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.

[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.

[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.

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.

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.

Use of complementary and alternative therapies in outpatients with Parkinson's disease in Argentina.

We interviewed 300 patients (54.7% male; mean age was 65.8 ± 9.5) attending the Movement Disorders Clinic at the Buenos Aires University Hospital to determine the prevalence of CATs use and their association with demographic, social, or disease-specific characteristics among patients with Parkinson's disease (PD) in Buenos Aires, Argentina. We found that 25.7% of the PD patients interviewed (77/300) stated they had used CATs to improve their PD symptoms whereas 38.0% (114/300) had used some CATs without any relation to PD, at least once in life. At the moment of the interview, CATs prevalence use was 50.6% in the former group and 25.0% in the latter. The use of CATs was much more frequent among women and more common in the 50- to 69-year age group. Friends and neighbors of the patients had most frequently recommended these therapies. No major association was observed between CATs use and the duration of the disease, side of initial involvement, PD phenotype, or the Hoehn and Yahr staging. Acupuncture, homeopathy, yoga, and therapeutic massage were the most widely used therapies. After the initiation of conventional treatment the use of massage, yoga, and acupuncture in patients using CATs to improve PD significantly increased. Neurologists should be aware and inquire about the use of CATs to rule out potentially harmful effects.

Vascular hemichorea/hemiballism and topiramate.

Although vascular hemichorea/hemiballism (HC/HB) has been reported to be self-limited, in some cases, it can be irreversible and severely disabling. The standard treatment includes typical and atypical neuroleptics and GABA-mimetic drugs. Topiramate is a new antiepileptic drug possessing a complex mechanism of action, including the enhancement of GABA-mediated inhibition. We describe a 71-year-old patient with HC/HB who markedly improved after topiramate treatment.