Clinical patterns of dystonia and choreoathetosis in participants with dyskinetic cerebral palsy.

AIM: The aim of the study was to map clinical patterns of dystonia and choreoathetosis and to assess the relation between functional classifications and basal ganglia and thalamus lesions in participants with dyskinetic cerebral palsy (CP). METHODS: In this cross-sectional study, 55 participants with dyskinetic CP (mean age 14y 6mo, SD 4y 1mo; range 6-22y) were assessed with the Dyskinesia Impairment Scale and classified with the Gross Motor Function Classification System (GMFCS), Manual Ability Classification System (MACS), and Communication Function Classification System (CFCS). RESULTS: Dystonia and choreoathetosis are simultaneously present. Median levels of dystonia (70.2%) were significantly higher than levels of choreoathetosis (26.7%) and both were significantly higher during activity than at rest (both p<0.01). High correlations were found between dystonia levels and GMFCS level (Spearman's rank correlation coefficient, rS =0.70; 95% confidence interval [CI] 0.53-0.81; p<0.01) and MACS (rS =0.65; 95% CI 0.47-0.81; p<0.01), and fair correlation with CFCS (rs =0.36; 95% CI=0.11-0.57; p<0.05). No significant correlation was found between choreoathetosis levels and motor classifications. Finally, higher choreoathetosis levels were found in participants with pure thalamus and basal ganglia lesions (p=0.03) than mixed lesions, but not for dystonia (p=0.41). INTERPRETATION: Dystonia and choreoathetosis increase during activity. However, dystonia predominates and seems to have a larger impact on functional abilities. Our findings further suggest that choreoathetosis seems to be more linked to pure thalamus and basal ganglia lesions than dystonia.

Exploración neurológica y atención primaria. Bloque II: motilidad voluntaria, funciones corticales superiores y movimientos anómalos / Neurological examination and primary care. Block II: voluntary motility. Higher cortical functions. Abnormal movements

La exploración neurológica es, junto con la anamnesis, la base del diagnóstico de la patología del sistema nervioso. El médico de Atención Primaria, con un acceso limitado a pruebas de alta resolución, debe de conocer los aspectos más importantes de dicha exploración, siendo importante que esta se realice de manera sistemática.En un primer bloque se abordó la exploración de los pares craneales, la sensibilidad, los signos meníngeos, el cerebelo y la coordinación, y en esta segunda parte se analizan la función motora, las funciones corticales superiores y los movimientos anómalos (AU)

The neurological examination is, along with the anamnesis, the basis of the diagnosis of nervous system disease. The Primary Care doctor, with limited access to high resolution tests, must know the most important aspects of this examination; the most important being that this is carried out systematically. In the first Block, the examination of the cranial pairs, sensitivity, meningeal signs, cerebellum and coordination were looked at. In this second Block, motor function, higher cortical functions and abnormal movements are analysed (AU)

Drooling of saliva in children with cerebral palsy-etiology, prevalence, and relationship to salivary flow rate in an Indian population.

The prevalence of drooling, the impact of various etiological factors on its severity, and its relationship to salivary flow rate were assessed in 113 individuals with cerebral palsy (CP). In this study, there were 74 males and 39 females with an age range of 6-18 years who were attending a special school in Chennai, India. The severity of drooling was assessed by visual examination; demographics and data regarding severity and control of drooling were collected via a questionnaire. Data regarding the type of CP and drugs taken by the children were obtained from the medical records maintained by the school. The unstimulated salivary flow rate of these children was determined through a passive method. The overall incidence of drooling was 48.7%, with 17.7% having severe drooling. Individuals with athetosis had the least severe amount of drooling. There was a significant relationship between ability to close the mouth and the severity of drooling. The severity of drooling was reduced with age. There was no significant difference in the mean salivary flow rate of those children who drooled and those who did not.

Paroxysmal kinesigenic choreoathetosis: a report of 26 patients.

Paroxysmal kinesigenic choreoathetosis (PKC) is a neurological condition which results in abnormal involuntary movements that are precipitated by sudden movement. Because of its rarity, large case series of PKC have not been published. We studied 26 patients with PKC, which represents the largest series thus reported. We reviewed our cases with respect to attack characteristics, aetiology, family history, and treatment response. Our population consisted of 23 men and 3 women. Seven patients had a family history of paroxysmal dyskinesia. None of our patients had clear evidence of symptomatic PKC. Two-thirds of our patients had attacks lasting between 30-60 s, and over one-half experienced one to ten attacks per day. Attack distribution varied widely, and most experienced pure dystonia rather than choreodystonic movements. Most patients responded very well to anticonvulsant therapy. We also report the PET results from two of our patients and Bereitschaftspotential abnormalities recorded from two others.

Paroxysmal kinesigenic choreoathetosis in Singapore and its relationship to epilepsy.

OBJECTIVES: To study the clinical characteristics of paroxysmal kinesigenic choreoathetosis (PKC) in our local population and its relationship to epilepsy. METHODS: We reviewed retrospectively 15 patients who were managed by neurologists in our department from 1982 to 1996. The literature was also reviewed to study the association between PKC and epilepsy. RESULTS: In our study, all the cases were idiopathic. The male to female ratio was 14:1 with all major races represented. Sixty percent of our patients suffered dystonic posturing rather than chorea, during the attacks. Twenty-one percent had a family history of a similar disorder which appeared to be of autosomal dominant inheritance. The sporadic form (79%) predominated in Singapore. One had a history of febrile fits while two had a history of epilepsy. We reviewed the available literature and found five other patients with idiopathic PKC also suffering from epilepsy. Of the 83 patients reviewed, 8% had epilepsy. This further strengthens the relationship between the two conditions. All our patients responded well to phenytoin at doses between 100 and 400 mg/day. CONCLUSION: PKC affected all three major races in our population with a high male to female ratio of 14:1. Seventy-nine percent of our cases were sporadic and 60% suffered dystonic posturing during attacks. Of the cases reviewed, 8% of patients with idiopathic PKC also had epilepsy.

Prevalence of chorea, dystonia and athetosis in Assiut, Egypt: a clinical and epidemiological study.

Involuntary movements originate from different parts of the nervous system. The character of movements depends upon the site of the lesion and the type of pathological change. The presence of more than one type of involuntary movement in a patient can cause confusion and difficulty in the proper classification of movement disorders, which then leads to problems in the differential diagnosis and appropriate treatment. This work was planned to estimate the prevalence and to study the aetiological factors of chorea, dystonia, athetosis and hemiballismus in Assiut, a representative community of Upper Egypt. This study was carried out on 7,000 families (42,000 subjects) representing different types of communities (2,000 families from urban, 2,000 families from suburban and 3,000 families from rural communities). All members of these samples were personally interviewed at home. Full clinical assessment and special investigations required for the diagnosis of different types of chorea, dystonia and athetosis were carried out in Assiut University Hospital. The prevalence rate for rheumatic chorea was 62/100,000 population and it was significantly higher (p < 0.01) among rural than urban and suburban populations, whereas Huntington's chorea had a prevalence rate of 21/100,000 with no significant difference between different areas studied (urban, suburban and rural). The other two common types of chorea were reported with prevalence rates of 12/100,000 for the encephalitic type and 17/100,000 for the atherosclerotic type. No single case of generalized dystonia was recorded and all cases were of the focal type of dystonia with a prevalence rate of 26/100,000 population. No significant differences were recorded between the different areas studied (urban, suburban and rural).(ABSTRACT TRUNCATED AT 250 WORDS)

Prospective data on the prevalence of abnormal involuntary movements among elderly people living in the community.

Trained raters from the Liverpool Continuing Health in the Community study interviewed 1070 people over the age of 65 in 1982-1983 using the Geriatric Mental State (GMS) examination. Three years later the cohort was re-interviewed, this time by psychiatrists trained in the GMS, who used the GMS and the History and Aetiology Schedule (HAS). The cohort had fallen in size to 875, because of mortality; 701 were re-interviewed. The cohort was followed up again 6 years after the first interview by trained nurse raters using the GMS (A3) and the Mini-Mental State Examination. Cases of mental disorder identified by the computer diagnostic program AGECAT were re-interviewed by psychiatrists along with a number of controls using the GMS and the HAS on the remaining 450 individuals. Observational behavioural ratings from the GMS and summary sheets were analysed along with AGECAT diagnoses and data on medication gained at the 3 assessments. The community prevalence of tardive dyskinesia and other movement disorders in elderly people over a 6-year follow-up appears to be very low (the community prevalence of tardive dyskinesia being 0.22% and akathisia 1.57%), is usually associated with organic mental disorder (and consequently higher mortality) and is furthermore not usually associated with antipsychotic medication.

Factors associated with choreoathetosis after cardiopulmonary bypass in children with congenital heart disease.

BACKGROUND: Choreoathetosis (CHO) after congenital heart surgery has been described since 1960. Risk factors and patient outcome have not been well defined. METHODS AND RESULTS: As our complexity of cases increased and management of pH on cardiopulmonary bypass (CPB) evolved, we noted the appearance of CHO among patients beginning in 1986. We reviewed the hospital course and follow-up of all 19 affected children, including eight younger patients (median age, 4.3 months) who developed a mild transient form of CHO, all of whom survived and had complete resolution of CHO; seven of these eight patients had deep hypothermic circulatory arrest (DHCA). Eleven older patients (median age, 16.8 months) developed severe persistent CHO; 11 had DHCA, 10 were cyanotic, seven had anatomic pulmonary atresia, and three others were physiologically analogous with a systemic to pulmonary artery shunt-dependent circulation. Five of six patients who had pertinent preoperative angiography had systemic to pulmonary collateral vessels arising from the head and neck arteries. Mortality in severe patients was 36% (four of 11); the seven survivors showed improvement, but only one had a normal neurological examination after 60 months of follow-up. When severe CHO patients were compared with 17 age- and diagnosis-matched patients without neurological complications, no differences were found in CPB or DHCA times, arterial blood gases, or hematocrits. Time from onset of CPB to onset of DHCA (time to shutoff) was shorter in the severe persistent CHO group than for comparison patients (22 +/- 7 versus 40 +/- 29 minutes, p = 0.053). CONCLUSIONS: Factors that may be associated with the development of severe persistent CHO include 1) age beyond early infancy, 2) cyanotic heart disease with systemic to pulmonary collaterals, particularly those arising from the head and neck vessels, and 3) the duration of the cooling period used in conjunction with deep hypothermic circulatory arrest. We advocate earlier reparative surgery, precise preoperative diagnosis and preoperative or intraoperative control of systemic to pulmonary artery collaterals, and further study of pH effects during CPB on development of CHO.

Choreoathetosis after deep hypothermia without circulatory arrest.

In 8 of 758 patients undergoing an intracardiac operation under cardiopulmonary bypass and hypothermia, choreoathetosis developed 3 to 7 days postoperatively. Before the onset of choreoathetosis, varying degrees of neurological dysfunction were noted. Electroencephalography and neuroimaging failed to detect any responsible functional or structural changes. Six patients are alive 1 to 3 years postoperatively, and their condition is improving. Two patients died of aspiration or sepsis. All patients were grouped based on factors identified as being possibly causative: depth of hypothermia, cooling time, flow rate, and repeated hypothermia. The incidence of choreoathetosis was significantly different in group A (rectal temperature greater than 25 degrees C) compared with group B (rectal temperature less than or equal to 25 degrees C) (0/295 versus 8/463; p = 0.02). Based on cooling time, the incidence of choreoathetosis was significantly different in group B1 (cooling time less than 1 hour) compared with group B2 (cooling time greater than or equal to 1 hour) (1/220 versus 7/243; p = 0.05). Based on flow rate during cooling, group B2 was further divided into the low-flow group (less than 1,500 mL.min-1.m-2) and the high-flow group (greater than or equal to 1,500 mL.min-1.m-2). Although not significant, the incidence of choreoathetosis was higher in the high-flow group (6/153 versus 1/90; p = 0.22). In group B patients having reoperation, the incidence of choreoathetosis was higher than in patients operated on for the first time (5/54 versus 3/409; p less than or equal to 0.0001).(ABSTRACT TRUNCATED AT 250 WORDS)

The changing panorama of cerebral palsy in Sweden 1954-1970. II. Analysis of the various syndromes.

From an unselected series of 560 Swedish cases of cerebral palsy, born 1954-1970, various data of etiologic and pathogenetic interest were analyzed in detail. Untraceable and prenatal factors were found to dominate within the group of spastic hemiplegia. Placental dysfunction in small-for-date babies and severe asphyxia were thought to be the two main pathogenetic factors among the patients with spastic tetraplegia. In spite of a significant decrease in the number of low birth weight children within the group of spastic diplegia, this syndrome was still very characteristic for the child born immature. Ataxic diplegic forms were found to have greater pathogenic similarities to spastic diplegia than to simple ataxia. In two-thirds of the children the latter syndrome was characterized by normal pregnancy, delivery and birth weight and an untraceable (genetic?) factor. Dyskinetic syndromes were mostly encountered after perinatal asphyxia.