Susan Curtiss

Cerebral hemispherectomy Hospital course, seizure, developmental, language, and motor outcomes

Objective: To compare hemispherectomy patients with different pathologic substrates for hospital course, seizure, developmental, language, and motor outcomes. Methods: The authors compared hemispherectomy patients (n = 115) with hemimegalencephaly (HME; n = 16), hemispheric cortical dysplasia (hemi CD; n = 39), Rasmussen encephalitis (RE; n = 21), infarct/ischemia (n = 27), and other/miscellaneous (n = 12) for differences in operative management, postsurgery seizure control, and antiepilepsy drug (AED) usage. In addition, Vineland Adaptive Behavior Scale (VABS) developmental quotients (DQ), language, and motor assessments were performed pre- or postsurgery, or both. Results: Surgically, HME patients had the greatest perioperative blood loss, and the longest surgery time. Fewer HME patients were seizure free or not taking AEDs 1 to 5 years postsurgery, but the differences between pathologic groups were not significant. Postsurgery, 66% of HME patients had little or no language and worse motor scores in the paretic limbs. By contrast, 40 to 50% of hemi CD children showed near normal language and motor assessments, similar to RE and infarct/ischemia cases. VABS DQ scores showed +5 points or more improvement postsurgery in 57% of patients, and hemi CD (+12.7) and HME (+9.1) children showed the most progress compared with RE (+4.6) and infarct/ischemia (−0.6) cases. Postsurgery VABS DQ scores correlated with seizure duration, seizure control, and presurgery DQ scores. Conclusions: The pathologic substrate predicted pre- and postsurgery differences in outcomes, with hemimegalencephaly (but not hemispheric cortical dysplasia) patients doing worse in several domains. Furthermore, shorter seizure durations, seizure control, and greater presurgery developmental quotients predicted better postsurgery developmental quotients in all patients, irrespective of pathology.

The development of language in genie: a case of language acquisition beyond the “critical period” ☆ ☆☆

Abstract The present paper reports on a case of a now-16-year-old girl who for most of her life suffered an extreme degree of social isolation and experiential deprivation. It summarizes her language acquisition which is occurring past the hypothesized “critical period” and the implications of this language development as related to hemispheric maturation and the development of lateralization. The results of a series of dichotic listening tests administered to her are included.

Phenotypic profiles of language-impaired children based on genetic/family history☆

Although etiological influences in developmental language impairment (dysphasia) are not well defined, a significant increase of family aggregation for the disorder has been reported. We report data from a large cohort of language-impaired (LI) children participating in the San Diego longitudinal study in which we examined whether children with or without positive family histories show different phenotypic profiles. Due to the longitudinal design of the study, questions pertaining to change over time are also addressed. Second, a subgroup of the most impaired children were reevaluated to obtain additional information pertaining to family history and phenotypic outcome. Approximately 70% of the LI children met criteria for inclusion as family history positive, with fathers reporting a history of language or learning problems one and a half to two times as frequently as mothers. LI children with or without a positive family history were not significantly different on language skills or IQ. However, subjects having a positive family history for developmental language/learning problems were significantly lower in socioeconomic status and were rated by parents and teachers as having more attention-related behavior problems than their family history negative counterparts. Similarly, family history positive LI children performed more poorly on standardized academic tests as well as on tests of auditory processing and attention.

Unexpected sex-ratios in families of language/learning-impaired children.

There is a well-documented propensity of males affected with developmental language/learning impairment. Results from this study demonstrate, unexpectedly, that this sex-ratio difference of males to females with developmental language/learning disorders was found to occur significantly only in families with a language/learning-impaired mother. In addition, a remarkably aberrant offspring sex-ratio was found in families of language/learning-impaired children who had an affected mother, but not father. Mothers who were developmentally language/learning-impaired had three times as many sons as daughters, and five times as many language/learning-impaired sons as daughters. Genetic and hormonal influences that might affect both sex-ratio and neuroanatomical development and disorders are discussed.

Rapid automatized naming and gesture by normal and language-impaired children.

This study investigates whether language-impaired (LI) children show deficits in rapid automatized naming and whether RAN performance is specific to verbal output (or to rapid motor output in general). A total of 67 LI and 54 age-matched control children were tested with the Rapid Automatized Naming (RAN) test (Denckla & Rudel, 1976) and with a manual version of the RAN (RAN-manual) in which subjects were required to provide a nonverbal, pantomime response. Subjects also completed tests of rapid oral and manual sequencing skills and standardized tests of reading ability. Each subject was tested at 4, 6, and 8 years old. The results showed that LI children perform significantly poorer on both versions of the RAN than age-matched controls. Correlations between RAN scores and tests of reading ability were significant for normal and LI subjects and were particularly high for 8-year-old LI children. RAN-manual scores also correlated with 8-year-old LI childrens reading scores. Further, RAN and RAN-manual scores for the LI children correlated significantly with these childrens manual sequencing abilities, whereas this was not the case for the control subjects. These findings suggest that LI childrens rapid sequential processing deficits are not limited to verbal output, but also generalize to other motoric domains.

Spoken language outcomes after hemispherectomy: factoring in etiology.

We analyzed postsurgery linguistic outcomes of 43 hemispherectomy patients operated on at UCLA. We rated spoken language (Spoken Language Rank, SLR) on a scale from 0 (no language) to 6 (mature grammar) and examined the effects of side of resection/damage, age at surgery/seizure onset, seizure control postsurgery, and etiology on language development. Etiology was defined as developmental (cortical dysplasia and prenatal stroke) and acquired pathology (Rasmussens encephalitis and postnatal stroke). We found that clinical variables were predictive of language outcomes only when they were considered within distinct etiology groups. Specifically, children with developmental etiologies had lower SLRs than those with acquired pathologies (p =.0006); age factors correlated positively with higher SLRs only for children with acquired etiologies (p =.0006); right-sided resections led to higher SLRs only for the acquired group (p =.0008); and postsurgery seizure control correlated positively with SLR only for those with developmental etiologies (p =.0047). We argue that the variables considered are not independent predictors of spoken language outcome posthemispherectomy but should be viewed instead as characteristics of etiology.

Dissociations Between Language and Cognition: Cases and Implications

An important issue for the cognitive sciences is whether grammar is to any nontrivial extent an autonomous cognitive system. Current cognitive hypotheses of language acquisition would argue against an autonomous linguistic system and would support the notion that language emerges from more general cognitive knowledge and is throughout its development fundamentally tied to a nonlinguistic cognitive base. This paper explores this issue and presents data from case studies of children showing clear dissociations between language and nonlanguage cognitive abilities. The implications of such data are discussed. The major implications appear to be that lexical and relational semantic abilities are deeply linked to broader conceptual development but morphological and syntactic abilities are not. The development of a normal linguistic system, however, one in which grammar is systematically related to meaning, requires concurrent and concomitant linguistic and nonlingustic cognitive development.

Age and Etiology as Predictors of Language Outcome following Hemispherectomy

We report on the effects of etiology and age on the linguistic outcomes in a large pediatric hemispherectomy population. Four populations were considered separately: cortical dysplasia (multilobar involvement), Rasmussen’s encephalitis, infarction as a primary etiology and, fourth, children who failed to develop language, regardless of etiology. We argue against the ‘the-earlier-the-better’ hypothesis and propose our own hypothesis that weds maturational factors to etiological factors to predict language outcomes following pervasive brain insult. The implications of our ‘critical impact point’ hypothesis are discussed.

Developmental outcome with and without successful intervention

A review of the literature on the developmental outcome of medically and surgically treated symptomatic infantile spasms (IS) indicates that poor seizure control, severe mental retardation, and marked behavioral disorders are found at long-term outcome of symptomatic IS. The 2-year outcome findings of the UCLA Pediatric Epilepsy Surgery Research Group in children with symptomatic infantile spasms and in children with early onset intractable symptomatic epilepsy other than IS (non-IS) demonstrate impaired development of cognition, language, and social communication despite improved seizure control. The social communication deficits of these children are similar to those found in autistic children. Finally, the underlying pathology of the resected brain, rather than ongoing seizures and seizure type, plays an important role in development of the remaining brain tissue and the outcome of early onset symptomatic IS and non-IS.

Grammatical comprehension, aphasic syndromes and neuroimaging

Abstract This paper presents grammatical comprehension, computerized tomography (CT) and positron emission tomography (PET) data from a group of 43 aphasic patients. Comparison of comprehension performance with structural lesion (CT) revealed a correlation between comprehension of syntax and one inferior frontal lobe region (Brocas area) and one temporal lobe region (Wernickes area). In contrast, comparison between comprehension performance and metabolic data demonstrated strong correlations between morphology and syntax comprehension and regional measures throughout the temporal, parietal and occipital lobes. The second aspect of the study looked at performance differences between diagnostic groups of aphasic patients. Very few behavioral differences were observed between aphasic groups (Brocas, Anomic, Conduction and Wernickes), other than differences in severity. On all measures, Brocas and Wernickes patients performed similarly. Overall, the findings support a model of brain—behavior relations in which language comprehension is represented by more widespread regions of the left hemisphere than is traditionally thought, and the aphasie syndromes differ from each other less than is traditionally believed.