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Biomedical subjects

R Goodman

Publications and source records attributed to R Goodman.

At least 73 records · Page 4Linked to original sources

Spinal myoclonus induced by an intrathecal catheter.

We report a case of spinal myoclonus induced by the tip of an intrathecal catheter in a 35-year-old patient with severe, adult-onset, generalized dystonia of unknown cause, treated for 2 years using intrathecal baclofen. One month after a falling episode, the patient developed focal myoclonus of the right proximal leg whenever she stood up from a seated position. The electrophysiologic recordings were compatible with spinal segmental myoclonus, originating at a focus corresponding to the L2-S2 segments. At this site, the tip of the intrathecal catheter was demonstrated by myelography to be in close proximity to the nerve roots and conus medullaris. The myoclonus resolved promptly once the catheter tip was withdrawn. We review the literature on spinal myoclonus and discuss the possible mechanisms of spinal myoclonus pertaining to the present case. This report represents an unusual complication of intrathecal catheter systems that, if recognized, can lead to prompt therapeutic intervention.

Adult↗

Do unilateral lesions of the developing brain have side-specific psychiatric consequences in childhood?

This study used a large epidemiological sample of children with lateralised brain lesions to establish whether damage to the developing human brain has side-specific psychiatric consequences. Parents and teachers completed behaviour questionnaires on 429 hemiplegic children and teenagers, with a subsample of 149 hemiplegic children also being assessed by parent and child interviews. Although childhood hemiplegia was accompanied by a high rate of psychopathology, children with right- and left-sided hemiplegias did not differ significantly on any dimensional or categorical measure of psychopathology. This absence of laterality effects, perhaps reflecting the developing brain's neuroplasticity, casts doubt on theories linking particular types of child or adult psychopathology to side-specific damage to the developing brain.

Journal Article↗

Multicenter typing comparison of sporadic and outbreak Clostridium difficile isolates from geographically diverse hospitals.

In a collaborative study by three laboratories, arbitrarily primed polymerase chain reaction (AP-PCR), HindIII restriction enzyme analysis (REA), and pulsed-field gel electrophoresis (PFGE) using SmaI were compared for typing of Clostridium difficile. The study included 30 isolates from nosocomial outbreaks in six geographically disparate hospitals and 15 isolates from sporadic cases of C. difficile diarrhea. REA distinguished a total of 23 types representing 10 groups; AP-PCR performed at Deaconess Hospital resolved 19 types; AP-PCR performed at the Centers for Disease Control resolved 15 types. Thirty isolates exhibited degradation of larger sized fragments during processing and therefore were nontypeable by PFGE; among the remaining 15 isolates, PFGE resolved 11 types. Outbreak isolates in five different hospitals represented REA group J and constituted a single AP-PCR strain. In summary, nosocomial outbreaks of C. difficile diarrhea in five hospitals were associated with a single genetic lineage as resolved by multiple strain typing systems.

Bacterial Typing Techniques↗

The Strengths and Difficulties Questionnaire: a research note.

A novel behavioural screening questionnaire, the Strengths and Difficulties Questionnaire (SDQ), was administered along with Rutter questionnaires to parents and teachers of 403 children drawn from dental and psychiatric clinics. Scores derived from the SDQ and Rutter questionnaires were highly correlated; parent-teacher correlations for the two sets of measures were comparable or favoured the SDQ. The two sets of measures did not differ in their ability to discriminate between psychiatric and dental clinic attenders. These preliminary findings suggest that the SDQ functions as well as the Rutter questionnaires while offering the following additional advantages: a focus on strengths as well as difficulties; better coverage of inattention, peer relationships, and prosocial behaviour; a shorter format; and a single form suitable for both parents and teachers, perhaps thereby increasing parent-teacher correlations.

Adolescent↗

Multiple myeloma. Diagnostic clues in a patient presenting with incapacitating arthralgias.

Multiple myeloma is characterized by the production of a monoclonal immunoglobulin, free monoclonal light chains, or both. Although bone pain is the classic presentation, multiple myeloma should be a consideration in differential diagnosis in elderly patients with arthritic complaints if other typical symptoms or laboratory abnormalities, such as anemia, hypercalcemia, and elevated ESR, are present. A combination of radiation therapy and chemotherapy is the usual treatment.

Arthralgia↗

Psychiatric problems in children with hemiplegia: cross sectional epidemiological survey.

OBJECTIVE: To examine the prevalence and predictors of psychiatric problems in children with hemiplegia. DESIGN: Cross sectional questionnaire survey of an epidemiological sample with individual assessments of a representative subgroup. The questionnaire survey was repeated on school age subjects four years later. SUBJECTS: 428 hemiplegic children age 2 1/2-16 years, of whom 149 (aged 6-10 years) were individually assessed. MAIN OUTCOME MEASURES: Psychiatric symptom scores and the occurrence of psychiatric disorder. RESULTS: Psychiatric disorders affected 61% (95% confidence interval 53% to 69%) of subjects as judged by individual assessments and 54% (49% to 59%) and 42% (37% to 47%) as judged from parent and teacher questionnaires, respectively. Few affected children had been in contact with child mental health services. The strongest consistent predictor of psychiatric problems was intelligence quotient (IQ), which was highly correlated with an index of neurological severity; age, sex, and laterality of lesion had little or no predictive power. CONCLUSION: Though most hemiplegic children have considerable emotional or behavioural difficulties, these psychological complications commonly go unrecognised or untreated. Comprehensive health provision for children with chronic neurodevelopmental disorders such as hemiplegia should be psychologically as well as physically oriented.

Child↗

Urokinase is required for the pulmonary inflammatory response to Cryptococcus neoformans. A murine transgenic model.

Urokinase (uPA) is hypothesized to provide proteolytic activity enabling inflammatory cells to traverse tissues during recruitment, and it is implicated as a cytokine modulator. Definitive evaluation of these hypotheses in vivo has previously been impossible because uPA could not completely and irreversibly be eliminated. This limitation has been overcome through the development of uPA-deficient transgenic mice (uPA-/-). Using these mice, we evaluated the importance of uPA in the pulmonary inflammatory response to Cryptococcus neoformans (strain 52D). C. neoformans was inoculated into uPA-/- and control mice (uPA+/+), and cell recruitment to the lungs was quantitated. The number of CFU in lung, spleen and brain was determined to assess clearance, and survival curves were generated. By day 21 after inoculation, uPA-/- mice had markedly fewer pulmonary inflammatory (CD45+), CD4+, and CD11b/CD18+ cells compared with uPA+/+ controls (P<0.0007); pulmonary CFUs in the uPA-/- mice continued to increase, whereas CFUs diminished in uPA+/+ mice(P<0.005). In survival studies, only 3/19 uPA+/+ mice died, whereas 15/19 uPA-/- mice died (p<0.001). We have demonstrated that uPA is required for a pulmonary inflammatory response to C. neoformans. Lack of uPA results in inadequate cellular recruitment, uncontrolled infection, and death.

Animals↗

Use of intrathecal baclofen in the treatment of patients with dystonia.

BACKGROUND: Continuous infusion of intrathecal (IT) baclofen is a highly effective standard therapy for severe spasticity of spinal origin. By contrast, there is limited clinical experience regarding the use of IT baclofen in treating patients with dystonia, and little is known regarding the indications for treatment, efficacy, and safety of IT baclofen in this disorder. OBJECTIVE: To study retrospectively the effects of IT baclofen in treating 25 patients with severe segmental or generalized dystonia. SETTING: Neurological Institute, Columbia-Presbyterian Medical Center, New York, NY. PATIENTS: Twenty-five patients with severe segmental or generalized dystonia that was refractory to oral medications underwent IT baclofen test dosing. In addition to dystonia, 17 patients had spasticity or painful spasms. Thirteen of 25 patients responded to the test doses of IT baclofen, according to unblinded neurological assessments that included the patient's subjective report; all 13 underwent implantation of a pump for continuous IT baclofen infusion. RESULTS: In contrast to reports of patients with spasticity of spinal origin, those with dystonia in the present series had a lower response rate to bolus IT baclofen doses and a smaller degree of clinical improvement. For 10 of the 13 responders to the test doses of IT baclofen, dystonia rating scale scores of videotaped examinations by blinded observers detected no significant change (P < .07) in severity of dystonia. Retrospective data from 11 of 13 patients with implantable pumps, followed up for a mean interval of 21 months after pump insertion, showed continuing efficacy in 6 individuals (55%), based on a determination of patient satisfaction; however, only 3 patients (27%) reported a sustained improvement in functional capacity. Five (38%) of the 13 patients with implantable pumps experienced severe complications that required hospitalization. CONCLUSIONS: Despite recent reports that have described the benefit in small numbers of patients with dystonia, we concluded that the role of IT baclofen in treating severe dystonia remains uncertain. Intrathecal baclofen may be more effective when dystonia is associated with spasticity or pain. In the present series, we detected no significant difference in the response to IT baclofen in patients with or without spasticity or pain, perhaps owing to the small sample size.

Baclofen↗

Rating child psychiatric caseness from detailed case histories.

Novel operationalized criteria were used to rate detailed psychiatric case histories on 151 schoolchildren with hemiplegia. Ratings of psychiatric caseness or non-caseness were reliable and valid; the method may be widely applicable. Caseness was defined both narrowly (in terms of social incapacity for the child) and broadly (in terms of disruption to others and distress too). Both definitions could be applied reliably to children of normal intelligence but broadly defined caseness was more reliable for children with low intelligence. Barely half of the psychiatric cases met operationalized criteria for one or more DSM-IV or ICD-10 diagnosis. The others mostly had partial or mixed syndromes that could, with clinical judgement, be assigned to "not otherwise specified' diagnoses. Even with clinical judgement, however, a substantial minority of children with low intelligence had clinically significant but undiagnosable abnormalities in social relatedness.

Affective Symptoms↗

Are only children different? A study of child psychiatric referrals. A research note.

For this study, 683 only children seen in a psychiatric clinic were compared with 2364 children from two-child families seen in the same clinic. From the age of 5 years, there were no differences between the two groups. Among the under-5s, a higher proportion of only children had problems which did not warrant a psychiatric diagnosis, and their parents were more often rated as being over-protective. Our findings suggest, however, that the parents of only children differ from other parents not in being more over-protective but in being more willing or able to seek medical advice.

Adolescent↗

Do incomplete ascertainment and recruitment matter? - a study in childhood hemiplegia.

London children with hemiplegia were ascertained from multiple sources . The effectiveness of ascertainment varied markedly between different sectors of London, and many families did not respond to a written appeal to participate in the research (though most did respond to a personal appeal). Subjects from areas with high and low ascertainment rates had very similar demographic, medical, cognitive and behavioral variables, and so did easy- and hard-to-recruit subjects. The characteristics of the sample as a whole closely resembled those of previous epidemiological samples of hemiplegic children. It would be rash to assume that incomplete ascertainment and recruitment are innocuous, even though they did not make this sample unrepresentative.

Adolescent↗

A twin study of congenital hemiplegia.

Twins were more than three times more common in a large sample of London children with congenital hemiplegia than in the general population. This over-representation of twins could largely be explained by their higher rate of preterm birth, though twin-specific risk factors, including the consequences of a co-twin's death in utero, may also have played a part. None of the 34 co-twins who survived infancy had hemiplegia or any other form of cerebral palsy. Among 155 siblings of singletons with congenital hemiplegia, no child had hemiplegia and only one had cerebral palsy. Perhaps it is chance rather then genetic liability or an adverse environment that primarily governs who does and does not become congenitally hemiplegic.

Adolescent↗

IQ and its predictors in childhood hemiplegia.

In a population-based sample of 149 children with hemiplegia, IQ was highly correlated with a simple-to-calculate index of neurological severity. Performance IQ was an average of 13 points lower then verbal IQ-a difference that seemed to reflect specific visuospatial difficulties rather than an effect of the motor disability itself. Side of lesion was not significantly related to either IQ or verbal-performance discrepancy. Neurological factors and social class had additive effects on IQ, suggesting that neurological damage neither amplified nor obliterated the impact of ordinary psychosocial and genetic factors on IQ.

Age of Onset↗

Clinical experience with Repotin, a locally produced recombinant human erythropoietin, in the treatment of anaemia of chronic renal failure in South Africa.

OBJECTIVE: To evaluate the efficacy and safety of Repotin, a locally produced recombinant human erythropoietin (rHuEPO), in the treatment of the anaemia of chronic renal failure (ACRF). DESIGN: The study consisted of two multicentre non-randomised open stages. SETTING: Renal units at several teaching hospitals in South Africa. PARTICIPANTS: Haemodialysis patients with haemoglobin (Hb) levels less than 8.0 g/dl were recruited. The first stage examined 26 patients during a 12-week period in which the dose of intravenous rHuEPO was adjusted according to haematological response. In the second stage 27 patients were stabilised with intravenous rHuEPO and then maintained at a Hb level above 8.0 g/dl by subcutaneous administration for up to 1 year. OUTCOME MEASURES: In both stages, outcome was measured by clinical examination, blood pressure, full haematological parameters and blood chemistry. RESULTS: In stage 1, all patients responded to therapy with a statistically significant increase in Hb from geometric means of 6.28 g/dl to 8.50 g/dl (geometric SDs of 1.17 and 1.20 respectively). The doses used ranged from 25 IU to 125 IU/kg (average 47.1). In the second stage, Hb levels reached a mean of 8.06 g/dl (SD 0.9) and were maintained at target range with an average dose of 55.5 IU/kg three times a week. Apart from changes in serum iron, ferritin (associated with increased haematopoiesis) and potassium, there were no significant alterations in blood chemistry. The incidence of adverse events reported during the 12-month second stage was no greater than that reported for other forms of rHuEPO therapy. CONCLUSION: Repotin is a safe and effective rHuEPO preparation for the treatment of ACRF.

Adult↗

The relationship between normal variation in IQ and common childhood psychopathology: a clinical study.

The relationship between normal variation in IQ and common psychopathology was examined in a sample of 339 5- to 16-year-olds who were seen at a tertiary psychiatric clinic. The mean IQ was 9.6 points lower (95% CI 5.5 to 13.6 points lower) for conduct than for emotional disorders, with mixed disorders in between. For these common disorders, the mean IQ was 6.0 points lower (95% CI 1.6 to 10.3 points lower) for females than males. IQ variation in the normal range was inversely related to a dimensional measure of conduct problems - an association that was not attributable to social class or mediated by scholastic attainments. Other dimensional measures of psychopathology - covering emotional symptoms, developmental immaturity and relationship difficulties - were not significantly correlated with IQ. Limitations of the study are discussed in the paper.

Adolescent↗

Questionnaire screening for comorbid pervasive developmental disorders in congenitally blind children: a pilot study.

Teachers and parents completed the Autism Behavior Checklist (ABC) on a clinical sample of 17 congenitally blind children. Of the 17 children, 4 had a definite or likely pervasive developmental disorder (PDD) as judged by independent case note review. The ABC was administered both in its original format and in a slightly modified format. Only teacher-completed ABCs detected group differences and had satisfactory test-retest reliability. The modified-format ABC completed by teachers detected 3 of the 4 children with PDDs without any false positives. Screening questionnaires may have a limited but useful role in locating subjects with blindness plus putative PDDs for further study.

Autistic Disorder↗