Generalized anosognosia, anosodiaphoria, and visual hallucinations with bilateral enucleation after severe bifrontal brain injury: a case report describing similarities with and differences from Anton syndrome.

Visual anosognosia, associated with confabulations and cortical blindness in the context of occipital lobe injury, is known as Anton syndrome. Patients with this syndrome strongly deny their vision loss and confabulate to compensate for both visual loss and memory impairments. In this article, we present a case of a patient with some similarities to Anton syndrome, however, with several differences in clinical presentation. Bifrontal brain injury, bilateral enucleation, affective indifference (anosodiaphoria), generalized anosognosia, and the conviction that vision will resume mark clear clinical differences with Anton syndrome. Differentiating these findings from Anton syndrome will help occupational therapists, neuropsychologists, speech-language pathologists, physical therapists, and physicians when assessing frontal lobe brain injury with total and partial visual loss. This case demonstrates that visual anosognosia and confabulations can occur without occipital lobe dysfunction or cortical blindness.

Insights into Anton Syndrome: When the brain denies blindness.

INTRODUCTION: Anosognosia, a neurological condition, involves a lack of awareness of one's neurological or psychiatric deficits. Anton Syndrome (AS), an unusual form of anosognosia, manifests as bilateral vision loss coupled with denial of blindness. This systematic review delves into 64 studies encompassing 72 AS cases to explore demographics, clinical presentations, treatments, and outcomes. MATERIALS AND METHODS: The study rigorously followed PRISMA guidelines, screening PubMed, Google Scholar, and Scopus databases without timeframe limitations. Only English human studies providing full text were included. Data underwent thorough assessment, examining patient demographics, etiological variables, and treatment modalities. RESULTS: Sixty-four studies met the stringent inclusion criteria. Examining 72 AS cases showed a median age of 55 (6 to 96 years) with no gender preference. Hypertension (34.7 %) and visual anosognosia (90.3 %) were prevalent. Stroke (40.3 %) topped causes. Management included supportive (30.6 %) and causal approaches (30.6 %). Improvement was seen in 45.8 %, unchanged in 22.2 %, and deterioration in 11.1 %. Anticoagulation correlated with higher mortality (p < 0.05). DISCUSSION: AS, an unusual manifestation of blindness, stems mainly from occipital lobe damage, often due to cerebrovascular incidents. The syndrome shares features with Dide-Botcazo syndrome and dates back to Roman times. Its causes range from strokes to rare conditions like multiple sclerosis exacerbation. Accurate diagnosis involves considering clinical presentations and imaging studies, distinguishing AS from similar conditions. CONCLUSION: This comprehensive review sheds light on AS's complex landscape, emphasizing diverse etiologies, clinical features, and treatment options. Tailored treatments aligned with individual causes are crucial. The study's findings caution against blanket anticoagulation therapy, suggesting a nuanced approach. Further research is pivotal to refine diagnostics and optimize care for AS individuals.

Afasia progresiva fluente: ¿una forma de presentación inicial de demencia semántica? / Fluent progressive aphasia: a form of inicial presentation of semantic dementia?

Se presenta el caso de un hombre de 57 años que presenta un cuadro degenerativo con afasia fluente y moderadas alteraciones de conducta, sin defecto semántico significativo, agnosia visual ni agrafia lexical y su estudio imagenológico cerebral. Se discuten los conceptos de afasia progresiva fluente y demencia semántica, concluyendo que la primera puede evolucionar hacia esta última. Nuestro caso se encontraría en esta situación. Se señala que la patología de las afasias progresivas es variable, siendo lo más frecuente una degeneración frontotemporal (con o sin inclusiones de proteína tau); con menor frecuencia corresponde a una enfermedad de Alzheimer o una degeneración corticobasal.

We report a 57-year-old man presenting with progressive fluent aphasia and behaviour disorder, with no visual semantic loss, visual agnosia, lexical agraphia, nor alexia. We include in this report the brain images, and we discuss the borders between fluent progressive aphasia and semantic dementia. We conclude that fluent progressive aphasia may be the first stage of semantic dementia, noting that the neuropathology of progressive fluent aphasia usually corresponds to frontotemporal lobar degeneration, with or without Tau protein inclusions, as in Alzheimer's Disease or corticobasal degeneration.

Un caso de agnosia visual aperceptiva de tipo integrativa / A case of apperceptive visual agnosia showing an integrative disorder

Presentamos un paciente (C.A.) de 38 años afectado de alteraciones en el reconocimiento visual secundarias a una lesión isquémica cerebral posterior bilateral (RNM). En el examen neuropsicológico encontramos dificultades en reconocer visualmente rostros, objetos y figuras de objetos, a pesar de que los puede ver, dibujar y describir. El reconocimiento táctil y auditivo fue normal. Nuestro paciente tiene una prosopoagnosia, una agnosia visual de objetos, una simultagnosia, una agnosia topográfica y una amnesia episódica global. El defecto en el reconocimiento de objetos y de rostros no responde sólo a un trastorno perceptivo (representación formal). El conocimiento semántico de objetos y rostros es correcto pues puede evocar verbalmente la imagen eidética de aquellos que no puede reconocer (representación semántica). El paciente falla en el estadio de integración jerarquización de las partes necesarias para el correcto apareamaiento entre la imagen visual que ingresa con la imagen eidética del conocimiento semántico correspondiendo a una agnosia visual de tipo integrativa

Agnosia auditiva surdez pura para palavras afasia de Wernicke sem alexia: aspectos clínicos e conceituais / Auditory agnosia, pure word deafness, Wernicke's aphasia without alexia: clinical and concepetial aspects

A agnosia auditiva e sindromes correlatas podem ter seu diagnóstico confundido com o das afasias. Descrevemos o caso de uma mulher de 32 anos que subitamente perdeu a capacidade de compreender a palavra falada e sons näo verbais,, embora a leitura e a escrita estivessem intactas e a fala espontânea fosse apenas muito discretamente disfásica. A investigaçäo mostrou isquemia extensa e bilateral do córtex temporal, secundária a anticoagulaçäo inadequada, após colocaçäo de prótese aórtica. O segundo paciente é um homem de 43 anos com súbita dificuldade para compreender a palavra falada, estando preservadas a compreensäo de sons näo verbais, leitura e escrita. A nosso ver, o primeiro caso corresponde à agnosia auditiva e o segundo à afasia de Wernicke sem alexia. Säo discutidos aspectos do diagnóstico diferencial e prognóstico das diferentes síndromes