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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 109

Molecular Physiology of Muscle Contraction Ebashi S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 134

GUEST LECTURE

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

EDUCATIONAL LECTURES

Type I Familial Amyloidotic Polyneuropathy (Japanese Type)

119

Araki S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 128 Functional as well as Pathophysiological Aspects. of the Basal Ganglia

YoshidaM............................................... 135

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

136

138

Sakamoto Y, Watanabe K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 139 Diagnosis of Neonatal Central Nervous System Infections

ToriiS ................................................. 139 Diagnosis of Neonatal Bacterial Meningitis

Imura S, Takada M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 140 Two-dimensional Ultrasonography of the Brain in Infancy:

Its Application to the Diagnosis of Intracranial Disorders Yano S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 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 ...... .... . .... ............... 141

Neurophysiological Examinations for the Diagnosis of Perinatal Brain Damage Watanabe K, Inokuma K, Takeuchi T, Negoro T . . . . . . . . . . . . . . . . . . . . .. 142

Electrophysiological Diagnosis in Newborn; Clinical Applications of Auditory Brainstem Response and Blink Reflex Yasuhara A, Yamada A, Kinoshita Y, Matsumura T. . . . . . . . . . . . . . . . . . . . 143

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

Treatment and Prognosis of Intracranial Hemorrhage and Asphyxia in Neonates Sugimoto T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 144

FREE PAPERS

1. Experimental Developmental Neurobiology Regional Membrane Phospholipid Composition in Developing Rat

Brain after Premature Birth Yokota K, Yamaguchi T .... ............... .. . . .............. 146

2 Effect of Theophylline on Myelination in the Brain of Young Rats Kamigaki M, Sunaga S, Sato Y, Okuyama K . . . . . . . . . . . . . . . . . . . . . . . .. 146

3 Enzyme-Linked Immunoadsorbent Assay for Myelin Basic Protein Tanaka K, Tomida N, Tanaka M, Sasahara A, Yamamoto H, Shimada M .............................................. 147

4 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

5 Effects of Phenytoin on Mouse Purkinje Cells-Comparison between Infantile and Adult Mice Suzuki M, Tamai!, Ohta H, Maekawa K ...... . . . ................. , 148

6 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

7 Experimental Study on CNS Toxicity of Vincristine Tomiwa K, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 149

8 Effects of Early Malnutrition and Rehabilitation on Brain Development -Golgi-Cox Study Yoshida A, Yoshioka H, Kasubuchi Y, Kusunoki T . . . . . . . . . . . . . . . . . . . . 149

9 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

II. Human Development 10 Posture of Kindergarten Children and Its Relationship to Behavioral

Problems Yuge M, Kanda T, Yamori Y. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 150

11 Latency and Persistence of Fersen Reflex in Infants Futagi Y, Ohtani K, Tanaka J, Shimizu H . . . . . . . . . . . . . . . . . . . . . . . . . . 151

12 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 The Passive Range of Motions in Developmentally Disabled Children Kisa T, Nakao Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152

14 Method of Feeding in Infancy and the Psychomotor Development at Preschool Age Mukai Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 152

15 An Attempt at Systematic Understanding of Benign Hypertonia and Dystonia in Infancy Kitazumi E, Enomoto S, Yokochi A, Yokochi K, Uchida T, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 153

16 Effects of Neonatal Hypoxia on Brain Development III. Behavioral Changes due to Total Asphyxia Yoshioka H, Ochi M, Yoshida A , Iino S, Fujiwara K, Ishimura K, Nishimura 0, Kusunoki T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 153

17 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

III. Neurophysiological Diagnostics 18 A Study on Pathophysiology of Myoclonus - Polysomnographical

Examination Yonezawa M, Tada H, Hasegawa M, Nomura Y, Segawa M, Yamashita K, Tanaka S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 154

19 An Electroencephalographic Study on Uncommon Chromosomal Abnormalities Nishibayashi N, Inouye H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 155

20 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

21 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

22 Analysis of Swallowing Disturbance in Cerebral Palsy by Superficial EMG and Respiratory Curve Analysis Hirayama Y, Takagi A , Wash ida T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 156

23 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

24 Normal Conduction Velocity of the Tibial Nerve in Children Abe J, Mimaki T, Onoe S, Tagawa T, Ono J, Yabuu chi H, Sugita T .. ....... 157

25 Topographic Mapping of Brainstem Auditory Evoked Responses in Humans Shimizu N, Hirasawa K ................ . ... . ................. 158

26 Four Cases of Prenatal Brain Stem Damage Identified by Auditory Brain Stem Responses Uchida T, Kitazumi E, Yokochi K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 158

27 A Visual Evoked Potential Study in Sturge-Weber Syndrome Iinuma K, Suzuki K, Ich inohasama Y, Hayamizu S . . . . . . . . . . . . . . . . . . . . 159

28 A Clinical Study of Short Latency SEP in Patients with Brain Dysfunction Kasagi S, Takakura H ..... . ... .. .. ... ..... . . ........ . .... ... 159

29 Developmental Change of Interhemispheric Coherence in Children Tomita Y, Kato E, Mori C, Abe K .... .. . . . . . . . . . . . . . . . . . . . . . . . .. 160

30 Polysomnography on Lissencephaly Hiura K, Hashim oto T, Fukuda K, Endo S, Tayama M, Mori A, Fujino K, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 160

Contents 95

31 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

32 Head Injuries and Electroencephalographic Findings Sogawa H, Ohara T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161

33 Developmental Study of the T-Wave of Achilles Tendon Reflex in Children Takakura H, Kasagi S ....................................... 162

34 The Auditory Evoked Palpebral Reflex: Clinical Application to Neonatal Brain Dysfunction Yamada A, Yasuhara A, Kinoshita Y, Matsumura T, Yasuhara M . . . . . . . . . . . 162

35 An Electrophysiological Study of Early-Infantile Epileptic Encephalopathy with Suppression-Bursts Yasuhara A, Yamada A, Kinoshita Y, Sugimoto T, Matsumura T . . . . . . . . . . . 163

36 Somatosensory Evoked Potentials and Auditory Brainstem Responses in Diabetes Mellitus Takeda K, Murata R, Matsuoka 0, Kin M, Aono S, Tani M, Isshiki G . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163

37 The Auditory Brainstem Response (ABR) in the Cat: Effects of Lesions Nakano S, Okuno T, Mikawa H, Kudo M . . . . . . . . . . . . . . . . . . . . . . . . . . . 164

38 Hemispheric Lateralization of Flash YEP Maps of Some Neurological Disorders in Pediatrics Hirasawa K, Shimizu N ......... . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 164

39 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165

40 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . 165

41 Developmental Changes of Topographic EEG Features in the Newborn Inokuma K, Watanabe K, Negoro T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166

42 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 ................................................ 166

43 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 .............................. 167

44 Development of Sleep Spindles in Normal Children - With Reference to Change in Their Distribution Miyajima T, Ogihara M, Hoshika A, Matsuno T, Honda T . . . . . . . . . . . . . . . . 167

45 The Effect of Neonatal Hyperbilirubinemia on the Auditory Brainstem Response Kuriyama M, Konishi Y, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168

46 Short-Latency Somatosensory Evoked Potentials (SSEP) in Pediatric Neurological Disorders - Degenerative Diseases Nakagawa Y, Kataoka K, Hojo H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168

47 On a Quantitative Method to Measure Cerebral Blood Flow in Newborn Infants (I) Ishiwa S, Gotoh K, Ono Y, Ogawa T ............................. 169

48 Auditory Brainstem Evoked Responses in Down Syndrome Miyao M, Yamamoto Y, Kamoshita S, Nihei K . . . . . . . . . . . . . . . . . . . . .. . . 169

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

IV. Neuroradiological Diagnostics 49

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V. 55

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VI. 63

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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 L VW /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 ................................................ .

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Contents 97

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VII. 68

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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 ...................................... .

VIII. Intellectual, Motor and Sensory Disturbances 71 Educational Attendance of Handicapped Children in the Suburban

Area of Tokyo

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Tohjo M, Ohtsuka M, Nagase M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182 72 Early Signs of Spastic Diplegia: Comparative Analysis on Video

Recording Kitazumi E, Enomoto S, Yokochi A, Yokochi K, Uchida T, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 182

73 Clinical Features for Developing Cerebral Palsy Kanda T, Yuge M, Suzuki J, Yamori Y, Fukase H . . . . . . . . . . . . . . . . . . . . . 183

74 An Investigation of Cerebral-Palsied Children in Regular Classrooms GakuhariH, Kodama K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 183

75 A Study on the Pathophysiology of Sudden Death in Severely-Handicapped Children Kono C, Oka E, Inoue H, Miyake S, Iyoda K, Ohtahara S . . . . . . . . . . . . . . . . 184

76 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 .......... 184

77 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185

78 A Statistical Study on Resident Deaths at Institutions for the Profoundly Retarded Nakamura H, Honda T, Uemura I, Nanba Y, Mita K, Okafa K . . . . . . . . . . . . . 185

79 On the Development of Mental Activity in the Monozygotic Twins with Early Infantile Brain Injury Hasegawa Y, Okada R, Tachi K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 186

IX. Convulsive Disorders (1): Febrile or Non-Epileptic 80 Etiological Aspects of Epilepsy and Febrile ConVUlsions-Pregnancy

and Perinatal Factors Katafuchi Y, Aoki N, Matsuishi T, Yamashita F .................. . .. , 186

81 Follow-Up Study of Children with Simple Febrile Convulsions and Epileptiform EEG Activities without Chronic Anticonvulsant Medication Kajitani T, Kimura T, Ide K ................... - - - - . - ..... - . . .. 187

82 Clinical and Electroencephalographic Study on Siblings of Patients with Epilepsies or Febrile Convulsions Exhibiting Rolandic Discharges Nakamura M, Kajitani T, Kumanomidou Y, Kimura T . . . . . . . . . . . . . . . . . . 187

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

83 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 ...................................... 188

84 Apnea Following Hyperventilation in Severely Mentally Retarded Children Miyazaki S, Hara K, Kumagai T, Takeuchi T, Hakamada S, Watanabe K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 188

85 A Case of Familial Paroxysmal Choreoathetosis Endo S, Suzue J, Hiura K, Kawano N, Hashimoto T, Tamura Y, Miyao M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 189

86 Epilepsy Following Febrile Convulsions Hirata Y, Somiya K, Nakano M, Ikuma M . . . . . . . . . . . . . . . . . . . . . . . . .. 189

X. Convulsive Disorders (2): Epilepsy - Clinical 87 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. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 190

88 A Long-Term Follow-Up Study of "Cryptogenic" Status Convulsivus in Childhood A waya Y, Fukuyama Y, Iwamoto H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 190

89 Visual Epileptic Seizures after Mild Head Injuries in Children Tamagawa K, Sasaki H, Aoki N, Shimizu H . . . . . . . . . . . . . . . . . . . . . . . .. 191

90 Headache and Convulsive Disorders: A Study on Pathophysiological Correlations Tada H, Goto M, Yonezawa M, Nomura Y, Segawa M . . . . . . . . . . . . . . . . . . 191

91 Behavioral Problems in Children with Epilepsy Matsuo M, Kurokawa T, Takaki S, Chen Y-J, Yoshida K ................ 192

92 A Floppy Child with Atonic Attacks Kuwajima K ............................................. 192

93 Risk of Seizures in Siblings of Children with Epilepsy Soda M, Nakai M, Takeshita K ................................. 193

94 Focal and Clonic Convulsions in Infancy Yokochi K, Uchida T ....................................... 193

95 Ictal EEG in Benign Epilepsy of Childhood with Rolandic Discharges Kumanomidou Y, Kajitani T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194

96 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194

97 Accidental Large Overdose of Sodium Valproate-A Case Report Horii Y, Ishimura K, lino S, Yoshida A, Ochi M, Yoshioka H, Kusunoki T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 195

98 A Case of Report of Valproic Acid-Induced Pure Red Cell Aplasia Sugano T, Tominaga M, Kasubuchi Y, Imashuku S . . . . . . . . . . . . . . . . . . . . 195

XI. Convulsive Disorders (3): West or Lennox Syndrome 99 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196

lOO Neuropathology of Infantile Spasms Satoh J, Mizutani T, Morimatsu Y, Tomimoto M, Sakamoto K, Shinohara T, Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 196

101 A Study on ACTH Therapy of Infantile Spasms 2nd Report Concerning Synthetic ACTH Hashimoto K, Tamamoto A, Ozaki N, Konishi K, Takaya K ............. , 197

Contents 99

102 Suppressive Effect of ACTH on the Serum Thyroxine, Level and Its Influence on the Clinical Effectiveness in Infantile Spasms Izumi T, Fukuyama Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 197

103 Effects of ACTH on Sleep of Patients with West Syndrome Ogiso M, Shimohira M, Kohyama J, Niwa T, Iwakawa Y, Segawa M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 198

104 Gamma-Globulin Therapy in Infantile Spasms Shiihara H, Michihiro N, Ogawa K, Coto K, Suzuki H, Utsumi Y, Ariizumi M, Baba K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 198

105 A Study of Treatment of Lennox-Gastaut Syndrome Watanabe Y, Sato S, Okumura K, Aoki T . . . . . . . . . . . . . . . . . . . . . . . . .. 199

106 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 ................ 199

XII. Convulsive Disorders (4): Treatments 107 Clorazepate Therapy for Refractory Seizures in Children

Mimaki T, Abe J, Onoe S, Tagawa T, Ono J, Yabuuchi H, Kamio M. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 200

108 Pharmacokinetics of Rectal Administration of Diazepam Solutions in Infants with Febrile Convulsions Miura H, Minagawa K, Yagi J ... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 200

109 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 201

110 Lidocaine for the Treatment of Status Epilepticus Miyata H, Tsubota T, Kuroda E, Takemoto H, Kanazawa Y, Ogino H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 201

111 Reevaluation of the Indication of Sodium Valproate Monotherapy in Childhood Epilepsy - Compared with Phenobarbital Monotherapy Hosaka S, Ogiso M, Iwakawa Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 202

112 Sodium Valproate and Clonazepam in the Treatment of Intractable Epilepsies Oki J, Tasaki T, Cho K, Yoshioka H . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 202

113 Abnormal Breath Test Results with 13C-Palmitate in Patients Treated with Valproic Acid Sakuragawa N, Suehiro M, Watanabe H . . . . . . . . . . . . . . . . . . . . . . . . . . .. 203

114 Serum Protein Binding of Phenobarbital in Childhood Tanaka J, Shimizu H, Futagi Y, Suzuki K . . . . . . . . . . . . . . . . . . . . . . . . .. 203

115 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 ...... . . . . . . . . . . . . . . . .. 204

116 Physical and Mental Development of Infants Born to Mothers Taken Antiepileptic Drugs Nomura Y, Takebe Y, Kaneko S . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 204

117 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 .............. 205

118 Relationship between Valproate Pharmacokinetics and Laboratory Data in Epileptic Children in the Steady-State Takebe Y, Haneda S, Kimura S, Koide N. . . . . . . . . . . . . . . . . . . . . . . . . .. 205

119 Study of Serum Immunoglobulins in Childhood Epilepsy Hibio S, Michihiro N, Ogawa K, Ohkubo 0, Utsumi Y, Ariizumi M, Baba K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 206

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Antico nvulsan ts Follow-Up Study of Plasma Ammonia Levels in Patients Treated

with Valproic Acid (VP A) Yoshimoto T, Harima Y ..................................... .

Sodium Valproate and Hyperammonemia Nishida N, Ono A, Yasuhara A, Ito N, Sugimoto T, Matsumura T, Sakane Y, Taniuchi K ............................ .

The Effects of Valproic Acid and Phenobarbital on Free Amino Acid Levels in Serum and Urine Honda T, Watanabe K, Sato Y, Tada H, Takahashi H, Kaneko K, Takahashi K, Ohtsuka C ..................................... .

Measurement of Free Phenobarbital Blood Levels Using an Amicon Centriflo Model CF 25 (Ultrafiltration Method) Fujii E, Okada K, Matsutaka K, Somqa T, Teshirogi T ................ .

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

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 ....................................... .

CNS Infections 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 Kataoka K, Nakagawa Y, Hojo H, Yoshioka H, Sawada T ........ . ...... .

Induction of Acute Myoclonic Encephalitis in Hamsters by SSPE Virus 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 Diagnosis and Treatment Kiyohara K, Nakajima M, Nonomiya S, Hayashi M, Sekino T, Ogiso M, Arai S, Iwakawa Y .................................. .

The Early Diagnosis of Herpetic Encephalitis - Analysis of 14 Cases Hamano K, Ponsot G, Plouin P ................................ .

Measles Encephalitis in Two Immunized Children Kurashige T, Tone Y, Kuramitsu M, Kitamura I ..................... .

Subacute Encephalitis Associated with Elevated HSV-l Titer Kawano Y, Ohya T ........................................ .

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, Koizumi F . ............................................. .

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

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138 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 . . . . . . . . . . . . . . . . . . . . . . . 215

139 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216

140 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216

141 Three Cases of Brainstem Encephalitis Miura J, Tachi N, Nakao T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 217

XV. Cerebrovascular Disorders 142 Clinical Features of Moyamoya Disease: Differences from Acute

Hemiplegia without the Moyamoya Vascular Network Kurokawa T, Chen Y-J, Kishikawa T, Kitamura K . . . . . . . . . . . . . . . . . . . . . 217

143 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

144 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

145 Correlation between CT Findings and Intelligence Quotient in Pediatric Patients with Moyamoya Disease Fukumoto T, Matsushima Y, Suzuki R, Hiratsuka H, Inaba Y . . . . . . . . . . . . . 219

146 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

147 100 Cases of Acute Hemiplegia in Childhood Okuno T, Hattori H, Takao T, Ito M, Konishi Y, Nakano S, Mikawa H . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 220

148 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

149 Studies on Ischemic Changes in Childhood Migraine Kato M, Takai K, Ohy a Y . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 221

150 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

151 Cerebrovascular Occlusive Disease in Children Fuwa I, Kajiwara H, Yokota A, Matsukado Y, Ishizu M, Teruhisa M, Taku K . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 222

152 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

XVI. Brain Tumors, Degenerative or Etiology Unknown 153 A Case of Myoclonus Epilepsy

Takahashi Y, Nakashima M, Taga T, Terasawa S, Ichihashi H, OrU T, Higashi T. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 223

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Three Cases with Progressive Dystonia and Low Density of Basal Ganglia Toyofuku T, Tamagawa K, Komiya K, Nagashima T, Beppu H, Ishihara K 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

Infantile Neuroaxonal Dystrophy Diagnosed by Peripheral Nerve Biopsy Sasaki K, Tachi N, Shoji R, Shinoda Mo o 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

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 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

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 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0 0

Vomiting in Newborn Infants - Developmental Study Shim omura C, Ishiwa S, Nakajima K, Suzuki Y A, Ogawa Too 0 0 0 0 0 0 0 0 0 0 0 0 0

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 0 0 0 0 0 0 0 0 0

Neuropsychiatric Problems Diagnosis of Delayed Speech

Yoshida K, Matsuo M, Kurokawa Too 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

Dexamethasone Suppression Test on Autistic Children Ohno Y, Hoshino Y, Yamamoto T, Watanabe K, Tachibana R, Murata S, Kaneko M, Kumashiro 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

Psychological Finding in Left-Handed Children with Epilepsy or Brain Dysfunction of Normal Intelligence Okada R, Hasegawa Y, Tachi K 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 ••• •••••

Rett Syndrome - A Clinical and Pathophysiological Study Nomura Y, Segawa M, Hasegawa M, Yonezawa M, Tada H o . . . ..... . 0 •••••••• • •• •• • • 0 •• • ••••••• • ••• ••• • 0 • • ••

Electroencephalographic and Computed Cranial Tomographic Findings in Children with Clumsiness or Minimal Brain Dysfun~tion Ochiai Y, Soeda A, Kurihara M, Aso M, Yamazaki Y, Tsuzura S ..... 0 • 0 0 ••

The Correlation between Personality Disorders and Soft Neurological Signs or Hyperkinetic Syndrome in Childhood Murata S, Hoshino Y, Kaneko M, Yashima Y, Kumashiro H 0 0 ••• 0 • 0 ••• •••

Neurologic Problems in Systemic Diseases The Possibility of Acute Encephalopathy Following Administration

of Calcium Hopantenate Sugimoto T, Yasuhara A, Nishida N, Matsumura T, Sakane Y . 0 ••••• 0 0 0 •••

Two Autopsied Cases of Sudden Death Associated with Calcium Hopantenate Togashi K, Miura Y, Ishiyama S, Konno K, Suzuki H .......... 0 •• 0 ••• 0 •

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 .... 0 0 •••••••• •••• • 0 •

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 • •••

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170 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 . . . . . . . . . . . . .. 231

171 A Surgical Case of an Infant with Intracranial Hemorrhage due to Vitamin K Deficiency Tanji H, Izumi H, Shoda M, Shiro H, Tanaka H ...................... 232

XIX. Neurometabolic Disorders 172 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 . . . . . . . . . . . . . . . . .. 232

173 Prenatal Metachromatic Leucodystrophy -Pathological Findings Okuno A, Eto Y, Maekawa K, Oyanagi S . . . . . . . . . . . . . . . . . . . . . . . . . .. 233

174 Clinical Manifestations of Galactosialidosis limori Y, Okamura Y, Sakuraba H, Yamanaka T, Suzuki Y. . . . . . . . . . . . . .. 233

175 Mental Development and Behavior Disorders in Histidinemic Children Yamamoto H, Fukuda Y, Osasa Y, Hase Y, Tsuruhara T, Oura T, Matsui S, Takesada M .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 234

176 Heterogeneity in I-Cell Disease Okada S, Kato T, Yutaka T, Midorikawa M, Yabuuchi H . . . . . . . . . . . . . . . . 234

177 Long-Term Follow-Up of Ten Patients with Lesch-Nyhan Syndrome Mizuno T .. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 235

178 Clinical and Biochemical Studies of 25 Patients with Lactic Acidosis and Progressive Neurological Deterioration Miyabayashi S, Narisawa K, Tada K, Kobayashi Y. . . . . . . . . . . . . . . . . . . . . 235

179 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 .................... 236

180 Cell Biological Diagnosis of Ataxia-Telangiectasia Katsura T, Ohta S, Shimada M, Shima A, Chishiro H, Kasahara Y .......... 236

181 Has Wilson Disease been Solved? Kaneko Y, Kaneko H, Kumashiro H . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 237

182 The Effects of Thyrotropin-Releasing Hormone on Monoamine Metabolism in Children with Nonprogressive Ataxic Syndrome Haraguchi H, Une Y, Hata M . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 237

183 Metabolic Changes of Catecholamines after L-DOPA Therapy in Hereditary Progressive Dystonia Shimoyamada Y, Ohmori H, Shiga S, Kawamura H, Kashii H, Kihara S .............................................. " 238

184 Plasma Catecholamine Levels at Syncope in Children Sumi K, Ito N, Nagaura T, Goto M, Yamatodani A . . . . . . . . . . . . . . . . . . .. 238

185 Effect of the Ketogenic Diet on Brain Monoamine Metabolism in Young Mice Otani K, Mimaki T, Yabuuchi H, Yamatodani A, Wada H . . . . . . . . . . . . . . .. 239

186 Report of Two Sisters with Perinatal Sudanophilic Leucodystrophy Hanawa H, Isoyama K, Kobayashi E, Ishikawa A, Yokoi S . . . . . . . . . . . . . .. 239

187 Cases of Fabry Disease in a Family Nakamura H, Maehara M, Sato C, Mizutani N, Watanabe K . . . . . . . . . . . . . . . 240

188 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 ....... . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 240

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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 .................................... .

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 ............................. .

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 ................ .

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Contents 105

206 Rehabilitation of Floppy Infants-with Special Reference to Congenital Muscular Dystrophy Including Fukuyarna Type Muscular Dystrophy Yamagata Y, Fujimoto T . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 249

207 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 . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 250

208 A Case of Duchenne Muscular Dystrophy with Ehlers-Danlos Syndrome Suganuma I, Sato C, Une Y, Haraguchi H, Saeki M . . . . . . . . . . . . . . . . . . .. 250

209 Delayed Muscle Fiber Type Differentiation in a Case of Congenital Myotonic Dystrophy Shikura K, Inukai K, Yoshizawa K, Nonaka I ...................... " 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 . . . . . . . . . . . . . . . . . . . . . .. 251

211 Modulation of Fiber Type Differentiation in Rat Soleus Muscle during Neonatal Hypothyroidism Sugie H, Sugie Y, Ohsawa M, Suzuki H, Fukuyama Y ............. . . . .. 252

212 Treatment of Polymyositis in Childhood with Particular Reference to Corticosteroid-Resistant Cases Yoshioka M, Kuroki S, Mizue H, Okuno T. . . . . . . . . . . . . . . . . . . . . . . . .. 252

213 Hereditary Peroneal Muscle Atrophy Presenting Severe Congenital Club Foot in Mouse Toyofuku T, Nonaka I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 253

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 . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 253

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 ................................................. 254

216 Biochemical Properties of Acetylcholine Receptor Purified from Human Muscle Momoi MY, Lennon VA. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 254

217 AMP Deaminase of Biopsied Muscles from Patients with Neuromuscular Disorders Nagao H, Morimoto T, Sano N, Takahashi M, Kida K, Matsuda H .......... 255

218 A Case of Congenital Myopathy with Marked Type II Muscle Fiber Hypoplasia Kuroki S, Ohkura K, Yoshioka M, Mizue H, Itagaki Y . . . . . . . . . . . . . . . . .. 255

219 Congenital Myopathy with Histological Characteristics of Muscle Fiber Immaturity Nonaka I, Fujita T " . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. 256

220 Histochemical Analysis of Fiber Type Distribution and Fiber Diameters in Normal Infants Saito Y, Nonaka I . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 256

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 . . . . . . . . . . . . . . . . . . . . . . . . . .. 257

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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 ............. . .. . .......... ... .

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 .. ...... ..... ...... . ... .

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