La historia olvidada del alzhéimer: recordando a Oskar Fischer

Introducción: La Enfermedad de Alzheimer (EA), es una patología neurodegenerativa progresiva que afecta la memoria y otras funciones cognitivas. Hasta ahora no existen tratamientos curativos ni modificadores de la enfermedad, por lo que el manejo está centrado en la prevención y en el tratamiento de factores que puedan contribuir a su evolución; las herramientas farmacológicas son escasas y tienen efectos modestos en la ralentización de la enfermedad. Se propone realizar una breve biografía de Oskar Fischer, describir el conflicto con Alois Alzheimer que se identifica en documentos científicos y mencionar los principales elementos de la teoría de Oskar Fischer. Método: Se realizó una revisión narrativa en las bases de datos Scielo, PubMed y Lilacs, con los términos "Oskar Fischer" y se encontró quince artículos publicados entre 1906 a 2023, los cuales fueron resumidos por los autores GS y NR. El artículo fue posteriormente revisado por los demás autores. Resultados: Se organizaron en secciones, partiendo con una breve biografía del autor, su interacción con Alois Alzheimer y un resumen de su teoría; lo descrito por Oskar Fischer en términos de las estructuras de placas y ovillos se considera como una de las principales teorías fisiopatológicas de la EA. Conclusiones: Oskar Fisher hizo un aporte invaluable y planteó conceptos clásicos con respecto a la EA, que, si bien no le valieron para ser reconocido en la posteridad, han permitido que en las investigaciones posteriores sea de gran importancia repensar estos conceptos e incluir otras posibilidades e hipótesis, para continuar en la profundización del conocimiento de la enfermedad.

Introduction: Alzheimer's disease (AD) is a progressive neurodegenerative pathology that affects memory and other cognitive functions. Until now, there are no curative or disease-modifying treatments, so management is focused on prevention and treatment of factors that may contribute to its evolution; pharmacological tools are scarce and have modest effects in slowing the disease. It is proposed to make a brief biography of Oskar Fischer, describe the conflict with Alois Alzheimer that is identified in scientific documents and mention the main elements of Oskar Fischer's theory. Method: A narrative review was carried out in the Scielo, PubMed and Lilacs databases, with the terms "Oskar Fischer" and fifteen articles published between 1906 and 2023 were found, which were summarized by the authors GS and NR. The article was subsequently reviewed by the other authors. Results: They were organized in sections, starting with a brief biography of the author, his interaction with Alois Alzheimer and a summary of his theory; what was described by Oskar Fischer in terms of the structures of plaques and tangles is considered one of the main pathophysiological theories of AD. Conclusions: Oskar Fisher made an invaluable contribution and raised classic concepts regarding AD, which, although they did not earn him recognition in posterity, have allowed subsequent research to be of great importance to rethink these concepts and include other possibilities and hypotheses, to continue deepening the knowledge of the disease.

A case of recurrent Miller-Fisher syndrome with multiple anti-ganglioside antibodies / 中国综合临床

Guillain-Barré syndrome (GBS) defines a kind of Immune-mediated acute inflammatory peripheral neuropathy. Miller-Fisher Syndrome (MFS) is a special variant of GBS, with mostly one-way course and rare clinical recurrence. Only a few recurrent cases have been reported in China. Here we report a case of a young male patient with double vision and progressive aggravation of limb numbness, acute onset, with symptoms of upper respiratory tract infection before onset, accompanied by pupil abnormalities and autonomic nervous dysfunction, who was was admitted to our hospital for similar symptoms 3 years ago and was improved by immunotherapy. The patient had a triad of “ataxia, areflexia and ophthalmoplegia”. Cerebrospinal fluid showed protein-cell separation. Serum anti-Sulfatides antibody IgM, anti-GT1a antibody IgG, anti-GQ1b antibody IgG and anti-GM3 IgM were positive. Recurrent MFS was diagnosed and the symptoms improved after immunotherapy. This case suggests that MFS is clinically heterogeneous, a few patients can present with relapse and generally have a better prognosis with immunotherapy. Pre-existing infection and anti-GQ1b antibody production may be predisposing factors for MFS recurrence.

Postmortem Interval Estimation Using Protein Chip Technology Combined with Multivariate Analysis Methods / 法医学杂志

OBJECTIVES@#To estimate postmortem interval (PMI) by analyzing the protein changes in skeletal muscle tissues with the protein chip technology combined with multivariate analysis methods.@*METHODS@#Rats were sacrificed for cervical dislocation and placed at 16 ℃. Water-soluble proteins in skeletal muscles were extracted at 10 time points (0 d, 1 d, 2 d, 3 d, 4 d, 5 d, 6 d, 7 d, 8 d and 9 d) after death. Protein expression profile data with relative molecular mass of 14 000-230 000 were obtained. Principal component analysis (PCA) and orthogonal partial least squares (OPLS) were used for data analysis. Fisher discriminant model and back propagation (BP) neural network model were constructed to classify and preliminarily estimate the PMI. In addition, the protein expression profiles data of human skeletal muscles at different time points after death were collected, and the relationship between them and PMI was analyzed by heat map and cluster analysis.@*RESULTS@#The protein peak of rat skeletal muscle changed with PMI. The result of PCA combined with OPLS discriminant analysis showed statistical significance in groups with different time points (P<0.05) except 6 d, 7 d and 8 d after death. By Fisher discriminant analysis, the accuracy of internal cross-validation was 71.4% and the accuracy of external validation was 66.7%. The BP neural network model classification and preliminary estimation results showed the accuracy of internal cross-validation was 98.2%, and the accuracy of external validation was 95.8%. There was a significant difference in protein expression between 4 d and 25 h after death by the cluster analysis of the human skeletal muscle samples.@*CONCLUSIONS@#The protein chip technology can quickly, accurately and repeatedly obtain water-soluble protein expression profiles in rats' and human skeletal muscles with the relative molecular mass of 14 000-230 000 at different time points postmortem. The establishment of multiple PMI estimation models based on multivariate analysis can provide a new idea and method for PMI estimation.

Evaluation of pediatric patients presenting with acute-onset unilateral transient acquired blepharoptosis / Avaliação de pacientes pediátricos apresentando blefaroptose transitória unilateral de início agudo

ABSTRACT Purpose: To evaluate the clinical features of pediatric patients with acute-onset, unilateral transient acquired blepharoptosis. Methods: In this retrospective study, the clinical records of patients between April 2015 and June 2020 were reviewed for evaluation of demographic features, accompanying neurological and ophthalmologic manifestations, symptom duration, etiological cause, and imaging findings. Patients with congenital and acquired blepharoptosis with chronic etiologies were excluded. Results: Sixteen pediatric patients (10 boys and 6 girls) with acquired acute-onset unilateral transient blepharoptosis were included in this study. The patients' mean age was 6.93 ± 3.16 years. The most commonly identified etiological cause was trauma in 7 patients (43.75%) and infection (para-infection) in 5 patients (31.25%). In addition, Miller Fisher syndrome, Horner syndrome secondary to neuroblastoma, acquired Brown's syndrome, and pseudotumor cerebri were identified as etiological causes in one patient each. Additional ocular findings accompanied blepharoptosis in 7 patients (58.33%). Blepharoptosis spontaneously resolved, without treatment, in all the patients, except those with Miller Fisher syndrome, neuroblastoma, and pseudotumor cerebri. None of the patients required surgical treatment and had ocular morbidities such as amblyopia. Conclusion: This study demonstrated that acute-onset unilateral transient blepharoptosis, which is rare in childhood, may regress without the need for surgical treatment in the pediatric population. However, serious pathologies that require treatment may present with blepharoptosis.

RESUMO Objetivo: Avaliar as características clínicas de pacientes pediátricos com blefaroptose adquirida unilateral, transitória e de início agudo. Métodos: Neste estudo retrospectivo, foram revisados prontuários clínicos entre abril de 2015 e junho de 2020. Os pacientes foram avaliados em termos de características demográficas, manifestações neurológicas e oftalmológicas associadas, duração dos sintomas, etiologia e achados de imagem. Foram excluídos pacientes com blefaroptose congênita e com blefaroptose adquirida de etiologia crônica. Resultados: Foram incluídos neste estudo 16 pacientes pediátricos (10 masculinos e 6 femininos) com blefaroptose adquirida transitória unilateral de início agudo. A média de idade dos pacientes foi de 6,93 ± 3,16 anos. As causas etiológicas mais comumente identificadas foram trauma em 7 pacientes (43,75%) e infecção (casos parainfecciosos) em 5 pacientes (31,25%). Além disso, a síndrome de Miller-Fisher, a síndrome de Horner secundária a neuroblastoma, a síndrome de Brown adquirida e pseudotumor cerebral foram determinados como causas etiológicas em um paciente cada uma. Achados oculares adicionais estavam associados à blefaroptose em 7 pacientes (58,33%). Foi observada a resolução espontânea da blefaroptose, sem tratamento, em todos os pacientes, exceto nos pacientes com síndrome de Miller-Fisher, neuroblastoma e pseudotumor cerebral. Nenhum paciente precisou de tratamento cirúrgico. Morbidades oculares, como ambliopia, não foram encontradas em nenhum paciente. Conclusão: Este estudo demonstrou que a blefaroptose transitória unilateral de início agudo, rara na infância, pode regredir sem a necessidade de tratamento cirúrgico na população pediátrica. No entanto, também não deve ser esquecido que patologias graves que requerem tratamento podem se apresentar com blefaroptose.

EMPOWERMENT OF FISHER-FOLK IN KANYAKUMARI DISTRICT

The main aim of the study is to study the socio-cultural empowerment of fisher-folk and the economic empowerment of fisher-folk in Kanyakumari district. Primary data has been used in this paper. Primary data were collected on the basis of questionnaire prepared on the light of the objectives of the study. The sizes of the samples are 150 fisher-folk. These sample respondents were selected by taking 30 from five villages of the study area. These samples were selected purposively. The questionnaire was designed with two main characteristics viz., open ended and close ended according to the nature of information. Collected data were analyzed through the use of various statistical tools like ANOVA. The data were analyzed in order to make the study more informative and useful to the readers. It is found that the important socio-cultural empowerment among the fisher-folk are in the age group of 20 to 30 years are attending cultural programmes and exercising the voting rights. Among the fisher-folk are in the age group of above 50 years, the important socio-cultural empowerment are extending the support for social causes and participation in the awareness programmes to eradicate anti social activities. Age wise there is a significant difference in the socio-cultural empowerment are identified in the case of participation in social programmes, attending social functions, exercising the voting rights and attending cultural programmes

Síndrome de Miller-Fisher durante el embarazo

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Miller-Fisher syndrome is a rare, acute, autoimmune, demyelinating disorder which is considered a variant of Guillain-Barré syndrome. The pathologic mechanism is unclear, but acute demyelinating polyneuropathies may be triggered by bacterial or viral infections, major surgical interventions, or vaccination. Pregnancy may be a trigger of the immune response causing the onset of the syndrome. Miller-Fisher syndrome is characterized by acute onset, with predominant involvement of the facial and cranial nerves resulting in ophthalmoparesis, ataxia, and areflexia/hyporeflexia. Diagnosis is based on clinical suspicion together with the determination of specific ganglioside antibodies and other laboratory and imaging tests. Treatment consists of intravenous immunoglobulin and plasmapheresis, together with supportive measures. There are few reports of the syndrome occurring in pregnant women. A case of Miller-Fisher syndrome during pregnancy is presented.

INTRODUCTION Miller-Fisher syndrome (MFS) is a rare disorder that is characterized by acute onset of ophthalmoparesis, ataxia and hyporeflexia / areflexia(1). It was recognized 60 years ago as a variant of Guillain-Barré syndrome (GBS). The annual incidence is 0.09 per 100,000 persons and affects more males than females with a 2:1 ratio(2). GBS usually follows Campylobacter jenuni, cytomegalovirus, Epstein-Barr and influenza virus infections or secondary to major surgery, pregnancy, or vaccination(3,4). MFS accounts for 5%-10% of GBS cases and may have a major autoimmune component due to the presence of anti-ganglioside antibodies. During the acute phase of the disease, these antibodies have a diagnostic sensitivity and specificity of 92% and 97%, respectively(5). MFS during pregnancy is rare and there are only reports of 5 cases in pregnant women. A case of Miller-Fisher syndrome during pregnancy is presented. CLINICAL CASE The patient was 16 years old, primigravida of 20 weeks, who was referred for presenting nausea and incoercible vomiting of five days of evolution, accompanied by double vision, generalized weakness, ataxia,

Disfagia e Disfonia em um caso de Síndrome de Miller Fisher: Avaliação fonoaudiológica / Dysphagia and Dysphonia in a case of Miller Fisher Syndrome: Speech therapy evaluation

RESUMO Relato de caso de um paciente do sexo masculino internado no pronto socorro de um hospital com síndrome de Miller Fisher (SMF). A SMF é caracterizada pela presença de uma tríade de sinais: oftalmoparesia, arreflexia e ataxia, podendo apresentar outros sinais menos frequentes como alterações de fala e de deglutição. A partir da avaliação fonoaudiológica foi possível identificar disfagia orofaríngea de grau grave, hipernasalidade vocal e outras alterações fonoarticulatorias. O paciente apresentou boa evolução a partir de uma terapêutica multiprofissional integrada, incluído atendimento fonoaudiológico.

SUMMARY The case report of a male patient admitted to the emergency hospital with the diagnosis of Miller Fisher syndrome (MF), is presented. MFS is characterized by the presence of a triad of signs: ophthalmoparesis, areflexia and ataxia, and may present other less frequent signs such as speech and swallowing disorders. On the basis of findings during the speech therapy evaluation it was possible to identify severe oropharyngeal dysphagia, vocal hypernasality and other speech disorders. The patient presented a good evolution following an integrated multi-professional treatment program, including speech-language therapy.

Clinical application of the new classification criteria in children with Miller Fisher syndrome and clinical characteristics analysis of different types of Miller Fisher syndrome / 中华实用儿科临床杂志

Objective:To investigate the clinical application of the new classification criteria in children with Miller Fisher syndrome (MFS), and to analyze clinical characteristics of different types of MFS.Methods:Clinical data of MFS patients hospitalized in the Department of Neurology, Beijing Children′s Hospital, Capital Medical University from January 2015 to December 2019 were collected and analyzed retrospectively, including demographic characteristics, clinical symptoms, neurological examination findings, laboratory examination data, diagnosis and treatment, and prognosis.The counting data was described by percentage, and the measurement data was described by median.Results:A total of 23 patients were included in the research, including 14 males and 9 females, with a median age of 4 years and 8 months.There were 3 cases of pure MFS; 5 cases of incomplete MFS, including 1 case of acute ptosis and 4 cases of acute ataxia neuropathy; 15 cases of overlapping syndrome, including 13 cases of MFS/Guillain Barre syndrome (GBS), 1 case of MFS/pharyngocervical brachial variant GBS(PCB GBS)and 1 case of MFS/GBS/Bickertaff brainstem encephalitis (BBE). In addition to Ⅲ, Ⅳ and Ⅵ cranial nerve palsy, 11 cases had the involvement of other cra-nial nerves, including 2 cases in pure MFS, 8 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Autonomic nervous dysfunction occurred in 6 cases.Respiratory muscle paralysis occurred in 6 cases, including 5 cases in MFS/GBS and 1 case in MFS/GBS/BBE.Graded by the Hughes scoring system (HG score), 3 cases with pure MFS were graded 4 points; 1 case with acute ptosis was graded 0; 3 cases with acute ataxia neuropathy were graded 2 points, and the other one was graded 3 points; 1 case with MFS/PCB GBS was graded 3 points; 10 cases with MFS/GBS were graded 4 points, 1 case was graded 3 points, and the other 2 cases were graded 2 points; 1 case with MFS/GBS/BBE was graded 4 points.Twenty-two patients were treated with intravenous immunoglobulin.The HG of all patients at discharge decreased at varying degree, which was graded 0 at 6 months of follow-up.Conclusions:The clinical application of the new diagnostic classification method is helpful to the accurate diagnosis of different types of MFS.More than half of MFS cases will develop into the overlapping syndrome.The overlapping of MFS and GBS or BBE is prone to the involvement of cranial nerves except for the external ophthalmic muscles, autonomic nerve dysfunction and respiratory muscle paralysis.The disease course of MFS varies, and its diagnosis should be comprehensively made.All cases of MFS in this study have a satisfactory prognosis.

Heart sound classification based on improved mel frequency cepstrum coefficient and integrated decision network method / 生物医学工程学杂志

Heart sound analysis is significant for early diagnosis of congenital heart disease. A novel method of heart sound classification was proposed in this paper, in which the traditional mel frequency cepstral coefficient (MFCC) method was improved by using the Fisher discriminant half raised-sine function (F-HRSF) and an integrated decision network was used as classifier. It does not rely on segmentation of the cardiac cycle. Firstly, the heart sound signals were framed and windowed. Then, the features of heart sounds were extracted by using improved MFCC, in which the F-HRSF was used to weight sub-band components of MFCC according to the Fisher discriminant ratio of each sub-band component and the raised half sine function. Three classification networks, convolutional neural network (CNN), long and short-term memory network (LSTM), and gated recurrent unit (GRU) were combined as integrated decision network. Finally, the two-category classification results were obtained through the majority voting algorithm. An accuracy of 92.15%, sensitivity of 91.43%, specificity of 92.83%, corrected accuracy of 92.01%, and F score of 92.13% were achieved using the novel signal processing techniques. It shows that the algorithm has great potential in early diagnosis of congenital heart disease.

Síndrome de Miller Fisher, un desafío diagnóstico de oftalmoplejía: reporte de un caso / Miller Fisher syndrome, a diagnostic challenge of ophthalmoplegia: a case report

El síndrome de Guillain-Barré (SGB), y sus derivados, entre ellos el síndrome de Miller Fisher (SMF); junto a otras patologías de origen neurológico como la Polineuropatía desmielinizante inflamatoria crónica (CIDP), las polineuropatías de causa metabólica, miastenia gravis, esclerosis lateral amiotrófica (ELA), síndrome de Lambert-Eaton, encefalopatía de Wernicke entre otras; presentan signos y síntomas neurológicos de presentación común. De este modo, la importancia del examen neurológico acabado; y los exámenes de apoyo diagnóstico como: laboratorio -destacando el líquido cefalorraquídeo (LCR)-, electromiografía, y toma de imágenes, son cruciales para esclarecer el diagnóstico. Así, es posible ofrecer un tratamiento de forma precoz, basado en la evidencia, y con el objetivo de disminuir la letalidad de la enfermedad. En el presente texto se plasma un subgrupo de patología de SGB, el SMF, el cual posee una incidencia significativamente baja, una clínica característica, y un pronóstico bastante ominoso sin un tratamiento adecuado. En el presente texto se plasma el reporte de un caso abordado en el Hospital San Pablo de Coquimbo, Chile.

Guillain-Barré syndrome (GBS) and its derivatives, including Miller Fisher syndrome (MFS), along others pathologies of neurological origin such as chronic inflammatory demyelinating polyneuropathy (CIDP), metabolic polyneuropathies, myasthenia gravis, amyotrophic lateral sclerosis (ALS), Lambert-Eaton syndrome, Wernicke's encephalopathy and well as others, have common neurological signs and symptoms. In this way, the importance of a thorough neurological examination, and supporting diagnostic tests such as: laboratory, -cerebrospinal fluid (CSF)-electromyography, and imaging, are crucial to clarify the diagnosis. Thus, it is possible to offer early, evidence-based treatment with an aim of reducing the disease's lethality. In the text below we present a subgroup of GBS pathology, MFS, which has a significantly low incidence, a characteristic clinical picture, and a rather ominous prognosis without adequate treatment. In the following text/paper is shown the report of a case approached in San Pablo Hospital, from Coquimbo, Chile.

Casos clínicos de los síndromes de Guillain-Barré y Miller-Fisher relacionados con la COVID-19 / Clinical cases of Guillain-Barré and Miller-Fisher syndromes linked to COVID-19

Introducción: la enfermedad por coronavirus del 2019 (COVID-19), causada por el nuevo coronavirus SARSCoV-2, se ha asociado con el desarrollo de enfermedades neurológicas como el síndrome de Guillain-Barré (SGB) y sus variantes. En el presente trabajo se reportan dos casos de síndromes desmielizantes asociados con la COVID-19. Casos clínicos: hombre de 53 años con SGB y mujer de 29 años con la variante del síndrome de Miller-Fisher (SMF), respectivamente. Ambos presentaron los signos y síntomas neurológicos clásicos de polineuropatía desmielinizante que caracterizan a estos síndromes. De las pruebas bioquímicas paraclínicas, el aumento de proteínas en líquido cefalorraquídeo fue distintiva. La positividad de la RT-qPCR para el SARS-CoV-2 indicó la asociación de los SGB y SMF con la COVID-19. Ambos pacientes se trataron con inmunoglobulina intravenosa y mostraron mejoría. La electromiografía realizada en semanas posteriores aún mostrabaafectación desmielinizante crónica. Conclusión: los casos de los SGB y SMF, junto con otros casos similares reportados en todo el mundo, proporcionan más evidencia para el SARS-CoV-2 como nueva posible etiología de estas raras enfermedades neurológicas.

Background: coronavirus disease 2019 (COVID-19), caused by the new coronavirus SARS CoV-2, has been associated with the development of neurological diseases such as Guillain-Barré syndrome (GBS) and its variants. In the present work, two cases of demyelinating syndromes associated with COVID-19 are reported. Clinical cases: 53-year-old male with GBS and and 29-yearold female with Miller-Fisher syndrome (MFS) variant, respectively. Both patients presented the classic neurological signs and symptoms of demyelinating polyneuropathy that characterizes the syndromes. From the paraclinical biochemical tests, the increase of proteins in cerebrospinal fluid was distinctive. The positivity of the RT-qPCR for SARSCoV-2 suggested the association of GBS and MFS with COVID-19. Both patients were treated with intravenous immunoglobulin showing improvement. Electromyography performed weeks ahead still showed chronic demyelinating involvement. Conclusion: The cases of GBS and MFS, along with other similar cases reported around the world, provide further evidence for SARS-CoV-2 as a new possible etiology of these rare neurological diseases.

Síndrome de Miller Fisher tratado con plasmaféresis durante el embarazo: reporte de un caso y revisión de la literatura / Miller Fisher syndrome treated with plasmapheresis during pregnancy: Case report and review of the literature

Resumen Objetivo: Reportar el caso de una paciente gestante con síndrome de Guillain-Barré (SGB) presentado en la variante denominada síndrome de Miller Fisher (SMF), y realizar una revisión en torno al diagnóstico, tratamiento y pronóstico de esta variedad de SGB durante la gestación. Materiales y métodos: Se presenta el caso de una gestante de 27 semanas con síndrome de Miller Fisher, quien fue tratada con plasmaféresis en un hospital militar de referencia, con evolución satisfactoria a los 15 días y continuación normal del embarazo, parto a las 38 semanas con recién nacido sano. Se realizó una búsqueda bibliográfica en bases de datos electrónicas: Medline vía PubMed, Lilacs, SciELO, ScienceDirect, Ovid, con los términos "Embarazo", "Síndrome de Miller Fisher", "Síndrome de Guillain-Barré". Se incluyeron cohortes, series y reportes de casos de mujeres gestantes con síndrome de Miller Fisher; se extrajo información sobre los métodos diagnósticos, el tratamiento utilizado y el pronóstico materno y perinatal. La búsqueda se hizo en junio de 2020, sin restricción por fecha, pero sí por tipo de idioma (español e inglés). Resultados: Se identificaron 423 títulos, tres estudios cumplieron los criterios de inclusión, los tres correspondieron a reportes de caso. Todos los casos mostraron seropositividad para antigangliósidos GQ1b positivos; en ningún caso hubo alteración imagenológica. Dos pacientes recibieron inmunoglobulina intravenosa y la tercera paciente se dejó en observación. Hasta el momento no se documentan complicaciones obstétricas. Conclusión: Existen pocos casos reportados de SMF durante la gestación, el diagnóstico se basa en el examen clínico; el tratamiento con inmunoglobulina IV representa la alternativa utilizada con mayor frecuencia. En el caso presentado se utilizó la plasmaféresis. Se desconoce el impacto de la variedad del síndrome de Miller Fisher sobre el curso normal de la gestación y sobre los resultados perinatales a largo plazo. Se requieren más estudios que aborden el diagnóstico, el tratamiento y el pronóstico de esta entidad.

Abstract Objective: To report the case of pregnant woman with Guillain-Barré syndrome (GBS) presenting as the Miller Fisher variant, and to review the literature on the diagnosis, treatment and prognosis of this GBS variant during gestation. Materials and Methods: Pregnant woman presenting at 27 weeks of gestation with Miller Fisher syndrome (MFS), treated in a military referral hospital with a satisfactory course after 15 days, continuation of normal pregnancy and delivery of a healthy neonate at 38 weeks. A search of the literature was conducted in the Medline via PubMed, Lilacs, SciELO, ScienceDirect and Ovid databases using the terms "Pregnancy," "Miller Fisher syndrome," "Guillain-Barré syndrome". Cohorts, case series and case reports of pregnant women with MFS were included. Data on diagnostic methods, treatment and maternal and perinatal prognosis were extracted. The search was made on June 2020, with no restriction by date, but restriction by language (Spanish and English). Results: Overall, 423 titles were identified, three studies met the inclusion criteria, the three of them corresponding to case reports. All cases were found to be seropositive for anti-GQ1b ganglioside antibodies. No imaging abnormalities were found in any of the cases. Two patients received IV immunoglobulin and the third patient was kept under observation. No obstetric complications have be documented so far. Conclusion: There are few cases of MFS reported during pregnancy. Intravenous immunoglobulin is the most frequently used treatment option. Plasmapheresis was used in the case presented here. The impact of the Miller Fisher variant on the normal course of gestation and on long-term perinatal outcomes is unknown. Further studies that look into the diagnosis, treatment and prognosis of this condition are required.

Síndrome de Evans Fisher / Evans fisher syndrome

Paciente femenina de 35 años de edad con color de la piel blanca, con antecedentes de cefalea migrañosa. Comenzó con manifestaciones purpúreas en forma de petequias y equimosis diseminadas por todo el cuerpo; palidez cutánea mucosa e íctero en piel y esclera, además de coluria. Se realizaron estudios hematológicos, humorales e inmunológicos. Es atendida en la sala de Medicina Interna del Hospital General Docente Comandante Pinares. Con evolución satisfactoria al tratamiento con esteroides y la inmunoglobulina intravenosa. Se realizó la discusión diagnóstica del caso confirmándose el Síndrome de Evans-Fisher, se revisa la bibliografía actualizada sobre la enfermedad(AU)

We report a case of a 35-year-old female patient, white skin color, with a history of migraine headache. She began with purple manifestations in the form of petechiae and ecchymoses spread throughout the body; mucous skin pallor and icterus in skin and sclera, as well as coluria. Hematological, humoral and immunological studies were carried out. She was treated in the Internal Medicine ward at Comandante Pinares General Teaching Hospital. She evolved satisfactorily to treatment with steroids and intravenous immunoglobulin. The diagnostic discussion of this case was carried out, confirming the Evans-Fisher Syndrome, the updated bibliography on the disease was reviewed(AU)

Síndrome anti-GQ1b en un niño con síndrome de Miller-Fisher-Bickerstaff / Anti-GQ1b syndrome. A child with Miller-Fisher-Bickerstaff syndrome

El síndrome anti-GQ1b reúne el síndrome de Miller-Fisher y la encefalitis del tronco cerebral de Bickerstaff, entre otras entidades. Tienen etiopatogenia común, constituida por la presencia de anticuerpos anti-GQ1b que reaccionan contra los sitios GQ1b del sistema nervioso según sea su accesibilidad. La prevalencia anual del síndrome de Miller-Fisher es de 0,09 casos por 100 000 habitantes por año y no existen estudios epidemiológicos sobre la encefalitis del tronco cerebral de Bickerstaff, que sería menos frecuente. De evolución natural hacia la mejoría, se beneficia del tratamiento con gammaglobulina endovenosa.Se presenta a un paciente de 12 años con síndrome de Miller-Fisher­Bickerstaff tras un episodio de diarrea aguda por Campylobacter jejuni en el que los anticuerpos anti-GQ1b resultaron positivos. Es nuestro objetivo comunicar sobre un síndrome de presentación poco habitual en pediatría a fin de advertir acerca de la necesidad de su sospecha precoz y solicitud de estudios de laboratorio específico

Miller-Fisher syndrome and Bickerstaff brainstem encephalitis, among others, constitute the anti-GQ1b syndrome, with a common immune pathophysiologic pathway characterized by the presence of anti-GQ1b antibodies, which react against the different nervous system GQ1b sites according to their different accessibility. The Miller-Fisher syndrome has a prevalence of 0.09 cases per 100 000 people-year but there are not epidemiological studies about Bickerstaff brainstem encephalitis, that it seems to be less frequent. In spite of having a good natural outcome, the immunoglobulin administration has been established as efficacious at improving it. A twelve-year-old boy suffering from Miller-Fisher-Bickerstaff syndrome after an acute Campylobacter jejuni diarrhea with positive titers of anti-GQ1b and anti-QGT1a antibodies is presented. We communicate a very uncommon pediatric disease with the aim of warning about the importance of its early suspicion and the need of specific laboratory determination

Síndrome de Miller Fisher: relato de caso / Miller Fisher syndrome: case report

A síndrome de Miller Fisher é uma desmielinização dos nervos cranianos e periféricos, gerando graves consequências para o paciente, como, por exemplo, redução ou ausência dos reflexos, paralisia do III, IV e VI nervos cranianos e ataxia. Este relato descreveu o caso de uma mulher de 51 anos, natural e procedente de Penápolis (SP), admitida em um hospital de Araçatuba (SP) com quadro de arreflexia, ataxia e oftalmoplegia. No contexto clínico, foi suspeitada a hipótese de síndrome de Miller Fisher e, assim, começou o processo de investigação, com base nos critérios diagnósticos. O caso foi diagnosticado como síndrome de Miller Fisher, e o tratamento teve início.

Miller Fisher Syndrome is a demyelinating disease affecting cranial and peripheral nerves, leading to severe problems to the patient, such as reduced or absent reflexes, III, IV and VI cranial nerves palsy, and ataxia. This report describes the case of a 51-year-old woman from the city of Penápolis, in the state of São Paulo, who was admitted to the hospital in the city of Araçatuba, in the same state, with ataxia, areflexia and ophthalmoplegia. In the clinical context, the suspicion of Miller Fisher Syndrome was raised, and then investigation ensued for the disease, based on the diagnostic criteria. After evaluation, Miller Fisher Syndrome was confirmed and treatment was started.

The correlation between the preoperative measurement of estimated cerebral perfusion pressure and estimated intracranial pressure as obtained by trans cranial doppler with modified fisher grade as seen in computed tomography scan head in patients with acute sub arachnoid hemorrahage undergoing surgical clipping

Background: In Aneurysmal Sub Arachnoid haemorrhage, precise Cerebral Perfusion Pressure (CPP) and Intracranial Pressure (ICP) measurement can only be achieved by an invasive monitoring device. The study aimed at non-invasively estimating the preoperative values of CPP and ICP by use of validated formulae. These estimated flow velocities (estimated CPP or eCPP and estimated ICP or eICP) of the Middle Cerebral Artery were obtained by Trans Cranial Doppler ultrasound and comparing it with the preoperative CT Head Fisher Scale. In the Institute Rimed Digi-Lite Trans Cranial Doppler machine was used for research and Siemens (Somatom) 64 CT Scanner from GE (Signa) was used to perform CT scan of patients.Methods: It is a prospective, observational study which was studied between July 2017 and December 2018 in Post Graduate Institute of Medical Education and Research, Chandigarh, India. This study is a secondary analysis of a prospective observational study which was primarily designed to evaluate the neurological outcome related to the effect of estimated Intracranial Pressure and estimated Cerebral Perfusion Pressure as measured by Trans Cranial Doppler in patients with a SAH. A total of 100 patients were recruited in this study.Results: There was significant correlation between estimated CPP and Fisher Grading. There was no strong correlation between the modified Fisher Grade and estimated ICP.Conclusions: This study was able to give a statistically significant correlation between eCPP and Fisher Grading (p value- 0.047), as the Modified Fisher grading increased, so did the eCPP, this observation was unique, and it went against the hypothesis. However, no statistically significant co-relation was seen during comparison of eICP and Fisher Grading (p value- 0.069).

Value of discriminant analysis for screening of clonorchiasis sinensis / 中国血吸虫病防治杂志

Objective To build a discriminant function of clonorchiasis sinensis using stepwise discriminant analysis, so as to investigate the feasibility of discriminant analysis for clonorchiasis sinensis screening. Methods Ten villages in Mulan County of Harbin City were sampled as the study sites using a cluster random sampling method. The fecal samples were collected from the permanent residents in the study sites and detected for Clonorchis sinensis eggs using the modified Kato-Katz smear technique. A questionnaire of clonorchiasis sinensis was designed. All data were entered into Epidata 3.1, and a discriminant analysis was performed using the software SPSS version 15.0. Variable were screened using the stepwise discriminant analysis, and the discriminant function was built using the Fisher’s discriminant analysis method. The effectiveness of the discriminant function for clonorchiasis sinensis screening was evaluated by comparison with the modified Kato-Katz smear method. Results Eight variables with statistical significance were included to build the discriminant function, including chronic cholecystitis, cholangitis, gender, eating raw fish, abdominal distension, ethnicity, abdominal pain and age, and the correction rate of the discriminant function was 88.75% to identify clonorchiasis sinensis. Conclusions The discriminant function of clonorchiasis sinensis can be rapidly and simply built based on the strong data processing and analysis capability of the SPSS software, which is rapid to screen clonorchiasis sinensis in Harbin City. Such a function has a high discriminant analysis capability, and provides insights into the establishment of rapid screening of clonorchiasis sinensis in other endemic areas.

Open quadricepsplasty in hyperextension deformity of knee in children with arthrogryposis

Arthrogryposis includes heterogeneous disorders, characterized by congenital contractures of multiple joints. Knee involvement is very common (38–90 % of patients with amyoplasia) ranging from soft-tissue contractures (in flexion or hyperextension) to subluxation and dislocation. Children who present late will require surgery involving quadricepsplasty or lengthening of the contracted quadriceps muscle. Curtis and Fisher describe an open V-Y lengthening of the quadriceps femoris with post-operative immobilization in 30-45 ° of flexion. The main issue of this technique was limited degree of flexion that can be achieved, and the fibrous replacement of the quadriceps muscle. This is a neglected case of hyperextension deformity and congenital dislocation of knee in children with arthrogryposis resistant to conservative management and successfully treated by open quadricepsplasty.

Clinical features of 19 children with Bickerstaff brainstem encephalitis / 中华儿科杂志

Objective@#To summarize the clinical features of Bickerstaff brainstem encephalitis (BBE) in children.@*Methods@#In this retrospective study, data of 19 patients with BBE (11 males and 8 females) were collected from Department of Neurology, Beijing Children′s Hospital from October 2015 to January 2018. The clinical features, treatment and prognosis were analyzed.@*Results@#The onset age of BBE ranged from 1 year and 8 months to 12 years and 11 months. There were 18 cases with preceding infection. The most common infection was upper respiratory tract infection (9 cases), followed by simple fever (5 cases). The most common initial neurological symptoms were lethargy or disturbance of consciousness (8 cases), followed by limb weakness (5 cases). There were 6 cases of simple BBE and 13 cases of BBE overlapping Guillain-Barré syndrome (GBS). Besides the characteristic triad of altered mental status, ataxia, and ophthalmoplegia, there were other symptoms including convulsion (5 cases), diplopia (3 cases), nystagmus (7 cases), facial muscular weakness (7 cases),bulbar palsy (13 cases) and autonomic nerve symptoms (9 cases). Hypo or areflexia was seen in 16 cases. Positive Babinski′s signs were seen in 8 cases. Hyponatremia was present in 10 cases in whom 4 showed severe hyponatremia. Albumin-cytological dissociation of cerebrospinal fluid was seen in 10 cases. The autoimmune antibodies were examined in all 19 patients. Anti-ganglioside antibodies including anti-GM1 IgG antibody was positive in 2 patients and one of whom was also found with positive anti-GD1b IgG antibody. Anti-GQ1b IgG antibody was present in 2 patients. Electromyography was performed in 14 cases and 8 cases, who were all BBE overlapping GBS, showed neurological damage. A total of 16 cases were monitored by video electroencephalography and 8 cases showed slow waves of background. In addition to, interictal focal discharge was detected in 2 cases. T2 fluid-attenuated inversion recovery (FLAIR) sequence abnormal signals were detected in 3 of 18 cases performed brain magnetic resonance imaging (MRI), and lesions involved with brainstem, basal ganglia, thalamus, cerebellum, corpus callosum and cerebral cortex. Lesions involved cervical and thoracic spinal cord were found in 1 out of 11 cases for whom spinal cord MRI was performed. All of the 4 cases who underwent enhanced MRI of spinal had partial nerve roots enhancement. All of the 19 patients received 1 to 2 courses of intravenous immunoglobulin therapy, and 2 cases also received plasma exchange. Fifteen cases received steroid therapy. The following-up period ranged from 3 months to 2.5 years. Two cases were lost to follow-up. Twelve cases achieved a full recovery within 3 months. Three cases recovered within 6 months. One case still had slight limb weakness and ataxia after 1 year and 8 months of follow-up, and another case had left autonomic nerve symptoms in the follow-up of 2 years and 3 months. Both of them were BBE overlapping GBS.@*Conclusions@#Children′s BBE is similar to that in adults, and is frequently found overlapped with GBS. Furthermore, it is sometimes accompanied by central nervous system demyelination disease. The antiganglioside antibodies are not often detectable. Immunoglobulin therapy could usually achieve good response. The prognosis of simple BBE is good in most situations. For BBE overlapping GBS, the more severe the limb weakness during the peak of disease is, the slower the recovery would be.

Prediction of pathological grading of Henoch-Schönlein purpura nephritis in children based on Fisher stepwise discriminant analysis / 中华实用儿科临床杂志

Objective@#To establish the pathological grades of Henoch-Schönlein purpura nephritis(HSPN) in children with diagnostic prediction models by stepwise Fisher discriminant in children.@*Methods@#Based on the investigation of 28 clinical indicators from 144 cases with HSPN came from Children′s Hospital of Chongqing Medical University, the sensitive indicators were found and stepwise Fisher discriminant model was established and its accuracy in predicting the pathological classification of HSPN was tested.@*Results@#There were 5 laboratory indicators and clinical manifestations with different pathological grades of HSPN.In children with pathological grade Ⅱ, Ⅲ and Ⅳ, 5 indicators were screened (P<0.05) and stepwise Fisher discriminant models were established.And the correct rate of comprehensive diagnosis was (61.371±8.740)% in 100 random sampling diagnostic simulations; in children with pathological grade Ⅲa and Ⅲb, 5 indicators were also screened (P<0.05) and stepwise Fisher discriminant models were established.And the correct rate of comprehensive diagnosis was (68.015±5.736)% in 100 random sampling diagnostic simulations.@*Conclusions@#The stepwise Fisher discriminant models established in this research have a better diagnostic accuracy in forecasting for pathological grade of HSPN, and have a certain guiding value on early treatment and prognosis evaluation of children with newly diagnosed HSPN.