- •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
58 4 Ancillary Tests in Neurology
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the pyramidal pathway to the muscles. Surface elec- |
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trodes placed on an arm or leg muscle are used to record |
Stimulation |
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the summed motor potentials. These potentials are |
right |
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larger and easier to record when the subject lightly con- |
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tracts the corresponding muscle beforehand. An abnor- |
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mality of the MEP implies a lesion in the peripheral or |
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P100: 147ms |
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central portion of the motor pathway (see Fig. 4.21). |
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Epilepsy, cardiac pacemakers, and ferromagnetic in- |
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tracranial implants are contraindications for trans- |
Stimulation |
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cranial magnetic stimulation for any purpose, including |
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MEP. |
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left |
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2µV |
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Electromyography |
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50ms |
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P100: 100ms |
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Principle. Electrical activity is recorded from a muscle |
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Fig. 4.19 Visual evoked potentials (VEP). A 38-year-old woman |
through bipolar needle electrodes, first at rest, and then |
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with multiple sclerosis and right optic neuritis. The cortical re- |
with light and maximal voluntary muscle contraction. |
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sponse on the right side is significantly delayed compared to the |
The recorded potentials are displayed visually on an |
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normal left side. |
oscilloscope and also converted into audible signals |
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from a loudspeaker. When the muscle is lightly con- |
Somatosensory evoked potentials (SSEP). When a re- |
tracted, the potentials arising from individual motor |
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petitive electrical stimulus is applied to the skin, im- |
units can be observed. (A motor unit is the set of muscle |
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pulses are generated at the terminal sensory branch of a |
fibers innervated by a single motor anterior horn cell by |
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peripheral nerve and conducted centrally via the pe- |
way of its multiple axon collaterals.) When the muscle is |
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ripheral nerve, nerve root, posterior columns/ |
strongly or maximally contracted, a large number of |
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spinothalamic tract, medial lemniscus, and thalamocor- |
motor unit potentials come together to form an interfer- |
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tical connections. A lesion at any point along this path- |
ence pattern. |
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way can alter the evoked potentials, which are recorded |
Insertional activity and spontaneous activity. The re- |
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first over Erb point (for the median n.) or the lumbar |
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spine (for the tibial n.), and then through a scalp elec- |
sting muscle is normally electrically silent; when the |
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trode in the parietal region on the side opposite the |
needle is inserted, there are normally only a few positive |
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stimulation. An example of delayed conduction in the |
sharp waves or fibrillations. Pathological spontaneous |
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central somatosensory pathway is shown in Fig. 4.20. |
activity of a muscle is manifested as prolonged in- |
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sertional activity as well as pathological fibrillation |
Motor evoked potentials (MEP). In this technique, a |
potentials and positive sharp waves (Fig. 4.22). This spon- |
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rapidly alternating magnetic field produced by a ring- |
taneous activity reflects denervation of the muscle. |
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shaped magnetic impulse generator induces a stimulat- |
Fasciculations and complex repetitive discharges are |
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ing electrical current in the motor cortex. Action poten- |
further forms of pathological spontaneous activity, as |
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tials are then generated in the cortex and travel down |
are myotonic repetitive discharges. |
Stimulation: right tibial n. |
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P40: 39.2ms |
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Cortical recording |
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1µV |
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Lumbar recording |
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2µV |
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Stimulation: left tibial n. |
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N22: 21.6ms |
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P40: 58.4ms |
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Cortical recording |
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1µV |
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Lumbar recording |
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2µV |
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N22: 21.2ms |
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10ms |
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Fig. 4.20 Somatosensory evoked potentials of the tibial n. A 44-year- old woman with multiple sclerosis. Normal lumbar N22 potential on both sides. The cortical P40 potential appears at a normal latency of 29.2 ms on the right, but is significantly delayed on the left, with a latency of 58.4 ms, and also abnormally small. These findings indicate impaired conduction in the spinothalamic pathways.
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
Electrophysiological Studies |
59 |
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Fig. 4.21 Motor evoked potentials in a 61-year-old man with cervical syringomyelia. Recording of motor potentials from the abductor digiti minimi m. after electrical stimulation of the ulnar n. at the wrist, the forearm, and the C8 root (tracings a−c). After cortical stimulation (d), the recorded motor evoked potential is reduced in amplitude and somewhat delayed. The calculated central motor conduction time (CMCT) of 9.2 ms is prolonged in comparison to the normal value of 8.7 ms. These findings suggest impaired conduction in the pyramidal tract in the cervical spinal cord.
Stimulus:
aWrist
bArm
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Root |
5 mV |
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16.1 ms
d Cortex
1.5 mV
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25.3 ms |
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CMCT = |
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10 ms |
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9.2 ms |
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4
Ancillary Tests
300μV |
5000μV |
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c |
d |
e |
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20ms |
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20ms |
Fig. 4.22 Different types of potentials in an electromyogram. a Normal motor unit potential. b Fibrillation potential in denervation. c Positive sharp waves in denervation. d Fragmented poly-
Electrical activity with voluntary contraction. Muscle action potentials are observed when the muscle is voluntarily contracted. The amplitude and duration of individual motor unit potentials are proportional to the size of the motor unit, i. e., the number of muscle fibers it contains. The more strongly a muscle is contracted, the more motor units will be recruited. When a large number of motor units are active, their potentials can no longer be seen individually. Instead, they summate to form a (complete) interference pattern (Fig. 4.23a).
The size and shape of electromyographic potentials are altered by many different types of neuromuscular disease. Myopathy is characterized by a diffuse loss of individual muscle fibers throughout the affected
phasic low-amplitude potential, as seen in reinnervation. e Abnormally prolonged and high-amplitude motor unit potential (“giant potential”) in chronic anterior horn cell disease.
muscle(s). Each motor unit potential is therefore of lower amplitude and shorter duration (Fig. 4.23d). In principle, all of the motor units are still present, but they contain fewer muscle fibers than before; thus, on maximal voluntary contraction of the muscle, the interference pattern is full, but of lower than normal amplitude. In contrast, in a neuropathic process (chronic denervation of a muscle), the motor units are larger than normal because of repeated denervation and reinnervation. When the nerve fiber to a particular motor unit degenerates, axon collaterals sprouting from the nerves of adjacent motor units take over the muscle fibers of the denervated unit, so that the surviving motor units actually contain more muscle fibers than before. Their
adfsköb
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme
adköb
All rights reserved. Usage subject to terms and conditions of license.
60 4 Ancillary Tests in Neurology
20ms
a
2mV |
20ms |
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b
1s |
2mV |
10ms |
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c
0.2mV |
20ms |
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d
2mV
Tabelle 4.6 Indications for EMG and ENG
Condition/suspected pathology
Suspected anterior horn cell disease
Suspected nerve root lesion
Suspected plexus lesion (differentiation from peripheral nerve lesion)
Focal peripheral nerve lesion
Polyneuropathy
Myopathy
Ischemic muscle damage
Myasthenia gravis
++ |
= |
indicated test |
+ |
= |
may be additionally useful |
Fig. 4.23 Various EMG findings. a Normal electromyogram with full interference pattern. b Individual oscillations in the reinnervation stage after a peripheral nerve injury. c Total denervation. Fibrillation potentials and positive sharp waves are seen. d Myopathy. Despite muscle weakness, there is a complete interference pattern. The individual potentials making up the interference pattern are of low amplitude; some of them are polyphasic and fragmented.
motor unit potentials are usually polyphasic and of increased amplitude and duration (Fig. 4.23b). Because of the reduced number of motor units, maximal voluntary contraction of a denervated muscle yields a markedly attenuated interference pattern, in which the individual action potentials of the remaining motor units appear as large oscillations.
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Electrical activity at the motor end plate. Abnormali- |
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ties of the motor end plate affecting neuromuscular |
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transmission are also revealed by EMG. On repetitive |
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0.4mV |
electrical stimulation of a peripheral motor nerve, the |
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recorded muscle action potential becomes smaller with |
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each stimulus (decrement phenomenon, Fig. 4.12, p. 53). |
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Indications. In disorders affecting muscle, EMG can be |
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used to determine whether the underlying pathological |
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process is located in the muscle itself (myopathic |
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process), in the nerve innervating it (neuropathic |
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process), or at the neuromuscular junction. It can also be |
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used to grade the severity of muscle denervation and |
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the extent of reinnervation. In combination with elec- |
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troneurography (see below), EMG is a very important |
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type of ancillary study for the diagnosis of neuromuscu- |
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lar diseases. The indications for these two methods are |
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listed side by side in Table 4.6. |
Electroneurography
Principle. Electroneurography is a method of measuring the motor and sensory conduction velocities of peripheral nerves. The result of measurement is always the
EMG (needle |
ENG |
Remarks |
myography) |
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++ |
Negative |
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+ |
++ (F wave) |
Imaging studies may be |
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more important |
+ |
++ (F wave) |
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++ |
++ |
Severity of injury, signs |
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of regeneration, localiza- |
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tion of injury |
+ |
++ |
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Normal |
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++ |
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Repetitive stimulation, |
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jitter phenomenon |
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.