Neurocognitive deficits in survivors of childhood acute myeloid leukemia.

BACKGROUND: Although treatment of acute myeloid leukemia (AML) contains neurotoxic agents, studies investigating neurocognitive outcomes in children with AML are sparse. We evaluated late cognitive effects in children treated with a high-dose cytarabine based regimen, focusing on general intellectual ability and specific neurocognitive domains. METHODS: We evaluated 12 survivors of childhood AML who were treated between 2006 and 2016 and completed the Wechsler Intelligence Scales. One-sample t-tests were used to compare full-scale intelligence quotient (FSIQ) and primary index scores to norms. The overall effect of index scores and subtests was examined with one-way ANOVA. Univariate analyses and multiple regression models examined demographic and clinical characteristics associated with FSIQ. RESULTS: Participants who underwent the Wechsler Intelligence Scale for Children demonstrated impairment on working memory index and participants who underwent the Wechsler Adult Intelligence Scale showed low score in the subtests that reflect working memory, whereas they exhibited no statistical differences versus the population means for FSIQ. There were no significant differences in the overall effect of index scores and subtests. On univariate analysis, FSIQ were related to time since diagnosis and age at assessment, and both were significant predictors of FSIQ on multiple linear regression. CONCLUSIONS: Survivors of childhood AML exhibited impairment of working memory, even if their FSIQ was within the normal range. Difficulties in specific cognitive domains are associated with reduced quality of life. It is important to identify survivors who are at risk and provide tailored interventions.

Cranial molding helmet therapy and establishment of practical criteria for management in Asian infant positional head deformity.

BACKGROUND: The growing number of infants with deformational plagiocephaly (DP) has raised clinical questions about which children, at what age, and how molding helmet therapy (MHT) should be performed especially in Japan. METHODS: A total of 1,011 Japanese pediatric head deformity infants had undergone MHT after being diagnosed with non-synostotic DP. Three ratios of left to right comparison (anterior, posterior, and overall) were created and analyzed comparing age of starting treatment, helmet wearing period, and severity of skull deformity before with after MHT. RESULTS: The averages of head symmetry ratios after treatment in all groups (for the occipital region) showed apparent improvement; t(930) = -60.86, p = 0.000. (t(932) = -57.8, p = 0.000.) In the "severe" deformation group, the earlier the treatment was started, the higher symmetry ratio recovery was obtained. Treatment was especially effective when started in 4-month-old infants. In contrast to the "severe" group, the "mild" deformation group showed that MHT was most effective if treatment started before 6 months of age. Again, the earlier the treatment was started, the higher symmetry ratio was achieved, but compared to the "severe" group, it had a modest effect when treatment was started in infants older than 8 months. CONCLUSION: This is the first large-scale molding helmet study reporting the method and efficacy in Japanese infants. It demonstrated that despite the structural and physiological differences from infants of other races, molding helmet therapy is effective in Asian-born infants, provided that intervention timing and recognition conditions are met.

Placental alkaline phosphatase levels in cerebrospinal fluid can have a decisive role in the differential diagnosis of intracranial germ cell tumors.

OBJECTIVE: Placental alkaline phosphatase (PLAP) in CSF can provide a very high diagnostic value in cases of intracranial germ cell tumors (GCTs), especially in pure germinomas, to the level of not requiring histological confirmation. Unlike other tumor markers, reliable data analysis with respect to the diagnostic value of PLAP serum or CSF levels has not been available until now. This is the first systematic and comprehensive study examining the diagnostic value of CSF PLAP in patients with intracranial GCTs. METHODS: From 2004 to 2014, 74 patients (average age 19.6 ± 10.6 years) with intracranial GCTs were evaluated using PLAP from their CSF and histological samples. Chemiluminescent enzyme immunoassay was utilized to measure CSF PLAP in the following tumor sites: pineal (n = 32), pituitary stalk, suprasellar (n = 16), basal ganglia (n = 15), intraventricular (n = 9), and cerebellar (n = 5) regions. In addition to classifying GCT cases, all patients underwent tumor biopsy for correlation with tumor marker data. RESULTS: PLAP in combination with other tumor markers resulted in extremely high sensitivity and specificity of the diagnostic value of intracranial GCTs. Intracranial GCT cases were classified into 1) germinomas, both "pure" and syncytiotrophoblastic giant cell types (n = 38); 2) nongerminomatous GCTs, choriocarcinomas (n = 9) and teratomas (n = 4); and 3) nongerminomas, other kinds of tumors (n = 23). Consequently, all patients received chemoradiation therapy based on elevation of PLAP and the histopathological results. It was also speculated that the level of PLAP could show the amount of intracranial germ cell components of a GCT. PLAP was 100% upregulated in all intracranial germinoma cases. The absence of CSF PLAP proved that the tumor was not a germinoma. CONCLUSIONS: The current study is the first systematic and comprehensive examination of the diagnostic value of the tumor marker PLAP in pediatric patients with intracranial GCT. Using the level of PLAP in CSF, we were able to detect the instances of intracranial germinoma with very high reliability, equivalent to a pathological diagnosis.

'Autistic' traits in non-autistic Japanese populations: relationships with personality traits and cognitive ability.

We explored the relationships between 'autistic' traits as measured by the AQ (Autism-Spectrum Quotient; Baron-Cohen et al., J. Autism Develop. Disord. (2001b) 31 5) and various personality traits or cognitive ability, which usually coincide with autistic symptoms, for general populations. Results showed the AQ was associated with tendencies toward an obsessional personality as defined by the TCI (Temperament and Character Inventory), higher depression and anxiety, and higher frequency of experience of being bullied. These results parallel the patterns in autism and corroborate the validity of the AQ for general populations. Contrary to our prediction, however, there was no relationship between the AQ and cognitive ability, such as theory of mind, executive functioning, and central coherence, suggesting the AQ does not reflect autism-specific cognitive patterns in general populations.

[Reading mind from pictures of eyes: theory of mind, language ability, general intellectual ability, and autism].

Some researchers have claimed that theory of mind or 'mind reading' ability, necessary for inferring the mental states of others, depends on language ability. However, previous tests of theory of mind were verbally demanding, and high correlations found might have been an artifact. This paper reports the performance of 22 children with autism, with varying degrees of dysfunction, on a test devised by Baron-Cohen, Jolliffe, Mortimore, and Robertson (1997), based on a relatively nonverbal theory of mind. The test involved inference of mental states from photographs of human eyes. Results indicated that mind reading ability was independent of language ability, general intelligence, or mental age. Test scores were, however, strongly related to the severity of autistic disorder. These results suggest both the independence of mind-reading ability from language ability and general intelligence, and a strong relationship between theory of mind deficit and the autistic disorder.

Reflexive orienting in response to eye gaze and an arrow in children with and without autism.

BACKGROUND: This study investigated whether another person's social attention, specifically the direction of their eye gaze, and a non-social directional cue, an arrow, triggered reflexive orienting in children with and without autism in an experimental situation. METHODS: Children with autism and typically developed children participated in one of two experiments. Both experiments involved the localization of a target that appeared to the left or right of the fixation point. Before the target appeared, the participant's attention was cued to the left or right by either an arrow or the direction of eye gaze on a computerized face. RESULTS: Children with autism were slower to respond, which suggests a slight difference in the general cognitive ability of the groups. In Experiment 1, although the participants were instructed to disregard the cue and the target was correctly cued in only 50% of the trials, both groups of children responded significantly faster to cued targets than to uncued targets, regardless of the cue. In Experiment 2, children were instructed to attend to the direction opposite that of the cues and the target was correctly cued in only 20% of the trials. Typically developed children located targets cued by eye gaze more quickly, while the arrow cue did not trigger such reflexive orienting in these children. However, both social and non-social cues shifted attention to the cued location in children with autism. CONCLUSION: These results indicate that eye gaze attracted attention more effectively than the arrow in typically developed children, while children with autism shifted their attention equally in response to eye gaze and arrow direction, failing to show preferential sensitivity to the social cue. Difficulty in shifting controlled attention to the instructed side was also found in children with autism.