Hyaluronidase-facilitated subcutaneous immunoglobulin 10% as maintenance therapy for chronic inflammatory demyelinating polyradiculoneuropathy: The ADVANCE-CIDP 1 randomized controlled trial.

BACKGROUND AND AIMS: ADVANCE-CIDP 1 evaluated facilitated subcutaneous immunoglobulin (fSCIG; human immunoglobulin G 10% with recombinant human hyaluronidase) efficacy and safety in preventing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) relapse. METHODS: ADVANCE-CIDP 1 was a phase 3, double-blind, placebo-controlled trial conducted at 54 sites in 21 countries. Eligible adults had definite or probable CIDP and adjusted Inflammatory Neuropathy Cause and Treatment (INCAT) disability scores of 0-7 (inclusive), and received stable intravenous immunoglobulin (IVIG) for ≥12 weeks before screening. After stopping IVIG, patients were randomized 1:1 to fSCIG 10% or placebo for 6 months or until relapse/discontinuation. fSCIG 10% was administered at the same dose (or matching placebo volume) and interval as pre-randomization IVIG. The primary outcome was patient proportion experiencing CIDP relapse (≥1-point increase in adjusted INCAT score from pre-subcutaneous treatment baseline) in the modified intention-to-treat population. Secondary outcomes included time to relapse and safety endpoints. RESULTS: Overall, 132 patients (mean age 54.4 years, 56.1% male) received fSCIG 10% (n = 62) or placebo (n = 70). CIDP relapse was reduced with fSCIG 10% versus placebo (n = 6 [9.7%; 95% confidence interval 4.5%, 19.6%] vs n = 22 [31.4%; 21.8%, 43.0%], respectively; absolute difference: -21.8% [-34.5%, -7.9%], p = .0045). Relapse probability was higher with placebo versus fSCIG 10% over time (p = .002). Adverse events (AEs) were more frequent with fSCIG 10% (79.0% of patients) than placebo (57.1%), but severe (1.6% vs 8.6%) and serious AEs (3.2% vs 7.1%) were less common. INTERPRETATION: fSCIG 10% more effectively prevented CIDP relapse than placebo, supporting its potential use as maintenance CIDP treatment.

Long-Term Remission With Low-Dose Rituximab in Myasthenia Gravis: A Retrospective Study.

OBJETIVE: Rituximab (RTX) is a therapeutic option, for patients with myasthenia gravis (MG) not responding to conventional immunosuppressive treatment. In this cohort, we evaluated long-term efficacy of RTX in the treatment of refractory generalized MG. METHODS: A retrospective study was performed in adult patients with refractory generalized MG and at least 24 months of follow-up, between January/2015 and October/2021. The Myasthenia Gravis Status and Treatment Intensity Score was used to assess outcomes, and CD19/CD20+ B-cell counts were monitored. RESULTS: Sixteen patients with MG (8 antiacetylcholine receptor+ and 8 muscle-specific antikinase+; mean age 45.5 ± 16.2 years) treated with low-dose RTX protocols were included. CD19/CD20 levels remained undetectable 12 months after induction, and no new relapses were observed during follow-up. CONCLUSIONS: Low-dose RTX infusions were sufficient to achieve undetectable CD19/20 cell counts and sustained clinical remission. In low and middle-income countries, the impact of low-dose RTX therapy represents a paradigm shift in decision-making for long-term treatment.

Childhood focal compressive mononeuropathies during the COVID-19 pandemic in Buenos Aires, Argentina.

INTRODUCTION/AIMS: Focal peripheral neuropathies are infrequently seen in pediatric patients. The COVID-19 pandemic has disrupted normal life for many people, including complete lockdowns and school closing for long periods of time in many countries, which prompted children to stay at home. Our aim is to assess whether there has been an increased incidence of focal compressive peripheral neuropathies in the pediatric population during COVID-19-associated lockdown. METHODS: Clinical, electrophysiological, and imaging characteristics were reviewed for patients referred to the electrodiagnostic (EDx) laboratory with suspicion of a focal neuropathy. The incidence of focal compressive peripheral neuropathies seen during the period of March to September 2020 was compared with the same time period in 2019. RESULTS: An increased incidence of focal neuropathies was seen in 2020 (31%) compared with 2019 (6.8%). During 2020, 7 fibular (peroneal) mononeuropathies and 2 ulnar neuropathies were diagnosed. Most patients with focal neuropathies were underweight and acknowledged prolonged screen time periods. Electrophysiological findings consisted of mostly demyelinating lesions with an overall good clinical outcome. DISCUSSION: In this study we raise awareness about a possible increased incidence of focal compressive peripheral neuropathies in children during COVID-19-associated lockdown, which may be prevented with changing positions during sedentary activities.

Re-emergent tremor provocation.

INTRODUCTION: Rest and re-emergent tremor (RET) in Parkinson's disease (PD) are known to be markedly variable. The aim of this study is to evaluate the effect of tremor provocation on RET latency and variability. METHODS: We performed a prospective observational study in 21 PD patients with RET. Evaluations were conducted by accelerometric analysis of hand movements with and without provocation by counting out loud backwards from 100, in the OFF state. Differences in RET pause duration, tremor power at peak frequency, root mean square (RMS) and slope of return of the tremor after the pause was measured. Inter- and intra-subject variability were also calculated. RESULTS: RET pause duration showed a 75% decrease after provocation (p < 0.001), which led to zero in 52% of cases, as compared to 9% in unprovoked measurements (p < 0.001). Provocation also led to a 2.57 time increase in tremor power (p < 0.001), 1.37 time increase in RMS (p < 0.001) and 2.47 time increase in slope (p < 0.001). A significant decrease in inter-subject variability was also observed (p = 0.001). CONCLUSION: Tremor provocation led to RET amplitude increase, pause shortening, and variability decrease. Therefore, while provocation can be recommended for the evaluation of rest tremor in clinical practice, it might well annul its value for identifying the pause prior to re-emergent tremor.

Latency of re-emergent tremor in Parkinson's disease is influenced by levodopa.

INTRODUCTION: Re-emergent tremor (RET) is a common form of postural tremor observed in Parkinson's disease (PD) patients. Recent studies have shown that administration of levodopa decreases RET amplitude. However, drug effects on tremor pause duration are less clear. METHODS: We performed a prospective observational study in PD patients with RET, subjected to acute levodopa challenge. Tremor activity was measured during OFF and ON states both clinically, as well as by using accelerometers taped to the back of both hands. Correlation between RET amplitude and pause duration, as well with MDS-UPDRS scores were investigated. The slope of gradual increase of postural tremor after the pause was also measured in the OFF and ON states. RESULTS: Significant inverse correlation between tremor amplitude and RET pause duration was observed in OFF (rs = -0.474, p = 0.030) and ON (rs = -0.569, p = 0.006) states. Levodopa reduced tremor amplitude (26%, p = 0.004) dampening slope gradient (22%, p = 0.029). Tremor pause duration also showed inverse correlation with postural tremor amplitude measured by MDS-UPDRS in OFF (rs = -0.311, p = 0.048) and ON (rs = -0.503, p = 0.020) states, as well as with total MDS-UPDRS Part III score (rs = -0.295, p = 0.009). Finally, accelerometric analysis proved to be more sensitive than visual inspection for detecting tremor pauses. CONCLUSION: Our results suggest RET pause duration is amplitude related, since levodopa-induced amplitude decrease led to pause prolongation, associated with decreased tremor intensity and slope gradient dampening.

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.

Experiencia en la cirugía del bocio tóxico / Experience in surgery of toxic goiter

Introducción: se hace recuento del hipertiroidismo y sus dos causas principales donde prima la enfermedad de Graves Basedow. Se mencionan su diagnóstico y tratamiento. Objetivo: determinar el comportamiento de la cirugía en el bocio tóxico en un grupo de trabajo del servicio de cirugía del Hospital Universitario Amalia Simoni de Camagüey. Métodos: se realizó un estudio retrospectivo transversal sobre bocio tóxico en 12 años con una muestra conformada por 57 pacientes operados en un grupo de trabajo. Se tuvieron en cuenta, aspectos clínicos, de diagnóstico y terapéuticos. Resultados: predominó el sexo femenino en un rango de edades de 21 a 40 años, así como la positividad de los complementarios realizados. Se expuso la preparación preoperatoria más utilizada, la técnica quirúrgica realizada y las complicaciones en el orden del 29,81 porciento. Conclusiones: la mayoría fueron mujeres, con edad media de 35 años, se subutilizaron algunos medios diagnósticos, la preparación preoperatoria más usada fue propiltiuracilo, propanolol y lugol. El proceder más realizado fue la tiroidectomía subtotal y las complicaciones no fueron ni elevadas ni graves(AU)

Introduction: a recount of hyperthyroidism and its two principal causes in which Graves-Basedow disease takes priority is made. Its diagnosis and treatment are also mentioned. Objective: to determine the surgical behavior of toxic goiter in a work group of the Surgery Service at Amalia Simony University Hospital in Camaguey. Methods: a transversal retrospective study about toxic goiter was conducted during 12 years. The sample was composed of 57 patients who were operated on by a work group. Clinical, diagnostic and therapeutic aspects were considered. Results: the female sex predominated between the ages of 21 and 40 years, as well as the positive results of the complementary tests performed. The most used preoperative preparation, the surgical technique applied and the complications were shown in the order of 29.81 percent. Conclusions: most of the patients were women, with a mean age of 35 years; some diagnostic methods were sub-utilized; the most used preoperative preparations were propylthiouracil, propranolol and lugol. The most preformed procedure was the subtotal thyroidectomy and the complications were neither high nor serious(AU)

Experiencia en la cirugía del bocio tóxico / Experience in surgery of toxic goiter

Introducción: se hace recuento del hipertiroidismo y sus dos causas principales donde prima la enfermedad de Graves Basedow. Se mencionan su diagnóstico y tratamiento. Objetivo: determinar el comportamiento de la cirugía en el bocio tóxico en un grupo de trabajo del servicio de cirugía del Hospital Universitario Amalia Simoni de Camagüey. Métodos: se realizó un estudio retrospectivo transversal sobre bocio tóxico en 12 años con una muestra conformada por 57 pacientes operados en un grupo de trabajo. Se tuvieron en cuenta, aspectos clínicos, de diagnóstico y terapéuticos. Resultados: predominó el sexo femenino en un rango de edades de 21 a 40 años, así como la positividad de los complementarios realizados. Se expuso la preparación preoperatoria más utilizada, la técnica quirúrgica realizada y las complicaciones en el orden del 29,81 porciento. Conclusiones: la mayoría fueron mujeres, con edad media de 35 años, se subutilizaron algunos medios diagnósticos, la preparación preoperatoria más usada fue propiltiuracilo, propanolol y lugol. El proceder más realizado fue la tiroidectomía subtotal y las complicaciones no fueron ni elevadas ni graves(AU)

Introduction: a recount of hyperthyroidism and its two principal causes in which Graves-Basedow disease takes priority is made. Its diagnosis and treatment are also mentioned. Objective: to determine the surgical behavior of toxic goiter in a work group of the Surgery Service at Amalia Simony University Hospital in Camaguey. Methods: a transversal retrospective study about toxic goiter was conducted during 12 years. The sample was composed of 57 patients who were operated on by a work group. Clinical, diagnostic and therapeutic aspects were considered. Results: the female sex predominated between the ages of 21 and 40 years, as well as the positive results of the complementary tests performed. The most used preoperative preparation, the surgical technique applied and the complications were shown in the order of 29.81 percent. Conclusions: most of the patients were women, with a mean age of 35 years; some diagnostic methods were sub-utilized; the most used preoperative preparations were propylthiouracil, propranolol and lugol. The most preformed procedure was the subtotal thyroidectomy and the complications were neither high nor serious(AU)

Lack of maintenance of gait pattern as measured by instrumental methods suggests psychogenic gait.

Fluctuation is a common feature of all psychogenic gait disorder (PGD) patterns. Whether this fluctuation involves only the degree of impairment or whether it affects the gait pattern itself remains an interesting question. We hypothesize that, on repeated measurements, both normal and abnormal gait may present quantitative differences while maintaining their basic underlying pattern; conversely, in psychogenic gait, the basic pattern appears not to be preserved. Using an optoelectronic system, data acquired from 19 normal subjects and 66 patients were applied to train a neural network (NN) and subsequently classify gait patterns into four different groups (normal, ataxic, spastic-paraparetic and parkinsonian). Five patients who fulfilled clinical criteria for psychogenic gait and six controls were then prospectively evaluated on two separate occasions, three months apart. Normal controls and ataxic, parkinsonian or spastic patients were correctly identified by the NN, and categorized within the corresponding groups at baseline as well as at a three-month follow-up evaluation. NN analysis showed that after three months, no PGD patient preserved the gait pattern detected at baseline, even though this finding was not clinically apparent. Modification of gait pattern detected by repeated kinematic measurement and NN analysis could suggest the presence of PGD, particularly in difficult-to-diagnose cases.

Tricobezoar gástrico: Presentación de un caso

Se presenta una paciente joven, de 28 años de edad, que acude a consulta de gastroenterología de la clínica popular "Simón Bolívar" por presentar náuseas, vómitos esporádicos, dolor abdominal, dispepsias y tumor abdominal. Al examen físico se constató una masa palpable en epigastrio, dura,móvil y dolorosa. Se le realizó ecografía abdominal que mostró una masa epigástrica, además, un embarazo. El examen contrastado de estómago y duodeno reveló defecto de lleno del estómago; la endoscopia digestiva alta evidenció un tricobezoar de gran tamaño que imposibilitó el avance del equipo. Tres meses después de realizársele cesárea se extrajo el tricobezoar gástrico a través de una gastrostomía anterior. La evolución fue satisfactoria y se le dio de alta con seguimiento por psiquiatría.

A 28-year-old female patient was admitted to the gastroenterology consult of the "Simon Bolivar Popular Clinic". Her symptoms were: nausea, sporadic vomiting, abdominal pain, dyspepsia, and an abdominal tumor. After medical examination, a hard, painful moving mass located in the epigastry was stated. An abdominal ecography was showed an epigastric mass, in addition pregnancy was also detected. A contrasted examination of the stomach and duodenum revealed a filling stomach defect. The high digestive endoscopy revealed a tricobezoar of large size that obstructed the advance of the equipment. Three months after making a Caesarean procedure, the gastric tricobezoar was extracted through a frontal gastrostomy. The patient evolution was satisfactory, and she was dismissed with psychiatric follow up.