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Colour association and "colour amnesia" in aphasia.

"Colour association" performance of 50 aphasic patients was investigated by means of a test in which they identified the characteristic colours of objects shown in line drawings. All aphasics with defects in colour association were impaired in reading comprehension. However, some (33%) retained normal aural comprehension. Approximately half the aphasics with receptive language impairment performed normally in colour association. The findings suggest that "colour amnesia" may be the result of a specific cognitive disturbance which is also responsible for a subtype of aphasic alexia.

Adult↗

When the left brain is not right the right brain may be left: report of personal experience of occipital hemianopia.

OBJECTIVES: To make a personal report of a hemianopia due to an occipital infarct, sustained by a professor of neurology. METHODS: Verbatim observation of neurological phenomena recorded during the acute illness. RESULTS: Hemianopia, visual hallucinations, and non-occipital deficits without extraoccipital lesions on MRI, are described and discussed. CONCLUSIONS: Hemianopia, due to an occipital infarct, without alexia, is not a disability which precludes a normal professional career. Neurorehabilitation has not been necessary.

Aortic Valve↗

Intact verbal description of letters with diminished awareness of their forms.

Visual processing and its conscious awareness can be dissociated. To examine the extent of dissociation between ability to read characters or words and to be consciously aware of their forms, reading ability and conscious awareness for characters were examined using a tachistoscope in an alexic patient. A right handed woman with 14 years of education presented with incomplete right hemianopia, alexia with kanji (ideogram) agraphia, anomia, and amnesia. Brain MRI disclosed cerebral infarction limited to the left lower bank of the calcarine fissure, lingual and parahippocampal gyri, and an old infarction in the right medial frontal lobe. Tachistoscopic examination disclosed that she could read characters aloud in the right lower hemifield when she was not clearly aware of their forms and only noted their presence vaguely. Although her performance in reading kanji was better in the left than the right field, she could read kana (phonogram) characters and Arabic numerals equally well in both fields. By contrast, she claimed that she saw only a flash of light in 61% of trials and noticed vague forms of stimuli in 36% of trials. She never recognised a form of a letter in the right lower field precisely. She performed judgment tasks better in the left than right lower hemifield where she had to judge whether two kana characters were the same or different. Although dissociation between performance of visual recognition tasks and conscious awareness of the visual experience was found in patients with blindsight or residual vision, reading (verbal identification) of characters without clear awareness of their forms has not been reported in clinical cases. Diminished awareness of forms in our patient may reflect incomplete input to the extrastriate cortex.

Awareness↗

Selective impairment of facial recognition due to a haematoma restricted to the right fusiform and lateral occipital region.

A 67 year old right handed Japanese man developed prosopagnosia caused by a haemorrhage. His only deficit was the inability to perceive and discriminate unfamiliar faces, and to recognise familiar faces. He did not show deficits in visual or visuospatial perception of non-facial stimuli, alexia, visual agnosia, or topographical disorientation. Brain MRI showed a haematoma limited to the right fusiform and the lateral occipital region. Single photon emission computed tomography confirmed that there was no decreased blood flow in the opposite left cerebral hemisphere. The present case indicates that a well placed small right fusiform gyrus and the adjacent area can cause isolated impairment of facial recognition. As far as we know, there has been no published case that has demonstrated this exact lesion site, which was indicated by recent functional MRI studies as the most critical area in facial recognition.

Aged↗

"Bottom-up" and "top-down" effects on reading saccades: a case study.

OBJECTIVE: To investigate the role right foveal/parafoveal sparing plays in reading single words, word arrays, and eye movement patterns in a single case with an incongruous hemianopia. METHODS: The patient, a 48-year-old right handed male with a macular sparing hemianopia in his left eye and a macular splitting hemianopia in his right eye, performed various reading tasks. Single word reading speeds were monitored using a "voice-trigger" system. Eye movements were recorded while reading three passages of text, and PET data were gathered while the subject performed a variety of reading tasks in the camera. RESULTS: The patient was faster at reading single words and text with his left eye compared with his right. A small word length effect was present in his right eye but not his left. His eye movement patterns were more orderly when reading text with his left eye, making fewer saccades. The PET data provided evidence of "top-down" processes involved in reading. Binocular single word reading produced activity in the representation of foveal V1 bilaterally; however, text reading with the left eye only was associated with activation in left but not right parafoveal V1, despite there being visual stimuli in both visual fields. CONCLUSIONS: The presence of a word length effect (typically associated with pure alexia) can be caused by a macular splitting hemianopia. Right parafoveal vision is not critically involved in single word identification, but is when planning left to right reading saccades. The influence of top-down attentional processes during text reading can be visualised in parafoveal V1 using PET.

Hemianopsia↗

Somesthetic disconnection syndromes in patients with callosal lesions.

Somesthetic disconnection syndromes were investigated in relation to the sites of lesions in the corpus callosum in 3 patients with callosal lesions, in order to identify the callosal regions responsible for the interhemispheric transfer of somesthetic information. Cases 1 and 2 with lesions in the posterior truncus exhibited transfer deficits of discriminative sensations between the left and right hands, left-sided tactile anomia and left-sided somesthetic alexia. Case 3 with lesions in the posteroventral part of the posterior truncus showed no signs of somesthetic disconnection syndromes. The results suggest the importance of the anterior and/or dorsal part of the posterior truncus of the corpus callosum for interhemispheric transfer of the discriminative sensations and integrated somesthetic information necessary for tactile naming and somesthetic reading.

Adult↗

Posterior cortical atrophy: clinical characteristics and differences compared to Alzheimer's disease.

BACKGROUND: Predominant and progressive complex visual disorders are often due to posterior cortical atrophy (PCA), a rare early-onset dementing syndrome presenting with visual complaints. In clinicopathological studies, PCA is most commonly considered a form of Alzheimer's disease (AD); no prior study has evaluated clinical differences between PCA and AD. METHODS: This study identified 15 patients who presented with progressive complex visual disorders and predominant occipitoparietal hypoperfusion on SPECT. These patients were retrospectively compared on clinical variables with 30 patients with clinically probable AD matched for gender, age and duration of illness. RESULTS: The PCA patients presented with alexia, elements of Balint's syndrome, apperceptive visual agnosia, dressing apraxia and environmental disorientation along with elements of Gerstmann's syndrome. Compared to the AD patients, the 15 PCA patients (mean age of onset 58 years, range 51-64) had significantly better verbal fluency, less memory difficulty, more depression and greater insight into their illness but similar familial and apolipoprotein E risk factors. In the PCA patients, MRI often showed occipitoparietal atrophy without detectable mesiotemporal atrophy. CONCLUSIONS: PCA is a distinct clinical syndrome and not just AD with prominent visual deficits. Compared to AD controls, PCA patients have better language and memory but more insight and depression and more posterior atrophy on MRI. These results indicate clinical criteria for the diagnosis of PCA and recommend specific interventions such as visual aids and antidepressant medications. Similar risk factors and course suggest that PCA is most commonly an early-onset posteriorly shifted AD variant.

Aged↗

Partial disconnection syndrome following penetrating stab wound of the brain.

A case of callosal disconnection syndrome caused by a penetrating stab wound of the brain is reported. A portion of the genu and the inferior splenium of the corpus callosum were sectioned by an ice pick without any other significant cerebral damage. We could detect incomplete disconnection left visual alexia by tachistoscopy. It would now appear that the inferior part of the splenium has a significant functional role in transmitting visual verbal codes from the right to the left cerebral hemisphere, but the remaining superior fibers of the splenium may carry some information, albeit less efficiently than the intact structure.

Adult↗

Visualizing the neural bases of a disconnection syndrome with diffusion tensor imaging.

Disconnection syndromes are often conceptualized exclusively within cognitive box-and-arrow diagrams unrelated to brain anatomy. In a patient with alexia in his left visual field resulting from a posterior callosal lesion, we illustrate how diffusion tensor imaging can reveal the anatomical bases of a disconnection syndrome by tracking the degeneration of neural pathways and relating it to impaired fMRI activations and behavior. Compared to controls, an abnormal pattern of brain activity was observed in the patient during word reading, with a lack of activation of the left visual word form area (VWFA) by left hemifield words. Statistical analyses of diffusion images revealed a damaged fiber tract linking the left ventral occipito-temporal region to its right homolog across the lesioned area of corpus callosum and stopping close to the areas found active in fMRI. The behavioral disconnection syndrome could, thus, be related functionally to abnormal fMRI activations and anatomically to the absence of a connection between those activations. The present approach, based on the "negative tracking" of degenerated bundles, provides new perspectives on the understanding of human brain connections and disconnections.

Adult↗

From orthography to phonetics: ERP measures of grapheme-to-phoneme conversion mechanisms in reading.

Neuroimaging has provided evidence that the first stages of visual word recognition activate a visual word-form center localized in the left extrastriate cortex (fusiform gyrus). Accordingly, neurological cases of patients suffering from pure alexia reported the left posterior occipital lobe as the possible locus of orthographic analysis. There is less agreement in the literature about which brain structures are involved in the subsequent stages of word processing and, in particular, their time course of activation. Functional magnetic resonance imaging and magnetic source imaging studies recently reported data that could indicate a dual route model of reading. These findings are particularly relevant to studies on the functional deficits associated with phonological and surface dyslexia. There is evidence for the existence of two different brain mechanisms supporting phonological processing in visual word recognition: one mechanism subserving "assembled phonology" for reading letter strings and another one subserving "addressed phonology" for reading meaningful words. However, available knowledge on the time course and neural locus of grapheme-to-phoneme conversion mechanisms in reading is still inadequate. In this study, we compared processing of meaningful and meaningless Italian words in a task requiring a phonemic/phonetic decision task. Stimuli were 1152 different orthographic stimuli presented in the central visual field. Half the stimuli were Italian words (with a high or low frequency of occurrence), the other half were meaningless strings of letters (legal pseudowords and letter strings). Event-related potentials were recorded from 28 scalp sites in 10 Italian university students. The task consisted of deciding about the presence/absence of a given "phone" in the hypothetical enunciation of word read: for example, "Is there a /k/ in cheese?". Results showed that lexical frequency and orthographical regularity affected linguistic processing within 150 msec poststimulus. Indeed, the amplitude of a centroparietal P150 varied as a function of stimulus type, being larger in response to high-frequency words than to low-frequency ones and to words and pseudowords than to letter strings. This component might index visual categorization processes and recognition of familiar objects, being highly sensitive to orthographic regularity and "ill-formedness" of words. The amplitude of the P150 was the same in response to well-formed meaningless and to meaningful words, when these latter had a low lexical frequency. This might indicate that highly familiar words are recognized as meaningful unitary visual objects at very early stages of processing, through a visual route to an orthographic input lexicon. Moreover, the amplitude of the negativity recorded between 250 and 350 msec showed an anteroposterior topographic dissociation for access to the phonemic representation of well- or ill-formed strings of characters. Brain responses were larger over the left occipito-temporal regions during reading of words and pseudowords and over the left frontal regions during reading of letter strings.

Adult↗

Occipital-callosal pathways in children: Validation and atlas development.

Diffusion tensor imaging and fiber tracking were used to measure fiber bundles connecting the two occipital lobes in 53 children of 7-12 years of age. Independent fiber bundle estimates originating from the two hemispheres converge onto the lower half of the splenium. This observation validates the basic methodology and suggests that most occipital-callosal fibers connect the two occipital lobes. Within the splenium, fiber bundles are organized in a regular pattern with respect to their cortical projection zones. Visual cortex dorsal to calcarine projects through a large band that fills much of the inferior half of the splenium, while cortex ventral to calcarine sends projections through a band at the anterior inferior edge of the splenium. Pathways projecting to the occipital pole and lateral-occipital regions overlap the dorsal and ventral groups slightly anterior to the center of the splenium. To visualize these pathways in a typical brain, we combined the data into an atlas. The estimated occipital-callosal fiber paths from the atlas form the walls of the occipital horn of the lateral ventricle, with dorsal paths forming the medial wall and the ventral paths bifurcating into a medial tract to form the inferior-medial wall and a superior tract that joins the lateral-occipital paths to form the superior wall of the ventricle. The properties of these fiber bundles match those of the hypothetical pathways described in the neurological literature on alexia.

Anatomy, Artistic↗

Rehabilitation of Balint's syndrome: a single case report.

Very little research has been done in the area of rehabilitation of the visual perceptual deficits observed after Balint's syndrome. This syndrome is characterized by difficulties with visual scanning, dysmetria secondary to visual perceptual deficits, and an inability to perceive more than 1 object at a time. This article describes the effects of a neuropsychological treatment protocol on a 23-year-old patient who suffered a fat embolism involving the territory of the posterior cerebral arteries. A neuropsychological evaluation carried out 12 months after the brain embolism disclosed Balint's syndrome, alexia without agraphia, visual agnosia, prosopagnosia, and memory impairments. The rehabilitation protocol included both visuoperceptual retraining and a functional program designed to provide rehabilitation in contexts that were meaningful to the patient. After 1 year of treatment, a second neuropsychological evaluation was carried out. Significant improvement was demonstrated in terms of both objective testing and the return of an integrated and productive lifestyle.

Adaptation, Psychological↗

Clinical, genetic, and neuropathologic characteristics of posterior cortical atrophy.

OBJECTIVE: To examine the clinical, genetic, and neuropathologic features of posterior cortical atrophy (PCA). DESIGN/METHODS: Using a broad definition of PCA as a syndrome with the insidious onset of visual dysfunction in the absence of primary ophthalmologic causes, the authors identified and then reviewed the presenting signs and symptoms, ApoE genotypes, tau haplotypes, and neuropathologic findings when available of PCA cases from two dementia research centers collected over the past 14 years. RESULTS: The authors identified 40 PCA cases. Their mean age at symptom onset was 60.5 +/- 8.9 years. There were twice as many women as men in the series. The principal types of visual impairment were simultanagnosia (82%) and visual field defect (47.5%). Acalculia, alexia, and anomia were also common. Insight was preserved in almost all (95%) early in the disorder. Neither apoE epsilon4 nor tau haplotype frequencies were different from typical Alzheimer disease (AD). Nine patients had died and underwent postmortem examination. Seven autopsied cases had AD pathology but when compared to typical AD, the neurofibrillary tangle (NFT) densities were significantly higher in Brodmann areas 17 and 18 (p < 0.05) and significantly lower in the hippocampus (p < 0.05). Two cases had corticobasal degeneration with maximal involvement of tau positive glial pathology in the posterior parietal lobe and Brodmann areas 17 and 18. CONCLUSIONS: PCA is a distinctive dementia syndrome in which the most pronounced pathologic involvement is in the occipitoparietal regions independent of the specific underlying pathology. AD was the most common pathologic cause, but its regional distribution differed from typical AD.

Adult↗

The cognitive profile of posterior cortical atrophy.

BACKGROUND: Posterior cortical atrophy (PCA) is a progressive dementia characterized by prominent disorders of higher visual processing, affecting both dorsal and ventral streams to cause Balint's syndrome, alexia, and visual agnosia. OBJECTIVE: To define the cognitive profile of PCA and compare to the typical, primary amnestic dementia of the Alzheimer's type (DAT). METHODS: The authors used standard cognitive tests and a novel battery designed to reflect dysfunction in both ventral (Object, Face & Color Agnosia Screen [OFCAS]) and dorsal (complex pictures and compound stimuli) visual streams. The authors identified 19 patients with PCA and compared their performance to a matched group of patients with DAT and normal controls. RESULTS: Patients with PCA were younger with marked impairment in visuospatial tasks, reading, and writing but relative preservation of memory compared to DAT using standard tests. Dorsal stream signs were most prevalent among the patients with PCA with no pure ventral stream syndromes found. All novel tests distinguished reliably between subjects with complex picture descriptions and processing of compound stimuli showing the most significant differences compared to DAT. CONCLUSIONS: PCA is predominantly a dorsal stream syndrome, distinct from typical DAT, which involves occipitotemporal regions over time.

Aged↗

Basal temporal language area demonstrated by electrical stimulation.

We report on a 38-year-old patient with intractable complex partial seizures originating in the dominant left medial temporal region. In the work-up for seizure surgery, arrays of subdural electrodes were placed, and electrical stimulation revealed marked language interference in a 2 X 2-cm area in the left basal temporal fusiform gyrus (3.5 to 5.5 cm posterior to the temporal tip). Complete receptive and expressive aphasia, inability to repeat, agraphia, and alexia were elicited, but visual memory was preserved, and no constructional apraxia was noted. Stimulation of the basal temporal gyrus at lower stimulus intensities produced a relatively selective and severe anomia.

Adult↗

Hemiplegia in posterior cerebral artery occlusion.

We report 4 patients with hemiplegia due to a posterior cerebral artery occlusion. Associated clinical signs were aphasia, alexia or a neglect syndrome, hemianopia, and hemisensory loss. Hemiplegia was due to infarction in the lateral midbrain. The level of the occlusion in the posterior cerebral artery may be located distal to the junction with the posterior communicating artery.

Aged↗

Clinical management of agnosia.

Agnosia is a neurological recognition deficit that affects a single modality. Visual agnosias include pure object agnosia, prosopagnosia, akinetopsia, and pure alexia. Auditory agnosias include pure word deafness, phonagnosia, and pure sound agnosia. New neuroimaging tools have permitted scientists to better understand the loci of lesions that cause various agnosias and from that knowledge to develop theories about the processing networks that contribute to perception and recognition in each modality. These research data, in turn, inform the rehabilitation process. By utilizing current knowledge about neuroprocessing networks, clinical professionals can differentially diagnose agnosias from aphasia and other cognitive deficits. Practical approaches to treatment of agnosia will follow once the diagnosis is established.

Agnosia↗

Two posterior neuropsychological syndromes revisited.

Of the higher disorders associated with posterior cerebral lesions two have received considerable attention in the past decade. These are prosopagnosia, the so-called agnosia for faces, and alexia without agraphia. Major aspects of these disorders are reviewed briefly.

Agnosia↗