- •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
54 4 Ancillary Tests in Neurology
Electrophysiological Studies
Fundamentals
Electrophysiological processes are an intrinsic part of all cellular activity (p. 4). Differences in electrical potential and changes in these differences over time can be amplified, displayed on an oscilloscope, and recorded on paper or in digitized form. Electroencephalography records the activity of cortical neurons and neuronal populations and electromyography that of muscle cells. The conduction of spontaneous or induced impulses in peripheral nerves is assessed by electroneurography. Repeated stimulation of the receptors of a particular sensory system (e. g., the retina, by visual stimuli) and simultaneous measurement of the resulting cortical activity enables determination of the conduction velocity within the sensory system in question (evoked potential studies). The complex electrophysiological phenomena that occur during sleep are registered by somnography (sleep studies). These electrophysiological diagnostic
techniques offer a practically riskfree means of assessing the functional state of the nervous system, though some of them are rather unpleasant for the patient. Despite the absence of risk, they should only be performed for strict indications, in accordance with the general principles outlined above on p. 45.
The techniques discussed in this chapter are in widespread use and belong to the diagnostic armamentarium of any clinical neurophysiologist.
Electroencephalography (EEG)
Principle. The surface EEG registers fluctuations in electrical potential that are generated by the cerebral cortex. These represent the sum of the excitatory and inhibitory synaptic potentials.
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
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Fig. 4.15 Normal EEG. a Monopolar recording, b bipolar recording.
Electrophysiological Studies |
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Ancillary Tests
Technique. Electrodes are placed on the scalp according to the internationally standardized 10−20 system (Fig. 4.14). The potential fluctuations at each electrode are recorded, either in bipolar mode (i. e., differences in potential between adjacent electrodes) or in unipolar mode (i. e., differences in potential between each electrode and a reference electrode). Their magnitude at the scalp is 10−100 μV. They are amplified and recorded on paper in 12 parallel channels. Fluctuations in electrical potential are classified by frequency. Certain maneuvers, e. g., opening and closing the eyes, hyperventilation, and rhythmic photic stimulation, affect the EEG tracing in characteristic ways and may induce pathological waves in patients with epilepsy.
Evaluation. A mainly occipital alpha rhythm is the major component of the EEG tracing in a normal, awake individual. There is a progressive slowing of frequencies during sleep, depending on the sleep stage (depth of sleep). The following EEG changes indicate a pathological process in the brain:
Fig. 4.14 Placement of EEG electrodes according to the 10−20 system (a−c from Masuhr K.F., Neumann M.: Neurologie, Hippokrates, Stuttgart 1992; d from Künkel H.: Das EEG in der neurologischen Diagnostik, in Schliack H., Hopf H.C.: Diagnostik in der Neurologie, Thieme, Stuttgart 1988). a Lateral view. The electrodes are placed at fixed percentage intervals between the nasion and the inion. b Frontal view. The preauricular points serve as reference points for the placement of the central transverse row of electrodes. C2 is the intersection of the central transverse and longitudinal rows. c Superior view. d Names of the electrodes in the 10− 20 system.
General changes. Slowing of the background rhythm in the awake patient is abnormal, as is acceleration of background activity (e. g., in the form of a beta rhythm). The latter is often due to medication use.
Focal findings. Slowing of background activity (e. g., in the form of theta or delta waves) limited to a circumscribed area of the brain reflects focal cortical disfunction. Findings of this type are often due to structural lesions of the brain (e. g., tumors).
Sharp waves and spikes. These characteristically shaped abnormal potentials are seen in persons with epilepsy. During a seizure, characteristic seizure-related potentials appear (spikes with a prolonged following wave— the “spike and wave” pattern). Pathological EEG changes are not necessarily demonstrable between seizures; thus, a normal interictal EEG does not rule out epilepsy.
An example of a normal EEG is shown in Fig. 4.15 and the most important graphoelements of the EEG are shown schematically in Fig. 4.16.
Indications. The main indications for EEG are summarized in Table 4.5. EEG changes are also seen in many other processes affecting the brain. The most important pathological EEG rhythms are shown in Fig. 4.16.
Polysomnography
Technique. Polysomnography is a special application of EEG in which the EEG is recorded simultaneously with a number of other electrophysiological parameters. It is used to assess sleep and sleep disturbances. The EEG
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4 Ancillary Tests in Neurology |
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Table 4.5 The main indications for electroencephalography |
changes that normally occur during sleep are related to |
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the progression of the individual through various sleep |
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Confirmation of the diagnosis of epilepsy |
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stages, including deep or REM sleep (REM = “rapid eye |
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Determination of the type of epilepsy that is present |
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movement”). The recorded parameters include eye |
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Brief, episodic impairment of consciousness of unknown etiology |
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movements (by electro-oculography), respiratory ex- |
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Longer-lasting disturbances of consciousness, delirium |
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cursion, airflow in the nostrils, muscle activity (by sur- |
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Metabolic disturbances |
face EMG), cardiac activity (by ECG), and the partial |
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Creutzfeldt−Jakob disease |
pressure of oxygen (by transcutaneous pulse oximetry) |
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Sleep studies (e. g., in suspected narcolepsy) |
(Fig. 4.17). These are displayed together with the EEG in |
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Designation |
Morphology |
Definition |
1 β rhythm |
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Regular sequence of |
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Regularly waxing and |
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waning waves |
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at 14–30 Hz |
3 |
α rhythm |
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Regular sequence of |
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waves at 8–13.3 Hz |
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ϑ rhythm |
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Regular sequence of |
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waves at 4–7 Hz |
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5 |
δ rhythm |
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Regular sequence of |
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waves at 1–3.5 Hz |
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6 |
δ activity |
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Irregular sequence of |
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polymorphic waves |
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at 1–3.5 Hz |
7 |
Subdelta |
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Wave with duration |
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wave |
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> 1 s |
8 |
Steep waves |
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Conspicuous, blunt, |
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(steep |
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steep individual waves |
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potential) |
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Sharp waves |
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Sharp and steep waves |
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of 80–250 ms duration, |
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potential) |
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usually steeper than |
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descending phase |
10 |
Spike |
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Sharp and steep wave |
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of duration < 80 ms |
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11 |
Polyspikes |
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Compact series |
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of spikes |
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12 |
Spike–wave |
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Complex consisting |
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complex |
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wave |
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13 |
Rhythmic |
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Sequence of regular |
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spikes and |
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spike–wave complexes |
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waves |
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at about 3 Hz |
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Sequence of comp- |
14 |
Sharp and |
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and slow waves of |
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slow waves |
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500–1000 ms dura- |
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2s |
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Fig. 4.16 The most important graphoelements in EEG: designations, morphology, and definitions (from Schliack H., Hopf H.C.: Diagnostik in der Neurologie, Thieme, Stuttgart 1988).
Indications. The most important indication for a sleep study is a clinical suspicion of sleep apnea syndrome (p. 171) on the basis of a characteristic history obtained from the patient or bed partner, together with related physical findings and a low partial pressure of oxygen measured during sleep by pulse oximetry. The typical polysomnographic finding in such patients is shown in Fig. 4.18. Polysomnography is also indicated for the diagnosis of narcolepsy, as well as for the assessment of excessive fatigue and daytime somnolence.
Evoked potentials
General principles. Evoked potentials are used to assess the integrity of individual functional systems (visual, auditory, somatosensory, or motor). The system under study is activated with a repeatedly delivered stimulus. The resulting fluctuations of electrical potential in the brain can be detected by summation of the potentials that are recorded when the excitatory stimulus has been delivered a large number of times. Evoked potentials provide evidence of whether impulse conduction in the system in question is intact from the site of stimulation all the way to the cerebral cortex. Sometimes a partial or total conduction block can be localized precisely between two relay stations for neural transmission within a particular system. In addition, evoked potentials may reveal subclinical lesions. The most important types of evoked potential for clinical practice are outlined in the following paragraphs.
Visual evoked potentials (VEP). The patient fixates on a video screen displaying a checkerboard pattern in which the white and black fields are regularly and periodically inverted, while electrical potentials are recorded through a needle electrode in the scalp at the occiput. Evoked potentials are obtained by summation; the largest fluctuation is a positive wave that appears 100 milliseconds after the stimulus. Delay of this wave is found early in the course of optic neuritis and persists thereafter (Fig. 4.19).
Auditory evoked potentials (AEP). A click stimulus delivered periodically to one ear induces the generation of neural impulses that travel along the auditory nerve to the brainstem, the thalamus, and finally the cerebral cortex. The electrophysiological response is measured from the vertex of the head in relation to a reference electrode on the earlobe. The normal AEP contains five different waves, each of which is generated by a different structure along the chain of impulse transmission.
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme All rights reserved. Usage subject to terms and conditions of license.
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Electrophysiological Studies |
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Fig. 4.17 Recording scheme for |
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C3-A1 |
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polysomnography. |
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Video recording |
EEG |
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EEG |
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ROC |
EOG |
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2-A2 |
LOC |
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Respiratory |
Nasal |
Masseter |
EMG |
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volume |
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Air flow |
“Submentalis” |
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Oral |
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Thoracic |
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ECG |
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Respiratory |
breathing |
Left |
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effort |
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Abdominal |
EMG |
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Biceps brachii |
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breathing |
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Right |
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Oxygen |
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Oximetry |
Left |
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Tests |
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saturation |
Tibialis anterior |
EMG |
Ancillary |
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Right |
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Fig. 4.18 Hypnogram. Polysomnography in a patient with REM-sleep-associated obstructive sleep apnea syndrome. 1 EEG frequency analysis. 2 Rapid eye movement (REM) sleep. 3 Submental muscle activity measured through a surface electrode. 4 Sleep stages. AWK = awake, REM = REM sleep, 1−4 = sleep stages 1−4. 5 Time axis. 6 Nasal/oral air flow and count (cnt) of apneic and hypopneic episodes per minute. 7 Transcutaneously measured oxygen saturation (upper curve) and frequency of desaturations by 4 % or more (lower curve). 8 ECG (bpm = beats per minute) and number of tachycardias, bradycardias, or extrasystoles. 9 Surface EMG from the masseter m. 10 Surface EMG from the right tibialis m. 11 Surface EMG from the left tibialis m. 12 Body position.
adfsköb
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thieme
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