Tumor de hipófise em paciente com hidrocefalia: um desafio diagnóstico / Pituitary tumor in a patient with hydrocephalus: a diagnostic challenge

RESUMO A hidrocefalia é definida como a dilatação ventricular pelo aumento da pressão intraventricular e intracraniana quando não tratada ou por insucesso do tratamento. Muitas vezes, leva ao dano das vias ópticas, podendo causar atrofia óptica, devido à proximidade dessas vias com o ventrículo lateral quando ocorre a dilatação. Assim como a hidrocefalia pode levar à atrofia óptica, outras patologias também podem. Tumores hipofisários compartilham desse mesmo sinal, além de causar hemianospsia bitemporal quando o tumor comprime quiasma óptico. Ademais, a hemianopsia bitemporal é o distúrbio visual mais comum encontrado em pacientes com tumor de hipófise. Os tumores de hipófise, por exemplo, geram manifestações clínicas que podem estar relacionadas à disfunção da glândula ou aos efeitos mecânicos da expansão tumoral. Sinais e sintomas visuais estão mais ligados ao efeito mecânico do tumor. Assim, muitas vezes, o paciente procura o oftalmologista antes do endocrinologista. Neste caso, analisaremos uma paciente portadora de hidrocefalia que apresentava, concomitantemente, um tumor hipofisário, e a investigação oftalmológica fez toda a diferença no tratamento da paciente.

ABSTRACT Hydrocephalus is defined as ventricular dilation caused by increased intraventricular and intracranial pressure when untreated or due to treatment failure. Optical pathways can often cause optic atrophy due to the proximity to the lateral hazard when dilation occurs. Hydrocephalus can lead to optic atrophy, as well as other pathologies. Pituitary tumors share this same sign, in addition to causing bitemporal hemianopia when it compresses the optic chiasm. In addition, bitemporal hemianopia is the visual disturbance most commonly found in patients with pituitary tumors. Pituitary tumors, for example, have clinical manifestations that may be related to gland dysfunction, or to mechanisms of tumor expansion. Visual signs and symptoms are more linked to the mechanical effect of the tumor. Therefore, the patient usually seeks the ophthalmologist before the endocrinologist. In this case, we analyzed a patient with hydrocephalus who presented, at the same time, a pituitary tumor, and the ophthalmological investigation made all the difference in the treatment of the patient.

Manifestaciones neuroftalmológicas en pacientes con enfermedad cerebrovascular isquémica / Neuro-ophthalmological manifestations in patients with ischemic cerebrovascular disease

La enfermedad cerebrovascular isquémica tiene una elevada incidencia y prevalencia en Cuba, y constituye la tercera causa de muerte en el país. Existen diferencias anatómicas y clínicas entre el infarto de la circulación anterior y la posterior. En ocasiones, los elementos distintivos que ayudan al diagnóstico topográfico de la enfermedad cerebrovascular isquémica son las manifestaciones neuroftalmológicas. Con el objetivo de profundizar en el conocimiento actual sobre las alteraciones neuroftalmológicas que se asocian a la enfermedad cerebrovascular isquémica, se realizó una revisión bibliográfica, donde se consultaron un total de 69 fuentes de información digital de los últimos 5 años. La circulación cerebral se divide de manera general en anterior y posterior. Los síntomas y signos principales del ictus que afecta la circulación anterior son la desviación conjugada de la mirada, la afectación de las sácadas, la hemianopsia homónima, la heminegligencia y la apraxia de la apertura ocular; mientras que las alteraciones asociadas a la afectación de la circulación posterior son el nistagmo, las anormalidades en la alineación y los movimientos oculares, así como la hemianopsia homónima con conservación macular. Se concluye que en la enfermedad cerebrovascular isquémica aparecen síntomas y signos como consecuencia de la afectación, tanto de la vía visual aferente, como de la eferente. La hemianopsia homónima es el signo más frecuente reportado(AU)

Ischemic cerebrovascular disease has a high incidence and prevalence in Cuba, and it is the third cause of death in the country. A number of anatomical and clinical differences distinguish anterior from posterior circulation infarction. On certain occasions the distinguishing elements that aid in the topographic diagnosis of ischemic cerebrovascular disease are its neuro-ophthalmological manifestations. With the purpose of gaining insight into the current knowledge about the neuro-ophthalmological alterations associated to ischemic cerebrovascular disease, a bibliographic review was conducted based on the analysis of 69 digital information sources from the last five years. Cerebral circulation is generally divided into anterior and posterior. The main symptoms and signs of the stroke that affects anterior circulation are conjugate gaze deviation, altered saccades, homonymous hemianopsia, heminegligence and eyelid opening apraxia, whereas the alterations associated to posterior circulation involvement are nystagmus, eye movement and alignment abnormalities, and homonymous hemianopsia with macular preservation. It is concluded that ischemic cerebrovascular disease presents symptoms and signs related to both the afferent and the efferent visual pathways. Homonymous hemianopsia is the most common sign reported(AU)

Traumatic chiasmal syndrome. Case report / Síndrome quiasmática traumática. Relato de caso

ABSTRACT Traumatic chiasmal syndrome is one of the rare etiologies of chiasmal syndrome, characterized by optic chiasm injury following head trauma. The main visual defect associated is bitemporal hemianopia with macular splitting; however, it can present with a variety of other visual defects and neurologic signs. The authors report a case of complete bitemporal hemianopia after head trauma, with multiple frontal and skull base fractures and no other neurologic deficits, or hypothalamic-pituitary abnormality. Most cases of traumatic chiasmal syndrome are caused by mechanical stretch or compression of the chiasma. Nevertheless, in this case, the radiologic findings excluded macroscopic disruption or external compression of the chiasma, raising the possibility of a contusion necrosis associated with functional impairment of the optic chiasma. Traumatic chiasmal syndrome must be considered in the differential diagnosis of patients presenting with complete bitemporal hemianopia after head injury caused by frontal and skull base fracture.

RESUMO A síndrome quiasmática traumática é uma das raras etiologias da síndrome do quiasma óptico, que se caracteriza pela presença de lesão do quiasma óptico causada por traumatismo craniencefálico. O principal defeito visual associado é a hemianopsia bitemporal com macular splitting. No entanto, pode se manifestar por uma variedade de outros defeitos visuais e sinais neurológicos. Os autores relatam um caso de hemianopsia bitemporal completa após traumatismo craniencefálico com múltiplas fraturas frontais e da base do crânio na ausência de outros défices neurológicos ou alterações do eixo hipotálamo-hipofisário. A maioria dos casos de síndrome quiasmática traumática é causada por estiramento mecânico ou compressão do quiasma. No entanto, no caso apresentado, os achados radiológicos excluíram lesão macroscópica ou compressiva do quiasma, levantando a possibilidade de uma necrose após contusão associada ao compromisso funcional do quiasma óptico. A síndrome quiasmática traumática deve ser considerada no diagnóstico diferencial de doentes que apresentam hemianopsia bitemporal completa após traumatismo craniencefálico, especialmente em casos de fratura do osso frontal e da base do crânio.

Digital Therapeutics: Emerging New Therapy for Neurologic Deficits after Stroke / 대한뇌졸중학회지

Digital therapeutics is an evidence-based intervention using high-quality software, with the sole purpose of treatment. As many healthcare systems are encountering high demands of quality outcomes, the need for digital therapeutics is gradually increasing in the clinical field. We conducted review of the implications of digital therapeutics in the treatment of neurological deficits for stroke patients. The implications of digital therapeutics have been discussed in four domains: cognition, speech and aphasia, motor, and vision. It was evident that different forms of digital therapeutics such as online platforms, virtual reality trainings, and iPad applications have been investigated in many trials to test its feasibility in clinical use. Although digital therapeutics may deliver high-quality solutions to healthcare services, the medicalization of digital therapeutics is accompanied with many limitations. Clinically validated digital therapeutics should be developed to prove its efficacy in stroke rehabilitation.

Bilateral Occipital Lobe Infarction Presenting as Bilateral Inferior Altitudinal Defects

PURPOSE: Horizontal visual field defects are generally caused by lesions before the optic chiasm, but we report a case with bilateral inferior altitudinal defects secondary to bilateral occipital lobe infarction. CASE SUMMARY: A 57-year-old male with a history of diabetes and hypertension presented with a month of blurring in the inferior visual field. His corrected visual acuity was 1.0 in the right eye and 0.63 in the left eye, and the intraocular pressure was normal in each eye. Pupillary response, ocular movement, and color vision tests were normal in both eyes. There was no specific finding of the optic disc and macula on fundus examination. Visual field examination revealed an inferior congruous homonymous hemianopia with horizontal meridian sparing and a left incongruous homonymous quadrantanopia. Optical coherence tomography for peripapillary retinal nerve fiber layer thickness revealed a mild decrease in the inferior disc of both eyes. Brain magnetic resonance imaging confirmed the presence of an acute infarction confined with upper medial calcarine fissures of bilateral occipital lobe and the right splenium of the corpus callosum, which were consistent with inferior altitudinal hemianopia and left superior incongruous quadrantanopia, respectively. Brain magnetic resonance angiography showed multiple stenosis of bilateral posterior cerebral arteries. CONCLUSIONS: The altitudinal visual field defects could be caused by the occipital lesion medial to the calcarine fissure, and unusual visual defects could be due to a combination of multiple lesions.

Homonymous Quadrantanopia Caused by Occipital Lobe Ulegyria

PURPOSE: We report a case of homonymous quadrantanopia caused by occipital lobe ulegyria. CASE SUMMARY: A 23-year-female was referred to our clinic because of a visual field defect incidentally discovered during preoperative evaluation for refractive surgery at another clinic. However, she did not report any symptoms. She had no systemic diseases. Visual acuity was 20/20 in both eyes, and the color vision test was normal. Both pupils exhibited normal responses to light and near stimulations. In fundus examinations, the right optic disc was normal and the left contained drusen. Automated perimetry revealed right lower homonymous quadrantanopia with macular sparing. Brain magnetic resonance imaging revealed areas of ulegyria involving the left occipital lobe, consistent with the visual field defect. A follow-up visual field test performed 5 months later yielded the same result. CONCLUSIONS: Neuroimaging should be performed in patients with homonymous visual field defects to determine the location and etiology of the brain lesions. Occipital lobe ulegyria can cause homonymous quadrantanopia in the absence of any neurological problem.

Ganglion Cell Analysis in an Optic Tract Syndrome Patient Previously Diagnosed with Glaucoma

PURPOSE: To report the results of ganglion cell analysis in a patient with optic tract syndrome who was previously diagnosed with glaucoma. CASE SUMMARY: A 32-year-old male, who had been diagnosed with glaucoma 12 years ago, but had not visited an ophthalmology clinic since then, came to our clinic for evaluation of his glaucoma. Both eyes showed an increased cup-to-disc ratio and temporal pallor of the disc. Retinal nerve fiber layer (RNFL) optical coherence tomography showed thinning of the superior, inferior, and temporal peripapillary RNFL in both eyes. On ganglion cell analysis (GCA), ganglion cell layer thinning in the nasal region of the right eye and in the temporal region of the left eye was observed. The visual field test showed right incongruous homonymous hemianopsia. After the atrophic change of the left optic tract was confirmed by orbit magnetic resonance imaging, he was diagnosed with left optic tract syndrome. CONCLUSIONS: We report the results of GCA in a case of optic tract syndrome, previously diagnosed as glaucoma. GCA can be useful when diagnosing optic tract syndrome.

Homonymous Hemianopia as the Initial Sign of Posterior Cortical Atrophy

No abstract available.

Clinical Characteristics of Stroke Mimics Presenting to a Stroke Center within the Therapeutic Window of Thrombolysis

This study aimed at identifying the frequency and final diagnoses of stroke mimics (SMs) among suspected stroke cases, and seeking differences in clinical & imaging features between SMs and true strokes. It was retrospective with data taken from an ongoing stroke registry for patients admitted to a Stroke Unit between 1 May and 31 October 2011. Baseline characteristics, clinical & imaging features, complications, and outcomes of SMs were compared to those with true strokes by appropriate statistical tests. The total number of cases admitted was 537. Only 232 (43.2%) of them presented during the critical time thrombolytic intervention. SMs comprised 15.9% of all suspected stroke cases. The commonest mimics were psychiatric disorders (43.2%), migraines (16.2%), seizures (8.1%), brain tumors (5.4%), and systemic infections (5.4%). SMs were younger, more common in females, with less evidence of preexisting hypertension (43.2% vs. 56.9% for true strokes). Hemianopia (p = 0.001), sensory inattention, neglect or both (p < 0.001) were more associated with true strokes, while cerebellar, brainstem signs or both (p = 0.045) excluding dysarthria were linked only to SMs. Acute ischemic signs in imaging scans were found linked to true strokes. Some clinical and imaging characteristics were found to differentiate to some extent between SMs and true strokes.

Chordoid Glioma of the Third Ventricle, a Rare Tumor with an Unexpected Outcome

Chordoid glioma is a rare tumor of the third ventricle whose imaging features are difficult to distinguish from other more common lesions in this location. There are only 83 cases described so far in the literature. Although gross total resection (GTR) is the treatment of choice, immediate postoperative mortality with this approach can be as high as 29%, and morbidity among survivors can reach 67%. We report a case of a male patient of advanced age, with a third ventricle mass lesion, who presented with a progressive right temporal hemianopia. Imaging was compatible with craniopharyngioma, meningioma or even metastasis. Chordoid glioma was not considered in the differential diagnosis. The patient underwent surgery and GTR was achieved. There were no postoperative complications, and the patient was discharged from the hospital three weeks later. Unexpectedly, two days afterwards, he suffered a major brainstem hemorrhagic stroke and, unfortunately, died.

O glioma cordoide é um tumor raro do terceiro ventrículo, e as suas características imagiológicas são difíceis de distinguir de outras lesões mais comuns nesta localização. Até a data presente, existem apenas 83 casos de gliomas cordoides descritos na literatura. A remoção macroscópica total destes tumores deve ser o tratamento de escolha; no entanto, a mortalidade pós-operatória imediata pode chegar aos 29%, e a morbilidade pode atingir os 67% entre os sobreviventes. Nós descrevemos o caso de um homem idoso com uma lesão tumoral no terceiro ventrículo, que se manifestou com uma hemianopsia temporal direita progressiva. Os exames de imagem eram compatíveis com craniofaringioma, meningioma ou até metástase. O glioma cordoide não foi considerado como uma das hipóteses no diagnóstico diferencial inicial. O paciente foi submetido a cirurgia, tendo-se obtido a remoção macroscópica total. Não houve qualquer complicação no período pós-operatório, e o paciente teve alta hospitalar após três semanas. Inesperadamente, dois dias após a alta clínica, o paciente sofreu um AVC hemorrágico do tronco cerebral, e acabou por falecer.

Occipital Lobe Metastasis of Hepatocellular Carcinoma Presenting as Homonymous Hemianopia

PURPOSE: To report brain metastasis of hepatocellular carcinoma presenting as homonymous hemianopia. CASE SUMMARY: A 51-year-old female with a history of hepatectomy and diagnosis of hepatocellular carcinoma (HCC) 19 months earlier was referred to our neuro-ophthalmology clinic for evaluation due to headache and decreased visual acuity over the past several months. Best visual acuity was 20/20, and the results of all other aspects of our examination were normal except Humphrey automatic perimetry, which showed complete left homonymous hemianopia. Brain magnetic resonance imaging showed a large mass in the right occipital lobe. Craniotomy and removal of tumor were performed. HCC was confirmed by histopathologic examination. CONCLUSIONS: Metastasis of hepatocellular carcinoma to the occipital lobe is extremely rare but can present as homonymous hemianopia. Therefore, clinicians should be aware of this when examining a patient with a history of HCC.

Posterior Type of Alzheimer's Dementia Presenting with Homonymous Hemianopsia

BACKGROUND: Alzheimer's disease is a chronic neurodegenerative condition, mostly affecting the medial temporal lobe and associated neocortical structures. In this report, we present a rare clinical manifestation of this disease. CASE REPORT: A 61-year-old female with word finding difficulty and memory disturbances was diagnosed with Alzheimer's disease. Two years later, she complained of right homonymous hemianopia without optic ataxia, ocular apraxia, and simultagnosia. No findings other than parenchymal disease were apparent in magnetic resonance imaging and laboratory tests. CONCLUSIONS: In this case, in a patient initially diagnosed with Alzheimer's dementia with progressive disease, we found only homonymous hemianopia, without signs of Balint's syndrome or Gerstmann's syndrome. After careful investigation showing that Alzheimer's dementia with visual symptom was not associated with parenchymal disease, we concluded a case of atypical variant of Alzheimer's disease.

Pituicytoma with Significant Tumor Vascularity Mimicking Pituitary Macroadenoma

A 19-year-old man presented with bitemporal hemianopsia and was found to have a large sellar and suprasellar tumor, resembling a pituitary macroadenoma. Emergency transsphenoidal approach was attempted because of rapid visual deterioration with headache. However, the approach was complicated and stopped by uncontrolled hemorrhage from the tumor. After conventional cerebral angiography and recognition of an unusual pathology, transcranial approach was achieved to prevent permanent visual loss. The final pathological diagnosis was pituicytoma with epithelioid features. Pituicytoma is a rare low-grade tumor (WHO Grade I) of pituicytes involving the sellar and suprasellar region, and originating from special glial cells of the neurohypophysis. Because of the high vascularity, the firm consistency, and invasion to surrounding neurovascular structures, a pituicytoma should be included in the differential diagnosis of a mass in the sellar and suprasellar area if the tumor shows high enhancement with vascular components. We report a case of rare pituicytoma mimicking a pituitary macroadenoma with massive hemorrhage to disturb surgery.

An Unruptured Anterior Communicating Artery Aneurysm Presenting with Left Homonymous Hemianopsia: A Case Report

Unruptured cerebral aneurysms sometimes present with visual symptomsdue to compression of the visual pathways. However, until now, unruptured anterior communicating artery (ACoA) aneurysms presenting visual field defects have been extremely rare. The authors report the case of a 51-year-old woman who presented with left homonymous hemianopsia. Radiological findings demonstrated an ACoA aneurysm filled with thrombus, that was compressing the optic chiasm and post-chiasmal tract. The patient underwent clipping of the aneurysm, which resolved the visual field defect. In cases of visual field defects, an ACoA aneurysm should be included in the differential diagnosis.

Pancreatic Choriocarcinoma with Multiple Metastases: a Case Report and Literature Review / 대한췌담도학회지

Non-gestational, extragonadal choriocarcinoma is a rare disease and pancreatic choriocarcinoma is an extremely rare disease. Choriocarcinoma of non-placental origin is a highly malignant carcinoma with poor prognosis. It is characterized by high serum human chorionic gonadotropin levels. There is no standard therapy for extragonadal choriocarcinoma. Herein, we report a 47-year-old woman who presented with acute pancreatitis and left hemianopsia and was diagnosed with pancreatic choriocarcinoma with multiple metastases in liver, lung, and brain. Although the patient was treated with best supportive care, she succumbed to cerebral edema and hypernatremia on the fifteenth day of hospitalization.

Caso de hemianopsia reversível no pós-parto decorrente de síndrome da encefalopatia reversível posterior (PRES) em gestante com eclâmpsia tardia / Reversible hemianopsia in postpartum due to posterior reversible encephalopathy syndrome in pregnant with late eclampsia

Resumo Objetivos: Descrever um caso de Síndrome da Encefalopatia Reversível Posterior em gestante diagnosticada com eclâmpsia tardia, bem como seu manejo clínico. Descrição do caso: Paciente feminina, 34 anos, em sua terceira gestação, iniciou com aumento dos níveis tensionais durante o trabalho de parto e, após onze dias de puerpério, apresentou quadro de diminuição da acuidade visual à direita, seguida de crise convulsiva e subsequente perda parcial da visão do olho direito. Após a realização de exames de imagem e descartada a possibilidade de acidente vascular encefálico, a paciente foi diagnosticada com Síndrome da Encefalopatia Reversível Posterior (PRES). Instituído o manejo clínico das crises convulsivas e hipertensivas, houve remissão completa dos sintomas e reversão do quadro clínico inicial. Conclusões: Uma vez adequadamente diagnosticada e tratada, a Síndrome da Encefalopatia Reversível Posterior pode apresentar evolução satisfatória, especialmente quando associada a um fator desencadeado agudamente, como a eclâmpsia.

Abstract Objectives: To describe a case of Posterior Reversible Encephalopathy Syndrome diagnosed in pregnant women with late-eclampsia, as well as its clinical management. Case description: A 34 years old patient in her third pregnancy had started with high blood pressure levels during labor; after eleven days postpartum, she presented a decreased right visual acuity; subsequently one episode of seizure followed by partial loss of vision in the right eye. After conducting tests and ruled out stroke, the patient was diagnosed as Posterior Reversible Encephalopathy Syndrome (PRES). Established the clinical management of seizures and hypertensive crisis, there was complete remission of symptoms and reversal of the initial clinical picture. Conclusion: Once properly diagnosed and treated, the Posterior Reversible Encephalopathy Syndrome can present satisfactory progress, especially when associated with an acutely triggered factor, as eclampsia.

Occipital Lobe Epilepsy with Hemicrania Epileptica

No abstract available.

Charles Bonnet Syndrome Following Trans-Sphenoidal Adenomectomy without Optic Nerve Atrophy

Charles Bonnet syndrome (CBS) can develop after trans-sphenoidal adenomectomy (TSA); however, the neural mechanisms remain unknown. Sensory deprivation and releasing phenomenon are both hypothetical explanations for this condition; however, there is no definite evidence that strongly supports either supposition. We report the first case of CBS after TSA without optic nerve atrophy. Postoperatively, the patient's vision seemed to be relatively well preserved, apart from the left-side hemianopsia in the right eye. Distinctive visual hallucinations only appeared when his eyes were closed, and these responded to quetiapine in a dose-dependent manner. Dose dependent change in colors and formation of hallucination was reported. Two weeks after quetiapine initiation, the patient's CBS was completely resolved. This unique case suggests that blocking sensory input from the periphery is more critical than neural damage of the bottom-up connection to the visual association cortex. In addition, quetiapine should be considered as a specific treatment for CBS.

Prominent Cognitive Dysfunction without Motor Impairment Following Anterior Choroidal Artery Infarction: a Case Report

Neurological deficits commonly associated with anterior choroidal artery infarction (AChAI) include hemiplegia, hemisensory loss, and homonymous hemianopsia, while neuropsychological and perceptual deficits are uncommon. Prominent cognitive function impairment has rarely been reported. Here, we report a case of AChAI with prominent cognitive function impairment without motor deterioration. In contrast to the typical clinical features of AChAI, near complete and rapid motor recovery was observed, while cognitive impairment persisted despite rehabilitation therapy.

Bitemporal Hemianopsia in Ethambutol-Induced Optic Neuropathy

PURPOSE: To report three cases with bitemporal hemianopsia after using ethambutol to treat tuberculosis. CASE SUMMARY: A 50-year-old male with chronic renal failure and tuberculous pleurisy, a 57-year-old male with diabetic retinopathy and pulmonary tuberculosis, and a 59-year-old male with diabetes and pulmonary tuberculosis were referred for evaluation due to decreased visual acuity for several months after taking ethambutol to treat tuberculosis. All 3 patients had abnormal color vision and visual evoked potential in both eyes. Visual field showed bitemporal hemianopsia with or without central scotoma. Brain imaging tests were normal. Although ethambutol was discontinued in all three patients, one patient with renal disease showed further decrease in visual acuity and visual field worsened to total field defect. CONCLUSIONS: Ethambutol-induced optic neuropathy is a wide spectrum disorder and based on our cases, can present as bitemporal hemianopsia mimicking compressive chiasmal lesions. A thorough history should be taken and immediate discontinuation of ethambutol is recommended in cases when bitemporal hemianopia occurs.