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PubMed2003

Lower esophagus in dyspeptic Iranian patients: a prospective study.

BACKGROUND: Gastroesophageal junction cancer has increased over time in Western countries. Gastroesophageal reflux disease (GERD) is considered to be a major risk factor. We prospectively studied the prevalence of clinical, histological and endoscopic GERD, and premalignant changes among dyspeptic Iranian patients referred for upper gastrointestinal endoscopy (UGIE). METHODS: Consenting patients referred for UGIE to our clinic were enrolled. Their symptoms were recorded, UGIE was conducted, and biopsies from all suspicious lesions and across the Z-line were taken. RESULTS: Of the 344 enrolled patients, 269 (135 women, 134 men; mean age: 41.6 years) were evaluated. One major GERD symptom (heart burn, acid regurgitation, dysphagia and chest pain) was seen in 209 (77.6%) patients, and 207 patients (76.1%) had endoscopic esophagitis. Thirteen patients (5%) had specialized intestinal metaplasia at the gastrointestinal junction (SIM-GEJ), and three had glandular dysplasia (two low-grade, one high-grade). No symptom could predict the presence of histological or endoscopic findings. Patients with dysplasia had more advanced degrees of endoscopic esophagitis. CONCLUSION: Gastroesophageal reflux disease is common among Iranian patients referred for diagnostic endoscopy. The prevalence of SIM-GEJ among this population was comparable to that reported in Western countries.

Adolescent↗

PubMed2000

Speech timing in Thai left- and right-hemisphere-damaged individuals.

An acoustic analysis of syllable duration in short Thai phrases was conducted to evaluate the effects of focal brain damage on the control of speech timing. Almost all 35 of the subjects had participated in each of four previous companion studies: 13 left-brain-damaged (6 nonfluent aphasics; 7 fluent aphasics), 14 right-brain-damaged patients, and 8 normal controls. Somewhat surprisingly, results revealed relatively normal timing patterns in 3-syllable phrases in all subject groups. A comparison of the current study and the four others, however, led us to conclude that Thai-speaking nonfluent aphasics exhibit a speech timing deficit regardless of the linguistic level of representation, whereas timing deficits in fluent aphasics appear to be restricted to units larger than a syllable. Speech timing, on the other hand, appears to be intact across the board in right-brain-damaged individuals. Findings are brought to bear on theories of temporal control in brain-damaged patients.

Acoustics↗

PubMedDate not supplied

Direct and indirect therapy for word sound deafness.

This paper evaluates two therapy programmes conducted with PK, an aphasic individual with word sound deafness. The first aimed directly to improve discrimination skills, using minimal pair tasks supported with lip reading. Disappointingly, there were no changes on discrimination tests after this therapy, even when PK could benefit from lip reading information. The second, indirect, programme of therapy aimed to change the communication behaviours of PK's wife. Strategies such as writing and simplifying information were modelled and practiced. A detailed information booklet, outlining the target strategies and explaining PK's main strengths and weaknesses supported the programme. Evaluation of this therapy involved pre- and post-therapy analysis of interactions between PK and his wife, with biographical questions forming the basis of the interactions. There were several changes after therapy on this measure. The number and length of communication breakdowns were reduced, and more questions were answered accurately. This study suggests that, in some cases of severe word sound deafness, indirect therapies may be most effective.

Aged↗

PubMed1987

Hearing loss among stroke patients.

Pure-tone hearing thresholds were measured by air conduction for 140 right hemiplegic and 103 left hemiplegic patients who had suffered a single stroke. Statistical and informal testing of the mean audiometric thresholds revealed that: (1) the ability of the aphasic patient to attend to and to perform the audiometric test was independent of the severity of the aphasic impairment; (2) aphasic patients did not suffer disproportionately greater hearing losses than did other right hemiplegic stroke patients; (3) no relation was apparent between the severity of the hearing loss and the test ear or side of hemiplegia after stroke; (4) the severity of high-frequency hearing loss among stroke patients was consistent with that found in the elderly male population at Framingham, MA (Moscicki, Elkins, Baum, and McNamara, Ear Hear 1985;6:184-90); and (5) the prevalence of hearing loss among the stroke patients was greater than that reported at Framingham, but was consistent with rates found among elderly nursing home residents.

Adult↗

PubMed1990

Temporal dimensions of consonant and vowel production: an acoustic and CT scan analysis of aphasic speech.

This study explored a number of temporal (durational) parameters of consonant and vowel production in order to determine whether the speech production impairments of aphasics are the result of the same or different underlying mechanisms and in particular whether they implicate deficits that are primarily phonetic or phonological in nature. Detailed analyses of CT scan lesion data were also conducted to explore whether more specific neuroanatomical correlations could be made with speech production deficits. A series of acoustic analyses were conducted including voice-onset time, intrinsic and contrastive fricative duration, and intrinsic and contrastive vowel duration as produced by Broca's aphasics with anterior lesions (A patients), nonfluent aphasics with anterior and posterior lesions (AP patients), and fluent aphasics with posterior lesions (P patients). The constellation of impairments for the anterior aphasics including both the A and AP patients suggests that their disorder primarily reflects an inability to implement particular types of articulatory gestures or articulatory parameters rather than an inability to implement particular phonetic features. They display impairments in the implementation of laryngeal gestures for both consonant and vowel production. These patterns seem to relate to particular anatomical sites involving Broca's area, the anterior limb of the internal capsule, and the lowest motor cortex areas for larynx and tongue. The posterior patients also show evidence of subtle phonetic impairments suggesting that the neural instantiation of speech may require more extensive involvement, including the perisylvian area, than previously suggested.

Aged↗

PubMed1986

On fluency measures in aphasic speech.

This study was conducted with two purposes. First, to examine the extent to which fluency measures are affected by conditions of speech production. The speech of an unselected sample of 18 aphasic subjects was collected in two situations: conversation and description of short picture stories. Words were uttered at a reduced rate in the story descriptions, which were also characterized by a greater use of verbs. However, the ranking of the subjects on the different measures of fluency was correlated in the two situations. The second aim was to analyze the relationship between different fluency measures: mean silence duration, speech tempo, subjective evaluation of fluency, and the use of grammatical morphemes. Rankings of the subjects on these measures were compared. The different indicators of fluency were positively correlated with each other. Moreover, clinical evaluations of fluency by Belgian and American clinicians were correlated with the objective measures. However, fluency measures did not correlate with the percentage of content words. Thus, reduction of fluency did not seem to be related to a selective loss of function words.

Adolescent↗

PubMed1993

The influence of depression, social activity, and family stress on functional outcome after stroke.

BACKGROUND AND PURPOSE: This study was designed to assess the quality of life after an active poststroke period of rehabilitation and to investigate the possibility of a return to a working environment for those still of working age. METHODS: The study was conducted on 180 consecutive patients affected by stroke who were hospitalized for the first time and discharged at least 1 year before the study. The group consisted of 65% men and 35% women with a mean age 65.29 years (SD, 11.22). The period between the stroke and the interview ranged from 12 to 196 months, with a mean of 37.5 months. The average Rankin score on discharge from the rehabilitation center was 2.718 (moderate handicap). The interview took place at home after consent obtained by telephone. The questionnaire included general and personal information regarding the individuals, their socioeconomic position, and scales for daily activity, depression, social activity, and stress produced in the family. The control group consisted of 167 age-matched subjects. RESULTS: A close correlation was observed in all patients between depression, social activity, and stress caused to relatives. The scores on the individual scales were clearly worse than those for control subjects. The patients received approximately 5 months of rehabilitation after the stroke. Differences emerged between men and women for depression and social activities, with the women scoring worse. In reference to daily life, 70% of prestroke ability was required on average after rehabilitation. The daily activity score at the time of the interview was also strongly influenced by the discharge score. The majority of patients were retired. Of the total, 20.64% returned to work, but not always to the same job and often after readapting to new conditions. Of this population, only 31.5% were women. With regard to the population aged younger than 65 years, 21.42% returned to work. Lesions in the dominant hemisphere do not necessarily seem to rule out return to work, even if associated with aphasia. The main discriminating element was the ability to understand language. The patients were often criticized by their cohabitants; the criticisms most often raised concerned apathy, irritability, and self-centeredness. Sexual activity was depressed in almost all cases. CONCLUSIONS: Despite the progress made in studying cerebral vasculopathies, patients in the aftermath of a stroke still seem to live unsatisfactorily, as they did many years ago. Useful measures include valid treatment against spasticity, psychological assistance, and greater social support.

Activities of Daily Living↗

PubMed2004

Validation of the aphasic depression rating scale.

BACKGROUND AND PURPOSE: The Aphasic Depression Rating Scale (ADRS) was developed to detect and measure depression in aphasic patients during the subacute stage of stroke. METHODS: Six experts selected an initial sampling of behavioral items from existing depression rating scales. Stroke patients (aphasic and nonaphasic) were assessed with these items by the rehabilitation staff, with the Hamilton Depression Rating Scale (HDRS) for nonaphasic patients only, by a psychiatrist, and by the rehabilitation staff with Visual Analog Scales (VAS). A second item selection was conducted after a regression algorithm was run including VAS as independent variables (criterion validity) and after their factorial structure was analyzed with a principal component analysis (factorial validity). The construct validity was evaluated with respect to the other depression assessments. A threshold for the diagnosis of depression was computed with respect to the psychiatrist's diagnosis. Interrater and test-retest reliability were assessed in 2 additional groups of aphasic patients. RESULTS: Eighty patients participated in the study (59 aphasic). Fifteen behavioral items from existing depression rating scales were selected, and 9 were retained after the validation process. ADRS correlated highly with VAS and HDRS (r=0.60 to 0.78, P=10(-4) to 10(-6)). With respect to the psychiatrist's diagnosis, the sensitivity and specificity of ADRS were 0.83 and 0.71, respectively, when the threshold was set at 9/32. Its factorial structure was comparable to HDRS structure. Interrater and test-retest reliability were high (average kappa coefficient of the 9 items=0.69). CONCLUSIONS: ADRS is a valid, reliable, sensitive, and specific tool for the evaluation of depression in aphasic patients during the stroke subacute phase.

Adult↗

PubMed1996

Prevalence of stroke in Kinmen.

BACKGROUND AND PURPOSE: Stroke is the second most common cause of death in Taiwan. We studied its prevalence, risk factors, and mortality in a rural Chinese population. METHODS: A door-to-door survey of stroke was conducted in two Kinmen Island townships with a total population of 26105 people. Our target population (n = 5061) consisted of all the registered residents in these townships who were aged > or = 50 years on August 1, 1993. All participants were given a standardized neurological examination and a questionnaire. RESULTS: The participation rate was 77.4% (n = 3915). Ninety-six cases of completed stroke were identified. Eighty-nine patients had one, 6 patients had two, and 1 patient had three episodes of stroke. The prevalence of stroke in persons aged > or = 50 years was 24.5 per 1000 (95% confidence interval, 19.7 to 29.3 per 1000). Prevalence increased with age. Statistically significant risk factors associated with stroke included hypertension, diabetes mellitus, and irregular heartbeats. Of the stroke survivors, 59% were independent in activities of daily living and 71% could walk independently. CONCLUSIONS: Compared with other countries, Taiwan has a moderately high prevalence of stroke. The risk factors for stroke in this rural region of a developing country are similar to those in developed countries. Most stroke survivors here perform their daily activities independently without outside assistance.

Activities of Daily Living↗

PubMed1992

Thematic analysis in agrammatic comprehension: syntactic structures and task demands.

Aphasics' ability to assign thematic roles during sentence comprehension was investigated. The study was motivated by the hypothesis that capacity limitations, e.g., due to slowed processing operations, interact systematically with certain aspects of structural analysis more than with others. Experiments using a sentence-picture matching paradigm were conducted with several agrammatic Broca's and paragrammatic Wernicke's patients. Experiment 1 tested comprehension of various syntatic structures. The astoundingly good performance in Experiment 1 for both patient groups was attributed to the low task demands of the experiment. Raising the task demands, by delaying presentation of the relevant picture set in Experiment 2, resulted in a significant decrement of comprehension performance for agrammatic Broca's aphasics, but not for Wernicke's aphasics. Putting the main verb in the sentence-final position, as in Experiment 3, affected Wernicke's aphasics', but not Broca's aphasics' performance. In principle, one explanation for the performance differences between Experiments 1 and 2 for Broca's patient might be a deficit in these patients' verbal memory capacity. Therefore, verbal memory span on different word categories was tested in an additional experiment. No difference, however, was found between the Broca's patients' and the Wernicke's patients' capacity for verbal memory despite their different sentence comprehension profiles. The present data are taken to support the view that Broca's aphasics' comprehension behavior is due to the processing demands imposed by structural inference chains and not to a general reduction in verbal memory.

Adult↗

PubMedDate not supplied

Psychometric testing of the revised 15-item Bakas Caregiving Outcomes Scale.

BACKGROUND: Family caregivers of stroke survivors experience a variety of negative social, emotional, and health-related outcomes as a result of providing care. OBJECTIVES: : To psychometrically test the revised 15-item Bakas Caregiving Outcomes Scale (BCOS) measuring life changes specifically resulting from providing care. The original 10-item BCOS was improved by adding five items addressing financial well-being, level of energy, role functioning, physical functioning, and general health. METHODS: Psychometric testing of the revised 15-item BCOS using a sample of 147 family caregivers of stroke survivors approximately 4 months after stroke was conducted to determine the quality of the items, internal consistency reliability, test-retest reliability, construct validity, and criterion-related validity. Most caregivers were women (78.9%), White (68.0%) or African American (29.9%), and either spouses (60.1%) or adult children (31.3%). RESULTS: Satisfactory evidence of internal consistency (alpha = .90) and 2-week test-retest reliability (intraclass coefficient [ICC] = .66; 95% confidence interval [CI] = 0.42-0.81) was provided, with item-to-total correlations ranging from .41 to .74. Unidimensionality was supported by confirmatory factor analysis with indices, indicating a good fit. Using hierarchical multiple regression, 36% of the BCOS variance was explained by constructs in the conceptual model [F(11,132) = 6.72, p < .001]. Criterion-related validity was supported by correlations with the 36-item Short Form (SF-36) General Health Subscale (r = .32, p < .001) and a criterion variable measuring how caregivers' lives had changed overall (r = .67, p < .001). DISCUSSION: The revised 15-item BCOS has evidence of satisfactory reliability and validity in family caregivers of stroke survivors. The BCOS is a valuable measure in research and can be used to identify priority areas for nursing interventions designed to improve caregivers' outcomes.

Adaptation, Psychological↗

PubMed1990

[Does circumscribed ischemic white matter lesion produce neuropsychological symptoms?].

In order to determine a possible role of ischemic white matter lesion in producing neuropsychological symptoms, we conducted a retrospective survey. Subjects were 206 consecutive patients who had been admitted to our neurology service under the diagnosis of ischemic stroke from April 1988 to November 1989 and had received brain CT as well as SPECT scans. From this cohort only patients that had circumscribed white matter lesion on CT scans were selected. Patients who had lesion in basal ganglia or thalamus were rigidly excluded. Eight subjects (3.9%) fulfilled above criteria, i.e. 5 with left hemispheric, 2 with right hemispheric and 1 with bilateral white matter lesions. Then we supplemented MRI information which had happened to be available for all the eight subjects. Exclusion of grey matter lesion based on MRI reduced the number of cases with exclusive white matter lesion into 4, i.e. 3 with left hemispheric and 1 with right hemispheric lesion. Of these, only one case with a discrete lesion in the genu of the left internal capsule had manifested mild neuropsychological impairments in naming, comprehension and writing. I123 amphetamine SPECT study revealed low uptake in the anterior half of the left middle cerebral artery in this case. We conclude that the role of a circumscribed white matter lesion as a cause of neuropsychological symptom seems to be much less clear than has been postulated.

Adult↗

PubMed2005

Contextual priming in semantic anomia: a case study.

The present case continues the series of anomia treatment studies with contextual priming (CP), being the second in-depth treatment study conducted for an individual suffering from semantically based anomia. Our aim was to acquire further evidence of the facilitation and interference effects of the CP treatment on semantic anomia. Based on the results of the study of , our hypothesis before the treatment was that our participant would show short-term interference and at most modest and short-term benefit from treatment. To acquire such evidence would not only be important for the choice of anomia treatment methods in individual patients, but would also prompt further development of the CP method. The CP technique used for our participant included cycles of repeating and naming items in three contextual conditions (semantic, phonological, and unrelated). As predicted, the overall improvement of naming was modest and short-term. Interestingly, the contextual condition that corresponded with the nature of our patient's underlying naming deficit (semantic) elicited immediate interference in the form of contextual naming errors, as well as short-term improvement of naming. Based on this and a recent study by , it appears that despite short-term positive effects, in its current form the CP treatment is not sufficient for those aphasics who have a semantic deficit underlying their anomia. The possible mechanism and directions for future research are discussed.

Aged↗

PubMed1998

Survival prolongation in HIV-associated progressive multifocal leukoencephalopathy treated with alpha-interferon: an observational study.

UNLABELLED: A retrospective chart review was conducted to determine the effect of alpha-Interferon (alpha-IFN) on disease progression, symptom palliation, and survival in HIV-associated Progressive Multifocal Leukoencephalopathy (PML). METHODS: Subjects were HIV seropositive patients diagnosed with PML at the Johns Hopkins Hospital between 1985 and July of 1986. Diagnostic criteria for PML included both clinical symptomatology and histologic or radiographic confirmation. All patients with concomitant CNS infections were excluded. Patients receiving a minimum treatment of 3 weeks of 3 million units of alpha-IFN daily were compared to untreated historical controls. From 104 PML cases reviewed, 77 met the defined criteria for PML. Twenty-one patients had received open-label alpha-IFN treatment in a non-randomized manner for at least 3 weeks, and 32 met criteria for inclusion in the untreated group as historical controls. Deceased treated patients were comparable to deceased untreated patients with respect to age, gender, race, HIV risk factors, AIDS-defining illnesses, and CD4+ counts. CD4+ counts and use of anti-retroviral medications within 6 months of PML onset were higher among those who were living at the time of the study. RESULTS: Among deceased patients, median survival of treated patients was 127.5 days longer than that of untreated patients (Chi-square=4.21, P=0.04). When living and deceased treated patients were combined, the median survival was 325 days (range 35 - 1634) versus 121 days (range 46 - 176) in untreated patients (Chi-square=13.47, P < 0.001). When survival times in untreated patients were left-censored to account for possible survivorship bias in treated patients, survival in treated patients remained significantly prolonged (325 days versus 175.5 days, Chi-square=4.65, P=0.03). In addition, use of alpha-IFN was associated with a significant delay in the onset of memory loss (Chi-square=8.59, P < 0.01). Seven alpha-IFN treated patients showed sustained remissions of several months to over a year, with documented improvements in mental status, aphasia, dysarthria, dysphagia, paresis, and dyscoordination. Moreover, four IFN-treated patients had evidence of MRI lesion regression, although this was not always correlated with clinical remission. Four of 32 untreated patients also reported transient symptomatic improvements. CONCLUSION: This open-label study suggests that alpha-IFN may delay progression, palliate symptoms, and significantly prolong survival in HIV-associated PML, and we therefore suggest that a controlled clinical trial is warranted.

AIDS-Related Opportunistic Infections↗

PubMed1983

Identification of tonal contrasts in Thai aphasic patients.

In tone languages pitch variations (tones) serve to distinguish the lexical meanings of words. This study was conducted to examine the extent and nature of impairment in the perception of tones by aphasic patients who were monolingual speakers of Thai, a tone language which has five contrastive tones (mid, low, falling, high, rising). Six subjects participated in the study: two Broca aphasics, one transcortical motor aphasic, one conduction aphasic, one right brain-damaged nonaphasic, and one normal control. Three sets of stimuli (two real-speech, one synthetic-speech) were presented for identification, each set containing five Thai words minimally distinguished by tone. Results of the perception tests indicated that the performance of all four left brain-damaged aphasics differed significantly from that of the normal control, while the performance of the right brain-damaged nonaphasic did not. The normal performance of the right brain-damaged nonaphasic patient on this tone identification task suggests that deficits in the perception of tone exhibited by left brain-damaged patients can be attributed specifically to pathology in the language dominant hemisphere rather than to a general brain-damage effect. No difference in performance among the left brain-damaged patients could be attributed to a specific type of aphasic syndrome. The pattern of tonal confusions of the aphasics in comparison to that of normals suggests that their deficit is primarily quantitative rather than qualitative. Although two (mid, low) of the five tones accounted for a large percentage of the aphasics' errors, no uniform rank order of tones in terms of identifiability could be established across aphasic subjects, which suggests that their deficit is general to all five tones rather than selective to individual tones.

Adult↗

PubMed1988

The right cerebral hemisphere: emotion, music, visual-spatial skills, body-image, dreams, and awareness.

Based on a review of numerous studies conducted on normal, neurosurgical and brain-injured individuals, the right cerebral hemisphere appears to be dominant in the perception and identification of environmental and nonverbal sounds; the analysis of geometric and visual space (e.g., depth perception, visual closure); somesthesis, stereognosis, the maintenance of the body image; the production of dreams during REM sleep; the perception of most aspects of musical stimuli; and the comprehension and expression of prosodic, melodic, visual, facial, and verbal emotion. When the right hemisphere is damaged a variety of cognitive abnormalities may result, including hemi-inattention and neglect, prosopagnosia, constructional apraxia, visual-perceptual disturbances, and agnosia for environmental, musical, and emotional sounds. Similarly, a myriad of affective abnormalities may occur, including indifference, depression, hysteria, gross social-emotional disinhibition, florid manic excitement, childishness, euphoria, impulsivity, and abnormal sexual behavior. Patients may become delusional, engage in the production of bizzare confabulations and experience a host of somatic disturbances such as pain and body-perceptual distortions. Based on studies of normal and "split-brain" functioning, it also appears that the right hemisphere maintains a highly developed social-emotional mental system and can independently perceive, recall and act on certain memories and experiences without the aid or active reflective participation of the left. This leads to situations in which the right and left halves of the brain sometime act in an uncooperative fashion, which gives rise to inter-manual and intra-psychic conflicts.

Aphasia↗

PubMed2003

Dose-volume prediction of radiation-related complications after proton beam radiosurgery for cerebral arteriovenous malformations.

OBJECT: The use of radiosurgery for the treatment of cerebral arteriovenous malformations (AVMs) and other lesions demands an accurate understanding of the risk of radiation-related complications. Some commonly used formulas for predicting risk are based on extrapolation from small numbers of animal experiments, pilot human treatment series, and theoretical radiobiological considerations. The authors studied the incidence of complications after AVM radiosurgery in relation to dose, volume, and other factors in a large patient series. METHODS: A retrospective review was conducted in 1329 patients with AVM treated by Dr. Raymond Kjellberg at the Harvard Cyclotron Laboratory (HCL) between 1965 and 1993. Dose and volume were obtained from HCL records, and information about patient follow up was derived from concurrent clinical records, questionnaires, and contact with referring physicians. Multivariate logistic regression with bootstrapped confidence intervals was used. Follow up was available in 1250 patients (94%); the median follow-up duration was 6.5 years. The median radiation dose was 10.5 Gy and the median treatment volume was 33.7 cm(3). Twenty-three percent of treated lesions were smaller than 10 cm(3). Fifty-one permanent radiation-related deficits occurred (4.1%). Of 1043 patients treated with a dose predicted by the Kjellberg isoeffective centile curve to have a less than 1% complication risk, 1.8% suffered radiation-related complications. Actual complication rates were 4.7% for 128 patients treated at Kjellberg risk centile doses of 1 to 1.8%, and 34% for 61 patients treated at risk centile doses of 2 to 2.5%. The fitted logistic model showed that complication risk was related to treatment dose and volume, thalamic or brainstem location, and patient age. CONCLUSIONS: The Kjellberg isoeffective risk centile curve significantly underpredicted actual risks of permanent complications after proton beam radiosurgery for AVMs. Actual risks were best predicted using a model that accounted for treatment dose and volume, lesion location, and patient age.

Adolescent↗

PubMed2006

Sydenham's chorea in western Pennsylvania.

OBJECTIVE: Chorea is characterized by involuntary, fleeting, irregular, nonrhythmic movements that flow from 1 body region to another. There are many causes of childhood chorea, including cerebrovascular accidents, collagen vascular diseases, drug intoxication, hyperthyroidism, Wilson's disease, Huntington's disease, and infectious agents. Although Sydenham's chorea (SC), a nonsuppurative sequela of group A streptococcal infection, is known to be a common cause of chorea, multiple laboratory and radiographic studies are often obtained to determine the cause of pediatric chorea. We conducted a retrospective chart review to determine the causes of childhood chorea seen in a large children's hospital in an area endemic for acute rheumatic fever (ARF). The utility of neuroimaging in establishing a final diagnosis of SC is discussed. METHODS: Patients who received a diagnosis of chorea between 1980 and 2004 at the Children's Hospital of Pittsburgh were identified from databases that are maintained by the divisions of Infectious Diseases and Cardiology and from the hospital's medical records department. Charts were abstracted retrospectively. All patients who had new-onset chorea and did not have any underlying neurologic disorders were included in this study. Patient demographic, clinical, laboratory, and imaging information was analyzed. Follow-up information was not found consistently and therefore was not included. Charts of patients with questionable diagnoses were reviewed with a neurologist. RESULTS: A total of 144 patients met the search criterion. Eleven patients had incomplete charts, and 6 charts could not be located. Thirty patients were excluded because they had preexisting neurologic diagnoses, eg, cerebral palsy. Fifteen patients were excluded because they were miscoded as having chorea. Eighty-two patients had new-onset chorea. The cause was SC (n = 79), postoperative cerebral ischemia (n = 1), and basal ganglion infarct (n = 2). Seventy-six (71%) children with SC were female. The mean age of presentation was 9.8 years (range: 5-14.5 years). Chorea was unilateral in 23 (30%) patients. Family history of ARF existed in 30% of patients. Neurologic symptoms of SC included behavior change (46%), dysarthria (67%), gait change (51%), deterioration of handwriting (29%), and headache (11%). Nonneurologic manifestations of ARF were carditis (44%), arthritis (11%), erythema marginatum (3%), and subcutaneous nodules (0%). Antecedent group A streptococcal infection was documented in 99% of patients who were tested by an elevated antistreptolysin O titer (n = 53), an elevated anti-deoxyribonuclease B titer (n = 7), a positive streptozyme (n = 53), or acute throat infection with Streptococcus pyogenes (n = 19). A total of 52 neuroimaging tests were obtained from 46 patients with SC. In patients with SC, brain MRI was abnormal in 8 of 32 patients, and brain computed tomography was abnormal in 1 of 20 patients. Abnormalities did not aid in diagnosis and included nonspecific increased signal (n = 2), nonspecific punctate lesions (n = 2), asymmetry of the hippocampal fissures, unrelated petrous bone anomaly, Arnold Chiari malformation, and medulloblastoma in a macrocephalic patient. Three patients with chorea that was not attributed to ARF had atypical presentations: 1 developed chorea after a perioperative hypoxic/ischemic central nervous system insult; 1 had an episode of disorientation, aphasia, and transient facial droop (angiography showed basal ganglia infarct); and 1 with hemichorea had basal ganglion infarct seen on MRI. CONCLUSIONS: Ninety-six percent of children who had acute chorea and presented to a large children's hospital in an area that is endemic for ARF had SC. These patients had characteristic demographic and clinical features of SC. The most common concurrent major Jones criterion was carditis. Arthritis, erythema marginatum, and subcutaneous nodules were uncommon in this population. Neuroimaging was obtained in 58% of patients with SC and did not aid in any of their diagnoses. The 3 patients with chorea that was not caused by SC had histories that were atypical for SC and warranted neuroimaging. SC can be readily diagnosed on the basis of history, physical examination, and laboratory evaluation; neuroimaging is not necessary and should be reserved for patients who have an atypical presentation, including hemichorea.

Acute Disease↗