Stroke in systemic lupus erythematosus: epidemiology, mechanism, and long-term outcome.

OBJECTIVE: To determine stroke prevalence, mechanisms, and long-term outcome in a cohort of Hispanic patients with systemic lupus erythematosus (SLE). METHODS: We analyzed demographical data, the timing between SLE diagnosis and stroke onset, stroke type, recurrence, and outcomes from an institutional database of 4451 patients with SLE followed from 1993 to 2018. RESULTS: We observed 139 strokes (3.1%), for an incidence rate of 1.25 per 1000 person-years: 81 (58.3%) acute ischemic stroke (AIS), 19 (13.7%) subarachnoid hemorrhage (SAH), 17 (12.2%) cerebral venous thrombosis, 13 (9.4%) intracerebral hemorrhage (ICH), and 9 (6.5%) transient ischemic attack. Median time from SLE diagnosis to acute stroke was 60 months (interquartile range 12-132 months). AIS had a bimodal presentation with 26% occurring within the first year and 30% >10 years after SLE diagnosis. In contrast, 75% of ICH cases occurred >3 years (and 34% >10 years) after SLE diagnosis. The most important cause of AIS was secondary antiphospholipid syndrome (48%). Hypertension was associated with 69% of ICH cases, while aneurysmal rupture was observed in 78% of SAH cases. Excellent recovery at hospital discharge was observed in 65%. Stroke recurrence was observed in 7%. The long-term all-cause fatality rate was 8%. CONCLUSIONS: The prevalence of stroke in this cohort was 3.1%. Ischemic strokes had a bimodal presentation, occurring either early after SLE diagnosis or after a several-year delay. Half of the hemorrhagic strokes occurred >10 years after the diagnosis of SLE. Clinical outcome was usually good with a relatively low recurrence rate.

Adherence to treatment in children with growth hormone deficiency, small for gestational age and Turner syndrome in Mexico: results of the Easypod™ connect observational study (ECOS).

BACKGROUND: Assessing adherence to growth hormone (GH) is challenging. The Easypod™ connect device delivers pre-set doses of recombinant human GH (r-hGH) and stores a digital record of adherence that can be shared with healthcare provider. We assessed adherence to r-hGH delivered with Easypod™ according to the approved pediatric indications for r-hGH: growth hormone deficiency (GHD), born small for gestational age (SGA) who failed to show catch-up growth and Turner syndrome (TS). METHODS: ECOS (NCT01555528) was a multicenter (24 countries), 5-year, longitudinal, observational study, which aimed to evaluate country-specific adherence to r-hGH therapy prescribed via the Easypod™ electronic injection device. The primary endpoint was yearly adherence. Secondary endpoints were height velocity, height velocity standard deviation scores (SDS), height, height SDS and IGF-1 concentrations. Clinical and auxological data were obtained from medical records and adherence from Easypod™ logs. RESULTS: This study included 147 Easypod™-naïve Mexican children assessed during 3 years (mean age: 9.96 ± 3.41 years, 56.8% boys, mean height SDS at baseline: - 2.17 ± 0.97): 118 with GHD, 24 SGA and 5 with TS. A total of 105 (71.4%) patients were GH naïve. Overall median adherence was > 90% over the first year of treatment and > 80% at 3 years. Adherence was not different by r-hGH indication or between GH-naïve or experienced patients. At 1-year follow-up, mean change in height SDS was 0.57 ± 0.34, whereas mean height velocity SDS was 2.85 ± 2.51. In all, 84.7% patients had normal IGF-1 concentrations at 1-year follow-up. Adherence was associated with change in height SDS (r = 0.239, p = 0.005) and height velocity SDS (r = 0.194, p = 0.027). CONCLUSION: Adherence rates with the Easypod™ device are high and maintained over time in GHD, SGA and TS Easypod™-naïve Mexican patients. High adherence is associated with better outcomes. Easypod™ assists physicians in monitoring adherence to r-hGH.

Pathophysiology of neurally-mediated syncope. / Fisiopatología del síncope neuralmente mediado.

INTRODUCTION: Neurally-mediated syncope (NMS) is defined as a transient loss of consciousness due to an abrupt and intermittent drop in blood pressure (BP). OBJECTIVES: This study describes the putative pathophysiological mechanisms giving rise to NMS, the role of baroreflex (BR), and the interaction of its main haemodynamic variables: heart rate (HR) and BP. DEVELOPMENT: Episodic dysregulation affects control over the haemodynamic variables (HR and BP) mediated by baroreflex mechanisms. During active standing, individuals experience a profound transient drop in systolic BP due to the effect of gravity on the column of blood and probably also because of reflex vasodilation. Abnormalities in the BR in NMS could be due to a more profound drop in BP upon standing, or to delayed or incomplete vasoconstriction resulting from inhibited or delayed sympathetic activity. CONCLUSIONS: Sympathetic hyperactivity is present in patients with NMS at rest and before syncope. During active standing or passive tilting, excessive tachycardia may be followed by bradycardia and profound hypotension. Recovery of systolic BP is delayed or incomplete.

Hospital arrival time and functional outcome after acute ischaemic stroke: results from the PREMIER study.

INTRODUCTION: Information regarding hospital arrival times after acute ischaemic stroke (AIS) has mainly been gathered from countries with specialised stroke units. Little data from emerging nations is available. We aim to identify factors associated with achieving hospital arrival times of less than 1, 3, and 6 hours, and analyse how arrival times are related to functional outcomes after AIS. METHODS: We analysed data from patients with AIS included in the PREMIER study (Primer Registro Mexicano de Isquemia Cerebral) which defined time from symptom onset to hospital arrival. The functional prognosis at 30 days and at 3, 6, and 12 months was evaluated using the modified Rankin Scale. RESULTS: Among 1096 patients with AIS, 61 (6%) arrived in <1 hour, 250 (23%) in <3 hours, and 464 (42%) in <6 hours. The factors associated with very early (<1 hour) arrival were family history of ischemic heart disease and personal history of migraines; in <3 hours: age 40-69 years, family history of hypertension, personal history of dyslipidaemia and ischaemic heart disease, and care in a private hospital; in <6 hours: migraine, previous stroke, ischaemic heart disease, care in a private hospital, and family history of hypertension. Delayed hospital arrival was associated with lacunar stroke and alcoholism. Only 2.4% of patients underwent thrombolysis. Regardless of whether or not thrombolysis was performed, arrival time in <3 hours was associated with lower mortality at 3 and 6 months, and with fewer in-hospital complications. CONCLUSIONS: A high percentage of patients had short hospital arrival times; however, less than 3% underwent thrombolysis. Although many factors were associated with early hospital arrival, it is a priority to identify in-hospital barriers to performing thrombolysis.

Prognosis of patients with severe cerebral venous thrombosis treated with decompressive craniectomy.

INTRODUCTION: Despite the highly favorable prognosis, mortality occurs in nearly 2% of patients with cerebral venous thrombosis (CVT), in which decompressive craniectomy (DC) may be the only way to save the patient's life. The aim of this report is to describe the risk factors, neuroimaging features, in-hospital complications and functional outcome of severe CVT in patients treated with DC. MATERIALS AND METHODS: Consecutive malignant CVT cases treated with DC from a retrospective third-level hospital database were analyzed. Demographic, clinical, and functional outcomes were analyzed. RESULTS: Twenty-six patients were included (20 female, age 35.4±12.1 years); 53.8% of the patients had acute CVT, with neurological focalization as the most common symptom in 92.3% of the patients. Superior sagittal sinus thromboses were found in 84.6% of cases. Bilateral lesions were present in 10 patients (38.5%). Imaging on admission showed a parenchymal lesion (venous infarction±hemorrhagic lesion)>6cm measured along the longest diameter in 25 patients (96.2%). Mean duration of clinical neurological deterioration was 3.5 days; eleven patients (42.3%) died during hospitalization. CONCLUSION: In patients with severe forms of CVT, we found higher mortality than previously reported. DC is an effective life-saving treatment with acceptable functional prognosis for survivors.

An expandable prosthesis with dual cage-and-plate function in a single device for vertebral body replacement: the clinical experience on 14 consecutive cases with vertebral tumors.

AIM: The aim of this paper was to test the hypothesis that an expandable prosthesis with dual cage-and-plate function can provide immediate and durable spine stabilization after corpectomy. METHODS: We designed an expandable vertebral body prosthesis with dual cage-and-plate function in a single device (JR-prosthesis). Anatomical studies were performed to design a titanium-made prosthesis. Cadaver assays were done with a stainless steal device to test fixation and adequacy to the human spine anatomy. Then, 14 patients with vertebral tumors (8 metastatic) underwent corpectomy and vertebral body replacement with the JR-prosthesis. RESULTS: All patients had neurological deficit, severe pain and spine instability (mean follow-up: 25.4 months). Mean pain score before surgery in a visual analog scale improved from 7.6 to 3.0 points after operation (P=0.002). All patients achieved at least one grade of improvement in the Frankel score (P=0.003), excepting the 3 patients with Frankel grade A presurgery. Two patients with renal cell carcinoma died during the following 4 days after surgery (renal failure and massive bleeding), the rest attained a painless and stable spine immediately and maintained for long periods. No significant infections or implant failures were registered. A non-fatal case of inferior vena cava surgical injury was observed (repaired during surgery without further complications). CONCLUSION: The JR-prosthesis stabilizes the spine immediately after surgery and for the rest of the patients' life. To our knowledge, this is the first report on the clinical experience of any expandable vertebral body prosthesis with dual cage-and-plate function in a single device. These observations await confirmation in different scenarios.

Prognosis of patients with severe cerebral venous thrombosis treated with decompressive craniectomy.

INTRODUCTION: Despite the highly favorable prognosis, mortality occurs in nearly 2% of patients with cerebral venous thrombosis (CVT), in which decompressive craniectomy (DC) may be the only way to save the patient's life. The aim of this report is to describe the risk factors, neuroimaging features, in-hospital complications and functional outcome of severe CVT in patients treated with DC. MATERIALS AND METHODS: Consecutive malignant CVT cases treated with DC from a retrospective third-level hospital database were analyzed. Demographic, clinical, and functional outcomes were analyzed. RESULTS: Twenty-six patients were included (20 female, age 35.4±12.1 years); 53.8% of the patients had acute CVT, with neurological focalization as the most common symptom in 92.3% of the patients. Superior sagittal sinus thromboses were found in 84.6% of cases. Bilateral lesions were present in 10 patients (38.5%). Imaging on admission showed a parenchymal lesion (venous infarction±hemorrhagic lesion)>6cm measured along the longest diameter in 25 patients (96.2%). Mean duration of clinical neurological deterioration was 3.5 days; eleven patients (42.3%) died during hospitalization. CONCLUSION: In patients with severe forms of CVT, we found higher mortality than previously reported. DC is an effective life-saving treatment with acceptable functional prognosis for survivors.

Citicoline for acute ischemic stroke in Mexican hospitals: a retrospective postmarketing analysis.

Some neuroprotective agents have shown benefits in animal models, but disappointing results in humans. Citicoline is used in several countries as coadjuvant treatment in acute ischemic stroke (AIS) patients; however, there are no retrospective postmarketing surveillances on the experience of citicoline in Mexico. The aim of this study was to evaluate the correlation between citicoline exposure and functional outcome at discharge and at 30 and 90 days post-stroke, in a retrospective case-control design on systematic descriptive databases from three referral hospitals. Clinical records of 173 consecutively registered patients were analyzed, 86 of whom were treated with citicoline within the first 48 h after AIS and the remaining 87 were untreated, randomly selected controls matched for age (+/- 5 years), gender and NIHSS (+/- 1 point) at hospital admission. Pretreatment conditions were similar between groups. Compared with controls, exposure to citicoline was associated with a significantly lower 30-day mean and median modified Rankin score (in both, P < 0.05). After paired multivariate analyses (controlled for NIHSS, age, gender, hospital arrival in < 24 h, thrombolysis and comorbidities) citicoline was independently associated with a lower 90-day mortality risk (P = 0.047) and with fewer in-hospital complications (mainly infections and sepsis, P = 0.001). In this observational study, citicoline use was associated with a better functional status and lower rates of short-term mortality, possibly due to fewer in-hospital systemic complications. The putative benefits should be interpreted as clinical associations, since this is not a randomized, controlled clinical trial.

Opsoclonus-myoclonus-ataxia syndrome associated with central nervous system HIV-1 escape phenomenon. / Síndrome opsoclono-mioclono-ataxia asociado a fenómeno de escape viral por virus de la inmunodeficiencia humana en el sistema nervioso central.

INTRODUCTION: Opsoclonus-myoclonus-ataxia (OMA) syndrome is a rare neurological disorder characterized by involuntary conjugate saccadic eye movements, myoclonus, and ataxia. Few reports exist on patients with HIV and OMA. CASE REPORT: A 41-year-old man diagnosed with HIV-1 infection in 1997 coursed with multiple anti-retroviral schemes as a consequence of poor adherence. In 2008 he presented an HIV-1 viral load of 100,000 copies/mL and a CD4+ T cell count of 10 cells/mm3. In 2013 our patient arrived with an 11-month history of progressive opsoclonus and ataxia. He had undetectable plasma HIV-1 RNA load and CD4+ of 606 cells/mm3. No opportunistic infections were found. Cerebrospinal fluid analysis showed mildly elevated protein concentration and HIV-1 viral load of 534 copies/mL. Cerebrospinal fluid co-receptor tropism test showed selective CCR5 usage. A brain magnetic resonance imaging showed hippocampal atrophy and T2-weighted hyperintensities. Our patient exhibited a dramatic recovery and cerebrospinal fluid HIV clearance after adjustment of anti-retroviral treatment based on genotyping resistance and tropism analyses. CONCLUSIONS: In patients with HIV presenting cengral nervous system dysfunction without opportunistic infections, cerebro-spinal fluid and plasma HIV-1 viral load, resistance and tropism tests should be performed to assess a potential viral escape and to design the appropriate anti-retroviral therapy in an individual patient basis.

TITLE: Síndrome opsoclono-mioclono-ataxia asociado a fenómeno de escape viral por virus de la inmunodeficiencia humana en el sistema nervioso central.Introducción. El síndrome opsoclono-mioclono-ataxia (OMA) es un trastorno neurológico infrecuente caracterizado por movimientos oculares conjugados sacádicos involuntarios, mioclonías y ataxia. Existen pocos casos en la bibliografía de pacientes con virus de la inmunodeficiencia humana (VIH) y OMA. Caso clínico. Varón de 41 años y diagnóstico de infección por el VIH-1 desde 1997, que cursó con múltiples esquemas antirretrovirales debido a una pobre adhesión al tratamiento. En 2008 presentó una carga viral de 100.000 copias/mL y una cuenta linfocitaria CD4+ de 10 células/mm3. En 2013 sufrió un cuadro progresivo de 11 meses de evolución caracterizado por opsoclonía y ataxia. En ese momento, su carga viral era indetectable, y la cuenta de CD4+, de 606 células/mm3. Se descartaron infecciones oportunistas. El examen del líquido cefalorraquídeo demostró hiperproteinorraquia leve y una carga viral de 534 copias/mL. El examen del tropismo de correceptor en el líquido cefalorraquídeo demostró un uso selectivo de CCR5. La resonancia magnética cerebral objetivó atrofia hipocámpica e hiperintensidades en las secuencias ponderadas en T2. El paciente mostró una recuperación clínica franca y un aclaramiento de la carga viral en el líquido cefalorraquídeo tras el ajuste de antirretrovirales basado en la resistencia de genotipo y el análisis de tropismo. Conclusiones. En pacientes con infección por el VIH y disfunción del sistema nervioso central sin infecciones oportunistas, debería llevarse a cabo una determinación de la carga viral en el plasma y el líquido cefalorraquídeo para descartar un potencial fenómeno de escape viral, así como exámenes de resistencia y tropismo para diseñar el tratamiento antirretroviral adecuado.

Mortalidad por enfermedad renal crónica y su relación con la diabetes en México / Chronic kidney disease mortality and its relation to diabetes in Mexico

Resumen OBJETIVO Analizar el efecto en México de la mortalidad producida por la enfermedad renal crónica secundaria a la diabetes mellitus. MATERIAL Y MÉTODO Estudio observacional efectuado de 1998 a 2014, en el que se tomó como base un registro nacional correspondiente a un lapso relativamente prolongado de 17 años, reconociendo a esta enfermedad en tanto entidad nosológica diferenciada, utilizando como metodología de análisis la minería de datos, y evitando en lo posible las ambigüedades o limitaciones detectadas en los estudios previamente publicados. RESULTADOS En las dos últimas décadas se han duplicado la prevalencia y las tasas de mortalidad por enfermedad renal crónica en la República Mexicana, lo que supone un elevado costo humano y financiero, además de que esta enfermedad reduce significativamente la calidad y la esperanza de vida de la población adulta mexicana. CONCLUSIONES Es necesario optimizar las estrategias de atención del paciente con enfermedad renal crónica sin descartar el uso de estrategias de prevención eficaces, dirigidas a la población general.

Abstract OBJECTIVE To analyze the impact in Mexico of the mortality produced by chronic renal failure secondary to diabetes mellitus. MATERIAL AND METHOD An observational study was done from 1998 to 2014 on the basis of a national registry corresponding to a relatively long period of 17 years, recognizing this pathology as a differentiated nosological entity, using the data mining as methodology of analysis, and avoiding as far as possible the ambiguities or limitations detected in previously published studies. RESULTS In the last two decades, the prevalence and mortality rates for chronic renal failure has doubled in Mexico, which means a high human and financial cost, in addition to the fact that this disease significantly reduces the quality of life and the life expectancy of the Mexican adult population. CONCLUSION It is necessary to optimize the care strategies for patients with chronic renal failure, without ruling out the use of effective prevention strategies focused on the general population.

[Neuromyelitis optica: a clinical update]. / Neuromielitis optica: actualizacion clinica.

INTRODUCTION: Neuromyelitis optica (NMO) or Devic's disease is an autoimmune, inflammatory and demyelinating central nervous system disorder that affects mainly to optic nerve and spinal cord. Recent advances have substantially permitted to expand the knowledge about this entity. AIM: To present a clinical update on the current understanding of the nature, progression, diagnosis and treatment of NMO. DEVELOPMENT: Due to its demyelinating nature and its recurrent behavior in most cases, NMO was first considered a form of multiple sclerosis (MS). However, recent findings have led to the conclusion that NMO is a distinct disorder, presenting important immunopathological, clinical, prognostic and therapeutic differences from MS. Fundamental in the under-standing of the disease was the recent discovery of antibodies directed against aquaporin-4 (anti-AQP4, also known as NMO-IgG), which are present in the majority of NMO cases clinically defined, and in a minority of patients with MS. Despite the knowledge on its immunopathogenesis and advances in diagnosis, the treatment of NMO is still challenging. CONCLUSION: NMO is a demyelinating disease different from MS. Current diagnostic criteria have been enriched with the recent description of the humoral disorder underlying NMO. However, current treatment options for NMO are far from being ideal.

Idiopathic hypertrophic cranial pachymeningitis successfully treated with weekly subcutaneous methotrexate.

Idiopathic hypertrophic cranial pachymeningitis is a very infrequent disorder. Adequate management is still a matter of debate. We describe the use of low-dose pulse methotrexate in treating a 63-year-old woman with idiopathic hypertrophic cranial pachymeningitis. A weekly scheme with subcutaneous methotrexate was tried. Clinical improvement occurred in one week. Total remission of the clinical and neuro-imaging abnormalities was evident 6 months later, with minimal side effects. The patient is in complete remission after one year of follow-up without treatment. Hence, low-dose weekly subcutaneous methotrexate may be safe and effective in inducing complete and sustained remission of this condition. The experience with subcutaneous methotrexate to treat this entity has never been reported.