Neurology

Clinical Syndromes 1 1 Clinical Syndromes in Neurology 2 Differentiation of Central and Peripheral Paresis . . . . . . . . . . . . . . . . 2 Bodily Distribution of Paresis . . . . . 3 Monoparesis . . . . . . . . . . . . . . . . . . 3 Hemiparesis . . . . . . . . . . . . . . . . . . . 4 Para- and Quadriparesis . . . . . . . 4 Lesions Affecting the Anterior Horn Ganglion Cells . . . . . . . . . . . . . . 5 Lesions Affecting a Spinal Nerve Root . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 5 Polyradiculopathy . . . . . . . . . . . . . . . 10 Polyneuropathy . . . . . . . . . . . . . . . . . 10 Plexus Lesions . . . . . . . . . . . . . . . . . . 10 Lesions of a Single Peripheral Nerve . . . . . . . . . . . . . . . . . . . . . . . . . . . 11 Dysfunction of the Neuromuscular Junction . . . . . . . . . . . . . . . . . . . . . . . . 11 Myopathy . . . . . . . . . . . . . . . . . . . . . . 11 Clinical Neurology 13 2 DiseasesMainlyAffecting the Brain and its Coverings 14 Characteristics of Diseases of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . . . 14 Congenital and Perinatally Acquired Diseases of the Brain . . . . . . . . . . . . . . . . . . . . . . . . . 14 Traumatic Brain Injury . . . . . . . . 45 Intracranial Hypertension and Brain Tumors . . . . . . . . . . . . . . . . . 56 Infectious Diseases of the Brain and Meninges . . . . . . . . . . . . . . . . 75 Disturbances of Cerebral Perfusion and Nontraumatic Intracranial Hemorrhage . . . . . 131 The Comatose Patient . . . . . . . . 221 Extrapyramidal Syndromes . . 235 Cerebellar Syndromes . . . . . . . 274 Metabolic Disorders with Cerebral Or Other Neurologic Involvement . . . . . . . . . . . . . . . . . 285 Systemic Diseases Affecting the Nervous System . . . . . . . . . 299 Dementing Disorders and Other Neuropsychological Syndromes . . . . . . . . . . . . . . . . . . 346 IX Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. 3 DiseasesMainlyAffecting the Spinal Cord 389 Characteristics of Diseases of the Spinal Cord . . . . . . . . . . . . . . . . 389 Ancillary Tests in Diseases of the Spinal Cord . . . . . . . . . . . . . . . . 389 Classification of Spinal Cord Syndromes . . . . . . . . . . . . . . . . . . . . 391 Congenital and Perinatally Acquired Lesions of the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . 393 Spinal Cord Trauma . . . . . . . . . . . . 395 Fundamentals of (Complete) Spinal Cord Transection Syndrome . . . . . . . . . . . . . . . . . . . 395 Practical Approach to Acute Traumatic Spinal Cord Transection . . . . . . . . . . . . . . . . . . 399 Acceleration Injury of the Spine (Whiplash Injury) . . . . . . . . . . . 402 Tumors and Other Masses Compressing the Spinal Cord . . . . . . . 405 General Aspects . . . . . . . . . . . . . 405 Types of Mass Compressing the Spinal Cord . . . . . . . . . . . . . . 406 Infectious, Allergic, and Toxic Diseases of the Spinal Cord and Its Coverings . . . . . . . . . . . . . . . . . . . 414 Infectious Diseases of the Spinal Cord . . . . . . . . . . . . . . . . . . 414 Myelitis . . . . . . . . . . . . . . . . . . . . . 416 Toxic Myelopathies . . . . . . . . . . 417 Circulatory Disorders of the Spinal Cord . . . . . . . . . . . . . . . . . . . . 418 Blood Supply of the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . 418 Spinal Cord Ischemia Due to Deficient Arterial Blood Flow . . . . . . . . . . . . . . . . . . . . . . . . 418 Spinal Cord Ischemia Due to Venous Disturbances . . . . . . . . . 423 Degenerative and Heredodegenerative Diseases Mainly Affecting the Spinal Cord . . . . . . . 425 Diseases Affecting the Anterior Horn Cells . . . . . . . . . . . . . . . . . . . 425 Spastic Spinal Paralysis . . . . . . 432 Amyotrophic Lateral Sclerosis (ALS) . . . . . . . . . . . . . . . . . . . . . . . . 434 Spinocerebellar Ataxias . . . . . . 437 Metabolic Disorders Mainly Affecting the Spinal Cord . . . . . . . 441 Vitamin B12 Deficiency and Funicular Myelosis . . . . . . . . . . . 441 Syringomyelia and Syringobulbia . . . . . . . . . . . . . . . . . . 443 Other Diseases of the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . 447 Radiation-Induced Myelopathy . . . . . . . . . . . . . . . . . 447 Decompression Myelopathy . . 447 4 Autonomic and Trophic Disorders 449 Acute Pandysautonomia . . . . . . . . 454 Familial Dysautonomia (Riley-Day Syndrome) . . . . . . . . . . 455 Botulism . . . . . . . . . . . . . . . . . . . . . . . 455 Insensitivity to Pain . . . . . . . . . . . . 456 Congenital Insensitivity to Pain . . . . . . . . . . . . . . . . . . . . . . 456 Congenital Sensory Neuropathy with Anhidrosis . . . . . . . . . . . . . 456 Sensory Radicular Neuropathy . . . . . . . . . . . . . . . . . . . . . . . 456 Pain Asymbolia . . . . . . . . . . . . . . 456 Sympathetic Syndromes . . . . . . . 457 Typical Manifestations . . . . . . . 457 Trophic Disorders . . . . . . . . . . . . . . 461 X Contents Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. 5 Demyelinating Diseases 465 Multiple Sclerosis . . . . . . . . . . . . . . 465 Typical Clinical Features . . . . . 466 Epidemiology . . . . . . . . . . . . . . . . 467 Clinical Features . . . . . . . . . . . . . 467 Ancillary Tests . . . . . . . . . . . . . . . 471 Prognosis . . . . . . . . . . . . . . . . . . . . 474 Pathologic Anatomy . . . . . . . . . 476 Etiology and Pathogenesis . . . 477 Treatment . . . . . . . . . . . . . . . . . . . 480 Differential Diagnosis . . . . . . . . 483 Other Demyelinating Diseases . . 484 Concentric Sclerosis (Balo´ ’s Disease) . . . . . . . . . . . . . . 484 Diffuse Sclerosis (Schilder’s Disease) . . . . . . . . . . 484 Acute Disseminated Encephalomyelitis (ADEM) . . . . . . . . . . . . . 484 Neuromyelitis Optica (Devic’s Disease) . . . . . . . . . . . . . 484 Subacute Myelo-optic Neuropathy (SMON) . . . . . . . . . 485 Hereditary Demyelinating Diseases . . . . . . . . . . . . . . . . . . . . . 485 6 Injury to the Nervous System by Specific Physical Agents 487 Electrical Injury . . . . . . . . . . . . . . . . 487 Gas Embolism . . . . . . . . . . . . . . . . . 488 Venous Gas Embolism . . . . . . . 488 Arterial Gas Embolism . . . . . . . 489 Injury Due to Ionizing Radiation . . . . . . . . . . . . . . . . . . . . . . 490 Radiation Injury to the Brain 490 Radiation Injury to the Spinal Cord . . . . . . . . . . . . . . . . . . . . . . . . 490 Radiation Injury to the Peripheral Nervous System . . . 491 Hypothermic Injury . . . . . . . . . . . . 491 7 Epilepsy, Other Episodic Disorders of Neurologic Function, and Sleep Disorders 493 Epilepsy . . . . . . . . . . . . . . . . . . . . . . . 493 History . . . . . . . . . . . . . . . . . . . . . . 493 Etiology and Pathogenesis . . . 494 Epidemiology . . . . . . . . . . . . . . . . 495 Ancillary Diagnostic Tests in Epileptology . . . . . . . . . . . . . . . . . 495 Classification of Epilepsy . . . . . 501 Individual Seizure Types . . . . . 504 Clinical Patterns of Epilepsy and Epileptic Syndromes . . . . . 506 Partial (Focal) Seizures . . . . . . . 517 Special Seizure Types and Seizure Etiologies . . . . . . . . . . . . 530 Procedure after a First Seizure or Multiple Seizures . . . . . . . . . 532 Treatment of Epilepsy . . . . . . . 532 Prognosis . . . . . . . . . . . . . . . . . . . . 546 Episodic Disturbances of Consciousness, Syncope, and Other Nonepileptic Episodes . . . . . . . . . . 547 Syncope and Drop Attacks . . . 550 Disturbances of Consciousness of Metabolic Origin . . . . . . . . . . 554 Drop Attacks . . . . . . . . . . . . . . . . 555 Episodic Nonepileptic Motor Phenomena . . . . . . . . . . . . . . . . . 556 Episodic Disorders that are Partly or Wholly Psychogenic 558 Contents XI Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Neurological Findings in the Unconscious Patient and in Psychogenic Pseudo-Neurological Conditions . . . . . . . . . . . . . . . . . . . . . 560 Neurological Findings in the Unconscious Patient . . . . . . . . . 560 The Neurological Examination in Psychogenic Pseudo- Neurological Conditions . . . . . 560 Sleep and Disturbances of Sleep . . . . . . . . . . . . . . . . . . . . . . . . . . 563 Sleep . . . . . . . . . . . . . . . . . . . . . . . . 563 Sleep Disorders . . . . . . . . . . . . . . 564 Hypersomnia . . . . . . . . . . . . . . . . 565 Sleep Apnea Syndrome . . . . . . 569 8 Polyradiculitis and Polyneuropathy 575 Polyradiculitis . . . . . . . . . . . . . . . . . 575 Classic Acute Polyradiculitis (Guillain-Barre´ Syndrome, Landry-Guillain-Barre´ Syndrome) . . . . . . . . . . . . . . . . . . 576 Atypical Polyradiculitis . . . . . . 580 Polyneuropathy . . . . . . . . . . . . . . . . 582 General Features . . . . . . . . . . . . . 582 Hereditary Polyneuropathy . . 588 Polyneuropathy Due to Metabolic Disorders . . . . . . . . . 598 Polyneuropathy Due to Improper or Inadequate Nutrition . . . . . . . . . . . . . . . . . . . . 604 Polyneuropathy Due to Vitamin B12 Malabsorption . . . 604 Autoimmune Polyneuropathy . . . . . . . . . . . . . . . . . . 605 Polyneuropathy Due to Infectious Disease . . . . . . . . . . . 606 Polyneuropathy Due to Arterial Disease . . . . . . . . . . . . . . 606 Polyneuropathy Due to Sprue and Other Malabsorptive Disorders . . . . . . . . . . . . . . . . . . . . . . . 608 Polyneuropathy Due to Exogenous Toxic Substances 609 Polyneuropathy of Other Causes . . . . . . . . . . . . . . . . . . . . . . 613 9 DiseasesAffecting the Cranial Nerves 617 Disturbances of Olfaction . . . . . . 623 Anosmia . . . . . . . . . . . . . . . . . . . . 623 True Combined Anosmia and Ageusia . . . . . . . . . . . . . . . . . . . . . 624 Cacosmia . . . . . . . . . . . . . . . . . . . . 624 Visual Disturbances of Neurologic Origin . . . . . . . . . . . . . . . . . . . . . . . . . 624 Loss of Vision . . . . . . . . . . . . . . . . 624 Visual Field Defects and Perceptual Disturbances . . . . . 625 Abnormal Findings in the Optic Disks . . . . . . . . . . . . . . . . . . 630 Oculomotor Disturbances . . . . . . 630 The Neuroanatomical Basis of Ocular Motility . . . . . . . . . . . . 634 Preliminary Remarks on the Examination of Ocular Motility . . . . . . . . . . . . . . . . . . . . . 636 Ancillary Tests in Oculomotor Disturbances . . . . . . . . . . . . . . . . 639 XII Contents Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. General Principles of Ocular Motility . . . . . . . . . . . . . . . . . . . . . 641 Some Diseases in which Oculomotor Disturbances are Prominent . . . . . . . . . . . . . . . . . . . 652 Diseases that Mimic Supranuclear Disturbances of Eye Movement . . . . . . . . . . . . . . . . . . 653 Lesions of the Cranial Nerves Subserving Eye Movement and their Brainstem Nuclei . . . . . . . 654 Ptosis . . . . . . . . . . . . . . . . . . . . . . . 662 Treatment of Oculomotor Disorders . . . . . . . . . . . . . . . . . . . . 663 Pupillary Disorders . . . . . . . . . . . . 664 Anatomy and Clinical Examination of the Pupils . . . . . . . . . . . 664 Abnormal Size and Shape of the Pupils . . . . . . . . . . . . . . . . . . . 664 Abnormalities of Pupillary Reactivity . . . . . . . . . . . . . . . . . . . 664 Trigeminal Disturbances . . . . . . . 670 Facial Nerve Disturbances . . . . . . 673 Cryptogenic Peripheral Facial Nerve Palsy . . . . . . . . . . . . . . . . . . 673 Bilateral Facial Nerve Palsy . . . 676 Melkersson-Rosenthal Syndrome . . . . . . . . . . . . . . . . . . . 677 Other Causes of Peripheral Facial Nerve Palsy . . . . . . . . . . . 677 Disturbances of Taste . . . . . . . . 678 Hemifacial Spasm ... . . . . . . . . 678 Facial Myokymia . . . . . . . . . . . . . 679 Facial Tic . . . . . . . . . . . . . . . . . . . . 679 Progressive Facial Hemiatrophy . . . . . . . . . . . . . . . . 679 Disturbances of the Vestibulocochlear Nerve (Statoacoustic Nerve, Auditory Nerve) . . . . . . . . . 680 Anatomy . . . . . . . . . . . . . . . . . . . . 680 Testing of Hearing . . . . . . . . . . . 680 Cochlear, Retrocochlear, or Central Hearing Loss? . . . . . . . . 682 Diseases Causing Hearing Loss . . . . . . . . . . . . . . . . . . . . . . . . . 683 Ancillary Tests in the Diagnostic Evaluation of Hearing Loss . . . 690 Tinnitus and Other Abnormal Sounds . . . . . . . . . . . . . . . . . . . . . . 690 Vertigo . . . . . . . . . . . . . . . . . . . . . . 691 Diseases Causing Prominent Vertigo . . . . . . . . . . . . . . . . . . . . . . 702 Glossopharyngeal and Vagus Nerve Dysfunction . . . . . . . . . . . . . 712 Accessory Nerve Palsy . . . . . . . . . . 713 Hypoglossal Nerve Palsy . . . . . . . 715 Multiple Cranial Nerve Palsies . . 716 Cranial Polyradiculitis . . . . . . . . 716 (Recurrent) Multiple Cranial Nerve Palsies . . . . . . . . . . . . . . . . 716 Progressive Palsies of Multiple Cranial Nerves . . . . . . . . . . . . . . . 716 Garcin Syndrome . . . . . . . . . . . . 716 Rarer Causes . . . . . . . . . . . . . . . . 716 10 Spinal Radicular Syndromes 717 General Symptoms and Signs . . . 718 Intervertebral Disk Disease as a Cause of Radicular Syndromes . . 728 Cervical Disk Herniation and Spondylosis . . . . . . . . . . . . . . . . . 729 Thoracic Radicular Syndromes . . . . . . . . . . . . . . . . . . 730 Lumbar Disk Herniation . . . . . 730 Mass Lesions in and Adjacent to the Spinal Nerve Roots . . . . . . . . . 738 Other Radicular Syndromes . . . . 739 Herpes Zoster . . . . . . . . . . . . . . . 739 Contents XIII Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. 11 Lesions of Individual Peripheral Nerves 741 General Clinical Features . . . . . 741 Ancillary Tests . . . . . . . . . . . . . . . 742 Classification and Quantification of Peripheral Nerve Lesions . . . . 746 Peripheral Nerve Regeneration . . . . . . . . . . . . . . . . . . . . . . . . . . 748 Pain Syndromes due to Peripheral Nerve Lesions . . . . . . . . . . . . . . . . . . 748 Brachial Plexus Palsies . . . . . . . . . 749 Traumatic Brachial Plexus Palsies . . . . . . . . . . . . . . . . . . . . . . 751 Other Causes of Brachial Plexus Palsy . . . . . . . . . . . . . . . . . 762 Differential Diagnosis of Brachial Plexus Palsies . . . . . . . 766 Long Thoracic Nerve .. . . . . . . . . . 767 Axillary Nerve . . . . . . . . . . . . . . . . . 767 Suprascapular Nerve . . . . . . . . . . . 768 Musculocutaneous Nerve . . . . . . 768 Radial Nerve . . . . . . . . . . . . . . . . . . . 768 Median Nerve . . . . . . . . . . . . . . . . . 771 Ulnar Nerve . . . . . . . . . . . . . . . . . . . 775 Lumbosacral Plexus . . . . . . . . . . . . 780 Genitofemoral and Ilioinguinal Nerves . . . . . . . . . . . . . . . . . . . . . . . . . 780 Femoral Nerve . . . . . . . . . . . . . . . . . 787 Lateral Femoral Cutaneous Nerve (Meralgia Paresthetica) . . . . . . . . . 788 Obturator Nerve . . . . . . . . . . . . . . . 789 Gluteal Nerves . . . . . . . . . . . . . . . . . 790 Sciatic Nerve . . . . . . . . . . . . . . . . . . . 791 Common Peroneal Nerve . . . . . . . 792 Tibial Nerve . . . . . . . . . . . . . . . . . . . 795 12 Headache and Facial Pain 797 General Aspects . . . . . . . . . . . . . . . . 798 History-Taking from Patients with Headache . . . . . . . . . . . . . . 798 Classification of Headache and Facial Pain . . . . . . . . . . . . . . 798 Examination of Patients with Headache . . . . . . . . . . . . . . 802 Pathogenesis of (Primary) Headache . . . . . . . . . . . . . . . . . . . 802 The Major Primary Headache Syndromes . . . . . . . . . . . . . . . . . . . . 803 Tension-Type Headache . . . . . . 803 Post-Traumatic Headache . . . . 804 Migraine . . . . . . . . . . . . . . . . . . . . 805 Headache in Organic Vascular Disease . . . . . . . . . . . . . . . . . . . . . . . . 815 Cranial Arterial Occlusion . . . . 815 Aneurysmal Subarachnoid Hemorrhage . . . . . . . . . . . . . . . . . 815 Arterial Hypertension . . . . . . . . 816 Pheochromocytoma . . . . . . . . . 816 Temporal Arteritis . . . . . . . . . . . 816 Spondylogenic Headache and Cervical Migraine . . . . . . . . . . . . . . 817 Neck-Tongue Syndrome . . . . . . 818 Other Symptomatic Forms of Headache . . . . . . . . . . . . . . . . . . . . . . 818 Headache Due to an Intracranial Mass . . . . . . . . . . . . . . . . . . . . . . . . 818 Headache Due to Intermittent Obstruction of CSF Flow . . . . . 819 Syndrome of Low Cerebrospinal Fluid Volume (Hypoliquorrhea) . . . . . . . . . . . . . . . . . . 819 Pseudotumor Cerebri . . . . . . . . 820 Headache Due to Ocular Disorders . . . . . . . . . . . . . . . . . . . . 820 Headache Due to Disorders of the Ear, Nose, and Throat . . 820 Headache Due to Systemic Disease . . . . . . . . . . . . . . . . . . . . . . 820 Psychogenic Headache . . . . . . . 821 Drug-Induced Headache . . . . . 821 XIV Contents Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Facial Pain . . . . . . . . . . . . . . . . . . . . . 821 Neuralgias . . . . . . . . . . . . . . . . . . . 821 Other Types of Facial Pain . . . . 825 13 Pain Syndromes of the Limbs and Trunk 833 Pain in the Shoulder and Arm (Cervicobrachialgia) . . . . . . . . . . . . 835 Pain Syndromes of the Trunk and Back . . . . . . . . . . . . . . . . . . . . . . 839 Pain in the Trunk . . . . . . . . . . . . 839 Back Pain . . . . . . . . . . . . . . . . . . . . 840 Pelvic Pain and Pain in the Lower Limb . . . . . . . . . . . . . . . . . . . . 843 Other Regional and Generalized Pain Syndromes . . . . . . . . . . . . . . . . 848 14 Myopathies 851 General Aspects . . . . . . . . . . . . . . . . 851 Muscular Dystrophies . . . . . . . . . . 861 Myotonias and Periodic Paralyses . . . . . . . . . . . . . . . . . . . . . . 877 The Myotonias . . . . . . . . . . . . . . . 877 Heterogeneous Syndromes Involving Excessive Muscle Fiber Activity . . . . . . . . . . . . . . . . 882 Periodic Paralyses ... . . . . . . . . 884 Metabolic Myopathies . . . . . . . . . 888 General Aspects of Energy Metabolism in Muscle . . . . . . . 888 Clinical Presentation and Diagnostic Evaluation of the Metabolic Myopathies . . . . . . . 889 Specific Metabolic Myopathies . . . . . . . . . . . . . . . . . . 891 Myoglobinuria . . . . . . . . . . . . . . . 893 Mitochondrial Encephalomyopathies . . . . . . . . . . . . . . . . . . . . . . . . 895 Congenital Myopathies . . . . . . . . . 899 Myositis . . . . . . . . . . . . . . . . . . . . . . . 901 Myopathy in Endocrine Diseases . . . . . . . . . . . . . . . . . . . . . . . 907 Muscular Manifestations of Electrolyte Disturbances . . . . . . . 908 Muscular Manifestations Due to Medications, Intoxications, and Nutritional Deficiencies . . . . 909 Disorders of Neuromuscular Transmission . . . . . . . . . . . . . . . . . . 911 Myasthenia Gravis (Myasthenia Gravis Pseudoparalytica, Erb- Goldflam Disease) . . . . . . . . . . . 911 Lambert-Eaton Myasthenic Syndrome . . . . . . . . . . . . . . . . . . . 922 Congenital Myasthenic Syndromes . . . . . . . . . . . . . . . . . . 923 Other Myasthenic Syndromes . . . . . . . . . . . . . . . . . . 924 Common Muscle Cramps . . . . . . . 924 15 References 925 See also http://www.thieme.com/mm-refs Contents XV Mumenthaler, Neurology © 2004 Thieme All rights reserved. Usage subject to terms and conditions of license. Appendix 927 Scales for the Assessment of Neurologic Disease 928 Detailed Instructions for the Unified Parkinson’s Disease Rating Scale (UPDRS) . . . . . . . . . . 930 Simplified Scale for Evaluating the Severity of Individual Signs of Parkinson’s Disease . . . . . . . . . . 938 Epworth Sleepiness Questionnaire . . . . . . . . . . . . . . . . . . 939 Barthel Index (of Disability) . . . . 940 Modified Rankin Scale (for Stroke) . . . . . . . . . . . . . . . . . . . . 941 Modified NIH Stroke Scale (adapted from Brett et al. and Lyden et al.) . . . . . . . . . . . . . . . . . . . 942 Expanded Disability Status Scale (DSS) for Multiple Sclerosis . . . . 946 Major Neurogenetic Diseases . . . 947 Glossary of Common Abbreviations in Neurology . . . . . . . . . . . .