- Preface to the twelfth edition
- Preface to the eleventh edition
- Preface to the first edition
- Contributors
- Glossary
- Section 1 Introduction
- Chapter 1 A short history of clinical neurology
- Chapter 2 The clinical approach
- Chapter 3 Investigations
- Chapter 4 Psychologically determined disorders
- Chapter 5 Toxic and environmental disorders
- Chapter 6 Principles of neurological rehabilitation
- Section 2 Developmental neurology
- Chapter 7 Clinical approach to developmental neurology
- Chapter 8 Development, degeneration, and regeneration of the central nervous system
- Chapter 9 Neurodevelopmental disorders
- Chapter 10 Neurometabolic disorders
- Chapter 11 Neurocutaneous syndromes
- Section 3 Disorders of special senses
- Chapter 12 Abnormal vision
- Chapter 13 Ocular motor disorders
- Chapter 14 Disorders of hearing
- Chapter 15 Vertigo and imbalance
- Chapter 16 Abnormalities of smell and taste
- Section 4 Pain
- Chapter 17 Neuropathic pain
- Chapter 18 Headache
- Chapter 19 Craniofacial pain
- Section 5 Nerve and muscle disease
- Chapter 20 The lower cranial nerves and dysphagia
- Chapter 21 Polyneuropathy
- Chapter 22 Focal peripheral neuropathy
- Chapter 23 The motor neurone disorders
- Chapter 24 Muscle diseases
- Section 6 Structural disease affecting brain, spinal cord, and nerve roots
- Chapter 25 Head injury
- Chapter 26 Raised intracranial pressure, cerebral oedema, and hydrocephalus
- Chapter 27 Tumours of the brain and skull
- Chapter 28 Spinal cord disorders
- Chapter 29 Cauda equina lesions, radiculopathies, and sphincter disorders
- Section 7 Seizures and alterations of consciousness and thought
- Chapter 30 Seizures and related disorders in children
- Chapter 31 Seizures, epilepsy, and other episodic disorders in adults
- Chapter 32 Sleep disorders
- Chapter 33 Coma
- Chapter 34 Neuropsychological disorders, dementia, and behavioural neurology
- Section 8 Vascular, demyelinating, inflammatory and degenerative disorders of the central nervous system
- Chapter 35 Cerebrovascular diseases
- Chapter 36 Vasculitis and collagen vascular diseases
- Chapter 37 Multiple sclerosis and other demyelinating diseases
- Chapter 38 Paraneoplastic disorders and neuroimmunology
- Chapter 39 Tremor, ataxia, and cerebellar disorders
- Chapter 40 Movement disorders
- Section 9 Neurological infection
- Chapter 41 Meningitis
- Chapter 42 Encephalitis and infectious encephalopathies
- Chapter 43 Intracranial space occupying infections and neurological HIV disease
- Index
(p. 1131) Multiple sclerosis and other demyelinating diseases
- Chapter:
- (p. 1131) Multiple sclerosis and other demyelinating diseases
- Author(s):
Alastair Compston
- DOI:
- 10.1093/med/9780198569381.003.0871
The oligodendrocyte–myelin unit subserves saltatory conduction of the nerve impulse in the healthy central nervous system. At one time, many disease processes were thought exclusively to target the structure and function of myelin. Therefore, they were designated ‘demyelinating diseases’. But recent analyses, based mainly on pathological and imaging studies, (re)emphasize that axons are also directly involved in these disorders during both the acute and chronic phases. Another ambiguity is the extent to which these are inflammatory conditions. Here, distinctions should be made between inflammation, as a generic process, and autoimmunity in which rather a specific set of aetiological and mechanistic conditions pertain. And there are differences between disorders that are driven primarily by immune processes and those in which inflammation occurs in response to pre-existing tissue damage.
With these provisos, the pathological processes of demyelination and associated axonal dysfunction often account for episodic neurological symptoms and signs referable to white matter tracts of the brain, optic nerves, or spinal cord when these occur in young people. This is the clinical context in which the possibility of ‘demyelinating disease’ is usually considered by physicians and, increasingly, the informed patient. Neurologists will, with appropriate cautions, also be prepared to diagnose demyelinating disease in older patients presenting with progressive symptoms implicating these same pathways even when there is no suggestive past history. Both in its typical and atypical forms multiple sclerosis remains by far the commonest demyelinating disease. But acute disseminated encephalomyelitis, the leucodystrophies, and central pontine myelinolysis also need to be considered in particular circumstances; and multiple sclerosis itself has a differential diagnosis in which the relapsing-remitting course is mimicked by conditions not associated with direct injury to the axon–glial unit. Since our understanding of the cause, pathogenesis and features of demyelinating disease remains incomplete, classification combines aspects of the aetiology, clinical features, pathology, and laboratory components. Whether the designation ‘multiple sclerosis’ encapsulates one or more conditions is now much debated. We anticipate that a major part of future studies in demyelinating disease will be further to resolve this question of disease heterogeneity leading to a new taxonomy based on mechanisms rather than clinical empiricism. But, for now, the variable ages of onset, unpredictable clinical course, protean clinical manifestations, and non-specific laboratory investigations continue to make demyelinating disease one of the more challenging diagnostic areas in clinical neurology.
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- Preface to the twelfth edition
- Preface to the eleventh edition
- Preface to the first edition
- Contributors
- Glossary
- Section 1 Introduction
- Chapter 1 A short history of clinical neurology
- Chapter 2 The clinical approach
- Chapter 3 Investigations
- Chapter 4 Psychologically determined disorders
- Chapter 5 Toxic and environmental disorders
- Chapter 6 Principles of neurological rehabilitation
- Section 2 Developmental neurology
- Chapter 7 Clinical approach to developmental neurology
- Chapter 8 Development, degeneration, and regeneration of the central nervous system
- Chapter 9 Neurodevelopmental disorders
- Chapter 10 Neurometabolic disorders
- Chapter 11 Neurocutaneous syndromes
- Section 3 Disorders of special senses
- Chapter 12 Abnormal vision
- Chapter 13 Ocular motor disorders
- Chapter 14 Disorders of hearing
- Chapter 15 Vertigo and imbalance
- Chapter 16 Abnormalities of smell and taste
- Section 4 Pain
- Chapter 17 Neuropathic pain
- Chapter 18 Headache
- Chapter 19 Craniofacial pain
- Section 5 Nerve and muscle disease
- Chapter 20 The lower cranial nerves and dysphagia
- Chapter 21 Polyneuropathy
- Chapter 22 Focal peripheral neuropathy
- Chapter 23 The motor neurone disorders
- Chapter 24 Muscle diseases
- Section 6 Structural disease affecting brain, spinal cord, and nerve roots
- Chapter 25 Head injury
- Chapter 26 Raised intracranial pressure, cerebral oedema, and hydrocephalus
- Chapter 27 Tumours of the brain and skull
- Chapter 28 Spinal cord disorders
- Chapter 29 Cauda equina lesions, radiculopathies, and sphincter disorders
- Section 7 Seizures and alterations of consciousness and thought
- Chapter 30 Seizures and related disorders in children
- Chapter 31 Seizures, epilepsy, and other episodic disorders in adults
- Chapter 32 Sleep disorders
- Chapter 33 Coma
- Chapter 34 Neuropsychological disorders, dementia, and behavioural neurology
- Section 8 Vascular, demyelinating, inflammatory and degenerative disorders of the central nervous system
- Chapter 35 Cerebrovascular diseases
- Chapter 36 Vasculitis and collagen vascular diseases
- Chapter 37 Multiple sclerosis and other demyelinating diseases
- Chapter 38 Paraneoplastic disorders and neuroimmunology
- Chapter 39 Tremor, ataxia, and cerebellar disorders
- Chapter 40 Movement disorders
- Section 9 Neurological infection
- Chapter 41 Meningitis
- Chapter 42 Encephalitis and infectious encephalopathies
- Chapter 43 Intracranial space occupying infections and neurological HIV disease
- Index