Paediatric Neurology, Second Edition presents management guidelines of neurological disorders in infants and children. This book is composed of 21 chapters that discuss the clinical examination, laboratory studies, and diagnosis of the injury, as well as the neurological analysis of a child. The opening chapters describe some of the examinations of infant and children, including the use of electroencephalography, electromyography, and measurement of the conduction velocity of peripheral nerves, examination of the spinal fluid, imaging techniques applicable to the neuromuscular system. A chapter emphasizes the treatment of convulsion. The next chapters explore the endocrine disorders causing neurological symptoms and signs; the malformations of the nervous and muscular systems; and the physical injury to the nervous system after the neonatal period. A chapter is devoted to the tumors of the nervous system. The closing chapter focuses on the abnormal muscle stiffness and muscle pains. The book can provide useful information to pediatricians, neurologists, students, and researchers.
Front Cover 1
Paediatric Neurology 4
Copyright Page 5
Table of Contents 10
Preface 6
Dedication 3
Acknowledgements 8
Introduction 12
Chapter 1. History – general outline 14
FAMILY HISTORY 14
PREGNANCY 14
LABOUR AND DELIVERY 15
NEONATAL PERIOD 15
DEVELOPMENT OF THE CHILD 15
ONSET AND COURSE OF THE DISEASE 16
References 17
Chapter 2. The neurological examination 18
Examination of the head 19
Cranial nerves 22
Muscle tone 24
Reflexes 25
Brudzinski's sign 28
Examination of older infants and children 31
Examination of the head 44
References 45
Chapter 3. Special examinations 46
Electroencephalography 46
Conduction velocity 62
Examination of the spinal fluid 64
Other laboratory studies 66
References 89
Chapter 4. Convulsions 90
Classifícatíon according to the type of fit 90
Classification according to the cause of the fít 105
Hypomagnesaemia 113
Colour plate section 114
Other metabolic disorders 118
Cardiovascular factors 120
Breath-holding spells 121
Programme for the examination of children with convulsions 122
The acute management of convulsions and status epilepticus outside the neonatal period 126
The long-term management of children with benign febrile convulsions 128
Long-term symptomatic antiepileptic therapy outside the newborn period 128
Prognosis 137
References 137
Chapter 5. Mental retardation 139
General causes of mental retardation 140
Medical causes of mental retardation 141
Plan of examination in cases of mental retardation 166
Treatment 169
References 170
Chapter 6. Generalized muscular hypotonia and flaccid weakness 172
General non-neurological conditions 172
Involvement of the nervous or muscular system, certain or postulated 175
Miscellaneous 224
References 225
Chapter 7. Abnormal muscle stiffness and/or muscle pains 227
The central nervous system 227
The muscle fibres 230
Localization unknown 235
Plan of examination in patients with abnormal muscle stiffness 235
References 236
Chapter 8. Localized weakness 237
Spastic weakness 237
Flaccid weakness 237
References 248
Chapter 9. Abnormal growth of the head 249
Abnormally small head or abnormally shaped head 249
Abnormally large head 255
References 272
Chapter 10. Ataxia 273
Ataxia present from infancy 274
Ataxia with acute onset during childhood 275
Ataxia with insidious onset during childhood 278
Management of the ataxic child 281
References 282
Chapter 11. Involuntary movements 283
Tremor 283
Choreoathetosis 284
Torsion dystonia 287
Myoclonic jerks 288
Tics 289
Management of the child with involuntary movements 289
References 290
Chapter 12. Cerebral palsy 291
Causes of cerebral palsy 291
Clinical picture 294
Differential diagnosis 305
Treatment 307
Prognosis 310
References 310
Chapter 13. Diseases characterized by progressive neurological and mental symptoms and signs 311
Conditions with early main manifestations caused by white matter dysfunction 318
Other conditions 322
Conditions dominated by ataxia 325
Other chronic viral infections of the brain 329
The management of a child showing progressive mental and neurological symptoms and signs 331
References 332
Chapter 14. Headache 334
Headache with acute onset and short duration 334
Persistent headache of more insidious onset 337
Intermittent headache 338
Management of the child with headache 343
References 344
Chapter 15. Malformations of the nervous and the muscular systems 345
Malformations of the brain and the cranium 345
Malformations of the spine, spinal cord, and nerve roots 350
Malformations of the peripheral nerves 356
Malformations of muscles 356
Management of the situation around a newborn infant in whom a malformation is apparent immediately after birth 357
References 359
Chapter 16. Infectious diseases and immunization 360
Acute purulent meningitis 360
Chronic meningitis and meningoencephalitis 362
Viral infection of the central nervous system 365
Neurological complications of common infectious diseases and immunizations 368
Brain abscess 378
Subdural abscess 379
References 379
Chapter 17. Tumours of the nervous system 381
Intracranial tumours 381
Description of the various types of tumour 382
Tumours extending both extradurally and intradurally 389
Tumours of the peripheral nerves 390
References 391
Chapter 18. Physical injury to the nervous system after the neonatal period 392
Head trauma 392
Spinal cord injury 395
Injuries to the nerve roots and the peripheral nerves 395
References 396
Chapter 19. Abnormal growth as a symptom of a neurological disorder 397
Short stature with normal body proportions 397
Abnormally rapid growth with normal body proportions or acromegalic features 397
Emaciation with normal body proportions or acromegalic features 400
Other types of disproportionate growth 403
Summary 403
References 403
Chapter 20. Endocrine disorders causing neurological symptoms and signs 405
Thyroid disorders 405
Disorders of the hypophysis and adrenal cortex 407
Parathyroid disorders 408
Insulinoma 409
References 409
Chapter 21. Miscellaneous neurological diseases affecting infants and children 410
Syringomyelia 410
Vascular abnormalities of the spinal cord 411
Hereditary sensory radicular neuropathy 412
Congenital analgia 412
Porphyria 413
Lead encephalopathy 413
Malignant hyperthermia 414
References 415
Index 416
History – general outline
Publisher Summary
This chapter presents the general outline of the taking of the history and its evaluation. All information about labor and delivery is important in the neurological history of a child. Although the incidence of birth injury has diminished with improved obstetric and neonatal care, it still appears to be the most common single cause of neurological symptoms and signs during childhood. It is found that although a birth injury may occur when delivery seems entirely normal, its incidence increases when there are complications such as premature birth, abnormal presentation, vacuum extraction, or evidence of fetal stress. Although the incidence of injuries is statistically increased, only a small proportion of infants born under unfavorable conditions will show permanent neurological symptoms and signs. The neonatal period may be complicated by jaundice, cyanotic spells, respiratory difficulties, apneic attacks, or a bleeding tendency, any of which may be an indication of a disorder that may lead to neurological abnormalities. Neurological signs may also have been recorded in the neonatal period such as abnormal cry, apathy, swallowing difficulties, inability to suck, abnormal muscle tone, tremor, or convulsions.
This chapter gives only a general outline of the taking of the history and its evaluation. All special aspects that are relevant to particular diseases described later will be discussed in connection with the disease. A history taken from the parents, as is the general rule in paediatrics, gives better information about family history, pregnancy, neonatal period, and early development than a history taken from the patient. The advantages of a history taken from the parents should be utilized even when the child is old enough to tell about his present symptoms. On the other hand, a history taken from the parents is always second-hand information and should, whenever possible, be supplemented by direct questioning of the child.
FAMILY HISTORY
The simple statement that there are no known diseases in the family is of no value. One must also know the number and sex of healthy family members to judge if there are individuals available who might have shown the abnormal trait. Information about consanguinity is also necessary, as consanguinity between parents increases the probability that the child will have a disease due to an autosomal recessive gene. In some situations tests are now available to diagnose the healthy carriers of an abnormal gene (see Chapter 6).
PREGNANCY
Events that occur during pregnancy may affect the fetus. Various infections, some of which the mother may have unknowingly, may pass to the fetus and cause active disease or permanent damage. Examples of such infections are syphilis, toxoplasmosis, cytomegalic inclusion body disease, rubella, and possibly other viral infections.
The possibility of the toxic influence of drugs became apparent during the 1960s when thalidomide caused malformations in children; this drug is no longer available. Antiepileptic medication, which usually has to be continued throughout pregnancy in a woman with epilepsy, may approximately double the risk of malformation in the child (see page 126), although these malformations do not usually involve the nervous system. Drugs with a cytostatic effect, such as azathioprine and cyclophosphamide, are even more dangerous to the developing fetus. Environmental toxins, particularly ethyl alcohol, may also seriously affect the child (see page 126).
Maternal disease, e.g. diabetes, may increase the incidence of malformations in the child, and untreated metabolic conditions, e.g. phenylketonuria (see page 127), may impair the development of the fetal brain. Smoking during pregnancy impairs the growth of the child but does not seem to cause malformations. Toxicosis of the mother increases the risk of placental insufficiency and thus the birth of an undernourished small-for-dates child.
Undernourishment of the mother during pregnancy, either general starvation due to lack of food or to lack of appetite and/or vomiting because of serious disease in the mother, or lack of specific substances such as vitamin B12 in a diet extremely lacking in all animal protein, may cause irreversible brain damage in the child. The incidence of abnormal children is slightly increased in pregnancies complicated by vaginal bleeding. It is debated whether this bleeding causes abnormalities in the child or whether it should be interpreted as a threatening abortion due to a primary abnormality of the fetus.
An estimation of the duration of the pregnancy, combined with an evaluation of the maturity of the newborn infant, is important. Preterm birth always carries a certain risk of intracranial bleeding and respiratory problems which may lead to asphyxia and brain injury. These risks are small for infants born after 34 or more weeks of pregnancy.
LABOUR AND DELIVERY
All information about labour and delivery is important in the neurological history of a child. Although the incidence of birth injury has diminished with improved obstetric and neonatal care, it still appears to be the most common single cause of neurological symptoms and signs during childhood. Although a birth injury may occur when delivery seems entirely normal, its incidence increases when there are complications such as premature birth, abnormal presentation, vacuum extraction, or evidence of fetal stress. It should, however, be emphasized that although the incidence of injuries is statistically increased, only a small proportion of infants born under unfavourable conditions will show permanent neurological symptoms and signs.
NEONATAL PERIOD
The neonatal period may be complicated by jaundice, cyanotic spells, respiratory difficulties, apnoeic attacks, or a bleeding tendency, any of which may be an indication of a disorder that may lead to neurological abnormalities. Neurological signs may also have been recorded in the neonatal period, e.g. abnormal cry, apathy, swallowing difficulties, inability to suck, abnormal muscle tone, tremor, or convulsions.
DEVELOPMENT OF THE CHILD
Questions about the psychomotor development of the child are a necessary part of the history. A few ‘milestones’ of the normal development of the infant born at term are briefly given here (see also Table 2.1, page 17). Information about these milestones is a necessary part of the developmental history. The motor development of the prematurely born infant is delayed, particularly during the first weeks and months, when the delay is roughly proportional to the degree of prematurity. The healthy premature baby usually catches up with the baby born at term during the first year of life.
The newborn baby is already able to follow a bright, slowly moving object with his eyes. He also turns his eyes towards the light. The infant starts to fix and give visual contact at the age of 4–6 weeks and to follow an object promptly a few weeks later. The first smile is seen at about the same time. Many newborn infants can lift the head in the prone position; all infants are expected to do so at 6 weeks of age. At 4 months of age the child can lift his head also in the supine position, can balance it when he is held upright, and can lift head and shoulders when lying on his belly. He reaches out for objects but is not yet able to grasp them deliberately; he examines and plays with his hands; he turns his eyes and head towards a sound; he can use his voice to express pleasure. A month or two later he starts to roll over. At the age of 7 months the child sits without support, he can grasp a rather large object in a clumsy way with his whole hand, and can transfer objects from hand to mouth. At 9–10 months of age the child starts to pull himself to standing and to walk holding on to furniture. He can pick up rather small things now, using the pinching grasp, and can transfer from hand to hand.
At 12–14 months of age most children start to walk without support and have a vocabulary of about three to ten words which may be understood by their parents. At 2 years of age the child is expected to be able to put at least two words together in a simple sentence and at 3–4 years to pronounce words distinctly and correctly without having to use baby-talk. The child is usually able to run at 2 years of age and to hop on one leg at 4½ years.
Normal psychomotor development usually seems to occur stepwise rather than smoothly. The normal variation is considerable. Thus, small deviations from the development outlined here should cause no concern. A thorough examination is suggested in the following situations:
1. If the child does not lift his head in the prone position or does not follow an object with his eyes at age 2 months.
2. If the child does not lift his head in the supine position at age 6 months.
3. If the child makes no attempts to sit at age 9 months or to walk at age 18 months.
4. If the child uses no words at all at age 18 months or does not try to form simple sentences at age 3.
The outlined deviations from the usual development do not establish that the child is developing abnormally,...
Erscheint lt. Verlag | 22.10.2013 |
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Sprache | englisch |
Themenwelt | Medizin / Pharmazie ► Medizinische Fachgebiete ► Gynäkologie / Geburtshilfe |
Naturwissenschaften ► Biologie ► Humanbiologie | |
Naturwissenschaften ► Biologie ► Zoologie | |
ISBN-10 | 1-4831-9196-6 / 1483191966 |
ISBN-13 | 978-1-4831-9196-6 / 9781483191966 |
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