- •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
Neuropsychological and Psychiatric Examination 39
Examination of the Autonomic Nervous System
Many clinical tests of the autonomic nervous system have been devised; not a few are rather cumbersome. We will merely mention some of them here: testing of pupillary reactivity after the local application of various substances, measurement of the rise in blood pressure after the administration of ephedrine, observation of changes in blood pressure with orthostasis or on a tilt table, observation and measurement of sweating after warming of the body or observation of local sweating with the aid of pilocarpine iontophoresis, measurement
of the pulse on inspiration and expiration or after the administration of 1 mg of atropine, assessment of voiding and erectile function (in males), etc. Such tests are generally used only in selected patients to answer specific questions. All patients, however, should be asked about possible disturbances of autonomic function when the history is taken (urination, defecation, sexual function, sweating).
Neurologically Relevant Aspects of the General Physical Examination
Many internal illnesses have neurological symptoms, sometimes as the main or sole manifestation of disease. The clinician performing a neurological examination should pay special attention to any potential symptoms or signs of a general, not exclusively neurological condition.
The patient’s general appearance may suggest a wasting illness, such as a malignant neoplasm, or an endocrinopathy. Abnormal pallor of the skin may be a sign of anemia and a straw-yellow coloration may indicate pernicious anemia due to vitamin B12 deficiency. The
skin should also be carefully inspected for evidence of neurocutaneous diseases, vasculitic processes, or collagen vascular disease, which, taken together, are not at all uncommon. Findings to look for include the café-au- lait spots of neurofibromatosis (von Recklinghausen disease), abnormal shape and quality of the nails, herpetic vesicles, etc. The cardiovascular examination is very important: the blood pressure must be measured, pulses felt in the upper and lower limbs, and vascular bruits listened for, particularly in the neck, the supraclavicular fossae, the abdomen, and the groin bilaterally. The patient should also be examined for possible organomegaly or lymphadenopathy.
3
The Neurological Examination
Neuropsychological and Psychiatric Examination
Psychopathological Findings
Many neurological illnesses are associated with psychiatric disturbances of greater or lesser severity. The organic neurological clinical picture is only complete once any psychopathological abnormalities that may be present have been thoroughly assessed and documented.
The examiner should first determine whether the patient is awake and alert. If not, he or she will be unable to receive and process incoming stimuli in the normal way. The patient may have a disturbance of consciousness of varying degrees of severity, ranging from drowsiness to coma, as described in Table 3.9.
In addition to the patient’s level of consciousness and attention, the examiner should assess his or her orientation, concentration, memory, drive, affective state, and cognitive ability. The overall psychopathological picture is composed of these elements. If mental functioning is disturbed by an underlying neurological illness (socalled psycho-organic syndrome or organic brain syn-
ARgo
drome), the manifestations often progress in a characteristic sequence, regardless of the etiology. At first, short-term and long-term memory, concentration, and attention are impaired; the patient is easily fatigued and has difficulty processing new information or performing complex tasks. Later, the patient becomes progressively disoriented, first to time, then to place, and then to person. Reactive depression is common at this stage. Ultimately, all spontaneous activity ceases; the patient loses interest, lacks drive, and in the end becomes permanently confused. Disturbances of this type can often be discerned in the patient’s behavior, growing increasingly evident to the examiner over the course of the clinical interview and physical examination. Further historical data from the patient’s family are often helpful. The MiniMental Status Test (Table 3.10) is widely used to assess cognitive function. For congenital psy- cho-organic abnormalities (mental retardation) and acquired forms (dementia), see p. 137.
ARgo leicht argo
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All rights reserved. Usage subject to terms and conditions of license.
40 |
3 The Neurological Examination |
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Table 3.9 Degrees of impairment of consciousness, and other abnormal states of consciousness |
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Designation |
Features |
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Normal consciousness |
oriented to place, time, and person (self), answers questions promptly and appropriately, follows com- |
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mands correctly |
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Drowsiness |
mostly awake, responds to questions and commands slowly but usually correctly (after repetition if nec- |
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essary), moves in response to a sufficiently intense stimulus, usually oriented and coherent |
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Somnolence |
mostly asleep, arousable with a moderately intense stimulus, generally requires repetition of questions |
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or commands but then responds correctly, reacts slowly and after a delay but usually correctly |
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Stupor |
asleep unless awakened, can only be awakened with a strong (auditory) stimulus or perhaps only with a |
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mechanical stimulus, cannot answer questions or follow commands or does so only after intense repeti- |
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tion, and then only incompletely |
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Coma |
unconscious, cannot be awakened, does not respond to a verbal or auditory stimulus, may respond to |
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painful stimuli of graded intensities with specific (localizing) self-defense, nonlocalizing withdrawal of a |
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limb, or abnormal flexion or extension responses, depending on the grade of coma (see also Table 2.54) |
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Confusion |
inappropriate spontaneous behavior and responses to questions and commands, deficient orientation |
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to place, time, and/or person (self); the confused patient may be fully conscious, less than fully con- |
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scious, or agitated (see below) |
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Agitation |
motor unrest, inappropriate spontaneous behavior, cannot be quieted by verbal persuasion, more or |
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less disoriented, does not follow commands appropriately |
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Table 3.10 MiniMental Status Test (after Folstein et al.)
Name of patient: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Date of birth: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Date of examination: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
1 point for each correct answer
Orientation in time
1.“What day of the week is it?”
2.“What is today’s date?”
3.“What is the current month?”
4.“What is the current season?”
5.“What year is it?”
Orientation to place
6.“Where are we (hospital, old age home, etc.)?”
7.“On what floor?”
8.“In what city?”
9.“In what state (province, canton, etc.)?”
10.“In what country?”
Retentiveness
“Please repeat the following words.”
(To be spoken at one word per second; to be performed only once)
11.“Lemon,
12.Key,
13.Ball.”
Attention and calculations
14.“Please count from 100 backward by sevens” (serial-7 test)
15.one point for each correct subtraction,
16.maximum five points
17. . . . . . .
18. . . . . . .
Recent memory
19.“Which three words
20.did you repeat earlier?”
21.one point for each word correctly recalled
. . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . .
. . . . . . . . . . . . . . . . . . . . . . . . . .
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Continued
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Neuropsychological and Psychiatric Examination 41
Table 3.10 MiniMental Status Test (after Folstein et al.) (continued)
Language, naming |
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22. |
“What is this?” (show a pencil) |
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23. |
“What is this?” (show a watch) |
. . . . . . . . . . . . . . . . . . . . . . . . . . |
24. |
“Please say after me: ‘No ifs, ands, or buts.’ ” |
. . . . . . . . . . . . . . . . . . . . . . . . . . |
Language comprehension, motor execution |
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25. |
“Take this piece of paper in your hand, |
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26. |
fold it down the middle, |
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27. |
and put it on the ground.” (each command to be given only once) |
. . . . . . . . . . . . . . . . . . . . . . . . . . |
Reading |
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28. |
“Please do what it says on this card.” (Show card—“Close your eyes”) |
. . . . . . . . . . . . . . . . . . . . . . . . . . |
Writing |
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29. |
“Write any sentence.” (the patient is given a piece of paper and something to write with) |
. . . . . . . . . . . . . . . . . . . . . . . . . . |
Drawing |
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30. |
“Please copy this drawing.” |
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(all 10 edges of the two pentagons must be drawn, |
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and the pentagons must overlap, for the patient |
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to receive one point for this task) |
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Level of wakefulness: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Total points achieved: . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Neuropsychological Examination
The neuropsychological examination is designed to detect cognitive deficits (particularly in the areas of language, recognition, and the performance of motor tasks) and disorders of perceptual processing that may be due to a focal cortical lesion.
The localizing significance of various neuropsychological deficits is illustrated in the diagram of Fig. 3.32.
Aphasia. Language disorders due to cortical disfunction are called aphasia and are generally caused by left-sided lesions. A basic distinction is drawn between abnormalities of language production (motor aphasia or Broca aphasia) and of language comprehension (sensory aphasia or Wernicke aphasia). In Broca aphasia, there is a paucity of spontaneous speech, even though the “organic apparatus” for speech production (phonation, respiration, vocal muscles) remains intact. In Wernicke aphasia, speech comprehension is impaired despite intact hearing and processing of nonlinguistic auditory signals.
Fig. 3.32 Cognitive deficits that typically result from focal brain lesions (diagram adapted from A. Schnider).
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Distractibility, perseveration |
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L |
Reduced drive |
Hyperverbalization |
R |
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Aphasia, |
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Aprosodia |
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agraphia, |
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alexia, |
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apraxia |
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Hemispatial neglect |
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Acalculia |
Amnesia |
Visuoconstructive |
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Finger agnosia, |
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deficit |
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right–left |
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confusion |
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Topographagnosia |
Object agnosia, |
Prosopagnosia |
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color anomia |
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ARgo |
ARgo leicht |
argo |
Mumenthaler / Mattle, Fundamentals of Neurology © 2006 Thiemealöb auch Argo
All rights reserved. Usage subject to terms and conditions of license.
3
The Neurological Examination
42 3 The Neurological Examination
The examiner begins to assess the patient’s spontaneous speech while taking the history; if necessary, the patient can be given specific language tasks, e. g., “Describe this picture.” Various kinds of abnormality may be noted. The patient’s utterances may be found to be unusually poor in meaning-bearing words and overloaded with connectives and “function words.” Sentences may be faultily constructed (paragrammatism). The flow of speech may be either considerably greater than normal or slow and hesitant (telegraphic speech). Individual words may be deformed in certain characteristic ways (e. g., sound substitutions or phonematic paraphasias, such as “cog” for “dog”), or words may be used in place of other words from the same semantic category (semantic paraphasias, e. g., “table” for “chair”). Some words may be replaced by invented pseudowords (neologisms). Impaired language comprehension may be manifested by the patient’s inability to point out various objects in the room, including parts of his or her own body, when these are named by the examiner. Complex commands are an even more sensitive functional test. The patient can be asked, for example, to place a certain named object in between two other named objects, or to interpret a complicated sentence, such as the following: “Not in the closet, but on top of it, was where he had placed his hat. Where was the hat?” Aphasic patients often make errors in the repetition of spoken sentences and in the naming of objects or parts of the body that are shown to them. Reading and writing may also be impaired.
Disturbances of spatial processing are usually caused by right-hemispheric lesions. Evidence for such a disturbance is present if the patient has unusual difficulty in spontaneously drawing or copying three-dimensional figures (cube, house, etc.). Deficits of this kind are often accompanied by neglect of the left side of space and the left half of the patient’s own body (hemispatial neglect).
Apraxia. Disturbances in the goal-directed execution of complex actions or sequences of actions are known as apraxia. If the individual components of a single action cannot be put together correctly, the patient is suffering from ideomotor apraxia. Different parts of the body can be affected individually. In facial apraxia, for example, the patient may be unable to follow a command to execute certain motor tasks with the face, e. g., drinking through a straw or clicking the tongue. A patient with ideomotor apraxia of the upper limbs may be unable to salute or to mime the action of slapping someone in the face; a patient with ideomotor apraxia of the lower
limbs may be unable to kick an imaginary football. In ideational apraxia, individual actions can be performed, but cannot be combined into more complex sequences. A patient might thus be unable to ready a letter for mailing, as this requires several steps: folding the letter, putting it in the envelope, sealing the envelope, and putting a stamp on it. Cortical lesions causing apraxia are usually on the left side.
Agnosia is an inability to recognize and correctly interpret incoming stimuli in a particular sensory modality even though sensation as such is intact. A patient with visual agnosia, for example, has no visual impairment but cannot recognize objects on sight; the patient can name an object only after feeling or hearing it (e. g., the jangling of a bunch of keys).
Special types of agnosia include an inability to recognize colors (color agnosia) or faces (prosopagnosia). The responsible lesion is in the visual association cortex, i. e., in the occipital or occipitotemporal region, in one or both hemispheres. Stereognosis is tested by putting a familiar object (key, pair of scissors) in the patient’s hand and asking him or her to palpate it and name it (with eyes closed). An inability to do this despite intact sensation is called tactile agnosia. Further special types of agnosia are finger agnosia and autotopagnosia (difficulty recognizing parts of one’s own body). Anosognosia is the denial or trivialization of one’s own neurological deficits, e. g., hemiplegia or even blindness.
Higher cognitive functions. More than just the basic neuropsychological functions described above must be intact so that the individual can thrive in his or her social environment and cope adequately with the demands of everyday life. A person’s fund of knowledge, memory, intelligence (by which we mean a capacity for abstract thought and problem solving), personality, and social behavior are all of vital importance, as are his or her mood and motivation. The assessment of these higher cognitive functions requires a careful weighing of historical information (particularly from persons in the patient’s social environment: family, friends, colleagues), in addition to certain standardized neuropsychological tests. There are specific tests for the patient’s fund of knowledge, logical thinking, and cognitive skills such as the recognition of differences, the formation of categories, and the interpretation of symbolic information, e. g., proverbs. These so-called higher “integrative” functions depend not only on an intact cerebral cortex, but also on other, deeper regions of the brain.
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