Ruth D Nass, M.D.

The clinical utility of the Social Responsiveness Scale and Social Communication Questionnaire in tuberous sclerosis complex
Granader, Yael E; Bender, Heidi A; Zemon, Vance; Rathi, Sipra; Nass, Ruth; Macallister, William S
2010 Jul;18(3):262-266, Epilepsy & behavior
Tuberous sclerosis complex (TSC) is often associated with epilepsy, mental retardation, and autism spectrum disorders (ASDs). Thus, screening for ASDs is important when evaluating these individuals. We examined the utility of the Social Responsiveness Scale (SRS) and Social Communication Questionnaire (SCQ), two measures for screening for ASDs, in a TSC population. Twenty-one children were evaluated, with 52.4% classified as having ASDs on the SRS and 42.9% classified as such on the SCQ. Number of antiepileptic drugs significantly correlated with SRS Total score, as did level of intellectual functioning. Evidence for convergent validity was obtained between the SRS and SCQ Total scores (r=0.605). Moreover, all SRS subscales correlated with SCQ Total score (r>0.400). All SCQ subscales except for Communication correlated with SRS total. Overall, the results demonstrate that these questionnaires appear to be effective screens for ASDs in a TSC population and are measuring similar constructs
— id: 110875, year: 2010, vol: 18, page: 262, stat: Journal Article,

Ethical perspectives in neurology: Ethical considerations in sudden unexplained death in epilepsy
Hamid, Hamada; Nass, Ruth
2010 ;16(3):242- 245, Continuum : lifelong learning in neurology
The cause of sudden unexplained death in epilepsy patients (SUDEP) is unknown, although a number of mechanisms have been suggested, including cardiac arrhythmia precipitated by seizure discharge acting via the autonomic nervous system, respiratory arrest, and neurogenic pulmonary edema. The risk of SUDEP varies with the severity of epilepsy and is highest among candidates for epilepsy surgery and those followed at epilepsy referral centers. The risk of SUDEP increases when seizures are frequent. Improved seizure control may reduce the risk of SUDEP for patients at high risk for it because of sub optimal seizure control. Thus, knowledge about SUDEP becomes increasingly relevant as the severity of epilepsy increases. The discussion of SUDEP will involve providing patients with more information, choices, and opportunities to prevent this rare but catastrophic complication of epilepsy. Awareness of SUDEP could influence more than medical decisions. Informing low-risk patients of SUDEP is more ethically preferable than withholding information because the potential benefits of increasing compliance to medication, reassuring patients and their families about the low risk, and introducing the ideas of advance directives outweigh the potential harm of causing anxiety.
— id: 111752, year: 2010, vol: 16, page: 242, stat: Journal Article,

Cognitive and behavioral abnormalities of pediatric diseases
Nass, Ruth D; Frank, Yitzchak
New York : Oxford University Press, 2010,
This book provides a detailed account of intellectual, other neuropsychological and behavioral manifestations of general pediatric diseases. The conditions discussed include the whole range of pediatric diseases - genetic syndromes, other congenital conditions, metabolic, endocrine, gastrointestinal, infectious, immunologic, toxic, trauma, and neoplastic, as well as sensory disabilities including deafness and blindness. Although the book is not intended to discuss cognitive and behavioral manifestations of conditions usually considered to be primary neurological disease, some of those, including cerebral palsy, muscular dystrophy, myotonic dystrophy and epilepsy, are included. Where possible, a translational approach is used, linking the behavioral and cognitive manifestations of these conditions, to the underlying structural, chemical or genetic abnormalities and their effect on the brain, and, in turn, on behavior and cognition. At the same time, included are significant psychosocial factors. Together, those factors have a major effect on patients' performance, including school performance, and on their families
— id: 2005, year: 2010, vol: , page: , stat: ,

Compulsive Spitting in a Child with Pervasive Developmental Disorder and Tourette's Disorder: Tic or Compulsion?
Nass, Ruth; Coffey, Barbara J
2010 Feb;20(1):71-73, Journal of child & adolescent psychopharmacology
— id: 107381, year: 2010, vol: 20, page: 71, stat: Journal Article,

Perinatal stroke
Stiles, Joan; Nass, Ruth D; Levine, Susan C; Moses, Pamela; Reilly, Judy S
Pediatric neuropsychology: Research, theory, and practice New York, NY, US: Guilford Press; US, 2010,
(from the chapter) This chapter examines the medical and neuropsychological effects of perinatal stroke (PS). It begins with an overview of the mechanisms and suspected causes of stroke in the perinatal period, and considers the medical consequences for the physical development of the motor and sensory systems. The remainder of the chapter focuses on the behavioral consequences of PS. The discussion of neuropsychological effects includes an overview of research using standardized measures of behavioral development, as well as a summary of experimental studies that examine specific aspects of development in greater detail. Finally, we review some very recent functional neuroimaging studies designed to examine alternative patterns of brain organization that can emerge after PS.
— id: 5338, year: 2010, vol: , page: 181, stat: Chapter,

Tuberous sclerosis complex: a tale of two genes
Nass, Ruth; Crino, Peter B
2008 Mar 18;70(12):904-905, Neurology
— id: 116236, year: 2008, vol: 70, page: 904, stat: Journal Article,

Effects of early focal brain injury on memory for visuospatial patterns: selective deficits of global-local processing
Stiles, Joan; Stern, Catherine; Appelbaum, Mark; Nass, Ruth; Trauner, Doris; Hesselink, John
2008 Jan;22(1):61-73, Neuropsychology
Selective deficits in visuospatial processing are present early in development among children with perinatal focal brain lesions (PL). Children with right hemisphere PL (RPL) are impaired in configural processing, while children with left hemisphere PL (LPL) are impaired in featural processing. Deficits associated with LPL are less pervasive than those observed with RPL, but this difference may reflect the structure of the tasks used for assessment. Many of the tasks used to date may place greater demands on configural processing, thus highlighting this deficit in the RPL group. This study employed a task designed to place comparable demands on configural and featural processing, providing the opportunity to obtain within-task evidence of differential deficit. Sixty-two 5- to 14-year-old children (19 RPL, 19 LPL, and 24 matched controls) reproduced from memory a series of hierarchical forms (large forms composed of small forms). Global- and local-level reproduction accuracy was scored. Controls were equally accurate on global- and local-level reproduction. Children with RPL were selectively impaired on global accuracy, and children with LPL on local accuracy, thus documenting a double dissociation in global-local processing
— id: 116237, year: 2008, vol: 22, page: 61, stat: Journal Article,

Frequency and predictors of autism in children with tuberous sclerosis complex
Zelleke, TZ; Rubin, MR; Cohen, BC; Lai, GL; LaJoie, JL; Miles, DM; Devinsky, OD; Zaroff, CZ; MacAllister, WM; Weiner, HW; Nass, RN
2008 SEP ;64(3):S115-S115, Annals of neurology
— id: 104238, year: 2008, vol: 64, page: S115, stat: Journal Article,

Mental retardation: Relationship to seizures and tuber burden in tuberous sclerosis
Zaroff, C; Barr, W; Devinsky, O; Miles, D; Nass, R
2006 MAR ;60(3):S127-S128, Annals of neurology
— id: 104247, year: 2006, vol: 60, page: S127, stat: Journal Article,

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex
Zaroff, Charles M; Barr, William B; Carlson, Chad; LaJoie, Josiane; Madhavan, Deepak; Miles, Daniel K; Nass, Ruth; Devinsky, Orrin
2006 Oct;15(7):558-562, Seizure
In patients with tuberous sclerosis complex (TSC), the high rates of mental retardation are associated with cortical tubers, seizure activity, and genetic factors. The goal of the study was to investigate the relationship between bilateral cortical tubers and seizure variables and mental retardation in individuals with TSC. The records of 27 patients with TSC (age 6 months to 33 years) undergoing neuropsychological assessment and the following clinical variables were examined: bilateral versus non-bilateral cortical tubers, the age of seizure onset, and presence of infantile spasms. Results were statistically analyzed. Bilateral cortical tubers (p=0.02) and early age of seizure onset (p=0.04) were significantly related to impaired cognitive functioning. Only one of the seven patients with normal cognitive functioning had bilateral tubers, whereas 13/21 patients with intellectual impairment had bilateral tubers. Patients with normal cognitive functioning experienced a mean age of seizure onset after 6 years. A trend was observed between infantile spasms and cognitive functioning (p=0.06); the lack of statistical significance likely reflects the small sample size. Neither age nor gender was related to cognitive status. Further investigation incorporating additional neuroimaging factors, antiepileptic treatment effects, and genetic variables, is needed
— id: 69073, year: 2006, vol: 15, page: 558, stat: Journal Article,

Evaluation and assessment issues in the diagnosis of attention deficit hyperactivity disorder
Nass, Ruth D
2005 Dec;12(4):200-216, Seminars in pediatric neurology
This article discusses the availability and appropriateness of various assessment techniques for diagnosing ADHD. In terms of the interview/history, the necessity and viability of using formal structured, semi structured, computerized and informal interviews for making an ADHD diagnosis are critiqued. The pros and cons of the various questionnaires frequently used to diagnose ADHD are addressed. The conundrum raised by multiple informants who often disagree is discussed, as are the effects of age and gender. The contributions of the medical and neurological examinations to the diagnosis of ADHD are considered. The utility of a neuropsychological assessment and of continuous performance testing to diagnosis is critiqued. Finally the lack of need for laboratory tests and the lack of necessity of neurological workup, specifically EEG and imaging, are argued
— id: 67851, year: 2005, vol: 12, page: 200, stat: Journal Article,

Mental retardation and relation to seizure and tuber burden in tuberous sclerosis complex
Zaroff, CM; Barr, W; Devinsky, O; Miles, D; Nass, R
2005 SEP ;46(11):166-166, Epilepsia
— id: 59587, year: 2005, vol: 46, page: 166, stat: Journal Article,

Social and affective impairments are important recovery after acquired stroke in childhood
Nass, Ruth D; Trauner, Doris
2004 Jul;9(6):420-434, CNS spectrums
Despite a congenital stroke, overall intelligence at school age is generally within the normal range. Language acquisition problems are more prominent when children are younger (<5 years of age) than when they are older. They are present after both right and left lesions, but appear to have different features. They are less apparent than in the child with a developmental language disorder. Acquired aphasia in childhood results in subtle and often persisting deficits. Children with congenital strokes are at risk for behavioral and psychiatric problems. Those with congenital right hemisphere strokes appear to be more difficult infants, but there is no clear side of lesion effect in older children. Children with congenital right hemisphere strokes have more prominent spatial difficulties than their left lesion counterparts. Evaluating both the process and the product highlights this. Increasing the difficulty of the task often brings out deficits in the right lesion group even when they seemingly recovered
— id: 45973, year: 2004, vol: 9, page: 420, stat: Journal Article,

Effects of mothers' autoimmune disease during pregnancy on learning disabilities and hand preference in their children
Ross, Gail; Sammaritano, Lisa; Nass, Ruth; Lockshin, Michael
2003 Apr;157(4):397-402, Archives of pediatrics & adolescent medicine
OBJECTIVES: To determine whether children (and particularly sons) of women with systemic lupus erythematosus (SLE) during pregnancy are more likely to have learning disabilities (LD) and be non-right-handed, and if maternal disease variables (ie, presence of maternal antibodies, disease activity level, and use of corticosteroids) predict the prevalence of LD in offspring. DESIGN: Case-controlled study with subjects matched by age and sex. PARTICIPANTS: We studied 58 children whose mothers had SLE during pregnancy and 58 children of healthy mothers. Measures Data collected included maternal disease variables in women with SLE during their pregnancies. All children took a standardized intelligence test (Wechsler Intelligence Scale for Children-III) and completed a modified version of the Edinburgh Hand Preference Questionnaire. They also took standardized tests of reading, arithmetic, and writing achievement. Learning disability was defined as having an academic achievement score of at least 1.5 SDs below the Full-Scale IQ. RESULTS: Sons of women with SLE were significantly more likely to have LD than daughters of women with SLE or children of either sex in the control group. Maternal SLE was not associated with non-right-handedness in sons or daughters. The presence of anti-Ro/La antibodies and disease activity (flare) in mothers during pregnancy were significantly related to higher prevalence of LD in offspring. CONCLUSIONS: Autoimmune disease in women during pregnancy is associated with an increased risk for LD in their sons. Maternal antibodies, particularly anti-Ro/La, likely affect the fetal brain of male offspring and result in later learning problems. These findings should promote greater awareness of the risk for LD in sons of women with autoimmune disease and the possible need for early educational intervention in those children
— id: 116238, year: 2003, vol: 157, page: 397, stat: Journal Article,

Attention deficit hyperactivity disorder and Tourette syndrome: what's the best treatment?
Nass, Ruth; Bressman, Susan
2002 Feb 26;58(4):513-514, Neurology
— id: 116239, year: 2002, vol: 58, page: 513, stat: Journal Article,

Perfusion brain scintigraphy studies in infants and children with malformations of the vein of Galen
Nass R; Kramer E; Molofsky W; Melnick J; de Hollisey M; Madrid M; Wisoff JH; Berenstein A
2001 Sep;17(9):519-523, Child's nervous system : ChNS
Cerebral perfusion brain scintigraphy obtained in six unselected patients (age newborn to 14 years) from among 50 children with vein of Galen malformations was used in conjunction with magnetic resonance imaging to determine the basis of the neurological and cognitive abnormalities in patients with vein of Galen malformations (VGMs). Five had a hemiparesis - persistent, transient, or alternating. Four were developmentally delayed. Two had so far been cognitively normal and acquired a neurological deficit, following an embolization procedure. The school age patient had a nonverbal learning disability. Three had epilepsy and/or an abnormal electroencephalogram. Magnetic resonance imaging documented only the VGM, hydrocephalus and atrophy; one child with perinatal asphyxia had periventricular leukomalacia. Perfusion brain scintigraphy was normal in two (a normal infant, and a toddler with a hemiparesis and aphasia). Abnormal findings included: left parietal hypoperfusion, fronto-temporal atrophy, patchy flow; left fronto-temporal hypoperfusion, left hemiatrophy, bilateral medial temporal hypoperfusion, right cerebellar hypoperfusion; right temporal hypoperfusion, patchy flow; right hemiatrophy, occipital hypoperfusion. Perfusion brain scintigraphy findings correlated better with focal neurological and cognitive defects than did magnetic resonance imaging
— id: 34709, year: 2001, vol: 17, page: 519, stat: Journal Article,

Behavioural profiles of children and adolescents after pre- or perinatal unilateral brain damage
Trauner, DA; Nass, R; Ballantyne, A
2001 MAY ;124(5):995-1002, Brain
Recent case reports of individuals with early-onset damage to the prefrontal cortex have suggested that such early insults could result in severely impaired social behaviour in later childhood and adolescence. The investigators speculated that the acquisition of complex social conventions and moral rules had been impaired, In a large cohort of children, we sought to determine whether early focal brain insults might result in clinically significant behavioural or emotional problems. This study reports on 39 children with pre- or perinatal-onset unilateral brain damage (focal lesion) from cerebral infarction or intraparenchymal haemorrhage, using the Achenbach Child Behavior Checklist to assess the presence or absence of behavioural and emotional difficulties. Two-thirds of the subjects had left hemisphere (LH) lesions and one-third had right hemisphere (RH) lesions, Age range was 4.0-15.4 years at the time of questionnaire completion. Their results were compared with those of 54 control children. Analyses were conducted on focal lesion versus controls, RH versus LH lesion, frontal versus non-frontal lesion, and seizure versus non-seizure groups. When the effect of IQ was partialled out, there were no significant differences on the nine Behavior Problem scales, the Internalizing-Externalizing dichotomy or the Total Problem score for any of the group comparisons. Our subjects showed no evidence of clinically significant behavioural or emotional problems, even when the frontal lobe was involved. Individuals with more extensive and bilateral damage may be at higher risk of significant behavioural and emotional dysfunction than were those in our study population. In future studies of brain-behaviour relationships in developing children, all potential causes for any observed behavioural abnormalities, such as genetic and environmental factors and toxin exposure, must be considered before concluding that specific anatomical lesions are causally related to specific behavioural outcomes
— id: 55057, year: 2001, vol: 124, page: 995, stat: Journal Article,

Acquired aphasia in children after surgical resection of left-thalamic tumors
Nass, R; Boyce, L; Leventhal, F; Levine, B; Allen, J; Maxfield, C; Salsberg, D; Sarno, M; George, A
2000 SEP ;42(9):580-590, Developmental medicine & child neurology
Five children (three males, two females; four right-, one left-handed; age range 6 to 14 years) who developed aphasia after gross-total excision of left predominantly thalamic tumors are reported. Three patients had Broca aphasia, one had mixed transcortical aphasia, and one patient had conduction aphasia. In the months after surgery, three children improved while receiving radiation and/or chemotherapy, although none recovered completely. Two patients with malignant tumors developed worsening aphasia when the tumor recurred, and later died. Two of three patients tested had visuospatial difficulties in addition to language deficits. Attention and executive functioning mere affected in three of three patients tested. Memory, verbal and/or visual functioning, were affected in four of four patients tested. Both patients who were tested showed transient right hemineglect. Two of two patients tested were probably apraxic. The wide range of deficits in these children highlights the importance of the thalamus and other subcortical structures in developing cognition
— id: 54506, year: 2000, vol: 42, page: 580, stat: Journal Article,

Autistic regression with rolandic spikes
Nass R; Devinsky O
1999 Jul;12(3):193-197, Neuropsychiatry neuropsychology & behavioral neurology
OBJECTIVE: This study was designed to further define the electroencephalographic abnormalities seen in the Landau-Kleffner syndrome variants and the associated clinical features. BACKGROUND: Landau-Kleffner syndrome is rare, but its putative variants are more common. METHOD: We report two patients with centro-temporal spikes, autistic epileptiform regression, and variably prominent oro-motor symptoms. RESULTS: The epileptic aphasia pattern found among patients with prominent Rolandic spikes may more frequently involve expressive language than is seen in the typical Landau-Kleffner syndrome, where verbal auditory agnosia is the rule. CONCLUSIONS: This clinical difference likely reflects the location of the epileptiform activity (centrotemporal as opposed to anterior or mid-temporal) on buccal-lingual function, vocalization, and language production
— id: 6181, year: 1999, vol: 12, page: 193, stat: Journal Article,

Outcome of multiple subpial transections for autistic epileptiform regression
Nass R; Gross A; Wisoff J; Devinsky O
1999 Jul;21(1):464-470, Pediatric neurology
Treatment options for atypical forms of Landau-Kleffner syndrome (LKS) are not well delineated. Many patients with typical LKS fail to respond to antiepileptic drug treatment, but some benefit from multiple subpial transections (MSTs). The authors report seven patients with autism or autistic epileptiform regression who responded in varying degrees to MSTs after failed medical management. These patients derived from an original cohort of 36 children (29 males, seven females, ranging from 2 years, 3 months to 11 years, 3 months, mean age = 5 years, 8 months) with a history of language delay or regression, as well as varying degrees of social and behavioral abnormalities, who were evaluated with video-electroencephalogram (EEG) monitoring over a 2-year period. Fifteen patients had clinical seizures (11 of the 19 children with autistic epileptiform regression and four of 12 autistic children). Epilepsy was refractory to medication in seven. Surgical treatment variously involved MSTs of the left neocortex in temporal, parietal, and frontal regions, often including regions within the classic perisylvian language areas. One patient also had a left temporal lobectomy. In all seven patients, seizure control or EEG improved after MSTs. Language, social, and overall behavior improved to a moderate degree, although improvements were temporary in most. Autistic epileptiform regression resembles LKS in that both may respond to MST. MST is used to treat epilepsy in eloquent regions. The responsiveness of autistic epileptiform regression to MST buttresses the argument that autistic epileptiform regression is a form of focal epilepsy
— id: 6167, year: 1999, vol: 21, page: 464, stat: Journal Article,

Moyamoya syndrome associated with congenital heart disease
Lutterman J; Scott M; Nass R; Geva T
1998 Jan;101(1 Pt 1):57-60, Pediatrics
OBJECTIVE. To describe the association between moyamoya syndrome and congenital heart disease and to discuss its clinical implications. Study Design. Retrospective analysis of a case series from two institutions. RESULTS. Five patients with moyamoya syndrome and structural congenital heart disease were identified. Coarctation of the aorta was present in 3 patients, in association with a ventricular septal defect (1 patient), aortic and mitral valve stenoses (1 patient), and tetralogy of Fallot (1 patient). Tetralogy of Fallot and a large paramembranous ventricular septal defect were found in the other 2 patients. Four patients underwent surgical repair of their congenital heart disease during the first year of life and 1 patient had balloon dilation of aortic coarctation at 5 years of age. In all patients, moyamoya syndrome was diagnosed after surgical intervention for congenital heart disease-at 6 months of age in 1 patient, at 2 years of age in 3 patients, and at 6 years in 1 patient. Strokes were the most common presenting sign (3 patients) followed by seizures (2 patients). By the age of 33 months, 4 of 5 patients had undergone cerebral revascularization surgery to halt the clinical progression of moyamoya syndrome. CONCLUSIONS. Moyamoya syndrome should be considered in the differential diagnosis of seizures and stroke in patients with structural congenital heart disease. Prompt diagnosis and surgical management of the occlusive cerebral angiopathy should lead to improved neurological outcome in these patients
— id: 28152, year: 1998, vol: 101, page: 57, stat: Journal Article,

Autism and autistic epileptiform regression with occipital spikes
Nass R; Gross A; Devinsky O
1998 Jul;40(7):453-458, Developmental medicine & child neurology
The electroencephalographic abnormalities seen in Landau-Kleffner syndrome (LKS) (language deterioration) are non-specific, and consist of a variety of epileptiform discharge patterns including continuous slow spike-wave discharges during sleep, focal sharp waves with spikes, and centrotemporal (rolandic) spikes. Similarly, the EEG abnormalities seen in autistic epileptiform regression (language and social/behavioral deterioration) are non-specific and overlap with those seen in LKS. By contrast, distinct epilepsy syndromes in otherwise normal children occur in the EEG-defined benign focal epilepsies of childhood. Occipital spikes or spike-wave present either in the older child with visual symptoms and headache or in the younger child with autonomic symptoms followed by brief or prolonged partial motor seizures. Seven young children (five from a consecutive series of 42) presenting clinically with autism or autistic regression and possible or definite seizures, whose EEGs revealed occipital spikes or spike-wave characteristic of the benign epilepsies, are reported. Although occipital spikes are commonly seen in young children as an age-dependent EEG-defined benign focal epilepsy, their high frequency in this population with cognitive difficulties suggests a possible causal relation. The effects of the epileptiform discharge on cognitive functioning presumably reflect extension into temporal and parietal lobes, rather than occipital disturbances per se
— id: 57214, year: 1998, vol: 40, page: 453, stat: Journal Article,

Conduction aphasia in a 3-year-old with a left posterior cortical/subcortical abscess
Nass R; Leventhal F; Levine B; Lebron D; Maxfield C; McCaul P; George A; Allen J
1998 Mar;62(1):70-88, Brain & language
A 3-year-old, right-handed girl developed a conduction-type aphasia following a second generalized seizure in the setting of a developing abscess involving left subcortical and cortical angular gyrus and arcuate fasciculus, and the posterior corpus callosum. The language disorder was fluent, characterized by age appropriate mean length of utterance and syntax, but with markedly reduced spontaneity of output, rapid rate of speech and mild dysarthria. Comprehension was relatively, but not completely spared. Naming, repetition, and reading (letters) were initially markedly impaired. Improvements in naming and repetition were associated with both literal and semantic paraphasias. Writing skills in the form of drawing were spared, but a mild apraxia to verbal command and imitation was initially present. Despite her young age, this child's fluent conduction aphasia and lesion localization were adult-like. Multimodal memory difficulties appeared to underlie what is best described as conduction aphasia
— id: 57215, year: 1998, vol: 62, page: 70, stat: Journal Article,

Temperament profiles of children with vein of Galen malformations
Nass R; Melnick J; Berenstein A
1998 Aug;13(8):387-390, Journal of child neurology
Unlike many brain injured children who are often rather difficult, toddlers and school-aged children with congenital vein of Galen malformations (n = 20; age range, 4 months to 12 years with a mean of 4 years) evidence a relatively positive temperament profile (based on the Temperament Scales of Carey, McDevitt, Fullard, Hegvik, Medoff-Cooper). They do not, however, differ from normal children with regard to the frequency of easy, difficult, slow to warm up, and intermediate temperament clusters. The temperament profile of children with vein of Galen malformations appears unaffected by additional neurologic abnormalities (hydrocephalus, abnormal developmental quotient, seizure disorder, focal cortical lesions) or other factors including age at testing, sex, or socioeconomic status. Contrary to the findings in adults of right hemisphere dominance for attention, presence of additional right-sided focal cortical pathology was not associated with greater activity level, distractibility, or impersistence. In contrast to the findings in adults and children of right hemisphere dominance for emotions in general, children with vein of Galen malformations and additional right hemisphere cortical damage are not more difficult than those with additional left cortical pathology or no additional focal cortical pathology. The fact that the pathology in vein of Galen malformations is subcortical may explain their relatively easier temperament and the absence of lateralization effects
— id: 7703, year: 1998, vol: 13, page: 387, stat: Journal Article,

Patterns of electroencephalographic abnormalities in autistic spectrum disorders: Correlation with clinical status and outcome
Nass, R; Gross, A; Devinsky, O
1998 SEP ;44(3):B56-B56, Annals of neurology
— id: 104278, year: 1998, vol: 44, page: B56, stat: Journal Article,

Autistic epileptiform regression: Response to multiple subpial resections
Nass, R; Gross, A; Wisoff, J; Devinsky, O
1998 SEP ;44(3):A24-A24, Annals of neurology
— id: 104277, year: 1998, vol: 44, page: A24, stat: Journal Article,

Language development in children with congenital strokes
Nass R
1997 Jun;4(2):109-116, Seminars in pediatric neurology
The congenital lesion population provides an excellent forum to investigate the issues of innate specialization and plasticity. Effects of early lesions on left and right hemisphere function reflect both the cognitive process under study and biological/neurological factors of lesion parameters and hemispheric maturation rates. In general, toddlers with both congenital left and right lesions show mild to moderate delays in language acquisition. School-age children with left hemisphere lesions have more problems with language and language-based academic skills than those with right hemisphere lesions, but the problems are subtle and do occur in both groups. This pattern stands in sharp contrast with the adult, who shows striking language deficits with acquired lesions only in the left perisylvian region. Thus, there is evidence in these studies of the immature nervous system for both innate specialization and plasticity, as well as a right hemisphere contribution to language acquisition and verbal cognition
— id: 56975, year: 1997, vol: 4, page: 109, stat: Journal Article,

Boys with Asperger's disorder, exceptional verbal intelligence, tics, and clumsiness
Nass R; Gutman R
1997 Oct;39(10):691-695, Developmental medicine & child neurology
Five boys with both Asperger's disorder and Tourette syndrome, exceptional verbal intelligence, and clumsiness are reported. Each presented at early elementary school age with a prominent complaint of social difficulties with peers. History was notable for a flapping stereotypy and the neurological examination revealed motor and/or vocal tics and numerous motor soft signs. Highly specialized interests were characteristics. Language prosody and/or pragmatics was impaired. Despite exceptional verbal intelligence, the children were not, according to their teachers and parents, faring well either socially or academically. Motor difficulties, manifested psychometrically as a significant performance IQ disadvantage, interfered with school performance and social adjustment. Tics, although not noted by parents in the clinical history, compounded their social difficulties. Asperger's disorder in these highly verbal children overlaps with pervasive developmental disorder (PDD) on account of the socioemotional difficulties and stereotypies seen in both. Asperger's disorder and Tourette syndrome overlap in these children on account of the tics. Finally, Asperger's disorder and the right-hemisphere-based learning disorders overlap on account of the visuoperceptual and attentional deficits that can occur in both
— id: 12240, year: 1997, vol: 39, page: 691, stat: Journal Article,

Magnetic resonance imaging in the congenital adrenal hyperplasia population: increased frequency of white-matter abnormalities and temporal lobe atrophy
Nass R; Heier L; Moshang T; Oberfield S; George A; New MI; Speiser PW
1997 Apr;12(3):181-186, Journal of child neurology
Congenital adrenal hyperplasia results from an adrenal enzyme deficiency, that causes an underproduction of glucocorticoids and sometimes mineralocorticoids and a resultant overproduction of androgens, until treatment with replacement glucocorticoids is instituted. The goal of this study was to determine the frequency and etiology of white-matter changes and temporal lobe atrophy demonstrable on magnetic resonance imaging (MRI) in a group of children and young adults with congenital adrenal hyperplasia. About one third of the patients evidenced white-matter abnormalities or temporal lobe atrophy. All patients, except one with a known stroke, had normal neurologic examinations. Exposure to excess exogenous glucocorticoids in the process of being treated for congenital adrenal hyperplasia is the most theoretically appealing explanation for these MRI findings. However, the relationship of MRI findings to treatment status (over-versus under-suppressed) does not run in clear parallel
— id: 7224, year: 1997, vol: 12, page: 181, stat: Journal Article,

The development of drawing in children with congenital focal brain injury: Evidence for limited functional recovery
Stiles, J; Trauner, D; Engel, M; Nass, R
1997 MAR ;35(3):299-312, Neuropsychologia
Children with pre- or perinatal injury to right hemisphere (RH) brain regions show impairment of spatial integrative functions similar to that observed among adults with comparable injury. Unlike adults, children show considerable improvement with development on a range of spatial construction tasks which require spatial integration. Such gains could reflect true recovery of spatial integrative abilities. Alternatively, the improvement could be more limited in scope, reflecting the development of compensatory strategies which are task specific and allow the children to circumvent, rather than overcome, their primary spatial disorders. The studies presented here examined this distinction within the context of drawing tasks in which the child was first asked to draw a house and then an impossible house. The impossible house task was designed to examine the extent to which children rely on graphic formulas in generating organized drawings. The results showed that while all of the children with RH injury make considerable progress in free drawing into the school age period, they are very reliant on the use of graphic formulas. When given a task which requires them to alter their drawings, they did not change the spatial configuration of the depicted object. Rather they found alternate ways to render the object 'impossible'. (C) 1997 Elsevier Science Ltd
— id: 53288, year: 1997, vol: 35, page: 299, stat: Journal Article,

Developmental stutter in a patient with callosal agenesis disappears during steroid therapy
Nass RD
1996 Sep;15(2):166-168, Pediatric neurology
A 10-year-old left-handed girl with a developmental stutter and agenesis of the corpus callosum with associated hydrocephalus ceased stuttering immediately upon initiation of steroid therapy for colitis. Steroid taper resulted in a recurrence of the stutter and resumption for treatment of recrudescent colitis caused its disappearance again. Baseline agenesis of the corpus callosum with hydrocephalus and the patient's course in the face of the known effects of steroids on white matter lend support to the hypothesis that stuttering reflects anomalous dominance and/or atypical interhemispheric connectivity, as evidenced by the fact that presumed alterations of white matter tracts affected speech rhythms/stuttering
— id: 12550, year: 1996, vol: 15, page: 166, stat: Journal Article,

Specific cognitive abilities in 2-year-old children with subependymal and mild intraventricular hemorrhage
Ross, G; Boatright, S; Auld, PAM; Nass, R
1996 OCT ;32(1):1-13, Brain & cognition
Subependymal and mild intraventricular hemorrhages (S/IVH) are likely to affect the subcortical and frontal cortex regions of the brain in premature infants. Damage to the subcortical and frontal areas has been associated with poorer performance in certain abilities, including visual attention, memory for location, and ability to change response set. This study investigated whether S/IVH, occurring at birth, affect these abilities in young children. Two-year-old premature children with S/IVH, premature children with normal neonatal head ultrasound scans, acid full term children with normal births were evaluated on a series of tasks. These tasks were a habituation/novelty preference task (visual attention), Piaget's invisible displacement task (memory for location), an object discrimination reversal task (ability to change response set), and the Bayley Scales of Infant Development (traditional infant test). Premature children with hemorrhage performed significantly less well on the task of ability to remember the last location of a hidden object and on the task of ability to reverse response set. Both groups of premature children performed significantly less well than full term children on systematic search for an object once the order of hiding was reversed. There were no significant differences among the three groups on the habituation/novelty preference task or on Bayley scores. Results suggest that both S/IVH and prematurity affect development of specific cognitive abilities. (C) 1996 Academic Press, Inc
— id: 98378, year: 1996, vol: 32, page: 1, stat: Journal Article,

Developmental change in spatial grouping activity among children with early focal brain injury: Evidence from a modeling task
Stiles, J; Stern, C; Trauner, D; Nass, R
1996 JUN ;31(1):46-62, Brain & cognition
Spatial construction skills were assessed in children with left (LH) or right (RH) hemisphere focal brain injury and control children. Children copied simple and complex block models which were rated on accuracy and spatial strategy. The accuracy of simple and complex constructions for 4- to 5-year-old children with LPI injury was indistinguishable from 3-year-old controls. However, although they were able to produce accurate complex constructions, the processes used by children with LH injury differed from those of normal children. On both simple and complex constructions, 4- to 5-year-old children with RH injury showed evidence of developmental delay. For both accuracy and process measures, children with RH injury performed at a level comparable to normal children at 3 years. A second group of children with LH and RH injury were tested at 5 to 6 years of age. Both lesion groups were indistinguishable from 4-year-old controls in terms of accuracy. However, both children with LH and RH injury used different spatial processes than did controls. This study emphasizes the importance of distinguishing between products of behavior and the processes which underlie them. It is in the dissociation of products and process of behavior that the subtle spatial construction deficits in this population of brain-injured subjects is revealed. (C) 1996 Academic Press, Inc
— id: 52877, year: 1996, vol: 31, page: 46, stat: Journal Article,

Etiologies of attention deficit hyperactivity disorder: Facts and myths
Nass R
1995 ;10(3):236-241, International pediatrics
— id: 8115, year: 1995, vol: 10, page: 236, stat: Journal Article,

Failure of steroid replacement to consistently normalize pituitary function in congenital adrenal hyperplasia: hormonal and MRI data
Speiser PW; Heier L; Serrat J; New MI; Nass R
1995 ;44(6):241-246, Hormone research
Exogenous glucocorticoid replacement in patients with congenital adrenal hyperplasia (CAH), who due to an adrenal 21-hydroxylase enzyme deficiency are unable to produce endogenous glucocorticoids, is aimed at normalizing hypothalamic-pituitary-adrenal function. Excess androgen production by the adrenals is thus decreased. Despite standard glucocorticoid replacement doses (12.5-40 mg, 10.5-27 mg/m2/day hydrocortisone equivalents) 4 of 7 patients ranging in age from 14 to 33 years had abnormalities of the pituitary on MRI. Three appeared to have microadenomas and 1 had an empty sella. Five (3 salt wasters, 2 simple virilizers) of these 7 patients had 60-min p.m. ovine corticotropin-releasing hormone (oCRH) stimulation studies. The mean (logarithm) area under the ACTH curve for 0-60 min after oCRH stimulation was significantly greater in patients than controls (p < 0.0001). Mean ACTH at each time point before and after oCRH stimulation was similarly greater in patients than controls (p < 0.05). Two of these patients had pituitary microadenomas, 1 had an empty sella; all 3 were salt wasters. Despite standard glucocorticoid replacement, adolescent and young adult patients with CAH tend to have high basal ACTH and ACTH hyperresponsiveness to oCRH, as well as structural abnormalities of the pituitary. The inevitable periods of under- and overexposure to glucocorticoids in CAH patients may over time cause abnormalities of the hypothalamic-pituitary-adrenal axis
— id: 6966, year: 1995, vol: 44, page: 241, stat: Journal Article,

Advances in learning disabilities
Nass R
1994 Apr;7(2):179-186, Current opinion in neurology
This review reports recent findings about diagnostic criteria, epidemiology, genetics, neuropsychological underpinnings, neuroanatomy, etiology, outcome, and treatment for the following: pervasive developmental disorder, the developmental language disorders, dyslexia, and dyscalculia. In addition, recent findings about neurological correlates, medical causes, and gender effects of learning disabilities are discussed
— id: 12986, year: 1994, vol: 7, page: 179, stat: Journal Article,

Acquired stuttering after a second stroke in a two-year-old
Nass R; Schreter B; Heier L
1994 Jan;36(1):73-78, Developmental medicine & child neurology
An almost two-year-old left-handed girl with a history of a left-hemisphere stroke at the age of one year developed a transient stutter with newly acquired white matter infarctions. Her course suggests that developmental stuttering may reflect anomalous dominance and/or atypical interhemispheric connectivity
— id: 57308, year: 1994, vol: 36, page: 73, stat: Journal Article,

Sex differences in learning abilities and disabilities
Nass RD
1993 ;43:61-77, Annals of dyslexia
Explores reasons why boys more often than girls are affected by cognitive developmental disorders of childhood. Differences in the etiology of learning disabilities, as well as sex differences in learning styles in boys vs girls may explain the male preponderance in the prevalence of learning disabilities. Complications of pregnancy and the newborn are more common in males. In addition, the effects of male hormones on the nervous system may explain some learning disabilities special to males. Males and females mature at different rates, early in life and later in puberty. These maturational differences could alter interhemispheric connections, again setting the stage and/or exaggerating some learning disabilities special to males.
— id: 8178, year: 1993, vol: 43, page: 61, stat: Journal Article,

LANDAU-KLEFFNER SYNDROME - TEMPORAL-LOBE TUMOR RESECTION RESULTS IN GOOD OUTCOME
NASS, R; HEIER, L; WALKER, R
1993 JUL-AUG ;9(4):303-305, Pediatric neurology
A child with a left temporal lobe tumor and Landau-Kleffner syndrome (acquired aphasia with a convulsive disorder) is reported because of his good postsurgical outcome
— id: 52251, year: 1993, vol: 9, page: 303, stat: Journal Article,

VISUAL-EVOKED POTENTIALS IN DYSLEXICS AND NORMALS - FAILURE TO FIND A DIFFERENCE IN TRANSIENT OR STEADY-STATE RESPONSES
VICTOR, JD; CONTE, MM; BURTON, L; NASS, RD
1993 SEP-OCT ;10(5):939-946, Visual neuroscience
We measured transient and steady-state checkerboard contrast-reversal visual evoked potentials (VEPs) in ten dyslexics, five patient controls, and 11 normals over a range of contrasts and luminances. Latency, amplitude, and phase measurements failed to distinguish the responses of dyslexics from those of normals or patient controls. Decreases in luminance or contrast resulted in an increased latency of the transient VEP in all groups, but these changes also did not distinguish the responses of dyslexics from those of the controls. Response variability was similar in dyslexics and normals, but was increased in subjects with attention deficit-hyperactivity disorder (ADHD). Performance on standardized psychometric testing did differentiate the dyslexics from controls, but did not correlate with VEP responses
— id: 52253, year: 1993, vol: 10, page: 939, stat: Journal Article,

Differential effects of congenital versus acquired unilateral brain injury on dichotic listening performance: evidence for sparing and asymmetric crowding
Nass R; Sadler AE; Sidtis JJ
1992 Oct;42(10):1960-1965, Neurology
We assessed dichotic speech and complex-pitch discrimination in nine young patients with unilateral left-hemisphere injury and eight young patients with unilateral right-hemisphere injury incurred in the pre-perinatal (congenital) period. As in adults with acquired unilateral lesions, both congenital lesion groups demonstrated poor performance on stimuli presented to the ear contralateral to the lesion. In overall performance on speech discrimination, however, the left-hemisphere congenital lesion group performed significantly better than the acquired-lesion group did. On complex-pitch discrimination, the right-hemisphere congenital lesion group performed significantly better than did the acquired-lesion group, but both left- and right-hemisphere congenital lesion groups were significantly worse at complex-pitch discrimination than were their age- and gender-matched normal controls. These results indicate that although congenital damage produces a 'lesion effect' in dichotic listening similar to that after damage acquired in adulthood, overall function is relatively spared. To the extent that complex-pitch discrimination is affected by congenital damage to either hemisphere but speech discrimination is not, the present results are consistent with an asymmetric form of crowding during reorganization after congenital unilateral brain damage
— id: 60854, year: 1992, vol: 42, page: 1960, stat: Journal Article,

The effects of precocious adrenarche on cognition and hemispheric specialization
Nass R; Baker S; Sadler AE; Sidtis JJ
1990 Sep;14(1):59-69, Brain & cognition
Cognitive skills were assessed in 13 females with a history of precocious adrenarche (PA). They were of average intelligence. In terms of lateralized cognitive skills, PA had no effect on verbal fluency. The spatial abilities of females with a history of PA, who had reached gonarche (were fully pubertal), were inferior to those of females tested in the midst of PA and to population controls. The physiologic/hormonal changes associated with normal adrenarche may curtail further specialization of the right hemisphere, resulting in a relative spatial deficit among females in general, who as a group reach adrenarche earlier than males. This spatial performance deficit is exaggerated in females with PA
— id: 60864, year: 1990, vol: 14, page: 59, stat: Journal Article,

Double disconnection effects resulting from infiltrating tumors
Sidtis JJ; Sadler AE; Nass RD
1989 ;27(11-12):1415-1420, Neuropsychologia
Patients with left hemisphere lesions deep in parietal or parietal-occipital regions close to the lateral ventricles have been reported to have impaired performance on left ear speech stimuli in dichotic listening tests. This loss has been termed 'paradoxical' because it presents at the ear ipsilateral to the lesion. Two patients with infiltrating tumors which involved the corpus callosum demonstrated that effect, but also demonstrated right ear extinction on a complex-pitch discrimination test that required right hemisphere processing. Since the side at which the impairment will be demonstrated depends upon the type of test used, the term 'paradoxical extinction' does not clearly describe this phenomena. It is suggested that the so-called paradoxical loss is better referred to as callosal extinction
— id: 60868, year: 1989, vol: 27, page: 1415, stat: Journal Article,

Dichotic complex-pitch and speech discrimination in 7- to 12-year-old children
Sidtis, John J; Sadler, Abigail E; Nass, Ruth D
1987 ;3(3-4):227-238, Developmental neuropsychology
Tested 43 right-handed children on dichotic complex-pitch and speech discrimination tests. For each age group (7 to 8 yrs, 9 to 10 yrs, and 11 to 12 yrs), adult patterns of perceptual asymmetries were observed: There was a right-ear advantage for speech and a left-ear advantage for complex-pitch, without significant changes either in the magnitude of perceptual asymmetries or in the frequency of occurrence of particular patterns of asymmetry as a function of age. For overall accuracy, a significant increase with age was found for speech but not for pitch discrimination. It is suggested that, despite the absences of significant age-related changes in perceptual asymmetries in these 2 tasks, they may undergo different developmental courses. Implications for cerebral specialization, cerebral lateralization, and perceptual asymmetry are noted.
— id: 28826, year: 1987, vol: 3, page: 227, stat: Journal Article,