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Biomedical subjects

Michael I Shevell

Publications and source records attributed to Michael I Shevell.

At least 19 recordsLinked to original sources
PubMed2006

A clinical and etiologic profile of spastic diplegia.

To identify the clinical and etiologic profile of children with spastic diplegia, the medical records of patients with spastic diplegia in a single practice over a 12-year period were systematically and retrospectively reviewed. Clinical factors and possible etiology based on investigations were identified. Univariate and binomial logistical regression analyses were undertaken to identify factors correlating with an etiologic determination. Chart review identified 54 children with spastic diplegia. There were 31 (57.4%) preterm children and 23 (42.6%) term children. Periventricular leukomalacia was diagnosed in 24 (44.4%) children (26.1% of term children, 58.1% of preterm children). An etiology was not identified in 25 (46.3%) children: 14 (60.9%) term children and 11 (35.5%) preterm children. Periventricular leukomalacia among all children correlated with a birth weight less than 2000 gm (P = 0.037), history of neonatal resuscitation (P = 0.004), and gestation less than 33 weeks (P = 0.001). Factors specifically associated with periventricular leukomalacia in term children were a problematic perinatal history (P = 0.011), a history of neonatal resuscitation (P = 0.011), and a history of neonatal respiratory distress (P = 0.046). Regression analysis revealed a correlation between an abnormal perinatal history and an etiology of periventricular leukomalacia among term children (odds ratio 8.67, 95% confidence interval 2.51-29.97, P = 0.001). Approximately half of all children with spastic diplegia encountered in clinical practice will have an etiology identified.

Adolescent↗
PubMed2006

Communicating a diagnosis of cerebral palsy: caregiver satisfaction and stress.

As part of the implementation of a population-based registry of children with cerebral palsy, caregiver satisfaction with the process by which diagnosis was originally communicated by a professional was assessed. Satisfaction with various aspects of the diagnosis process was assessed using a five-point Likert scale and related to child, family, and situational characteristics. Measures were then correlated with current caregiver stress as measured objectively by the Parenting Stress Inventory. During the registration process, 59 consecutive caregivers (55 mothers) were questioned. Overall, 62% (35/59) were satisfied with the disclosure process, with satisfaction ranging from 69% (41/59-hopefulness) to 92% (54/59-honesty) for professional qualities, and from 61% (36/59-sufficient information provided) to 78% (46/59-understandable) for disclosure content. Satisfaction was related to the quantity and content of information given at the disclosure session. Parenting Stress Inventory scores, both total and for parental distress, correlated significantly with both the severity of the child's cerebral palsy and caregiver satisfaction with varying elements of the disclosure process. Overall caregiver satisfaction with the process by which a diagnosis of cerebral palsy is given appears to be good. Together with the severity of a child's intrinsic cerebral palsy, it appears to relate to later parental adjustment to a setting of chronic disability, suggesting a portal through which improvements in information delivery may result in better familial adaptation to disability.

Adult↗
PubMed2006

"A cold wind coming": Heinrich Gross and child euthanasia in Vienna.

Medicine in the German Third Reich became an instrument of Nazi political philosophy that included racial purification using biologic measures. This included an involuntary euthanasia program directed at mentally and physically disabled children. One such effort existed at the Am Spiegelgrund Hospital in Vienna under the direction of Dr Heinrich Gross. This article details the scientific and policy origins of child euthanasia in Vienna, the use of scientific materials obtained for personal and professional gain, and the postwar attempts to reconcile with this abject past.

Austria↗
PubMed2006

Diagnosing Sotos syndrome in the setting of global developmental delay and macrocephaly.

Sotos syndrome (cerebral gigantism) is characterized by macrocephaly, global developmental delay, characteristic facial dysmorphology, and a markedly advanced bone age. The purpose of this study was to describe the prevalence of Sotos syndrome in a consecutive series of patients with global developmental delay, which might modify our laboratory evaluation approach to this particular clinical situation. For a 10-year inclusive interval, the case records of all consecutive patients referred for global developmental delay in a single pediatric neurology practice were reviewed. Patients with macrocephaly were defined by an age- and gender-adjusted head circumference greater than or equal to the 98th percentile. Possible clinical factors associated with eventual diagnosis of Sotos syndrome in this group of macrocephalic children were tested with a two-tailed Fisher exact test. Of 261 children with global developmental delay, 18 (7%) had documented macrocephaly. Of these 18 children, 3 (17%) had an advanced bone age and were diagnosed with Sotos syndrome. In patients with global developmental delay and concomitant macrocephaly, Sotos syndrome is not uncommon. Assessment of bone age is a simple screening test for diagnosis of this entity and should be undertaken routinely in children with macrocephaly and global developmental delay even in the absence of other distinctive syndromic clinical features.

Abnormalities, Multiple↗
PubMed2006

Analysis of clinical features predicting etiologic yield in the assessment of global developmental delay.

OBJECTIVE: Global developmental delay is a common reason for presentation for neurologic evaluation. This study examined the role of clinical features in predicting the identification of an underlying cause for a child's global developmental delay. METHODS: Over a 10-year inclusive interval, the case records of all consecutive children <5 years of age referred to a single ambulatory practice setting for global developmental delay were systematically reviewed. The use of clinical features in predicting the identification of a specific underlying cause for a child's delay was tested using chi2 analysis. RESULTS: A total of 261 patients eventually met criteria for study inclusion. Mean age at initial evaluation was 33.6 months. An underlying cause was found in 98 children. Commonest etiologic groupings were genetic syndrome/chromosomal abnormality, intrapartum asphyxia, cerebral dysgenesis, psychosocial deprivation, and toxin exposure. Factors associated with the ability to eventually identify an underlying cause included female gender (40 of 68 vs 58 of 193), abnormal prenatal/perinatal history (52 of 85 vs 46 of 176), absence of autistic features (85 of 159 vs 13 of 102), presence of microcephaly (26 of 40 vs 72 of 221), abnormal neurologic examination (52 of 71 vs 46 of 190), and dysmorphic features (44 of 84 vs 54 of 177). In 113 children without any abnormal features identified on history or physical examination, routine screening investigations (karyotype, fragile X molecular genotyping, and neuroimaging) revealed an underlying etiology in 18. CONCLUSIONS: Etiologic yield in an unselected series of young children with global developmental delay is close to 40% overall and 55% in the absence of any coexisting autistic features. Clinical features are readily apparent that may enhance an expectation of a successful etiologic search. Screening investigations may yield an underlying cause.

Child, Preschool↗
PubMed2006

The clinical spectrum of developmental language impairment in school-aged children: language, cognitive, and motor findings.

OBJECTIVE: Our goal was to evaluate detailed school-age language, nonverbal cognitive, and motor development in children with developmental language impairment compared with age-matched controls. METHODS: Children with developmental language impairment or normal language development (controls) aged 7 to 13 years were recruited. Children underwent language assessment (Clinical Evaluation of Language Fundamentals-4, Peabody Picture Vocabulary-3, Goldman-Fristoe Test of Articulation-2), nonverbal cognitive assessment (Wechsler Intelligence Scale for Children-IV), and motor assessment (Movement Assessment Battery for Children). Exclusion criteria were nonverbal IQ below the 5th percentile or an acquired language, hearing, autistic spectrum, or neurologic disorder. RESULTS: Eleven children with developmental language impairment (7:4 boys/girls; mean age: 10.1 +/- 0.8 years) and 12 controls (5:7 boys/girls; mean age: 9.5 +/- 1.8 years) were recruited. Children with developmental language impairment showed lower mean scores on language (Clinical Evaluation of Language Fundamentals-4--developmental language impairment: 79.7 +/- 16.5; controls: 109.2 +/- 9.6; Goldman-Fristoe Test of Articulation-2--developmental language impairment: 94.1 +/- 10.6; controls: 104.0 +/- 2.8; Peabody Picture Vocabulary-3--developmental language impairment: 90.5 +/- 13.8; controls: 100.1 +/- 11.6), cognitive (Wechsler Intelligence Scale for Children-IV--developmental language impairment: 99.5 +/- 15.5; controls: 113.5 +/- 11.9), and motor measures (Movement Assessment Battery for Children percentile--developmental language impairment: 12.7 +/- 16.7; controls: 66.1 +/- 30.6) and greater discrepancies between cognitive and language scores (Wechsler Intelligence Scale for Children-IV/Clinical Evaluation of Language Fundamentals-4--developmental language impairment: 17.8 +/- 17.8; controls: 1.2 +/- 12.7). Motor impairment was more common in children with developmental language impairment (70%) than controls (8%). CONCLUSIONS: Developmental language impairment is characterized by a broad spectrum of developmental impairments. Children identified on the basis of language impairment show significant motor comorbidity. Motor assessment should form part of the evaluation and follow-up of children with developmental language impairment.

Child↗
PubMed2006

Screening for developmental delay in the setting of a community pediatric clinic: a prospective assessment of parent-report questionnaires.

OBJECTIVES: Our goal for this study was to prospectively test whether parent-completed questionnaires can be effectively used in the setting of a busy ambulatory pediatric clinic to accurately screen for developmental impairments. Specific objectives included (1) assessing the feasibility of using parent-report instruments in the setting of a community pediatric clinic, (2) evaluating the accuracy of 2 available screening tests (the Ages and Stages Questionnaire and Child Development Inventory), and (3) ascertaining if the pediatrician's clinical judgment could be used as a potential modifier. METHODS: Subjects were recruited from the patient population of a community clinic providing primary ambulatory pediatric care. Subjects without previous developmental delay or concerns noted were contacted at the time of their routine 18-month-old visit. Those subjects who agreed to participate were randomly assigned to 1 of 2 groups and completed either the Ages and Stages Questionnaire or Child Development Inventory. The child's pediatrician also completed a brief questionnaire regarding his or her opinion of the child's development. Those children for whom concerns were identified by either questionnaire underwent additional detailed testing by the Battelle Development Inventory, the "gold standard" for the purposes of this study. An equal number of children scoring within the norms of the screening measures also underwent testing with the Battelle Development Inventory. RESULTS: Of the 356 parents contacted, 317 parents (90%) agreed to participate. Most parents correctly completed the Ages and Stages Questionnaire (81%) and the Child Development Inventory (75%). Predictive values were calculated for the Ages and Stages Questionnaire and the Child Development Inventory (sensitivity: 0.67 and 0.50; specificity: 0.39 and 0.86; positive predictive value: 34% and 50%; negative predictive value: 71% and 86%, respectively). Incorporating the physician's opinion regarding the developmental status of the child did not improve the accuracy of the screening questionnaires. CONCLUSIONS: Three important conclusions were reached: (1) parent-completed questionnaires can be feasibly used in the setting of a pediatric clinic; (2) the pediatrician's opinion had little effect in ameliorating the accuracy of either questionnaire; and (3) single-point accuracy of these screening instruments in a community setting did not meet the requisite standard for development screening tests as set by current recommendations. This study raises important questions about how developmental screening can be performed, and we recommend additional research to elucidate a successful screening procedure.

Ambulatory Care Facilities↗
PubMed2006

Equivalence reliability of the Vineland Adaptive Behavior Scale between in-person and telephone administration.

There is a critical need to establish cost effective ways to monitor developmental progress of children at risk for developmental disabilities. The Vineland Adaptive Behavior Scale (VABS) is a well-known functional measure used for both clinical and research purposes. The objective of this study was to examine the equivalence reliability of the VABS using two different administration methods; in-person versus telephone interviews. Fifty children with or at-risk for developmental disability were tested (mean age of 77.5 +/- 18.5 months) using both interview formats. Correlations between in-person and telephone interview scores were extremely high for all subdomains including Communication (ICC = 0.99), Daily Living Skills (ICC = 0.98), Socialization (ICC = 0.96), Motor (ICC = 0.98) as well as the Adaptive Behavior Composite score (ICC = 0.99). The ability to collect reliable information on a child's developmental progress using a telephone interview format is critical, given current service delivery constraints.

Adaptation, Psychological↗
PubMed2005

Motor function at school age in children with a preschool diagnosis of developmental language impairment.

OBJECTIVES: To evaluate fine motor (FM) and gross motor (GM) function shortly after school entry in children with a preschool diagnosis of developmental language impairment (DLI). STUDY DESIGN: A cohort of children (n = 70) diagnosed at pre-school age with DLI was reevaluated in elementary school. Language, cognitive, and motor outcomes were assessed through the use of the Battelle Developmental Inventory (BDI). Language was further assessed through the use of the Vineland Adaptive Behavior Scale, Peabody Picture Vocabulary, and Expressive One Word Picture Vocabulary Tests. Performance below -1.5 SD of the normative mean on any test was considered to represent impairment. RESULTS: Forty-three children (mean age, 7.4 +/- 0.7 years) underwent reassessment at a mean of 3.8 +/- 0.7 years after initial preschool assessment. Mean scores for BDI motor domains (FM, 78.3 +/- 11.4; GM, 84.9 +/- 13.3) fell below normative values. Twenty-two children (52%) had motor impairment (FM, 17 of 42; GM, 15 of 42); 35 of 43 (81%) continued to have language impairment. BDI communication raw scores correlated most strongly with FM (rho = 0.73, P < .001) and GM (rho = 0.58, P = .003) raw scores but showed only moderate correlations with cognitive raw scores (rho = 0.41, P = .05). CONCLUSIONS: Impaired motor function is an important comorbidity in DLI. Factors critical to motor performance may also contribute to language deficits in DLI.

Child, Preschool↗
PubMed2005

Outcomes at school age of preschool children with developmental language impairment.

In a prospective study, preschool children diagnosed with developmental language impairment were systematically reassessed during the early school years with standardized developmental (Battelle Developmental Inventory) and functional measures (Vineland Adaptive Behavior Scale). Of an original cohort of 70 children assessed and diagnosed at a mean age of 3.6 +/- 0.7 years, 43 were reassessed at a mean age of 7.4 +/- 0.7 years. Group performance on the Battelle overall was 71.9 +/- 8.2 with the lowest sub-domain score in communication at 69.5 +/- 8.9. On the Battelle, 67% of children fell below the 1.5 standard deviation (S.D.) cutoff signifying significant developmental concerns. Between 36% (gross motor) and 83% (communication) of the cohort performed at least 1.5 S.D. below the normative mean on the individual domains of the Battelle. Seventy-four percent were impaired in two or more domains of the Battelle. The group mean on the Vineland overall was 81.1 +/- 16.9 with between 19% (socialization) to 48% (communication) of the cohort scoring more than 1.5 S.D. below the mean on each of the sub-domains. Almost half of the cohort (20/42, 48%) manifested functional impairment in at least two domains of the Vineland. Univariate and multivariate analysis of potential predictor variables identified only female sex as being predictive of significantly poorer performance on the Vineland communication sub-domain and the Vineland total score. Children with early developmental language impairment demonstrate persistent impairments in developmental and functional skills at school entry not limited to language. Deficits remain especially evident in the communication sub-domain. These results have implications with respect to later prognostication, family counseling, and devising a programmatic approach to this group of children.

Child↗
PubMed2005

Corrected head circumference centiles as a possible predictor of developmental performance in high-risk neonatal intensive care unit survivors.

The aim of this study was to evaluate the predictive value of corrected head circumference (HC) centiles at 2 years of age with respect to developmental performance in a series of high-risk neonatal intensive care unit (NICU) survivors with microcephaly. The study used a retrospective review of the clinical files of children seen in a clinic devoted to the follow-up of all high-risk survivors of a hospital's level III NICU. All children with microcephaly (occipital-frontal circumference below the 2nd centile for sex) at 2 years of age were identified. The HC obtained at 2 years was corrected to the ages for which the absolute HC corresponded to either the 50th or 2nd centile for the child's sex. Of 312 high-risk patients followed, 38 (12.2%) were microcephalic. Fifteen performed below the 50th age-corrected HC centile (severe developmental delay), 12 performed between the 50th and 2nd age-corrected HC centile (moderate developmental delay), and 11 performed above the 2nd age-corrected HC centile (mild developmental delay). The absolute value of HC measurement was not a predictor of developmental performance. Of all clinical factors evaluated, only coexisting epilepsy was found to be a significant predictor of less than the 50th age-corrected HC centile developmental performance (chi2=6.134, p=0.01). We conclude that in a high-risk population, the presence of microcephaly implies developmental impairment, though neither the absolute HC measurement nor the corrected HC centile is predictive. Coexisting epilepsy in this context appears to worsen developmental outcome.

Cephalometry↗
PubMed2005

Developmental screening.

An estimated 5 to 10% of the pediatric population has a developmental disability. The current strategy to identify these children is through developmental surveillance, a continuous procedure in which the health professional observes the infant, takes a developmental history, and elicits any concerns that the caregiver might have. However, identification of delayed children is ineffective when based solely on routine surveillance. A necessary adjunct is developmental screening: the process of systematically identifying children with suspected delay who need further assessment. Screening tests greatly improve the rate of identification. With the advent of intervention programs and the support of organizations such as the American Academy of Pediatrics, the topic of developmental screening is a timely and essential one. This review aims to describe the properties of screening tests, to evaluate the available tools for developmental screening while providing a representative sample of the currently available developmental tests, and, finally, to evaluate the efficacy of intervention programs, a needed prerequisite to justify screening.

Adolescent↗
PubMed2005

Type I spinal muscular atrophy can mimic sensory-motor axonal neuropathy.

Spinal muscular atrophy is a group of allelic autosomal recessive disorders characterized by progressive motoneuron loss, symmetric weakness, and skeletal muscle atrophy. It is traditionally considered a pure lower motoneuron disorder, for which a current definitive diagnosis is now possible by molecular genetic testing. We report two newborns with a clinical phenotype consistent with that of spinal muscular atrophy type I and nerve conduction studies and electromyography suggesting more extensive sensory involvement than classically described with spinal muscular atrophy. Molecular testing confirmed spinal muscular atrophy in patient 1 but not in patient 2. Thus, in the setting of a suspected congenital axonal neuropathy, molecular testing might be necessary to distinguish spinal muscular atrophy type I from infantile polyneuropathy.

Diagnosis, Differential↗
PubMed2005

Profile of pediatric hemiparesis.

Our objective was to determine the clinical spectrum of pediatric hemiparesis by identifying the relative frequency of various diagnoses and comorbid conditions seen in these children. Case records of all patients with hemiparesis in a single practice over an 11-year period were reviewed with reference to clinical features, etiologic determination, and comorbid conditions. Ninety-two children were identified: 73 (79.3%) had a congenital hemiparesis and 19 (20.7%) had an acquired hemiparesis. An abnormal perinatal history (P = .003), prematurity (P = .016), and younger age at onset of symptoms (P < .001) were associated with a congenital hemiparesis. The overall etiologic yield was 83.7% (82.2% in the congenital and 89.5% in the acquired). The top four etiologic entities were cerebrovascular ischemia (40.2%), periventricular leukomalacia (18.5%), intracranial hemorrhage (16.3%), and cerebral dysgenesis (13%). Factors predictive of establishing an underlying etiology included birth prior to 34 weeks' gestation (P = .034), global developmental delay (P = .048), epilepsy (P = .024), and having appropriate imaging modalities (P = .001). Half of these children had a concurrent global developmental delay, associated epilepsy (odds ratio 3.67; 95% confidence interval 1.40-9.72), and prematurity (odds ratio 5.41; 95% confidence interval 1.56-18.80). A third of these children developed epilepsy. Multivariate predictive factors for epilepsy included global developmental delay (odds ratio 4.20; 95% confidence interval 1.44-12.27), cerebrovascular ischemia (odds ratio 5.10; 95% confidence interval 1.76-14.77), and term birth (odds ratio 3.87; 95% confidence interval 1.20-12.56). The majority of children with hemiparesis have a congenital etiology. The diagnostic yield is higher than previously reported; however, specific underlying etiologies need to be better determined. Comorbid conditions of global developmental delay and epilepsy have a high prevalence in this population, contributing to overall morbidity.

Adolescent↗
PubMed2005

Factors predictive of outcome in childhood epilepsy.

To identify early predictive factors of outcome in childhood epilepsy, the case records of all children with new-onset epilepsy presenting to a single neurology practice over a 10-year interval were reviewed. Only children with more than 2 years of follow-up were included. Cox regression analysis was used to identify factors predictive of remission (successful cessation of medication). One hundred ninety-six children (mean age 7.6 +/- 3.7 years at first seizure, mean follow-up 55 +/- 30 months) were identified. Ninety-eight of 196 children (50%) had an idiopathic epilepsy, 63 of 196 (32.1%) had cryptogenic epilepsy, and 35 of 196 (17.9%) had remote symptomatic epilepsy. At final assessment, 52.6% were in remission, 12.8% had a poor outcome (recurrent seizures on therapeutic antiepileptic drug levels within 6 months prior to the final assessment), and 6.9% were intractable (more than one seizure/month over 1 year with failure of three or more anticonvulsants). One year after initiating treatment, factors associated with a lower probability of remission included seizure recurrence in the 6- to 12-month interval after therapy initiation (hazard ratio 0.24), multiple seizure types (hazard ratio 0.40), and mental retardation at onset (hazard ratio 0.19). Factors predictive of a poor outcome included seizure recurrence in the 6- to 12-month interval after therapy initiation (odds ratio 21.6), more than one seizure type (odds ratio 8.9), and global developmental delay at onset (odds ratio 8.9). Factors predictive of intractability included multiple seizure types (hazard ratio 6.5), mental retardation at onset (hazard ratio 7.2), and seizure recurrence in the first 6 to 12 months of treatment (hazard ratio 70). It appears that response in the first 6 to 12 months on antiepileptic medication is predictive of outcome. Clinical features of the underlying epilepsy and concurrent neurologic conditions were independently associated with intractability and a lower probability of remission.

Adolescent↗
PubMed2004

The predictive value of a preschool diagnosis of developmental language impairment.

OBJECTIVE: To evaluate language and cognitive outcomes in elementary school children with a prior preschool diagnosis of developmental language impairment (DLI). DESIGN/METHODS: A cohort of preschool children, consecutively diagnosed with isolated language impairment, was reassessed in elementary school. Measures used were the communication domains of the Battelle Developmental Inventory (BDI) and the Vineland Adaptive Behavior Scale, Peabody Picture Vocabulary and Expressive One Word Picture Vocabulary. Cognition was assessed using the BDI cognitive domain. Language impairment was defined as performance more than 1.25 SD below normative means on a language measure. Specific language impairment (SLI) was defined as language impairment concurrent with a cognitive score not more than one SD below the normative mean. RESULTS: A total of 43/70 (61%) children were reassessed. Mean age in preschool was 3.6 +/- 0.7 years and 7.4 +/- 0.7 years at follow up. A total of 36/43 (84%) showed persistent language impairment. The mean BDI cognitive domain score was 80.0 +/- 14.2 (15/42 below -2 SDs). Only 11/42 (26%) children met current research criteria for SLI, 24/42 (57%) had language impairment but had cognitive scores more than one SD below normative means, and 4/42 (10%) had normal language and cognitive skills. No factors could be identified at intake that predicted language outcome using univariate or multivariate analysis. CONCLUSIONS: While a preschool diagnosis of developmental language impairment predicted persisting language impairment, the specificity of this impairment did not persist. This suggests either undiagnosed cognitive impairment in preschool children with apparently isolated language impairment or an evolving profile of more global developmental impairment.

Age Factors↗
PubMed2004

Familial retinal migraines.

Approximately 25% of sufferers of retinal migraine are thought to have a positive family history. Retinal migraines can cause both transient, and rarely permanent, unilateral monocular visual loss. This report of familial retinal migraines furthers our understanding of this particular migraine subtype. Two families with retinal migraines are reported suggesting an autosomal-dominant inheritance pattern with variable expression and penetrance.

Adolescent↗