Kiyoko Watanabe

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.

Combined Treatment of Steroids and Cyclosporine in Kimura Disease

Kimura disease is a rare but distinctive chronic eosinophilic inflammatory disorder that is characterized by tumor-like lesions in the soft tissue and lymph nodes of the head and neck or parotid gland. Recently, many immunopathogenetic features of underlying T lymphocytes and related cytokines have been noted in Kimura disease. However, few previous studies have investigated the serial levels of cytokines in children. In this report we describe an 11-year-old Japanese boy with relapsing Kimura disease. Before the diagnosis of Kimura disease, the patient had a swelling on his left neck. Steroids were effective, but the tumor relapsed within a few months as the steroids were tapered. He was treated with steroids and cyclosporine. This treatment was done by measuring serial levels of serum soluble interleukin-2 receptor, interleukin-4, interleukin-5, and eosinophil cationic protein. These results suggest the activation of T-helper cells and T-helper 2 cytokines, that after activated B cells and eosinophilic infiltration play an important role in Kimura disease, and that cyclosporine suppresses the activity of this disease.

Predicting executive function task scores with the Rey-Osterrieth Complex Figure.

The Boston Qualitative Scoring System (BQSS) is one of the scoring methods of the Rey-Osterrieth Complex Figure Test (ROCF). With BQSS, 17 qualitative ratings are generated, and subsequently 6 summary scores are calculated by combining several qualitative scores. Previously we showed that 5 of 6 BQSS summary scores were correlated with the scores of the several executive function tests in children. The objective of this study was to develop a new summary score which correlates with the executive function test scores more strongly than the existing summary scores. For this purpose, we conducted multiple regression analysis to predict PEN, CA, DSM of the Wisconsin Card Sorting Test Keio Version, and the Mazes scores from WISC-III, by the BQSS qualitative scores derived from ROCF drawings in copy condition. The subjects were 78 children with various neuropsychological disorders (5 years 5 months-14 years 11 months; mean: 9 years 2 months; F 22, M 56). Significant predictive models were generated for PEN, CA, and Mazes scores. Among them the models for the Mazes scores were the most accurate. The second model for the Mazes scores was most suitable for a new summary score. Its degree-of-freedom-adjusted coefficient of multiple determination and multiple correlation coefficient reached 0.467 and 0.695, respectively. A new summary score should be applied in future studies to evaluate its clinical usefulness.

Executive function in children with pervasive developmental disorder and attention-deficit/hyperactivity disorder assessed by the Keio version of the Wisconsin card sorting test

The Wisconsin card sorting test (WCST) comprehensively examines executive function (EF). The Keio version of the WCST (KWCST) uses fewer cards and presents them in two steps, separated by a short pause during which an instruction is given. Being of short duration, this test is suitable for children with pervasive developmental disorder (PDD) and attention-deficit/hyperactivity disorder (AD/HD), yet few studies have examined the performance of children with such developmental disorders in the second step of a two-step test such as the KWCST. Using the KWCST, this study compares EF in children with PDD (n=52), or AD/HD (n=46) to that in typically developing (TD) children (n=52). Scores for the six indices of this test, including numbers of response cards until the first category achieved (NUCA), total errors (TE), and non-perseverative errors of Nelson (NPEN), were analyzed using ANOVA. Compared to the TD group, scores in the PDD and/or AD/HD groups were significantly lower for all indices except NUCA and NPEN for the first step, and lower for all indices except NUCA for the second step. Moreover, significantly fewer improvements in TE were seen in the PDD group, and significantly fewer improvements in NPEN were seen in the AD/HD group, compared with TD. This study suggests that both PDD and AD/HD make it difficult for children to utilize their experience in the first step and to effectively apply the instruction given before the second step. It also suggests that the two-step nature of the KWCST is clinically important.

A case report of neonatal onset multisystemic inflammatory disease in Japan treated with continuous hemodiafiltration and steroid pulse therapy.

Abstract:  Neonatal onset multisystem inflammatory disease (NOMID), which is also known as chronic infantile neurological cutaneous and articular syndrome, is a rare disease that is characterized by the triad of cutaneous rash, chronic meningitis and arthropathy. The long‐term prognosis is poor, with progressive deafness and visual impairment, and worsening of the central nervous system manifestations. Some fatal cases have been reported secondary to infection, vasculitis and amyloidosis. We recently managed a typical case of NOMID where the combined treatment of continuous hemodiafiltration and steroid pulse therapy was effective. The patient showed repeated flare ups of fever, arthralgia and meningitis accompanied with high levels of cytokines. The effect was temporary, but useful in recovery from such a serious condition.

Reading skills of Japanese second-graders.

Background:  A few studies have explored the prevalence of dyslexia among children who speak Japanese as their native language by evaluating them individually by means of reading‐based tasks. The present study was designed to clarify the frequency of suspected dyslexia among second‐graders attending ordinary classes.

Bilaterally independent epileptic spasms in a case of Aicardi syndrome

A girl with Aicardi syndrome was observed to have two distinct types of asymmetric epileptic spasms, as detected by ictal video-EEG recording at three months of age. When the two types of spasm concurred, they showed no mutual interactions based on either clinical or EEG aspects. This observation does not support the hypothesis that the brainstem always plays an initiating role in generating spasms.

Cryptogenic West syndrome and subsequent mesial temporal lobe epilepsy

We report on a male patient who experienced a previously unreported sequence of cryptogenic West syndrome in infancy and subsequent mesial temporal lobe epilepsy. His complex partial seizures were consistently characterised by motionless staring with brief right eye blinking. Scalp electroencephalography (EEG) showed bilateral temporal spikes which were dominant on the right side. Magnetic resonance imaging (MRI) revealed no organic brain lesion. Invasive EEG recording captured seizures with right hippocampal onset. The patient became seizure-free following right temporal lobectomy at 27 years, 8 months of age. Pathological examination of the resected specimen revealed corpora amylacea and gliosis in the temporal cortex but no clear findings of hippocampal sclerosis. It is suggested that an epileptogenic lesion causing MRI-negative mesial temporal lobe epilepsy may give rise to apparent cryptogenic West syndrome in infancy.

135. Bilateral independent ictal EEG activity in a patient with Aicardi syndrome

Subclinical rhythmic electrographic discharges of adults (SREDA) is a rare pattern reported in fewer than 0.05% of patients. We documented SREDA-like discharges in a patient with Parkinson’s disease (PD). A 59-year-old woman was admitted to our hospital due to slowly progressive motor disturbance of her left arm. On examination, she had left-dominant rigidity without autonomic and cognitive dysfunctions. Brain MRI showed a mild diffuse cerebral atrophy and SPECT revealed the hypoperfusion in the right thalamus and bifrontal areas. Her symptoms improved by L-DOPA, and thus we diagnosed her with PD. She underwent EEGs twice. In both tests, we found the repetitive discharges characterized by an abrupt onset during hyperventilation: occipital dominant theta waves preceded by delta waves appeared and evolved repeatedly, lasting 4–5 min. While these discharges were observed, her consciousness was alert and she had no symptoms. These discharges seemed to be SREDA, however, they lasted for long time and showed slower frequencies.