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.

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.

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.

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.

Incidence and Symptomatology of Vascular Crossed Aphasia in Bengali.

BACKGROUND: Crossed aphasia (CA) refers to aphasia following a right-hemispheric lesion in right-handed individuals. It has been suggested that the prevalence of CA differs with language, although its worldwide incidence, as reported by most studies, is less than 3%. OBJECTIVE: To find the incidence of CA in the Bengali language. METHODS: From 2016 to 2018, in a hospital located in a Bengali-speaking area of eastern India, 515 cases of first-ever stroke were documented, out of which 208 patients presented with aphasia (40.38%) according to their scores on the Bengali version of the Western Aphasia Battery. RESULTS: Among the patients with aphasia, 14 (6.73%; 8 men and 6 women) presented with CA. Of these, 10 were diagnosed with Broca aphasia and four with transcortical motor aphasia. No patient presented with Wernicke aphasia. CONCLUSIONS: The relatively high incidence of CA in our study suggests that bi-hemispheric language representation may be more prevalent in Bengali speakers than in speakers of other languages. The absence of crossed Wernicke aphasia in our study participants may represent a left-hemispheric advantage for receptive language abilities in Bengali speakers. Further studies are required to clarify whether idiosyncrasies in the Bengali language may be responsible for the differential brain representation of language seen in our study participants.

Cognitive Control and Criminogenic Cognitions in South Asian Gamblers.

It has been suggested that criminogenic thinking is likely to be a correlate of gambling. The primary aim of the current study was to assess whether gamblers and non-gamblers differ on measures of cognitive control and criminogenic cognitions. The secondary aim was to assess the association between cognitive control and criminogenic cognitions amongst gamblers and non-gamblers. The sample included 159 male participants (78 gamblers and 81 non-gamblers) with an age range from 15 to 25 years (M = 20.07, SD = 2.77), recruited from different snooker clubs in Lahore, Pakistan. Participants were individually administered color word interference test (CWIT) taken from Delis Kaplan executive function system, Lie Bet Questionnaire, and Criminogenic Cognition Scale in order to assess cognitive control, gambling, and criminogenic thinking patterns respectively. Raw scores of completion time and number of errors on four conditions of CWIT were taken as the measures of cognitive control. Results from repeated measure ANOVA indicated that gamblers committed significantly more errors on cognitive control measure and scored significantly higher on two criminogenic cognition subscales including notion of entitlement and insensitivity to the impact of crime. Furthermore, age and education were also found to be significant covariates of specific criminogenic cognitions and cognitive control respectively. Results from correlational analyses showed that error measure, but not the time measure, of cognitive control was significantly and positively correlated with short term orientation, notion of entitlement, negative attitude toward authority, and insensitivity to the impact of crime. Implications of the findings are suggested along with future directions.

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.

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.

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.

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.

Patterns of stuttering in a Spanish/English bilingual: A case report.

Stuttering patterns may differ when comparing two languages. In bilinguals, specific patterns of stuttering in each one of the languages may potentially be found. This study reports on the case of a 27-year-old Spanish/English simultaneous bilingual whose dominant language is English. Speech and language testing was performed in both languages (language repetition, language understanding, vocabulary, reading, verbal fluency, spontaneous speech and conversational speech). Some differences in the stuttering distribution were found: stuttering in adjectives, adverbs and conjunctions occurred at least twice as much in Spanish as in English; stuttering was also more frequent in verbs in Spanish. Some hypotheses are presented to explain the differences in stuttering severity in both languages. It seems that when comparing stuttering in two different languages in the same subject, it is possible to find similarities in the stuttering pattern, suggesting general stuttering laws; but also differences, associated not only with language-specific idiosyncrasies but also with the individual's bilingualism characteristics.

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.

A review of conduction aphasia.

In this paper, a historical overview of the interpretation of conduction aphasia is initially presented. It is emphasized that the name conduction aphasia was proposed by Wernicke and was interpreted as a disconnection between the temporal and frontal brain language areas; this interpretation was re-taken by Geschwind, attributing the arcuate fasciculus the main role in speech repetition disturbances and resulting in the so-called Wernicke-Geschwind model of language. With the introduction of contemporary neuroimaging techniques, this interpretation of conduction aphasia as a disconnection syndrome due to an impairment of the arcuate fasciculus has been challenged. It has been disclosed that the arcuate fasciculus does not really connect Wernicke's and Broca's areas, but Wernicke's and motor/premotor frontal areas. Furthermore, conduction aphasia can be found in cases of cortical damage without subcortical extension. It is concluded that conduction aphasia remains a controversial topic not only from the theoretic point of view, but also from the understanding of its neurologic foundations.

The role of the arcuate fasciculus in conduction aphasia.

In aphasia literature, it has been considered that a speech repetition defect represents the main constituent of conduction aphasia. Conduction aphasia has frequently been interpreted as a language impairment due to lesions of the arcuate fasciculus (AF) that disconnect receptive language areas from expressive ones. Modern neuroradiological studies suggest that the AF connects posterior receptive areas with premotor/motor areas, and not with Broca's area. Some clinical and neurophysiological findings challenge the role of the AF in language transferring. Unusual cases of inter-hemispheric dissociation of language lateralization (e.g. Broca's area in the left, and Wernicke's area in the right hemisphere) have been reported without evident repetition defects; electrocortical studies have found that the AF not only transmits information from temporal to frontal areas, but also in the opposite direction; transferring of speech information from the temporal to the frontal lobe utilizes two different streams and conduction aphasia can be found in cases of cortical damage without subcortical extension. Taken altogether, these findings may suggest that the AF is not required for repetition although could have a subsidiary role in it. A new language network model is proposed, emphasizing that the AF connects posterior brain areas with Broca's area via a relay station in the premotor/motor areas.

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.

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.

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.

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.