Chorea Patients Research Articles

Impact of Chorea on Self-care Activity, Employment, and Health-care Resource Use in Patients with Huntington's Disease.

Background: Chorea is recognized as a prototypic motor feature of Huntington’s disease (HD), but its effect on health-related quality of life (HRQoL) has not been fully explored. This study describes the impact of chorea on HRQoL in patients with HD.Objective: To determine the impact of HD-related chorea on employment, self-care activities, activities of daily living, and health-care resource utilization (HCRU).Methods: Data were drawn from the Adelphi HD Disease Specific Programme, a real-world point-in-time survey of 144 neurologists and 427 patients in the United States between July and October 2017. HD patients with and without chorea were identified and examined for differences in employment status, reasons for employment changes, self-care activities, and modifications to cope with involuntary movements. Bivariate tests and inverse probability weighted regression adjustment methods were used to determine differences in outcomes between patients with and without chorea.Results: HD patients with (n=287) and without (n=140) chorea were identified. Patients with chorea were less likely to be employed full-time (16.7% vs 25.7%; P<0.04) and more likely to be on long-term sick leave (17.4% vs 5.0%; P<0.01). The onset of motor symptoms in HD-related chorea patients coincided with a change in employment status (42.7% vs 20.8%; P<0.01). Among those still working (n=145), more than two-fifths of patients with chorea required changes to their workplace and required these changes more frequently (45% vs 17%; P<0.001). HD patients with chorea required aid to help them get around significantly more frequently than those without chorea (55% vs 34%; P<0.001).Discussion: These results demonstrate that HD patients with chorea experienced greater negative impact to employment, self-care activities, and HCRU than patients without chorea experienced. These patients were more likely to stop working due to motor, cognitive, and behavioral symptoms; require modifications in the home and workplace; and need more assistance from caregivers than patients without chorea.Conclusions: Patients with HD-related chorea have greater detriments to emotional, interpersonal, and professional functioning that could be improved by reducing chorea.

Impact of Chorea on Self-care Activity, Employment, and Health-care Resource Use in Patients with Huntington’s Disease

Background: Chorea is recognized as a prototypic motor feature of Huntington’s disease (HD), but its effect on health-related quality of life (HRQoL) has not been fully explored. This study describes the impact of chorea on HRQoL in patients with HD. Objective: To determine the impact of HD-related chorea on employment, self-care activities, activities of daily living, and health-care resource utilization (HCRU). Methods: Data were drawn from the Adelphi HD Disease Specific Programme, a real-world point-in-time survey of 144 neurologists and 427 patients in the United States between July and October 2017. HD patients with and without chorea were identified and examined for differences in employment status, reasons for employment changes, self-care activities, and modifications to cope with involuntary movements. Bivariate tests and inverse probability weighted regression adjustment methods were used to determine differences in outcomes between patients with and without chorea. Results: HD patients with (n=287) and without (n=140) chorea were identified. Patients with chorea were less likely to be employed full-time (16.7% vs 25.7%; P&lt;0.04) and more likely to be on long-term sick leave (17.4% vs 5.0%; P&lt;0.01). The onset of motor symptoms in HD-related chorea patients coincided with a change in employment status (42.7% vs 20.8%; P&lt;0.01). Among those still working (n=145), more than two-fifths of patients with chorea required changes to their workplace and required these changes more frequently (45% vs 17%; P&lt;0.001). HD patients with chorea required aid to help them get around significantly more frequently than those without chorea (55% vs 34%; P&lt;0.001). Discussion: These results demonstrate that HD patients with chorea experienced greater negative impact to employment, self-care activities, and HCRU than patients without chorea experienced. These patients were more likely to stop working due to motor, cognitive, and behavioral symptoms; require modifications in the home and workplace; and need more assistance from caregivers than patients without chorea. Conclusions: Patients with HD-related chorea have greater detriments to emotional, interpersonal, and professional functioning that could be improved by reducing chorea.

Adult-onset sporadic chorea: real-world data from a single-centre retrospective study

BackgroundAdult-onset sporadic chorea includes a wide and heterogeneous group of conditions whose differential diagnosis and treatments are often challenging and extensive.ObjectivesTo analyse retrospectively cases of adult-onset sporadic chorea from a single Italian centre to provide insights for a practical approach in the management of these patients.MethodsA total of 11,071 medical charts from a 9-year period (2012–2020) were reviewed, identifying 28 patients with adult-onset sporadic chorea (genetic forms excluded). All available data regarding phenomenology, diagnostic workup, aetiology, treatments, and long-term outcome from this cohort were collected and analysed.ResultsAdult-onset sporadic chorea occurred more frequently in females and presented with an acute-subacute onset. Cerebrovascular diseases accounted for 68% of aetiology; further causes were structural brain lesions, internal diseases, and other movement disorder syndromes. Clinical course was mild, with spontaneous resolution or minimal disturbances in 82% of cases. Neuroimaging was fundamental to diagnose 76% of adult-onset sporadic chorea, an appropriate clinical examination contributed to the 14% of diagnoses, whereas basic laboratory tests to the 10%.ConclusionsRevision of real-world data of adult-onset sporadic chorea patients from a single Italian cohort suggests that an accurate clinical examination, neuroimaging, and routine laboratory tests are useful to identify those cases underlying potentially severe but treatable conditions. Although in the majority of cases adult-onset sporadic chorea has mild clinical course and good response to symptomatic treatments, it is essential to run a fast diagnostic workup.

Evaluation of clinical findings and treatment options of Sydenham chorea patients

Background/Aim: Sydenham chorea is an autoimmune neurological disorder of the childhood which occurs due to cross-reaction of antibodies against group A beta-hemolytic streptococci in the basal ganglia. We evaluated patients with Sydenham’s chorea, treatment options, recovery duration, and relapses to determine whether there is any relationship between biochemical parameters such as erythrocyte sedimentation rate (ESR), serum Anti Streptolysin Antibody (ASA), and patients’ clinical course. Methods: This case series includes patients with Sydenham chorea who visited the pediatric neurology outpatient clinics between May 2013 and September 2018. Neurologic examination was performed by a pediatric neurologist, and electrocardiography and echocardiography were performed by a pediatric cardiologist. ESR and ASA levels, treatment options, and clinical course of the disease were evaluated. Results: Sixteen patients, with nine females (56.3%) and seven males (43.7%) were included in this study. The most seen chorea type was hemichorea. The median ASA and ESR values of the patients were 619 IU/ml (278.25-794.75) and 17.5 mm/h (7.25-27), respectively. Their median age and time until recurrence were 12 (9-14.25) years and 16 months (9-18), respectively. The median recovery period was 5.5 months (3-6). Diazepam and haloperidol were the most used secondary treatment options. Mitral insufficiency (MI) was the most frequent finding (56.3%). Benzathine penicillin and secondary prophylaxis, e.g., haloperidol, diazepam were our treatment agents of choice. ASA levels were lower among patients treated with steroids (U=9, z=-2.38, P=0.017). Age was moderately positively correlated with recovery period (age r (14) = 0.738, P=0.001)), while no correlation was found between age and ESR, or recurrence period (P=0.98, P=0.33, respectively). The recovery period of generalized chorea was longer than that of hemichorea (U=10.5, z=-1.96, P=0.05). Recurrence was not related to ASA levels, ESR levels, age, or recovery period (P=0.73, P=0.89, P=0.78, P=0.83 respectively). Conclusion: High ASA levels may not indicate steroid need, and the recovery period increases with age and in cases of generalized chorea. Instead of benzathine penicillin or other known secondary prophylactic agents, azithromycin and levetiracetam can be used in hypersensitive patients.

A reversible lentiform nucleus T1 hyperintensity in hypoglycemic chorea

Acute to subacute onset chorea is commonly due to stroke, metabolic, post-infectious, and autoimmune disorders. Chorea in diabetes (DM) patient is mostly due to hyperglycemia, and few cases were reported due to hypoglycemia. The mechanism at the cellular level is still evolving. The MRI changes in diabetic chorea patients are variably reported. It is important to discuss our patient with hypoglycemic hemichorea because of unique features like low BMI, recently diagnosed DM on sulphonylurea with a history of hypoglycemic episodes, and acute left hemichorea that was controlled with neuroleptics, and her MRI brain showed reversible lentiform nucleus T1 hyperintensity without diffusion restriction and blooming on SWI.

Sydenham Chorea: Putaminal Enlargement

Our aim in this study was to evaluate the efficacy of magnetic resonance imaging (MRI) studies in the detection of brain regions effected by Sydenham chorea and to determine whether they provided data regarding the pathogenesis of Sydenham chorea. To this end, we assessed basal ganglia structures in Sydenham chorea patients and control group by quantitative MRI volumetric analysis. Patients with a recent onset of chorea and control subjects matched for age and gender were included in the study. Medical history, laboratory tests, and physical and neurologic examinations were reviewed. All MRIs were considered within normal limits. High-resolution T1-weighted 3D magnetization-prepared rapid acquisition of gradient echo scans were used for quantitative volumetric assessment of the brain via the "volBrain" method. Twenty-four subjects with Sydenham chorea (16 girls and 8 boys, aged between 7 and 16 years) and 35 control subjects were evaluated. Mean age was 11.25 ± 2.89 years for Sydenham chorea patients and 10.58 ± 2.53 years for the controls. No significant difference was found relative to globus pallidus, caudate, and thalamic volumes between patients with Sydenham chorea and controls. The relative mean total, left, and right putamen volumes were significantly larger in patients with Sydenham chorea compared to controls (P = .003, P = .018, P = .001, respectively). Selective neuroanatomic differences in putamen among other basal ganglia structures and significant increases in size are consistent with a hypothesis of a cross-reactive antibody-mediated inflammation of the putamen as being the pathophysiologic mechanism for this disorder.

Kore Ön Tanılı Hastaların Değerlendirilmesi: Sydenham Koresi ve Ayırıcı Tanılar

Objective: Our aim is to evaluate patients being referred with an initial diagnosis of chorea according to their clinical, laboratory features and final diagnoses while emphasizing cardiological findings of patients with Sydenham chorea. Method: Children aged 4-18 years who were referred to Okmeydanı Research and Training Hospital Pediatric Neurology department with an initial diagnosis of acute, subacute chorea between January 2017-January 2020 were retrospectively included. Chronic chorea and diseases associated with chronic chorea were excluded from the study. Data concerning clinical, laboratory features, cardiological findings, etiologies, treatments, recurrence rates and follow-ups of patients were recorded. Descriptive statistical analysis were performed using SPSS 21.0. Results: Fifteen patients has been referred with the initial diagnosis of chorea. Mean age of the patients was 11.5±2.2 years. Ten (67%) patients were females, 5 (33%) patients were male. After admission, 8 (54%) patients were diagnosed with Sydenham chorea, and 2 (13%) patients with recurrent Sydenham chorea. During physical examination, 5 (33%) patients did not have chorea, and 3 cases had tic disorder. Out of 8 patients with new diagnosis of Sydenham chorea, 3 (37.5%) patients had subclinical carditis, and 5 (62.5%) patients clinical carditis. Chorea had been treated with one of haloperidol/biperiden, valproic acid and prednisolon options. The treatment of 6 patients attending regular follow-up visits was stopped 2-6 months later. Chorea of two patients recurred during our follow-up, and one of our newly diagnosed Sydenham chorea patients had been recognized as antiphospholipid antibody syndrome after recurrence. Conclusion: Sydenham chorea is the most common cause of acquired chorea in childhood. Most of the time it is self limiting. Differential diagnosis of chorea must be kept in mind especially when there is a recurrence.

Significance of silent carditis and investigation of follow-up signs in acute rheumatic fever.

This study aimed to demonstrate the importance of ECHO in the diagnosis and long-term follow-up of silent carditis. This study included 182 (157 arthritis; 25 chorea) patients out of 214 patients who had been diagnosed with acute rheumatic fever for the first time. All of the patients were scanned with ECHO between specific intervals. While there was no recovery observed in isolated aortic insufficiency during long-term follow-up, recovery in isolated mitral insufficiency was found. As the follow-up time of the mitral and aortic insufficiencies became longer, there was an increase in recovery in aortic insufficiency. Follow-up results of silent carditis support that ECHO should be among the major criteria used to diagnose rheumatic carditis.

Auditory p300 event-related potentials in children with Sydenham's chorea.

P300 event-related potentials (ERPs), objective measures related to cognitive processing, have not been studied in Sydenham's chorea (SC) patients. To assess cognitive impairment with P300 ERPs. Seventeen patients with SC and 20 unaffected healthy children were included. Stanford-Binet test was used for psychometric assessment, and odd-ball paradigm was used for auditory ERPs. There was no significant difference in P300 latencies between the SC-pretreatment group, SC-posttreatment group and control group (p>0.05). Mean interpeak latencies in SC-pretreatment group and SC-posttreatment group showed significant prolongation compared with the control group (p<0.05). Mean interpeak latencies in SC-posttreatment group were significantly decreased compared with SC-pretreatment group (p<0.05). Compared to controls, patients did not show significant difference in Stanford-Binet intelligence examination. This report suggests that interpeak latencies and amplitudes of P300 ERPs could be useful for detecting and monitoring cognitive impairment in SC patients.

Safety and Efficacy of Tetrabenazine and Use of Concomitant Medications During Long-Term, Open-Label Treatment of Chorea Associated with Huntington's and Other Diseases.

Although tetrabenazine, a drug that depletes presynaptic dopamine by inhibiting vesicular monoamine transporter 2 (VMAT2), was approved by the U.S. Food and Drug Administration in 2008 for the treatment of chorea associated with Huntington's disease (HD), there is a paucity of data on its long-term efficacy and safety. Approximately 2,000 patients with a variety of hyperkinetic movement disorders had been treated with open-label tetrabenazine at the Movement Disorders Clinic, Baylor College of Medicine, since 1979. Tetrabenazine was usually started at 12.5 mg/day, and the dosage was gradually increased (up to 300 mg/day). Responses were rated by the investigator 1-5, with 1 = marked chorea reduction, excellent improvement in function; 2 = moderate chorea reduction, very good improvement in function; 3 = fair chorea improvement, only mild improvement in function; 4 = poor or no response for chorea and function; and 5 = worsening chorea, some functional deterioration. Efficacy and safety were analyzed retrospectively. By 2004, 98 HD chorea patients had received tetrabenazine for a mean of 3.1 years (range ≤1-11.4 years). Of those with valid ratings, 75% had either marked or very good responses (rating 1 or 2) at their optimal dosages. The most common adverse events occurring in ≥5% of the patients were somnolence (39%), insomnia (33%), depression (31%), accidental injury (26%), and dysphagia (19%). Efficacy and safety were comparable to results for non-HD chorea patients. Tetrabenazine treatment was associated with long-term improvement in chorea. Adverse event rates were comparable to those reported from controlled trials.

Apparent Relative Hypermetabolism of Selective Brain Areas in Huntington Disease and Importance of Reference Region for Analysis

We investigated the pattern of reduced and increased metabolism in symptomatic Huntington chorea (HD) patients and assessed the influence of intensity normalization method. FDG brain PET was performed in 11 manifest HD patients and 11 age-matched controls. Images underwent statistical parametric mapping analysis after global normalization or with white matter or pons as reference region. Region of interest analysis of FDG uptake ratios was also performed using total region or white matter as reference. Correlation between regional uptake ratios to that of the caudate was evaluated by linear regression analysis. Although statistical parametric mapping analysis identified striatal hypometabolism and thalamic hypermetabolism in HD patients with all methods, substantially different FDG uptake patterns were shown depending on the method of intensity normalization. Global normalization displayed large areas of relative normalized hypermetabolism across the cerebral cortex and cerebellum. With white matter as reference, relative normalized hypermetabolism was observed in the thalamus and a much more limited area of the cortex. With pons as reference, relative normalized hypermetabolism was largely confined to the thalamus and a small area of the cerebellum. These results demonstrate apparent relative normalized hypermetabolism in selective brain regions in manifest HD patients. Furthermore, the choice of reference region for intensity normalization is critical for accurate monitoring of this metabolic change with FDG PET.

Tetrabenazine for Moderate vs. Severe/Disabling Chorea Associated with Huntington's Disease (HD) (P06.032)

Objective: To assess dosing, efficacy, and safety of tetrabenazine for patients with moderate vs. severe/disabling HD-chorea. Background At study initiation (1979), tetrabenazine had not yet been approved in the US (2008). Design/Methods: In an open-label study, patients with hyperkinetic movement disorders were evaluated at Baylor College of Medicine. Tetrabenazine was used “last resort,” when other medications failed to provide satisfactory control. For HD-chorea patients, all previous chorea treatments were discontinued before tetrabenazine initiation. Patients were initially hospitalized, and tetrabenazine was started at 12.5 mg/day (≤300 mg/day maximum). Dosage was increased every 3 days, until a dosage-limiting AE occurred, and then down-titrated to greatest tolerated dosage. Visits were 6 weeks after hospitalization, and every 3 months thereafter. Responses were rated on a scale of 1–5 (1 = marked chorea reduction, excellent improvement in function; 2 = moderate chorea reduction, very good improvement in function; 3 = fair chorea improvement, only mild improvement in function; 4 = poor/no response for chorea/function; 5 = worsening chorea/some functional deterioration). 1 Results: By 2004, 98 HD-chorea patients had participated. At baseline, 44 had moderate and 54 had severe/disabling chorea. 45% with moderate vs. 61% with severe/disabling chorea received tetrabenazine >2 years. Average daily dosages (SD; range of mean dosages) were 60.5 mg (25.9; 16.9–138.1) and 74.8 mg (45.0; 21.4–225.5) for moderate and severe/disabling chorea, respectively. On optimal dosages, 71% of moderate chorea patients achieved a marked or very good rating (any time point) vs. 78% for severe/disabling. Five most common AEs (moderate, severe/disabling) were somnolence (43%, 22%), insomnia (16%, 15%), depression (23%, 11%), akathisia (7%, 15%), and nervousness (7%, 13%). Conclusions: Tetrabenazine dosing is highly individualized, independent of chorea severity. Responses to tetrabenazine and AE rates were similar for patients grouped by chorea severity. 1 Jankovic J, et al. Neurology . 1988;38:391–4. Supported by: Lundbeck Inc. Disclosure: Dr. Shen has received personal compensation for activities with Lundbeck Inc as an employee. Dr. Clarence-Smith has received personal compensation for activities with Lundbeck Research USA, Inc. Dr. Hunter has received personal compensation for activities with Lundbeck Research USA, Inc. as a consultant. Dr. Jankovic has received personal compensation for activities with Allergan, Inc., Chelsea Therapeutics, Serono Inc., Merz Pharma, Lundbeck Research USA, Inc, Teva Neuroscience as a consultant. Dr. Jankovic has received personal compensation in an editorial capacity for Medlink: Neurology in Clinical Practice. Dr. Jankovic has received research support from Allergan, Inc, Allon Therapeutics, Ceregene, Inc., Chelsea Therapeutics; Diana Helis Henry Medical Research Foundation, Serono Inc., Huntington9s Disease Society of America, Huntington Study Group, Impax Pharmaceuticals, Ipsen Limited, Lundbeck Research USA, Inc.

Striatal Microinjection of Sydenham Chorea Antibodies: Using a Rat Model to Examine the Dopamine Hypothesis

We hypothesized that injection of anti-basal antibodies from patients with Sydenham's chorea into rats' striatum will induce behavioral and histological changes. Antibodies from eight Sydenham's chorea patients and eight age-matched controls were injected into the left caudate of 16 rats. Apomorphine- and amphetamine-induced rotations were performed on days 10 and 17, respectively, followed by immunohistochemical studies. Antibodies from patients with Sydenham's chorea, but not controls, bound to a ∼50-kDa molecule in the striatum extract; immunohistology staining demonstrated specific binding to cellular component(s) in rats' striatum. Contrary to our hypothesis, we could not detect in the rats injected with Sydenham's chorea antibodies changes in rotational behavior or immunohistochemistry staining for dopaminergic or GABAergic markers. Injection of small quantities of anti-neuronal antibodies present in patients with Sydenham's chorea into rat striatum is insufficient to alter motor behavior or cause detectable cellular changes.

The Prevalence of Neuropsychiatric Disorders in Sydenham's Chorea

Sydenham's chorea is a rare movement disorder associated with streptococcal infection. The co-occurrence of neuropsychiatric symptoms has raised the question of whether streptococcal infection could trigger these symptoms without chorea. This study evaluated the prevalence of behavioral diagnoses before, during, and after the onset of chorea in a cohort of children with a history of Sydenham's chorea for whom demographic and clinical data were available. In all, 28 Sydenham's chorea patients were evaluated, with a mean age of 10.3 years. Retrospective analysis was performed for subject demographics, streptococcal titers, and presence of arthritis and carditis. Structured diagnostic interviews were performed on 14 available patients and parents. Streptococcal titers and duration of treatment for chorea were compared between groups. The prevalence of attention deficit hyperactivity disorder before and after chorea was 30 and 37%, respectively. The proportion of children meeting combined, subthreshold, and suprathreshold criteria before, during, and after Sydenham's chorea was 71, 79, and 79% for anxiety and 19, 69, and 44% for depression. Streptococcal antibody titers and duration of treatment did not correlate with attention deficit hyperactivity disorder, depression, or anxiety disorders. During and after the diagnosis of Sydenham's chorea, clinicians should be vigilant for signs and symptoms of anxiety, depression, and attention deficit hyperactivity disorder.

EXTRAPYRAMIDAL SIGNS ARE A COMMON FEATURE OF SPINOCEREBELLAR ATAXIA TYPE 17

Spinocerebellar ataxia type 17 (SCA17) is a CAG repeat expansion disorder in the TATA-binding protein (TBP) gene.1 Previous studies have shown that SCA17 is a rare cause of ataxia, especially in the East.2,3 SCA17 may show nonataxic manifestations, such as choreic movement, cognitive decline, dystonia, parkinsonism, and pyramidal tract signs. Previously we reported that SCA17 is not rare among our parkinsonian patients (0.9%).4 Thus, we examined the frequency of SCA17 among Korean patients with ataxia or chorea as a main phenotype. ### Methods. Gene samples obtained from the Movement Disorder Gene Bank of Seoul National University Hospital collected until April 2007 were analyzed. All patients were native Koreans. Written informed consent was obtained from all subjects before blood sampling for gene studies, and the genetic study was approved by our Institutional Review Board. Patients with a positive gene test for SCA1, 2, 3, 6, 7, dentatorubropallidoluysian atrophy, or Huntington disease (HD) were excluded. In both groups, acquired causes were excluded. We did not exclude the patients with familial history of ataxia or chorea. Finally, 661 ataxia patients and 98 chorea patients were included (2 patients had both ataxia and chorea). The method of gene analysis used is described in the previous report.4 ### Results. The 2 patients with both ataxia and chorea were found to have …

Huntington's disease: genetic heterogeneity in black African patients.

Huntington's disease (HD) has been reported to occur rarely in black patients. A new genetic variant- Huntington's disease-like 2 (HDL2)--occurring more frequently in blacks, has recently been described. The absence of an expanded trinucleotide repeat at the chromosome 4 HD locus was previously regarded as a way of excluding classic HD. The objective of this paper is to describe a number of black patients with genetically proven HD and to review its occurrence in Africa. Eleven black families (12 subjects), with genetically proven HD, are described: 9 from the Dr George Mukhari Hospital, and 2 from private practice in Tshwane. Chorea was present in all 12 patients and cognitive decline in 9. Nine had an age of onset between 30 and 50 years. Six families exhibited expansion of the trinucleotide repeat at the chromosome 4, IT 15 gene (HD), and 5 a junctophilin (JPH3) trinucleotide expansion at chromosome 16 (HDL2). The HDL2 subtype showed a tendency towards a later age of onset. The clinical presentation of the two genotypes (i.e. HD and HDL2) appears to be similar. The actual rate of occurrence of HD in blacks may require re-assessment. Considering the number of Huntington's chorea patients occurring in our area (Garankuwa), the possibility of clustering of the condition arises.

Sydenham's Chorea: A Clinical Follow-Up of 65 Patients

Sydenham's chorea, the neurological manifestation of rheumatic fever, is the most common acquired chorea of childhood. In this retrospective study, the authors aim to present the clinical and laboratory findings of 65 Sydenham's chorea patients, followed up in a clinic over less than 7 years. The mean age at the onset of the symptoms was 11.7 +/- 2.6 years (range, 6-17 years). Of the patients, 63% were female and 37% were male (male/female: 1.7/1). Chorea was generalized in 78.5% of the patients, right hemichorea in 12.3%, and left hemichorea 9.2%. There was a history of rheumatic fever in 30.8% of the patients. Echocardiographic study showed cardiac valve involvement in 70.5% of 61 patients. Brain magnetic resonance imaging, which was performed on only 18 patients, was evaluated as normal in all. Electroencephalography was also performed on only 18 patients and showed abnormal waves in 50% of them. Pimozide was mostly the first choice of drug therapy. Nevertheless, drug therapy was not needed in 18.5% of the patients. The recovery period of the first attack of the chorea was 1 to 6 months in 51.7% of the patients. The recurrence rate was 37.9%. In conclusion, Sydenham's chorea is still an important health problem in Turkey with respect to its morbidity.

HLA class II DR and DQ genotypes and haplotypes associated with rheumatic fever among a clinically homogeneous patient population of Latvian children

The HLA system is being paid more and more attention because it is very significant in polymorphous immunological reactions. Several studies have suggested that genetic susceptibility to rheumatic fever (RF) and rheumatic heart disease (RHD) is linked to HLA class II alleles. We hypothesized that HLA class II associations within RHD may be more consistent if analysed amongst patients with a relatively homogeneous clinical outcome. A total of 70 RF patients under the age of 18 years were surveyed and analysed in Latvia. HLA genotyping of DQA1, DQB1 and DRB1 was performed using PCR with amplification with sequence-specific primers. We also used results from a previous study of DQB1 and DRB1 genotyping. In the RF patients, HLA class II DQA1*0401 was found more frequently compared to DQA1*0102. In the RF homogeneous patient groups, DQA1*0402 has the highest odds ratio. This is also the case in the multivalvular lesion (MVL) group, together with DQA1*0501 and DQA1*0301. In the chorea minor patients, DQA1*0201 was often found. Significant HLA DQA1 protective genotypes were not detected, although DQA1 genotypes *0103/*0201 and *0301/*0501 were found significantly and frequently. In the distribution of HLA DRB1/DQA1 genotypes, *07/*0201 and *01/*0501 were frequently detected; these also occurred significantly often in the MVL group. The genotype *07/*0201 was frequently found in Sydenhamn's chorea patients that had also acquired RHD, but DRB1*04/DQA1*0401 was often apparent in RF patients without RHD. In the distribution of HLA DQA1/DQB1 genotypes, both in RF patients and in the homogeneous patient groups, the least frequent were *0102/*0602-8. The genotype DQA1*0501 with the DQB1 risk allele *0301 was often found in the MVL group. The genotype *0301/*0401-2 was frequently found in the RF and Sydenhamn's chorea patient groups. The haplotype *07-*0201-*0302 was frequently found in RF and homogeneous patient groups, including the MVL group. In addition, haplotypes *04-*0401-*0301 and *04-*0301-*0401-2 were frequent amongst patients with Sydenhamn's chorea. The protective alleles DQA1*0102 and DQB1*0602-8 in the haplotype DRB1*15 were less frequently found in RF patients. The results of the present study support our hypothesis and indicate that certain HLA class II haplotypes are associated with risk for or protection against RHD and that these associations are more evident in patients in clinically homogeneous groups.

Migraine Headache in Patients with Sydenham's Chorea

We compared the frequency of migraine among Sydenham's chorea (SC) patients, rheumatic fever (RF) patients without neurological symptoms and matched controls. Migraine was more frequent in SC patients (12/55, 21.8%) than in controls (9/110, 8.1%) and as common as in the RF group (10/55, 18.2%). Our data are in agreement with previous studies reporting higher frequency of migraine in other basal ganglia disorders, such as essential tremor and Tourette's syndrome.

Serum from Sydenham's chorea patients modifies intracellular calcium levels in PC12 cells by a complement‐independent mechanism

The proposed pathogenesis of Sydenham's chorea (SC) is an autoantibody-mediated basal ganglia dysfunction. Our study has shown that incubation of PC12 cells with complement-inactivated serum from SC patients was associated with a significant increase in Ca2+ levels evoked by KCl stimulus (mean +/- SEM, 341.0 +/- 8.7% of fluorescence intensity, arbitrary units) when compared with incubation with control serum (313.8 +/- 8.7% of fluorescence intensity, arbitrary units; P = 0.01). The increase in Ca2+ levels determined by SC patients sera correlated directly with the enzyme-linked immunosorbent assay optical density values for anti-basal ganglia antibodies. Our study supports the hypothesis that antibodies against basal ganglia in SC may cause their dysfunction.