Deltoid muscle strength and autonomic dysfunction as independent risk factors for invasive mechanical ventilation in patients with Guillain-Barré syndrome.

BACKGROUND: Almost a third of patients with Guillain-Barré Syndrome (GBS) require mechanical ventilation, increasing mortality by 15-30% and proving poor functional outcomes. The Erasmus GBS Respiratory Insufficiency Score (EGRIS) is the most frequently used scale to assess probability of respiratory insufficiency within the first week of admission. We aim to determine other clinical and electrophysiological prognostic factors for invasive mechanical ventilation (IMV) in patients with GBS. MATERIAL AND METHODS: A cross-sectional ambispective cohort study was carried out in a referral center in Mexico City, from January 2015 to December 2019. Baseline demographics, MRC score, Hughes scale, EGRIS, dysautonomia and nerve conduction studies were performed on admission in GBS patients that required IMV. A multivariable analysis for IMV and a survival analysis for independent walk in prolonged-IMV (>14 days) were performed. RESULTS: Forty-nine (32%) out of 153 GBS patients required IMV. Statistically significant prognostic factors in multivariable analysis were deltoid muscle strength ≤2 [OR 7.1 (1.6-31.1)], EGRIS [OR 2.5 (1.3-4.6)] and autonomic dysfunction [OR 6.6 (2.0-22.0)]. Electrodecrement <1 mV in the compound muscle action potential (CMAP) of distal motor median nerve was more prevalent in prolonged-IMV patients (44.8% vs. 21%, p = .049). A significant minor prevalence of prolonged-IMV patients regain independent walk at 6 months using the Kaplan-Meier method (log rank test p < .001). CONCLUSIONS: We provide new specific clinical (deltoid muscle strength and autonomic dysfunction) and electrophysiological variables to discriminate GBS patients that will require IMV.

Very low peroneal nerve compound muscle action potential amplitude predicts poor outcome in patients with Guillain-Barré syndrome: a prospective cohort.

INTRODUCTION: Twenty percent of patients with Guillain-Barré syndrome (GBS) have poor outcomes despite proper management. The aim of the study was to characterize electrophysiological factors related to poor outcome in patients with GBS. METHODS: We conducted an observational study from a prospective cohort of 91 patients with GBS in a tertiary healthcare center in Mexico, from 2017 to 2019. Demographics and nerve conduction studies were performed on admission, and a 3-month follow-up for GBS disability score was ensued, allocating patients in good (GBS disability score ≤ 2) and poor outcome (GBS disability score ≥ 3) groups. A logistic regression analysis for independent walk at 3 months was performed. Kaplan-Meier estimator curves for independent walk in very low (< 20% LLN) and low-normal ( ≥20% LLN) peroneal nerve CMAPs are presented. RESULTS: From the 91 GBS patients included, 37 (40.6%) did not regain independent walk at 3 months. Axonal variants were more common in the poor outcome group (31.4% vs 59.4%, p = 0.01) as well as AIDP variants with motor conduction block (6.6% vs 42.4%, p = 0.018). Univariable analysis was statistically significant for very low median, ulnar, tibial, and peroneal CMAP amplitudes in poor outcome patients; however, multivariable analysis was only significant for very low peroneal nerve CMAP amplitude (OR 3.6 [1.1-11.5, p = 0.024]). Conversely, a greater proportion of GBS patients with low-normal CMAPs recovered independent walk at 90 days (75% vs 30%, p < 0.001). CONCLUSION: Severe axonal injury of the peroneal nerve, axonal, and AIDP with motor conduction block variants predicts worse functional outcome regarding independent walk at 3 months.

Inpatient Delirium in Guillain-Barré Syndrome: Frequency and Clinical Characteristics in a Mexican Hospital.

Background Delirium has a prevalence of 14%-56% in hospitalized patients. Risk factors include advanced age, invasive mechanical ventilation (IMV), and prolonged intensive care unit stay. Neuropsychiatric symptoms have been reported to be related to autoimmune responses secondary to Guillain-Barré syndrome (GBS) with direct involvement of the central nervous system (CNS) or to delirium. There are few reports of the frequency of delirium in patients with Guillain-Barré syndrome (GBS). Objective To describe the frequency of and the characteristics associated with delirium in patients with GBS. Material and methods A single-center, cross-sectional study was conducted in patients with GBS diagnosis between 2015 and 2019. The diagnosis of delirium was made using the Diagnostic and Statistical Manual of Mental Disorders, 5th Edition (DSM-5) criteria. We compared patients with and without delirium. We performed both univariate and multivariate analyses to identify factors associated with delirium. Results A total of 154 patients with GBS were included, of which 20 (12.9%) fulfilled the DSM-5 criteria for delirium. The mean age was 48 ± 18.2 years, the median Medical Research Council (MRC) sum score was 29.3 ± 21.9 points, 65% had bulbar cranial nerve involvement, 80% presented autonomic dysfunction, 85% had ICU stay, and 90% had mechanical ventilation requirement. In the multivariate analysis, the following were the independent factors for the development of delirium: age ≥ 60 (odds ratio (OR): 5.7; 95% confidence interval (CI): 1.3-23.5), time from symptom onset to admission ≤ 3 days (OR: 4.3; 95% CI: 1.1-16.8), autonomic dysfunction (OR: 13.1; 95% CI: 3-56), and intensive care unit stay (OR: 9.5; 95% CI: 2.1-42.6). Conclusion Delirium is not frequent in patients with Guillain-Barré syndrome. Patients with advanced age, rapid motor progression, bulbar cranial nerve involvement, prolonged intensive care unit stay, and mechanical ventilation need are more likely to present delirium.

Prognostic Factors in Elderly Patients With Guillain-Barré Syndrome: Does Age Matter?

INTRODUCTION: Twenty to 40% of Guillain Barré syndrome (GBS) patients will not be able to walk independently despite effective treatment. Older patients carry additional risks for worse outcomes. METHODS: A single center, ambispective cohort study was performed. Only subjects ≥18 years with a 3-month follow-up were included. Elderly patients were considered as a whole if ≥ 60 years. Demographics, CSF and nerve conduction studies were compared. A binomial logistic regression and Kaplan-Meier analyses were carried out to estimate good prognosis (Hugues ≤2) at 3-month follow-up. RESULTS: From 130 patients recruited, 27.6% were elderly adults. They had a more severe disease, higher mEGOS and more cranial nerve involvement. Age ≥70 years, invasive mechanical ventilation and axonal subtype, portrayed an unfavorable 3-month outcome. Further analysis demonstrated an earlier recovery in independent walk at 3 months for patients <70 years. CONCLUSIONS: Elderly patients with GBS have a more severe disease at admission and encounter worse prognosis at 3-month follow-up, especially those above 70 years.

Mitochondrial Encephalomyopathy, Lactic Acidosis, and Stroke-like Episodes (MELAS) Syndrome: Frequency, Clinical Features, Imaging, Histopathologic, and Molecular Genetic Findings in a Third-level Health Care Center in Mexico.

INTRODUCTION: Mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome, is a multisystemic entity of mitochondrial inheritance. To date, there is no epidemiological information on MELAS syndrome in Mexico. CASE SERIES: A retrospective, cross-sectional design was employed to collect and analyze the data. The clinical records of patients with mitochondrial cytopathies in the period ranging from January 2018 to March 2020 were reviewed. Patients who met definitive Yatsuga diagnostic criteria for MELAS syndrome were included to describe frequency, clinical, imaging, histopathologic, and molecular studies. Of 56 patients diagnosed with mitochondrial cytopathy, 6 patients met definitive Yatsuga criterion for MELAS (10.7%). The median age at diagnosis was 34 years (30 to 34 y), 2 females and the median time from onset of symptoms at diagnosis 3.5 years (1 to 10 y). The median of the number of stroke-like episodes before the diagnosis was 3 (range, 2 to 3). The main findings in computed tomography were basal ganglia calcifications (33%), whereas in magnetic resonance imaging were a lactate peak in the spectroscopy sequence in 2 patients. Five patients (84%) had red-ragged fibers and phantom fibers in the Cox stain in the muscle biopsy. Four patients (67%) had presence of 3243A>G mutation in the mitochondrial MT-TL1 gene. One patient died because of status epilepticus. CONCLUSIONS: MELAS syndrome represents a common diagnostic challenge for clinicians, often delaying definitive diagnosis. It should be suspected in young patients with stroke of undetermined etiology associated with other systemic and neurological features.

Classic and overlapping Miller-Fisher syndrome: clinical and electrophysiological features in Mexican adults.

Classic and overlapping Miller-Fisher syndrome (MFS) have divergent clinical courses. Few studies have addressed the electrophysiological evaluation of MFS patients, most of them carried out in Asia. This work describes and compares their clinical and neurophysiological characteristics. From a Guillain-Barré syndrome (GBS) patient cohort, we made a selection of twenty MFS cases. We defined classic and overlapping MFS, as stated by Wakerley et al. (Nat Rev Neurol 10(9):537-544, 2014). We describe and compare clinical, biochemical, and electrodiagnostic parameters between groups. Seventy-five percent were men, mean age was 42.2 ± 13.6 years, and 45% had a Hughes score ≥ 3. MFS/GBS was the most frequent clinical subtype with 50%. Almost one-third had unaltered electrophysiological studies. Comparative analysis between groups showed statistically significant differences in length of stay, dysautonomia presence, and treatment type. Kaplan-Meier survival analysis showed that 100% of the patients had an independent walk at 3 months. This study reports Mexican MFS patient's characteristics and represents the most extensive case series in Latin America. We observed a high proportion of overlapping syndromes, a good recovery profile, and no significant severe complications.

Síndrome de Vulpian-Bernhardt. Frecuencia, características clínicas y electrofisiológicas en un centro de atención de tercer nivel en México / Vulpian-Bernhardt syndrome. Its frequency and clinical and electrophysiological features in a tertiary care centre in Mexico

INTRODUCCIÓN: El síndrome de Vulpian-Bernhardt (SVB) es un fenotipo clínico atípico e infrecuente de la esclerosis lateral amiotrófica (ELA) que condiciona un importante retraso diagnóstico, por lo que reconocer sus características clínicas y electrofisiológicas tiene relevancia. MATERIALES Y MÉTODOS: Estudio retrospectivo y transversal. Se revisaron los expedientes clínicos de pacientes con diagnóstico de ELA en el período de enero de 2017 a diciembre de 2019. Se incluyeron los que cumplían criterios para SVB para describir su frecuencia, características clínicas y electrofisiológicas. RESULTADOS: Veinte pacientes (15,8%) cumplieron los criterios para el SVB; el 55% eran mujeres; la edad de inicio de los síntomas era de 46,6 ± 12,9 años; presentaba tabaquismo el 40%; la mediana de retraso del diagnóstico fue de 24 (12-96) meses; la mediana en afectarse un segundo segmento corporal fue de 24 (12-132) meses, que fue el lumbosacro en el 65%; el promedio en la escala Revised Amyotrophic Lateral Sclerosis Functional Rating Scale fue de 27 ± 7 puntos; el 45% cumplía los criterios de El Escorial para ELA definida en el momento del diagnóstico y el 58,8%, los de Awaji. Se contó con 19 estudios de neuroconducción y 17 electromiogramas, y se encontró una razón abductor digiti minimi-abductor pollicis brevis (APB/ADM) < 0,6 en el 63% (mano dividida). CONCLUSIONES: Existe un retraso importante en el diagnóstico de enfermedades de la motoneurona en general y de SVB en particular. Calcular la razón APB/ADM y aplicar los criterios de Awaji en el estudio de electrofisiología puede ser de gran ayuda para aumentar la certeza diagnóstica en esta entidad clínica

INTRODUCTION: Vulpian-Bernhardt syndrome (VBS) is an atypical rare clinical phenotype of amyotrophic lateral sclerosis (ALS) that causes a significant delay in diagnosis, and thus it is important to recognise its clinical and electrophysiological features. MATERIALS AND METHODS: Retrospective cross-sectional study. We reviewed the clinical records of patients diagnosed with ALS in the period from January to December 2019. Those meeting criteria for VBS were included so as to describe their frequency as well as their clinical and electrophysiological features. RESULTS: Twenty patients (15.8%) met criteria for VBS; 55% were female; age at onset of symptoms was 46.6 ± 12.9 years; 40% were smokers; median delay in diagnosis was 24 (12-96) months; median time to involvement of the second body segment was 24 (12-132) months, which was lumbosacral in 65%; mean Revised Amyotrophic Lateral Sclerosis Functional Rating Scale score was 27 ± 7 points; 45% met the El Escorial criteria for ALS defined at diagnosis and 58.8% met the Awaji criteria. There were 19 nerve conduction studies and 17 electromyograms, and an abductor digiti minimi–abductor pollicis brevis (ADM/APB) ratio < 0.6 was found in 63% (split hand). CONCLUSIONS: There is a significant delay in the diagnosis of motor neuron diseases in general and more particularly in VBS. Calculating the ADM/APB ratio and applying the Awaji criteria in the electrophysiology study can be a valuable aid to increase diagnostic certainty in this clinical entity