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Language disorders: clinical classification and neurovascular substrate.

The most prevalent cause of focal brain disease is stroke, Neuroanatomic localization in aphasia--and by inference, the identification of brain regions associated with language functions--is largely determined by, and confounded with, left cerebral hemisphere vascular territories. Traditional nosology of the aphasias, alexias, and agraphias is not always logically coherent and ignores major neurolinguistic boundries, yet it retains the clinical utility of permitting reliable and valid anatomic inferences concerning the underlying neuropathologic substrate.

Aphasia↗

[Disconnection and recognition of faces. A case with lesions of the left visual cortex and the splenium].

We report the clinical findings in a case of alexia without agraphia, due to a left posterior cerebral artery infarction, in which C.T. showed occipital and splenial lesions. There were in addition a right homonymous hemianopsia and agnosia for colors, objects and pictures, with both associative and disconnective aspects. The hypothesis of an occipito-occipital disconnection, depriving the left visual associative areas of inputs, could explain this agnosia. The most striking findings in this case was, besides an anomia for faces, a peculiar difficulty to recognize her own face. The patient seemed to recognize herself. The patient spoke about herself in the third person, giving genuine autobiographical details, without being able to say: "it is me". This phenomenon is discussed in terms of disconnection: the right hemisphere sending information from the semantic field evoked by her own face, through the anterior part of the corpus callosum, to the left hemisphere. We discuss the hemispheric contribution within cognitive experience, and the role of language concerning the conscious experience in man.

Brain↗

[Associative visual agnosia--a case report].

A 61 year-old right-handed man, a president of a company fell downstairs and was found unconscious on January 1, 1985. He was admitted to a hospital and he had been unresponsive for 10 days. As he recovered from the consciousness disturbance, he complained of difficulties in discrimination of common objects and in recognition of familiar faces. The patient was admitted to our hospital for the closer examination on July 29, 1985. There was no abnormality in the general physical examination on admission. Neurological examinations revealed no significant deficits except visual field disturbance. In the results of WAIS, the verbal IQ was 108, but the performance IQ was unmeasurable because of a presence of object agnosia. His spontaneous speech was fluent without paraphasia, perseveration or dysprosody. His auditory comprehension of spoken language and repetition were spared. Neuropsychological examinations revealed an impairment in the recent verbal memory, alexia, agraphia, object agnosia, color naming difficulty, prosopagnosia and visuospatial constructional disability. The patient exhibited three major neuropsychological characteristics. Firstly, the recognition of the objects which were presented by the photograph or line drawing was more severely impaired than the real objects. Secondly, the object naming difficulty was more pronounced in the presentation at an unusual angle than in the free presentation. Thirdly, the patient made poor performance in reproduction of the models by photographic presentation as compared with the presentation of the real models in the three-dimensional block construction test. The CT scan performed on January 4, 1985 demonstrated subcortical hematomas with surrounding edema in the temporo-occipital regions of both hemispheres.(ABSTRACT TRUNCATED AT 250 WORDS)

Agnosia↗

MRI of callosal disconnection due to cerebral infarction.

Magnetic resonance imaging (MRI) was performed to study two cases of callosal disconnection due to cerebral infarction. Both cases had pure alexia and right homonymous hemianopsia. Midsagittal inversion recovery (IR) images demonstrated that the corpus callosum was completely intact, and coronal IR images revealed involvement of the lingual gyrus, fusiform gyrus, forceps major, inferior longitudinal fascicle, and optic radiation in the dominant cerebral hemisphere in both cases. Midline sagittal images provided direct visualization of the corpus callosum but did not provide sufficient information for the study of callosal disconnection syndromes. Coronal reconstructions aided in visualizing the relationship of lesions to the adjacent structures of cerebral white and gray matter. MRI may be one of the most useful tools for understanding the process of interhemispheric transference of information.

Aged↗

[Transient agraphia due to a left parieto-occipital glioma in a right-handed patient: amnesic or "pure" agraphia? (author's transl)].

The authors investigate a case of agraphia induced by a left parieto-occipital tumor (glioma) in a right-handed 67 year-old patient. After three successive neuropsychological examinations it still proves immensely difficult to determine whether defects of spontaneous writing, dictation and copy are due to apraxia, alexia or motor disturbances. The authors discuss whether the clinical findings indicate a "pure agraphia" syndrome or "amnesic agraphia". Finally, problems of aetiology and lesional localisation are examined in the light of the literature. (Acta neurol. belg., 1977, 77, 321-330).

Adrenal Cortex Hormones↗

[Aphasia caused by left paramedian thalamic infarction. Anatomo-clinical case].

A 67 year-old right-handed man presented with an aphasia of acute onset associated to paralysis of vertical eye movements, mild cerebellar right dysmetria and right hemiparesis without hemianopia. CT scan showed two low density areas in the left thalamus and left occipital lobe. Neuropsychological examination revealed a non fluent aphasia with normal repetition, semantic paraphasias, perseverations and good comprehension of verbal and written commands. There was no alexia. Writing was impaired only by motor disturbances. Three weeks after the onset, tetraparesis and impairment of consciousness followed by a locked in syndrome supervened. Death occurred four months after onset. Neuropathological examination showed a left paramedian thalamic infarct involving the ventrolateral, dorso-medial and intralaminar nuclei, sparing the pulvinar. There was an occipital infarct sparing the calcarine scissura and multiple infarctions in the pons and the cerebellum. Broca's and Wernicke's areas were spared. We suggest that the involvement of medial nuclei could be partly responsible of language disturbances in thalamic aphasia.

Aged↗

[Impaired visual perception in progressive multifocal leukoencephalopathy; a clinical diagnosis based on sequential computerized axial tomography (author's transl)].

Progressive multifocal leukoencephalopathy (PML) is a viral illness affecting principally cerebral white matter of patients, who have diminished immunologic resistance. A patient is presented, whose initial complaint was blurred vision. Relentless progression over a period of seven months to left hemianopsia, prosopagnosia, alexia with agraphia and eventually cortical blindness was observed. Accompanying the visual impairment was a steady deterioration of mental function. Sequential computerized axial tomography defined low density lesions in the occipital white matter with sparing of the cortical ribbon. As hemianopsia progressed to cortical blindness, the scans confirmed the evolution of bilaterial occipital lesions. The diagnosis was made clinically and established on histological and cultural grounds. Computerized tomography seems to be valuable in diagnosis and monitoring progress of PML.

Aged↗

[Efficacy of nidus embolization of large arterio-venous malformation prior to surgical excision].

Although it is undoubted that surgical excision is the ideal treatment for arterio-venous malformation, its large nidus and deep localization make it difficult in some instances. To make surgical procedure easier, efficacy of nidus embolization or of obstruction of feeding arteries by detachable balloon technique is reported. A case presented here is such a case with large arterio-venous malformation which was successfully removed surgically following artificial nidus embolization with silastic spheres. On May 12th, 1983, a 27-year-old housewife was admitted to our department with chief complaints of episodes of alexia, apraxia, and aphasia lasting for a few minutes during 4 years prior to admission. Neurological examination on admission showed no abnormalities. Left internal carotid angiogram taken on admission showed a large arterio-venous malformation in the left parieto-temporo-occipital region fed by three branches of the left middle cerebral artery and two branches of the left posterior cerebral artery (Fig. 2a, 3a). To enable successful excision without neurological deficits, nidus embolization with silastic spheres was performed on June 2, 1983. Forty silastic spheres of 1.5 mm in diameter were injected via a catheter introduced in the left internal carotid artery and a second injection of 48 silastic spheres of 2.0 mm in diameter was performed after confirmation of no stray of silastic spheres in the arteries unrelated to the nidus. Embolization procedure was interrupted after the second injection, because of stray of silastic sphere into the posterior internal frontal artery of the left anterior cerebral artery.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

[Anterior choroidal artery syndrome. Neuropsychological study of 4 cases].

A neuropsychological study was carried out in 4 cases of infarction in the territory of the anterior choroïdal artery. In 3 cases the lesions were on the right side. A syndrome of the minor hemisphere was present with severe visual neglect, constructional apraxia, alexia due to disorders of visuo-spatial strategy, anosognosia and motor impersistence. In the case with a left-sided lesion there was no neglect but a mild aphasia with impaired fluency, semantic paraphasias, perseveration and a decreased psycho-linguistic ability. The mechanisms of neglect in right-sided lesions are considered.

Aged↗

[Transcortical sensory aphasia produced by lesions of the anterior basal ganglia area].

We reported three cases of an aphasic syndrome caused by unusual lesion distribution. Our patients, language disorders could be summarized as transcortical sensory aphasia and showed following symptoms; (1) fluent paraphasic verbal output, (2) anomia which was not facilitated by cueing, (3) impaired comprehension of spoken language, (4) preserved capacity of repetition, (5) preserved ability of reading aloud with impaired comprehension of the written material and (6) agraphia. In addition, all had no associated physical neurological signs such as hemiparesis or hemianopsia. All were right handed. All three cases showed the similar lesion distribution by computed tomographic scanning of the brain. All had low density areas in the anterior portion of the left basal ganglia including the head of the caudate nucleus, the anterior portion of the putamen, the anterior portion of the anterior limb of the internal capsule and the nearby white matter. Case 2 also had the small right hemisphere lesion in the white matter near the anterior portion of the lateral ventricle. Transcortical sensory aphasia with this lesion distribution has not been reported. We attributed the causative damage to lesions of the white matter and not to lesions of the basal ganglia per se. It was also speculated that fluent aphasia can be produced by the anteriorly situated white matter lesion if issuing fibers from the Broca's area were spared. Finally a possible anatomoclinical correlation for "transcortical alexia" (preserved oral reading and impaired reading comprehension) was attempted. The symptom is probably a reflection of the fact that the posterior speech area including the angular gyrus was left intact.

Aged↗

[Granular cell tumor of a cerebral hemisphere: value of gliofibrillary protein acid assay].

A patient with a left parieto-occipital tumor presented generalized convulsions, and aphasia with alexia and agraphia. Diagnosis was confirmed by CT scan. Partial excision only was possible and was followed by radiotherapy. Histology showed the tumor to be composed of moderate and large sized cells with a granular PAS+ cytoplasm. Protoplasmic astrocytes were numerous at the periphery. Electron microscopy demonstrated typical features of Abrikossof's granular cell tumor: heterogeneous granulations constituted mainly of secondary lysosomes, autophagic vacuoles, multivesicular bodies, and rare angular bodies. The granular cells were interspersed with astrocytes, the feet of which were implanted on the capillary basal membrane. Immunoperoxidases assay of gliofibrillary protein acid demonstrated high levels in the intercellular prolongations, considered here to be astrocytic, but weak concentrations in the granular cells. This suggests uptake by the latter cells of adjacent astrocytic gliofibrillary protein acid, especially as the action of an anti-albumin serum on the tumor demonstrated that the granular cells can take up extracellular proteins by a passive mechanism. These findings stand against an astrocytic origin of the tumor. Comparison with other granular cell tumors, particularly pituicytomas, provides evidence of morphological unity, but cerebral granular cell tumors are rich in astrocytes, whereas these are absent in pituicytomas. The oncogenic unity of these tumors has not been established.

Brain Neoplasms↗

[Visual agnosia without right hemianopia in a right-handed patient (author's transl)].

A right-handed 77-year-old man presented with a left homonymous lateral hemianopsia involving the macula. Consciousness, memory, and oral language were normal, but the patient experienced great difficulties in identifying objects and images by sight, even though he recognized their geometrical shapes. He also had prosopagnosia, and alexia without agraphia but no agnosia for colours. The patient died suddenly 6 weeks after the onset of the disorders. Pathological examination demonstrated the presence of a bilateral occipital infarct involving on the right, the inferior border of the calcarine fissure, and the lingual and fusiform gyri, and on the left the fusiform gyrus. This supports the role in man of the inferior occipitotemporal convolutions in the perceptual integration of visual informations, and suggests that this region of the cortex is analogous with the inferotemporal cortex of monkeys which has been shown to be a determinant for the elaboration of visual objects.

Aged↗

[Confabulatory anomia from visual and tactile agnosia in a case of multi-infarct dementia. Neuropsychological study (author's transl)].

Observation of a patient suffering from a double softening of the brain (inner side of right temporal lobe, left occipital lobe). Clinically, Korsakoff's syndrome, right quadranopsia, pure alexia, tactile and visual agnosia concerning objects, colors and faces. Relative integrity of elementary perceptions (matching tests), impairments of stimulus identification (Lissauer's associative agnosia). Phenomena of sensoriphasic interactions are reported: normal speech in conversation, contrasting with confabulant anomia when naming or describing visual or tactile stimuli: then, escaping the control of sight, a relative "autonomy" of speech appears, with word perseverations and semantic "slipping" and filiations. Discussion of optical aphasia.

Agnosia↗

Eclamptogenic Gerstmann's syndrome in combination with cortical agnosia and cortical diplopia.

Cortical blindness is defined as a loss of vision due to bilateral retrogeniculate lesions (geniculocalcarine blindness). Gerstmann's syndrome is a combination of disorientation for left and right, finger agnosia, and profound agraphia, alexia, and acalculia. It is due to a lesion in the left angular gyrus, situated at the confluence of the temporal, parietal, and occipital lobes. We report on a patient who suffered from severe underdiagnosed eclampsia and who developed bilateral extensive medial temporal, parietal, and calcarine ischemic infarctions during an eclamptic fit. In addition, ischemia destroyed the left angular gyrus. The combination of these lesions led to Gerstmann's syndrome with additional cortical agnosia and cortical diplopia. For the first few months following the ischemic insult, the patient had been cortically blind. Thereafter, the patient slowly regained a visual acuity of 0.1 in both eyes. She then experienced monocular and binocular diplopia. Her ocular motility was normal; there was no phoria or tropia. Monocular and binocular diplopia slowly became less severe over the following year. Now, 2 years after the incident, the patient has a visual acuity of 0.2 in both eyes and no double vision. However, the handicapping symptoms of Gerstmann's syndrome, which make leading a normal life impossible, have persisted--the patient still cannot cope alone, mainly due to the severe disorientation for left and right. The picture of cortical agnosia, cortical diplopia, and Gerstmann's syndrome is a very rare combination. Visual recovery and rehabilitation in cortical blindness are severely affected and made difficult by the symptoms of Gerstmann's syndrome. In our case the reason for such a dramatic clinical picture was eclampsia, whose prodomes had not been diagnosed in time.

Adult↗

[Posterior cortical atrophy with progressive visual agnosia].

Interest in progressive focal cerebral syndromes associated with classical degenerative diseases has increased in recent years. Descriptions of posterior cortical atrophy with progressive visual agnosia are relatively rare. We present 5 patients (2 women) ranging in age between 57 and 72 years old. In all cases symptoms began and progressed with no known etiology. All cases were sporadic. The main clinical signs are difficulty in recognizing objects, colors, persons or places; topographical disorientation and visual memory alterations; alexia, simultagnosia, loss of ocular fixing and optic ataxia. Some patients presented other disturbances of praxis or memory and 2 progressed to global dementia. Language function was preserved and behavioral disturbances did not develop. The amplitude of the P100 visual evoked potential was low but latency was normal in 4 patients and prolonged in 1. Brain images showed atrophy and hypoperfusion in the parieto-occipital area. The neuropathology status of these patients is unknown.

Adult↗

[A study of 82 cerebral infarctions in the area of posterior cerebral arteries].

Of 598 consecutive non-selected cases of cerebral infarction included in a stroke registry, 82 cases (54 men and 28 women, mean age 66 +/- 14 years) of spontaneous and isolated posterior cerebral artery (PCA) territory infarction (right PCA in 36, left PCA in 35 and both in 11) were identified on the basis of CT combined with MRI in 51 cases. Infarction was superficial in 25 (group A), combined deep-superficial in 23 (group B) and deep in 34 (group C). Of 48 superficial lesions, 29 were massive while 19 were restricted to the territory of one branch. Of 57 deep lesions, 21 were located in the inferolateral thalamic territory, 10 in the paramedian thalamic territory, 12 in other midbrain or thalamic territories, and 14 in a combination of various midbrain and/or thalamic territories. Of 41 patients with unilateral superficial involvement, 39 had homonymous visual field defect. Unawareness of the visual defect and visual release hallucinations were observed with the same frequency in right and left lesions. Of 7 patients with bilateral superficial involvement, only 5 had bilateral visual field defect including incomplete cortical blindness in 3. The frequency of confusional state (n = 24) did not differ significantly in left versus right sided lesions while it was significantly higher in superficial or combined versus deep lesions (p = 0.05). Of 18 clinically evaluable patients with left PCA territory infarct, 14 had speech disorders including pure alexia in only one case. Of 15 patients with right territory infarction, 10 had spatial judgement disorders.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗

[Post-traumatic interhemispheric disconnection syndrome].

A left-handed man presented with a partial hemispheric disconnection syndrome of traumatic origin composed by a left hand unilateral agraphia, a left hand tactile anomia, a left visual field anomia, a left visual field alexia and a partial left ear extinction for verbal material. MRI of the brain showed lesion involving the trunk of the corpus callosum.

Adult↗