Akinetopsia (visual motion blindness) associated with brain metastases: a case report.

Akinetopsia is a rare neurological syndrome characterized by an impaired perception of movement, often resulting from brain damage due to ischemia, epilepsy, or medication. It is also known as visual motion blindness, and patients with this condition are unable to perceive motion normally even with perfect visual acuity. This report aims to present a case of a patient in their late 40 s who developed akinetopsia and also an impairment in movement perception of objects without emitting sounds, after experiencing a late relapse of breast cancer with the occurrence of multiple brain metastases. The patient also experienced visual hallucinations, night terrors, and difficulty forming anterograde memory. Neuroimaging with MRI revealed severe brain damage, especially in the middle temporal area of the visual cortex. Akinetopsia is a rare phenomenon, and this is the first known case of its association with brain metastases.

Relationship Between Brain Tumors and Intracranial Aneurysms: A Systematic Review.

AIM: To investigate the possible relationship between intracranial aneurysms and brain neoplasms. MATERIAL AND METHODS: A comprehensive literature review involving a search of the databases PubMed and Embase to identify relevant articles was conducted in March 2021. The initial search retrieved 451 articles. After deduplication and screening of abstracts, 56 articles were selected. After reading of the full texts, 19 articles were included in the review. RESULTS: There insufficient evidence to support that people with brain neoplasms have a higher incidence rate of IAs. However, the prevalence of IAs appears to be higher in patients with pituitary tumors than in the general population. The key factors affecting prognosis were tumor type in patients with unruptured aneurysms and progression of subarachnoid hemorrhage in individuals with ruptured aneurysms. Treatment should be individualized according to patient age, tumor pathology, location, and aneurysm rupture risk. CONCLUSION: There is a lack of evidence to affirm that the existence of brain neoplasm plays a role in the formation and rupture of intracranial aneurysms. Additionally, there is insufficient evidence to confirm a greater prevalence of intracranial aneurysms in individuals with brain tumors. The association of these two disorders does not appear to worsen patient outcome. Prognosis depends on tumor pathology for malignant cases and on subarachnoid hemorrhage in patients with ruptured aneurysms.

Posterior fossa choroidplexus papilloma in the pediatric population: case series and literature review.

Choroid plexus papillomas (CPPs) are rare benign neoplasms which are particularly uncommon in the posterior fossa in children. We herein present a case series of five patients treated at a tertiary care hospital. A comprehensive literature review was also carried out. The patients treated at the tertiary care hospital were aged between 4 and 16 years. Gross total resection (GTR) was initially achieved in two patients. All patients showed clinical improvement. Moreover, 27 articles published between 1975 and 2021 were selected for the literature review, totaling 46 patients; with the 5 patients previously described, the total sample was composed of 51 cases, With a mean age was 8.2 years. The lesions were located either in the fourth ventricle (65.3%) or the cerebellopontine angle (34.7%). Hydrocephalus was present preoperatively in 66.7% of the patients, and a permanent shunt was required in 31.6% of the cases. The GTR procedure was feasible in 64.5%, and 93.8% showed clinical improvement. For CPPs, GTR is the gold standard treatment and should be attempted whenever feasible, especially because the role of the adjuvant treatment remains controversial. Neuromonitoring is a valuable tool to achieve maximal safe resection. Hydrocephalus is common and must be recognized and promptly treated. Most patients will need a permanent shunt. Though there is still controversy on its efficacy, endoscopic third ventriculostomy is a safe procedure, and was the authors' first choice to treat hydrocephalus.

Os papilomas do plexo coroide (PPCs) são neoplasias benignas raras e, na população pediátrica, são particularmente incomuns na fossa posterior. Apresentamos uma série de casos de cinco pacientes atendidos em um hospital terciário. Além disso, foi realizada uma ampla revisão da literatura. Os pacientes atendidos no hospital terciário tinham entre 4 e 16 anos. Ressecção macroscópica total (RMT) foi inicialmente realizada em dois pacientes. Todos os pacientes apresentaram melhora clínica. Além disso, 27 artigos publicados entre 1975 e 2021 foram selecionados para a revisão da literatura, totalizando 46 pacientes. Somados à série de casos atuais, encontramos 51 pacientes, com média de idade de 8,2 anos. As lesões localizavam-se no quarto ventrículo (65,3%) ou no ângulo pontocerebelar (34,7%). Hidrocefalia estava presente no pré-operatório em 66,7% dos pacientes, e derivação ventricular permanente foi necessária em 31,6% dos casos. A RMT foi possível em 64,5%, e 93,8% tiveram melhora clínica. Para os CPPs, a RMT é o tratamento padrão-ouro e deve ser tentado sempre que possível, especialmente porque ainda existem controvérsias quanto ao papel do tratamento adjuvante. A neuromonitorização é uma ferramenta importante para se atingir a máxima ressecção segura. A hidrocefalia é comumente vista nesses pacientes e deve ser identificada e tratada. A maioria dos pacientes irá precisar de uma derivação permanente. Apesar de persistirem controvérsias sobre sua eficácia, a terceiro-ventriculostomia endoscópica foi a primeira escolha para tratar a hidrocefalia na experiência dos autores e é uma opção segura.

Comparação de desfechos clínicos e correlação entre funcionalidade e tempo de ventilação mecânica de pacientes em uti / Comparison of clinical outcomes and correlation between functionality and time of mechanical ventilation in icu patients / Comparación de los resultados clínicos y correlación entre la funcionalidad y el tiempo de ventilación mecánica de los pacientes en uti

O objetivo deste estudo foi comparar os desfechos clínicos dos pacientes em suporte ventilatório invasivo por período curto e prolongado e correlacionar funcionalidade e tempo de ventilação mecânica (VM). Estudo documental retrospectivo, realizado na UTI neurocirúrgica de um hospital escola. Dos prontuários clínicos foram coletados: idade, sexo, hipótese diagnóstica de internação, tempo de internação e de VM em dias, o desfecho sucesso ou falha da extubação e o nível de funcionalidade. Os prontuários foram divididos em grupo um (GI): pacientes em VM por até três dias e grupo dois (GII): pacientes em VM por mais de três dias. Foram analisados 210 prontuários, 73% dos pacientes permaneceram menos de três dias em VM. A idade média de GI foi 51,8±15,5 anos e GII 48,7±16,3 anos (p=0,20), prevalência do sexo masculino em GI (59%) e GII (68%). O acidente vascular cerebral foi o diagnóstico mais prevalente no GI (18%) e o tumor cerebral no GII (21%) e hipertensão arterial, a comorbidade mais prevalente em GI (28%) e GII (25%). O GII permaneceu maior tempo (p<0,0001) em VM e internação na UTI que o GI e percentual de sucesso no desmame/extubação menor (p=0,01) que o GI. Não houve correlação significativa entre funcionalidade e tempo de VM em GI e GII (p>0,05). Os pacientes em suporte ventilatório invasivo por período prolongado evoluíram com maior permanência em VM, maior tempo de internação na UTI e menor taxa de sucesso no desmame/extubação. O tempo de permanência em suporte ventilatório invasivo não interferiu na funcionalidade desses pacientes.

The aim of this study was to compare the clinical outcomes of patients on short- and long-term invasive ventilatory support and to correlate functionality and duration of mechanical ventilation (MV). Retrospective documental study, carried out in the neurosurgical ICU of a teaching hospital. The following were collected from the clinical records: age, gender, diagnostic hypothesis of hospitalization, length of hospital stay and MV in days, the outcome of success or failure of extubation and the level of functionality. The medical records were divided into group one (GI): patients on MV for up to three days and group two (GII): patients on MV for more than three days. A total of 210 medical records were analyzed, 73% of the patients remained on MV for less than three days. The mean age of GI was 51.8±15.5 years and GII 48.7±16.3 years (p=0.20), male prevalence in GI (59%) and GII (68%). Stroke was the most prevalent diagnosis in GI (18%) and brain tumor in GII (21%) and hypertension was the most prevalent comorbidity in GI (28%) and GII (25%). GII remained longer (p<0.0001) in MV and ICU admission than GI and the percentage of success in weaning/extubation was lower (p=0.01) than GI. There was no significant correlation between functionality and time on MV in GI and GII (p>0.05). Patients on invasive ventilatory support for a long period evolved with longer MV stays, longer ICU stays and lower weaning/extubation success rates. The length of stay on invasive ventilatory support did not interfere with the functionality of these patients.

El objetivo de este estudio fue comparar los resultados clínicos de los pacientes con soporte ventilatorio invasivo a corto y largo plazo y correlacionar la funcionalidad y el tiempo de ventilación mecánica (VM). Se trata de un estudio documental retrospectivo, realizado en la UCI neuroquirúrgica de un hospital universitario. Se recogieron los siguientes datos de las historias clínicas: edad, sexo, hipótesis diagnóstica, duración de la estancia y tiempo de VM en días, el resultado éxito o fracaso de la extubación y el nivel de funcionalidad. Las historias clínicas se dividieron en el grupo uno (GI): pacientes bajo VM hasta tres días y el grupo dos (GII): pacientes bajo VM durante más de tres días. Se analizaron 210 historias clínicas, el 73% de los pacientes permanecieron menos de tres días con VM. La edad media de GI fue de 51,8±15,5 años y la de GII de 48,7±16,3 años (p=0,20), con prevalencia masculina en GI (59%) y GII (68%). El ictus fue el diagnóstico más prevalente en GI (18%) y el tumor cerebral en GII (21%) y la hipertensión, la comorbilidad más prevalente en GI (28%) y GII (25%). El GII permaneció más tiempo (p<0,0001) en la VM y la estancia en la UCI que el GI y el porcentaje de éxito en el destete/extubación fue menor (p=0,01) que el GI. No hubo correlación significativa entre la funcionalidad y el tiempo de VM en GI y GII (p>0,05). Los pacientes con soporte ventilatorio invasivo a largo plazo evolucionaron con una mayor estancia en la VM, una mayor estancia en la UCI y una menor tasa de éxito de destete/extubación. La duración de la estancia con soporte ventilatorio invasivo no interfirió en la funcionalidad de estos pacientes.

Peritoneal metastasis of a brainstem anaplastic ganglioglioma in a 2-year-old boy: case report and literature review.

Anaplastic gangliogliomas (AGG) are rare tumors of the central nervous system (CNS) that commonly affect children and young adults, with an unusual infratentorial presentation, which is related to hydrocephalus and a worse prognosis. We report a case of a brainstem AGG in a 2-year-old boy who underwent a ventriculoperitoneal shunting (VPS) and later presented peritoneal metastasis. We also reviewed the related literature. Even though rare, disease dissemination through VPS should be sought in patients with CNS tumors and VPS who develop new abdominal symptoms. The early diagnosis and intervention may minimize morbidity and improve quality of life of such patients.

Acute Intracranial Hypertension During Pregnancy: Special Considerations and Management Adjustments.

Pregnancy is associated with a number of pathophysiological changes (including modification of vascular resistance, increased vascular permeability, and coagulative disorders) that can lead to specific (eclampsia, preeclampsia) or not specific (intracranial hemorrhage) neurological complications. In addition to these disorders, pregnancy can affect numerous preexisting neurologic conditions, including epilepsy, brain tumors, and intracerebral bleeding from cerebral aneurysm or arteriovenous malformations. Intracranial complications related to pregnancy can expose patients to a high risk of intracranial hypertension (IHT). Unfortunately, at present, the therapeutic measures that are generally adopted for the control of elevated intracranial pressure (ICP) in the general population have not been examined in pregnant patients, and their efficacy and safety for the mother and the fetus is still unknown. In addition, no specific guidelines for the application of the staircase approach, including escalating treatments with increasing intensity of level, for the management of IHT exist for this population. Although some of basic measures can be considered safe even in pregnant patients (management of stable hemodynamic and respiratory function, optimization of systemic physiology), some other interventions, such as hyperventilation, osmotic therapy, hypothermia, barbiturates, and decompressive craniectomy, can lead to specific concerns for the safety of both mother and fetus. The aim of this review is to summarize the neurological pathophysiological changes occurring during pregnancy and explore the effects of the possible therapeutic interventions applied to the general population for the management of IHT during pregnancy, taking into consideration ethical and clinical concerns as well as the decision for the timing of treatment and delivery.

Alternative language paradigms for functional magnetic resonance imaging as presurgical tools for inducing crossed cerebro-cerebellar language activations in brain tumor patients.

OBJECTIVES: Crossed cerebro-cerebellar BOLD activations have recently come to light as additional diagnostic features for patients with brain tumors. The covert verb generation (VG) task is a widely used language paradigm to determine these language-related crossed activations. Here we demonstrate these crossed activations in two additional language paradigms, the semantic and phonological association tasks. We propose the merit of these tasks to language lateralization determination in the clinic as they are easy to monitor and suitable for patients with aphasia. METHODS: Patients with brain tumors localized at different cortical sites (n = 71) performed three language paradigms, namely the VG task as well as the semantic (SA) and phonological (PA) association tasks with button-press responses. Respective language activations in disparate cortical regions and the cerebellum were assigned laterality. Agreements in laterality between the two new tasks and the verb generation task were tested using Cohen's kappa. RESULTS: Both tasks significantly agreed in cortical and cerebellar lateralization with the verb generation task in patients. Additionally, a McNemar test confirmed the presence of crossed activations in the cortex and the cerebellum in the entire subject population. CONCLUSION: We demonstrated that the semantic and phonological association tasks resulted in crossed cerebro-cerebellar language lateralization activations as those observed due to the covert verb generation task. This may suggest the possibility of these tasks being used conjointly with the traditional verb generation task, especially for subjects that may be unable to perform the latter. KEY POINTS: • The semantic and phonological association tasks can be useful as additional presurgical fMRI language lateralization paradigms for brain tumor patients along with the standard verb generation task. • All three tasks also confirm the presence of crossed cerebro-cerebellar language activations in the current subject population.

[Germinoma in a patient with emaciation and growth retardation. Case report]. / Germinoma en un paciente con emaciación y retraso de crecimiento. A propósito de un caso.

Central nervous system tumors are the second most frequent oncological disease among children and teenagers. Among the intracranial tumors, the germ cells ones are infrequent. The symptoms they cause are headaches, nausea and vomiting, hormonal deficits, visual disturbances, weight loss, poor growth and early puberty. Less frequently, they produce movement or psychiatric disorders. Some of these tumors can be asymptomatic for a long period leading to a late diagnosis. The case of a 14-year-old female patient is presented. She showed weight loss and growth failure, with wrong diagnosis of eating disorder. After proper study methods, we arrived to central nervous system germinoma diagnosis. Because this pathology is rare and has a variable form of presentation, it requires that the family involved and the health team to be alert, to avoid delays in diagnosis and treatment.

Los tumores del sistema nervioso central representan la segunda enfermedad oncológica más habitual en niños y adolescentes. Entre los tumores intracraneales, los de células germinales son infrecuentes. Los síntomas que desencadenan son cefalea, náuseas, vómitos, déficits hormonales, alteraciones visuales, pérdida de peso, pobre crecimiento y pubertad precoz. Menos frecuentemente, producen trastornos del movimiento o psiquiátricos. Algunos de estos tumores pueden ser asintomáticos un largo período, lo que desencadena un diagnóstico tardío. Se presenta a una paciente femenina de 14 años con pérdida de peso y falla del crecimiento, con diagnóstico erróneo de trastorno de la conducta alimentaria. Tras estudios pertinentes, se arribó al diagnóstico de germinoma del sistema nervioso central. Al ser esta patología infrecuente y de presentación variable, requiere alto sentido de alerta por parte de la familia involucrada y del equipo de salud para evitar retrasos en el diagnóstico y el tratamiento.

Secondary stabbing headache associated with intracranial tumors, aneurysms, and arteriovenous malformation: An alarming warning sign.

BACKGROUND: Stabbing headache (SH) is considered as a pure primary headache, but according to a few clinical observations it could also be secondary. Over the past decades, we have been observing the complaint of SH in patients with intracranial vascular and neoplastic lesions. OBJECTIVE: To describe a series of patients with intracranial lesions who experienced SH. METHODS: This is a cross-sectional, retrospective study of 34 patients with intracranial lesions associated with SH, admitted at Hospital das Clínicas, Federal University of Pernambuco, Brazil. RESULTS: In this series of 34 patients [29 women, 44 ± 12 years (mean ± SD)] with secondary SH, the causes were intracranial neoplasms (n = 31), cerebral aneurysms (n = 2), or arteriovenous malformation (n = 1). Pituitary tumor (n = 18), meningioma (n = 6), and vestibular schwannomas (n = 4) were the most prevalent types of intracranial neoplasms. All these lesions had intimate contact with the dura mater, including an oligodendroglioma, the only intra-axial tumor in the series. A characteristic in the secondary SH is the crescendo pattern (12/34, 35%), progressing from infrequent attacks to recurrent crises occurring several times a day. The SH lasted from 5 days to 60 months (15 ± 18 months, mean ± SD) until the correct diagnosis [16/34 (47%) of the patients ≤6 months]. The SH was triggered by the movement of the head (5/34, 15%) or Valsalva maneuver (1/34). After surgery, suppression of the SH was observed. In a few of the patients to whom dexamethasone was prescribed, the SH subsided within a few days. CONCLUSION: This study was able to identify clinical red flags associated with intracranial lesions and secondary SH, for example, recent onset of SH, exclusively unilateral (ipsilateral) at the same location, crescendo pattern, triggered by head movements, or Valsalva maneuver.

Gastrointestinal Hemorrhage: A Manifestation of the Telomere Biology Disorders.

OBJECTIVE: To describe the clinical features, therapeutic interventions, and patient outcomes of gastrointestinal (GI) hemorrhage in individuals with a telomere biology disorder, including dyskeratosis congenita, Hoyeraal-Hreidarsson syndrome, Revesz syndrome, and Coats plus. STUDY DESIGN: Clinical Care Consortium for Telomere Associated Ailments members were invited to contribute data on individuals with telomere biology disorders at their institutions who experienced GI bleeding. Patient demographic, laboratory, imaging, procedural, and treatment information and outcomes were extracted from the medical record. RESULTS: Sixteen patients who experienced GI hemorrhage were identified at 11 centers. Among 14 patients who underwent genetic testing, 8 had mutations in TINF2, 4 had mutations in CTC1 or STN1, and 1 patient each had a mutation in TERC and RTEL1. Ten patients had a history of hematopoietic cell transplantation. The patients with Coats plus and those without Coats plus had similar clinical features and courses. Angiodysplasia of the stomach and/or small bowel was described in 8 of the 12 patients who underwent endoscopy; only 4 had esophageal varices. Various medical interventions were trialed. No single intervention was uniformly associated with cessation of bleeding, although 1 patient had a sustained response to treatment with bevacizumab. Recurrence was common, and the overall long-term outcome for affected patients was poor. CONCLUSIONS: GI bleeding in patients with telomere biology disorders is associated with significant morbidity and with vascular ectasias rather than varices.

Glioblastoma de células gigantes: informe de un caso / Giant cell glioblastoma: case report

Antecedentes: El Glioblastoma (GB) o astrocitoma grado IV, es un tumor agresivo que se origina de células gliales, con alto grado de malignidad, prevalencia menor al 1% en fosa posterior e incidencia menor al 0.5% de todos los GB. Actualmente se describen alrededor de 75 casos a nivel mundial. Descripción del caso clínico: Femenina, 24 años, referida a emergencia de Neurocirugía del Hospital Escuela Universitario, presentó cefalea holocraneana intensa, vómitos, náuseas, visión borrosa, vértigo y anorexia. Al examen neurológico mostró discreta adiadococinesia derecha y signos de papiledema. La tomografía axial computarizada cerebral evidenció lesión heterogénea en vermis extendido a hemisferio cerebeloso derecho, por lo que se realizó craniectomía suboccipital, abordaje transcerebelar, con citorreducción tumoral, encontrando masa vascularizada con componente quístico. Estudio anatomopatológico evidenció glioblastoma multiforme variante de células gigantes, confirmado con tinción de inmunohistoquímica (PFGA, CD34+ y vimentina). Paciente con buena evolución clínica postquirúrgica, egresada sin déficit neurológico. 16 meses después, presentó síndrome de recidiva tumoral y complicaciones, por lo que se reintervino en 4 ocasiones, posterior a recibir 30 dosis de radioterapia y 12 ciclos de quimioterapia, se reingresó con deterioro neurológico progresivo, signos meníngeos y síndrome de Parinaud, escala de Karnofsky (30 puntos), realizándose derivación ventrículo-peritoneal por compresión del IV ventrículo e hidrocefalia obstructiva secundaria, luego desarrolló neumonía intrahospitalaria, falleciendo a las dos semanas. Conclusiones: Es importante identificar la variante biológica del glioblastoma de forma temprana, para determinar pronóstico y acciones terapéuticas que influirán en la calidad de vida, así como la supervivencia...(AU)

Analysis of Intraoperative Seizures During Bipolar Brain Mapping in Eloquent Areas: Intraoperative Seizures in brain mapping.

OBJECTIVES: Awake Craniotomy (AC) is a very well described technique that is performed to make an adequate tumor resection preserving the functionality of the patient. Intraoperative Seizures (IS) are reported as a failure of such procedure. We analyze the incidence and risk factor during AC. METHODS: We made a review of the database of the National Institute of Neurology and Neurosurgery between January 2017 and May 2019 for intrinsic tumors located in eloquent areas of the brain. An analysis of ISconcerning the clinical history, clinical presentation, imaging techniques, histological findings and surgical technique was made. The factors associated with Mapping Failure (MF) were also evaluated. RESULTS: 45 patients were included of whom 7 patients (15.6%) developed IS after cortical-subcortical stimulation, 5 presented partial motor seizures (11.1%) and 2 experimented generalized secondary seizures (4.5%). Of the patients that had a MF, one patient (14%) was due to generalized tonic-clonic seizures which couldn't be managed by cold saline irrigation and administration of anti-seizures drugs and was then converted to a general anesthetic technique. We observed that the patients that had a bigger tumoral volume (112.2 cm3 85.3, P = 0,07) had a bigger positive relation in presenting IS, having a peak sensibility and specificity above 70 cc (ROC). CONCLUSIONS: In our analysis IS are more common in patients with high presurgical tumor volume. Even though the majority of the patients that presented IS didn't develop MF, it is important to acknowledge that the multidisciplinary group in the operating room must be prepared to detect these complications, treat them promptly and avoid MF.

Dysembryoplastic neuroepithelial tumor and temporal arachnoid cyst on a child with epilepsy. Which one to operate? Case report and literature review.

A 6 year-old male, with seizures characterized by abnormal epigastric sensation, behavioral arrest, upper extremities search automatisms and secondary tonic-clonic generalization. Magnetic resonance imaging showed a hypointense cystic extra-axial image with an increase in the thickness of the convolutions in the first and second gyri of the right frontal lobe. It was decided to resect the frontal lesion with transoperative motor mapping. Morphological and immunohistochemical findings corresponded to dysembryoplastic neuroepithelial tumor with focal cortical dysplasia. Adequate semiology, analysis of the electroencephalogram, and imaging studies allowed treating adequately the cortical dysplasia. At present, the patient is seizure-free without medication (Engel IA).

Niño de 6 años con crisis caracterizadas por sensación epigástrica, arresto conductual, automatismos de búsqueda y generalización tónico-clónica secundaria. La resonancia magnética mostró una imagen extraaxial quística y un aumento del grosor de las circunvoluciones del primer y segundo giros del lóbulo frontal derecho. Se decidió resecar primero la lesión frontal con guía por mapeo transcortical intraoperatorio. Los hallazgos morfológicos e inmunohistoquímicos mostraron un tumor neuroepitelial disembrioplásico con displasia cortical focal. La semiología, el análisis del electroencefalograma y la imagen permitieron orientar el tratamiento. Actualmente el paciente está libre de crisis y sin medicamentos (Engel IA).