Junri Hattori

Are pervasive developmental disorders and attention-deficit/hyperactivity disorder distinct disorders?

We studied the relationship between patients with attention-deficit/hyperactivity disorder (ADHD) and those with pervasive developmental disorders (PDD), using the High-Functioning Autism Spectrum Screening Questionnaire (ASSQ) and ADHD Rating Scale-IV. The ASSQ scores of the PDD group and the ADHD group were significantly higher than the control group. Furthermore, the PDD group scored higher than the ADHD group. Both groups also showed higher scores than the control group in all three domains, that is, restricted and repetitive behavior, social interaction, and communication problem. The PDD and the ADHD group showed no significant difference in the domains of communication problem, and restricted and repetitive behavior. The PDD group had a higher score than the ADHD group only in the social interaction domain. In total score, inattention score, and hyperactivity/impulsivity score on the ADHD Rating Scale-IV, both groups were significantly higher than the control group. Between the ADHD and the PDD groups, there was no significant difference in the three scores. The patients with strictly diagnosed ADHD had many PDD-related symptoms, and the patients with PDD had many ADHD-related symptoms. It therefore seems difficult to make a distinction between ADHD and PDD by using the present diagnostic criteria in the DSM-IV. We should evaluate each patient in terms of both sets of criteria.

The Rey-Osterrieth Complex Figure as a Measure of Executive Function in Childhood

In adults, the Rey-Osterrieth Complex Figure (ROCF) can be used for the assessment of not only visuoconstructional ability and visual memory, but also executive function in adults. We studied whether ROCF scores also correlated with executive function in childhood. The subjects consisted of 56 patients with various neurological diseases (5 years 7 months-14 years 11 months; mean: 8 years 8 months; M 42, F 14) whose full-scale IQs were 70 or higher (mean: 93.3). All subjects underwent the Wechsler Intelligence Scale for children third edition (WISC-III) and various psychological tests focusing on executive function. We evaluated the ROCF with the Boston Qualitative Scoring System (BQSS). We calculated the partial correlation coefficients between the BQSS Summary Scores and representative scores of other executive function tests, using age as the control variable. Among the scores of the various examinations, the Perseverative Errors of Nelson of the Wisconsin Card Sorting Test, the raw scores of the Mazes (WISC-III), the Digit Span (WISC-III), and the Block Design (WISC-III) were correlated significantly with two to four BQSS Summary Scores (P<0.05 or P<0.01). In contrast, the scores of the Trail Making Test, the Stroop Test, and the Commission Error of Continuous Performance Test-II did not show a significant correlation with any of the BQSS Summary Scores. The ROCF evaluated with the BQSS reflects not only visuoperceptual ability and visuoconstructional ability, but also executive function, especially planning and organization. However, a new BQSS Summary Score should be devised since no existing BQSS index specifically reflects executive functions with an outstandingly strong correlation.

A screening test for the prediction of Dravet syndrome before one year of age.

Purpose: Our aim was to develop a screening test to predict Dravet syndrome before the first birthday based on the clinical characteristics of infants and the SCN1A mutation analysis.

Long-term follow-up of vitamin B6-responsive West syndrome

We performed a clinical and electroencephalographic follow-up study on 25 patients with West syndrome that was responsive to vitamin B(6) (eight cryptogenic patients and 17 symptomatic patients) who were older than 3 years at the last follow-up. All cryptogenic patients and 13 symptomatic patients were seizure free at the last follow-up. All cryptogenic patients and seven symptomatic patients had intelligent quotient or developmental quotient scores of 75 or higher. The recurrence of clinical seizures was always associated with increases in epileptic discharges. We could successfully discontinue pyridoxal phosphate administration in four cryptogenic and four symptomatic patients who were 1 year, 8 months to 24 years old.

Symptoms related to ADHD observed in patients with pervasive developmental disorder.

To elucidate attention-deficit/hyperactivity disorder (ADHD)-related factors observed in high-function pervasive developmental disorder (PDD) and their impact on daily life, we classified high-function PDD patients according to the Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition (DSM-IV) criteria for ADHD with the exception of the exclusion criteria (coexistence with PDD), and studied the relationship between ADHD-related aspects and daily behavior problems using the Child Behavior Checklist (CBCL). The subjects were divided into two groups: Group 1, eight patients less than 6 years of age; and Group II, eight patients 6 years of age and older. Six patients in Group II met the criteria for ADHD. Five of them were classified as having the predominantly inattentive type of ADHD, and the remaining patient exhibited the combined type. However, no patient in Group I met the criteria for ADHD, suggesting the low sensitivity of the DSM-IV criteria for assessing the inattentiveness of preschoolers. The analyses of the correlation between corresponding items in the DSM-IV criteria and CBCL scores suggested that ADHD-related symptoms in high-function PDD patients have an impact on their daily lives.

Photosensitive fits elicited by TV animation: An electroencephalographic study

Background: We carried out a clinical and electroencephalographic (EEG) study to reveal the pathophysiology of acute symptoms elicited by a TV animation program.

Utility of the scalp-recorded ictal EEG in childhood epilepsy.

Summary:  Purpose: To evaluate the usefulness of the scalp‐recorded ictal EEGs in diagnosing childhood epilepsy.

Long-term follow-up of an individual with vitamin B6-dependent seizures.

We report on a 31-year-old female with vitamin B6-dependent seizures whose seizure onset was in the neonatal period. Her elder brother had the same disorder and died in infancy. Administration of vitamin B6 was initiated in the postnatal period. At the age of 12 years 1 month, 2 months after withdrawal of vitamin B6, visual seizures began to occur frequently. Myoclonic seizures and occasional generalized convulsive seizures were also observed. At the same time, photoparoxysmal response and spontaneous diffuse spike-wave bursts were seen on her EEG. Myoclonic seizures were provoked by intermittent photic stimulation during the EEG. It is distinctive that visual seizures were one of the main seizure types in this patient, that her clinical course was relatively benign, and that she has normal intellectual outcome.

A developmental study of scores of the Boston Qualitative Scoring System

To elucidate developmental changes of the Summary Scores of the Boston Qualitative Scoring System (BQSS) for the Rey-Osterrieth Complex Figure (ROCF). One hundred healthy children aged 6-16 (average 9.7 +/- 2.4; 60 boys, 40 girls). The ROCF was administered and graded based on the BQSS. The subjects were classified in four age-groups: 6- and 7-year-olds; 8- and 9-year-olds; 10- and 11-year-olds; and 12- and 16-year-olds. The differences in Summary Scores were examined among age-groups. All BQSS Summary Scores except Delayed Retention showed clear developmental changes. The Copy Presence Accuracy, the Immediate Presence Accuracy, the Delayed Presence Accuracy, and the Immediate Retention showed continuous development throughout childhood, though some differences were noted in the age ranges during which each score showed the most rapid development. The Organization score showed a somewhat peculiar pattern, with rapid development during the age ranges of 8-9 and 10-11 and with no distinctive development before and after these ranges. Five of the six BQSS Summary Scores showed clear developmental changes with a different developmental pattern in each score. Especially, the Organization score was unique and was supposed to reflect a different underlying process than the other Summary Scores, which might relate to the local and global processing style. BQSS is a promising tool for the evaluation of higher brain functions in childhood.

Combined use of sphenoidal electrodes and the dipole localization method for the identification of the mesial temporal focus

We attempted to sub‐classify four cases who show temporal spikes on standard scalp electroencephalogram (EEG), using sphenoidal electrodes and the dipole localization method. In a case with mesial temporal epilepsy, spikes showed phase reversal in a sphenoidal electrode, and the spike dipoles were estimated to be in the mesial temporal lobe. In a case with lateral temporal epilepsy, spikes showed no phase reversal in a sphenoidal electrode, and the spike dipoles were estimated to be in the lateral temporal lobe. In two cases out of four, spikes showed phase reversal in sphenoidal electrodes, whilst the dipoles were estimated to be in the frontal lobe. Clinical features also suggested a diagnosis of frontal lobe epilepsy. In one of the two cases in which frontal lobe epilepsy was suspected, ictal dipoles as well as interictal spike dipoles indicated participation of the frontal lobe in the genesis of seizures. Nevertheless, only mesial temporal lobectomy was performed based on results obtained by invasive subdural electrodes. As a result, seizures were not controlled. Although sphenoidal electrodes were useful for differentiating between mesial and lateral temporal lobe foci, it is advisable to use them in combination with the dipole localization method to identify frontal lobe foci.