Osvaldo Jm Nascimento

Guillain-Barré syndrome associated with the Zika virus outbreak in Brazil

Zika virus (ZIKV) is now considered an emerging flavivirosis, with a first large outbreak registered in the Yap Islands in 2007. In 2013, a new outbreak was reported in the French Polynesia, with associated cases of neurological complications including Guillain-Barré syndrome (GBS). The incidence of GBS has increased in Brazil since 2015, what is speculated to be secondary to the ZIKV infection outbreak. The gold-standard test for detection of acute ZIKV infection is the polymerase-chain reaction technique, an essay largely unavailable in Brazil. The diagnosis of GBS is feasible even in resource-limited areas using the criteria proposed by the GBS Classification Group, which is based solely on clinical grounds. Further understanding on the relationship of ZIKV with neurological complications is a research urgency.

Zika Virus Infection in the Elderly: Possible Relationship with Guillain-Barré Syndrome

The Zika virus (ZIKV) outbreak in French Polynesia, in 2013, and in Brazil, in 2015, was correlated with neurological complications, which comprised, among others, congenital microcephaly and Guillain-Barré syndrome (GBS), which includes a group of acute autoimmune neuropathies generally reported after respiratory or gastrointestinal infectious diseases. Despite being relatively rare, the incidence rate of GBS rises with age, which makes GBS more frequent in the elderly, in whom it is also a more severe disease with slower recovery than in younger patients. Different forms of GBS have been described having diagnostic confirmation of a previous infection with the ZIKV virus. Although we do not have enough evidence that elderly people are a particularly susceptible population to developing GBS following ZIKV infection, this is plausible. We should consider this possibility, particularly taking into account that aging subjects are more susceptible to infections. In this context, a deeper understanding of how the immune system in the elderly functions in relation to ZIKV infection is necessary, as well as an understanding of what kind of alterations of the nervous system such an infection triggers in the elderly, beyond GBS. This will be relevant for better therapeutic interventions and for designing vaccine candidates that can be applied in an aging population, particularly those prone to develop ZIKV-induced autoimmunity.

Scrutinizing brain magnetic resonance imaging patterns in Angelman syndrome.

BACKGROUND Global developmental delay, lack of speech, and severe epilepsy are the characteristic hallmarks of Angelman syndrome (AS). The purpose of this study was to explore the utility of brain magnetic resonance imaging (MRI) as an ancillary tool for the diagnosis of AS. MATERIAL AND METHODS Brain MRI images of nine laboratory-confirmed patients with AS from a neurorehabilitation center in Rio de Janeiro were reviewed. Each MRI was assessed by a set of two experienced neuroradiologists following a predefined protocol. RESULTS The main neuroimaging findings revealed in our study were: Thinning of the corpus callosum in five patients; enlargement of lateral ventricles in four patients; and, cerebral atrophy with frontal and temporal predominance in one patient. All patients presented with an increased signal intensity in T2-weighted images and fluid-attenuated inversion recovery (FLAIR) sequences. CONCLUSION The lack of specific changes in the brain MRI of children with AS observed in this case series rendered brain MRI a less helpful complementary test. Thus, a definitive diagnosis of AS could only be established on molecular biology that was undertaken based on the clinical suspicion of AS.

The broad clinical spectrum of hereditary neuropathy with liability to pressure palsy (HNPP)

editorial The broad clinical spectrum of hereditary neuropathy with liability to pressure palsy (HNPP) H ereditaty neuropathy with liability to pressure palsy (HNPP) is an autosomal dominant neuropathy encompassing a broad clinical phenotype spectrum, which usually makes its diagnosis difficult in an exclusive clinical basis. Recurrent sensory and motor neuropathy in a single nerve beginning in adolescence or young adulthood is characteristically seen. In most cases this recurrent clinical evolution is due to a PMP22 gene deletion. Mononeuropathies resulting from nerve pressure in entrapment points are the most common presentation, but an almost symmetric polyneuropathy can eventually be seen 1. Suggestive electrophysiological findings that draw attention for HNPP diagnosis are in consideration. DNA mutations tests are diagnostic, but not always available. Although it is an autosomal dominant condition, family history can be lacking in many cases, especially in Brazil, a country with a strong migration process and with different ethnics groups. In a recent comparison of causes of neuropathy in peripheral neuropathy reference centers in the United State of America and South-America (NA-SA project) we have observed a higher percentage of diagnoses of inherited neuropathies in the USA in comparison with Brazil: NA 292 (26.7%), SA 103 (10%) 2. In an American international reference center of inherited neuropathy an epidemiological study, including 787 Charcot-Marie-Tooth disease (CMT) patients, showed that only 67% received molecular diagnosis 3. This result points that the diagnosis is performed mainly in clinical/ neurophysiological basis. This edition of Arquivos de Neuropsiquiatria includes an elegant study by Oliveira et al. 4 reporting the clinical and neurophysiological features of HNPP due to the 17p11.2 deletion in 39 Brazilian patients, looking for its diagnostic characteristics. In this series, family history was present in 59% of cases, showing that clinical presentation, recurrence of signs and symptoms , and neurophysiological findings are strong elements for diagnostic suspicion. A progressive course is uncommon, but was reported in 20% of their HNPP patients 4. Older presentation of HNPP was seen in this series, in contrast with others. The first clinical manifestation was the classical painless muscle weakness with at least one episode of acute nerve paralysis (61,5% of cases). This painless acute or subacute mononeuropathy is the classical phenotype 1. Pes cavus and nerve thickening, seen in CMT patients, was rare in Oliveira et al. 4 HNPP series. Heterogeneity in clinical presentation and disease course is the rule in this neuropathy 1,5. Atypical presentations …

Prolonged Physical Effort Affects Cognitive Processes During Special Forces Training

This study aimed to investigate the effects of strenuous physical exertion on biomarkers of muscle damage, on physical and mental fatigue, and on cognitive processes. Seventeen military (males 24–40 years old) were tested cognitively at six time points, while they were progressively exhausted over the course of 102 h of continued operations. Three types of variables were analyzed: biomarkers of muscle damage [serum levels of creatine kinase (CK) and lactate dehydrogenase (LDH)], reported physical fatigue (PF) and mental fatigue (MF), and cognitive processes [(verbal reasoning (VR), numerical reasoning (NR) and spatial reasoning (SR) and short-term memory (STM)]. The results revealed significant increases in CK, LDH, PF and MF. On the other hand, we found significant decreases in VR, NR, SR and STM, which were negatively correlated MF. Our results show additional evidences about the impact of strenuous physical exertion on muscle damage, physical and mental fatigue, and cognitive processes.

Tension pneumocephalus and rhinorrhea related to chronic sinusitis

1Medical residents of Neurology, Department of Neurology, Federal Fluminense University, Niterói RJ, Brazil; 2MD, PhD, Professor of Neurology, Department of Neurology, Federal Fluminense University, Niterói RJ, Brazil; 3Ophthalmologist, Post-graduating program in Neurology and Neuroscience, Federal Fluminense University, Niterói RJ, Brazil; 4Radiologist at Antonio Pedro Hospital, Federal Fluminense University, Niterói RJ, Brazil. Correspondence: Victor de Almeida Kosac; Rua Doutor Paulo César 25 / apto. 1.608; 24240-000 Niterói RJ Brasil; E-mail: victorkosac@yahoo.com.br Conflict of interest: There is no conflict of interest to declare. Received 18 March 2012; Received in final form 26 November 2012; Accepted 03 December 2012. 1. Lefranc M, Peltier J, Demuynkc F, et al. Tension pneumocephalus and rhinorrhea revealing spontaneous cerebrospinal fluid fistula of the anterior cranial base. Neurochirurgie 2009;55:340-344. 2. Webber-Jones JE. Tension pneumocephalus. J Neurosci Nurs 2005;37: 272-276. References A 42-year-old woman presented with a sudden-onset severe headache associated with vomiting and a persistent aqueous rhinorrhea. She had an allergic chronic sinusitis. There was no history of head trauma. Neurologic exam disclosed papilledema. The computed tomography (CT) scan showed opacification of the left sphenoid sinus and pneumocephalus extending from frontal region until convexity, compressing the supratentorial ventricular system (Fig 1). The cerebrospinal fluid (CSF) fistula was found in the left pterygoid process (Fig 2). Air probably came through the dural defect, and may have followed the CSF flow circuit. The physiopathology can be explained by bone defect, absence of nasal mucosa, and minor traumas1,2.

Encefalopatia mioclonica pos-anoxica (sindrome de Lance-Adams): estudo anatomopatológico de dois casos

Foram feitos estudos neuropatologicos em dois casos de pacientes que apresentaram encefalopatia mioclonica pos-anoxica (sindrome de Lance-Adams). O encefalo mostrou lesoes neuronais difusas, comprometendo principalmente o cortice, talamo e estruturas sub-talâmicas, desde lesoes discretas caracterizadas pela presenca de vacuolos intracitoplasmaticos (primeira alteracao vista na anoxia) ate neuronios totalmente degenerados, notando-se varios neuronios com lesoes classicas de isquemia. A presenca de material de inclusao anfofilica discretamente PAS positiva observado no citoplasma neuronal foi diferente das inclusoes verificadas nos casos de epilepsia mioclonica com corpusculos de Lafora. Nao conseguimos identificar a constituicao destas inclusoes, apesar de serem feitos diferentes metodos de coloracao. Nao encontramos tambem, na literatura, referencia a tal tipo de inclusao. Foram tambem encontradas alteracoes vasculares, consistindo de vasos proliferados com celulas endoteliais tumefeitas. Tais celulas apresentaram-se com disposicao anarquica, provavelmente devidas a anoxia isquemica. A diferenca dos achados anatomopatologicos, entre os casos 1 e 2, sao provavelmente decorrentes do mecanismo diverso da instalacao da anoxia.Two cases of Lance-Adams syndrome with anatomopathologic study are reported. There were evidences of diffuse neuronal degeneration in the brain. These changes were most seen in the neurones of the cortical layers, thalamus and subthalamic nuclei. The cells changes were similar of those seen in ischaemic disease. Some neurones showed intracytoplasmatic inclusions staining with the P.A.S. method. These inclusions were readily distinguished from the Lafora bodies.