Intraoperative monitoring of sensory part of the trigeminal nerve using blink reflex during microvascular decompression for trigeminal neuralgia.

Intraoperative monitoring during cerebellopontine angle surgery is widely accepted. While techniques which monitor cranial motor nerves are commonly used, monitoring the sensory afferents has been challenging. Considering the reflex arc, blink reflex (BR) might be useful in monitoring the sensory part of the trigeminal nerve, the brainstem connections and the facial nerve. We describe the case of a patient who developed hemifacial hypoesthesia after microvascular decompression surgery for trigeminal neuralgia. Intraoperative BR showed a severe loss of R1 amplitude. BR might be a useful intraoperative technique to monitor the sensory part of the trigeminal nerve.

Peripheral neuropathy in 30 duodopa patients with vitamins B supplementation.

OBJECTIVES: Peripheral neuropathy (PN) is a significant concern and potential cause of withdrawal in patients with Parkinson's disease (PD) treated with Levodopa/Carbidopa Intestinal Gel (LCIG) infusion. Vitamin B deficiency and/or hyperhomocysteinemia levodopa-related are considered possible causative factors. In this study, we evaluated PN incidence in LCIG-PD patients treated since the beginning of infusion with vitamins B supplementation. MATERIALS & METHODS: In this prospective open-label pilot study, 30 consecutive patients with PD on LCIG infusion were evaluated with clinical, neurophysiological, and biochemical assessments for a mean follow-up of 42.4 months (range 24-72). All evaluations were repeated every 6 months. RESULTS: At baseline, 21 of 30 presented no signs or symptoms of PN, and 9 of 30 had pre-existing chronic PN. In whole population, a progressive worsening in nerve conduction studies of sural sensory and peroneal motor nerves was observed during the long-term follow-up. 4 of 21 patients, with normal clinical, electrophysiological assessment at baseline, developed distal symmetrical axonal polyneuropathy that remained asymptomatic during the long-term follow-up. Patients with pre-existing PN (9 of 30) showed a mild worsening of electrophysiological features during the period of observation. In none PN was cause of discontinuation of LCIG therapy. No incident cases of acute-subacute PN were documented. No correlation was found with age, sex, Levodopa dosage, duration of levodopa exposure, and homocysteine plasma levels. CONCLUSION: In this consecutive series of 30 patients with PD on LCIG infusion, with early and continuous vitamins B integration, we observed a low rate (19%) of new onset peripheral polyneuropathy that remained stable after long-term follow-up. Larger studies, controlled, with blinded evaluation, are needed to confirm these findings.

Very Early Carotid Endarterectomy After Intravenous Thrombolysis.

OBJECTIVE/BACKGROUND: The timing of carotid endarterectomy (CEA) after thrombolysis is still a matter of debate. The aim of this study was to analyse a cohort of patients undergoing urgent endarterectomy after intravenous thrombolysis for acute ischaemic stroke. METHODS: This was an observational study. Prospective databases were reviewed and matched to identify patients who underwent CEA early after intravenous thrombolysis (2009-14). The focus was carotid surgery performed within 12 hours of stroke onset in patients with a high grade (≥70%) symptomatic carotid stenosis, associated with vulnerable plaques or stroke in evolution, and evidence of a significant salvageable ischaemic penumbra on perfusion computed tomography scan. Demographic and clinical information, as well as data on relevant outcomes were extracted. RESULTS: Thirty four consecutive stroke patients who underwent CEA within 2 weeks of thrombolysis for acute ischaemic stroke and ipsilateral high grade carotid stenosis were identified. In 11 patients the surgical procedure was performed within 12 hours of the onset of symptoms. All patients showed a clinical improvement after combined treatment. The 3 month outcome was favourable (modified Rankin Scale ≤ 2) in 10 patients. No haemorrhagic complications were registered. There was neither peri-operative stroke nor stroke within 3 months of surgery. One patient died from acute myocardial infarction 3 days after intervention. CONCLUSION: This experience suggests that very early CEA after thrombolysis, aimed at removing the source of potential embolisation and restoring blood flow, may be safe and can lead to a favourable outcome.

Emerging issues on selection criteria of levodopa carbidopa infusion therapy: considerations on outcome of 28 consecutive patients.

Many studies confirmed the efficacy and safety of continuous infusion of intrajejunal levodopa/carbidopa gel (CIILG) for advanced Parkinson's disease (PD). Although this treatment is widely used, definite inclusion/exclusion criteria do not exist. In this prospective open-label study, we evaluated the long-term outcome in 28 consecutive patients and sought to detect any predictive factor to identify the best candidates for CIILG therapy. The assessment was carried out routinely at baseline, after 6 months and every year with UPDRS III-IV, FOG Questionnaire, non-motor symptoms scale, PD questionnaire (PDQ-8), cognitive and psychiatric status evaluation (MMSE, FAB, NPI) and caregiver's quality of life. 17/28 patients reached the 24-month follow-up. A statistically significant beneficial effect was shown on motor complications in short- and long-term follow-up, also on axial symptoms like gait disturbances. A concomitant improvement in PDQ8 score was observed, with a parallel mild amelioration, but not significant, on Caregivers QoL. When classified according to their outcome on QoL, the only predictive positive factor was less severe at Neuropsychiatric Inventory (NPI) score at baseline. Considering the improvement in motor scores (duration of "off" period), the more advanced age was associated with a poorer outcome. Our results confirmed a sustained efficacy and safety in long-term follow-up and suggest that younger age at operation and absence or mild presence of psychiatric/behavioural symptoms could be considered valid predicting factors in selecting the best candidates for this efficacious therapy.

Explosive-aggressive behavior related to bilateral subthalamic stimulation.

We describe a patient with Parkinson's Disease who underwent bilateral subthalamic nucleus deep brain stimulation and later presented with episodes of aggressive behavior disorder with disturbed impulse control and an inability to control anger likely related to the deep brain stimulation "switch-on stimulation". We hypothesize that increasing voltage intensity could influence neighboring passing fibers coming from basal limbic system that are involved in the regulation of affect and emotional behavior. We suggest investigating these neuropsychological disturbances considering their influence on quality of life after surgery.

Observational clinical study in juvenile-adult glycogenosis type 2 patients undergoing enzyme replacement therapy for up to 4 years.

The objective of this study was to describe a large Italian cohort of patients with late-onset glycogen storage disease type 2 (GSDII) at various stages of disease progression and to evaluate the clinical effectiveness of alglucosidase alpha enzyme replacement therapy (ERT). Previous studies showed in late-onset patients ERT efficacy against placebo and variable response in uncontrolled studies. Seventy-four juvenile or adult GSDII patients were treated with ERT in a multicenter open label, non-randomized study, from 12 months up to 54 months. Recombinant human alpha glucosidase (rh-GAA) was injected by intravenous route at 20 mg/kg every second week. Patients were divided into three groups according to ERT duration: Group A received treatment for 12-23 months (n = 16), Group B for 24-35 months (n = 14), and Group C for more than 36 months (n = 44). Clinical assessment included a 6-min walk test (6MWT), forced vital capacity (FVC), the Walton and Gardner-Medwin score, the number of hours of ventilation, body mass index, echocardiography and blood creatine kinase (CK). Included in our cohort were 33 males and 41 females (M:F = 0.8:1), with a mean age at first symptoms of 28.3 years (range 2-55 years) and a mean age of 43 years at study entry (range 7-72 years). Seven wheelchair bound patients, as well as 27 patients requiring ventilation support, were included. After treatment we could observe an increase in distance walked on the 6MWT in the large majority of patients (48/58; 83%), with an overall mean increase of 63 m (from 320 ± 161 to 383 ± 178 m). After treatment in the majority of patients FVC was improved or unchanged (45/69; 65%). In ventilated patients we observed an improvement in average number of hours off the ventilator (from 15.6 to 12.1 h). Six patients stopped mechanical ventilation and two others started it. The effect of therapy was not related to ERT duration. Nine of 64 patients (13%) that underwent to echocardiography showed a variable degree of cardiac hypertrophy (left ventriculum or septum), and a positive effect was observed after 36 months of ERT in one adult case. Discontinuation of treatment occurred in four patients: one drop-off case, one patient died for a sepsis after 34 months of treatment and two patients stopped ERT for worsening of general clinical condition. Mild adverse effects were observed in four cases (5%). This study represents the largest cohort of late-onset GSDII patients treated with ERT, and confirm a positive effect of treatment. These results, obtained in a large case series on therapy, indicate a favourable effect of ERT therapy, even in more advanced stage of the disease.

Avaliação e manejo inicial da insuficiência respiratória aguda na criança / Assessment and initial management of acute respiratory failure in children

O comprometimento do sistema respiratório é uma das principais causas de atendimento a crianças nos serviços de emergência, com a gravidade variando desde quadro leve e autolimitado até doença fatal. A insuficiência respiratória aguda não reconhecida é a principal causa de parada cardíaca na população pediátrica. A capacidade de produzir um diagnóstico precoce, reconhecer e manejar adequadamente o comprometimento respiratório em crianças é habilidade essencial para médicos que trabalham em serviços de urgência. O objetivo deste artigo é apresentar uma abordagem, por meio de uma avaliação sistemática, que permita diagnóstico precoce e manejo inicial do comprometimento respiratório agudo em crianças atendidas em serviços de emergência.

Diseases of the respiratory system are some of the leading causes of child care in emergency services, with severity ranging from mild and self-limiting to life-threatening. Undetected acute respiratory failure is the main cause of cardiac arrest in children. The ability to recognize, produce an early diagnosis, and properly manage respiratory impairments in children are essential skills for doctors working in emergency services. The purpose of this article is to present an approach to systematic evaluation so as to enable early diagnosis and initial management of acute respiratory failure in children in emergency services.

Sequência rápida de intubação: uma abordagem prática para o pediatra em situações de urgência / Rapid sequence intubation: a practical approach for the pediatrician in the emergency room

A intubação é, na Pediatria, um dos principais procedimentos realizados em situações de urgência. E sua sequência rápida ? que compreende a administração sequencial ou simultânea de um agente indutor (analgésico e sedativo) e um relaxante neuromuscular ? é prática pouco comum entre os pediatras. No entanto, muitos estudos têm mostrado que a mesma melhora muito as condições de intubação, reduzindo complicações doprocedimento em si. Esse artigo se propõe a apresentar uma atualização das principais medicações disponíveis bem como as indicações de uso de cada uma nas situações de intubação na sala de urgência e CTI.

A intubação é, na Pediatria, um dos principais procedimentos realizados em situações de urgência. E sua sequência rápida - que compreende a administração sequencial ou simultânea de um agente indutor (analgésico e sedativo) e um relaxante neuromuscular - é prática pouco comum entre os pediatras. No entanto, muitos estudos têm mostrado que a mesma melhora muito as condições de intubação, reduzindo complicações doprocedimento em si. Esse artigo se propõe a apresentar uma atualização das principais medicações disponíveis bem como as indicações de uso de cada uma nas situações de intubação na sala de urgência e CTI.

Intubation is one of the key procedures in emergency situations in Pediatrics. Rapid sequence intubation, which comprises the simultaneous or sequential administration of inducing agents (sedatives and analgesics) and neuromuscular relaxants, is not a common practice among pediatricians, despite many studies showing that it greatly improvesintubating conditions and even reduces complications in the procedure. This article presents an update of the main available drugs as well as the indicated uses in airway management in the ICC.

Choque séptico em pediatria: manejo incial do paciente no pronto-atendimento ou enfermarias pediátricas / Septic shock in pediatrics: initial management of patients in the emergency room or pediatric wards

Choque séptico e sepse grave são as principais causas de morbidade e mortalidade em crianças. Iniciativas que promovem o diagnóstico precoce e tratamento agressivo pelos médicos que fazem o primeiro atendimento podem reduzir a mortalidade relacionada à sepse de maneira significativa. Este artigo é uma atualização terapêutica sobre o tratamento do choque séptico em pediatria na primeira hora após o diagnóstico, direcionada para médicos que atuam em serviços de emergências e enfermarias pediátricas.

Severe sepsis and septic shock are major causes of morbidity and mortality in children. Early diagnosis and aggressive treatment by doctors who provide the first care can reduce sepsis-related mortality significantly. This is therapeutic update on the treatment of septic shock in pediatrics at the first hours after diagnosis, targeted to physicians who work in emergency departments and pediatric wards.

Ultra-som na avaliaçäo da doença nodular da tireóide: experiência de um grupo multidisciplinar / Ultrasonography in the evaluation of thyroid nodular disease: experience of a multidisciplinary group

O objetivo do trabalho foi correlacionar as características ecográficas dos nódulos tireóideos aos achados citológicos, para determinar a importância da ultra-sonografia na doença nodular da tireóide. Foram realizadas punçöes aspirativas por agulha fina orientadas por ultra-som, após avaliaçäo e classificaçäo ecográfica de 436 nódulos. Obteve-se celularidade adequada para análise em 401 nódulos (92 por cento), procedendo-se à correlaçäo ecográfico/citológica. A classificaçäo utilizada permitiu distribuir as características ecográficas de acordo com o risco de malignidade, identificando nódulos Grau III císticos com foco sólido (citologia positiva em 23,8 por cento) e sólidos hipoecóicos (40 por cento) como suspeitos e os Grau IV (95,2 por cento) como altamente suspeitos. Os nódulos Grau II sólidos iso e hiperecóicos (5 por cento) e mistos (1 ,9 por cento), em acordo com a literatura, foram considerados provavelmente benignos. O ultra-som forneceu auxílio importante à avaliaçäo clínica e determinaçäo da conduta a ser tomada, devendo fazer parte dos protocolos para avaliaçäo inicial e acompanhamento da doença nodular da tireóide.