High-flow carotid cavernous fistula and 'white eye': A rare and easily misdiagnosed presentation - A case report and literature review.

The three classic symptoms of carotid cavernous fistula (CCF) are pulsating exophthalmos, bruit and conjunctival chemosis. Here, we present a clinical case of isolated abducens nerve palsy due to a high-flow CCF in an 84-year-old woman, without the typical congestive orbito-ocular features. It was a diagnostic challenge because, for patients older than 50 years with cardiovascular risk factors, ischaemic mononeuropathy is the most frequent aetiology. This case illustrates the least common type of CCF that can be easily misdiagnosed. Physicians should consider fistula as a possible diagnosis in a patient with isolated abducens nerve palsy even without the classic triad.

[Cerebral infarction in young patients: clinical characteristics and evolution in an Argentine cohort]. / Infarto cerebral en pacientes jóvenes: características clínicas y evolución en una cohorte argentina.

INTRODUCTION: Stroke (CVA) in young adults comprises approximately 10% of all cerebrovascular events. The information available on the recurrence of a new event in this population and particularly in Latin America is limited. Our objective was to examine the presence of stroke recurrence after having presented a stroke. METHODS: A retrospective cohort study was carried out, including patients with arterial cerebral infarctions between the ages of 18 and 55, between January 2005 and May 2020. The main outcome was the recurrence of a cerebrovascular attack. RESULTS: 138 patients were included during a median follow-up of 24 months. The 52.2% (n = 72) were male patients and 73.4% (n = 94) had an initial NIHSS score of less than 4. The 38% (n = 52) had a history of arterial hypertension and 13, 1% (n = 18) history of previous stroke / TIA. 13% (n = 18) presented recurrence during their follow-up. DISCUSSION: The recurrence of neurovascular events occurs predominantly in patients with a history of previous stroke/TIA, probably secondary to diseases that are difficult to diagnose.

Introducción: El ataque cerebral (ACV) en adultos jóvenes comprende aproximadamente el 10% de todos los eventos cerebrovasculares. La información disponible sobre la recurrencia de un nuevo evento en esta población y particularmente en Latinoamérica es limitada. Nuestro objetivo fue examinar la presencia de recurrencia de ACV luego de haber presentado un infarto cerebral. Métodos: Se realizó un estudio de cohorte retrospectivo, incluyendo pacientes con infartos cerebrales arteriales en edades comprendidas entre 18 y 55 años, entre enero de 2005 a mayo de 2020. El resultado principal fue la recurrencia de un ataque cerebrovascular. Resultados: Se incluyeron 138 pacientes durante una mediana de seguimiento de 24 meses. El 52.2% (n = 72) de sexo masculino y el 73,4% (n = 94) tuvo un NIHSS inicial menor a 4. El 38% (n = 52) tenía antecedentes de hipertensión arterial y 13,1% (n = 18) antecedentes de ACV / ataque isquémico transitorio (AIT) previo. El 13% (n = 18) presento recurrencia durante su seguimiento. Discusión: La recurrencia de los eventos neurovasculares ocurren predominantemente en pacientes con antecedentes de ACV/AIT previo, probablemente secundario a enfermedades de difícil diagnóstico.

Endoscopic retrograde cholangiopancreatography for the management of choledocholithiasis in older patients.

BACKGROUND: Older adults are increasing in number and frequently seeking hospital care for acute illness. We aimed to measure the utilization and safety of ERCP in patients aged 85 and older in our hospital. METHODS: This is a single-site, retrospective, observational and descriptive study. We obtained data about admissions and ERCP utilization from our hospital database. We reviewed medical and procedural records of patients aged 85 or older who underwent ERCP for choledocholithiasis between 2013 and 2019. We evaluated technical and medical adverse events after ERCP. RESULTS: 494 ERCP for choledocholithiasis were performed during this period and 154 (31%) were aged 85 or older. We identified 567 (4.8%) admissions for biliary tract diseases in the older population, and 27% of cases had required ERCP. In older patients, the rate of technical adverse events was around 10%. There was no statistical difference between the older and younger groups regarding technical complications (8.8 vs 9.7%; p=0.7). Besides, in 36% of cases, a medical event, decompensated comorbidity or geriatric syndromes appeared after ERCP. The overall mortality for any cause at six months was nearly 20%, and the survival rate was significantly lower in patients who develop adverse events (technical or medical). CONCLUSIONS: Our hospital experiences frequent utilization of ERCP by patients aged 85 and older. Although technical adverse event rates are similar to those of younger adults, medical events appear frequently. We plan to strengthen our care plans for older adults and hope to reduce the medical complications experienced post ERCP.

[Stiff person syndrome associated with thymoma]. / Síndrome de la persona rígida asociado a timoma.

Stiff-person syndrome is a rare neurological condition characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostration. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detection of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We report a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

El síndrome de persona rígida es un cuadro neurológico infrecuente caracterizado por rigidez muscular de tronco y extremidades y espasmos musculares gatillados por estímulos sensoriales o emocionales, que progresa hacia la postración. Cuenta con un mecanismo fisiopatogénico con base inmunológica, en el cual los autoanticuerpos, como el antiGAD65, cumplen un rol central. Asimismo, la detección de dichos anticuerpos corrobora el diagnóstico ante un paciente con cuadro clínico sugestivo. Un 4 a 6% de los casos tienen neoplasias subyacentes. El tratamiento se basa en el manejo sintomático, inmunomodulador y de la enfermedad de base en los casos paraneoplásicos. Reportamos un caso de síndrome de persona rígida clásico asociado a timoma y describimos las características principales de esta entidad.

Síndrome de la persona rígida asociado a timoma / Stiff person syndrome associated with thymoma

Resumen El síndrome de persona rígida es un cuadro neuro lógico infrecuente caracterizado por rigidez muscular de tronco y extremidades y espasmos musculares ga tillados por estímulos sensoriales o emocionales, que progresa hacia la postración. Cuenta con un mecanismo fisiopatogénico con base inmunológica, en el cual los autoanticuerpos, como el antiGAD65, cumplen un rol central. Asimismo, la detección de dichos anticuerpos corrobora el diagnóstico ante un paciente con cuadro clínico sugestivo. Un 4 a 6% de los casos tienen neoplasias subyacentes. El tratamiento se basa en el manejo sintomático, inmunomodulador y de la enfermedad de base en los casos paraneoplásicos. Reportamos un caso de síndrome de persona rígida clásico asociado a timoma y describimos las características principales de esta entidad.

Abstract Stiff-person syndrome is a rare neurological condi tion characterized by muscular rigidity of the trunk and extremities and muscle spasms triggered by sensory or emotional stimuli, which progresses towards prostra tion. It has a pathophysiogenic mechanism with an immunological basis, in which autoantibodies, such as antiGAD65, play a central role. Likewise, the detec tion of these antibodies corroborates the diagnosis in a patient with a suggestive clinical picture. Four to 6% of cases have underlying neoplasms. Treatment is based on symptomatic, immunomodulatory, and underlying disease management in paraneoplastic cases. We re port a case of classic stiff person syndrome associated with thymoma and review the main characteristics of this entity.

Pseudotumoral Presentation of Cerebral Amyloid-Beta Angiopathy: Case Report and Review of Literature.

OBJECTIVE: Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare and potentially treatable encephalopathy that usually affects people older than 50 years old and has an acute or subacute clinical presentation characterized by rapidly evolving cognitive decline, focal deficits and seizures. In a small subset of patients the disease can adopt a pseudotumoral form in the neuroimages that represents a very difficult diagnostic challenge. METHODS: Here in we report a patient with a tumour-like presentation of histopathologically confirmed CAA-RI. RESULTS: We also conducted a search and reviewed the clinical and radiological features of 41 cases of pseudotumoral CAA-RI previously reported in the literature in order to identify those characteristics that should raise diagnostic suspicions of the disease, there by avoiding unnecessary surgical treatments. CONCLUSION: The therapy of CAA-RI with steroids is usually effective and clinical and radiological remission can be achieved in the first month in approximately 70% of cases.

[Long-term psychosocial impact in young stroke survivors: a frequent but often under diagnosed complication]. / Impacto psicosocial del ataque cerebral en pacientes jóvenes: una complicación frecuente y habitualmente sub-diagnosticada.

INTRODUCTION: Approximately 15% of all strokes occur in young patients, affecting them in the most productive years of their lives. Currently, there is limited information (particularly in Latin America) regarding the long-term psychosocial consequences of stroke in young patients. Therefore, the objective of our study was to analyze the functional impact of stroke in this group of patients, regarding both cognitive and psychosocial aspects. MATERIAL AND METHODS: A Beck Depression Inventory (BDI) was administered to outpatients with ischemic stroke between 16 and 55 years of age in two centers of Argentina. The following variables were compared in depressed and non-depressed individuals: NIHSS, modified Rankin Score, Mini-Mental State Examination, Barthel Index, as well as clinical-demographic variables. A BDI score greater than 10 was considered as marker of depression. RESULTS: Thirty-four patients with cerebral infarction were included, 67% (n = 23) were women, mean age: 45.53 ± 9.78 years (range: 21-59). Eleven patients (33%) had depression; 50% of the population was unable to continue with their previous job and 15% divorced or separated from their partner after the vascular event. The stroke recurrence rate was 0.03%. CONCLUSIONS: A high proportion of patients with depressive symptoms was observed. It should be noted that, in the majority, symptoms of depression had not been recognized and the patients were under-treated. Likewise, depression spread persistently after several years of the cerebrovascular event. Likewise, a significant proportion of patients were not able to re-insert themselves into their usual work activity. Moreover, stroke also had an important impact on their affective relationships. Treatment of depression after stroke should be particularly considered in these individuals due to their long-term survival, and should be offered to achieve the highest possible quality of recovery after stroke.

Ischemic stroke of the "hand knob area": A case series and literature review.

Isolated hand paresis may reflect an infarction of the "hand knob area", which represents less than 1% of all ischemic strokes. In this type of stroke, a potential source of embolism is often identified. There are no large case series regarding this topic in Latin America. Herein we present the largest cohort of this entity in the region and we compare our results with those previously published. We analyzed all stroke patients admitted to our hospital between May 2015 - June 2018. Only patients with motor +/- sensory deficits restricted to the hand and ischemic stroke confirmed by MRI were included. We assessed stroke mechanism, clinical characteristics and outcome. From 339 patients admitted with ischemic stroke, 12 (3.53%) were included (9 men, 75%). Mean age: 60.4 years-old (range:24-79). Localization of stroke: 8 patients (66%) precentral gyrus, 3 (25%) postcentral; in 1 both gyri were affected. Stroke mechanism according to TOAST classification was as follows: two patients (16%) large artery atherosclerosis, two cardioembolic, one other determined etiology (thrombophilia), seven (58%) undetermined etiology (SUE). Nine patients (75%) received antiplatelets and statins, and three (25%) anticoagulants. The mean follow-up period was 11 months (range 1-26). Stroke recurrence was observed in one patient. At follow up, eight patients (66%) had a modified Rankin Score (mRS) of 0 and one a mRS of 1. In conclusion, in this series the most prevalent stroke mechanism was SUE, mainly embolic stroke of undetermined source. The outcome was excellent regardless of stroke mechanism.

Impact of Infarct Size on Blood Pressure in Young Patients with Acute Stroke.

BACKGROUND: Hypertension can be found in up to 80% of patients with acute stroke. Many factors have been related to this phenomenon such as age, history of hypertension, and stroke severity. The aim of our study was to determine the relationship between infarct volume and blood pressure, at admission, in young patients with acute ischemic stroke. MATERIALS AND METHODS: Patients younger than 55 years old admitted within 24 hours of ischemic stroke were included. Socio-demographic variables, systolic blood pressure, diastolic blood pressure, and infarct volume at admission were assessed. Statistical analysis: mean and SEM for quantitative variables, percentages for qualitative, and Spearman correlations (p value < 0.05 was considered statistically significant). RESULTS: Twenty-two patients (12 men), mean age: 44.64 ± 1.62 years. The most frequent vascular risk factors were: hypertension, smoking, and overweight (40.9%). Mean systolic and diastolic blood pressure on admission were: 143.27 ± 6.57 mmHg and 85.14 ± 3.62 mmHg, respectively. Infarct volume: 11.55 ± 4.74 ml. Spearman correlations: systolic blood pressure and infarct volume: p = 0.15 r: -0.317; diastolic blood pressure and infarct volume: p = 0.738 r: -0.76. CONCLUSION: In our series of young patients with acute ischemic stroke, large infarct volume was not associated with high blood pressure at admission.

Convexity Subarachnoid Hemorrhage Secondary to Adalidumab in a Patient with Ulcerative Colitis.

The TNF-α antagonists are the drugs used for the treatment of ulcerative colitis (UC). Nontraumatic convexity subarachnoid hemorrhage is an infrequent nonaneurysmal subtype of subarachnoid bleeding caused mainly by reversible cerebral vasoconstriction syndrome (RCVS), cerebral amyloid angiopathy, and posterior reversible encephalopathy syndrome (PRES). We present a 26-year-old female patient with a diagnosis of UC taking Adalimumab. She received her last doses the same day she was admitted to our hospital for an acute severe UC exacerbation. Steroids were added to the treatment. Five days after admission she presented a thunderclap headache with photophobia, nausea, and vomiting. An MRI was performed showing left frontal convexity subarachnoid hemorrhage and hyperintense lesions on T2-weighted and FLAIR sequences located in both occipital lobes, left cerebellar hemisphere, and brainstem. Digital angiography was unremarkable. Adalimumab was discontinued but persisted on treatment with steroids. The patient evolved with complete resolution of her symptoms and was discharged with a normal neurological exam. Two months later, she was asymptomatic and her MRI revealed superficial siderosis secondary to cSAH with resolution of white matter hyperintensities. Convexity subarachnoid hemorrhage in our patient could be secondary to PRES or to RCVS. Analogous MRI findings can be observed in both syndromes, along with similar clinical and angiographic findings. This suggests that both conditions may reflect different manifestations of the same pathology, in which vascular tone and endothelial dysfunction play a major role. To our knowledge, this is the first report of a patient with severe UC and convexity subarachnoid hemorrhage associated with Adalimumab.

Blood pressure evolution in young patients with acute ischemic stroke: a new model for understanding the natural course of spontaneous hypertension?

BACKGROUND: Approximately 80% of patients suffering an acute ischemic stroke develop transient hypertension. The physiopathological mechanism remains unclear. Due to the lack of vascular risk factors, young adults could be a useful model for understanding blood pressure (BP) evolution in this setting. METHODS: Patients between 18 and 55 years old admitted with an acute ischemic stroke were included. BP was evaluated during the following 48 h. Hypertension was defined as: systolic blood pressure (SBP) ≥140 mmHg or diastolic blood pressure (DBP) ≥90 mmHg. Patients were divided into two groups: RF group included those who had a previous vascular risk factor and/or other medical condition known to affect the autonomic function; noRF group included patients without any of the previously stated conditions. RESULTS: Thirty-nine patients were included: mean age: 44.26 years old, 21 were males (53.8%). Mean SBP and DBP at admission were 139.77 ± 5.35 mmHg (range: 90-243) and 84.44 ± 3.02 mmHg (range: 60-128), respectively; 43.58% patients were hypertensive at admission. SBP decreased significantly during the first 48 h (p = 0.044) for the entire population. RF group has higher SBP (p = 0.009) and DBP (p = 0.011) at admission than the noRF group. CONCLUSION: Most patients were normotensive at admission and BP fell spontaneously despite BP at admission. Young patients without medical conditions that could alter the autonomic system function could be a useful model for understanding acute hypertension in ischemic stroke.

Treatable inherited rare movement disorders.

There are many rare movement disorders, and new ones are described every year. Because they are not well recognized, they often go undiagnosed for long periods of time. However, early diagnosis is becoming increasingly important. Rapid advances in our understanding of the biological mechanisms responsible for many rare disorders have enabled the development of specific treatments for some of them. Well-known historical examples include Wilson disease and dopa-responsive dystonia, for which specific and highly effective treatments have life-altering effects. In recent years, similarly specific and effective treatments have been developed for more than 30 rare inherited movement disorders. These treatments include specific medications, dietary changes, avoidance or management of certain triggers, enzyme replacement therapy, and others. This list of treatable rare movement disorders is likely to grow during the next few years because a number of additional promising treatments are actively being developed or evaluated in clinical trials. © 2017 International Parkinson and Movement Disorder Society.

Convexity subarachnoid hemorrhage: clinical features and etiology of an Argentinian cohort.

Nontraumatic convexity subarachnoid hemorrhage is an increasingly recognized subtype of subarachnoid bleeding. OBJECTIVE: Our aim was to describe the etiology and clinical features of a cohort of patients with convexity subarachnoid hemorrhage. METHODS: We retrospectively analyzed all cases of convexity subarachnoid hemorrhage admitted to our hospital between January 2012 and April 2017. Demographic features, clinical characteristics, complementary investigations, etiology and mortality were assessed. Twenty patients (65% females) were identified. Mean age: 53 years (range, 15-86 years). RESULTS: Symptoms on admission: headache (65%), sensory and/or motor symptoms (50%) and seizures (35%). Commonest causes: cerebral vein thrombosis (20%), reversible cerebral vasoconstriction syndrome (20%) and cerebral amyloid angiopathy (20%). Two patients died. CONCLUSION: Convexity subarachnoid hemorrhage may be related to a wide spectrum of etiologies. In our patients, an increased prevalence of cerebral vein thrombosis was observed. Mortality was low and not related to the bleeding itself.

Convexity subarachnoid hemorrhage: clinical features and etiology of an Argentinian cohort / Hemorragia subaracnóidea da convexidade: características clínicas e etiologia de uma coorte argentina

ABSTRACT Nontraumatic convexity subarachnoid hemorrhage is an increasingly recognized subtype of subarachnoid bleeding. Objective: Our aim was to describe the etiology and clinical features of a cohort of patients with convexity subarachnoid hemorrhage. Methods: We retrospectively analyzed all cases of convexity subarachnoid hemorrhage admitted to our hospital between January 2012 and April 2017. Demographic features, clinical characteristics, complementary investigations, etiology and mortality were assessed. Twenty patients (65% females) were identified. Mean age: 53 years (range, 15-86 years). Results: Symptoms on admission: headache (65%), sensory and/or motor symptoms (50%) and seizures (35%). Commonest causes: cerebral vein thrombosis (20%), reversible cerebral vasoconstriction syndrome (20%) and cerebral amyloid angiopathy (20%). Two patients died. Conclusion: Convexity subarachnoid hemorrhage may be related to a wide spectrum of etiologies. In our patients, an increased prevalence of cerebral vein thrombosis was observed. Mortality was low and not related to the bleeding itself.

RESUMO A hemorragia subaracnóidea não traumática da convexidade é um subtipo cada vez mais reconhecido de sangramento subaracnóideo. Objetivo: Nosso objetivo foi descrever a etiologia e as características clínicas de uma coorte de pacientes com hemorragia subaracnóidea da convexidade. Métodos: Foram analisados retrospectivamente todos os casos de hemorragia subaracnóidea da convexidade admitidos em nosso hospital entre janeiro de 2012 e abril de 2017. Foram avaliados os aspectos demográficos, características clínicas, investigações complementares, etiologia e mortalidade. Vinte pacientes (65% mulheres) foram identificados. Média de idade: 53 anos (intervalo, 15-86). Resultados: Sintomas na admissão: dor de cabeça (65%), sintomas sensitivos e/ou motores (50%) e convulsões (35%). Causas mais comuns: trombose venosa cerebral (20%), síndrome de vasoconstrição cerebral reversível (20%) e angiopatia amilóide cerebral (20%). Dois pacientes morreram. Conclusão: A hemorragia subaracnóidea da convexidade pode estar relacionada a um amplo espectro de etiologias. Em nossos pacientes, observou-se uma maior prevalência de trombose venosa cerebral. A mortalidade foi baixa e não relacionada à própria hemorragia.

Experiences and lessons learned for planning and supply of micronutrient powders interventions.

Realistic planning for a nutrition intervention is a critical component of implementation, yet effective approaches have been poorly documented. Under the auspices of "The Micronutrient Powders Consultation: Lessons Learned for Operational Guidance," 3 working groups were formed to summarize experiences and lessons across countries regarding micronutrient powders (MNP) interventions for young children. This paper focuses on programmatic experiences in the planning stages of an MNP intervention, encompassing assessment, enabling environment and adaptation, as well as considerations for supply. Methods included a review of published and grey literature, key informant interviews, and deliberations throughout the consultation process. We found that assessments helped justify adopting an MNP intervention, but these assessments were often limited by their narrow scope and inadequate data. Establishing coordinating bodies and integrating MNP into existing policies and programmes have helped foster an enabling environment and support programme stability. Formative research and pilots have been used to adapt MNP interventions to specific contexts, but they have been insufficient to inform scale-up. In terms of supply, most countries have opted to procure MNP through international suppliers, but this still requires understanding and navigating the local regulatory environment at the earliest stages of an intervention. Overall, these findings indicate that although some key planning and supply activities are generally undertaken, improvements are needed to plan for effective scale-up. Much still needs to be learned on MNP planning, and we propose a set of research questions that require further investigation.

Alcohol-Sensitive Generalized Dystonia.

We report the case of a 29-year-old male patient with a generalized and progressive dystonia that led him unable to stand. Multiple antidystonic treatments were tried without benefit. Alcohol test was positive with a dramatic improvement. To the best of our knowledge, this is the first reported case of generalized dystonia without other clinical manifestations sensitive to alcohol.

Analysis of D216H polymorphism in Argentinean patients with primary dystonia.

The D216H polymorphism (rs1801968) in TOR1A has been suggested as a risk factor for developing primary dystonia in German subjects not carrying the deletion c.904-906delGAG (∆GAG). However, this association could not be confirmed in other populations with different ethnic backgrounds. The purpose of this study is to evaluate the D216H polymorphism in an Argentinean cohort of 40 patients with primary dystonia and 200 unrelated control subjects. The authors could observe a significantly higher frequency of the H216 variant in dystonic patients lacking ∆GAG as compared with controls.

Vaginal estrogen supplementation during Depo-Provera initiation: a randomized controlled trial.

BACKGROUND: Irregular bleeding is often cited as the reason for discontinuation of depot-medroxyprogesterone acetate (DMPA) after the first injection. Estrogen supplementation during DMPA initiation may decrease bleeding and improve continuation. STUDY DESIGN: This prospective, randomized, controlled trial evaluated estrogen supplementation during DMPA initiation. Women initiating DMPA were randomized to receive an estradiol vaginal ring for 3 months versus DMPA alone. Bleeding diaries and questionnaires at three and 6 months assessed bleeding, continuation and ring acceptability. RESULTS: Seventy-one participants enrolled; 49 completed the first follow-up period. The median number of bleeding or spotting days was 16 in the estrogen ring group (n=26) versus 28 in the DMPA alone group (n=23) (p=.19). Seventy-seven percent of the intervention group received a second injection compared with 70% in the DMPA alone group (p=.56). For each additional day of bleeding and/or spotting reported, women were 3% less likely to receive a second injection (OR 0.97, 95% CI 0.94-0.99). Acceptability of the vaginal ring was high among those in the intervention group. CONCLUSIONS: Vaginal estrogen supplementation during DMPA initiation is acceptable to women and may decrease total bleeding.