
- •Overview
- •Preface
- •Translator’s Note
- •Contents
- •1. Fundamentals
- •Microscopic Anatomy of the Nervous System
- •Elements of Neurophysiology
- •Elements of Neurogenetics
- •General Genetics
- •Neurogenetics
- •Genetic Counseling
- •2. The Clinical Interview in Neurology
- •General Principles of History Taking
- •Special Aspects of History Taking
- •3. The Neurological Examination
- •Basic Principles of the Neurological Examination
- •Stance and Gait
- •Examination of the Head and Cranial Nerves
- •Head and Cervical Spine
- •Cranial Nerves
- •Examination of the Upper Limbs
- •Motor Function and Coordination
- •Muscle Tone and Strength
- •Reflexes
- •Sensation
- •Examination of the Trunk
- •Examination of the Lower Limbs
- •Coordination and Strength
- •Reflexes
- •Sensation
- •Examination of the Autonomic Nervous System
- •Neurologically Relevant Aspects of the General Physical Examination
- •Neuropsychological and Psychiatric Examination
- •Psychopathological Findings
- •Neuropsychological Examination
- •Special Considerations in the Neurological Examination of Infants and Young Children
- •Reflexes
- •4. Ancillary Tests in Neurology
- •Fundamentals
- •Imaging Studies
- •Conventional Skeletal Radiographs
- •Computed Tomography (CT)
- •Magnetic Resonance Imaging (MRI)
- •Angiography with Radiological Contrast Media
- •Myelography and Radiculography
- •Electrophysiological Studies
- •Fundamentals
- •Electroencephalography (EEG)
- •Evoked potentials
- •Electromyography
- •Electroneurography
- •Other Electrophysiological Studies
- •Ultrasonography
- •Other Ancillary Studies
- •Cerebrospinal Fluid Studies
- •Tissue Biopsies
- •Perimetry
- •5. Topical Diagnosis and Differential Diagnosis of Neurological Syndromes
- •Fundamentals
- •Muscle Weakness and Other Motor Disturbances
- •Sensory Disturbances
- •Anatomical Substrate of Sensation
- •Disturbances of Consciousness
- •Dysfunction of Specific Areas of the Brain
- •Thalamic Syndromes
- •Brainstem Syndromes
- •Cerebellar Syndromes
- •6. Diseases of the Brain and Meninges
- •Congenital and Perinatally Acquired Diseases of the Brain
- •Fundamentals
- •Special Clinical Forms
- •Traumatic Brain injury
- •Fundamentals
- •Traumatic Hematomas
- •Complications of Traumatic Brain Injury
- •Intracranial Pressure and Brain Tumors
- •Intracranial Pressure
- •Brain Tumors
- •Cerebral Ischemia
- •Nontraumatic Intracranial Hemorrhage
- •Infectious Diseases of the Brain and Meninges
- •Infections Mainly Involving the Meninges
- •Infections Mainly Involving the Brain
- •Intracranial Abscesses
- •Congenital Metabolic Disorders
- •Acquired Metabolic Disorders
- •Diseases of the Basal Ganglia
- •Fundamentals
- •Diseases Causing Hyperkinesia
- •Other Types of Involuntary Movement
- •Cerebellar Diseases
- •Dementing Diseases
- •The Dementia Syndrome
- •Vascular Dementia
- •7. Diseases of the Spinal Cord
- •Anatomical Fundamentals
- •The Main Spinal Cord Syndromes and Their Anatomical Localization
- •Spinal Cord Trauma
- •Spinal Cord Compression
- •Spinal Cord Tumors
- •Myelopathy Due to Cervical Spondylosis
- •Circulatory Disorders of the Spinal Cord
- •Blood Supply of the Spinal Cord
- •Arterial Hypoperfusion
- •Impaired Venous Drainage
- •Infectious and Inflammatory Diseases of the Spinal Cord
- •Syringomyelia and Syringobulbia
- •Diseases Mainly Affecting the Long Tracts of the Spinal Cord
- •Diseases of the Anterior Horns
- •8. Multiple Sclerosis and Other Myelinopathies
- •Fundamentals
- •Myelin
- •Multiple Sclerosis
- •Other Demyelinating Diseases of Unknown Pathogenesis
- •9. Epilepsy and Its Differential Diagnosis
- •Types of Epilepsy
- •Classification of the Epilepsies
- •Generalized Seizures
- •Partial (Focal) Seizures
- •Status Epilepticus
- •Episodic Neurological Disturbances of Nonepileptic Origin
- •Episodic Disturbances with Transient Loss of Consciousness and Falling
- •Episodic Loss of Consciousness without Falling
- •Episodic Movement Disorders without Loss of Consciousness
- •10. Polyradiculopathy and Polyneuropathy
- •Fundamentals
- •Polyradiculitis
- •Cranial Polyradiculitis
- •Polyradiculitis of the Cauda Equina
- •Polyneuropathy
- •Fundamentals
- •11. Diseases of the Cranial Nerves
- •Fundamentals
- •Disturbances of Smell (Olfactory Nerve)
- •Neurological Disturbances of Vision (Optic Nerve)
- •Visual Field Defects
- •Impairment of Visual Acuity
- •Pathological Findings of the Optic Disc
- •Disturbances of Ocular and Pupillary Motility
- •Fundamentals of Eye Movements
- •Oculomotor Disturbances
- •Supranuclear Oculomotor Disturbances
- •Lesions of the Nerves to the Eye Muscles and Their Brainstem Nuclei
- •Ptosis
- •Pupillary Disturbances
- •Lesions of the Trigeminal Nerve
- •Lesions of the Facial Nerve
- •Disturbances of Hearing and Balance; Vertigo
- •Neurological Disturbances of Hearing
- •Disequilibrium and Vertigo
- •The Lower Cranial Nerves
- •Accessory Nerve Palsy
- •Hypoglossal Nerve Palsy
- •Multiple Cranial Nerve Deficits
- •12. Diseases of the Spinal Nerve Roots and Peripheral Nerves
- •Fundamentals
- •Spinal Radicular Syndromes
- •Peripheral Nerve Lesions
- •Fundamentals
- •Diseases of the Brachial Plexus
- •Diseases of the Nerves of the Trunk
- •13. Painful Syndromes
- •Fundamentals
- •Painful Syndromes of the Head And Neck
- •IHS Classification of Headache
- •Approach to the Patient with Headache
- •Migraine
- •Cluster Headache
- •Tension-type Headache
- •Rare Varieties of Primary headache
- •Symptomatic Headache
- •Painful Syndromes of the Face
- •Dangerous Types of Headache
- •“Genuine” Neuralgias in the Face
- •Painful Shoulder−Arm Syndromes (SAS)
- •Neurogenic Arm Pain
- •Vasogenic Arm Pain
- •“Arm Pain of Overuse”
- •Other Types of Arm Pain
- •Pain in the Trunk and Back
- •Thoracic and Abdominal Wall Pain
- •Back Pain
- •Groin Pain
- •Leg Pain
- •Pseudoradicular Pain
- •14. Diseases of Muscle (Myopathies)
- •Structure and Function of Muscle
- •General Symptomatology, Evaluation, and Classification of Muscle Diseases
- •Muscular Dystrophies
- •Autosomal Muscular Dystrophies
- •Myotonic Syndromes and Periodic Paralysis Syndromes
- •Rarer Types of Muscular Dystrophy
- •Diseases Mainly Causing Myotonia
- •Metabolic Myopathies
- •Acute Rhabdomyolysis
- •Mitochondrial Encephalomyopathies
- •Myositis
- •Other Diseases Affecting Muscle
- •Myopathies Due to Systemic Disease
- •Congenital Myopathies
- •Disturbances of Neuromuscular Transmission−Myasthenic Syndromes
- •15. Diseases of the Autonomic Nervous System
- •Anatomy
- •Normal and Pathological Function of the Autonomic Nervous System
- •Sweating
- •Bladder, Bowel, and Sexual Function
- •Generalized Autonomic Dysfunction
- •Index

Other Ancillary Studies |
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Indications. The velocity and flow profile (laminar or turbulent) of the blood flowing within a particular vessel depend, among other things, on the vessel’s caliber and on the nature of its wall. Ultrasound studies aid in the detection of vascular stenosis and occlusion, vessel wall irregularities, abnormalities of the speed and direction of blood flow, and turbulent flow. Insonation of the extraand intracranial vessels (e. g., of the middle cerebral a. through the thin bone of the “temporal window,” or of the basilar a. through the foramen magnum) yields an informative picture of the current state of blood flow in the brain (Fig. 4.27). This diagnostic technique is inexpensive, non-invasive, and free of risk.
Fig. 4.27 c MR angiography reveals occlusion of the internal carotid artery.
Other Ancillary Studies
Cerebrospinal Fluid Studies
Technique. Cerebrospinal fluid is usually obtained by lumbar puncture (LP) below the level of the conus medullaris, i. e., at L4−5 (occasionally at L3−4 or L5−S1). Suboccipital puncture is fraught with a much higher rate of complications and is performed only when meningitis is suspected and no fluid can be obtained by lumbar puncture (“dry tap”), or when LP is contraindicated because of a known purulent process in the lumbar region. LP is performed with sterile technique on a patient in the lateral decubitus position (or, occasionally, sitting up). The recommended positioning is shown in Fig. 4.28. The physician performing the puncture measures the CSF pressure with a manometer and visually assesses the color of the fluid. The laboratory tests to be performed include cell count, glucose and protein content, and others (esp. cultures), depending on the clinical situation. The most important CSF tests are listed in Table 4.7.
Normal CSF values are listed in Table 4.8 together with the corresponding serum values.
Indications. Lumbar puncture is useful in the diagnosis of diseases affecting the meninges, the brain and spinal cord, and the nerve roots, which can manifest themselves with changes in the biochemical or cellular properties of the cerebrospinal fluid. The most important abnormal CSF findings are listed in Table 4.9.
Table 4.7 Clinically relevant CSF studies
Routinely performed tests pressure
color (turbidity? xanthochromia? bloody tinge?) cell count and differential
protein glucose
Tests to be performed under special circumstances immunoglobulins
IgG−albumin index oligoclonal bands
measurement of specific IgG, IgA, and IgM against Borrelia, parasites, and viruses
cultures: bacterial, fungal, viral, mycobacterial gram and Ziehl−Neelsen staining, touch prep VDRL and FTA tests for syphilis
cytological examination for malignant cells
DNA amplification (polymerase chain reaction) in suspected tuberculosis or viral diseases
cystatin C in amyloid angiopathy
antineuronal antibodies in suspected paraneoplastic syndromes
4
Ancillary Tests
Fig. 4.28 Patient position for lumbar puncture. |
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64 4 Ancillary Tests in Neurology
Contraindications. Intracranial hypertension is the most important contraindication to lumbar puncture. Before any LP is performed, the patient’s optic discs should be inspected with an ophthalmoscope to rule out papilledema. Nor should an LP ever be performed if the platelet count is below 5000/μl. It should only rarely be performed, for strict indications and with extreme caution, in anticoagulated patients or when the platelet count is below 20 000/μl.
Complications of lumbar puncture are rare overall. If the patient harbors an intracranial mass causing elevated intracranial pressure, CSF removal may be followed by herniation of parts of the brain into the ten-
Table 4.8 Normal CSF values and corresponding serum values in adults1
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CSF |
Serum |
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Pressure |
5−18 cm H2O |
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Volume |
100−160 ml |
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Osmolarity |
292−297 mosm/l |
285−295 mosm/l |
Electrolytes |
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Na |
137−145 mmol/l |
136−145 mmol/l |
K |
2.7−3.9 mmol/l |
3.5−5.0 mmol/l |
Ca |
1.0−1.5 mmol/l |
2.2−2.6 mmol/l |
Cl |
116−122 mmol/l |
98−106 mmol/l |
pH |
7.31−7.34 |
7.38−7.44 |
Glucose |
2.2−3.9 mmol/l |
4.2−6.4 mmol/l |
CSF/serum glucose |
0.5−0.6 |
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ratio1 |
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Lactate |
1−2 mmol/l |
0.6−1.7 mmol/l |
Total protein |
0.2−0.5 g/l |
55−80 g/l |
Albumin |
56−75 % |
50−60 % |
IgG |
0.010−0.014 g/l |
8−15 g/l |
IgG index2 |
0.65 |
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Leukocytes |
4/μl |
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Lymphocytes |
60−70 % |
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1Because there is normally an equilibrium between CSF and serum, it is advisable to measure CSF and serum values at the same time.
2IgG index = [CSF IgG (mg/l) serum albumin (g/l)]/[serum IgG (mg/l) CSF albumin (mg/l)]
Table 4.9 CSF analysis: main indications and findings
torial notch or the foramen magnum, potentially resulting in death. If an intraspinal mass is present, preexisting paraparesis may worsen after LP. After the procedure is performed, persistent leakage of CSF out of the subarachnoid space through the puncture hole(s) in the dura mater may result in symptomatic intracranial hypotension with orthostatic headache. Other possible complications include iatrogenic infection and epidural hematoma, potentially causing cauda equina syndrome.
Tissue Biopsies
Muscle biopsy is justified in patients with neuromuscular disease when the clinical history, physical examination, and electromyographic, chemical, and/or genetic studies fail to yield a sufficiently precise diagnosis. The biopsy should be performed under local anesthesia in a muscle that is known to be affected by the disease process, but is not so atrophic as to reduce the chance of a diagnosis. In many cases, a needle biopsy alone suffices. Depending on the clinical situation, histochemical and/or electron-microscopic study of the tissue specimen may be indicated in addition to conventional histological staining.
Nerve biopsy is performed under local anesthesia. A relatively unimportant sensory nerve is chosen for biopsy, usually the sural n. The ensuing sensory deficit on the lateral edge of the foot is generally an acceptable price to pay for a firm diagnosis, but the patient must be informed of it before granting his or her consent to the procedure. Part of the specimen is used to make a teased preparation in which nerve fibers and their myelin sheaths can be seen over a certain length of nerve. More importantly, very thin cross-sections of the nerve are prepared, which can be microscopically examined for various abnormalities, including disordered myelination or inflammatory changes of the vasa nervorum.
Brain biopsy is performed by a neurosurgeon, usually with stereotactic technique, for very strict indications.
Condition/suspected pathology |
Appearance |
Cell count and type |
Protein |
Pressure |
Special remarks |
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Purulent meningitis |
turbid |
mostly |
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possibly |
LP is urgent—the most |
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granulocytes |
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important diagnostic |
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study |
Chronic meningitis |
clear |
mostly lympho- |
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possibly |
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cytes |
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Encephalitis |
clear |
mostly lympho- |
possibly |
possibly |
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cytes |
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Subarachnoid hemorrhage |
bloody— |
erythrocytes |
possibly |
possibly |
xanthochromia in 6 |
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xanthochromic |
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hours to 6 days |
Intracerebral hemorrhage |
xanthochromic |
possibly erythro- |
normal |
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LP not indicated |
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cytes |
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Subdural hematoma |
xanthochromic |
usually normal |
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normal, , |
LP not indicated |
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Low CSF pressure syndrome |
clear |
normal |
to |
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aspirate if no spon- |
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taneous CSF flow |
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Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.

Other Ancillary Studies |
65 |
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Its purpose is the histological diagnosis of (potentially treatable) structural alterations of the brain whose presence has been revealed by imaging studies, but whose precise nature is nonetheless unclear. Examples are brain tumors and inflammatory processes.
Perimetry
Perimetry is used to detect visual field defects (p. 181).
Goldmann perimetry is a dynamic method in which moving spots of light of variable size and intensity are
presented in the patient’s visual field, starting in the periphery and moving toward the center. The findings associated with different types of visual field defect are illustrated in Fig. 3.6, p. 19.
Static computed perimetry is performed with the socalled Octopus apparatus. The brightness of a stationary light source is increased until the patient can see it. The measured brightness thresholds at all tested points in the visual field can be displayed visually as raw numbers, on a gray scale, or as a pseudo-three-dimensional visual field “landscape.” Illustrative findings in a case of homonymous quadrantanopsia are shown in Fig. 4.29.
Fig. 4.29 Automatic (Oc- |
a |
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topus) perimetry in right |
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Gray scale of measured values |
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Gray scale of measured values |
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homonymous hemianop- |
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30° |
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30° |
sia. a Gray-scale represen- |
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tation of the visual field |
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defect. b Differential value |
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chart representing the loss |
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of light sensitivity at each |
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point in the visual field, |
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measured in decibels (dB), |
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as compared to the aver- |
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age local sensitivity in a |
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normal control population. |
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There is no measurable |
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loss at the points marked |
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with solid black squares. |
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See also Fig. 3.6. |
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b |
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Differential values |
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Differential values |
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30° |
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30° |
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4
Ancillary Tests
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Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme
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All rights reserved. Usage subject to terms and conditions of license.