Proceedings of the 25th Annual Meeting of the Japanese Society of Child Neurology

Proceedings of the 25th Annual Meeting of the Japanese Society of Child Neurology

Proceedings of the 25th Annual Meeting of the Japanese Society of Child Neurology Tochigi, July 7-9, [983 PREFACE Kamoshita S 108 SPECIAL LECTURE...

900KB Sizes 1 Downloads 29 Views

Proceedings of the 25th Annual Meeting of

the Japanese Society of Child Neurology Tochigi, July 7-9, [983

PREFACE

Kamoshita S

108

SPECIAL LECTURES A Quarter-Century of the Japanese Society of Child Neurology Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Molecular Physiology of Muscle Contraction Ebashi S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ..

109

134

GUEST LECTURE CSF Dynamics in Hydrocephalus- With Special Attention to External Hydrocephalus Barlow CF . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

119

EDUCATIONAL LECTURES Type I Familial Amyloidotic Polyneuropathy (Japanese Type) Araki S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Functional as well as Pathophysiological Aspects. of the Basal Ganglia YoshidaM...............................................

CHILD NEUROLOGY TOPICS Acute and Long-Term Side Effects of Antineoplastic Treatment in Children Hanefeld F, Harten G, Langermann H-J, Riehm H, Stephani U . ............ . Well-known and Less Well-known Malformations of the Hindbrain. Their Morphology and Relationship to "Malformation" Hydrocephalus Schmitt HP . .................................................... . SYMPOSIA Problems in Perinatal Neurology - Diagnosis of Neonatal Brain Damage Preface: Present Situation and Future of Perinatal Neurology Sakamoto Y, Watanabe K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Diagnosis of Neonatal Central Nervous System Infections ToriiS . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Diagnosis of Neonatal Bacterial Meningitis Imura S, Takada M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Two-dimensional Ultrasonography of the Brain in Infancy: Its Application to the Diagnosis of Intracranial Disorders Yano S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ..

128

135

136 138

139 139 140

141

Contents 93

Two-dimensional Ultrasonography of the Brain in Infancy: Its Application to the Diagnosis of Intracranial Disorders Yokochi K, Hokazono Y, Inukai K ...... .... . .... . . . . . . . . . . . . . . . Neurophysiological Examinations for the Diagnosis of Perinatal Brain Damage Watanabe K, Inokuma K, Takeuchi T, Negoro T . . . . . . . . . . . . . . . . . .... . Electrophysiological Diagnosis in Newborn; Clinical Applications of Auditory Brainstem Response and Blink Reflex Yasuhara A, Yamada A, Kinoshita Y, Matsumura T. . . . . . . . . . . . . . . . . . Pathogenesis and Prevention of Perinatal Hypoxic Brain Damage and Intraventricular Hemorrhage Takashima S, Ando Y, Mito T, Otani K, Yokota K, Kondo I, Iwao H, Oguri R, Sueishi K, Tanaka K . . . . . . . . . . . . . . . . . . . . . . . . . . Treatment and Prognosis of Intracranial Hemorrhage and Asphyxia in Neonates Sugimoto T . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . . .. .

141 142 143 . .

.144 . . . . . .. . 144 . . . .

.. .

FREE PAPERS

1.

2 3 4

5 6 7 8 9

II. 10

11 12

Experimental Developmental Neurobiology Regional Membrane Phospholipid Composition in Developing Rat Brain after Premature Birth Yokota K, Yamaguchi T .... . . . . . . . . . . . . . . . .. . . . . . . . . . . . . . . . . 146 Effect of Theophylline on Myelination in the Brain of Young Rats Kamigaki M, Sunaga S, Sato Y, Okuyama K . . . . . . . . . . . . . . . . . ... . . 146 . . . Enzyme-Linked Immunoadsorbent Assay for Myelin Basic Protein Tanaka K, Tomida N, Tanaka M, Sasahara A, Yamamoto H, Shimada M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 147 Prevention of Brain Dysfunction by Supplementary Zinc in Rat with Fetal Alcohol Syndrome: Effect on RNA and Protein Contents and Appearance of Confronting Cisternae Tanaka H, Inomata K, Arima M . . . . . . . . . . . . . . . . . . . . . . . . . .... . . .147 . Effects of Phenytoin on Mouse Purkinje Cells-Comparison between Infantile and Adult Mice Suzuki M, Tamai!, Ohta H, Maekawa K ...... . . . . . . . . . . . . . . . . . . . . , 148 Synaptic Changes in Purkinje Cell Dendritic Spines of Mouse Cerebellum after Neonatal Administration of Cytocine Arabinoside Ohno M, Kawasaki H, Utsunomiya T, Abe Y, Yamano T, Shimada M . . . . . ... 148 Experimental Study on CNS Toxicity of Vincristine Tomiwa K, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . 149 . . . . .. Effects of Early Malnutrition and Rehabilitation on Brain Development -Golgi-Cox Study Yoshida A, Yoshioka H, Kasubuchi Y, Kusunoki T . . . . . . . . . . . . . . . . . . 149 . . Biochemical Study of Experimental Microcephaly. III. Developmental Changes in Catecholamines, Serotonin and GABA in Experimental Microcephaly Yamasaki S, A be Y, Oya N, Shimada M ... .. .. . .. .... . . . . . . . . . . . .. 150 Human Development Posture of Kindergarten Children and Its Relationship to Behavioral Problems Yuge M, Kanda T, Yamori Y. . . . . . . . . . . . . . . . . . . . . . . . . . . .150 . . . . . . . Latency and Persistence of Fersen Reflex in Infants Futagi Y, Ohtani K, Tanaka J, Shimizu H . . . . . . . . . . . . . . . . . . . . . . 151 . . . . Effects of Posture on the Functional and Postural Laterality of Pre-Term Infants in the Early Life Konishi Y, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151

94 Brain & Development, Vol 6, No 2, 1984

. .

13 14 15

16

17

III. 18

19 20 21 22 23

24 25 26 27 28 29 30

The Passive Range of Motions in Developmentally Disabled Children Kisa T, Nakao Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152 . . . . Method of Feeding in Infancy and the Psychomotor Development at Preschool Age Mukai Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152 .. . . . An Attempt at Systematic Understanding of Benign Hypertonia and Dystonia in Infancy Kitazumi E, Enom oto S, Yokochi A, Yokochi K, Uchida T, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . 153 . . . .. . Effects of Neonatal Hypoxia on Brain Development III. Behavioral Changes due to Total Asphyxia Yoshioka H, Ochi M, Y oshida A , Iino S, Fujiwara K, Ishimura K, . . .... . . 153 . . . . Nishimura 0, Kusuno ki T . . . . . . . . . . . . . . . . . . . . . . . Postural Changes in Ventral Suspension during the Neonatal Period and Early Infancy Morikawa Y, Sai H, Hino T, Fujita H, Izumi H, Kimura K, Miyoshi K, Mizuta R . . . . . . . . . .. . . .. . . . . . . . . . . . . . . . . . . .... . .154 . . . . .

.. . ..

. .

.

. .

. . .

Neurophysiological Diagn ostics A Study on Pathophysiology of Myoclonus - Polysomnographical Examination Yonezawa M, Tada H, Hasegawa M, Nomura Y, Segawa M, . . . . . . . . . . . . . . . . .. . . . . 154 .. . .. . Yamashita K, Tanaka S . . . . . . . . . . An Electroencephalographic Study on Uncommon Chromosomal Abnormalities Nishibayashi N, Inouye H . . . . . . . . . . . . . . . . . . . . . . . . . . . . .155 . . . . . . . . A Clinical Study of EEG Power Spectra in Children with Hemiplegia -Prognostic Correlation to Similarity between the Two Hemispheres Fujiwara J, Kawashima K, Nakahara S, Nakata Y . . . . . . . . . . . . . . . . . .ISS . . . On the Effects of Pentoxifylline upon the EEG Activity of Early Infantile Autism: Quantitative Pharmaco-Electroencephalographic Study Suzuki M, Nakazima K, Sugiyama A, Nakashita Y, Ogawa T . . . . . . . . . . . . 156 . . Analysis of Swallowing Disturbance in Cerebral Palsy by Superficial EMG and Respiratory Curve Analysis Hirayama Y, Takagi A , Wash ida T . . . . . . . . . . . . . . . . . . . . . . . . . 156 . . . . . . The Correlation of Waveform between Fontanelle Pressure and Systemic Pressure - Observation of Waveform during Operations for Congenital Heart Disease Nakashima 0 , Shirouzu T, Kuramoto N . ... . . . . . . . . . . . . . . . . . . .157 . . . . Normal Conduction Velocity of the Tibial Nerve in Children Abe J, Mimaki T, On oe S, Tagawa T, Ono J, Yabuu chi H, Sugita T .. . . . . . . . 157 Topographic Mapping of Brainstem Auditory Evoked Responses in Humans Shimizu N, Hirasawa K . . . . . . . . . . . . . . . . . ... . . . . . . . . . . . . . . . . . . 158 Four Cases of Prenatal Brain Stem Damage Identified by Auditory Brain Stem Responses Uchida T, Kitazumi E, Yo kochi K . . . . . . . . . . . . . . . . . . . . . . . . . . 158 . . . . . A Visual Evoked Potential Study in Sturge-Weber Syndrome Iinuma K, Suzuki K, Ich inohasama Y, Hayamizu S . . . . . . . . . . . . . . . . 159 ... . A Clinical Study of Short Latency SEP in Patients with Brain Dysfunction Kasagi S, Takakura H ..... . ... .. .. ... ..... . . . . . . . . . . . .... ... 159 Developmental Change of Interhemispheric Coherence in Children Tomita Y, Kato E, Mori C, Abe K .... .. . . . . . . . . . . . . . . . . . . .. . . . . 160 . Polysomnography on Lissencephaly Hiura K, Hashim oto T, Fukuda K, Endo S, Tayama M, Mori A, Fujino K, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160 . . . . . .

Contents 95

31

32 33 34 35 36

37 38 39

40

41 42

43 44 45 46 47 48

A Long-Term Polygraphic Study Using Ambulatory EEG Monitoring - Apneic Spells in a Case of Arnold-Chiari II Malformation during the Day and Night Kumagai K, Kureha Y, Obata J, Niwa N, Okuyama M, Horita H, Maekawa K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Head Injuries and Electroencephalographic Findings Sogawa H, Ohara T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Developmental Study of the T-Wave of Achilles Tendon Reflex in Children Takakura H, Kasagi S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Auditory Evoked Palpebral Reflex: Clinical Application to Neonatal Brain Dysfunction Yamada A, Yasuhara A, Kinoshita Y, Matsumura T, Yasuhara M . . . . . . . . . . . An Electrophysiological Study of Early-Infantile Epileptic Encephalopathy with Suppression-Bursts Yasuhara A, Yamada A, Kinoshita Y, Sugimoto T, Matsumura T . . . . . . . . . . . Somatosensory Evoked Potentials and Auditory Brainstem Responses in Diabetes Mellitus Takeda K, Murata R, Matsuoka 0, Kin M, Aono S, Tani M, Isshiki G . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Auditory Brainstem Response (ABR) in the Cat: Effects of Lesions Nakano S, Okuno T, Mikawa H, Kudo M . . . . . . . . . . . . . . . . . . . . . . . . . . . Hemispheric Lateralization of Flash YEP Maps of Some Neurological Disorders in Pediatrics Hirasawa K, Shimizu N . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Short Latency Somatosensory Evoked Potentials Following Peroneal Nerve Stimulation Hashimoto T, Tayama M, Mori A, Hiura K, Fukuda K, Endo S, Fujino K, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Short Latency Somatosensory Evoked Potentials in Patients with Anticonvulsant Therapy Tayama M, Hashimoto T, Kawano N, Hiura K, Suzue J, Endo S, Fukuda K, Mori A, Fuzino K, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . Developmental Changes of Topographic EEG Features in the Newborn Inokuma K, Watanabe K, Negoro T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Follow-Up Study of Neurologically Abnormal Infants Using the Auditory Brain Stem Response Part I: Process to Spastic Paralysis in Infants with One-Sided Deafness KoikeK . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Polygraphic Studies of Abortive Sudden Infant Death Syndrome Fukuda K, Mori A, Tayama M, Endo S, Suzue J, Hiura K, Kawano N, Hashimoto T, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Development of Sleep Spindles in Normal Children - With Reference to Change in Their Distribution Miyajima T, Ogihara M, Hoshika A, Matsuno T, Honda T . . . . . . . . . . . . . . . . The Effect of Neonatal Hyperbilirubinemia on the Auditory Brainstem Response Kuriyama M, Konishi Y, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Short-Latency Somatosensory Evoked Potentials (SSEP) in Pediatric Neurological Disorders - Degenerative Diseases Nakagawa Y, Kataoka K, Hojo H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . On a Quantitative Method to Measure Cerebral Blood Flow in Newborn Infants (I) Ishiwa S, Gotoh K, Ono Y, Ogawa T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Auditory Brainstem Evoked Responses in Down Syndrome Miyao M, Yamamoto Y, Kamoshita S, Nihei K . . . . . . . . . . . . . . . . . . . . .. . .

96 Brain & Development, Vol 6, No 2, 1984

161 161 162

162 163

163 164 164

165

165 166

166 167 167 168

168 169 169

IV.

49 50

51 52

53 54

V. 55 56 57

58 59 60 61 62 VI.

63 64 65 66

Neuroradiological Diagnostics Neuroradiological Study of Human Brain in the Fetal Period Imanishi M, Tanikake T, Kyoi K, Utsumi S, Fukuzumi A, Iwasaki S, Otani R . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . The Morphometrical Changes Seen on Computed Tomography with Increasing Age in Down Syndrome: Small Posterior Fossa and Calcification of the Globus Pallidus Ieshima A, Kisa T, Yoshino K, Takashima S, Takeshita K . . . . . . . . . . . . . . . Subdural Effusion (Hematoma) Accompanied by Enlargement of Subarachnoid Spaces in Infancy Aoki N, Masuzawa H, Sato J . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Evaluation of Shunt Patency Sato J, Masuzawa H, Mirna T, Inoue T . . . . . . . . . . . . . . . . . . . . . . . . . . . Classification of Low Density Areas in the White Matter on Computerized Tomography Narazaki 0, Hanai T, Fukuda M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Positron Emission CT in Childhood Epilepsy Michihiro N, Utsumi Y, Ariizumi M, Sato K, lio M . . . . . . . . . . . . . . . . . . . Congenital Anomalies of the CNS Slit Ventricle as a Shunt Complication - Some Considerations on Pathogenesis and Therapeutic Problems in the Developing Brain Sato N, Sato H, Hojo H, Yamasaki S, Tamaki N, Matsumoto S . . . . . . . . . . . Cerebral Malformation of Thanatophoric Dwarfism: Including a Golgi Study Shigematsu H, Otani K, Takashima S, Ieshima A . . . . . . . . . . . . . . . . . . . . . Pathology of Holoprosencephaly on Immunohistochemical Observation of Heterotopic Glioneural Tissue in the Subarachnoid Space Tanaka J, Takada K, Ito H, Tsujimoto Y . . . . . . . . . . . . . . . . . . . . . . . . . . Congenital Cyst in the Tentorial Region Nakahara N, Masuzawa T, Iida K, Shimabukuro H, Yamamoto H, Iwasa H, Sato F . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ... . .. An Autopsy Case of Cerebro-hepato-renal Syndrome of Zellweger Iwamoto H, Hayashi M, Sasaki Y, Shiihara H, Onishi S . . . . . . . . . . . . . . . . A Long-Term Follow-Up Study of Spina Bifida Uchida T, Yokochi A, Yokochi K, Kitazumi E, Enomoto S, KodamaK . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Monoamine Metabolites in Cerebrospinal Fluid and Intraventricular Cerebrospinal Fluid Pressure in Childhood Hydrocephalus Ito H, Chin M, Oiwa Y, Okada K, Ito T, Miwa T . . . . . . . . . . . . . . . . . . . . . Multicystic Encephalopathy - A Poly etiologic Condition in Early Infancy: Morphologic, Pathogenetic and Clinical Aspects Schmitt HP . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Neonatal Neurology Histographic Analysis of Computed Tomography Scans in Term Newborn Infants with Perinatal Asphyxia Nakada E, Tanaka M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Evaluation of the LVW /HW Ratio in Preterm and Term Infants with Real-Time Ultrasound Tatsuno M, Okuyama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ... Pathological Studies on the Brain in Massive Aspiration Syndrome (MAS) Itoh H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Neuropathological Studies on the Brain of Autopsied Infants, Dying of Gastric Perforation (GP) and Necrotizing Enterocolitis (NEC) Itoh H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

.

170

.

170

.

171

.

171

.

172

.

172

.

173

.

174

.

175

.

175

.

176

.

176

.

177

.

177

.

178

.

178

.

179

.

179

Contents 97

67

VII. 68

69

70 VIII. 71 72

73 74 75 76 77

78 79

IX. 80 81

82

Diagnosis of Neonatal Convulsions Using a Real-Time Ultrasound Scanner Yokota K, Hirata T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Neurocutaneous Syndromes The Cystic Renal Lesions in Epileptic Patients with Tuberous Sclerosis Watanabe Z, Ogawa S, Tsuchiya M, Murakami M, Hashimoto K, Ueda Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Abnormal Cerebral Vessels in von Recklinghausen Disease: Report of Three Cases Takenaka N, Toya S, Shiobara R, lisaka Y, Ootani M, Miyahara Y, Doumoto Y, Inoue H, Okui S, Ohira T . . . . . . . . . . . . . . . . . . . . . . . . . . . . Cutis Verticis Gyrata and Its Precursors in Children with Mental Retardation Tachibana H, Sugio Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Intellectual, Motor and Sensory Disturbances Educational Attendance of Handicapped Children in the Suburban Area of Tokyo Tohjo M, Ohtsuka M, Nagase M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Early Signs of Spastic Diplegia: Comparative Analysis on Video Recording Kitazumi E, Enomoto S, Yokochi A, Yokochi K, Uchida T, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Clinical Features for Developing Cerebral Palsy Kanda T, Yuge M, Suzuki J, Yamori Y, Fukase H . . . . . . . . . . . . . . . . . . . . . An Investigation of Cerebral-Palsied Children in Regular Classrooms GakuhariH, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. A Study on the Pathophysiology of Sudden Death in SeverelyHandicapped Children Kono C, Oka E, Inoue H, Miyake S, Iyoda K, Ohtahara S . . . . . . . . . . . . . . . . Possible Risk Factors and Signs of Mental Retardation: Comparative Study of Mentally Retarded Children and Controls Matsuishi T, Yoshimura K, Yoshida A, Yamashita F, Shiotsuki Y . . . . . . . . . . The Prevalence Rate and an Etiological Survey of Mental Retardation (MR) in Japanese School Children Shiotsuki Y, Matsuishi T, Yoshimura K, Yamashita F, Yano K, Tokimasa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Statistical Study on Resident Deaths at Institutions for the Profoundly Retarded Nakamura H, Honda T, Uemura I, Nanba Y, Mita K, Okafa K . . . . . . . . . . . . . On the Development of Mental Activity in the Monozygotic Twins with Early Infantile Brain Injury Hasegawa Y, Okada R, Tachi K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Convulsive Disorders (1): Febrile or Non-Epileptic Etiological Aspects of Epilepsy and Febrile ConVUlsions-Pregnancy and Perinatal Factors Katafuchi Y, Aoki N, Matsuishi T, Yamashita F . . . . . . . . . . . . . . . . . . . .. , Follow-Up Study of Children with Simple Febrile Convulsions and Epileptiform EEG Activities without Chronic Anticonvulsant Medication Kajitani T, Kimura T, Ide K . . . . . . . . . . . . . . . . . . . - - - - . - ..... - . . .. Clinical and Electroencephalographic Study on Siblings of Patients with Epilepsies or Febrile Convulsions Exhibiting Rolandic Discharges Nakamura M, Kajitani T, Kumanomidou Y, Kimura T . . . . . . . . . . . . . . . . . .

98 Brain & Development, Vol 6, No 2,1984

180

180

181 181

182

182 183 183 184 184

185 185 186

186

187

187

83

84

85 86 X.

87

88 89 90 91 92 93 94 95 96

97 98 XI.

99

lOO 101

Qualitative Analysis of Paroxysmal Discharges in Electroencephalograms of Febrile Convulsions and Their Prognosis Kimiya S, Seki T, Yamawaki H, Suzuki N, Maezawa M, Yamada T, Tachibana Y, Hirose M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Apnea Following Hyperventilation in Severely Mentally Retarded Children Miyazaki S, Hara K, Kumagai T, Takeuchi T, Hakamada S, Watanabe K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. A Case of Familial Paroxysmal Choreoathetosis Endo S, Suzue J, Hiura K, Kawano N, Hashimoto T, Tamura Y, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Epilepsy Following Febrile Convulsions Hirata Y, Somiya K, Nakano M, Ikuma M . . . . . . . . . . . . . . . . . . . . . . . . .. Convulsive Disorders (2): Epilepsy - Clinical A Clinical Study of Convulsions Including Status Convulsivus in Pediatric Emergencies Ko Y, Yabiku M, Uchida H, Hara T, Shiomi M, Imaishi H, Okuni H, Sugita T. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Long-Term Follow-Up Study of "Cryptogenic" Status Convulsivus in Childhood A waya Y, Fukuyama Y, Iwamoto H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Visual Epileptic Seizures after Mild Head Injuries in Children Tamagawa K, Sasaki H, Aoki N, Shimizu H . . . . . . . . . . . . . . . . . . . . . . . .. Headache and Convulsive Disorders: A Study on Pathophysiological Correlations Tada H, Goto M, Yonezawa M, Nomura Y, Segawa M . . . . . . . . . . . . . . . . . . Behavioral Problems in Children with Epilepsy Matsuo M, Kurokawa T, Takaki S, Chen Y-J, Yoshida K . . . . . . . . . . . . . . . . A Floppy Child with Atonic Attacks Kuwajima K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Risk of Seizures in Siblings of Children with Epilepsy Soda M, Nakai M, Takeshita K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Focal and Clonic Convulsions in Infancy Yokochi K, Uchida T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Ictal EEG in Benign Epilepsy of Childhood with Rolandic Discharges Kumanomidou Y, Kajitani T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Premenstrual Tension with Autonomic Seizure: Biochemical, Electroencephalographic and Endocrinological Studies Kureha Y, Numaguchi S, Obata J, Okabe T, Eto Y, Kumagai K, Maekawa K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Accidental Large Overdose of Sodium Valproate-A Case Report Horii Y, Ishimura K, lino S, Yoshida A, Ochi M, Yoshioka H, Kusunoki T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Report of Valproic Acid-Induced Pure Red Cell Aplasia Sugano T, Tominaga M, Kasubuchi Y, Imashuku S . . . . . . . . . . . . . . . . . . . . Convulsive Disorders (3): West or Lennox Syndrome A Longitudinal Study on Early-Infantile Epileptic Encephalopathy with Suppression-Bursts Ohtsuka Y, Yamatogi Y, Ichiba N, Terasaki T, Yoshida H, Matsuda M, Ohtahara S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Neuropathology of Infantile Spasms Satoh J, Mizutani T, Morimatsu Y, Tomimoto M, Sakamoto K, Shinohara T, Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. A Study on ACTH Therapy of Infantile Spasms 2nd Report Concerning Synthetic ACTH Hashimoto K, Tamamoto A, Ozaki N, Konishi K, Takaya K . . . . . . . . . . . . . ,

188

188 189 189

190 190 191 191 192

192 193 193 194

194 195 195

196

196

197

Contents 99

102 103 104 105 106

XII. 107

108 109 110 111

112 113 114 115 116 117

118 119

Suppressive Effect of ACTH on the Serum Thyroxine, Level and Its Influence on the Clinical Effectiveness in Infantile Spasms Izumi T, Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Effects of ACTH on Sleep of Patients with West Syndrome Ogiso M, Shimohira M, Kohyama J, Niwa T, Iwakawa Y, Segawa M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Gamma-Globulin Therapy in Infantile Spasms Shiihara H, Michihiro N, Ogawa K, Coto K, Suzuki H, Utsumi Y, Ariizumi M, Baba K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. A Study of Treatment of Lennox-Gastaut Syndrome Watanabe Y, Sato S, Okumura K, Aoki T . . . . . . . . . . . . . . . . . . . . . . . . .. Effect of ACTH Therapy on Peripheral Nerve Conduction Velocity in Patients with Infantile Spasms Shinozaki K, Izumi T, Ochiai T, A waya Y, Fukuyama Y . . . . . . . . . . . . . . . .

Convulsive Disorders (4): Treatments Clorazepate Therapy for Refractory Seizures in Children Mimaki T, Abe J, Onoe S, Tagawa T, Ono J, Yabuuchi H, Kamio M. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Pharmacokinetics of Rectal Administration of Diazepam Solutions in Infants with Febrile Convulsions Miura H, Minagawa K, Yagi J ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Lidocaine in the Treatment of Status Epilepticus Sai H, Morikawa Y, Izumi H, Miyoshi K, Kimura K, Fujita H, Hino T, Mizuta R, Coto K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Lidocaine for the Treatment of Status Epilepticus Miyata H, Tsubota T, Kuroda E, Takemoto H, Kanazawa Y, Ogino H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Reevaluation of the Indication of Sodium Valproate Monotherapy in Childhood Epilepsy - Compared with Phenobarbital Monotherapy Hosaka S, Ogiso M, Iwakawa Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Sodium Valproate and Clonazepam in the Treatment of Intractable Epilepsies Oki J, Tasaki T, Cho K, Yoshioka H . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Abnormal Breath Test Results with 13C-Palmitate in Patients Treated with Valproic Acid Sakuragawa N, Suehiro M, Watanabe H . . . . . . . . . . . . . . . . . . . . . . . . . . .. Serum Protein Binding of Phenobarbital in Childhood Tanaka J, Shimizu H, Futagi Y, Suzuki K . . . . . . . . . . . . . . . . . . . . . . . . .. Anticonvulsant Effect of Pyridoxal Phosphate in ddY Mice Takeda H, Komatsu M, Matsui T, Nakazawa M, Kuniya S, Miyake S, Yokoyama S, Kodama S, Matsuo T ...... . . . . . . . . . . . . . . . .. Physical and Mental Development of Infants Born to Mothers Taken Antiepileptic Drugs Nomura Y, Takebe Y, Kaneko S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. The Serum Enzyme Activity (LAP, ALP) in Liver of Infantile Patients with AED-Correlation of Combination, Serum Concentration of AED and Serum Enzyme Activity Miyamoto S, Hisanaga M, Utsumi S, Takemura K, Kirino Y . . . . . . . . . . . . . . Relationship between Valproate Pharmacokinetics and Laboratory Data in Epileptic Children in the Steady-State Takebe Y, Haneda S, Kimura S, Koide N. . . . . . . . . . . . . . . . . . . . . . . . . .. Study of Serum Immunoglobulins in Childhood Epilepsy Hibio S, Michihiro N, Ogawa K, Ohkubo 0, Utsumi Y, Ariizumi M, Baba K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ..

100 Brain & Development, Vol 6, No 2, 1984

197 198 198 199 199

200 200 201 201

202 202 203 203 204

204

205

205 206

XIII.

Antico nvulsan ts

120

Follow-Up Study of Plasma Ammonia Levels in Patients Treated with Valproic Acid (VP A)

121

Sodium Valproate and Hyperammonemia

122

The Effects of Valproic Acid and Phenobarbital on Free Amino Acid Levels in Serum and Urine

Yoshimoto T, Harima Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Nishida N, Ono A, Yasuhara A, Ito N, Sugimoto T, Matsumura T, Sakane Y, Taniuchi K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Honda T, Watanabe K, Sato Y, Tada H, Takahashi H, Kaneko K, Takahashi K, Ohtsuka C . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

123

Measurement of Free Phenobarbital Blood Levels Using an Amicon Centriflo Model CF 25 (Ultrafiltration Method)

124

Effects of Phenobarbital and Phenytoin on Lipid Peroxidation in Rats Ono J, Mimaki T, Nagaura T, Abe J, Onoe S, Tagawa T, Yabuuchi H . . . . . . . . Effect of l~-Hydroxycholeca1ciferol on Bone Development in Epileptic Children: Follow-Up Study with a Microdensitometer Onoe S, Mimaki T, Tanaka Y, Yamaoka K, Ishida M, Seino Y,

Fujii E, Okada K, Matsutaka K, Somqa T, Teshirogi T . . . . . . . . . . . . . . . . . 125

Yabuuchi H, Yamashita G . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

126

XIV. 127

128

A Study on Blood Levels of Antiepileptics before and Two Hours after Administration Nakazawa M, Kuniya S, Matsui T, Komatsu M, Yokoyama S, Kodama S, Matsuo T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Subacute Sclerosing Panencephalitis (SSPE) Treated with ~-Type Interferon (IF) Kataoka S, Hayashi T, Hirose G . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Interferon Therapy for SSPE Nishimura 0, Nakagawa M, Fujiwara K, Yoshioka H, Sawada T,

Kusunoki T, Kishida T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Inosiplex and Interferon Treatment in 2 Patients with SSPE

130

Induction of Acute Myoclonic Encephalitis in Hamsters by SSPE Virus

Kataoka K, Nakagawa Y, Hojo H, Yoshioka H, Sawada T . . . . . . . . . . . . . . . . Sugita T, Okuni H, Imaishi H, Shiomi M, Hara T, Uchida H, Yabiku M, Ko E, Ueda S, Shiraki K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Three Cases of Herpes Simplex Encephalitis with Reflection on the

133

The Early Diagnosis of Herpetic Encephalitis - Analysis of 14 Cases Hamano K, Ponsot G, Plouin P . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Measles Encephalitis in Two Immunized Children

134

Subacute Encephalitis Associated with Elevated HSV-l Titer

135

A Case of SSPE after Measles Vaccination Miyake S, Katsumata N, Hayashi M, Iwamoto H . . . . . . . . . . ... . ... . ... . An Autopsy Case of Subacute Sclerosing Panencephalitis with Hypogammaglo bulinemia Kyoya S, Irimichi H, Matsushima A, Kimura A, Fukase S,

136

137

207

208 208

209

209

210

210 211

211

Diagnosis and Treatment

Kiyohara K, Nakajima M, Nonomiya S, Hayashi M, Sekino T, Ogiso M, Arai S, Iwakawa Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

132

207

CNS Infections

129

131

206

212 212

Kurashige T, Tone Y, Kuramitsu M, Kitamura I . . . . . . . . . . . . . . . . . . . . . .

213

Kawano Y, Ohya T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

213

214

Koizumi F . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

214

Matsumura T, Yasuda M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

215

Three Cases of Congenital Cytomegalovirus Infection Ito N, Taniguchi H, Matsuzaki S, Sugimoto T, Iwase S,

Contents 101

138 139

140

141 XV. 142 143 144

145 146

147 148

149

150

151 152

XVI. 153

Rotavirus and Central Nervous System Ushijima H, Tajima T, Tagay a M, Nonaka C, Fujita S, Togo T, Hiraiwa M, Ohmi K, A be T, Nakajo M . . . . . . . . . . . . Indications for CT Examination in Purulent Meningitis - Investigation of Cases with Subdural Effusion Ishimura K, Fujiwara K, Ino S, Yoshida A, Ochi M, Yoshioka H, Kusunoki T . . . . . . . . . . . . . . . . . . . . . . . . . ... Two Cases of Severe Herpes Simplex Virus Infections Confirmed by Virus Isolation Hara T, Ito N, Sonoda N, Sugimoto T, Matsumura T, Ito T, Ohyama A . . . . . . . . . . . . . . . . . . . . . ... . . . . . Three Cases of Brainstem Encephalitis Miura J, Tachi N, Nakao T . . . . . . . . . . . . . . . . . . . . .

.. . ...

.215 . . . .

. . . . . . . . 216 ........

....

. . .216 .. . ...

..

. . . . . . . 217 . . . .. ...

Cerebrovascular Disorders Clinical Features of Moyamoya Disease: Differences from Acute Hemiplegia without the Moyamoya Vascular Network Kurokawa T, Chen Y-J, Kishikawa T, Kitamura K . . . . . . . . ......... 217 . ... EEG Power Spectra and Gas Analysis of Arterial Blood during and after Hyperventilation in Moyamoya Disease in Children Nakahara S, Kawash ima K, Fujiwara J, Matsumura A , Nakata Y . . . . . . . . . . . 218 Mechanism of EEG Abnormality Caused by Hyperventilation in Children with Moyamoya Disease-Hemodynamic Study by the 133Xe Inhalation Method Tagawa T, Naritomi H, Mimaki T, Abe J, Onoe S, Ono J, Yabuuchi H, Sum i K, Sawada T . . . . . . . . . . . . . . . . . . . . . . . . . . 218 . . . . . . . Correlation between CT Findings and Intelligence Quotient in Pediatric Patients with Moyamoya Disease Fukumoto T, Matsushima Y, Suzuki R, Hiratsuka H, Inaba Y . . . . . . . . . . . . . 219 Long-Term Surgical Results of Moyamoya Disease in Childhood Yoshizawa T, Kikuchi H, Karasawa J, Nishiya M, Ihara I, Yamashita T, Nagata I, Naruo Y, !kota T, Mitsugi T, Sh ishido H, Inoue S, Hashimoto K, Minamikawa J, Miyamoto S . . . . . . . . . . . . . . . . . . . 219 100 Cases of Acute Hemiplegia in Childhood Okuno T, Hattori H, Takao T, Ito M, Konishi Y, Nakano S, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220 . . .. .. . . A Study on Prognosis of Acute Hemiplegia in Childhood (AHC): Particularly with Reference to Age of Onset, Sex of Patient and Side of Lesion Honda K, Takehiro H, Miyake S, Ike Y, Kimura S, Numora Y . . . . . . . . . . . . 220 . Studies on Ischemic Changes in Childhood Migraine Kato M, Takai K, Ohy a Y . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . 221 . ... Problems of Spontaneous Intracerebral Hematoma in Children -Comparison with Intracerebral Hematoma due to Cerebral Arteriovenous Malformation Nagai K, Ikeda Y, Miki T, A zuma S, Ito H, Miwa T .... . . . . . . . . . . . . .. . , 221 Cerebrovascular Occlusive Disease in Children Fuwa I, Kajiwara H, Yokota A, Matsukado Y, Ishizu M, Teruhisa M, Taku K . . . . . . . . . . . .. . . .. ..... . . . . . . . . . . . . . . . 222 . .. . . .. . A Nine-Year-Old Boy.Who was Diagnosed as Cerebrovascular Disease at a Relatively Early Stage by Serial EEGs Yamada T, Seki T, Kimiya S, Maezawa M, Tachibana Y . . . . . . . . . . . .. . . . 222 . Brain Tumors, Degenerative or Etiology Unknown A Case of Myoclonus Epilepsy Takahashi Y, Nakashima M, Taga T, Terasawa S, Ichihashi H, OrU T, Higashi T. . . . . . . . . . . . . . . . . . . . . . .

102 Brain & Development, Vol 6, No 2,1984

. . ....

. . . . . 223 ......

.

154

Three Cases with Progressive Dystonia and Low Density of Basal Ganglia Toyofuku T, Tamagawa K, Komiya K, Nagashima T, Beppu H, Ishihara K Infantile Neuroaxonal Dystrophy Diagnosed by Peripheral Nerve Biopsy Sasaki K, Tachi N, Shoji R, Shinoda Mo o A Case of Childhood Multiple Sclerosis with Ring-Like Contrast Enhancement on CT Morimoto T, Sano N, Takahashi M, Habara S, Nagao H, Matsuda H, Fukui K, Beppu K, Shoda To o A Case Report of Focal Nodular Myositis Associated with Various Symptoms such as Lipogranuloma Subcutanea, Anterior Mediastinitis and Nodular Masses in Lung and Brain Nobukuni Y, Tsuchiya H, Ohtani Y, Miike T, Matsuda I Vomiting in Newborn Infants - Developmental Study Shim omura C, Ishiwa S, Nakajima K, Suzuki Y A, Ogawa Too A Case of Peculiar White Matter Disease of Childhood Associated with Cortical Blindness, Cortical Deafness and Other Higher Brain Dysfunction Sawamura Y, Matsuura T, Fukushima R, Tashiro K, Takamura H 0

155

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

156

0

157

158

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

XVII.

160

Neuropsychiatric Problems Diagnosis of Delayed Speech Yoshida K, Matsuo M, Kurokawa Too Dexamethasone Suppression Test on Autistic Children Ohno Y, Hoshino Y, Yamamoto T, Watanabe K, Tachibana R, Murata S, Kaneko M, Kumashiro H Psychological Finding in Left-Handed Children with Epilepsy or Brain Dysfunction of Normal Intelligence Okada R, Hasegawa Y, Tachi K Rett Syndrome - A Clinical and Pathophysiological Study Nomura Y, Segawa M, Hasegawa M, Yonezawa M, Tada H o . . . ..... . Electroencephalographic and Computed Cranial Tomographic Findings in Children with Clumsiness or Minimal Brain 0

161

0

162

0

163

0

164

0

0

• • • • • • • •

0

0



0

0

0

0

0

••

0

0

0

0

0

0

••



0



0

0

0

0

0

0

0

0

0

0

••

0

0

0

0



0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

• • • • • • •

0

0

00

0

0

0

0

0

0

0

0

0

0

0

0

0

0

00

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

•• •

•••

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

0

•••

0

0

0

0

0

0

0

0

0

0

0

0

0



0

0

0

223

0

0

0

0

0

0

0

0

0

159

0

0

0

0

0

0

0

0

225

226

0

0

0

0



0





••

0



0

0

••

226

0

0

•••••

224

225

0

0

0

0

0

0

0

0

224

0

227 227 228

Dysfun~tion

Ochiai Y, Soeda A, Kurihara M, Aso M, Yamazaki Y, Tsuzura S ..... 165 The Correlation between Personality Disorders and Soft Neurological Signs or Hyperkinetic Syndrome in Childhood Murata S, Hoshino Y, Kaneko M, Yashima Y, Kumashiro H XVIII. Neurologic Problems in Systemic Diseases 166 The Possibility of Acute Encephalopathy Following Administration of Calcium Hopantenate Sugimoto T, Yasuhara A, Nishida N, Matsumura T, Sakane Y . 167 Two Autopsied Cases of Sudden Death Associated with Calcium Hopantenate Togashi K, Miura Y, Ishiyama S, Konno K, Suzuki H . . . . . . . . . . 168 Transient Multiple High Density Areas Demonstrated by Computed Tomography in a Case of Childhood Leukemia Sano N, Nagao H, Morimoto T, Takahashi M, Habara S, Matsumoto S, Kuzume K, Hayashi M, Matsuda H .... 169 Cranial Computerized Tomography (CCT) and Neuropsychological Findings in Long-Term Survivors of Acute Lymphoblastic Leukemia Hanefeld F, Harten G, Habermalz E, Henze G, Langermann H-J, Stephani U, Riehm H 0

0

•••

0

0

0

••••

0

0

0

0

0

0



0

0



0

0 •••

0

0

0

0

0



•••

•••

•••••

0

•••

0

0

••

• • • • • • • •



0

0



0



0

0

0

0

0

•••

••••

0 ••

0





0

0

228

229

229 230





•••

230

231

Contents 103

170

171

XIX. 172

173 174 175

176 177 178 179 180 181 182 183

184 185 186 187 188

A Boy with SLE Nephritis Showing Symptoms of Degenerative Disorder and Good Response to Methylpredonisolone Pulse Therapy lijima K, Yoshikawa N, Yokoyama S, Kodama S, Matsuo T . . . . . . . . . . . . .. A Surgical Case of an Infant with Intracranial Hemorrhage due to Vitamin K Deficiency Tanji H, Izumi H, Shoda M, Shiro H, Tanaka H . . . . . . . . . . . . . . . . . . . . . . Neurometabolic Disorders Niemann-Pick Disease, Type A: Pathological, Histochemical, Ultrastructural and Biochemical Studies in an Infant and a Fetus Fukuda M, Tanaka A, Iwamura C, Isshiki G, Nakamura S, Hase Y, Kobayashi Y, Matsumoto M, Kitagawa Y, Morihana Y . . . . . . . . . . . . . . . . .. Prenatal Metachromatic Leucodystrophy -Pathological Findings Okuno A, Eto Y, Maekawa K, Oyanagi S . . . . . . . . . . . . . . . . . . . . . . . . . .. Clinical Manifestations of Galactosialidosis limori Y, Okamura Y, Sakuraba H, Yamanaka T, Suzuki Y. . . . . . . . . . . . . .. Mental Development and Behavior Disorders in Histidinemic Children Yamamoto H, Fukuda Y, Osasa Y, Hase Y, Tsuruhara T, Oura T, Matsui S, Takesada M .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Heterogeneity in I-Cell Disease Okada S, Kato T, Yutaka T, Midorikawa M, Yabuuchi H . . . . . . . . . . . . . . . . Long-Term Follow-Up of Ten Patients with Lesch-Nyhan Syndrome Mizuno T .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Clinical and Biochemical Studies of 25 Patients with Lactic Acidosis and Progressive Neurological Deterioration Miyabayashi S, Narisawa K, Tada K, Kobayashi Y. . . . . . . . . . . . . . . . . . . . . Carnitine Deficiency Differentiated from Reye Syndrome Sugiyama N, Morishita H, Maki N, Ogawa Y, Wada Y, Yamamoto M, Hosoe A, Nishimura Y, Kobayashi S, Kamoshita S . . . . . . . . . . . . . . . . . . . . Cell Biological Diagnosis of Ataxia-Telangiectasia Katsura T, Ohta S, Shimada M, Shima A, Chishiro H, Kasahara Y . . . . . . . . . . Has Wilson Disease been Solved? Kaneko Y, Kaneko H, Kumashiro H . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. The Effects of Thyrotropin-Releasing Hormone on Monoamine Metabolism in Children with Nonprogressive Ataxic Syndrome Haraguchi H, Une Y, Hata M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. Metabolic Changes of Catecholamines after L-DOPA Therapy in Hereditary Progressive Dystonia Shimoyamada Y, Ohmori H, Shiga S, Kawamura H, Kashii H, Kihara S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . " Plasma Catecholamine Levels at Syncope in Children Sumi K, Ito N, Nagaura T, Goto M, Yamatodani A . . . . . . . . . . . . . . . . . . .. Effect of the Ketogenic Diet on Brain Monoamine Metabolism in Young Mice Otani K, Mimaki T, Yabuuchi H, Yamatodani A, Wada H . . . . . . . . . . . . . . .. Report of Two Sisters with Perinatal Sudanophilic Leucodystrophy Hanawa H, Isoyama K, Kobayashi E, Ishikawa A, Yokoi S . . . . . . . . . . . . . .. Cases of Fabry Disease in a Family Nakamura H, Maehara M, Sato C, Mizutani N, Watanabe K . . . . . . . . . . . . . Two Siblings with f3-Galactosidase-Neuraminidase Deficiency Whose Clinical Symptoms Appeared during Preschool Years Takeda E, Kuroda Y, Tomita T, Kobashi H, Ito M, Watanabe T, Hashimoto T, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ..

104 Brain & Development, Vol 6, No 2, 1984

231 232

232 233 233

234 234 235 235 236 236 237 237

238 238 239 239 240 . .

240

189

190 191 192

193 194 195 196

197 198

199 XX.

200

201 202 203 204 XXI.

205

Enzymatic Studies of Sialidase in a Case of Severe Infantile Sialidosis with Apparently Normal Sialidase Activity Yutaka T, Okada S, Kato T, Nishigaki M, Oshima T, Yabuuchi H, Sugino M, Yamano K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Reye-Like Syndrome with Dicarboxylic Aciduria Woo M, Hirabayashi Y, Yoshino M, Ebina R, Nakano H, Sugimoto T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Citrullinemia been Treated as an Epilepsy Origuchi Y, Ushijima T, Sakaguchi M, Miyoshino S, Matsuda I . . . . . . . . . . . A Female Case of Ornithine Transcarbamylase Deficiency with Marked Abnormalities of Brain Computed Tomographic Scan Takayanagi M, Ohtake A, Ogura N, Hoshino M . . . . . . . . . . . . . . . . . . . . . . A Case of Partial Defect of Pyruvate Dehydrogenase Hayashi M, Tatsuno M, Misugi N, Iwamoto H, Koike Y, Koike M . . . . . . . . . . Ultramicroscopic Findings of Lymphocytes in Congenital Metabolic Disease Ito N, Monma N, Satodate R, Ito M, Fujiwara T . . . . . . . . . . . . . . . . . . . . . A Case of Congenital Lactic Acidosis without Enzyme Deficiency Itakura Y, Tsuchiyama A, Tamura Y, Tachi N, Tsukazaki H, Oyanagi K, Nakao T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case Study of Schizophrenia with Wilson Disease, and D-Penicillamine Treatment during Pregnancy Watanabe Y, Okumura K, Sasamoto A, Aoki T . . . . . . . . . . . . . . . . . . . . . . A Case of Menkes Kinky Hair Syndrome with Recurrent Subdural Hematoma Hanaoka Y, Sakai A, Kurata S, Mizuno M . . . . . . . . . . . . . . . . . . . . . . . . . Lowe Syndrome with Infantile Spasms which Showed Clinical and Electroencephalographical Improvement after Alkali Therapy -Introduction of a Newly Developed Technique for Biochemical Diagnosis of Lowe Syndrome Kimiya S, Seki T, Yamawaki H, Suzuki N, Maezawa M, Yamada T, Tachibana Y, Matsuo N . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Two Cases of Adrenoleukodystrophy Diagnosed by Increased Serum Hexacosanoic Acid Ida H, Eto T, Maekawa K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

.

241

.

241

.

242

.

242

.

243

.

243

.

244

.

244

.

245

.

245

.

246

.

246

.

247

.

247

.

248

.

248

Progressive Muscular Dystrophy Lipid Compositions of Plasma, Erythrocyte and Muscle in a Patient and His Mother with Myotonic Dystrophy Terai K, Ito M, Oyama K, Fujiwara T, Monma N, Ito N . . . . . . . . . . . . . . . . .

249

Spinal Cord, Peripheral Nerves & Myasthemia Four Cases of Hereditary Motor and Sensory Neuropathy in Childhood - Light and Electron Microscopic Studies of Sural Nerve Biopsies Sugie Y, Hara M, Sugama M, Kosaka S, Shibuya T, Sugie H, Shishikura K, Ohsawa M, Suzuki H, Fukuyama Y . . . . . . . . . . . . . . . . . . . . Three Cases of Hereditary Motor and Sensory Neuropathy in a Family (HMSN Type III) Kudo H, Terada M, Abe T, Naito H, Nihei K, Nonaka I . . . . . . . . . . . . . . . . A Case of Congenital Hypomyelination Polyneuropathy Asoh S, Takemura T, Inoue K, Misugi N . . . . . . . . . . . . . . . . . . . . . . . . . . Sensory and Motor Neuropathy in Childhood Misugi N, Iwamoto H, Hayashi M, Miyake S . . . . . . . . . . . . . . . . . . . . . . . . Congenital Myasthenia Associated with Skeletal Abnormalities and Cataract in Two Sisters Okabe I, Kobayashi S, Kamoshita S . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

Contents 105

206

207

208 209

Rehabilitation of Floppy Infants-with Special Reference to Congenital Muscular Dystrophy Including Fukuyarna Type Muscular Dystrophy Yamagata Y, Fujimoto T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . Histopathological Study of Muscle Biopsies in Fukuyama Type Congenital Muscular Dystrophy (FCMD) - Special Reference to Calcium Staining by Kashiwa's Method Suyama A, Yabe K, Sugie Y, Sugie H, Osawa M, Suzuki H, Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . A Case of Duchenne Muscular Dystrophy with Ehlers-Danlos Syndrome Suganuma I, Sato C, Une Y, Haraguchi H, Saeki M . . . . . . . . . . . . . . . . . . Delayed Muscle Fiber Type Differentiation in a Case of Congenital Myotonic Dystrophy Shikura K, Inukai K, Yoshizawa K, Nonaka I . . . . . . . . . . . . . . . . . . . . . .

..

249

..

250

..

250

"

251

XXII. Metabolic and Other Muscle Disorders 210 The Panorama of Atypical Types of Muscular Diseases in the San-In District of Japan Tanaka T, Takeshita K, Kasagi S, Nakashima T . . . . . . . . . . . . . . . . . . . . . .. 211 Modulation of Fiber Type Differentiation in Rat Soleus Muscle during Neonatal Hypothyroidism Sugie H, Sugie Y, Ohsawa M, Suzuki H, Fukuyama Y . . . . . . . . . . . . . . . . .. 212 Treatment of Polymyositis in Childhood with Particular Reference to Corticosteroid-Resistant Cases Yoshioka M, Kuroki S, Mizue H, Okuno T. . . . . . . . . . . . . . . . . . . . . . . . .. 213 Hereditary Peroneal Muscle Atrophy Presenting Severe Congenital Club Foot in Mouse Toyofuku T, Nonaka I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 214 Urinary Excretion of 3-Methylhistidine in Children with Neuromuscular Diseases Kumagai T, Takeuchi T, Hara K, Miyazaki S, Shionoya T, Watanabe K, Komatsu K, Mizutani N . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 215 Histochemical Study of Biopsied Muscles from Nemaline Myopathy and Congenital Fiber Type Disproportion - Behavior of Type 2B Fibers Miike T, Tamari H, Ohtani Y, Ishitsu T, Matsuda I, Miyoshino S, Une Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216 Biochemical Properties of Acetylcholine Receptor Purified from Human Muscle Momoi MY, Lennon VA. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 217 AMP Deaminase of Biopsied Muscles from Patients with Neuromuscular Disorders Nagao H, Morimoto T, Sano N, Takahashi M, Kida K, Matsuda H . . . . . . . . . . 218 A Case of Congenital Myopathy with Marked Type II Muscle Fiber Hypoplasia Kuroki S, Ohkura K, Yoshioka M, Mizue H, Itagaki Y . . . . . . . . . . . . . . . . .. 219 Congenital Myopathy with Histological Characteristics of Muscle Fiber Immaturity Nonaka I, Fujita T " . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 220 Histochemical Analysis of Fiber Type Distribution and Fiber Diameters in Normal Infants Saito Y, Nonaka I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221 Successful Carnitine Treatment in Two Siblings of Lipid Storage Myopathy with Hypertrophic Cardiomyopathy Matsuishi T, Terasawa K, Hirata K, Yoshino M, Nonaka I, Sugiyama N, Ohta K, Ohtaki E, Hirose F . . . . . . . . . . . . . . . . . . . . . . . . . ..

106 Brain & Development, Vol 6, No 2, 1984

251 252 252 253

253

254 254 255 255 256 256

257

222

223 224 225 226

XXIII .

227

228 229

Ataxia Telangiectasia: Reduced Pyruvate Dehydrogenase Activity with Lipid Storage in Type 1 Muscle Fibers Terasawa K, Matsuishi T, Ishihara 0, Sakaguchi Y, Sugiyama N . . . . . . . . . . . Acute Fulminant Viral Myositis Associated with Encephalitis and Myoglobinuria Ohtani Y, Miike T, Ishitsu T, Matsuda I, Migita M, Nishihara S .. ......... A Case of Myositis Following Measles Infection in a Boy with Cataract and Retinal Pigmentary Degeneration Kobayashi S, Kamoshita S, Nihei K ..... .... ..... . . . . . . . ...... .. A Case of Probable Muscle Glycogenosis Associated with Dilated Cardiomyopathy Fujita T, Fujino 0, Hashimoto K, Hirayama T, Ueda Y, Nonaka I . . . . . . . . . Two Females with Congenital Myopathy with Obesity, Marked Fatty Infiltration, Mild Myogenic Change and Abnormal Fiber-Type Distribution Y okochi K, Kodama K, Nonaka I . . . . . . . . . . . . . . .. . . . . . . . . . . . ...

.

257

.

258

.

258

.

259

.

259

Therapeutic Trials Clinical and Neuroendocrinological Evaluation of Thyrotropin Releasing Hormone Tartrate in the Treatment of Psychologic Disorders of Childhood and Adolescence Kawano N, Miyao M ... . . . . . . . . . . . . . . . . . . . . . . . . . ... . . . . . . . . . Effectiveness of Pemoline in Attention Deficit Disorder Hara H, Ohta M, Shimizu Y, Nishihara K, Koda Y .. .. . ... ... .. . .. .... . Therapeutic Trial of TRH-Tartrate for Consciousness Disturbance in Children Abe T, Terada M, Kudo H, Naito H, Nihei K .. ...... ..... ...... . ... .

260 260 261

Contents 107