Separated at birth: Rediscovering the lost emotions in Luria's Working Brain.

Aleksandr Luria repeatedly emphasised the importance of emotions and the right hemisphere in his neuropsychological writings. It is surprising, therefore, that Luria's most influential book, The Working Brain, appears to lack an explicit section on these topics. This is especially notable because of a comment in the book's English-language Introduction, by Karl Pribram, referencing Luria's thoughts about precisely this material. Remarkably, it seems that Luria did write such an explicit chapter, in the original Russian edition. However, in the English-language version, the relevant sections were separated, embedded elsewhere without chapter headings, and altered, presumably following an explicit translation decision. The present paper tracks the nature of these changes and, 50 years later, presents the material for the first time translated and reunited in English, as Luria intended. After the translation, we offer a brief commentary, on the ways in which Luria's ideas were in some respects prescient, and in other respects less well-informed about the brain basis of emotions and the right hemisphere. This reunification offers an interesting time capsule on the opinions of one of neuropsychology's greatest minds, on a topic which Luria admits had, at the time, only a modest empirical foundation.

Bilingualism as a protective factor in aphasia.

BACKGROUND: Bilingualism may affect the profile of cognitive disturbances associated with stroke. Its impact on aphasia severity, however, is in need of substantiation. AIMS: To determine the relationship between bilingualism and vascular aphasia severity. METHODS: This is an observational cross-sectional study conducted on people with post-stroke aphasia. Our sample included 155 monolingual and 53 bilingual vascular aphasia patients. They were selected in a neurological hospital in Kolkata (West Bengal, India). RESULTS: The Bengali version of Western Aphasia Battery (BWAB) was used in this study. Aphasia severity was compared between monolingual and bilingual participants. The overall difference in the mean aphasia quotient (AQ) between bilingual and monolingual participants was statistically significant (p = 0.009). It was also found that in bilingual participants, aphasia was less severe in certain subgroups, namely: higher lesion volume, male gender, and sub-cortical stroke, while in none of the subgroups a monolingual advantage was documented. CONCLUSION: Current results suggest that bilingualism represents a protective factor in vascular aphasia; this effect is observed particularly in some aphasia subgroups.

Twenty years of Colombian experience with enzymatic screening in patients with features of mucopolysaccharidosis.

Mucopolysaccharidoses (MPSs) are a group of genetic alterations whose effect is the progressive intralysosomal accumulation of glycosaminoglycans. Affected individuals are deficient in one or more lysosomal enzymes which, depending on the MPS, may cause coarse facial features, short stature, multiple skeletal dysplasia, joint stiffness, or developmental delay. Their diagnosis is mostly performed late or incorrectly, and it represents a challenge since it requires specialized tests only performed in major cities. This makes it difficult for patients to have access to physicians since their geographical location is distant and therefore, the use of samples collected in solid-phase represents an advantage for the study of high-risk populations. In addition, epidemiological information about rare diseases, especially in Latin America, is scarce or inconsistent. Our aim was to report the experience of 20 years of selective screening by assessing enzyme activity and reporting incidence values of MPS in Colombia. This study validated a group of fluorometric endpoint techniques in 8239 patients. The samples were dried blood spots (DBS) collected on filter paper and leukocyte extracts. Reference values in the Colombian population for α-l-iduronidase, iduronate 2-sulfatase, α-N-acetylglucosaminidase, N-acetylglucosamine-6-sulfate sulfatase, ß-galactosidase, arylsulfatase B, and ß-glucuronidase were established in leukocyte extracts, and patients reference ranges were updated in the case of DBS samples. Incidence values were calculated for each MPS and the distribution of cases across the country is also shown. This study offers very useful information for the health system, the scientific community, and it facilitates the diagnosis of these disorders. This is indispensable when seeking to develop new diagnostic or treatment approaches for patients.

Linguistic profiles of variants of primary progressive aphasia.

BACKGROUND: Several subtypes of primary progressive aphasia (PPA) have been proposed. Most reports use small samples, and few have included Spanish-speaking participants. AIM: To analyze the language profile and nonlinguistic deficits in a large sample of PPA Spanish monolingual participants. METHOD: 177 individuals were diagnosed with PPA in a sample consisting of 69 men and 108 women (Mage = 66.40 years, SD = 9.30). The participants were assessed using the Spanish versions of the Western Aphasia Battery Revised (SWAB-R) and the Boston Diagnostic Aphasia Examination (SBDAE). Non-verbal reasoning was evaluated with the Raven's Colored Progressive Matrices. RESULTS: 41.8% of the sample met the criteria for the logopenic variant (lvPPA), while 28.2% met the criteria for semantic (svPPA), 15.3% for lexical (lxvPPA), and 14.7% for nonfluent/agrammatic (nfvPPA) variants. Language difficulties were similar in all variants except for lxvPPA. Scores on Spontaneous Language, Auditory Comprehension, Repetition, and Naming were significantly higher for the lxvPPA group. Raven's Colored Progressive Matrices scores were significantly lower in lvPPA. Years of education correlated with all test scores, while age was negatively associated with naming. When the PPA variants were classified according to the traditional aphasia classification, discrepancies were evident. Furthermore, the most frequent type of aphasia was Amnesic, while the least frequent was Wernicke's aphasia. CONCLUSION: The SWAB-R is useful in describing the clinical characteristics of aphasia for each variant of PPA, but quantitative scores from this battery are not capable of distinguishing between variants of PPA, with the exception of lxvPPA.

What do neuropsychological tests assess?

Background. Contemporary neuroimaging techniques, particularly fMRI and PET, have demonstrated that cognitive abilities do not strictly depend on specific brain areas, but rather on complex brain circuits or systems.Methods. Using PubMed and Google Scholar databases, a search for functional studies (fMRI and PET) during the performance of several neuropsychological tests was done. The pattern of brain activity found during the solution of some executive functions, language, memory, calculation, and visuospatial/visuoconstructive abilities is reviewed.Results. Brain activity supporting the performance in these tests is usually quite extended, and involves not only those brain areas traditionally assumed in neuropsychology, but also other cortical and sometimes subcortical regions.Conclusions. Most neuropsychological tests are simultaneously evaluating different cognitive abilities associated with the activity of diverse brain areas. "Cognitive/anatomical" correlations could only be established for some relatively simple functions. This change in the understanding about the brain organization of cognition has not been reflected in the interpretation of the neuropsychological tests yet. The interpretation of neuropsychological tests should be based not only in clinical observations but also in functional studies. This is a necessary further step in clinical neuropsychology.

Acalculia in Aphasia.

BACKGROUND: Patients with aphasia can present a type of acalculia referred to as aphasic acalculia. AIMS: To investigate the correlation and to test regression models for one- and two-digit calculation skills using verbal and nonverbal predictors. METHODS AND PROCEDURES: We selected an aphasia sample of 119 men and 81 women with a mean age of 57.37 years (SD = 15.56) and an average level of education of 13.52 years (SD = 4.08). Spanish versions of the Western Aphasia Battery and Boston Diagnostic Aphasia Examination, plus a Written Calculation test, were individually administered. The calculation section of the Western Aphasia Battery and the Written Calculation tests were used to pinpoint calculation difficulties. OUTCOMES AND RESULTS: Calculation difficulties were more severe in Global and Mixed non-fluent aphasia; they were very similar in Broca, Conduction, and Amnesic Aphasia. All correlations between the two calculation subtests and the other subtests of the Western Aphasia Battery were statistically significant. Calculation subtests correlated negatively with age and positively with schooling. Sex and time post-onset did not show any correlation with the calculation scores. Education, Reading, Block Design, and Raven's Colored Progressive Matrices were significant predictors of Western Aphasia Battery Calculation. Writing was the only significant predictor of the Written Calculation scores. CONCLUSIONS: Nonverbal abilities were predictors of calculation tests, whereas agraphia defects were predictors of the Written Calculation test. Therefore, calculation abilities can be regarded both as written language-dependent and verbal language-independent.

Determination of Reference Values for Alpha-N-Acetylglucosaminidase Activities in Patients with Sanfilippo Type B Disease and Control Population in Colombia

Abstract Sanfilippo B is a lysosomal disorder characterized by the pathological accumulation of heparan sulfate. It is caused by mutations in the NAGLU gene that codes for the alpha-N-acetylglucosaminidase enzyme. The objective of this study was to determine the reference values and frequency of Sanfilippo B in Colombia through an enzyme analysis of leukocytes extracts. We aim to inform the community and the health system so that they can work in a preventive way, providing an early diagnosis of patients and thus providing an appropriate management of the symptoms. We carried out an endpoint assay that indirectly quantifies NAGLU activity through the cleavage of 4-methylumbelliferone from the 4-methylumbelliferyl-2-acetamido-2-deoxy-α-D-glucopyranoside substrate. The activity of 463 healthy volunteers (Range: 0.6 - 4 nmol/mg/h, Median: 1.69 +/- 0.73) as well as 462 patients referred for clinical suspicion, was calculated. From the last group, 7 cases turned out to be positive (Range: 0 - 0.24 nmol/mg/h, Median: 0.13 +/- 0.09). The cut-off point according to ROC analysis between affected patients and controls was 0.42 nmol/mg/h. To our knowledge, this study is the first in Colombia where an estimated frequency of Sanfilippo type B is calculated by providing enzyme activity ranges and a cut-off point.

A Longitudinal Study of Aphasia Due to Pure Sub-Cortical Strokes.

INTRODUCTION: Contemporary neuro-imaging techniques have significantly advanced our understanding of the brain organization of language and the involvement of subcortical areas in aphasia. However, articles on sub-cortical aphasia, particularly in non-western languages, remain to be few and far between. We set out to explore aphasia typology in sub-cortical strokes among Bengali-speaking population with a focus towards providing a longitudinal view over a period of 3 months post-stroke. METHODS: Bengali version of Western Aphasia Battery (BWAB) was used to assess and classify language dysfunction in our study participants. Conventional brain imaging techniques (CT scan & MRI) were used to detect and localize strokes. Uni-variate analysis for categorical variable (location versus aphasia typology) was performed using Chi square and Fischer's exact test (as applicable). Directional measures were calculated using lambda and Goodman-Kruskal tau (Range of - 1 to + 1). Boot strapping was applied while calculating the directional measures because of inadequate numbers in some sub-sections the sample. RESULTS: Frequency of sub-cortical aphasia was observed to be 29.80% (62/208) in the index study. Four location of strokes were associated with language dysfunction, of which putamen (53.23%) was the commonest followed by striato-capsular region (33.87%). Thalamus and peri-ventricular white matter (PVWM) strokes (6.45% each) were infrequent in our sample of sub-cortical aphasia. Global aphasia (30/62, 48.38%) was the most frequent type observed in acute phase while Broca's aphasia (23/53, 43.39%) dominated among the follow up cases. Aphasia recovery (with follow up AQ of 90.0 or more) was observed in 12 (22.64%) patients of whom majority (8/12) had striato-capsular strokes. CONCLUSION: The present paper illustrates the epidemiological aspects as well as longitudinal course aphasia following pure sub-cortical strokes.

Dyskinetopsic Palinopsia: Palinopsia Accompanied by Moving Afterimages.

Palinopsia refers to the abnormal persistence, or recurrence, of visual images after a visual stimulus has subsided. We describe here a case of palinopsia accompanied by a visual motion perception disorder as manifested by moving afterimages. A 71-year-old man presented to us after having experienced acute-onset, vivid, visual hallucinations for 1 week. A detailed history revealed that he was hallucinating multiple living and nonliving objects. He also complained of a persistence of afterimages, particularly in the left visual field. He reported that, on a few occasions, while sitting by the window in his room, he had seen a moving car on the road; immediately after the car had disappeared from his sight, he had then seen the same car moving backward at almost the same speed-as if the driver had applied the reverse gear. A neuropsychological assessment did not reveal any deficits in attention, language, or episodic memory. Visual field testing by confrontational perimetry suggested left hemianopia. An MRI of the brain revealed an arteriovenous malformation in the medial part of the right occipital lobe, affecting both the lingual gyrus and the inferior occipital gyrus. Palinopsia has generally been described in reference to static afterimages. In our case, not only was the afterimage that was perceived by the patient in motion, but the direction of the movement was also opposite to that of the actual object. We propose the term dyskinetopsic palinopsia, or simply motion-related palinopsia, for this particular condition.

Análisis de beta-glucosidasa en sangre seca recolectada en papel filtro (DBS), reporte de un nuevo método aplicado a población control y pacientes con sospecha de enfermedad de Gaucher / Beta-Glucosidase analysis in collected dried blood spots (DBS), report of a new method applied to the control population and patients with suspicion of Gaucher disease / Análise de β-glucosidase em sangue seco coletado em papel filtro (DBS), relatório de um novo método aplicado à população controle e a pacientes com suspeita de doença de Gaucher

Resumen: La enfermedad de Gaucher (GD) es el trastorno de almacenamiento lisosomal que se caracteriza por la deficiencia en la actividad enzimática de la β-glucosidasa (BGLU), lo que produce la acumulación de glucosilceramida en las células. Su diagnóstico se orienta a la valoración de la enzima en los leucocitos afectados. Se han realizado estudios en DBS para la actividad de BGLU en el seguimiento de poblaciones de alto riesgo; sin embargo, presentan interferencias relacionadas a leucopenias severas o expresión aumentada de la isoforma neutra de la enzima BGLU, molécula no relacionada con GD. El objetivo de este estudio fue la estandarización de un método de tamizaje en DBS (punch: 5 mm) con el uso de 4-metilumbeliferil-β-D-glucósido y conduritol-β-epóxido. Se analizaron muestras de DBS de 395 individuos con sospecha clínica (población de alto riesgo o AR), 151 controles y 16 pacientes afectados, usando la elución de un corte de 5 mm (≈10 μl de sangre) en 300 μl de Tritón X-100/(0,5 %). Como resultados, se obtuvieron los rangos, AR: 0,84-26,92 nmol/ml/h, controles: 3,56- 8,92 nmol/ml/h (M = 5,56, DS = 1,15) y pacientes confirmados con GD: 0,82- 2,88 nmol/ml/h (M = 1,64, DS = 0,57). El punto de corte entre deficientes y controles fue 3,22 nmol/ml/h, obtenido a partir de análisis ROC (99 % confianza, 100 % sensibilidad y 100 % especificidad). El protocolo permitió evidenciar la deficiencia en todos los casos de GD, confirmados mediante el análisis en paralelo de la enzima en aislamiento leucocitario. Se recomienda el uso del CBE y realizar la elución del corte a 5 mm, a fin de llevar a cabo la valoración enzimática con un volumen mayor aproximado de sangre y en ausencia de la actividad generada por la isoforma neutra.

Abstract: Gaucher disease (GD) is a lysosomal storage disorder characterized by a deficiency in the enzymatic activity of β-glucosidase (BGLU), resulting in the accumulation of glucosylceramide in cells. Its diagnosis is aimed at checking the enzyme in the affected leukocytes. Studies have been conducted on dried blood spots (DBS) for bglu activity to monitor high-risk populations; however, they exhibit interferences related to severe leukopenias or increased expression of the neutral bglu isoform, a molecule not related to gd. This study intends to standardize a screening method on dbs (punch: 5 mm) using 4-methylumbelliferyl-β-D-glucoside and conduritol-β-epoxide (CβE). dbs samples from 395 individuals clinically suspected of gd (high-risk or hr population), 151 controls, and 16 affected patients were analyzed using the elution of 5 mm punches (≈10 μl of blood) in 300 μl of Triton X-100/ (0.5 %). As a result, the following ranges were obtained; HR: 0.84-26.92 nmol/ml/h, controls: 3.56-8.92 nmol/ml/h (M = 5.56, SD = 1.15), and patients with confirmed GD: 0.82-2.88 nmol/ml/h (M = 1.64, SD = 0.57). The cut-off point between patients with gd and controls was 3.22 nmol/ml/h, obtained from roc analysis (99 % ci, 100 % sensitivity, and 100 % specificity). The protocol revealed a deficiency in all gd cases, confirmed by parallel bglu analysis in isolated leukocytes. The use of cbe and the elution of 5 mm punches are recommended for enzymatic evaluation with a higher approximate volume of blood and in the absence of neutral isoform activity.

Resumo: A doença de Gaucher (GD) é o trastorno de armazenamento lisosomal caracterizado pela deficiência na atividade enzimática da β-glucosidase (BGLU), o que produz a acumulação de glucossilceramida nas células. Seu diagnóstico está orientado à avaliação da enzima nos leucócitos afetados. Foram realizados estudos em dbs para a atividade de BGLU no seguimento de populações de alto risco; contudo, são apresentadas interferências relacionadas a leucopenias graves ou a expressão aumentada da isoforma neutra da enzima BGLU, molécula não relacionada com GD. O objetivo deste estudo foi a padronização de um método de tamisação emdbs (punch: 5 mm) com o uso de 4-metilumbeliferil-β-D- glicosídeo e conduritol-β-epóxido. Foram analisadas amostras de dbs de 395 indivíduos com suspeita clínica (população de alto risco ou ar), 151 controles e 16 pacientes afetados, usando a eluição de um corte de 5 mm (≈10 μl de sangue) em 300 μl de Tritão X-100/(0,5 %). Como resultados, foram obtidos os intervalos: AR: 0,84-26,92 nmol/ml/h, controles: 3,56-8,92 nmol/ml/h (M = 5,56, DS = 1,15) e pacientes confirmados com GD: 0,82- 2,88 nmol/ml/h (M = 1,64, DS = 0,57). O ponto de corte entre deficientes e controles foi 3,22 nmol/ml/h, obtido a partir de análise ROC (99 % confiança, 100 % sensibilidade e 100 % especificidade). O protocolo permitiu evidenciar a deficiência em todos os casos de GD, confirmados mediante a análise em paralelo da enzima em isolamento leucocitário. É recomendado o uso do cbe e a realização da eluição do corte a 5 mm, a fim de implementar a avaliação enzimática com um volume maior aproximado de sangue e em ausência da atividade gerada pela isoforma neutra.

Alexia and agraphia in Spanish.

BACKGROUND: Every language has certain specific idiosyncrasies in its writing system. Cross-linguistic analyses of alexias and agraphias are fundamental to understand commonalities and differences in the brain organization of written language. Few reports of alexias and agraphias in the Spanish language are currently available. AIMS: To analyse the clinical manifestations of alexias and agraphias in Spanish, and the effect of demographic variables. METHODS & PROCEDURES: Spanish versions of the Western Aphasia Battery (WAB) and Boston Diagnostic Aphasia Examination (BDAE) were used for language assessment. Lesion localization was obtained by using computed axial tomography and magnetic resonance imaging. The final sample included 200 patients: 195 (97.5%) right-handed and five (2.5%) left-handed; 119 men and 81 women with a mean age of 57.37 years (SD = 15.56), education of 13.52 years (SD = 4.08), and mean time post-onset of 6.58 months (SD = 12.94). Using the WAB, four quotients were calculated: aphasia quotient (AQ), reading-writing quotient (RWQ), language quotient (LQ) and cortical quotient (CQ). OUTCOMES & RESULTS: The types of aphasia were: global = 11 patients (5.5%), Broca = 31 (15.5%), Wernicke = 30 (15.0%), conduction = 22 (11.0%), transcortical sensory = 17 (8.5%), transcortical motor = 3 (1.5%), amnesic or anomic = 54 (27.0%) and mixed non-fluent = 32 (16.0%). The degree of oral and written language impairment differed across the various aphasia types. Most severe reading and writing difficulties were found in global, mixed non-fluent and transcortical motor aphasia; fewer difficulties were observed in amnesic, Broca and conduction aphasia. The severity of the written language impairments paralleled the severity of the oral language disturbances. Age negatively, while schooling positively, correlated with the scores in reading and writing tests. No effect of sex and time since onset was found. CONCLUSIONS & IMPLICATIONS: In Spanish-speaking aphasia patients, difficulties in reading and writing are similar to oral language difficulties. This similarity of performance is mostly based on severity rather than the participants' patterns of errors. What this paper adds What is already known on the subject There is limited information about alexia and agraphia in Spanish. What this paper adds to existing knowledge An extensive study with a large sample of patients. What are the potential or actual clinical implications of this work? The study contributes to the clinical management of patients with reading and writing disturbances.

Gerstmann Syndrome.

PURPOSE OF REVIEW: Gerstmann (left angular gyrus) syndrome includes the tetrad of finger agnosia (inability to distinguish, name, and recognize the fingers), agraphia (acquired disturbance in the ability to write), acalculia (loss of the ability to perform arithmetical operations and use numerical concepts), and right-left disorientation (right-left discrimination defect when using language). There is some disagreement regarding its exact localization, but it most likely involves the left angular gyrus with a probable subcortical extension. This article reviews recent research on the clinical aspects of this syndrome. RECENT FINDINGS: During the last years, just some few new reports of Gerstmann syndrome are found in neurological and neuropsychological literature. Most of the reports are single-case reports. An association between Gerstmann syndrome and the so-called semantic aphasia has been pointed out. Two different explanations to this unusual syndrome have been recently proposed: (1) the pathological process is located in the left parietal white matter disconnecting separate cortical networks and (2) it represents a disturbance in the ability to verbally mediate some spatial knowledge. Although Gerstmann syndrome continues as a controversial syndrome, and most of the reports are single case reports, recently two different explanations have been advance the understanding of this polemic but fascinating syndrome.

COVID-19 Pandemic: A Neurological Perspective.

Even though severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) has been observed to principally affect the respiratory system, neurological involvements have already been reported in some published work. We have reviewed original articles, case reports, and existing open-source data-sets to delineate the spectrum of neurological disorders potentially observed in SARS-CoV-2 positive cases. Neurological involvement in coronavirus disease 2019 (COVID-19) corresponds to three situations: (a) neurological manifestations of viral infection, (b) post-infective neurological complications, and (c) infection in patients with neurological co-morbidity. Neurological manifestations can further be subdivided into the central nervous system (headache, dizziness, alteration of the sensorium, ataxia encephalitis, stroke, and seizures) and peripheral nervous system (skeletal muscle injury and peripheral nerve involvement including hyposmia and hypogeusia) symptomatology. Post-infective neurological complications include demyelinating conditions. Reduced mobility and dementia as co-morbidities may predispose a patient to have a viral infection. It is concluded that the pandemic of COVID-19 presents for a neurologist some unique challenges. We observe that SARS-CoV-2 may have various neurological manifestations and in many cases, neurological features may precede typical respiratory symptoms.

A perspective on research, diagnosis, and management of lysosomal storage disorders in Colombia.

Lysosomal storage diseases (LSDs) are a group of about 50 inborn errors of metabolism characterized by the lysosomal accumulation of partially or non-degraded molecules due to mutations in proteins involved in the degradation of macromolecules, transport, lysosomal biogenesis or modulators of lysosomal environment. Significant advances have been achieved in the diagnosis, management, and treatment of LSDs patients. In terms of approved therapies, these include enzyme replacement therapy (ERT), substrate reduction therapy, hematopoietic stem cell transplantation, and pharmacological chaperone therapy. In this review, we summarize the Colombian experience in LSDs thorough the evidence published. We identified 113 articles published between 1995 and 2019 that included Colombian researchers or physicians, and which were mainly focused in Mucopolysaccharidoses, Pompe disease, Gaucher disease, Fabry disease, and Tay-Sachs and Sandhoff diseases. Most of these articles focused on basic research, clinical cases, and mutation reports. Noteworthy, implementation of the enzyme assay in dried blood samples, led to a 5-fold increase in the identification of LSD patients, suggesting that these disorders still remain undiagnosed in the country. We consider that the information presented in this review will contribute to the knowledge of a broad spectrum of LSDs in Colombia and will also contribute to the development of public policies and the identification of research opportunities.

Analysis of an Unusual Case of Nonfluent Aphasia With Predominantly Posterior Perisylvian Lesion: An Apparent Paradox.

Lesion site-aphasia type discordance has garnered increasing interest in cognitive neuroscience over the last century. Diaschisis, the network concept of cognitive functions, and interindividual variability are among the plausible explanations cited in the literature for such unusual clinical cases. We describe here the case of a nonfluent type of aphasia following an ischemic stroke predominantly affecting the left posterior perisylvian cortex in a right-handed Bengali-speaking woman. The patient's comprehension was well preserved; however, she presented with a severe motor speech defect. MRI revealed an ischemic lesion in the left parietotemporal area, with slight involvement of the postero-inferior frontal cortex. We suggest two plausible explanations for this lesion-aphasia discordance: Our patient had bilateral representation of language receptive functions in her brain, and additional areas neighboring the classical Broca area may support some critical mechanisms of speech production. Taken together, these explanations may clarify why our patient was able to maintain the ability to decode language even though her language production was significantly affected.

Hallucinatory palinopsia and paroxysmal oscillopsia as initial manifestations of sporadic Creutzfeldt-Jakob disease: A case study.

BACKGROUND: Heidenhain variant of Cruetzfeldt Jacob Disease is a rare phenotype of the disease. Early and isolated visual symptoms characterize this particular variant of CJD. Other typical symptoms pertaining to muti-axial neurological involvement usually appear in following weeks to months. Commonly reported visual difficulties in Heidenhain variant are visual dimness, restricted field of vision, agnosias and spatial difficulties. We report here a case of Heidenhain variant that presented with very unusual symptoms of palinopsia and oscillopsia. CASE PRESENTATION: A 62-year-old male patient presented with symptoms of prolonged afterimages following removal of visual stimulus. It was later on accompanied by intermittent sense of unstable visual scene. He underwent surgery in suspicion of cataratcogenous vision loss but with no improvement in symptoms. Additionally he developed symptoms of cerebellar ataxia, cognitive decline and multifocal myoclonus in subsequent weeks. On the basis of suggestive MRI findings in brain, typical EEG changes and a positive result of 14-3-3 protein in CSF, he was eventually diagnosed as sCJD. CONCLUSION: This case adds to the tally of handful reports of Heidenhain variant CJD in literature, particularly from India. Two atypical initial symptoms, namely hallucinatory palinopsia and paroxysmal oscillopsia were observed in the index case. Possible explanations of such phenomena in CJD have been explored in light of the available studies.

Age, executive functioning, and decision-making styles in adults: a moderated mediation model.

The current study aimed to assess: i) whether executive functioning (EF) mediates the association of age with different decision-making (DM) styles in adults, and ii) whether these mediational associations change with age in adulthood. Our sample included 195 adults (110 young adults and 85 middle-aged adults; 95 males) selected from different government, semi-government, and private sector organizations. They were assessed on a self-report measure of General Decision-making Styles and on two EF tests: the Design Fluency Test and the Color-Word Interference Test from the Delis-Kaplan Executive Functions System. Results indicated that EF mediated the association of age with three decision-making styles including dependent, avoidant, and spontaneous DM. However, a conditional indirect effect of EF was significant only for spontaneous DM, indicating stronger indirect effects for middle-aged adults than for young adults. The findings highlight the idea that EF is an important factor in DM, particularly during middle adulthood.

A cross-linguistic comparison of category verbal fluency test (ANIMALS): a systematic review.

BACKGROUND: Cross-linguistic information about performance in neuropsychological verbal tests is extremely scarce. It has been suggested that verbal fluency test using animal fluency test is one of the few tests fulfilling the fundamental criteria desirable in a robust neuropsychological test. OBJECTIVE: To compare and establish cross-linguistic information about performance in the animal fluency test. RESULTS: In an extensive search, it was found that norms for the semantic fluency test using the category ANIMALS are available in 15 different languages. These languages represent a relatively broad spectrum of world languages, including not only Indo-European languages, but also Semitic, Sino-Tibetan, Austroasiatic, Dravidian, and even Amerindian languages. Normative data in these 15 languages are analyzed and the results are compared. CONCLUSIONS: It is concluded that (a) pure linguistic factors, such as type of language and word-length, seemingly do not significantly affect the performance in this test; (b) two major demographic variables-age and education-account for a significant percentage of the variance in this test; the effect of sex seems to be neglectable; (c) in bilinguals, when the native language has few speakers and/or is a marginalized language, a very low score can be observed in the native language, even lower than in the second language; (d) there is a frequently overlooked major factor accounting for differences in cognitive test performance: the effort made in performing the test. It depends on the significance given to the test performance. Effort is quite variable across cultures and is higher in psychometric-oriented societies.