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)

Pattern of corneal disorders in Ekiti: a tertiary eye center experience

Purpose: The cornea is the most significant refractive medium in the eye. Pathologies affecting the cornea usually have a great impact on vision. The etiology of corneal disorder varies from one geographical location to another. The objective of this study was to determine the pattern of corneal disorders at Ekiti State University Teaching Hospital, Ado-Ekiti. Materials and Methods: A retrospective study of case records of patients with cornea disorders over a 5-year period was carried out. Demographic characteristics, presenting visual acuity, and risk factor for cornea disorders were retrieved. Data were entered into the Statistical Package for the Social Sciences version 20, and statistical significance was inferred at P < 0.05. Results: Corneal disorders accounted for 3.3% of the eye disorders seen during the period of study. The median age was 37 years. Males outnumbered females giving a ratio of 1.9:1 and the age range from 0.25 to 92 years. There were more females than males in the 11­20 years' age group. Students (84, 25.4%) and artisans (62, 18.8%) were the two leading occupational groups. Infectious cases constituted 27.2% of the cases. Visual acuity at presentation was <3/60 in 131 (39.7%) cases. Foreign body entry was the leading etiologic agent in 101 (30.6%) cases. Conclusion: Half of the patients were blind at presentation, and many of them presented after more than 1 week of the onset of symptoms. Corneal foreign body, trauma, and vernal keratoconjunctivitis were the leading known predisposing factors. There will be need to emphasize more on the role of protective eye devices among our people, especially those who engage in outdoor activities

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.

's experience in the treatment of cortical blindness with acupuncture: report of medical cases / 中国针灸

's experience is introduced in the treatment of cortical blindness with the comprehensive acupuncture therapy for benefiting and resolving stasis. The comprehensive therapy includes acupuncture at Sishencong (EX-HN 1), Xinming No.1 (Extra), Shang Jianming, Chengqi (ST 1), Xinming No.2 (Extra), optic area and visual area (scalp acupuncture); acupoint injection applied alternatively at Qiuhou (EX-HN 7) and Taiyang (EX-HN 5); the auricular point paste stimulation at Brain Point, Shenmen (TF), Gan (CO), Shen (CO), Yan (LO), Eye No.1 and Eye No.2; as well as the tapping therapy with skin needle at Zhengguang No.1 and Zhengguang No.2. Additionally, 3 typical cases of cortical blindness are introduced. The coordination of the different acupuncture techniques is analyzed so as to explain its actions as benefiting , resolving stasis, promoting blood circulation and nourishment of the eyes.

Late-Onset Group B Streptococcal Meningitis Complicated with Extensive Cerebral Infarction

Group B streptococcus (GBS) is the leading cause of neonatal morbidity and mortality. Late-onset GBS disease commonly manifests as occult bacteremia or meningitis. Approximately 50% of survivors of late-onset meningitis have long-term neurologic sequelae. Cerebrovascular complications are often associated with unfavorable clinical outcomes of GBS meningitis. There have been a few reports of cerebral infarction accompanied by GBS meningitis. We report a 29-day-old girl with severe, widespread cerebral infarction due to late-onset GBS meningitis. Isolated GBS strain from this patient was serotype III, ST-19. Currently, she has cortical blindness and significant developmental delay.

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.

Severe Cerebral Vasospasm in Patients with Hyperthyroidism

Cerebral vasospasm associated with hyperthyroidism has not been reported to cause cerebral infarction. The case reported here is therefore the first of cerebral infarction co-existing with severe vasospasm and hyperthyroidism. A 30-year-old woman was transferred to our hospital in a stuporous state with right hemiparesis. At first, she complained of headache and dizziness. However, she had no neurological deficits or radiological abnormalities. She was diagnosed with hyperthyroidism 2 months ago, but she had discontinued the antithyroid medication herself three days ago. Magnetic resonance imaging and angiography showed cerebral infarction with severe vasospasm. Thus, chemical angioplasty using verapamil was performed two times, and antithyroid medication was administered. Follow-up angiography performed at 6 weeks demonstrated complete recovery of the vasospasm. At the 2-year clinical follow-up, she was alert with mild weakness and cortical blindness. Hyperthyroidism may influence cerebral vascular hemodynamics. Therefore, a sudden increase in the thyroid hormone levels in the clinical setting should be avoided to prevent cerebrovascular accidents. When neurological deterioration is noticed without primary cerebral parenchyma lesions, evaluation of thyroid function may be required before the symptoms occur.

Neonatal Group B Streptococcal Meningitis Complicated with Secondary Hypopituitarism: A Case Report

Group B Streptococcus (GBS) commonly causes neonatal meningitis and sepsis. In infants with late-onset meningitis, fever, irritability or lethargy or both, poor feeding, and tachypnea are common initial signs. Major neurologic sequelae are observed in 29% of children, the most serious including global or profound mental retardation, spastic quadriplegia, cortical blindness, deafness, uncontrolled seizures, hydrocephalus, and hypothalamic dysfunction. We report a 14-day-old full-term female infant who presented with grunting and irritability to the emergency room and was diagnosed with GBS meningitis subsequently complicated with central diabetes insipidus and secondary hypopituitarism. Central diabetes insipidus should be ruled out in infants with complicated GBS meningitis.

A Case of Posterior Reversible Encephalopathy Syndrome with Bilateral Visual Impairment

PURPOSE: To report a case of recovery of bilateral cortical blindness in a patient with posterior reversible encephalopathy syndrome. CASE SUMMARY: A 46-year-old female visited the ophthalmology department due to abrupt visual acuity decrease. Ten days earlier, she had received conservative management due to anemia caused by menorrhagia and uterine prolapse. She underwent a gynecological operation to remove a uterine myoma two days previously, and was given a blood transfusion postoperatively because of excessive bleeding. After the transfusion, she complained of acute blurred vision. Her best corrected visual acuity (BCVA) was hand motion 10 cm in both eyes. There were no abnormal specific findings except retinal dot hemorrhage at the temporal side in the left eye on fundus examination. Her pupillary light reflex was normal and optical coherence tomography examination was unremarkable in both eyes. However, flash visual evoked potential findings showed reduced P100 amplitude in both eyes and she was diagnosed with posterior reversible encephalopathy syndrome based on brain magnetic resonance imaging. After close observation without any treatment, the symptoms gradually improved. Finally, her BCVA recovered to 1.0 and P1 and P100 amplitudes were restored to normal range in both eyes at 16 weeks from the first diagnosis. CONCLUSIONS: A patient complained of bilateral visual loss without other neurological symptoms after chronic blood loss and blood transfusion. She recovered visual acuity completely with prompt diagnosis of posterior reversible encephalopathy syndrome and close observation only.

Transient Cortical Blindness Following Vertebral Angiography: A Case Report / 신경중재치료의학

Transient cortical blindness (TCB) is a rare but well-known complication of cerebral angiography. Its pathophysiology remains uncertain. We would like to report a case of TCB in a patient during a follow up vertebral angiogram for post-coil embolization of left posterior inferior cerebellar artery aneurysm. Patient's vision was resumed spontaneously within 24 hours after angiography, with no residual neurological deficit in subsequent clinical follow up. Multi-modality imaging evaluation including vertebral angiography, brain CT and MRI performed on same day are presented.

Posterior Cortical Atrophy with Acute Onset and Rapid Progressive Visual Symptoms: A Case Report

BACKGROUND: Posterior cortical atrophy (PCA) is characterized by slowly progressive early onset dementia with cortical visual dysfunction and disproportionate atrophy of the posterior cortex. CASE REPORT: A 55-year-old right-handed woman developed visuo-spatial impairments that progressed rapidly into cortical blindness over the following 3 months. Neuro-psychological evaluation revealed Gerstmann syndrome and severe constructional impairments with all components of Balint syndrome. However, her memory, insight, and judgment were preserved. Her brain MRI was normal. However, 18F fluorodeoxyglucose positron emission tomography revealed a marked hypometabolism in the bilateral parieto-occipital region. CONCLUSIONS: Although rapid progression of visuo-spatial dysfunction without memory impairment occurred, we considered PCA as well.

Arnold-Chiari Malformation Type III With Meningoencephalocele: A Case Report

Arnold-Chiari malformation type III (CM III) is an extremely rare anomaly with poor prognosis. An encephalocele with brain anomalies as seen in CM II, and herniation of posterior fossa contents like the cerebellum are found in CM III. The female infant was a twin, born at 33 weeks, weighing 1.7 kg with a huge hydrocele on the craniocervical junction. After operations were performed, she was referred to the department of rehabilitation medicine for poor motor development, swallowing dysfunction, and poor eye fixation at 22 months. The child was managed with neurodevelopmental treatment, oromotor facilitation, and light perception training. After 14 months, improvement of gross motor function was observed, including more stable head control, rolling, and improvement of visual perception. CM III has been known as a condition with poor prognosis. However, with the improvement in operative techniques and intensive rehabilitations, the prognosis is more promising than ever before. Therefore, more attention must be paid to the rehabilitation issues concerning patients with CM III.

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.

Cortical Blindness Following Spinal Surgery: Very Rare Cause of Perioperative Vision Loss

A 38-year-old man was operated with posterior spinal decompression and pedicle screw instrumentation for his L2 fracture with incomplete neurological deficit. In the recovery, he complained of blindness in both eyes after twelve hours. Computed tomographic scan and magnetic resonance angiography revealed bilateral occipital lobe infarcts. He remained permanently blind even after three years follow-up. Though rare, perioperative vision loss is a potential complication following spine surgery in prone position. We report a rare occurrence of cortical blindness following lumbar spine surgery.

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.

Anton's Syndrome and Eugenics

Anton's syndrome is arguably the most striking form of anosognosia. Patients with this syndrome behave as if they can see despite their obvious blindness. Although best known for his description of asomatognosia and visual anosognosia, Gabriel Anton (1858-1933) made other significant contributions to the clinical neurosciences, including pioneering work in neurosurgery, neuropsychology, and child psychiatry. However, it has not been recognized in the English literature that Anton was also a dedicated advocate of eugenics and racial hygiene. This paper provides a case of Anton's syndrome and puts the works of Gabriel Anton into their historic context.

Transient cortical blindness after heart surgery in a child patient: A case report / 대한마취과학회지

Visual loss occurring after pediatric cardiac surgery employing cardiopulmonary bypass (CPB) is relatively rare but the risk is substantial. Compromised cerebral perfusion due to a CPB related micro-embolization and inflammatory vascular changes as well as reduced oxygen carrying capacity in hemodilution and hypothermia during CPB might be major contributing factors to the development of postoperative visual loss after cardiac surgery with CPB. A case of immediate but transient postoperative visual loss was encountered in a 21-month-old male who underwent tricuspid valve surgery. Despite routine intraoperative measures to maintain an adequate perfusion pressure throughout the procedure, postoperative computed tomography revealed a subacute infarct in his occipital lobe. Recovery began on postoperative day 28, and the patient's vision was restored by 31 days.

A Case of Complete Recovery of Visual Loss Associated With Electroconvulsive Therapy

PURPOSE: To report a case of complete recovery within 1 day of cortical blindness associated with electroconvulsive therapy (ECT). CASE SUMMARY: A 15-year-old girl, who had no ophthalmologic disease, complained of bilateral visual loss immediately after ECT. At the first ophthalmologic examination, best corrected visual acuity was 0.03 in both eyes. Findings of anterior segment and fundus, movement of external ocular muscles, and pupillary light reflex were normal. Visual field examination showed generalized reduction of sensitivity and increased fixation loss in both eyes. Optical coherence tomography revealed no abnormalities. Electroretinography was normal, and a slightly prolonged latency of P100 was noted in the right and left flash visual evoked potentials. Without any specific treatment, the patient's vision improved gradually after 18 hours of ECT and was recovered completely, with vision of 1.0 in both eyes the next day. There were no abnormalities in visual evoked potentials or the visual field. CONCLUSIONS: Transient visual loss can occur after ECT, because of cortical blindness by transient cerebral ischemia, and can be recovered completely without specific treatment.