Maria Lucia Brito Ferreira

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.

Alicata disease: neuroinfestation by Angiostrongylus cantonensis in Recife, Pernambuco, Brazil

. Once infested, the larvae pen-etrate the vasculature of the intestinal tract and pro-mote an inflammatory reaction with eosinophilia and lymphocytosis. This produces rupture of the blood-brain barrier, changes to nervous tissue and damage to the Purkinje cells in the cerebellum, thereby promot-ing eosinophilic meningoencephalitis or Alicata disease

Cognitive deficits in multiple sclerosis: a systematic review

OBJECTIVE To present the results of prospective and retrospective studies on multiple sclerosis patients cognitive dysfunctions, as well as to discuss the batteries of neuropsychological tests used in these investigations. METHOD A systematic review was performed involving 40 studies published from 1997 to 2009, in PubMed, Scopus, Ovid, ISI Web of Knowledge, Scientific Electronic Library on line (Scielo) and Latin-American and Caribbean Center of Health Sciences Informations database. Clear description of multiple sclerosis patients cognitive deficits evaluation, study design, sample size; inclusion-exclusion and discontinuation criteria; instruments for neuropsychological evaluation, diagnostic methods, evaluated functions and detailed statistical analysis had been the inclusion criteria. RESULTS There is consensus on cognitive impairment of multiple sclerosis patients, especially on memory, speed processing, executive function, attention and concentration domains. One has identified use of 23 batteries and 74 neuropsychological tests, despite the recommendation of Consortium of Multiple Sclerosis Centers to the application of MACFIMS battery. CONSIDERATIONS The absence of the uniformization for multiple sclerosis patients cognitive evaluation battery has precluded to achieve evidences to recommend its incorporation on diagnostic routine. Nevertheless this tendency is already outlined.

Epidemiologia de 118 casos de esclerose múltipla com seguimento de 15 anos no centro de referência do Hospital da Restauração de Pernambuco

The prevalence of multiple sclerosis (MS) has a considerable variability all over the world. According to Kurtzke, South America is considered to have low prevalence (minor than 5 cases: 100000 habitants). OBJECTIVE: To describe the MS epidemiology on a reference center, compared to eight Brazilian series. METHOD: according to a descriptive, prospective, longitudinal, prevalence study, we analyzed data of 118 patients, who attended at the Reference Center for Attention to Patients with Demielinizating Diseases of Hospital da Restauracao (CRAPPDD-HR) - Recife - Pernambuco, Brazil, from January 1987 to March 2002, to have MS diagnosed according to Poser criteria. RESULTS: The patients were followed up by 1 to 15 years. Ninety five (80.5%) patients were in treatment with immune-modulators. The prevalence was 1,36:100000 habitants. We have identified: predominance of mulattoes (110,93.2%), 82 (82.2%) cases clinically defined, 15 (12.7%) cases laboratorial defined and 6 (5.1%) cases clinically probable; a number of maximum bouts equal to 46; more frequent mild EDSS for patients with 1 to 10 years of MS of remittent-recurrent (RR) form. Gender rate of 83 cases of RR form was equivalent to the general one, however on RR with secondary progressive form form, the rate was 2.5 times greater for females than for males, and on primary progressive form, 5.5 times greater for males. CONCLUSION: Although some results of this research had been similar to that of eight studies used as standards for comparison, the differences will require new approaches.

Manifestações neurológicas de dengue: estudo de 41 casos

UNLABELLED The increase of encephalitis and meningoencephalitis in patients with dengue, diagnosed at a public hospital, Neurology State reference, we adopted systematic data collection. OBJECTIVE The objective was to present 41 cases of neurological manifestations of dengue and to compare data with literature. METHOD This is a descriptive study, retrospective from March to July 1997 and, prospective, from February to May 2002, analyzing damaged neurological regions and diagnostic of 41 patients. RESULTS Involved regions were brain (5/7 cases - 71.4%, in 1997, and 20/34 cases - 58.8%, in 2002), spinal cord (2/34 cases - 5.9% in 2002) and peripheral nerves (2/7 cases - 28.6% in 1997 and 12/34 cases - 35.3% in 2002). There was no meningeal involvement. According to topography, there was encephalic and peripheral nerves diagnosis in both periods and, exclusively in 2002, spinal cord damage. Cerebral hemorrhage and acute disseminated encephalomyelitis were diagnosed for the first time, as well as hemifacial spasm worsening as possible neurological manifestation of dengue. CONCLUSION This is the third casuistics in dengue and nervous system. Three dengue complications were diagnosed, for the first time registered on the literature.

Pediatric multiple sclerosis: analysis of clinical and epidemiological aspects according to National MS Society Consensus 2007

OBJECTIVE To describe the epidemiological and clinical characteristics of child/adolescence multiple sclerosis (MS). METHOD According to a descriptive, cohort study, with comparison of groups, data of 31 cases of child/adolescent MS, diagnosed at State Reference Center for Demyelinating Diseases-Hospital da Restauração, Recife, Pernambuco, Brazil, from 1987 to July 2007, were analyzed. The variables were: sex, initial symptoms, time for diagnosis, time of disease onset (early childhood, later childhood and adolescence), time of follow-up, number of relapses, relapses index and disability. Using SPSS software, version 13.0, t Student and Mann-Whitney tests were performed, with significance level of 0.05. RESULTS There were 3 (9.7%) cases of early childhood MS, 9 (29%), of late childhood MS, and 19 (61.3%), of adolescence MS. The general sex rate female: male was 1.8:1, varying according to age of onset. The predominant deficits were motor (12; 38.7%) and brainstem/cerebellum (7; 22.5%) especially on subsequent relapses of relapsing/remitting form. Time for diagnosis and average relapses index were higher in early childhood than in adolescence class (p=0.049 and p=0.028, respectively). Disability was higher for primary and secondary MS, as well as for early childhood. CONCLUSION Early childhood MS presents proper and different characteristics from adults, consisting in a difficult diagnosis that demands aid of expert neurologist on MS.

CONSENSO EXPANDIDO DO BCTRIMS PARA O TRATAMENTO DA ESCLEROSE MÚLTIPLA III. Diretrizes baseadas em evidências e recomendações

There has been unprecedented advances in knowledge of multiple sclerosis (MS) in the last few years. A new set of criteria for its diagnosis and a bunch of recent clinical trials with disease-modifying agents (DMA) have been published. All of that has made it necessary to update and expand the previous consensus for MS treatment as formulated by the Brazilian Committee for Treatment and Research in Multiple Sclerosis (BCTRIMS) two years ago. The BCTRIMS Expanded Consensus emphasizes the need to (1) consider MS treatment on an individual basis; (2) educate patients about the potential benefits and risks of treatment; (3) monitor drugs side effects; (4) have a signed Informed Consent Form; (5) consider the relative cost of the drug. The various clinical possibilities and the indications of the DMA and other immunointerventions are considered according to classes of evidences and types of recommendations. The BCTRIMS Expanded Consensus on Treatment of MS may turn out to be a model to other developing countries.

Neuromyelitis optica with onset in childhood and adolescence.

BACKGROUND Neuromyelitis optica with onset before the age of 18 years is a relatively rare, yet potentially devastating condition. The objective of the present study was to contribute to the study of early-onset neuromyelitis optica with a case series. PATIENTS Data were collected from medical records of Brazilian neurologists caring for patients with neuromyelitis optica occurring in childhood and adolescence. RESULTS Twenty-nine patients with neuromyelitis optica occurring before the age of 18 years and fulfilling the diagnostic criteria were identified. The average age at disease onset was 13 years and the patients had had an average disease duration of 6 years. The expanded disability scale score at the latest consultation was, on average, 4.7, and one patient had died from the disease. The 29 patients had had an average 4.5 relapses during the disease, accounting for 0.75 relapses per year, irrespective of the medication used. All patients were using one or more of the following medications: azathioprine, prednisone, immunoglobulin, and glatiramer acetate. CONCLUSIONS Neuromyelitis optica with onset in childhood and adolescence is a poorly understood condition that is often disabling and difficult to manage.

Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study

The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients’ demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect immunofluorescence (IIF) with a composite substrate of mouse tissues in 200 NMOSD cases was positive in people with NMO (95/162; 58.6%), longitudinally extensive transverse myelitis (10/30; 33.3%) and bilateral or recurrent optic neuritis (8/8; 100%). No association of NMO-IgG antibody positivity was found with gender, age at onset, ethnicity, early or late onset forms, disease course, or long-term severe disability. The relative frequency of NMO among relapsing-remitting MS (RRMS) + NMO cases in SA was 14.0%. Despite the high degree of miscegenation found in SA, MS affects three quarters of all patients with IIDD, mainly white young women who share similar clinical characteristics to those in Western populations in the northern hemisphere, with the exception of ethnicity; approximately one-third of all cases occur among non-white individuals. At the last assessment, the majority of RRMS patients showed mild disability, and the risk for secondary progression was significantly superior among those of African ethnicity. NMO comprises 11.8% of all IIDD cases in SA, affecting mostly young African-Brazilian women, evolving with a recurrent course and causing moderate or severe disability in both ethnic groups. The South-North gradient with increasing NMO and non-white individuals from Argentina, Paraguay, Brazil and Venezuela confirmed previous studies showing a higher frequency of NMO among non-white populations.

Multiple sclerosis starting before the age of 18 years: the Brazilian experience

Multiple sclerosis (MS) starting in childhood and adolescence poses a challenge for diagnosis and management of the disease. The aim of the present study was to assess the characteristics of early onset MS in Brazilian patients. Methods Retrospective data collection from specialized MS units. Results From 20 MS units in 11 Brazilian states, 117 cases of MS starting before the age of 18 years were collected. These patients had an average of 10 years of disease duration, still typically with low disability and one relapse every 2.5 years. The mean age for disease onset was 13.7 years. Conclusion The present study introduces a large series of Brazilian cases of pediatric MS. Although some patients presented a very severe form of MS, on the whole the group of patients with MS starting in childhood or adolescence presented a relatively mild form of this disease in Brazil.