- •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
50 |
4 Ancillary Tests in Neurology |
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Tabelle 4.2 MRI signal intensities of normal and abnormal structures (after Edelmann)1 |
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Tissue |
T1-weighted image |
T2-weighted image |
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Contrast enhancement with gadolinium− |
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DTPA |
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Low concentration |
Very bright |
Bright |
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High concentration |
Intermediate to dark |
Very dark |
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Hematoma |
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Hyperacute |
Intermediate |
Intermediate to bright |
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Acute |
Intermediate to dark |
Dark to very dark |
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Subacute |
Bright rim, intermediate |
Bright rim, dark center, later all bright |
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Chronic |
Dark rim, bright center, later all dark |
Dark rim, bright center, later all dark |
1 Bright = hyperintense, dark = hypointense, intermediate = isointense in comparison to brain tissue
only if it is excited by two radio wave pulses arriving one after the other at the same location. If the blood rapidly passes through the imaging plane, the bit of blood that received the first excitatory pulse has already flowed away by the time the second pulse arrives and no signal is generated—the vessel appears dark (there is a “flow void”). However, if the blood flows slowly enough to receive both pulses in the imaging plane, the vessel appears bright. When gradient-echo sequences are used, flowing blood always appears bright, while stationary tissue appears dark. Computer algorithms can combine the individual sectional images, processing them to generate a projectional image resembling a conventional angiogram; this is a magnetic resonance angiogram (Fig. 4.7). With MR angiography, an occluded carotid artery, for example, can be diagnosed noninvasively. Contrast-en-
a
b
Fig. 4.7 MR angiography of the intracranial vessels. a Coronal and b axial projections. The arteries in this study are normal except for hypoplasia of the main stem of the right anterior cerebral a. (arrow).
hanced MR angiography is currently being performed increasingly often. In this technique, the signal is produced not by the flowing of the blood per se, but by the contrast medium in the bloodstream.
The indications for MRI and CT scanning of the brain and spinal cord are listed and compared with each other in Table 4.1.
Angiography with Radiological Contrast Media
Diagnostic imaging of the cerebral blood vessels is indicated when a vascular stenosis, occlusion, or malformation is suspected as the cause of a neurological illness.
Methods. Conventional arteriography, also known as angiography with radiological contrast media, is indicated for certain special purposes, e. g., the preoperative visualization of intracranial aneurysms or arteriovenous malformations. This type of study involves the introduction of an intra-arterial catheter by way of the femoral a. along a guide wire all the way up to the great vessels supplying the brain. Contrast medium is injected into these vessels while fluoroscopic images are simultaneously obtained. The image changes from one second to the next, as the contrast medium distributes itself in the vascular system of the brain. All of the images are digitized and an image obtained before any contrast medium was injected is subtracted from each to generate a digital subtraction angiogram, which shows nothing but the blood vessels supplying the head and brain (both extraand intracranial). Contrast medium can be injected into the carotid a. to display the anterior circulation (Fig. 4.8), or into the vertebral a. to display the posterior circulation (Fig. 4.9).
The blood vessels of the spinal cord can also be studied angiographically, e. g., for the diagnosis and treatment of spinal arteriovenous malformations or fistulae.
Intravenous angiography has largely been abandoned.
The potential complications of angiography include hemorrhage or dissection at the femoral puncture site, the detachment of atherosclerotic plaques from arterial walls by the tip of the catheter, and the induction of vasospasm with consequent cerebral ischemia, possibly leading to stroke. The contrast media that are used can also have side effects.
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
Imaging Studies
a |
b |
c |
Fig. 4.8 Normal digital subtraction angiogram of the intracranial anterior circulation (carotid distribution). a Anteroposterior projection. b Lateral projection. c Venous phase, lateral projection. a and b: 1 MCA = middle cerebral a., 2 ICA = internal carotid a., 3 ACA = anterior cerebral a., 4 pericallosal a. c: 1 Superior
Fig. 4.9 Selective angiography of the left vertebral a. a Arterial phase, anteroposterior projection. b Arterial phase, lateral projection.
1 posterior cerebral a.
2 superior cerebellar a.
3 anterior inferior cerebellar a. (AICA)
4 left vertebral a.
5 basilar a.
6 posterior inferior cerebellar a. (PICA)
The general rule, when a diagnostic study of the blood vessels is desired, is to choose the type of study that is expected to yield sufficient information for effective diagnosis and treatment while putting the patient at the lowest risk. MR angiography (Fig. 4.10) and Doppler ultrasonography (Fig. 5.61) now suffice for most purposes.
The indications of cerebral angiography are listed in Table 4.3.
cerebral vv. (rolandic and Trolard), 2 superior sagittal sinus, 3 inferior sagittal sinus, 4 septal v., 5 thalamostriate v., 6 internal cerebral v., 7 straight sinus, 8 v. of Labbé = inferior anastomotic v., 9 basal v. of Rosenthal, 10 cavernous sinus, 11 inferior petrosal sinus, 12 lateral sinus, 13 jugular v.
Table 4.3 Indications for angiography of the intracranial vessels
Visualization of saccular aneurysms
Visualization of arteriovenous malformations and fistulae
Detailed representation of saccular aneurysms (after diagnosis by MRI, as an aid to treatment by neurosurgical or interventional neuroradiological methods)
Detailed representation of arteriovenous malformations (after diagnosis by MRI, as an aid to treatment by neurosurgical or interventional neuroradiological methods)
Visualization of other vascular anomalies:
moya−moya
agenesis of vessels and other developmental anomalies
vascular stenosis or occlusion
arterial dissection
Fig. 4.10 Arteriovenous malformation on the surface of the cervical spinal cord. The malformation is visible in this T2-weighted MR image as a void in the midst of the bright CSF signal of the subarachnoid space.
51
4
Ancillary Tests
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52 4 Ancillary Tests in Neurology
Myelography and Radiculography |
Diagnostic Techniques of |
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Technique. Radiculomyelography (the visualization of |
Nuclear Medicine |
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intraspinal structures with contrast medium) is gener- |
CSF Scintigraphy/Isotope Cisternography |
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ally performed after the injection of 10−15 ml of water- |
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soluble contrast medium into the subarachnoid space |
Technique. The subarachnoid space is entered with a |
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via lumbar puncture—or, rarely, via suboccipital punc- |
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ture. The passage of contrast medium through the sub- |
fine needle in the suboccipital or lumbar region and a |
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arachnoid space, including the nerve root sleeves, can |
radiolabeled substance, e. g., human albumin labeled |
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then be followed on the radiologic image and any ob- |
with 131I, is injected into the cerebrospinal fluid. The |
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structions to the flow of contrast medium can be iden- |
radioactive contrast medium should be detectable one |
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tified (e. g., spinal tumors). The nerve roots appear as |
to two hours later in the basal cisterns, four to six hours |
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filling voids within the nerve root sleeves. The bony |
later over the cerebral convexity, and 24 hours later in |
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spine is seen on the myelographic images as well and |
the superior sagittal sinus. In normal individuals, it is |
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can be evaluated at the same time. |
never detected in the lateral ventricles. |
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The indications for myelography and radiculography |
The indications for this type of study are, for example, |
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are listed in Table 4.4 together with those of other, com- |
the localization of a fistula through which CSF is leaking |
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peting types of study. CT and MRI have now replaced |
from the subarachnoid space into the nasal cavity |
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radiculomyelography for many of its earlier indications. |
(where it can be detected on a nasal tampon), or the de- |
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monstration of malresorptive hydrocephalus, in which |
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Findings. Some of the more common myelographic |
contrast medium can be seen to enter the lateral ven- |
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findings are depicted schematically in Fig. 4.11. Further |
tricles (Fig. 4.12). |
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myelographic images can be found elsewhere in this |
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book: lumbar intervertebral disk herniation, Fig. 12.7, |
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p. 212; cervical myelopathy, Fig. 7.8, p. 148; spinal cord |
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tumors, Figs. 7.4−7.7, p. 147.
Table 4.4 Indications for contrast myelography as compared with other imaging techniques
Condition/suspected pathology |
Plain |
CT |
MRI |
Contrast myRemarks |
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radio- |
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elography, |
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graphs |
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radiculogra- |
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phy, myelo-CT |
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Pain without neurologic deficit |
++ |
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Clinically localizable radiculopathy |
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+++ |
++ |
Plain films may be |
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useful, e. g., in |
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vertebral body |
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tumors |
Clinically evident lumbar radiculopathy with unclear CT findings |
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++ |
Suspected radiculopathy, but no clear segmental localization |
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+ |
+++ |
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Suspected spinal cord compression |
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++ |
+++ |
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Suspected spinal stenosis |
++ |
++ |
+++ |
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Clinically evident spinal stenosis |
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++ |
+++ |
Suspected myelopathy due to cervical spondylosis |
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+ |
+++ |
+ |
Suspected myelitis or demyelination |
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+++ |
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+++ = most suitable study, usually adequate for diagnosis; |
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++ = study generally useful; |
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+= study occasionally necessary or indicated in addition to other tests
Fig. 4.11 Typical findings in contrast myelography (schematic diagram).
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
Imaging Studies 53
Fig. 4.12 CSF scintigram in a patient with malresorptive hydrocephalus. After injection of iodine-131-labeled human albumin into the cisterna magna, the radioactive contrast medium refluxes into the lateral ventricles, because of slow CSF flow.
a |
b |
Fig. 4.13 SPECT studies. a Normal study. b SPECT in a patient with Alzheimer disease. Hypoperfusion is seen bilaterally in the parietal and temporal lobes, particularly on the right. Cf. normal finding in a. c This SPECT study in a patient with medically intractable com-
4
Ancillary Tests
c
plex partial seizures, performed after the intravenous administration of 180 MBq of 133I-iomazenil, reveals diminished binding to benzodiazepine receptors in the left temporal region.
SPECT
Technique. Single photon emission computed tomography uses either a 99m-technetium compound or 133I-am- phetamine as a tracer. The purpose of this type of study is to measure regional cerebral blood flow.
Indications. SPECT can be performed to demonstrate reduced perfusion of the brain, e. g., in stroke or in Alzheimer disease, which is associated with reduced activity in the temporoparietal region (Fig. 4.13a, b). It can also be used to detect focal pathological processes of other kinds, e. g., epileptogenic foci (Fig. 4.13c).
PET
Technique. Positron emission tomography uses the short-lived positron-emitting radionuclides 11C, 14O, or 18F. This type of study can therefore only be performed near a cyclotron in which these isotopes are produced. PET can be used to produce quantitative tomographic images of regional cerebral blood flow (rCBF), cerebral blood volume (CBV), oxygen consumption (the cerebral metabolic rate for oxygen = CMRO2), and glucose consumption (CMR−Glu).
Indications. With PET, physicians can perform biochemical studies in vivo. The radioactive labeling of substances metabolized in the human brain makes it possible to measure their concentration and kinetics in specific brain areas. Thus, for example, the localization and concentration of injected DOPA can be studied in patients with suspected Parkinson disease.
adfsköb
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme
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All rights reserved. Usage subject to terms and conditions of license.