by the activities of daily living portion of the United Parkinson's Disease Rating Scale. In addition, the active treatment group was able to reduce total levodopa dose by 30% (p < 0.05). Pramipexole was well tolerated and the side effects reported were typical of other dopamine agonists. We conclude that pramipexole has antiparkinsonian effects which make it potentially useful in the treatment of motor fluctuations in PD.

Aged↗

Persistent neuroleptic-induced rigidity and dystonia in AIDS dementia complex: a clinico-pathological case report.

Patients with AIDS dementia complex (ADC) appear to have an increased likelihood of developing acute onset parkinsonism and dystonia when treated with dopamine antagonists. It has been hypothesized, based on clinical evidence, that hypersensitivity to these drugs in ADC is probably related to direct invasion of the basal ganglia by the HIV virus and a secondary alteration in dopaminergic mechanisms. We report the first pathological description of a patient with ADC who developed acute onset, generalized rigidity and dystonia after a brief trial of low dose neuroleptic therapy administered for psychotic symptoms. An unusual clinical feature of this case was the persistence of his movement disorder. Pathological examination revealed a generalized encephalitic process with substantial neuronal loss observed primarily in the medial and lateral globus pallidus. Correlation with a current model of basal ganglia pathophysiology and other disorders with pallidal lesions is discussed. Clinical and pathological features of this case confirm the previous contention and indicate that dopamine antagonists should be utilized with extreme caution in patients with ADC.

AIDS Dementia Complex↗

Platelet morphology in Parkinson's disease: an electron microscopic study.

There are no peripheral diagnostic markers for Parkinson's disease (PD). However, recent studies of platelets in PD patients indicate that mitochondrial and monoamine oxidase function may be abnormal. This investigation examines platelets in PD from a morphological standpoint utilizing transmission electron microscopy (EM). Fourteen PD patients (seven treated, seven untreated) and seven age matched controls had platelets separated from other blood components, fixed in a standardized fashion and examined by EM. Platelets (in the activated form because they were collected in glass tubes) were evaluated at magnifications of 15,000x and 40,000x. Abnormalities observed in treated and untreated PD patients included the presence of numerous large intracytoplasmic vacuoles formed from the open canalicular system. Morphometric examination performed at 40,000x magnification indicated that the mean area of vacuoles and the cytoplasmic volume percent of platelets occupied by vacuoles were significantly greater in PD (p < 0.05) than controls. However, differences observed between treated and untreated PD groups suggest that these changes could be caused by the disease or the treatment or both. No abnormalities were found in relation to mitochondria, storage granules and glycogen. From EM assessment, we conclude that platelets in PD are morphologically abnormal.

Adult↗

Clozapine: a 2-year open trial in Parkinson's disease patients with psychosis.

We treated 17 patients with Parkinson's disease (PD) complicated by psychosis with the atypical antipsychotic drug, clozapine, for 6 to 24 months (mean, 15 months) in a prospective, open-label trial. At 3-month intervals we evaluated patients, using a simplified brief Psychiatric Rating Scale (PRS), the motor examination portion of the Unified Parkinson's Disease Rating Scale, and the Mini-Mental State Examination (MMSE). Mean PRS score was significantly improved when compared with baseline over 1 year (p < 0.01) and nonsignificantly improved for the second year. We maintained the levodopa dose at levels that were 17 to 68% higher than baseline, and the mean motor examination score improved by 11 to 22% in the first 15 months. Clozapine dosage utilized in the trial ranged from 6.25 mg every other day to 150 mg/d. Adverse effects, including sedation and confusion, were common. These results demonstrate that clozapine therapy can be effective in treating psychosis in PD patients over 1 to 2 years. The decline in efficacy in the second year was most likely related to an increase in daily levodopa dose, progression of dementia (illustrated by a decline in MMSE score), and an inability of PD patients to tolerate higher doses of clozapine.

Aged↗