Persistent rheumatic chorea

A 79-year-old woman had rheumatic chorea that persisted after age 5 years and increased in severity at age 73. The continued chorea and late decompensation could have been due to incomplete functional resolution in the basal ganglia and decreasing reserve of striatal neurons with age. Sydenham's chorea persisting into late life has not been reported previously.     

Successful treatment course with carbamazepine despite initial significant leukopenia: case report

A case of carbamazepine-induced leukopenia with subsequent successful carbamazepine management is presented. Despite WBC suppression to a level of 2000/cu mm on an initial trial of carbamazepine, a challenge with a much lower dose and gradual titration permitted a full treatment course with therapeutic blood levels. Although many physicians are wary of the leukopenia associated with carbamazepine, it is a distinct entity from the rare agranulocytosis and aplastic anemia that have been associated with the drug. With careful clinical management, carbamazepine can be successfully used in patients with neurologic disorders despite significant WBC suppression.     

Successful treatment of sexual disinhibition in dementia with carbamazepine -- a case report

Sexual disinhibition is a disturbing behavioral symptom in dementia. At present, there are no treatment guidelines available. Here we present the successful use of carbamazepine in a 78-year old AD patient with hypersexual behavior. The efficacy of carbamazepine in this case is in parallels to its effects on aggression and agitation in dementia and supports the important role of anticonvulsants in the management of behavioral disturbances in demented patients.     

Acute treatment of Huntington's chorea with lisuride

The authors studied the effects of lisuride hydrogen maleate (lisuride) on the hyperkinesias of 11 patients suffering from Huntington's chorea (HC). In all patients, acute injection of 150 micrograms of the drug induced a marked temporary improvement of the abnormal involuntary movements; the favourable drug-effect was more pronounced in the patients with a less severe degree of hyperkinesia. The antichoreic activity of the drug was prevented by pretreatment with haloperidol (2 mg) or sulpiride (400 mg), both injected intramuscularly 30 min before lisuride administration. The authors suggest the improvement of the motor disturbance induced in HC by lisuride may be explained on the basis of its preferential action on a subset of brain dopaminergic receptor.     

Corticosteroid treatment in patients with Sydenham's chorea

Sydenham's chorea occurs in approximately 10% of acute rheumatic fever and is one of its major manifestations. The disease may last for weeks or months, with a high risk of recurrence; usually only supportive treatment is recommended. This report describes five children diagnosed with Sydenham's chorea and treated with a short course of corticosteroids. Marked improvement of the involuntary movements was observed within 24-48 hours, with complete resolution within 7-12 days after commencement of treatment; there were no relapses. Larger, possibly comparative studies are necessary, but in the meantime treatment with corticosteroids in patients with Sydenham's chorea should be considered.     

The treatment of Huntington's chorea with belladonna alkaloids

Conclusions Although the series of cases presented is very small, the consistently beneficial results obtained by the use of belladonna alkaloids in the treatment of Huntington's chorea is most encouraging. It cannot be claimed that the belladonna alkaloids will prevent or arrest the chronic progressive degeneration. However, they do offer marked relief from the incapacitating neurological symptoms. This improvement in the somatic sphere is reflected in the psychic sphere. Personality changes are less prominent, and there is lessening in the apparent emotional and intellectual deterioration. Hospitalization does not become necessary so early and possibly may be prevented entirely. Hospitalized patients may be improved to the point where they can again return to the community. Further, it is believed that this treatment will prolong the lives of individuals suffering with Huntington's chorea by deferring the inevitable terminal complications.Presented at the interhospital conference, Syracuse Psychopathic Hospital, April 22, 1947.     

Comparison of the efficacy of carbamazepine,haloperidol and valproic acid in the treatment of children with Sydenham's chorea: clinical follow-up of 18 patients

In order to compare and contrast the efficacy of haloperidol, carbamazepine, and valproic acid in the treatment of Sydenham's chorea a prospective study including 18 cases of this disorder was undertaken. Age of patients ranged from 7 to 15 years. Ten children were female and 8 were male. All but one had generalized, either symmetric or asymmetric chorea. The patients were divided in three equal groups, and were given a standardized dose of each of the drugs built-up over a week. Following therapy, the six children receiving valproic acid showed remarkable improvement, without side effects. Five patients receiving carbamazepine showed improvement without side effects. Only three of the patients that received haloperidol improved. In the 4 cases that did not show clinical improvement after one week of treatment, therapy with valproic acid led to disappearance of the symptoms in a lapse that ranged from 4 to 7 days. Recurrence related to discontinuation of treatment was observed in two patients. In view of the present results we recommend valproic acid as the first choice drug to treat Sydenham chorea.     

Successful treatment with gabapentin in the presence of hypersensitivity syndrome to phenytoin and carbamazepine: a report of three cases.

We report three consecutive patients with hypersensitivity syndrome (HSS) due to phenytoin and carbamazepine and successful treatment with gabapentin. HSS is a rare but potentially fatal reaction to multiple drugs including several anticonvulsants. Cross-reactivity among drugs may occur. Immediate withdrawal of the offending drug is the most important step in treatment. Benzodiazepines acutely and, after resolution of the hepatitis, valproic acid have been successfully used for seizure control in patients with HSS. Our cases indicate that gabapentin is also a safe anticonvulsant in HSS.     

Long-term treatment of juvenile Huntington's chorea with dipropylacetic acid.

Since the proposed mode of action of dipropylacetic acid, an anticonvulsant, is to increase central nervous system gamma-aminobutyric acid levels, we used this agent to treat identical twins with juvenile Huntington's chorea. Their clinical status did not improve immediately after they received dipropylacetic acid. Furthermore, long-term administration (over a year) of high doses of the agent (up to 2,400 mg per day; 92 mg per kilogram per day) did not seem to alter the slow progression of their disease. Prior to treatment with dipropylacetic acid, the twins had normal cerebrospinal fluid gamma-aminobutyric acid levels. In addition, cerebrospinal fluid 5-hydroxyindoleacetic acid and homovanillic acid were determined before and after 18 hours of high-dose probenecid. The former showed a normal threefold to fourfold increase after probenecid administration, but homovanillic acid had a distinctly subnormal turnover after probenecid, with only a threefold rather than the normal tenfold increase.     

The treatment of affective disorder with carbamazepine: prophylactic synergism of lithium and carbamazepine combination.

1. In order to examine the prophylactic interaction between lithium and carbamazepine (CBZ), 18 patients who had been treated prophylactically with a combination of lithium and CBZ (combination therapy), lithium alone (Li-therapy), or CBZ alone (CBZ-therapy) were investigated in terms of episode occurrence. 2. The results revealed that the duration of symptoms and the frequency of hospitalization per year were significantly lower in the combination therapy than in both the Li-therapy and the CBZ-therapy. 3. In 7 out of 18 patients, the best prophylactic effect was obtained during combination therapy and none of the parameters measured was definitely inferior to those measured during the two single therapies. 4. During combination therapy, serum lithium level was significantly lower than during Li-therapy in the CBZ responders, and the combination of the two drugs enabled the required concentrations of lithium to be decreased. 5. It was concluded that synergistic action and a decrease in required concentrations of lithium can be expected with the combined use of lithium and CBZ, especially in responders to CBZ.     

Sydenham's chorea: clinical findings and comparison of the efficacies of sodium valproate and carbamazepine regimens

BACKGROUND: Sydenham's chorea is still the most frequently seen form of acquired chorea in childhood in developing world despite the use of antibiotics. It is a debilitating illness lasting for weeks or months and requires drug therapy. OBJECTIVE: To evaluate and compare the efficacies of sodium valproate and carbamazepine in the treatment of the choreiform movements in Sydenham's chorea. DESIGN: A prospective trial carried out with 24 children with Sydenham's chorea. Patients: Twenty-four patients were divided into two groups having similar demographic and clinical properties. One group (n = 17) was given carbamazepine (15 mg/kg per day) and the other (n = 7) was given sodium valproate (20-25 mg/kg per day). As soon as the symptoms were taken under control, doses of the drugs were tapered slowly. The duration of the drug use was recorded. The time of response to therapy was compared between the groups and the patients were monitored for the adverse effects. RESULTS: There was no significant difference between the groups with respect to the time of clinical improvement and time of complete remission, duration of the therapy and the recurrence rates. Clinical improvement began by 8.0 +/- 4.0 days in sodium valproate and 7.4 +/- 8.2 days in carbamazepine group (P = 0.88). In the whole group no adverse effect was seen due to the drugs. CONCLUSION: Carbamazepine and valproic acid are equally effective and safe drugs in the treatment of choreiform movements in Sydenham chorea.     

Tetrabenazine treatment for Huntington's disease-associated chorea

Tetrabenazine (TBZ), a monoamine depleter and dopamine receptor blocker, is used to treat a variety of hyperkinetic movement disorders. The objective was to study the efficacy and tolerability of TBZ for chorea associated with Huntington's disease (HD). Nineteen patients (12 female), mean age 56.3 +/- 12.4 years (range 37-76 years) diagnosed with HD were prospectively evaluated at initial and follow-up visits using a modified Abnormal Involuntary Movement Scale (AIMS). Patients were videotaped, and the randomized videotapes were rated with the motor subset of the AIMS by two investigators who were blinded to treatment assignment. Eighteen patients completed and were rated after 5.9 +/- 3.3 months (range 2-11) at a final mean TBZ dose of 62.5 +/- 37.4 mg/day (range 25-150). The blinded videotaped motor scores showed that 15 were better on TBZ, 2 were better before TBZ, and 1 was unchanged (p < 0.001, Wilcoxon signed rank test). The mean score improved from 16.2 +/- 4.8 to 12.8 +/- 4.4. Adverse events included akathisia, insomnia, constipation, depression, drooling, and subjective weakness. All 18 of these patients have continued to take TBZ since completion of the study. TBZ was well tolerated and resulted in a significant improvement in modified AIMS scores in HD patients. These results support the use of TBZ for chorea in patients with HD.     

Withdrawal from controlled carbamazepine therapy followed by further carbamazepine treatment in patients with dementia.

BACKGROUND: The aim of this study was to assess the effects of withdrawal from placebo and carbamazepine administered for agitation associated with dementia and to assess safety, tolerability, and efficacy of subsequent ongoing carbamazepine therapy. METHOD: We previously reported the results of a 6-week, randomized, parallel-group study of placebo versus carbamazepine in 51 nursing home patients with dementia who were agitated; 47 subjects completed that study. This report first presents the results of withdrawal from that experimental treatment assessed by (blinded) observations 3 weeks later (N = 45 remaining). The primary outcome measure was the Brief Psychiatric Rating Scale. Secondary outcome measures addressed other aspects of behavior, cognition, function, safety, and tolerability. Patients were then treated with carbamazepine for an additional 6 weeks (N = 32 remaining) or 12 weeks (N = 25 remaining), with the same assessments performed. RESULTS: Patients who had previously shown behavioral improvement with carbamazepine therapy reverted to their baseline state after washout, whereas there was no change in the patients previously treated with placebo. There were no other significant effects of washout. During subsequent therapy with carbamazepine at a modal dose of 300 mg/day, there were 2 deaths and 4 other adverse events resulting in dropout. Neither of the deaths, and only 1 serious adverse experience, was judged to be related to carbamazepine. There were a variety of nonserious adverse experiences during the trial. Behavior ratings showed ongoing improvement in agitation and aggression, as well as in other aspects of psychopathology. CONCLUSION: The washout data provided independent confirmation of efficacy found in the prior placebo-controlled phase of this trial. Ongoing treatment was not associated with unexpected toxicity and was associated with improvement in measures of agitation and aggression that appeared to continue for up to 12 weeks. These findings confirm and extend results from earlier placebo-controlled studies.