Contents 1 Normal Histology and Commonly Used Stains 2 Basic Pathologic Reactions

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1 Contents 1 Normal Histology and Commonly Used Stains Cells of the Nervous System Neurons Astrocytes Oligodendrocytes Schwann Cells Ependyma Microglia Supporting Tissues General Architecture of the Nervous System Grey and White Matter Peripheral Nerve Ganglia Skeletal Muscle Commonly Used Stains in Neuropathology Tinctorial Stains Immunohistochemical Preparations References Basic Pathologic Reactions Neurons Neuronal Atrophy and Neuronal Loss Hypoxic-Ischaemic Change Central Chromatolysis Abnormal Inclusions Axonal Alterations Astrocytes Gliosis Alzheimer Type II Astrocyte Rosenthal Fibers ix

2 x Contents Eosinophilic Granular Bodies Corpora Amylacea Oligodendrocytes Demyelination Inclusions Ependyma Atrophy Discontinuity Granulations Microglia Diffuse Microglial Activation and Microglial Nodules References Requesting and Interpreting Pathological Tests Neurosurgical Specimens Muscle and Nerve Biopsies Cerebrospinal Fluid Skin Biopsies Other Samples Vascular Diseases Vascular Anatomy Arterial Supply Venous Drainage Diseases Affecting the Blood Vessels Atherosclerosis Small Vessel Disease Amyloid Angiopathy Hereditary Vascular Diseases Vasculitides Vascular Malformations Aneurysms Consequences of Cerebrovascular Diseases Ischaemia and Infarction Haemorrhage References Infections Bacterial Infections Acute Bacterial Meningitis Abscess Chronic Bacterial Infections Viral Infections Aseptic Meningitis Encephalitis or Encephalomyelitis... 65

3 Contents xi 5.3 Fungal Infections Parasitic Infections Protozoal Infections Metazoal/Helminthic Infections Role of Brain Biopsy in Infectious Diseases References Tumours of Central Nervous System Classification Sending Specimens to the Laboratory Gliomas Astrocytic Tumours Genetics of Gliomas Non-astrocytic Gliomas Neuronal Tumours With or Without Glial Features Gangliocytoma and Ganglioglioma Dysembryoplastic Neuroepithelial Tumour Central Neurocytoma Desmoplastic Infantile Astrocytoma/Ganglioglioma Dysplastic Gangliocytoma of the Cerebellum (Lhermitte-Duclos Syndrome) Embryonal Tumours Medulloblastoma Primitive Neuroectodermal Tumour Atypical Teratoid/Rhabdoid Tumour Pineal Region Tumours Choroid Plexus Tumours Meningeal Tumours Meningiomas Other Tumours of the Meninges Pituitary Region Tumours Lymphomas Tumours of the Peripheral Nerves Other Tumours Affecting the CNS References Demyelinating Diseases Multiple Sclerosis Classic Multiple Sclerosis Acute Multiple Sclerosis Concentric Sclerosis Neuromyelitis Optica (Devic s Disease) Acute Disseminated Encephalomyelitis Acute Haemorrhagic Leukoencephalopathy Trigeminal Neuralgia References

4 xii Contents 8 Epilepsy Rasmussen s Encephalitis Focal Cortical Dysplasia Hippocampal Sclerosis Sudden Unexpected Death in Epilepsy (SUDEP) References Muscle Diseases Muscle Biopsy Technique Laboratory Preparation Normal Structure of Muscle General Pathological Reactions Muscular Dystrophies Dystrophin-Related Muscular Dystrophies Limb Girdle Muscular Dystrophies Congenital Muscular Dystrophies Other Muscular Dystrophies Congenital Myopathies Myofibrillar Myopathies Metabolic Myopathies Inflammatory Muscle Disease Toxic Myopathies Neurogenic Disorders References Peripheral Nerve Diseases Nerve Biopsy Technique Laboratory Preparation Normal Structure of Peripheral Nerve General Pathological Reactions Inflammatory Neuropathies Guillain-Barre Syndrome Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) Multifocal Motor Neuropathy with Conduction Block Idiopathic Perineuritis Sarcoidosis Vasculitis Neuropathies Associated with Infectious Disease Leprosy Human Immunodeficiency Virus Lyme Disease Diphtheria Toxic Neuropathies Metabolic and Nutritional Neuropathies

5 Contents xiii Diabetes Mellitus Uraemia Neuropathies Associated with Paraprotein Amyloid Neuropathies Miscellaneous Neuropathies Paraneoplastic Neuropathy Critical Illness Neuromyopathy Carpal Tunnel Syndrome Hereditary Neuropathies Skin Biopsy in Small Fibre Neuropathies References Metabolic, Toxic and Nutritional Diseases Metabolic Diseases Hypoglycaemia Electrolyte Imbalance Hepatic Encephalopathy Wilson s Disease Uraemic Encephalopathy Amino Acid Disorders Urea Cycle Disorders Porphyrias Lysosomal and Peroxisomal Disorders Mitochondrial Disorders Toxic Disorders Ethanol Nutritional Deficiencies References Neurodegenerative Disorders Dementia Alzheimer s Disease Dementia with Lewy Bodies Frontotemporal Lobar Degeneration Prion Diseases Disorders Predominantly Affecting the Basal Ganglia Huntington s Disease Neurodegeneration with Brain Iron Accumulation Neuroacanthocytosis Disorders Predominantly Affecting the Midbrain Parkinson s Disease Progressive Supranuclear Palsy Multiple System Atrophy Corticobasal Degeneration Other Disorders

6 xiv Contents 12.4 Cerebellar Degeneration Motor Neuron Diseases Motor Neuron Disease X-Linked Bulbar Spinal Muscular Atrophy (Kennedy s Disease) Spinal Muscular Atrophy Hereditary Spastic Paraparesis References Trauma Focal Injury Scalp Injury Skull Fractures Brain Contusions and Lacerations Traumatic Intracranial Haemorrhage Extradural Haemorrhage Subdural Haemorrhage Subarachnoid Haemorrhage Parenchymal Haemorrhage Intraventricular Haemorrhage Other Localised Injury Focal Vascular Injury Pituitary Gland Infarction Brain Stem Avulsion Cranial Nerve Avulsion Diffuse Brain Injury Diffuse Axonal Injury Diffuse Vascular Injury Brain Swelling and Ischaemia Fat Embolism Missile Head Injury Inflicted Head Injury in Childhood Chronic Traumatic Encephalopathy Spinal Cord Injury References Paediatric Diseases Congenital Malformations Neural Tube Defects Chiari Malformations Disorders of Forebrain Induction Neuronal Migration Disorders Encephaloclastic Defects Microcephaly, Megalencephaly, Hemi-megalencephaly Cerebellar, Brainstem and Spinal Cord Malformations

7 Contents xv 14.2 Hydrocephalus Perinatal Diseases Hypoxic-Ischaemic Neuronal Injury White Matter Lesions Cerebral Haemorrhages Hypoglycaemia Kernicterus Infections Rare Neurodegenerative Diseases References Autopsies References Appendix 1. Suggested Reading Index

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