Hiroyuki Takada

Epileptic seizures induced by animated cartoon, "Pocket Monster".

Summary: Purpose: A large number of children had fits while watching the animated cartoon television (TV) program “Pocket Monster.” To elucidate the seizures associated with the TV program, we administered a questionnaire survey in Aichi Prefecture, Japan.

Prognostic value of positron emission tomography in cryptogenic West syndrome

The relationship between positron emission tomography (PET) findings and developmental or seizure outcome was examined in 17 infants (11 males, six females; mean age at onset of spasms 7 months, range 3 to 26 months) with newly diagnosed cryptogenic West syndrome. The predictive value of PET in these infants was assessed. PET was performed in the infants at the onset of spasms and 3 months after initial therapy using 18F‐labelled 2‐deoxy‐2‐fluoro‐D‐glucose. A third PET was performed at 18 months of age if the second scan was abnormal. All infants were followed up until at least 3 years of age. Cortical hypometabolism was detected in 11 infants on the first PET and in five infants on the second. Rate of developmental delay at the last follow‐up was significantly higher in infants with hypometabolism on the second PET than in those without PET abnormalities (p<0.05). Rate of seizure occurrence after initial treatment was higher in infants with cortical hypometabolism on the second PET, but the difference was not statistically significant. Results suggest that when PET after the initial treatment shows no abnormalities, even though the first PET shows hypometabolism, infants with cryptogenic West syndrome may have a favourable developmental or seizure outcome. PET may be a useful tool in evaluating the prognosis in infants with cryptogenic West syndrome.

A Follow-up Survey on Seizures Induced by Animated Cartoon TV Program “Pocket Monster”

Summary:  Purpose : To identify the short‐term outcome of patients who had seizures while watching an animated cartoon TV program, “Pocket Monster,” on December 16, 1997.

Epileptic Spasms Preceded by Partial Seizures with a Close Temporal Association

Summary: Purpose: To investigate the distinctive features of patients with West syndrome who had partial seizures followed by epileptic spasms (PS‐ES).

Inflammatory pathological changes in a 2-year-old boy with Charcot-Marie-Tooth disease.

We report histopathological findings in a 2-year-old boy with Charcot-Marie-Tooth (CMT) 1A, which had some similarities to those of chronic inflammatory demyelinating polyneuropathy. These findings are unusual in adult CMT patients but are reported in some patients with corticosteroid-responsive hereditary motor and sensory neuropathy (HMSN) that are characterized by rapid worsening of symptoms. We administered betamethasone based on the inflammatory pathological features but no improvement was seen. Active demyelination is also reported as an early morphological feature in HMSN. It is probable that edema, active demyelination and a few onion bulb formations, which were recognized in this patient, are characteristic early histological changes of HMSN rather than those of corticosteroid-responsive HMSN.

Epilepsies after Pocket Monster Seizures

Summary:  Background: We conducted a 5‐year follow‐up study in patients with a seizure induced by animated cartoon “Pocket Monster.”

Distinct interactions of Sox5 and Sox10 in fate specification of pigment cells in medaka and zebrafish

Mechanisms generating diverse cell types from multipotent progenitors are fundamental for normal development. Pigment cells are derived from multipotent neural crest cells and their diversity in teleosts provides an excellent model for studying mechanisms controlling fate specification of distinct cell types. Zebrafish have three types of pigment cells (melanocytes, iridophores and xanthophores) while medaka have four (three shared with zebrafish, plus leucophores), raising questions about how conserved mechanisms of fate specification of each pigment cell type are in these fish. We have previously shown that the Sry-related transcription factor Sox10 is crucial for fate specification of pigment cells in zebrafish, and that Sox5 promotes xanthophores and represses leucophores in a shared xanthophore/leucophore progenitor in medaka. Employing TILLING, TALEN and CRISPR/Cas9 technologies, we generated medaka and zebrafish sox5 and sox10 mutants and conducted comparative analyses of their compound mutant phenotypes. We show that specification of all pigment cells, except leucophores, is dependent on Sox10. Loss of Sox5 in Sox10-defective fish partially rescued the formation of all pigment cells in zebrafish, and melanocytes and iridophores in medaka, suggesting that Sox5 represses Sox10-dependent formation of these pigment cells, similar to their interaction in mammalian melanocyte specification. In contrast, in medaka, loss of Sox10 acts cooperatively with Sox5, enhancing both xanthophore reduction and leucophore increase in sox5 mutants. Misexpression of Sox5 in the xanthophore/leucophore progenitors increased xanthophores and reduced leucophores in medaka. Thus, the mode of Sox5 function in xanthophore specification differs between medaka (promoting) and zebrafish (repressing), which is also the case in adult fish. Our findings reveal surprising diversity in even the mode of the interactions between Sox5 and Sox10 governing specification of pigment cell types in medaka and zebrafish, and suggest that this is related to the evolution of a fourth pigment cell type.

A pilot study on benign partial epilepsy in children with complex partial seizures.

In a review of 145 children with partial onset epilepsy, the authors were able to determine a focus of children whose complex partial seizures (CPS) ran a benign course, who had no identifiable lesion on scanning and whose EEG focus was not fixed, but tended to shift. The authors suggest that a benign form of CPS in children can be recognised.

Correlations Between the Distribution of Cortical Hypometabolism and Clinical Manifestations in Patients with West Syndrome

Purpose: To examine the relation between the distribution of cortical hypometabolism and clinical manifestations in patients with West syndrome (WS).

A Comparison Between Seizure Manifestations and Outcome in Frontal Lobe Epilepsy of Childhood Onset

Purpose: The seizure manifestations of frontal lobe epilepsy (FLE) vary considerably, and the correlation between the ictal symptoms and seizure prognosis has not yet been fully elucidated. We retrospectively compared seizure manifestations with outcomes of the seizures in FLE of childhood onset.