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Epilepsy & Behavior Reports 2021Acquired visual agnosia in the context of continuous spikes and waves during slow sleep (CSWS) is rarely described. We present a case of an almost 7-year-old boy who...
BACKGROUND
Acquired visual agnosia in the context of continuous spikes and waves during slow sleep (CSWS) is rarely described. We present a case of an almost 7-year-old boy who lost his ability to name pictures and recognize familiar faces. Initial encephalography (EEG) revealed sleep induced epileptiform activity with a spike-wave index (SWI) of 100%, predominanting in the left posterior head region.
METHODS
Serial neuropsychological testing with concomitant EEG was done during the first 18 months of treatment with intravenous methylprednisolone. We administered intelligence scales, verbal tasks (memory, fluency), visual tasks (drawings, search, face recognition), and tasks requiring visual-verbal integration (picture naming, visual closure).
ANALYSES
Neuropsychological recovery studied with reliable cognitive change cut-offs and 95% confidence intervals.
RESULTS
With treatment, there was an improvement of the EEG pattern (SWI reduction to 45%), followed by a relapse (SWI 82%). Neuropsychological measures in part synchronized with improvement, stability, and fluctuating values. Significant increases were seen on Verbal Comprehension Index and semantic memory. Visual Spatial Index remained unchanged (67 to 73). Naming pictures showed only limited change. Interpreting degraded pictures remained extremely difficult.
DISCUSSION
Acquired visual agnosia may be seen in children with CSWS. Early recognition, prompt accurate treatment and tailored neuropsychological assessment remain crucial.
PubMed: 34258574
DOI: 10.1016/j.ebr.2021.100465 -
Cortex; a Journal Devoted To the Study... Aug 2022Developmental prosopagnosia (DP) is a neurodevelopmental disorder associated with difficulties in the perception and recognition of faces. However, the extent to which...
Developmental prosopagnosia (DP) is a neurodevelopmental disorder associated with difficulties in the perception and recognition of faces. However, the extent to which DP affects non-face object is an ongoing debate. In this study, we asked whether pareidolic objects (which give rise to the perception of a face) are also affected in DP. First, we compared performance in DPs (n = 30) and controls (n = 27) on a recognition task with faces, pareidolic objects and non-pareidolic objects (bottles). The pareidolic objects had either similar or dissimilar image statistics to faces. Consistent with our understanding of DP, we found that the pattern of recognition across items between DPs and controls was lowest for faces. Interestingly, there was also a low correlation between DPs and controls for pareidolic-similar objects that was similar to faces. In contrast, there were higher correlations between DPs and controls for pareidolic-dissimilar objects and bottles, which were both significantly different to faces. These findings suggest that the deficit in DP involves processing image properties that are common to faces. Next, using an individual differences approach across a large group of neurotypical adults (n = 94), we found that face recognition covaried with the recognition of pareidolic-similar objects, but not with pareidolic-dissimilar objects or non-pareidolic objects. Together, these findings support the idea that a representation based on image properties plays an important role in the perception and recognition of objects and faces and that the deficit in the perception of some object categories in DP could be explained by their similarity to the image properties found in faces.
Topics: Adult; Facial Recognition; Humans; Individuality; Pattern Recognition, Visual; Prosopagnosia; Recognition, Psychology
PubMed: 35576670
DOI: 10.1016/j.cortex.2022.04.011 -
Cognitive linguistic Treatment in Landau Kleffner Syndrome: Improvement in Daily Life Communication.Child Neurology Open 2021We report a case study of cognitive linguistic treatment in a teenager with chronic severe Landau Kleffner Syndrome. The effect of speech and language therapy in LKS is...
We report a case study of cognitive linguistic treatment in a teenager with chronic severe Landau Kleffner Syndrome. The effect of speech and language therapy in LKS is rarely examined and our case is unique in that we use an effective approach in adult aphasia to treat language deficits in aphasia in LKS. The results show successful acquisition of a considerable amount of new words as well as improved communication in daily life. However, auditory verbal agnosia, the most prominent feature in LKS, persisted. Cognitive linguistic treatment seems a promising treatment to improve spoken language production in LKS, but more research is needed to optimize speech and language therapy of auditory verbal agnosia and auditory language comprehension in children with LKS.
PubMed: 34124282
DOI: 10.1177/2329048X211022196 -
Cortex; a Journal Devoted To the Study... Aug 2019Aphasia typically is associated with comparable difficulties in written and spoken modalities of language expression and comprehension. In contrast, auditory verbal...
Aphasia typically is associated with comparable difficulties in written and spoken modalities of language expression and comprehension. In contrast, auditory verbal agnosia is the disproportionate difficulty comprehending spoken compared to written language, also typically greater than difficulties with spoken and written language expression, in the absence of a primary sensory deficit. The terms pure word deafness and auditory verbal agnosia are often used synonymously. However, the broader term of auditory agnosia more accurately reflects difficulty processing both speech and non-speech sounds whereas individuals with auditory verbal agnosia (pure word deafness) have preserved processing of environmental sounds. Auditory agnosia is reported in the stroke literature, but rarely reported in progressive neurologic disorders. Here, we report a case of a woman who presented with what is best described as a prominent auditory deficit in the context of an initially unclassifiable, or mixed, primary progressive aphasia (PPA) with accompanying apraxia of speech. Her clinical presentation shared features with auditory agnosia, although sensory functioning was not formally assessed. We report clinical and neuroimaging data spanning 6 years and subsequent autopsy results. She presented at 65 years of age, 5 years post onset of symptoms that included insidious and progressive difficulties thinking of words, constructing sentences, pronouncing words, and understanding instructions. She had disproportionate difficulty with comprehension of spoken compared to written language. She eventually developed features of the nonfluent/agrammatic variant of PPA, as well as an apraxia of speech. Imaging with [18F]-fluorodeoxyglucose (FDG)-PET revealed progression of bilateral (left greater than right) hypometabolism involving the frontal, temporal (predominantly the lateral superior gyrus), and parietal lobes, that eventually included the supplementary motor area, anterior cingulate, and caudate. Autopsy revealed pathological lesions consistent with corticobasal degeneration.
Topics: Aged; Agnosia; Aphasia, Primary Progressive; Comprehension; Female; Humans; Language; Neuropsychological Tests; Speech Perception
PubMed: 30878181
DOI: 10.1016/j.cortex.2019.01.021 -
Behavior Research Methods Feb 2022We present an expanded version of a widely used measure of unfamiliar face matching ability, the Glasgow Face Matching Test (GFMT). The GFMT2 is created using the same...
We present an expanded version of a widely used measure of unfamiliar face matching ability, the Glasgow Face Matching Test (GFMT). The GFMT2 is created using the same source database as the original test but makes five key improvements. First, the test items include variation in head angle, pose, expression and subject-to-camera distance, making the new test more difficult and more representative of challenges in everyday face identification tasks. Second, short and long versions of the test each contain two forms that are calibrated to be of equal difficulty, allowing repeat tests to be performed to examine effects of training interventions. Third, the short-form tests contain no repeating face identities, thereby removing any confounding effects of familiarity that may have been present in the original test. Fourth, separate short versions are created to target exceptionally high performing or exceptionally low performing individuals using established psychometric principles. Fifth, all tests are implemented in an executable program, allowing them to be administered automatically. All tests are available free for scientific use via www.gfmt2.org .
Topics: Face; Facial Recognition; Humans; Neuropsychological Tests; Pattern Recognition, Visual; Prosopagnosia; Psychometrics; Recognition, Psychology
PubMed: 34159512
DOI: 10.3758/s13428-021-01638-x -
Frontiers in Neurology 2021Consensus criteria on corticobasal degeneration (CBD) include alien limb (AL) phenomena. However, the gist of the behavioral features of AL is still "a matter of... (Review)
Review
Consensus criteria on corticobasal degeneration (CBD) include alien limb (AL) phenomena. However, the gist of the behavioral features of AL is still "a matter of debate." CBD-related AL has so far included the description of involuntary movements, frontal release phenomena (frontal AL), or asomatognosia (posterior or "real" AL). In this context, the most frequent symptoms are language and praxis deficits and cortical sensory misperception. However, asomatognosia requires, by definition, intact perception and cognition. Thus, to make a proper diagnosis of AL in the context of CBD, cognitive and language dysfunctions must be carefully verified and objectively assessed. We reviewed the current literature on AL in CBD and now propose that the generic use of the term AL should be avoided. This catchall AL term should instead be deconstructed. We propose that the term AL is appropriate to describe clinical features associated with specific brain lesions. More discrete sets of regionally bound clinical signs that depend on dysfunctions of specific brain areas need to be assessed and presented when posing the diagnosis. Thus, in our opinion, the AL term should be employed in association with precise descriptions of the accompanying involuntary movements, sensory misperceptions, agnosia-asomatognosia contents, and the presence of utilization behavior. The review also offers an overview of functional magnetic resonance imaging-based studies evaluating AL-related phenomena. In addition, we provide a complementary set of video clips depicting CBD-related involuntary movements that should not mistakenly be interpreted as signs of AL.
PubMed: 34566830
DOI: 10.3389/fneur.2021.661130 -
Iranian Journal of Child Neurology 2019Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan...
Mucopolysaccharidosis type III (MPS III; Sanfilippo syndrome) is a metabolic disorder characterized by a lysosomal enzyme deficiency in the catabolic pathway of heparan sulfate. The patients with mucopolysaccharidosis type III usually present with declined neurocognitive functions such as speech and hearing loss. Subtle somatic features of patients with mucopolysaccharidosis type III can lead to diagnostic delay and consequently, a greater neurocognitive deterioration may happen. Herein, we report a 9-yr-old boy referred to Loghman Hospital, Tehran, Iran, in 2018. He had developed normally up to four yr of age when his symptoms initiated with behavioral disturbances such as auditory agnosia and decreased verbal communication. Progression of his symptoms to seizure and ataxia, brain perfusion scan and electroencephalography features strongly suggested landau-Kleffner syndrome. However, results of gene sequencing analysis and high urinary glycosaminoglycan excretion confirmed mucopolysaccharidosis type III as his final diagnosis. This case strongly recommends screening for metabolic disorders such as mucopolysaccharidosis type III in the patients diagnosed as having landau-Kleffner syndrome.
PubMed: 31327975
DOI: No ID Found -
Neuropsychology Sep 2020Anosognosia, or unawareness, for memory loss has been proposed to underlie cognitive functions such as memory and executive function. However, there is an inconsistent...
OBJECTIVE
Anosognosia, or unawareness, for memory loss has been proposed to underlie cognitive functions such as memory and executive function. However, there is an inconsistent association between these constructs. Recent studies have shown that compromise ongoing self-monitoring of one's memory associates with anosognosia for memory loss. Yet to date it is unclear which memory monitoring mechanisms are impaired in these patients. In this study, we examined the extent to which or (e.g., ability to monitor the temporal relevance of a memory) and (e.g., the ability to distinguish which memories stem from internal as opposed to external sources) are associated with awareness of memory deficits.
METHOD
A total of 35 patients ( = 69 years; = 14 years of education) with memory difficulties following a stroke were recruited from outpatient clinics. Patients were assessed with measures of self-awareness of memory difficulties, cognitive abilities and 2 experimental paradigms assessing source and temporal monitoring.
RESULTS AND CONCLUSION
Results showed that patients unaware of their memory difficulties were more likely to externalize the source of their memories. Specifically, those unaware of their deficits were more likely to assign an external source to memories that were internally produced (e.g., imagined). No differences were observed in relation to temporal monitoring between patients aware and unaware of their deficits. This study informs current theoretical models of self-awareness of memory loss. Future studies should attempt to replicate these findings and explore different memory monitoring mechanisms in relation to anosognosia for memory loss. (PsycInfo Database Record (c) 2020 APA, all rights reserved).
Topics: Aged; Aged, 80 and over; Agnosia; Awareness; Cognition; Executive Function; Female; Humans; Male; Memory; Memory Disorders; Middle Aged; Neuropsychological Tests; Psychomotor Performance; Reaction Time; Self Concept; Stroke; Time Perception
PubMed: 32852998
DOI: 10.1037/neu0000643 -
Neurological Sciences : Official... Feb 2021Acquired prosopagnosia is usually a consequence of bilateral or right hemisphere lesions and is often associated with topographical disorientation and dyschromatopsia....
Acquired prosopagnosia is usually a consequence of bilateral or right hemisphere lesions and is often associated with topographical disorientation and dyschromatopsia. Left temporo-occipital lesions sometimes result in a face recognition disorder but in a context of visual object agnosia with spared familiarity feelings for faces, usually in left-handers. We describe a patient with a left temporo-occipital hemorrhagic lesion unexpectedly resulting in a deficit of face familiarity, which could represent a mild form of associative prosopagnosia. Our patient failed to feel familiarity feelings even with very well-known famous faces but had neither visual object agnosia nor defects with semantics or naming of celebrities. This was confirmed even when the patient was re-tested a year later. We speculate that a graded lateralization of face processing could be at the basis of occasional cases of prosopagnosia.
Topics: Agnosia; Emotions; Humans; Pattern Recognition, Visual; Prosopagnosia; Recognition, Psychology
PubMed: 32648048
DOI: 10.1007/s10072-020-04581-5 -
Neurology Aug 2021
Topics: Adult; Brain Mapping; Drug Resistant Epilepsy; Electric Stimulation; Female; Gerstmann Syndrome; Humans
PubMed: 34187861
DOI: 10.1212/WNL.0000000000012441