Transient Global Amnesia Recurrence: Prevalence and Risk Factor Meta-analysis.

Background and Objectives: Transient global amnesia (TGA) is an acute amnestic disorder with unclear pathophysiology. Although considered a benign phenomenon, the possibility of a recurrence is a major concern for the patient. Our objective is to identify the prevalence and risk factors of relapse to help clinicians counsel patients about it. Methods: According to the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidance, we screened 1,658 studies from MEDLINE, Lilacs, and Embase databases, published from 1985 to April 2021, in English or Spanish. We included 36 observational case-control and cohort studies that included patients with TGA according to the Caplan or Hodges and Warlow diagnostic criteria. We performed a meta-analysis with a random effect model for proportions and calculation of odds ratio (OR) for identified risk factors. Methodological quality was assessed according to the Newcastle-Ottawa scale. Results: We identified 4,514 TGA cases and 544 recurrence events (12.73%). A follow-up had no effect on its variance. We identified a statistically significant association between recurrence and sexual activity as a trigger, a personal history or current state of migraine and depression (OR 1,481 95% CI [1.0341-2.1222] p = 0.04; OR = 2.0795 95% CI [1.3892-3.1128] p = 0.003; and OR = 4.4871 95% CI [1.890-10.651] p = 0.0288, respectively). Discussion: The analysis showed that approximately 1 of 8 participants may experience recurrence, with an increased risk in the case of a history or current state of migraine, depression, or sexual intercourse before the event. A personal history of migraine and depression was associated with 2 and 4 times risk, respectively.

Treatment-related fluctuations in Guillain-Barré syndrome​: clinical features and predictors of recurrence / Fluctuaciones relacionadas al tratamiento en el Síndrome de Guillain-Barré: características clínicas y predictores de recurrencia

ABSTRACT Background: A treatment-related fluctuation (TRF) in a patient with Guillain-Barré syndrome (GBS) is defined as clinical deterioration within two months of symptom onset following previous stabilization or improvements with treatment. Objective: To investigate the clinical characteristics and factors that could increase the risk of relapse of GBS in patients with and without TRFs. Methods: Retrospective review of medical records of patients (>18 years) with GBS evaluated between January/2006 and July/2019. Demographic and clinical characteristics, ancillary studies, treatment received, and the clinical course of patients with and without TRFs were analyzed. Results: Overall, 124 cases of GBS were included; seven (5.6%) presented TRFs. GBS-TRF cases were triggered more frequently by infectious mononucleosis (28.57 vs. 8.55%; p=0.01). GBS-TRF were initially treated with plasmapheresis more frequently than those without TRF (14.29 vs. 1.70%; p=0.0349). Combined treatment (71.43 vs. 4.27%; p<0.001) and corticosteroids (42.86 vs. 1.71%; p<0.001) were more commonly used in the GBS-TRF group. GBS-TRF patients presented a higher median initial disability score (4 vs. 2; p=0.01). Conclusions: Patients with GBS triggered by infectious mononucleosis and a high degree of initial disability have higher chances of developing TRFs. Although patients with TRF were treated with plasmapheresis more often, the total number was too low to suggest a link between plasma exchange and TRF.

RESUMEN Antecedentes: Una fluctuación relacionada al tratamiento (FRT) en un paciente con síndrome de Guillain-Barré (SGB) se define como un deterioro clínico dentro de los dos meses posteriores al inicio de los síntomas después de una estabilización previa o mejoría con el tratamiento. Objetivo: Investigar las características clínicas y los factores que podrían incrementar el riesgo de recaída, comparando pacientes con SGB, con y sin FRT. Métodos: Revisión retrospectiva de historias clínicas de pacientes (>18 años) con SGB evaluados entre enero/2006 y julio/2019. Se analizaron las características demográficas y clínicas, los estudios complementarios, el tratamiento recibido y la evolución clínica de los pacientes con y sin FRT. Resultados: Se incluyeron 124 casos de SGB en el total; 7 (5,6%) presentaron FRT. Los casos de SGB con FRT se desencadenaron con mayor frecuencia por mononucleosis infecciosa (28,57 vs. 8,55%; p=0,01). Los casos de SGB con FRT se trataron inicialmente con plasmaféresis con más frecuencia que aquellos sin FRT (14,29 vs. 1,70%; p=0,0349). El tratamiento combinado (71,43 vs. 4,27%; p<0,001) y los corticosteroides (42,86 vs. 1,71%; p<0,001) se utilizaron con mayor frecuencia en el grupo de SGB con FRT. Los pacientes con FRT presentaron una escala de discapacidad inicial mediana más alta (4 vs. 2; p=0,01). Conclusiones: Aquellos SGB desencadenados por mononucleosis infecciosa y un alto grado de discapacidad inicial tienen una mayor probabilidad de desarrollar FRT. Aunque los pacientes con FRT fueron tratados con plasmaféresis con mayor frecuencia, el número total fue demasiado bajo para sugerir un vínculo entre la plasmaféresis y FRT.

Treatment-related fluctuations in Guillain-Barré syndrome​: clinical features and predictors of recurrence.

BACKGROUND: A treatment-related fluctuation (TRF) in a patient with Guillain-Barré syndrome (GBS) is defined as clinical deterioration within two months of symptom onset following previous stabilization or improvements with treatment. OBJECTIVE: To investigate the clinical characteristics and factors that could increase the risk of relapse of GBS in patients with and without TRFs. METHODS: Retrospective review of medical records of patients (>18 years) with GBS evaluated between January/2006 and July/2019. Demographic and clinical characteristics, ancillary studies, treatment received, and the clinical course of patients with and without TRFs were analyzed. RESULTS: Overall, 124 cases of GBS were included; seven (5.6%) presented TRFs. GBS-TRF cases were triggered more frequently by infectious mononucleosis (28.57 vs. 8.55%; p=0.01). GBS-TRF were initially treated with plasmapheresis more frequently than those without TRF (14.29 vs. 1.70%; p=0.0349). Combined treatment (71.43 vs. 4.27%; p<0.001) and corticosteroids (42.86 vs. 1.71%; p<0.001) were more commonly used in the GBS-TRF group. GBS-TRF patients presented a higher median initial disability score (4 vs. 2; p=0.01). CONCLUSIONS: Patients with GBS triggered by infectious mononucleosis and a high degree of initial disability have higher chances of developing TRFs. Although patients with TRF were treated with plasmapheresis more often, the total number was too low to suggest a link between plasma exchange and TRF.

Transverse Myelitis in Systemic Lupus Erythematosus: Clinical Features and Prognostic Factors in a Large Cohort of Latin American Patients.

BACKGROUND: Acute transverse myelitis (ATM) is an infrequent but severe complication of systemic lupus erythematosus (SLE). The purpose of study was to describe clinical features and prognostic factors of patients with SLE-related ATM. METHODS: In this medical records review study, data were collected from 60 patients from 16 centers seen between 1996 and 2017 who met diagnostic criteria for SLE and myelitis as defined by the American College of Rheumatology/Systemic International Collaborating Clinics and the Working Group of the Transverse Myelitis Consortium, respectively. Objective neurological impairment was measured with American Spinal Injury Association Impairment Scale (AIS) and European Database for Multiple Sclerosis Grade Scale (EGS). RESULTS: Among patients included, 95% (n = 57) were female, and the average age was 31.6 ± 9.6 years. Myelitis developed after diagnosis of SLE in 60% (n = 36). Symmetrical paraparesis with hypoesthesia, flaccidity, sphincter dysfunction, AIS = A/B, and EGS ≥ 8 was the most common presentation. Intravenous methylprednisolone was used in 95% (n = 57), and 78.3% (n = 47) received intravenous cyclophosphamide. Sensory/motor recovery at 6 months was observed in 75% (42 of 56), but only in 16.1% (9 of 56) was complete. Hypoglycorrhachia and EGS ≥ 7 in the nadir were associated with an unfavorable neurological outcome at 6 months (p < 0.05). A relapse rate during follow-up was observed in 30.4% (17 of 56). Hypoglycorrhachia and hypocomplementemia seem to be protective factors for relapse. Intravenous cyclophosphamide was associated with time delay to relapse. CONCLUSIONS: Systemic lupus erythematosus-related ATM may occur at any time of SLE course, leading to significant disability despite treatment. Relapses are infrequent and intravenous cyclophosphamide seems to delay it. Hypoglycorrhachia, hypocomplementemia, and EGS at nadir are the most important prognostic factors.

Intravenous Chlorpromazine as Potentially Useful Treatment for Chronic Headache Disorders.

OBJECTIVE: The aim of this study was to describe a group of patients with chronic headache disorders (CH) and medication overuse headache (MOH) treated with intravenous chlorpromazine (IVC). We hypothesized that IVC is an effective and safe addition to well-known treatment strategies for CH and MOH management. INTRODUCTION: Up to 4% of the general population could experience CH. Most cases occur in women, in association with MOH. To date, evidence to support different treatment strategies is lacking. Although IVC is frequently used in the emergency room (ER), documentation on its use as supportive treatment for CH and for withdrawal management of MOH is poor. METHODS: A retrospective cohort of patients hospitalized to receive treatment for CH in a specialized neurological center in Argentina was analyzed. RESULTS: A total of 35 CH patients were included. Of the 35 patients, 33 (94%) patients also presented MOH. Patients reported only minor side effects to IVC administration (mainly drowsiness and symptomatic hypotension). Three months after inpatient treatment, the number of ER visits made by these patients decreased from an average of 2.8 in the 3 months prior to hospitalization to 0.7 after it (72%, P = .009). Headache frequency decreased in 20/34 (59%) patients during the same time period. Pain levels had dropped from a mean of 8 points at admission (in the scale of 1-10) to 2 points at discharge. In the first 3 months of follow-up, the average number of days per month in which patients experienced headache decreased from 28.9 to 15.4 days (53.3%, P < .0001). CONCLUSION: In this particular group of inpatients, there were no significant safety issues with IVC administration and the study might suggest that the efficacy of IVC as an add-on treatment for CH and MOH.

Acute amnestic syndromes.

Acute amnestic syndromes are usually rare clinical events occurring in emergency situations. Etiological diagnosis can be challenging and underlying causes diverse. They can be transient and totally reversible, or accompanied by other neurological symptoms resulting in serious and irreversible brain damage. Pathophysiology of these syndromes mainly corresponds to structural or functional alteration of memory circuits, including those in the hippocampus. One of the most frequent forms is transient global amnesia (TGA), characterized by sudden onset of anterograde amnesia lasting less than 24  hours, in the absence of other neurological signs or symptoms. Another acute and transient memory disorder is transient epileptic amnesia (TEA), due to focal crisis activity. Stroke injuries occurring at strategic memory-related sites can also present as sudden episodes of amnesia. In addition to neurological etiologies, amnesia may be a symptom of a psychiatric disorder (dissociative amnesia). Traumatic brain injuries, autoimmune encephalitis and acute toxic metabolic disorders can also cause amnesia and should be included among the differential diagnoses. In this review, we summarize the most relevant clinical findings in acute amnestic syndromes, and discuss the different ancillary tests needed to establish a correct diagnosis and management as well the best treatment options. Relevant anatomical and pathophysiological aspects underlying these conditions will be also be presented.

[Multidisciplinary rehabilitation for adult patients with stroke]. / Rehabilitación multidisciplinaria para pacientes adultos con accidente cerebrovascular.

It is estimated that two thirds of people who have suffered a stroke have sequels that condition their quality of life. The rehabilitation of the stroke is a complex process, which requires the multidisciplinary approach of specialized professionals (doctors, kinesiologists, nurses, occupational therapists, phonoaudiologists, neuropsychologists and nutritionists). Currently, the practices carried out are a consequence of the combination of evidence and consensus, most of them through international stroke rehabilitation guides. The objective of this review is to adjust the international recommendations on stroke rehabilitation to what is applied to daily practice, in order to unify the criteria of the recommendations and to reduce the variability of the practices carried out. This work is a review of the literature on stroke rehabilitation guides developed in the last 10 years. Each section was supervised by different professionals specialized in these areas. We analyze the time and organization necessary to develop rehabilitation, recommendations for motor, cognitive and visual rehabilitation, the management of dysphagia and nutrition, the approach of comorbidities (venous thrombosis, skin ulcers, pain, psychiatric disorders and osteoporosis) and the necessary tasks to favor the return to the activities of daily life.

Se estima que dos tercios de las personas que han sufrido un accidente cerebrovascular (ACV) tienen secuelas que condicionan su calidad de vida. La rehabilitación del ACV es un proceso complejo, que requiere de un equipo multidisciplinario de profesionales especializados (médicos, kinesiólogos, enfermeros, terapistas ocupacionales, fonoaudiólogos, neuropsicólogos y nutricionistas). Actualmente, las prácticas realizadas en rehabilitación son consecuencia de la combinación de evidencia y consenso, siendo la mayoría aportadas a través de guías internacionales de rehabilitación en ACV. El objetivo de esta revisión es ajustar las recomendaciones internacionales sobre rehabilitación a lo aplicado a la práctica diaria, a fin de unificar criterios en las recomendaciones y reducir la variabilidad de las prácticas empleadas. En este trabajo, se realizó una revisión de la literatura sobre las guías de rehabilitación en ACV realizadas en los últimos 10 años y cada apartado fue supervisado por distintos profesionales especializados en dichas áreas. Se analizaron los tiempos y organización necesaria para desarrollarla, las recomendaciones para la rehabilitación motora, cognitiva y visual, el tratamiento de la disfagia y nutrición, de las comorbilidades (trombosis venosa, úlceras cutáneas, dolor, trastornos psiquiátricos, osteoporosis) y las tareas necesarias para favorecer el retorno a las actividades de la vida diaria.

Rehabilitación multidisciplinaria para pacientes adultos con accidente cerebrovascular / Multidisciplinary rehabilitation for adult patients with stroke

Se estima que dos tercios de las personas que han sufrido un accidente cerebrovascular (ACV) tienen secuelas que condicionan su calidad de vida. La rehabilitación del ACV es un proceso complejo, que requiere de un equipo multidisciplinario de profesionales especializados (médicos, kinesiólogos, enfermeros, terapistas ocupacionales, fonoaudiólogos, neuropsicólogos y nutricionistas). Actualmente, las prácticas realizadas en rehabilitación son consecuencia de la combinación de evidencia y consenso, siendo la mayoría aportadas a través de guías internacionales de rehabilitación en ACV. El objetivo de esta revisión es ajustar las recomendaciones internacionales sobre rehabilitación a lo aplicado a la práctica diaria, a fin de unificar criterios en las recomendaciones y reducir la variabilidad de las prácticas empleadas. En este trabajo, se realizó una revisión de la literatura sobre las guías de rehabilitación en ACV realizadas en los últimos 10 años y cada apartado fue supervisado por distintos profesionales especializados en dichas áreas. Se analizaron los tiempos y organización necesaria para desarrollarla, las recomendaciones para la rehabilitación motora, cognitiva y visual, el tratamiento de la disfagia y nutrición, de las comorbilidades (trombosis venosa, úlceras cutáneas, dolor, trastornos psiquiátricos, osteoporosis) y las tareas necesarias para favorecer el retorno a las actividades de la vida diaria.

It is estimated that two thirds of people who have suffered a stroke have sequels that condition their quality of life. The rehabilitation of the stroke is a complex process, which requires the multidisciplinary approach of specialized professionals (doctors, kinesiologists, nurses, occupational therapists, phonoaudiologists, neuropsychologists and nutritionists). Currently, the practices carried out are a consequence of the combination of evidence and consensus, most of them through international stroke rehabilitation guides. The objective of this review is to adjust the international recommendations on stroke rehabilitation to what is applied to daily practice, in order to unify the criteria of the recommendations and to reduce the variability of the practices carried out. This work is a review of the literature on stroke rehabilitation guides developed in the last 10 years. Each section was supervised by different professionals specialized in these areas. We analyze the time and organization necessary to develop rehabilitation, recommendations for motor, cognitive and visual rehabilitation, the management of dysphagia and nutrition, the approach of comorbidities (venous thrombosis, skin ulcers, pain, psychiatric disorders and osteoporosis) and the necessary tasks to favor the return to the activities of daily life.

Diagnosis of Rapidly Progressive Dementia in a Referral Center in Argentina.

INTRODUCTION: Rapidly progressive dementia (RPD) is a broadly defined clinical syndrome. Our aim was to describe clinical and ancillary study findings in patients with RPD and evaluate their diagnostic performance for the identification of nonchronic neurodegenerative rapidly progressive dementia (ncnRPD). METHODS: We reviewed clinical records and ancillary methods of patients evaluated for RPD at our institution in Buenos Aires, Argentina from 2011 to 2017. We compared findings between chronic neurodegenerative RPD and ncnRPD and evaluated the diagnostic metrics using receiver operating characteristic curves. RESULTS: We included 104 patients with RPD, 29 of whom were chronic neurodegenerative RPD and 75 of whom were ncnRPD. The 6-month time to dementia cutpoint had a sensitivity of 89% and specificity of 100% for ncnRPD, with an area under the receiver operating characteristic curve of 0.965 (95% confidence interval=0.935-0.99; P<0.001). A decision tree that included time to dementia, brain magnetic resonance imaging, and cerebrospinal fluid analysis identified ncnRPD patients with a sensitivity of 100%, specificity of 79%, positive predictive value of 93%, and negative predictive value of 100% overall. DISCUSSION: RPD is a clinical syndrome that comprises different diagnoses, many of them for treatable diseases. Using the time to dementia, brain magnetic resonance imaging, and cerebrospinal fluid analysis when triaging these patients could help identify those diseases that need to be studied more aggressively.

Espectro clínico de la leucoencefalopatía multifocal progresiva: diferencias y similitudes en pacientes con y sin virus de la inmunodeficiencia humana / The clinical spectrum of progressive multifocal leukoencephalopathy: differences and similarities in patients with and without human immunodeficiency virus

Objetivo. Analizar los hallazgos clínicos, exámenes complementarios y pronóstico de los pacientes con leucoencefalopatía multifocal progresiva (LMP) atendidos en nuestra institución, comparando las poblaciones con y sin virus de la inmunodeficiencia humana (VIH) asociado. Pacientes y métodos. Estudio retrospectivo de historias clínicas de pacientes con LMP probable o definitiva. Se analizaron variables clínicas, estudios complementarios (líquido cefalorraquídeo, resonancia magnética cerebral) y variables pronósticas. Mediante pruebas estadísticas no paramétricas se realizó la comparación entre las poblaciones con y sin VIH. Resultados. Se incluyó a 14 pacientes con diagnóstico de LMP definitiva y uno probable. Nueve pacientes presentaron LMP asociada a VIH; cinco, otras condiciones de inmunoafectación (dos, leucemia linfática crónica; uno, esclerosis múltiple; uno, neuromielitis óptica; y uno, neurosarcoidosis); y uno, sin condición inmunosupresora evidente. La población con VIH presentó con mayor frecuencia lesiones de la sustancia blanca heterogéneas de aspecto «sucio» (77,7% frente a 16,67%; p = 0,0247) en la resonancia magnética cerebral. No se identificaron otras diferencias significativas en las restantes variables analizadas. Conclusión. El VIH/sida es la patología más frecuente asociada a LMP. Con el uso de fármacos inmunomoduladores se describe su aparición en una variedad de otras enfermedades. Las lesiones de la sustancia blanca heterogéneas de aspecto «sucio» fueron significativamente más frecuentes en pacientes con VIH

Aim. To analyse the clinical findings, complementary examinations and prognosis of patients with progressive multifocal leukoencephalopathy (PML) treated in our institution, comparing populations with and without associated human immunodeficiency virus (HIV). Patients and methods. A retrospective study of the medical records of patients with probable or definite PML was carried out. Clinical variables, complementary studies (cerebrospinal fluid, magnetic resonance imaging of the brain) and prognostic variables were analysed. Non-parametric statistical tests were used to compare HIV-positive and HIV non-positive populations. Results. Fourteen patients with definite and one probable diagnosis of PML were included. Nine patients had PML associated with HIV; five had other immunosuppressive conditions (two, chronic lymphatic leukaemia; one, multiple sclerosis; one, neuromyelitis optica; and one, neurosarcoidosis); and one, no obvious immunosuppressive condition. The population with HIV presented heterogeneous dirty-appearing white matter lesions more frequently (77.7% versus 16.67%; p = 0.0247) in the cerebral MRI. No other significant differences were identified in the remaining variables analysed. CONCLUSION. HIV/AIDS is the pathology most frequently associated with PML. With the use of immunomodulator drugs its appearance is reported in a variety of other diseases. Heterogeneous dirty-appearing white matter lesions were significantly more common in HIV patients

Cerebellar Ataxia With Extreme Photophobia Associated With Anti-SOX1 Antibodies.

Anti-SOX1 antibodies are associated with diverse neurological syndromes, targeting both the central (paraneoplastic cerebellar degeneration) and peripheral nervous systems (Lambert Eaton myasthenic syndrome, paraneoplastic neuropathy). Although the pathogenic role of these antibodies remains unclear, their strong association with underlying neoplastic disease (mainly small-cell lung cancer) has designated them as onconeural antibodies. Here, we present a case of cerebellar ataxia with marked photophobia, with severe atrophy of the cerebellum and brain stem, associated with anti-SOX1 antibodies without evidence of an underlying malignancy. Although anti-SOX1-associated cerebellar syndrome is infrequent, investigation of these antibodies should be considered as a part of the diagnostic algorithm if more common causes have been ruled out. Extensive brain stem lesions causing disruption of the trigeminal pathway and its connections with the pretectal area might explain the underlying mechanism of the associated photophobia. Early recognition of anti-SOX1 antibodies, exclusion of underlying neoplasm, and prompt initiation of immunotherapy are essential to achieve a better outcome.

Transient global amnesia: clinical features and prognostic factors suggesting recurrence.

OBJECTIVE: The risk of recurrence of new amnesia events in patients having previously experienced transient global amnesia (TGA) ranges between 2.9-23.8%. Our objective was to search for recurrence predictors in TGA patients. METHODS: Retrospective analysis to identify recurrence predictors in a cohort of 203 TGA patients from a single center in Buenos Aires, Argentina, diagnosed between January 2011 and March 2017 Clinical features and complementary studies (laboratory results, jugular vein Doppler ultrasound and brain MRI) were analyzed. Comparison between patients with recurrent versus single episode TGA was performed, applying a multivariate logistic regression model. RESULTS: Mean age at presentation was 65 years (20-84); 52% were female. Median time elapsed between symptom onset and ER visit was two hours, with the average episode duration lasting four hours. Mean follow-up was 22 months. Sixty-six percent of patients referred to an identifiable trigger. Jugular reflux was present in 66% of patients; and 22% showed images with hippocampus restriction on diffusion-weighted MRI. Eight percent of patients had TGA recurrence. Patients with recurrent TGA had a more frequent history of migraine than patients without recurrence (37.5% vs. 14%; p = 0.03). None of the other clinical characteristics and complementary studies were predictors of increased risk of recurrence. CONCLUSIONS: Patients with migraine may have a higher risk of recurrent TGA. None of the other clinical characteristics evaluated allowed us to predict an increased risk of recurrence. Although the complementary studies allowed us to guide the diagnosis, they did not appear to have a significant impact on the prediction of recurrence risk.

The role of infections in multiple sclerosis.

Several lines of evidence suggest that multiple sclerosis (MS), like other autoimmune diseases, may be triggered by microbial infections. Pathogens associated with development or exacerbation of MS include bacteria, such as Chlamydia pneumoniae, Staphylococcus aureus-produced enterotoxins that function as superantigens, and viruses of the Herpesviridae (Epstein-Barr virus and human herpes virus 6) and human endogenous retrovirus families. However, to date, no single pathogen has been accepted as causal agent. In addition, common upper respiratory, gastrointestinal, and urogenital tract infections have also been associated with MS exacerbations. Although evidence of an infectious etiology as cause of MS in humans remains inconclusive, microbial agents may modulate the neuroimmunological system of genetically susceptible individuals. Decoding the epidemiological contribution of different microorganisms to MS, along with their pathogenic mechanisms, may help develop new treatment strategies and prevent relapses.

Transient global amnesia: clinical features and prognostic factors suggesting recurrence / Amnesia global transitoria: características clínicas y factores pronósticos sugestivos de recurrencia

ABSTRACT The risk of recurrence of new amnesia events in patients having previously experienced transient global amnesia (TGA) ranges between 2.9-23.8%. Objective: Our objective was to search for recurrence predictors in TGA patients. Methods: Retrospective analysis to identify recurrence predictors in a cohort of 203 TGA patients from a single center in Buenos Aires, Argentina, diagnosed between January 2011 and March 2017 Clinical features and complementary studies (laboratory results, jugular vein Doppler ultrasound and brain MRI) were analyzed. Comparison between patients with recurrent versus single episode TGA was performed, applying a multivariate logistic regression model. Results: Mean age at presentation was 65 years (20-84); 52% were female. Median time elapsed between symptom onset and ER visit was two hours, with the average episode duration lasting four hours. Mean follow-up was 22 months. Sixty-six percent of patients referred to an identifiable trigger. Jugular reflux was present in 66% of patients; and 22% showed images with hippocampus restriction on diffusion-weighted MRI. Eight percent of patients had TGA recurrence. Patients with recurrent TGA had a more frequent history of migraine than patients without recurrence (37.5% vs. 14%; p = 0.03). None of the other clinical characteristics and complementary studies were predictors of increased risk of recurrence. Conclusions: Patients with migraine may have a higher risk of recurrent TGA. None of the other clinical characteristics evaluated allowed us to predict an increased risk of recurrence. Although the complementary studies allowed us to guide the diagnosis, they did not appear to have a significant impact on the prediction of recurrence risk.

RESUMEN El riesgo de recurrencia de nuevos eventos de amnesia en pacientes que han experimentado previamente Amnesia Global Transitoria (AGT) oscila entre el 2.9-23.8%. Objetivo: Nuestro objetivo fue buscar predictores de recurrencia en pacientes con AGT. Métodos: Análisis retrospectivo de una cohorte de 203 pacientes con AGT de un único centro en Buenos Aires, Argentina, diagnosticados entre enero-2011 y marzo-2017 Se analizaron las características clínicas y los estudios complementarios (laboratorio, Doppler de vena yugular y RM encéfalo). Se comparó el grupo de AGT recurrente versus episodio único, aplicando un modelo de regresión logística multivariada. Resultados: la edad promedio de presentación fue de 65 años (20-84); 52% mujeres. La mediana del tiempo transcurrido entre el inicio de los síntomas y la visita a la sala de emergencia fue de 2 horas, con una duración promedio del episodio de 4 horas. El seguimiento medio fue de 22 meses. 66% de los pacientes tuvieron un desencadenante identificable. El reflujo yugular estuvo presente en el 66% de los pacientes y el 22% mostró imágenes restrictivas en DWI a nivel hipocampal. 8% de los pacientes presentaron recurrencia. Los pacientes con AGT recurrente tuvieron un historial de migraña más frecuente (37.5% vs. 14%; p=0.03). Ninguna de las otras características clínicas y estudios complementarios fueron predictores de mayor riesgo de recurrencia. Conclusiones: los pacientes con migraña pueden tener un mayor riesgo de recurrencia de AGT. Ninguna de las otras características clínicas evaluadas nos permitió predecir un mayor riesgo de recurrencia. Aunque los estudios complementarios nos permitieron orientar el diagnóstico, no pareció tener un impacto significativo en la predicción del riesgo de recurrencia.

Clinical Correlations of Positive Herpes Simplex PCR in Cerebrospinal Fluid.

OBJECTIVES: Herpes simplex viruses (HSV) can produce encephalitis (HSE), which requires early detection, typically using polymerase chain reaction (PCR) in cerebrospinal fluid (CSF). However, other neurological conditions not directly caused by HSV may also present with a positive HSV PCR in the CSF (NCNHPCR+). We aimed to analyze the clinical features of both groups of patients (HSE vs. NCNHPCR+) and to consider the potential relevance of this finding in the latter. METHODS: A retrospective analysis of clinical presentation, workup (CSF, EEG, and MRI) and outcome of patients with an HSV+ result in CSF was conducted from Jan-2007 to Sep-2015 in our institution. Patients under 18 years and those with nonencephalitic HSV associated disorders were excluded. Group comparison between HSE and NCNHPCR+ patients was conducted using parametric and nonparametric tests accordingly. RESULTS: Sixteen HSE and 23 NCNHPCR+ patients were included. Patients with HSE presented a higher incidence of headache (87.5% vs. 43.5%; P=0.008), meningeal symptoms (50% vs. 17.4%; P=0.04), pleocytosis (75% vs. 18%; P=0.001), EEG abnormalities (46.67% vs. 22%; P=0.02) and typical MRI findings (50% vs. 0%; P<0.001), whereas 35% of patients with NCNHPCR+ had an underlying immunologic disorder (35% vs. 0%; P=0.012). CONCLUSIONS: The pathogenic role of HSV in NCNHPCR+ is uncertain. This finding must be interpreted in the appropriate clinical, EEG, and neuroimaging context. Immunocompromise and neuroinflammation states could be related to a higher presence of HSV in CSF.

Chronic and Subacute Meningitis: Differentiating Neoplastic From Non-Neoplastic Etiologies.

BACKGROUND AND PURPOSE: Although incidence rates vary, infectious, autoimmune, and neoplastic diseases can all cause chronic and subacute meningitis (CSM). We report a Latin-American, single center, CSM case series, analyzing the main clinical characteristics as well as ancillary diagnostic methods differentiating neoplastic from non-neoplastic etiologies. METHODS: Retrospective review of CSM cases from a single center in Buenos Aires, Argentina. RESULTS: Seventy patients with CSM diagnosis were identified, 49 with neoplastic and 21 with non-neoplastic meningitis. A history of previous cancer was significantly higher in neoplastic cases, whereas prevalence of autoimmune disease and fever was more common in non-neoplastic meningitis. C-reactive protein values were higher in non-neoplastic CSM, as was pleocytosis in cerebrospinal fluid analysis. The most frequent etiologies were breast and lung cancer for neoplastic meningitis cases; and idiopathic, tuberculous, and fungal infection for non-neoplastic cases. CONCLUSIONS: Chronic and subacute meningitis diagnosis is challenging in daily neurological practice. The results we report contribute information from Latin America regarding etiologies of CSM, which can be identified after a comprehensive evaluation in a majority of cases.

The role of surgery in primary central nervous system lymphomas.

Background Primary central nervous system lymphomas (PCNSL) are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients.

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Demographic characteristics, clinical manifestations, cerebrospinal-fluid (CSF) findings, treatment and clinical evolution were analyzed. Nerve conduction studies were performed in all patients with at least 12 months follow-up. A total of 91 patients were included (AIDP, n = 77; A-CIDP, n = 14). The median age was 55.5 years in patients with A-CIDP vs 43 years in AIDP (P = .07). The history of diabetes mellitus was more frequent in A-CIDP (29% vs 8%, P = .04). No significant differences between groups were observed with respect to: human immunodeficiency virus (HIV) status, presence of auto-immune disorder or oncologic disease. Cranial, motor and autonomic nerve involvement rates were similar in both groups. Patients in the A-CIDP group showed higher frequency of proprioceptive disturbances (83% vs 28%; P < .001), sensory ataxia (46% vs 16%; P = .01), and the use of combined immunotherapy with corticoids (29% vs 3%; P = .005). There were no significant differences in CSF findings, intensive care unit (ICU) admission, or mortality rates. During the first 8 weeks both entities are practically indistinguishable. Alterations in proprioception could suggest A-CIDP. Searching for markers that allow early differentiation could favor the onset of corticotherapy without delay.

The role of surgery in primary central nervous system lymphomas / El rol de la cirugía en los linfomas primarios del sistema nervioso central

ABSTRACT Background Primary central nervous system lymphomas (PCNSL) are infrequent. The traditional treatment of choice is chemotherapy. Complete resections have generally not been recommended, because of the risk of permanent central nervous system deficits with no proven improvement in survival. The aim of the current study was to compare survival among patients with PCNSL who underwent biopsy versus surgical resection. Methods A retrospective study was conducted on 50 patients with a confirmed diagnosis of PCNSL treated at our center from January 1994 to July 2015. Results Patients in the resection group exhibited significantly longer median survival time, relative to the biopsy group, surviving a median 31 months versus 14.5 months; p = 0.016. Conclusions In our series, patients who had surgical resection of their tumor survived a median 16.5 months longer than patients who underwent biopsy alone.

RESUMO Introducción Los linfomas primarios del sistema nervioso central (LPSNC) son infrecuentes. Tradicionalmente el tratamiento de elección es la quimioterapia. Existe un paradigma de no indicar resección, por el riesgo de déficit neurológico sin aumento de la sobrevida. El objetivo del presente estudio es comparar la sobrevida de pacientes con LPSNC sometidos a biopsia versus resección. Métodos Estudio retrospectivo que incluye 50 pacientes con diagnóstico confirmado de LPSNC tratados en nuestra Institución desde enero de 1994 hasta julio de 2015. Resultados Los pacientes del "grupo resección" mostraron una sobrevida media significativamente mayor respecto a los del "grupo biopsia"; 31 meses versus 14,5 meses respectivamente, p = 0,016. Conclusiones En nuestra serie, los pacientes que con resección quirúrgica de su tumor tuvieron una sobrevida media de 16,5 meses superior que los pacientes biopsiados.

Susac Syndrome: A differential diagnosis of white matter lesions.

INTRODUCTION: Susac Syndrome is an autoimmune endotheliopathy affecting capillaries and precapillary arterioles of the brain, inner ear and retina. The classic symptom triad includes visual disturbances, hypoacusia, and encephalopathy, but is rarely fully manifest at onset. The syndrome typically follows an active fluctuating monophasic course. Typical imaging findings on brain magnetic resonance show central fiber microischemias/microinfarctions of the corpus callosum known as "snow balls", as well as lineal infarcts and upper callosal fiber involvement. Fluorescein angiography and tonal audiometry are important tools to confirm diagnosis, even in patients who are asymptomatic for visual or auditory disturbances. We describe 8 patients with Susac Syndrome treated at our center and compare findings to those of other published case series. METHODS: Eight adults with Susac Syndrome diagnosed between January 2007 and August 2016 at our center, in Buenos Aires, Argentina are described. Magnetic Brain Resonance, fluorescein angiography, tonal audiometry, a complete serologic battery and lumbar puncture were performed to all patients. RESULTS: The majority of patients were males, and average age at diagnosis was 37.5 years (range: 22-52 yrs). Two patients presented full clinical triad at onset, while the remaining developed visual and/or auditory disturbances later during the course of disease. All cases manifested varied neurological symptoms including pyramidal and/or sensory tract symptoms, amnesic disorders, ataxia and vertigo. Psychiatric manifestations such as disinhibition, aggressive behavior, paranoid ideation and hallucinations were also present. Seven patients suffered at least one relapse. Typical central corpus callosum fiber ischemias/infarcts, also known as snowball lesions were visible in all patients. Spokes, icicles, periventricular lesions and internal capsule "string of beads" patterns were observed on diffusion weighted images. Four cases presented fornix microischemias/microinfarctions, 5 juxtacortical involvement and 3 infratentorial infarcts. Leptomeningeal involvement was evident in only 1 patient after rabies vaccination. Fluorescein angiography was abnormal in 7 cases. Tonal audiometry revealed unilateral hypoacusia in 5 patients, 3 with low frequency hearing loss. Immunosuppressive treatment was indicated in all cases. Patient follow up ranged between 6 months and 9 years, only 1 patient was lost to follow up after three years. CONCLUSION: Susac Syndrome is probably both underdiagnosed and misdiagnosed. Early and aggressive immunosuppressive treatment is indicated in order to prevent potential disabilities.