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GLOSSARY OF
DESCRIPTIVE TERMINOLOGY FOR ICTAL SEMIOLOGY
Warren T. Blume, MD, FRCPC - Chair
Hans O. Lüders
Eli Mizrahi
Carlo Tassinari
Walter van Emde Boas
Jerome Engel, Jr., - Ex-officio
TABLE OF CONTENTS
INTRODUCTION
PRINCIPLES FOR TERMS AND DEFINITIONS
DATA SOURCES
I. GENERAL TERMS
1.0 SEMEIOLOGY
(SEMIOLOGY)
2.0 EPILEPTIC
SEIZURE
3.0 ICTUS
4.0 EPILEPSY
5.0 FOCAL
6.0 GENERALISED
7.0 CONVULSION
II. TERMS DESCRIBING EPILEPTIC SEIZURE SEMEIOLOGY
1.0 MOTOR
1.1 ELEMENTARY
MOTOR
1.1.1 TONIC
1.1.1.1
EPILEPTIC SPASM
1.1.1.2
POSTURAL
1.1.1.2.1
VERSIVE
1.1.1.2.2
DYSTONIC
1.1.2 MYOCLONIC
1.1.2.1
NEGATIVE MYOCLONIC
1.1.2.2
CLONIC
1.1.2.2.1
JACKSONIAN MARCH
1.1.3 TONIC-CLONIC
1.1.3.1
GENERALISED TONIC-CLONIC SEIZURE
1.1.4 ATONIC
1.1.5 ASTATIC
1.1.6 SYNCHRONOUS
1.2 AUTOMATISM
1.2.1 OROALIMENTARY
1.2.2 MIMETIC
1.2.3 MANUAL
OR PEDAL
1.2.4 GESTURAL
1.2.5 HYPERKINETIC
1.2.6 HYPOKINETIC
1.2.7 DYSPHASIC
1.2.8 DYSPRAXIC
1.2.9 GELASTIC
1.2.10
DACRYSTIC
1.2.11
VOCAL
1.2.12
VERBAL
1.2.13
SPONTANEOUS
1.2.14
INTERACTIVE
2.0 NON-MOTOR
2.1 AURA
2.2 SENSORY
2.2.1 ELEMENTARY
2.2.1.1
SOMATOSENSORY
2.2.1.2
VISUAL
2.23.1.3
AUDITORY
2.2.1.4
OLFACTORY
2.2.1.5
GUSTATORY
2.2.1.6
EPIGASTRIC
2.2.1.7
CEPHALIC
2.2.1.8
AUTONOMIC
2.2.2 EXPERIENTIAL
2.2.2.1
AFFECTIVE
2.2.2.2
MNEMONIC
2.3.2.3
HALLUCINATORY
2.2.2.4
ILLUSORY
2.3 DYSCOGNITIVE
3.0 AUTONOMIC
EVENTS
3.1 AUTONOMIC
AURA
3.2 AUTONOMIC
SEIZURE
4.0 SOMATOTOPIC
MODIFIERS
4.1 LATERALITY
4.1.1 UNILATERAL
4.1.1.1
HEMI-
4.1.2 GENERALISED
(syn. "bilateral")
4.1.2.1
ASYMMETRICAL
4.1.2.2
SYMMETRICAL
4.2 BODY PART
4.3 CENTRICITY
4.3.1 AXIAL
4.3.2 PROXIMAL
LIMB
4.3.3 DISTAL
LIMB
5.0 MODIFIERS
AND DESCRIPTORS OF SEIZURE TIMING
5.1 INCIDENCE
5.1.1 REGULAR,
IRREGULAR
5.1.2 CLUSTER
5.1.3 PROVOCATIVE
FACTOR
5.1.3.1
REACTIVE
5.1.3.2
REFLEX
5.2 STATE DEPENDENT
5.3 CATAMENIAL
6.0 DURATION
6.1 STATUS
EPILEPTICUS
7.0 SEVERITY
8.0 PRODROME
9.0 POSTICTAL
PHENOMENON
9.1 LATERALISING
(TODD'S (OR BRAVAIS')) PHENOMENON
9.2 NON-LATERALISING
PHENOMENA
9.2.1 IMPAIRED
COGNITION
9.2.2 ANTEROGRADE
AMNESIA
9.2.3 RETROGRADE
AMNESIA
9.2.4 PSYCHOSIS
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INTRODUCTION
This glossary intends to provide a standard terminology
for health care workers to communicate what is observed and what a patient
reports during a seizure. As this terminology is descriptive and phenomenological,
its use would not imply or require knowledge of ictal pathophysiology,
any pathological substrate or etiology.
Many terms are adjectives modifying "seizure" which itself
is defined under "general terms". This pertains to seizures with single
or multiple components.
Terms in this glossary, e.g. "seizure", "ictus", which
have widespread applicability in other fields of clinical neuroscience,
are herein defined according to their references to epilepsy.
Some terms of this glossary are "fundamental" i.e. they
encompass other more precise words. These can be used as the sole descriptor
when data to more precisely characterise a phenomenon are not available.
Such include aura, automatism, experiential, motor and sensory.
A seizure will often consist of two or more phenomena
occurring simultaneously or sequentially and would be described accordingly.
Quantitative terms, such as duration of motor events,
are not intended as immutable confines, but as clarifying guides to describe
clinically observed events.
Scientific progress dictates an evolution of terms to
retain their relevance. However, needs of communication in everyday life
require that changes be gradual and evolutionary than abrupt and revolutionary.
The use of synonyms in this glossary reflects incidences where gradual
changes are likely.
Terminology in some areas remains unresolved. Therefore,
we view this glossary as a dynamic process for which feedback will be
welcomed.
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PRINCIPLES FOR TERMS AND DEFINITIONS
In developing the "lexique" of this report we adopted and
applied the following principles.
Terms and definitions should:
- Contain features which distinguish or modify seizure entities.
- Be descriptive of the phenomena involved.
- Comply with terminology of clinical neuroscience.
- Employ current terminology and definitions wherever possible.
- Contain new terms only if necessary.
- Be easily translatable to other languages.
- Be readily understood and employed by potential users.
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DATA SOURCES
Adams RD, Victor M. Principles of Neurology. McGraw Hill
Book, New York, 1993, 5th edition.
Aicardi, J. Epilepsy in Children. International Review
of Child Neurology Series. Raven Press, New York, 1986, 1994.
Benson, D.F. The Neurology of Thinking. Oxford University
Press, New York, 1994, pp 224-225.
Blume, WT, Berkovic S, Dulac O. (1997) Search for a better
classification of the epilepsies. In: Epilepsy: A Comprehensive Textbook,
Vol. 1. Edited by J. Engel Jr. and T.A. Pedley, Lippincott-Raven Publishers,
Philadelphia, Chapter 69, pp 779-789.
Delgado-Escueta, A.V., Serratosa, J.M., Medina, M.T.
Myoclonic seizures, and progressive myoclonus epilepsy syndromes. In:
The Treatment of Epilepsy: Principles and Practice, 2nd ed. Edited by
E. Wyllie, Williams & Wilkins, Baltimore, 1996, pp 467-483.
Engel, J.Jr. Seizures and Epilepsy. F.A. Davis Company,
Philadelphia, 1989.
Engel, J.Jr. and Pedley, T.A. Epilepsy: A Comprehensive
Textbook, Volumes 1-3, Lippincott-Raven Publishers, Philadelphia, 1997.
Gastaut, H. and Broughton, R. Epileptic Seizures. Clinical
and Electrographic Features, Diagnosis and Treatment. Charles C. Thomas,
Springfield, Ill, 1972.
Gloor, P. Consciousness as a neurological concept in
epileptology: a critical review. Epilepsia 1986;27 (Suppl 2):S14-S26.
Gloor, P. The Temporal Lobe and Limbic System. Oxford
University Press, 1997.
Hopkins AP, Michael WF. Spinal myoclonus. J Neurol Neurosurg
Psychiatry 1974;37:1112-1115.
Luders H, et al. Semiological seizure classification.
Epilepsia 1998;39:1006-1013.
Moscovitch, M. Information processing and the cerebral
hemispheres. In: Gazzaniga MS, ed. Handbook of Behavioral Neurobiology:
Neuropsychology. Vol 2. New York, NY: Plenum Publishing Corp; 1979:379-446.
Penfield, W. and Jasper, H.H. Epilepsy and Functional
Anatomy of the Human Brain. Little, Brown and Company, 1954.
Pryse-Phillips, W. Companion to Clinical Neurology, Little,
Brown and Company, 1995.
Rowland, L.P. Merritt's Textbook of Neurology, 8th Edition.
Lea & Febiger, Philadelphia, 1989.
So, N. Epileptic auras. In: The Treatment of Epilepsy:
Principles and Practice, 2nd ed. Edited by E. Wyllie, Williams & Wilkins,
Baltimore, 1996, pp 376-384.
Wolf, P. Epileptic Seizures and Syndromes. John Libbey
& Company Ltd., 1994.
Young, G.B. Coma and Impaired Consciousness: A Clinical
Perspective. McGraw-Hill, New York, 1998.
Young, G.B. and Pigott, S.E. Neurobiological basis of
consciousness. Archives of Neurology 1999;56:153-157.
Some non-medical texts:
The Concise Oxford Dictionary. 7th edition. Edited by
J.B. Sykes. Clarendon Press; Oxford, England, 1982.
Dictionnaire Encyclopedique. Petit Larousse Illustre.
Librairie Larousse, Paris, 1973.
Dictionary of Canadian English. The Senior Dictionary.
Edited by W.S. Avis, P.D. Drysdale, R.J. Gregg, M.H. Scargill. W.J. Gage
Limited; Toronto, Canada, 1967.
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I. GENERAL TERMS
1.0 SEMEIOLOGY (SEMIOLOGY)
That branch of linguistics concerned with signs and symptoms.
2.0 EPILEPTIC SEIZURE
Manifestation(s) of epileptic (excessive and/or hypersynchronous), usually
self-limited activity of neurons in the brain.
3.0 ICTUS
A sudden neurological occurrence such as a stroke or an epileptic seizure.
4.0 EPILEPSY
a) Epileptic Disorder: A chronic neurological condition characterised
by recurrent epileptic seizures.
b) Epilepsies: Those conditions involving chronic recurrent epileptic
seizures that can be considered epileptic disorders.
5.0 FOCAL (syn. partial)
A seizure whose initial semiology indicates, or is consistent with, initial
activation of only part of one cerebral hemisphere.
6.0 GENERALISED (syn. bilateral)
A seizure whose initial semiology indicates, or is consistent with, more
than minimal involvement of both cerebral hemispheres.
7.0 CONVULSION
Primarily a lay term. Episodes of excessive, abnormal muscle contractions,
usually bilateral, which may be sustained or interrupted.
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II. TERMS DESCRIBING EPILEPTIC SEIZURE SEMEIOLOGY
These are descriptors of seizures unless specified otherwise.
1.0 MOTOR
Involves musculature in any form. The motor event could consist of an
increase (positive) or decrease (negative) in muscle contraction to produce
a movement.
Unless noted, the following terms are adjectives modifying
"motor seizure" or "seizure" e.g. "tonic motor seizure or dystonic seizure",
and whose definitions can usually be understood as prefaced by: "refers
to ...".
1.1 ELEMENTARY MOTOR
A single type of contraction of a muscle or group of muscles that is usually
stereotyped and not decomposable into phases. (However, see Tonic-Clonic,
an elementary motor sequence).
1.1.1 TONIC
A sustained increase in muscle contraction lasting a few seconds to minutes.
1.1.1.1 EPILEPTIC SPASM (Formerly
Infantile Spasm)
Noun: A sudden flexion, extension or mixed extension-flexion of predominantly
proximal and truncal muscles which is usually more sustained than a myoclonic
movement but not as sustained as a tonic seizure i.e. about 1 sec. Limited
forms may occur: grimacing, head nodding.. Epileptic spasms frequently
occur in clusters.
1.1.1.2 POSTURAL
Adoption of a posture which may be bilaterally symmetrical or asymmetrical
(as in a "fencing posture").
1.1.1.2.1 VERSIVE
A sustained, forced conjugate ocular, cephalic and/or truncal rotation
or lateral deviation from the midline.
1.1.1.2.2 DYSTONIC
Sustained contractions of both agonist and antagonist muscles producing
athetoid or twisting movements which when prolonged may produce abnormal
postures.
1.1.2 MYOCLONIC (adjective);
MYOCLONUS (noun)
Sudden, brief (< 100 msec) involuntary single or multiple contraction(s)
of muscles(s) or muscle groups of variable topography (axial, proximal
limb, distal).
1.1.2.1 NEGATIVE MYOCLONIC
Interruption of tonic muscular activity for < 500 msec without evidence
of antecedent myoclonia.
1.1.2.2 CLONIC
Myoclonus which is regularly repetitive, involves the same muscle groups,
at a frequency of about 2-3 c/sec, and is prolonged. Synonym: rhythmic
myoclonus.
1.1.2.2.1 JACKSONIAN MARCH
Noun: Traditional term indicating spread of clonic movements through contiguous
body parts unilaterally.
1.1.3 TONIC-CLONIC
A sequence consisting of a tonic followed by a clonic phase. Variants
such as clonic-tonic-clonic may be seen.
1.1.3.1 GENERALISED TONIC-CLONIC
SEIZURE (syn. bilateral tonic-clonic seizure (Formerly "Grand Mal"
Seizure))
Noun: Bilateral symmetrical tonic contraction then bilateral clonic contractions
of somatic muscles usually associated with autonomic phenomena.
1.1.4 ATONIC
Sudden loss or diminution of muscle tone without apparent preceding myoclonic
or tonic event lasting one to two seconds or more, involving head, trunk,
jaw or limb musculature.
1.1.5 ASTATIC
Loss of erect posture that results from an atonic, myoclonic or tonic
mechanism. Synonym: drop attack.
1.1.6 SYNCHRONOUS (Asynchronous)
Motor events occurring (not) at the same time or at the same rate in sets
of body parts.
1.2 AUTOMATISM
Noun: A more or less coordinated, repetitive, motor activity usually occurring
when cognition is impaired and for which the subject is usually amnesic
afterwards. This often resembles a voluntary movement, and may consist
of inappropriate continuation of ongoing preictal motor activity.
The following adjectives are usually employed to modify
"automatism".
1.2.1 OROALIMENTARY
Lip smacking, lip pursing, chewing, licking, tooth grinding or swallowing.
1.2.2 MIMETIC
Facial expression suggesting an emotional state, often fear.
1.2.3 MANUAL OR PEDAL
- Indicates principally distal components, bilateral or unilateral.
- Fumbling, tapping, manipulating movements.
1.2.4 GESTURAL
Often unilateral.
- Fumbling or exploratory movements with the hand directed toward self
or environment.
- Movements resembling those intended to lend further emotional tone
to speech.
1.2.5 HYPERKINETIC
- Involves predominantly proximal limb or axial muscles producing irregular
sequential ballistic movements, such as pedalling, pelvic thrashing,
rocking movements.
- Increase in rate of ongoing movements or inappropriately rapid performance
of a movement.
1.2.6 HYPOKINETIC
A decrease in amplitude and/or rate or arrest of ongoing motor activity.
1.2.7 DYSPHASIC
Impaired communication involving language without dysfunction of relevant
primary motor or sensory pathways, manifested as impaired comprehension,
anomia, paraphasic errors or a combination of these.
1.2.8 DYSPRAXIC
Inability to perform learned movements spontaneously or on command or
imitation despite intact relevant motor and sensory systems and adequate
comprehension and cooperation.
1.2.9 GELASTIC
Bursts of laughter or giggling, usually without an appropriate affective
tone.
1.2.10 DACRYSTIC
Bursts of crying.
1.2.11 VOCAL
Single or repetitive utterances consisting of sounds such as grunts or
shrieks.
1.2.12 VERBAL
Single or repetitive utterances consisting of words, phrases or brief
sentences.
1.2.13 SPONTANEOUS
Stereotyped, involve only self, and are virtually independent of environmental
influences.
1.2.14 INTERACTIVE
Not stereotyped, involve more than self, and are environmentally influenced.
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2.0 NON-MOTOR
2.1 AURA
Noun: A subjective ictal phenomenon that, in a given patient, may precede
an observable seizure; if alone, constitutes a sensory seizure.
2.2 SENSORY
A perceptual experience not caused by appropriate stimuli in the external
world. Modifies "seizure or "aura".
2.2.1 ELEMENTARY
A single, unformed phenomenon involving one primary sensory modality,
e.g. somatosensory, visual, auditory, olfactory, gustatory, epigastric,
or cephalic.
2.2.1.1 SOMATOSENSORY
Tingling, numbness, electric shock sensation, pain, sense of movement,
or desire to move.
2.2.1.2 VISUAL
Flashing or flickering lights, spots, simple patterns, scotomata, or amaurosis.
2.2.1.3 AUDITORY
Buzzing, drumming sounds or single tones.
2.2.1.4 OLFACTORY
Odour, usually disagreeable.
2.2.1.5 GUSTATORY
Taste sensations including acidic, bitter, salty , sweet or metalic.
2.2.1.6 EPIGASTRIC
Abdominal discomfort including nausea, emptiness, tightness, churning,
butterflies, malaise, pain, and hunger; sensation may rise to chest or
throat. Some phenomena may reflect ictal autonomic dysfunction.
2.2.1.7 CEPHALIC
Sensation in the head such as light headedness, tingling or headache.
2.2.1.8 AUTONOMIC
A sensation consistent with involvement of the autonomic nervous system,
including cardiovascular, gastrointestinal, sudomotor, vasomotor and thermoregulatory
functions. (Thus, "autonomic aura"; cf. "autonomic seizure" 3.0).
2.2.2 EXPERIENTIAL
Affective, mnemonic or composite perceptual phenomena including illusory
or composite hallucinatory events; these may appear alone or in combination.
Included are feelings of depersonalisation. These phenomena have subjective
qualities similar to those experienced in life but are recognised by the
subject as occurring outside of actual context.
2.2.2.1 AFFECTIVE
Components include: fear, depression, joy and (rarely) anger.
2.2.2.2 MNEMONIC
Components which reflect ictal dysmnesia such as: feelings as familiarity
(déjà-vu) and unfamiliarity (jamais-vu).
2.2.2.3 HALLUCINATORY
A creation of composite perceptions without corresponding external stimuli
involving visual, auditory, somatosensory, olfactory and/or gustatory
phenomena. Example: "hearing" and "seeing" people
talking.
2.2.2.4 ILLUSORY
An alteration of actual percepts involving the visual, auditory, somatosensory,
olfactory or gustatory systems.
2.3 DYSCOGNITIVE
The term describes events in which: 1) disturbance of cognition is the
prominent or most apparent feature, and 2a) two or more of the following
components are involved, or 2b) contributions of such components remain
undetermined. Otherwise, use more specific term e.g. mnemonic experimental
seziure or "hallucinatory experimental seizure."
Components of cognition:
- perception: symbolic conception of sensory information
- attention: appropriate selection of a principal perception or task
- emotion: appropriate affective significance of a perception
- memory: ability to store and retrieve percepts or concepts
- executive function: anticipation, selection, monitoring of consequences,
initiation of motor activity including praxis, speech
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3.0 AUTONOMIC EVENTS
3.1 AUTONOMIC AURA
A sensation consistent with involvement of the autonomic nervous system,
including cardiovascular, gastrointestinal, sudomotor, vasomotor and thermoregulatory
functions. (see 2.3.1.8).
3.2 AUTONOMIC SEIZURE
An objectively documented and distinct alteration of autonomic nervous
system function involving cardiovascular, pupillary, gastrointestinal,
sudomotor, vasomotor and thermoregularity functions.
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4.0 SOMATOTOPIC MODIFIERS
4.1 LATERALITY
4.1.1 UNILATERAL
Exclusive or virtually exclusive involvement of one side as a motor, sensory
or autonomic phenomenon.
4.1.1.1 HEMI-
A prefix to other descriptors e.g. hemiclonic.
4.1.2 GENERALISED (syn. "bilateral")
More than minimal involvement of each side as a motor, elementary sensory
or autonomic phenomenon.
Motor component: further modified as:
4.1.2.1 ASYMMETRICAL
Clear distinction in quantity and/or distribution of behaviour on the
two sides.
4.1.2.2 SYMMETRICAL
Virtual bilateral equality in these respects.
4.2 BODY PART
Refers to area involved i.e. arm, leg, face, trunk and other.
4.3 CENTRICITY
Modifier describes proximity to the body axis.
4.3.1 AXIAL
Involves trunk, including neck.
4.3.2 PROXIMAL LIMB
Signifies involvement from shoulders to wrist, hip to ankle.
4.3.3 DISTAL LIMB
Indicates involvement of fingers, hands, toes, and/or feet.
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5.0 MODIFIERS AND DESCRIPTORS OF SEIZURE
TIMING
The following terms are listed in the form (adjective, noun, verb) according
to principal usage; as adjective unless specified.
5.1 INCIDENCE
Noun: Refers to the number of epileptic seizures within a time period
or the number of seizure days per unit of time.
5.1.1 REGULAR, IRREGULAR
Consistent (inconsistent) or predictable (unpredictable, chaotic) intervals
between such events.
5.1.2 CLUSTER
1) Noun: Incidence of seizures within a given period (usually one or a
few days) which exceeds the average of incidence over a longer period
for the patient.
2) Verb: To vary in incidence as above.
5.1.3 PROVOCATIVE FACTOR
Noun: Transient and sporadic endogenous or exogenous element capable of
augmenting seizure incidence in persons with chronic epilepsy and evoking
seizures in susceptible non-epileptic individuals.
5.1.3.1 REACTIVE
Occurring in association with transient systemic perturbation such as
intercurrent illness, sleep loss or emotional stress.
5.1.3.2 REFLEX
Objectively and consistently demonstrated to be evoked by a specific afferent
stimulus or by activity of the patient. Afferent stimuli can be: elementary,
i.e. unstructured (light flashes, startle, a monotone) or elaborate i.e.
structured, (a symphony). Activity may be elementary, e.g. motor
(a movement); or elaborate, e.g. cognitive function (reading, chess
playing), or both (reading aloud).
5.2 STATE DEPENDENT
Occurring exclusively or primarily in the various stages of drowsiness,
sleep, or arousal.
5.3 CATAMENIAL
Seizures occurring principally or exclusively in any one phase of the
menstrual cycle.
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6.0 DURATION
Time between the beginning of initial seizure manifestations, such as
the aura, to the cessation of experienced or observed seizure activity.
Does not include non-specific seizure premonitions or postictal states.
6.1 STATUS EPILEPTICUS
A seizure which shows no clinical signs of arresting after a duration
encompassing the great majority of seizures of that type in most patients
or recurrent seizures without resumption of baseline central nervous system
function interictally.
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7.0 SEVERITY
A multicomponent assessment of a seizure by observers and the patient.
Components primarily of observer assessment include: duration,
extent of motor involvement, impairment of cognitive interaction with
environment intraictally, maximum number of seizures per unit of time.
Components primarily of patient assessment: extent of injury;
emotional, social and vocational consequences of the attack.
8.0 PRODROME
A preictal phenomenon. A subjective or objective clinical alteration,
e.g. ill-localised sensation or agitation, that heralds the onset of an
epileptic seizure but does not form part of it.
9.0 POSTICTAL PHENOMENON
A transient clinical abnormality of central nervous system function that
appears or becomes accentuated when clinical signs of the ictus have ended.
9.1 LATERALISING (TODD'S (OR BRAVAIS'))
PHENOMENON
Any unilateral postictal dysfunction relating to motor, speech, somatosensory
and/or integrative functions including visual, auditory or somatosensory
neglect phenomena.
9.2 NON-LATERALISING PHENOMENA
Impaired cognition, amnesia, psychosis.
9.2.1 IMPAIRED COGNITION
Decreased cognitive performance involving one or more of: perception,
attention, emotion, memory, execution, praxis, speech (cf Dyscognitive,
2.3).
9.2.2 ANTEROGRADE AMNESIA
Impaired ability to remember new material.
9.2.3 RETROGRADE AMNESIA
Impaired ability to recall previously remembered material.
9.2.4 PSYCHOSIS
Misinterpretation of external world in an awake, alert person; involves
thought disorder of emotion and socialisation.
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