Síndrome de Anton-Babinsky / Anton-Babinski Syndrome

Introducción: El síndrome de Anton-Babinsky es un trastorno neuropsiquiátrico poco frecuente, que se manifiesta por anosognosia y ceguera cortical, debido a lesiones en las áreas visuales asociativas de la corteza occipital sin presentar afectación en la vía visual. En adultos mayores sus manifestaciones clínicas suelen ser atípicas y la valoración geriátrica integral permite orientar el diagnóstico, que se puede asociar con síndromes geriátricos. Objetivo: Describir las manifestaciones clínicas, síndromes geriátricos, paraclínicos y tratamiento de un paciente con Síndrome de Anton-Babinsky. Caso clínico: Paciente de 85 años, quien durante un postoperatorio inmediato de cirugía ocular (pterigión) presentó alteración fluctuante del estado de conciencia, alucinaciones visuales, disminución de la agudeza visual bilateral y anosognosia. La analítica sanguínea no reportó alteraciones y la tomografía computarizada de cráneo documentó isquemia occipital bilateral, se le diagnosticó síndrome de Anton-Babinsky. Conclusiones: El síndrome de Anton-Babinsky puede tener presentación atípica a través de síndromes geriátricos. La valoración geriátrica integral permite realizar un diagnóstico y manejo multicomponente oportuno con el objetivo de influir en el pronóstico tanto a corto como a largo plazo(AU)

Introduction: Anton-Babinski syndrome is a rare neuropsychiatric disorder, with a manifestation of anosognosia and cortical blindness, due to lesions in the associative visual areas of the occipital cortex without presenting visual pathway impairment. In elderly adults, its clinical manifestations are usually atypical and a comprehensive geriatric assessment allows to guide the diagnosis, which can be associated with geriatric syndromes. Objective: To describe the clinical manifestations, geriatric syndromes, paraclinical findings and treatment of a patient with Anton-Babinski syndrome. Clinical case: This is the case of an 85-year-old patient who, during the immediate postoperative period after ocular surgery (pterygium), presented a fluctuating alteration of consciousness, visual hallucinations, decreased bilateral visual acuity and anosognosia. Blood analysis reported no alterations and cranial computed tomography documented bilateral occipital ischemia; the patient was diagnosed with Anton-Babinski syndrome. Conclusions: Anton-Babinski syndrome may have an atypical presentation through geriatric syndromes. Comprehensive geriatric assessment allows for timely multicomponent diagnosis and management with the aim of influencing both short- and long-term prognosis(AU)

Visual loss as first clinical manifestation of X-linked adrenoleukodystrophy / Perda visual como primeira manifestação clínica de adrenoleucodistrofia ligada ao X

Abstract X-linked adrenoleukodystrophy (X-ALD) represents a group of diseases characterized by the accumulation of very long chain fattyacids (VLCFAs) in the tissues. Its clinical manifestations are usually manifold. Visual changes may be present, but they often appear later in the disease. We describe here the case of a 9-year-old boy with X-ALD, whose first symptom was visual loss, which began at 8 years of age. His ophthalmologic evaluation revealed no alterations. Shortly thereafter, he suffered a head injury. The magnetic resonance imaging of brain revealed findings that led to the suspicion of X-ALD. The plasma VLCFA dosage confirmed this diagnosis. This report aims toshow that in cases of visual loss with a normal ophthalmic examination, a high index of suspicion should be given for conditions suchas X-ALD, since it affects the cortical routes related to vision. Fundoscopy findings appear late in X-ALD.

Resumo A adrenoleucodistrofia ligada ao X (X-ALD) representa um grupo de doenças caracterizadas pelo acúmulo de ácidos graxos de cadeia muito longa (VLCFAs) nos tecidos. Suas manifestações clínicas costumam ser múltiplas. Alterações visuais podem estar presentes, contudo costumam surgir mais tardiamente na doença. Descrevemos aqui o caso de um menino de 9 anos com X-ALD, cujo primeiro sintoma foi perda visual, iniciada aos 8 anos de idade. A sua avaliação oftalmológica não revelou alterações. Pouco tempo depois, ele sofreu um traumatismo craniano. A imagem de ressonância magnética de encéfalo revelou achados que levaram a suspeita de X-ALD. A dosagem dos VLCFAs no plasma confirmou este diagnóstico. Este relato tem como objetivo mostrar que em casos de perda visual com um exame oftalmológico normal, deve-se ter um alto índice de suspeita para condições como a X-ALD, pois a mesma afeta as vias corticais relacionadas à visão. Nessa doença, os achados da fundoscopia aparecem mais tardiamente.

Ceguera cortical como manifestación inicial de endocarditis trombótica no bacteriana / Cortical blindness as initial manifestation of nonbacterial thrombotic endocarditis

La endocarditis trombótica no bacteriana, antiguamente conocida como endocarditis marántica, es una entidad infrecuente en la que se desarrollan vegetaciones estériles, compuestas por fibrina en las válvulas del corazón. Suele diagnosticarse en el momento de la autopsia o en enfermedades oncológicas avanzadas. Las neoplasias malignas más frecuentemente asociadas con esta entidad son las de pulmón, páncreas, estómago y adenocarcinomas de origen primario desconocido. Es necesario descartar la endocarditis infecciosa y establecer la presencia de vegetaciones valvulares mediante ecocardiografía. Presentamos el caso de una paciente con diagnóstico reciente de adenocarcinoma de estómago en estadio avanzado que presentó ceguera cortical e imágenes compatibles con isquemia cerebral. El ecocardiograma transesofágico mostró dos vegetaciones en válvula mitral. Los hemocultivos fueron negativos. Se enfatiza la importancia de sospechar endocarditis trombótica no bacteriana en enfermos con cáncer y embolismo sistémico.

Nonbacterial thrombotic endocarditis, formerly known as marantic endocarditis, it is an infrequent entity in which sterile, fibrin vegetations develop on heart valve leaflets. It is often diagnosed at the time of autopsy or in late-stage malignancies. The most common malignancies associated with nonbacterial thrombotic endocarditis are lung, pancreatic, gastric cancer and adenocarcinomas of an unknown primary site. Diagnosis requires ruling out infective endocarditis and establishing the presence of valvular vegetations using echocardiography. We report the case of a patient with a recent diagnosis of advanced gastric adenocarcinoma who presented with cortical blindness. The computed tomography was compatible with cerebral ischemia. The transoesophageal echocardiogram showed two vegetations in mitral valve. Blood cultures were negative. We emphasize the importance of suspecting nonbacterial thrombotic endocarditis in patients with cancer and systemic embolism.

[Cortical blindness as initial manifestation of nonbacterial thrombotic endocarditis]. / Ceguera cortical como manifestación inicial de endocarditis trombótica no bacteriana.

Nonbacterial thrombotic endocarditis, formerly known as marantic endocarditis, it is an infrequent entity in which sterile, fibrin vegetations develop on heart valve leaflets. It is often diagnosed at the time of autopsy or in latestage malignancies. The most common malignancies associated with nonbacterial thrombotic endocarditis are lung, pancreatic, gastric cancer and adenocarcinomas of an unknown primary site. Diagnosis requires ruling out infective endocarditis and establishing the presence of valvular vegetations using echocardiography. We report the case of a patient with a recent diagnosis of advanced gastric adenocarcinoma who presented with cortical blindness. The computed tomography was compatible with cerebral ischemia. The transoesophageal echocardiogram showed two vegetations in mitral valve. Blood cultures were negative. We emphasize the importance of suspecting nonbacterial thrombotic endocarditis in patients with cancer and systemic embolism.

Seneca and the First Description of Anton Syndrome.

Seneca was a Roman philosopher, politician, and mentor to the young Nero. He later fell in disgrace and was sentenced to death by the Emperor. Seneca left many texts, one of the most influential being his Moral Letters to Lucilius (63 CE). In Letter 50, he describes the case of Harpaste, his wife's foolish slave who acutely became blind. She denied her illness and argued irrationally about room darkness, constantly asking attendants to change her quarters. Harpaste's case, consisting of acutely acquired blindness and anosognosia in the presence of relatively well-preserved cognition, fulfills the clinical criteria for the diagnosis of Anton syndrome, and probably constitutes its first description.

Cortical blindness secondary to posterior reversible encephalopathy syndrome, recovered by successful blood pressure management.

We report a case of cortical blindness secondary to posterior leukoencephalopathy syndrome, which was successfully treated by blood pressure management. A 66-year-old white man presented at the emergency room complaining of severe frontal headache, disorientation, and progressive blurred vision. The initial physical examination disclosed a blood pressure of 200/176 mmHg. One hour later the patient exhibited mental confusion and bilateral blindness. Computed tomography and magnetic resonance imaging were performed, and a systemic workup was conducted, resulting in a diagnosis of posterior leukoencephalopathy syndrome. This syndrome is a rare manifestation of systemic hypertension that requires proper diagnosis and management to avoid irreversible brain damage. Early recognition of this condition and prompt control of the patient's blood pressure are essential because they may bring about a reversal of the syndrome, which may otherwise result in permanent brain damage.

Cortical blindness secondary to posterior reversible encephalopathy syndrome, recovered by successful blood pressure management / Cegueira cortical secundária à síndrome de encefalopatia reversível posterior recuperada pelo controle bem sucedido da pressão arterial

ABSTRACT We report a case of cortical blindness secondary to posterior leukoencephalopathy syndrome, which was successfully treated by blood pressure management. A 66-year-old white man presented at the emergency room complaining of severe frontal headache, disorientation, and progressive blurred vision. The initial physical examination disclosed a blood pressure of 200/176 mmHg. One hour later the patient exhibited mental confusion and bilateral blindness. Computed tomography and magnetic resonance imaging were performed, and a systemic workup was conducted, resulting in a diagnosis of posterior leukoencephalopathy syndrome. This syndrome is a rare manifestation of systemic hypertension that requires proper diagnosis and management to avoid irreversible brain damage. Early recognition of this condition and prompt control of the patient's blood pressure are essential because they may bring about a reversal of the syndrome, which may otherwise result in permanent brain damage.

RESUMO Relatamos um caso de cegueira cortical secundário à síndrome de leucoencefalopatia posterior recuperado pelo controle bem sucedido da pressão arterial. Um homem branco de 66 anos de idade compareceu à emergência com queixa de dor de cabeça frontal severa, desorientação e embaçamento progressivo da visão. O exame físico inicial revelou uma pressão arterial de 200/176 mmHg. Uma hora depois, o paciente apresentou confusão mental e cegueira bilateral. Após a tomografia e a ressonância nuclear magnética, o tratamento sistêmico foram realizados e o diagnóstico de síndrome da leucoencefalopatia posterior foi realizado. A síndrome de leucoencefalopatia posterior é uma manifestação rara de hipertensão sistêmica que requer um diagnóstico e gerenciamento adequados para evitar danos cerebrais irreversíveis. O reconhecimento precoce desta condição e o controle imediato da pressão arterial são essenciais porque podem levar à reversão da síndrome, o que, de outra forma, pode resultar em dano cerebral permanente.

Amaurosis cortical aguda secundaria a preeclampsia / Acute cortical blindness secondary to preeclampsia

La amaurosis cortical aguda es una complicación dramática y poco común de la preeclampsia. Se presenta un caso de paciente de 29 años con diagnóstico de preeclampsia grave quien describió deterioro de la agudeza visual repentino en el puerperio inmediato. El fondo de ojo fue normal. La paciente identificaba la luz intensa. Las pupilas estaban reactivas y no se observo la presencia de nistagmo. Se le realizó una resonancia magnética cuyos resultados fueron normales, por lo que se realizó el diagnóstico de amaurosis cortical aguda.

Acute cortical blindness is an uncommon and dramatic complication of preeclampsia. We present a case of a 29 years-old patient with diagnosis of severe preeclampsia who described a sudden loss of visual acuity during immediate puerperium. Fundi were normal. Pupils were reactive and there was no nystagmus A magnetic resonance were performed with normal results, because diagnosis of acute cortical blindness was done.

Transient cortical blindness post angiography--a case report.

A 56-year old female reported having had a fall two weeks prior to presentation. Computed Tomography (CT) scan showed an acute right-sided convexity subdural haematoma. A computed tomography angiogram revealed no vascular anomaly. One hour post procedure she had bilateral cortical blindness. Her vision subsequently was fully restored. A diagnosis of transient cortical blindness was made. Transient cortical blindness is a rare but recognized complication ofintra-arterial injection of iodinated contrast agents.

Síndrome de Anton, el ciego que cree ver / Anton syndrome

Las manifestaciones clínicas del ACV son muy variables, no sólo la clásica hemiplejia facio-braquio-crural, déficit sensitivo contralateral y afasia, sino que también puede presentarse, en menos frecuencia, por déficit neurológicos de variada índole, tal es el caso del síndrome de Anton, en que debido a un afección del territorio cortical visual primario bilateral (área 17 de Brodmann) y al área de asociación vecina, caracterizado por ceguera cortical y anosognosia. Comunicamos el caso de un hombre que presenta este síndrome, que debido a dos ACVs, uno antiguo y otro reciente, cuyo diagnóstico fue confirmado por TAC de cerebro.

Clinical manifestations of stroke are highly variable, not only the classic hemiplegia facio-brachio-crural, contralateral sensory loss and aphasia, but can also occur, less frequently, neurological deficits of various kinds, as in the case of the syndrome in Anton, a condition that due to the territory bilateral primary visual cortex (Brodmann area 17) and the neighboring association area, characterized by cortical blindness and anosognosia. We report the case of a man who has this syndrome, due to two LCAs, one old and one recent, whose diagnosis was confirmed by brain CT.

Idiopathic childhood occipital epilepsy of Gastaut: a review and differentiation from migraine and other epilepsies.

The purpose of this review is to provide guidance for appropriate diagnosis and management of idiopathic childhood occipital epilepsy of Gastaut. The typical clinical features are visual seizures that typically consist of brief elementary visual hallucinations, which are mainly multicolored and circular. Ictal blindness and deviation of the eyes are also common symptoms. The seizures are usually frequent and diurnal. The electroencephalography is the only investigation with abnormal results, showing occipital spikes and often occipital paroxysms demonstrating fixation-off sensitivity. Brain magnetic resonance imaging is used to exclude symptomatic occipital epilepsy. Patients usually respond well to antiepileptic medication and about two-thirds remit by the age of 16 years. Idiopathic childhood occipital epilepsy of Gastaut is frequently misdiagnosed as migraine with visual aura, acephalgic, or basilar migraine. Differentiation from symptomatic occipital epilepsy, particularly when children are otherwise normal, can be difficult. Most children need prophylactic antiepileptic medication.

Causes of visual impairment in children: a study of 3,210 cases.

PURPOSE: To determine causes of visual impairment in children at the Low Vision Service of the Ophthalmic Clinic at the University of São Paulo and at the Brazilian Association for the Visually Impaired People (Laramara), located in São Paulo, Brazil. PATIENTS AND METHODS: This study evaluated 3,210 visually impaired children (49% female, 51% male; average age, 5.9 years). Visual impairment was present in 57% (visually impaired group) and 43% presented another associated disability (multiple disability group). RESULTS: The main causes of visual impairment in the visually impaired group were toxoplasmic macular retinochoroiditis (20.7%), retinal dystrophies (12.2%), retinopathy of prematurity (11.8%), ocular malformation (11.6%), congenital glaucoma (10.8%), optic atrophy (9.7%), and congenital cataracts (7.1%). The main causes of visual impairment in the multiple disability group were optic atrophy (37.7%), cortical visual impairment (19.7%), toxoplasmic macular retinochoroiditis (8.6%), retinopathy of prematurity (7.6%), ocular malformation (6.8%), congenital cataracts (6.1%), and degenerative disorders of the retina and macula (4.8%). The retina was the most frequently affected anatomic site in the visually impaired group (49.2%) and the optic nerve in the multiple disability group (39%). CONCLUSION: Primary, secondary, and tertiary prevention efforts for childhood blindness and visual rehabilitation must be considered in Latin America.

Amaurosis bilateral cortical en preeclampsia severa / Cortical blindness in severe preeclampsia

Se presenta un caso clínico con diagnóstico final de preeclampsia severa que debuta con una complicación excepcional, la amaurosis bilateral cortical. Se discute esta presentación poco frecuente, realizándose una revisión actualizada.

[The contribution of neuropsychology to the diagnosis and treatment of learning disorders]. / Aportes de la neuropsicología al diagnóstico y tratamiento de los trastornos de aprendizaje.

INTRODUCTION: In its broadest sense, the basic concept of Neuropsychology is that all behaviour originates in the brain; according to Luria, it depends on functional systems constituted by different areas of the brain which are inter related. Each area deals with a particular function which is part of the whole (for example, the cortical area for vision deals with reading). Which cortical areas make up the functional system depends on how the person acquired a certain type of behaviour. DEVELOPMENT: The neuropsychological model, when applied to learning disorders, assumes that they are the expression of specific cerebral dysfunction due to genetic or environmental factors which have altered the development of the nervous system. Pennington recognizes five functional systems or nodules related to intellectual function. Each of these corresponds to a clearly defined zone of the cerebrum and impaired function of any zone causes specific learning disorders. Thus, the left perisylvan region deals with neurolinguistic function and disorders of this causes dyslexia. The hippocampal area of both hemispheres is related to memory and changes in this lead to disorders of memory. Right hemisphere dysfunction causes dyscalculia (posterior right hemisphere) and behaviour disorders (anterior right hemisphere) which may present together or separately. The dysexecutive syndrome is due to frontal lobe changes and is characterized by attention deficit, poor planning and anticipation, defective abstraction and other behaviour disorders. Although these are the best defined and most commonly seen syndromes in clinical practice, it would be logical to expect that there may be as many types of disorder as there are neural systems or subsystems involved in particular types of learning or behaviour, which may be altered. In support of these concepts we report four clinical cases seen by us in the Italian Hospital, Buenos Aires.