Takeshi Tsuji

Current treatment of west syndrome in Japan

About 10 years have passed since a previous survey on the treatment of West syndrome in Japan. To elucidate current practice, a questionnaire was sent to 113 institutes. It included (1) the drugs used for the treatment, (2) their dosage, and (3) the dosage and the schedule of adrenocorticotropic hormone therapy. Response rate was 51.3%. Adrenocorticotropic hormone, valproic acid, vitamin B6, and zonisamide were frequently used. Vitamin B6 was used most frequently as the first-choice drug followed by valproic acid, zonisamide, and adrenocorticotropic hormone. The most frequently used dose of synthetic adrenocorticotropic hormone-Z was 0.0125 mg/kg/d. Adrenocorticotropic hormone was administered every day for 2 weeks and then tapered off in more than 80% of the institutes. Although therapeutic strategy and drug usage have not changed largely during these 10 years, 2 alterations were observed: an increased use of zonisamide and a shortened duration of adrenocorticotropic hormone therapy.

Multiple pathways for noxious information in the human spinal cord.

Abstract To investigate the pathways of noxious information in the spinal cord in humans, we recorded cortical potentials following the stimulation of A‐delta fibers using a YAG laser applied to two cutaneous points on the back at the C7 and Th10 level, 4 cm to the right of the vertebral spinous process. A multiple source analysis showed that four sources were activated; the primary somatosensory cortex (SI), bilateral parasylvian region (Parasylvian), and cingulate cortex. The activity of the cingulate cortex had two components (N2/P2). The mean peak latencies of the activities obtained by C7 and Th10 stimulation were 166.9 and 186.0 ms (SI), 144.3 and 176.8 ms (contralateral Parasylvian), 152.7 and 185.5 ms (ipsilateral Parasylvian), 186.2 and 215.8 ms (N2), and 303.0 and 332.3 ms (P2). Estimated spinal conduction velocities (CVs) of the respective activities were 16.8, 9.3, 8.7, 10.1 and 10.7 m/s. CV of SI was significantly faster than the others (P < 0.05). Therefore, our results suggested that noxious signals were conveyed through at least two distinct pathways of the spinal cord probably reaching distinct groups of thalamic nuclei. Further studies are required to clarify the functional significance of these two pathways.

Prognostic factors in acute encephalopathy with reduced subcortical diffusion

OBJECTIVES Acute encephalopathy with reduced subcortical diffusion (AED) covers a spectrum including not only typical acute encephalopathy with biphasic seizures and late reduced diffusion (AESD) but also atypical AESD with monophasic clinical course, or more severe subtypes. Aim of this study is to analyze prognostic factors of AED. MATERIALS & METHODS We recruited 33 children with AED, that is, widespread diffusion restriction in cortical and subcortical structures. Their clinical courses, laboratory data, MRI, and the efficacy of treatment were analyzed retrospectively. RESULTS Of the 33 children, 20 were males and the mean age at diagnosis was 22 months. Eighteen children had good outcome and 15 had poor outcome. Univariate analysis showed loss of consciousness 24 h after the onset, prolonged seizure at the onset, and mechanical ventilation to be weak predictors of poor outcome. Maximal aspartate aminotransferase, alanine aminotransferase, and creatinine kinase levels were significantly higher in the poor outcome group. Multivariate analysis showed loss of consciousness 24 h after the onset and prolonged seizure at the onset to be poor predictors of AED. Treatment with steroids and/or immunoglobulins did not result in better outcome. CONCLUSION Prolonged seizure at the onset and loss of consciousness 24 h after the onset were seen at early stages of severe AED. Using these features, a prospective study of early intervention in AED should be conducted to further analyze the efficacy of its treatment.

ACTH therapy for generalized seizures other than spasms

PURPOSE We reviewed our experience with ACTH for the treatment of intractable generalized seizures other than spasms. METHODS Efficacy and adverse effects of ACTH against intractable generalized seizures other than spasms were retrospectively investigated in 15 patients treated between 1991 and 2003. The median age of the patients at ACTH therapy was 42 months. The targeted type of seizure was brief tonic in 6, atypical absence in 6, atonic in 2, and myoclonic in 1. The dose of ACTH was 0.01-0.015 mg/kg in most patients. The effect of ACTH was determined at the end of ACTH therapy, and 3 months and 1 year after ACTH therapy. RESULTS Seizure freedom was obtained in 13 of 15 patients. However, 6 of them had a recurrence of seizures within 3 months after ACTH therapy. ACTH was the most effective in patients with atypical absence seizures. Statistical analysis showed that the number of injection and total dose of ACTH were smaller in patients with atypical absence seizures than in those with brief tonic seizures. Adverse effects were frequent but serious one was not observed. CONCLUSION ACTH therapy was effective in patients with intractable generalized seizures other than spasms. However, its efficacy was often transient. ACTH can be useful for generalized seizures other than spasms but its limitation should be considered.

Micturition induced seizures: ictal EEG and subtraction ictal SPECT findings.

We report an 8-year-old girl with seizures induced by micturition. Her seizures were characterized by extension of upper extremities with preserved consciousness. Magnetic resonance imaging and interictal electroencephalograms were normal. Ictal electroencephalogram showed low voltage fast waves without clear focal features followed by frontal dominant rhythmic multiple spikes. Subtraction ictal single photon emission computed tomography revealed a significant increase in blood flow in the mesial frontal area.

Evaluation of brain maturation in pre-term infants using conventional and amplitude-integrated electroencephalograms

OBJECTIVE To investigate the accuracy of conventional electroencephalograms (cEEGs) and amplitude-integrated EEGs (aEEGs) in the evaluation of brain maturation in normal pre-term infants. In addition, extrauterine acceleration of brain maturation was investigated. METHODS A total of 129 EEGs recorded between 27 and 37 weeks of postconceptional age (PCA) from 37 clinically stable pre-term infants were analysed. One expert investigator blindly estimated PCA using cEEG and total cerebral function monitoring (CFM) score on aEEG. The estimated PCA (ePCA) on aEEG was calculated from a regression equation. RESULTS The linear regression analysis showed a significant correlation between chronological PCA (cPCA) and ePCA on cEEG and between cPCA and total CFM score on aEEG. The estimation gap was smaller on cEEG, which was between -2 and +2 weeks in 96.9%. No significant correlation was observed between postnatal age and the estimation gap on cEEG or aEEG. CONCLUSIONS Estimated brain maturation was well correlated with cPCA on cEEG and aEEG. However, the evaluation of brain maturation was much more accurate on cEEG than aEEG. Extrauterine acceleration of brain maturation was not observed on either cEEG or aEEG. SIGNIFICANCE cEEG and aEEG will be valuable tools for evaluation of brain maturation in pre-term infants.

Acute encephalopathy with 2009 pandemic flu: Comparison with seasonal flu

To clarify the features of acute encephalopathy associated with 2009 pandemic flu. We identified 51 patients with acute encephalopathy with seasonal flu from the data base accumulated by Tokai Pediatric Neurology Society. We also collected 10 patients with acute encephalopathy with 2009 pandemic flu. The clinical course, laboratory data, neuroimaging findings, treatment, and the outcome of these patients were recruited using a structured research form. These data were compared between the two groups. The age was larger in the 2009 pandemic flu group (median, 109.5months) than in the seasonal flu group (median, 44months). There was no significant difference in other demographic data, neurologic symptoms, laboratory and neuroimaging findings, and treatment. Various degrees of neurologic sequelae including death were observed in 32% of the patients in the seasonal flu group, and in 50% in the 2009 pandemic flu groups. The analyses of patients with ages of 6years or older revealed that moderate or more severe sequelae were more frequent in patients with 2009 pandemic flu. Acute encephalopathy with 2009 pandemic flu occurred mainly among children with 6years of age or older, and the outcome was worse in this age group compared with acute encephalopathy with seasonal flu.

Callosal lesions and delirious behavior during febrile illness.

We retrospectively reviewed electroencephalography and magnetic resonance imaging findings for 21 children exhibiting delirious behavior during febrile illness. Among these, five patients had transient callosal lesions with or without white matter lesions on diffusion-weighted images. We compared the clinical characteristics, duration, and components of delirious behavior, the duration and severity of reduced consciousness, and EEG findings among patients with or without callosal lesions. No significant differences were detected in these items according to the presence or absence of callosal lesions. Adding insight into the pathogenesis of this condition, our study revealed that callosal lesions are not uncommon in patients exhibiting delirious behavior during febrile illness.

Amplitude-integrated electroencephalography in preterm infants with cystic periventricular leukomalacia

AIM This study aimed to assess amplitude-integrated electroencephalography (aEEG) findings in preterm infants with cystic periventricular leukomalacia (cPVL) in the early neonatal period. METHODS We analyzed five infants with cPVL, whose gestational age was between 27 and 30 weeks, and 15 matched control infants. Two-channel (C3-O1 and C4-O2) aEEG was obtained by digital conversion from a conventional electroencephalogram, which was recorded at days 0-5, 6-13, and 21-34 in each infant. We evaluated the averaged two-channel values of several measurements using visual and quantitative analyses. RESULTS Infants with cPVL had a significant higher maximal upper-margin amplitude value, with a median of 47.5 μV (range of 42.5-60) compared with the control infants (median, 33.8; range, 23.8-50) in the second visual-analysis record. Infants with cPVL also had a significantly higher mean upper-margin amplitude value, with a median of 18.8 μV (range, 17.7-23.2) compared with the control infants (median, 16.3; range, 10.3-19.0) in the second quantitative-analysis record. CONCLUSIONS We demonstrated that the upper-margin amplitude of aEEG in infants with cPVL was significantly higher than that in the control infants at 6-13 days after birth.

Delirious behavior in children with acute necrotizing encephalopathy

OBJECTIVE To clarify the features of delirious behavior in patients with acute necrotizing encephalopathy. METHODS We retrospectively evaluated the clinical course of 38 children with acute necrotizing encephalopathy diagnosed on the basis of neuroradiological findings. The patients were divided into two groups according to the presence or absence of delirious behavior. We compared clinical features, laboratory data, neuroimaging findings, and outcome between those with and without delirious behavior. In patients with delirious behavior, chronological sequence of neurological symptoms and the characteristics of delirious behavior were investigated. RESULTS Delirious behavior was observed in 8 patients. Patient characteristics or most laboratory data on admission were not significantly different between those with and without delirious behavior. Brainstem lesions were more frequent in patients with delirious behavior than in those without. In contrast, lesions in lentiform nuclei, cerebral hemisphere, or cerebellum were relatively more frequent in patients without delirious behavior. It was the initial neurological symptom in 7 of 8 patients. Stupor and seizures were observed after delirious behavior in most patients. CONCLUSIONS Delirious behavior was not uncommon in children with acute necrotizing encephalopathy. Brainstem lesions may be related to the development of delirious behavior of children with acute necrotizing encephalopathy.