Verbal auditory agnosia in children. 1977

I Rapin, and S Mattis, and A J Rowan, and G G Golden

Four (possibly five) boys are described with a profound comprehension deficit for acoustic language, leading to severe or complete abolition of expressive speech. One boy had presumed megalencephaly from birth but was of superior intelligence. He had a severe articulation deficit from early childhood, with delayed acquisition of speech. Another boy is thought to have a small angiomatous anomaly in the depth of the left parietal lobe. No brain lesions are known in the other three. Evidence for bilateral brain dysfunction consists of minor motor abnormalities in three boys, oromotor deficits in two boys, and bilaterally synchronous diffuse or independent focal paroxysmal discharges in the EEG of three, possibly four, of the boys. Seizures have occurred in only three boys, and have been easily controlled with anticonvulsants. One boy with a grossly abnormal EEG has had no clinical seizures to date and has not benefited frome one year of anticonvulsant therapy. Two of the boys are brothers, including the boy in whome the diagnosis is questionable since speech was never normal and since he has had neither seizures for an abnormal EEG. The severity of EEG abnormalities did not correlate closely with the course of the language deficit. The relationship of this syndrome to acquired aphasia in children, to Wernicke's aphasia and pure word deafness in adults, and to developmental lagnuage disability with predominantly receptive deficits, is discussed. One child illustrated the close association between writing and phonologic encoding and decoding operations, and two children the preservation of linguistic skills provided the acoustic channel was by-passed and language presented visually. This latter point has been emphasized because of its implications for the remedial education of children with this syndrome.

UI MeSH Term Description Entries
D007223 Infant A child between 1 and 23 months of age. Infants
D008297 Male Males
D002648 Child A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL. Children
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D004569 Electroencephalography Recording of electric currents developed in the brain by means of electrodes applied to the scalp, to the surface of the brain, or placed within the substance of the brain. EEG,Electroencephalogram,Electroencephalograms
D004827 Epilepsy A disorder characterized by recurrent episodes of paroxysmal brain dysfunction due to a sudden, disorderly, and excessive neuronal discharge. Epilepsy classification systems are generally based upon: (1) clinical features of the seizure episodes (e.g., motor seizure), (2) etiology (e.g., post-traumatic), (3) anatomic site of seizure origin (e.g., frontal lobe seizure), (4) tendency to spread to other structures in the brain, and (5) temporal patterns (e.g., nocturnal epilepsy). (From Adams et al., Principles of Neurology, 6th ed, p313) Aura,Awakening Epilepsy,Seizure Disorder,Epilepsy, Cryptogenic,Auras,Cryptogenic Epilepsies,Cryptogenic Epilepsy,Epilepsies,Epilepsies, Cryptogenic,Epilepsy, Awakening,Seizure Disorders
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000377 Agnosia Loss of the ability to comprehend the meaning or recognize the importance of various forms of stimulation that cannot be attributed to impairment of a primary sensory modality. Tactile agnosia is characterized by an inability to perceive the shape and nature of an object by touch alone, despite unimpaired sensation to light touch, position, and other primary sensory modalities. Auditory Agnosia,Finger Agnosia,Sensory Agnosia,Tactile Agnosia,Visual Agnosia,Agnosia for Pain,Agnosia for Smell,Agnosia for Taste,Agnosia for Temperature,Anosognosia,Auditory Agnosia, Congenital,Body-Image Agnosia,Congenital Auditory Agnosia,Developmental Agnosia,Gustatory Agnosia,Ideational Agnosia,Olfactory Agnosia,Position Agnosia,Somatosensory Agnosia,Time Agnosia,Topographical Agnosia,Visual Agnosia for Objects,Visual Disorientation Syndrome,Visuospatial Agnosia,Agnosia for Tastes,Agnosia, Auditory,Agnosia, Body-Image,Agnosia, Congenital Auditory,Agnosia, Developmental,Agnosia, Finger,Agnosia, Gustatory,Agnosia, Ideational,Agnosia, Olfactory,Agnosia, Position,Agnosia, Sensory,Agnosia, Somatosensory,Agnosia, Tactile,Agnosia, Time,Agnosia, Topographical,Agnosia, Visual,Agnosia, Visuospatial,Agnosias,Agnosias, Auditory,Agnosias, Body-Image,Agnosias, Congenital Auditory,Agnosias, Developmental,Agnosias, Finger,Agnosias, Ideational,Agnosias, Olfactory,Agnosias, Position,Agnosias, Sensory,Agnosias, Somatosensory,Agnosias, Tactile,Agnosias, Time,Agnosias, Topographical,Agnosias, Visual,Agnosias, Visuospatial,Anosognosias,Auditory Agnosias,Auditory Agnosias, Congenital,Body Image Agnosia,Body-Image Agnosias,Congenital Auditory Agnosias,Developmental Agnosias,Finger Agnosias,Ideational Agnosias,Olfactory Agnosias,Position Agnosias,Sensory Agnosias,Somatosensory Agnosias,Syndrome, Visual Disorientation,Syndromes, Visual Disorientation,Tactile Agnosias,Time Agnosias,Topographical Agnosias,Visual Agnosias,Visual Disorientation Syndromes,Visuospatial Agnosias
D001037 Aphasia A cognitive disorder marked by an impaired ability to comprehend or express language in its written or spoken form. This condition is caused by diseases which affect the language areas of the dominant hemisphere. Clinical features are used to classify the various subtypes of this condition. General categories include receptive, expressive, and mixed forms of aphasia. Aphasia, Acquired,Dysphasia,Word Deafness,Alogia,Anepia,Aphasia, Ageusic,Aphasia, Auditory Discriminatory,Aphasia, Commisural,Aphasia, Functional,Aphasia, Global,Aphasia, Graphomotor,Aphasia, Intellectual,Aphasia, Mixed,Aphasia, Post-Ictal,Aphasia, Post-Traumatic,Aphasia, Progressive,Aphasia, Semantic,Aphasia, Syntactical,Dejerine-Lichtheim Phenomenon,Dysphasia, Global,Lichtheim's Sign,Logagnosia,Logamnesia,Logasthenia,Acquired Aphasia,Ageusic Aphasia,Ageusic Aphasias,Alogias,Anepias,Aphasia, Post Ictal,Aphasia, Post Traumatic,Aphasias, Commisural,Auditory Discriminatory Aphasia,Auditory Discriminatory Aphasias,Commisural Aphasia,Commisural Aphasias,Deafness, Word,Dejerine Lichtheim Phenomenon,Discriminatory Aphasia, Auditory,Discriminatory Aphasias, Auditory,Functional Aphasia,Functional Aphasias,Global Aphasia,Global Aphasias,Global Dysphasia,Global Dysphasias,Graphomotor Aphasia,Graphomotor Aphasias,Intellectual Aphasia,Intellectual Aphasias,Lichtheim Sign,Lichtheims Sign,Logagnosias,Logamnesias,Logasthenias,Mixed Aphasia,Mixed Aphasias,Phenomenon, Dejerine-Lichtheim,Post-Ictal Aphasia,Post-Ictal Aphasias,Post-Traumatic Aphasia,Post-Traumatic Aphasias,Progressive Aphasia,Progressive Aphasias,Semantic Aphasia,Semantic Aphasias,Sign, Lichtheim's,Syntactical Aphasia,Syntactical Aphasias
D001307 Auditory Perception The process whereby auditory stimuli are selected, organized, and interpreted by the organism. Auditory Processing,Perception, Auditory,Processing, Auditory

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