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We present a 20-year-old patient with subglottic and tracheal stenosis was taken for a tracheal resection and end-to-end anastomosis. The patient's neck was positioned in hyperflexion using chin stitches to minimize tension at the anastomosis. On post-operative period, the patient developed paresthesias in upper and lower extremities associated with motor weakness. Magnetic resonance imaging was performed showing lesions compromising ventral spinal cord at the level of C4-C5 and C6-C7. Chin stitches were removed and neck flexion was reduced. The patient remained in the intensive care unit with vasopressors, physical therapy and intravenous fluid-therapy to maintain mean arterial pressure above 90 mmHg. After 3 weeks, the patient was discharged with no neurologic deficit. There are few cases reported of acute ischemic spinal injury following tracheal reconstruction. If this complication arises, neck posture should be corrected, maintenance of MAP above 90 mmHg and implementation of early physical therapy is key to improve neurologic outcomes.

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Background Spinal epidural abscess is a rare but serious condition that can cause spinal cord compression and neurological deficits. Case Description and Methods The article reports a case of a 31-year-old patient who presented with an infectious cellulitis in the left hand, which progressed to a spinal epidural abscess. The diagnosis was confirmed by clinical examination and magnetic resonance imaging. Treatment involved laminectomy, after which the patient had complete recovery of neurological deficits. This article is a case report with a literature review. Patient data and images were collected by the researchers who participated in the patient's care. The literature was reviewed by one of the researchers based on the search for articles in the PubMed database. For the research, the following keywords were inserted: "Spinal epidural empyema," "Spinal epidural abscess." Conclusion Spinal epidural abscess is often underdiagnosed, which can lead to delays in treatment and serious complications. The relationship between cellulitis and spinal epidural abscess may be related to the spread of infection through the lymphatic or blood system.

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Adaptor protein complex 4 (AP-4) is a heterotetrameric complex that promotes export of selected cargo proteins from the trans-Golgi network. Mutations in each of the AP-4 subunits cause a complicated form of Hereditary Spastic Paraplegia (HSP). Herein, we report that ApoER2, a receptor in the Reelin signaling pathway, is a cargo of the AP-4 complex. We identify the motif ISSF/Y within the ApoER2 cytosolic domain as necessary for interaction with the canonical signal-binding pocket of the µ4 (AP4M1) subunit of AP-4. AP4E1- knock-out (KO) HeLa cells and hippocampal neurons from Ap4e1-KO mice display increased co-localization of ApoER2 with Golgi markers. Furthermore, hippocampal neurons from Ap4e1-KO mice and AP4M1-KO human iPSC-derived cortical i3Neurons exhibit reduced ApoER2 protein expression. Analyses of biosynthetic transport of ApoER2 reveal differential post-Golgi trafficking of the receptor, with lower axonal distribution in KO compared to wild-type neurons, indicating a role of AP-4 and the ISSF/Y motif in the axonal localization of ApoER2. Finally, analyses of Reelin signaling in mouse hippocampal and human cortical KO neurons show that AP4 deficiency causes no changes in Reelin-dependent activation of the AKT pathway and only mild changes in Reelin-induced dendritic arborization, but reduces Reelin-induced ERK phosphorylation, CREB activation, and Golgi deployment. This work thus establishes ApoER2 as a novel cargo of the AP-4 complex, suggesting that defects in the trafficking of this receptor and in the Reelin signaling pathway could contribute to the pathogenesis of HSP caused by mutations in AP-4 subunits.

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Objetivo: Relatar e compreender o cuidado de enfermagem à pessoa com paraplegia secundária a acidente automobilístico. Método:Estudo qualitativo descritivo, tipo relato de caso, realizado em hospital público de Feira de Santana/BA. Procedeu-se à entrevista, com uma participante em situação de paraplegia. Utilizou-se a elaboração da SAE e suas etapas considerando os aspectos da Teoria de Betty Neuman. Resultados:O relato da paciente e informações obtidas em prontuário demonstraram desinformações e condutas soltas que dificultam o cuidado, alimentavam sentimento de angústia e tristeza à paciente. Conclusão:proporcionou o entendimento acerca do indivíduo vítima de politrauma e suas necessidades, entendendo o adoecer com transcurso multifatorial onde segundo a teoria de Betty Neuman o ambiente e o indivíduo dialogam entre si surtindo efeito positivo e negativo sob o equilíbrio do corpo humano

Objective: To report and understand nursing care for individuals with paraplegia secondary to car accidents. Method:Descriptive qualitative study, in the form of a case report, conducted at a public hospital in Feira de Santana/BA. An interview was conducted with a participant in a paraplegic situation. The Nursing Process (NP) was developed, and its stages were considered, taking into account aspects of Betty Neuman's Theory. Results:The patient's account and information obtained from medical records revealed misinformation and disjointed behaviors that hindered care, fostering feelings of anguish and sadness in the patient. Conclusion:This study provided an understanding of individuals suffering from polytrauma and their needs, understanding illness as a multifactorial process where, according to Betty Neuman's theory, the environment and the individual interact, having both positive and negative effects on the balance of the human body.

Objetivo: Informar y comprender el cuidado de enfermería para personas con paraplejia secundaria a accidentes automovilísticos. Método:Estudio cualitativo descriptivo, tipo informe de caso, llevado a cabo en un hospital público en Feira de Santana/BA. Se realizó una entrevista con una participante en situación de paraplejia. Se utilizó el desarrollo del Proceso de Enfermería (PE) y sus etapas considerando los aspectos de la Teoría de Betty Neuman. Resultados:El relato de la paciente y la información obtenida de los registros médicos revelaron desinformación y conductas desarticuladas que dificultaron el cuidado, fomentando sentimientos de angustia y tristeza en la paciente. Conclusión:Este estudio proporcionó una comprensión de las necesidades de individuos que sufren politraumatismos, entendiendo la enfermedad como un proceso multifactorial donde, según la teoría de Betty Neuman, el entorno y el individuo interactúan, teniendo efectos tanto positivos como negativos en el equilibrio del cuerpo humano.

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Abstract Background Cerebellar ataxias comprise sporadic and genetic etiologies. Ataxia may also be a presenting feature in hereditary spastic paraplegias (HSPs). Objective To report a descriptive analysis of the frequency of different forms of cerebellar ataxia evaluated over 17 years in the Ataxia Unit of Universidade Federal de São Paulo, Brazil. Methods Charts of patients who were being followed from January 2007 to December 2023 were reviewed. We used descriptive statistics to present our results as frequencies and percentages of the overall analysis. Diagnosed patients were classified according to the following 9 groups: sporadic ataxia, spinocerebellar ataxias (SCAs), other autosomal dominant cerebellar ataxias, autosomal recessive cerebellar ataxias (ARCAs), mitochondrial ataxias, congenital ataxias, X-linked ataxias, HSPs, and others. Results There were 1,332 patients with ataxias or spastic paraplegias. Overall, 744 (55.85%) of all cases were successfully diagnosed: 101 sporadic ataxia, 326 SCAs, 20 of other autosomal dominant cerebellar ataxias, 186 ARCAs, 6 X-linked ataxias, 2 mitochondrial ataxias, 4 congenital ataxias, and 51 HSPs. Conclusion This study describes the frequency of cerebellar ataxias in a large group of patients followed for the past 17 years, of whom 55% obtained a definitive clinical or molecular diagnosis. Future demographic surveys in Brazil or Latin American remain necessary.

Resumo Antecedentes Ataxias cerebelares compreendem as etiologias esporádicas e genéticas. Ataxia também pode ser uma característica das paraplegias espásticas hereditárias (HSPs). Objetivo Relatar uma análise descritiva da frequência das diferentes formas de ataxias cerebelares avaliadas ao longo de 17 anos no Setor da Ataxias da Universidade Federal de São Paulo, Brasil. Métodos Prontuários de pacientes acompanhados de janeiro de 2007 a dezembro de 2023 foram revisados. Usamos análise descritiva para apresentar nossos resultados como frequências e percentuais. Os pacientes foram classificados de acordo com os 9 grupos seguintes: ataxias esporádicas, ataxias espinocerebelares (SCA), outras ataxias cerebelares autossômicas dominantes, ataxias cerebelares autossômicas recessivas (ARCA), ataxias mitocondriais, ataxias congênitas, ataxias ligadas ao X, PEH e outros. Resultados Foram avaliados 1.332 pacientes. Desse total, 744 tiveram um diagnóstico definitivo: 101 ataxias esporádicas, 326 SCA, 20 outras ataxias cerebelares autossômicas dominantes, 186 (ARCA), 6 ataxias ligadas ao X, 2 ataxias mitocondriais, 4 ataxias congênitas e 51 HSP. Conclusão Esse estudo descreve a frequência e a etiologia das ataxias em um grande grupo de pacientes acompanhados nos últimos 17 anos, dos quais 55% obtiveram diagnóstico clínico ou molecular definitivos. Estudos demográficos futuros do Brasil ou da América Latina continuam sendo necessários.

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Abstract Background Hereditary or familial spastic paraplegias (SPG) comprise a group of genetically and phenotypically heterogeneous diseases characterized by progressive degeneration of the corticospinal tracts. The complicated forms evolve with other various neurological signs and symptoms, including movement disorders and ataxia. Objective To summarize the clinical descriptions of SPG that manifest with movement disorders or ataxias to assist the clinician in the task of diagnosing these diseases. Methods We conducted a narrative review of the literature, including case reports, case series, review articles and observational studies published in English until December 2022. Results Juvenile or early-onset parkinsonism with variable levodopa-responsiveness have been reported, mainly in SPG7 and SPG11. Dystonia can be observed in patients with SPG7, SPG11, SPG22, SPG26, SPG35, SPG48, SPG49, SPG58, SPG64 and SPG76. Tremor is not a frequent finding in patients with SPG, but it is described in different types of SPG, including SPG7, SPG9, SPG11, SPG15, and SPG76. Myoclonus is rarely described in SPG, affecting patients with SPG4, SPG7, SPG35, SPG48, and SPOAN (spastic paraplegia, optic atrophy, and neuropathy). SPG4, SPG6, SPG10, SPG27, SPG30 and SPG31 may rarely present with ataxia with cerebellar atrophy. And autosomal recessive SPG such as SPG7 and SPG11 can also present with ataxia. Conclusion Patients with SPG may present with different forms of movement disorders such as parkinsonism, dystonia, tremor, myoclonus and ataxia. The specific movement disorder in the clinical manifestation of a patient with SPG may be a clinical clue for the diagnosis.

Resumo Antecedentes As paraplegias espásticas hereditárias ou familiares (SPG) compreendem um grupo de doenças geneticamente e fenotipicamente heterogêneas caracterizadas por degeneração progressiva dos tratos corticospinais. As formas complicadas evoluem com vários outros sinais e sintomas neurológicos, incluindo distúrbios do movimento e ataxia. Objetivo Resumir as descrições clínicas de SPG que se manifestam com distúrbios do movimento ou ataxias para auxiliar o clínico na tarefa de diagnosticar essas doenças. Métodos Realizamos uma revisão da literatura, incluindo relatos de casos, séries de casos, artigos de revisão e estudos observacionais publicados em inglês até dezembro de 2022. Resultados O parkinsonismo juvenil ou de início precoce com resposta variável à levodopa foi relatado principalmente em SPG7 e SPG11. A distonia pode ser observada em pacientes com SPG7, SPG11, SPG22, SPG26, SPG35, SPG48, SPG49, SPG58, SPG64 e SPG76. O tremor não é um achado frequente em pacientes com SPG, mas é descrito em diferentes tipos de SPG, incluindo SPG7, SPG9, SPG11, SPG15 e SPG76. A mioclonia é raramente descrita em SPG, afetando pacientes com SPG4, SPG7, SPG35, SPG48 e SPOAN (paraplegia espástica, atrofia óptica e neuropatia). SPG4, SPG6, SPG10, SPG27, SPG30 e SPG31 podem raramente apresentar ataxia com atrofia cerebelar. E SPG autossômico recessivo, como SPG7 e SPG11, também pode apresentar ataxia. Conclusão Indivíduos com SPG podem apresentar diferentes formas de distúrbios do movimento, como parkinsonismo, distonia, tremor, mioclonia e ataxia. O distúrbio específico do movimento na manifestação clínica de um paciente com SPG pode ser uma pista clínica para o diagnóstico.

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Objective: to outline the profile of risk groups for spinal cord injury (SCI) at the Hospital de Clinicas de Campinas by an epidemiological survey of 41 patients with SCI. Methods: Data from patients with SCI were collected and analyzed: demographic data, level of neurological injury, visual analogue scale (VAS), and the current American Spinal Injury Association (ASIA) impairment scale (AIS), using questionnaires, medical records, and imaging tests. Fisher's exact test was used to assess the relationship between categorical variables, Spearman's correlation coefficient was used for numerical variables, and the Mann-Whitney and Kruskal-Wallis tests were used to analyze the relationship between categorical and numerical variables, with significance level of 5%. Results: There was a prevalence of 82.9% of men, a mean age of 26.5 years, and traffic accidents as the cause of SCI in 56.1% of cases. Conclusion: Results suggest the importance of SCI prevention campaigns directed at this population. Level of Evidence II, Retrospective Study.

Objetivo: Traçar o perfil dos grupos de risco para trauma raquimedular (TRM) do Hospital das Clínicas de Campinas através de levantamento epidemiológico de 41 pacientes vítimas de TRM. Métodos: Foram coletados e analisados dados demográficos, nível da lesão neurológica, escala visual analógica (EVA) e American Spinal Injury Association impairment scale (AIS) atuais, através da aplicação de questionários, análise de prontuários e de exames de imagem. Para avaliar a relação entre as variáveis categóricas foi utilizado o teste exato de Fisher; para as variáveis numéricas foi utilizado o coeficiente de correlação de Spearman; e para a análise da relação entre variáveis categóricas e numéricas foram utilizados os testes de Mann-Whitney e Kruskal-Wallis, adotando nível de significância de 5%. Resultados: Houve prevalência de 82,9% do sexo masculino, média de idade de 26,5 anos e de 56,1% casos de TRM causados por acidente automobilístico. Conclusão: Os resultados sugerem a importância da realização de campanhas de prevenção ao TRM voltadas para essa população. Nível de Evidência II, Estudo Retrospectivo.

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Spinal cord ischemia due to decreased cord perfusion is a devastating complication in patients with thoracoabdominal dissection following frozen elephant trunk (FET) repair surgery. However, rare occurrence of spinal cord ischemia leading to paraplegia after long-term follow-up of FET repair has been reported. Here, we describe a case of spinal cord ischemia resulting in paraplegia nine years after hybrid total arch repair with FET. Cerebrospinal fluid drainage and serial treatment were utilized to decrease intraspinal pressure and increase blood flow to the spinal cord. Three months after the onset of paraplegia and with treatment and rehabilitation, the patient recovered to walk.

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Las normativas legales peruanas como es la Ley 29973, busca la promoción, protección de las personas con discapacidad en el ámbito laboral logrando así el desarrollo, igualdad e inclusión social, política que actualmente aplicado en las instituciones públicas ni privadas en Perú. Objetivo. Analizar la influencia de la discapacidad física en la inserción laboral en Perú. Materiales y Métodos. La metodología que se utilizó estuvo enmarcada en el enfoque cuantitativo, el tipo de investigación fue básica causal explicativo, el método hipotético deductivo, el diseño de la investigación fue no experimental de corte transeccional. La población fue de 61 usuarios, la técnica utilizada para la recolección de datos fue la encuesta y como instrumento el cuestionario, los datos se analizaron mediante el software estadístico SPSS. El procesamiento estadístico fue no paramétrico de Chi-Cuadrada. Resultados. Como resultado de la investigación fue que el 31,1% de los usuarios manifestaron que no están insertados en el mercado laboral ya que la discapacidad física que poseen es de tipo paraplejia, es decir, que, ambas piernas carecen de movilidad lo cual es una restricción para su desarrollo y dificulta su integración en el mercado laboral. Conclusiones. La discapacidad influye significativamente en la inserción laboral de los usuarios de la Oficina Municipal de Atención a las Personas con Discapacidad.

Peruvian legal regulations such as Law 29973, seeks the promotion and protection of people with disabilities in the workplace, thus achieving development, equality and social inclusion, a policy that is currently applied in public and private institutions in Peru. Objective. To analyze the influence of physical disability on labor market insertion in Peru. Materials and Methods. The methodology used was framed in the quantitative approach, the type of research was basic causal explanatory, the method was hypothetical deductive, the research design was non-experimental of transectional cut. The population consisted of 61 users, the technique used for data collection was the survey and the instrument was the questionnaire; the data were analyzed using SPSS statistical software. The statistical processing was nonparametric Chi-Square. Results. As a result of the research, 31.1% of the users stated that they are not inserted in the labor market since the physical disability they have is paraplegia, that is, both legs lack mobility, which is a restriction for their development and hinders their integration in the labor market. Conclusions. Disability has a significant influence on the labor market insertion of the users of the Municipal Office of Attention to People with Disabilities.

As normas legais peruanas, como a Lei 29973, buscam a promoção e a proteção de pessoas com deficiência no local de trabalho, alcançando assim o desenvolvimento, a igualdade e a inclusão social, uma política que atualmente é aplicada em instituições públicas e privadas no Peru. Objetivo. Analisar a influência da deficiência física na inserção laboral no Peru. Materiais e métodos. A metodologia utilizada foi enquadrada no enfoque quantitativo, o tipo de pesquisa foi causal explicativa básica, o método foi hipotético dedutivo, o desenho da pesquisa foi de corte transeccional não experimental. A população foi de 61 usuários, a técnica utilizada para a coleta de dados foi a pesquisa e como instrumento o questionário, os dados foram analisados com o uso do software estatístico SPSS. O processamento estatístico foi o qui-quadrado não paramétrico. Resultados. Como resultado da pesquisa, 31,1% dos usuários afirmaram que não estão inseridos no mercado de trabalho porque a deficiência física que possuem é a paraplegia, ou seja, falta mobilidade nas duas pernas, o que é uma restrição para o seu desenvolvimento e dificulta a sua integração no mercado de trabalho. Conclusões. A deficiência tem uma influência significativa na inserção no mercado de trabalho dos usuários da Secretaria Municipal de Atenção às Pessoas com Deficiência.

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ABSTRACT Introduction: Paraplegia may develop as a result of spinal cord ischemia-reperfusion injury in patients who underwent thoracoabdominal aortic surgery. The objective of this research is to determine the neuroprotective effects of ginsenoside Rd pretreatment in a rat model of spinal cord ischemia-reperfusion injury. Methods: Sprague-Dawley rats (n=36) were randomly assigned to three groups. The sham (n=12) and control (n=12) groups received normal saline orally. The Rd group (n=12) received ginsenoside Rd (100 mg/kg) orally 48 hours before the induction of spinal cord ischemia. Spinal cord ischemia was induced by aortic occlusion using a Fogarty balloon catheter in the Rd and control groups. A neurological assessment according to the motor deficit index and a histological evaluation of the spinal cord were performed. To evaluate the antioxidant activity of ginsenoside Rd, malondialdehyde levels and superoxide dismutase activity were determined. Further, the tissue levels of tumor necrosis factor-alpha and interleukin-1 beta were measured. Results: The Rd group showed significantly lower motor deficit index scores than did the control group throughout the entire experimental period (P<0.001). The Rd group demonstrated significantly greater numbers of normal motor neurons than did the control group (P=0.039). The Rd group exhibited decreased malondialdehyde levels (P<0.001) and increased superoxide dismutase activity (P=0.029) compared to the control group. Tumor necrosis factor-alpha and interleukin-1 beta tissue levels were significantly decreased in the Rd group (P<0.001). Conclusion: Ginsenoside Rd pretreatment may be a promising treatment to prevent ischemia-reperfusion injury in patients who undergo thoracoabdominal aortic surgery.

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OBJECTIVE: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data. METHODS: Cross-sectional study was carried out in two university hospitals in Brazil. Two groups participated in the study: the case group (n = 28) with a confirmed genetic diagnosis for SPG4 and a control group (n = 17) matched for sex and age. The speech assessment of both groups included: speech task recording, acoustic analysis, and auditory-perceptual analysis. In addition, disease severity was assessed with the Spastic Paraplegia Rating Scale (SPRS). RESULTS: In the auditory-perceptual analysis, 53.5% (n = 15) of individuals with SPG4 were dysarthric, with mild to moderate changes in the subsystems of phonation and articulation. On acoustic analysis, SPG4 subjects' performances were worse in measurements related to breathing (maximum phonation time) and articulation (speech rate, articulation rate). The articulation variables (speech rate, articulation rate) are related to the age of onset of the first motor symptom. CONCLUSION: Dysarthria in SPG4 is frequent and mild, and it did not evolve in conjunction with more advanced motor diseases. This data suggest that diagnosed patients should be screened and referred for speech therapy evaluation and those pathophysiological mechanisms of speech involvement may differ from the length-dependent degeneration of the corticospinal tract.

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INTRODUCTION: Paraplegia may develop as a result of spinal cord ischemia-reperfusion injury in patients who underwent thoracoabdominal aortic surgery. The objective of this research is to determine the neuroprotective effects of ginsenoside Rd pretreatment in a rat model of spinal cord ischemia-reperfusion injury. METHODS: Sprague-Dawley rats (n=36) were randomly assigned to three groups. The sham (n=12) and control (n=12) groups received normal saline orally. The Rd group (n=12) received ginsenoside Rd (100 mg/kg) orally 48 hours before the induction of spinal cord ischemia. Spinal cord ischemia was induced by aortic occlusion using a Fogarty balloon catheter in the Rd and control groups. A neurological assessment according to the motor deficit index and a histological evaluation of the spinal cord were performed. To evaluate the antioxidant activity of ginsenoside Rd, malondialdehyde levels and superoxide dismutase activity were determined. Further, the tissue levels of tumor necrosis factor-alpha and interleukin-1 beta were measured. RESULTS: The Rd group showed significantly lower motor deficit index scores than did the control group throughout the entire experimental period (P<0.001). The Rd group demonstrated significantly greater numbers of normal motor neurons than did the control group (P=0.039). The Rd group exhibited decreased malondialdehyde levels (P<0.001) and increased superoxide dismutase activity (P=0.029) compared to the control group. Tumor necrosis factor-alpha and interleukin-1 beta tissue levels were significantly decreased in the Rd group (P<0.001). CONCLUSION: Ginsenoside Rd pretreatment may be a promising treatment to prevent ischemia-reperfusion injury in patients who undergo thoracoabdominal aortic surgery.

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ABSTRACT Objective: to outline the profile of risk groups for spinal cord injury (SCI) at the Hospital de Clinicas de Campinas by an epidemiological survey of 41 patients with SCI. Methods: Data from patients with SCI were collected and analyzed: demographic data, level of neurological injury, visual analogue scale (VAS), and the current American Spinal Injury Association (ASIA) impairment scale (AIS), using questionnaires, medical records, and imaging tests. Fisher's exact test was used to assess the relationship between categorical variables, Spearman's correlation coefficient was used for numerical variables, and the Mann-Whitney and Kruskal-Wallis tests were used to analyze the relationship between categorical and numerical variables, with significance level of 5%. Results: There was a prevalence of 82.9% of men, a mean age of 26.5 years, and traffic accidents as the cause of SCI in 56.1% of cases. Conclusion: Results suggest the importance of SCI prevention campaigns directed at this population. Level of Evidence II, Retrospective Study.

RESUMO Objetivo: Traçar o perfil dos grupos de risco para trauma raquimedular (TRM) do Hospital das Clínicas de Campinas através de levantamento epidemiológico de 41 pacientes vítimas de TRM. Métodos: Foram coletados e analisados dados demográficos, nível da lesão neurológica, escala visual analógica (EVA) e American Spinal Injury Association impairment scale (AIS) atuais, através da aplicação de questionários, análise de prontuários e de exames de imagem. Para avaliar a relação entre as variáveis categóricas foi utilizado o teste exato de Fisher; para as variáveis numéricas foi utilizado o coeficiente de correlação de Spearman; e para a análise da relação entre variáveis categóricas e numéricas foram utilizados os testes de Mann-Whitney e Kruskal-Wallis, adotando nível de significância de 5%. Resultados: Houve prevalência de 82,9% do sexo masculino, média de idade de 26,5 anos e de 56,1% casos de TRM causados por acidente automobilístico. Conclusão: Os resultados sugerem a importância da realização de campanhas de prevenção ao TRM voltadas para essa população. Nível de Evidência II, Estudo Retrospectivo.

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Introducción: Los trastornos funcionales son un reto clínico en la atención de pacientes con déficits neurológicos. Pueden generar manifestaciones clínicas típicas y gran discapacidad. Para su diagnóstico se requiere de una alta sospecha inicial asociado a una batería de examen físico completa. Hallazgos clínicos: Presentamos el caso de una paciente indígena embarazada de 19 años, con pérdida de la fuerza en sus extremidades inferiores, un nivel sensitivo y pérdida del control de los esfínteres. Luego de una evolución estacionaria con estudios imagenológicos e infectocontagiosos dentro de la normalidad. Se sospechó el diagnóstico de un trastorno neurológico funcional por lo que se realizó la prueba del Spinal Injuries Center (SIC) el cual fue positivo. Tratamiento y evolución: Realizamos una intervención multidisciplinar, incluyendo el servicio de rehabilitación, neurología, psiquiatría y psicología. Se utilizaron intervenciones con movimientos articulares, fortalecimiento muscular, estimulación eléctrica y psicoterapia. Posteriormente se obtuvo la recuperación completa de la paciente antes del alta hospitalaria, con la exigencia de seguimiento ambulatorio, además de una inserción satisfactoria en las actividades sociales y familiares. Conclusiones: Este caso refleja la importancia de un análisis neurológico detallado, el conocimiento de diferentes herramientas de semiología y el reto diagnóstico de los trastornos funcionales en neurológicos. La intervención de un equipo multidisciplinar favorece abordajes multidimensionales y resultados clínicos favorables.

Introduction: Functional disorders pose a clinical challenge in the care of patients with neurological deficits. They can generate typical clinical manifestations and great disability. Diagnosis requires a high initial suspicion together with comprehensive physical examination. Clinical Findings: We present the case of a 19-year-old pregnant indigenous patient, with loss of strength in her lower extremities, with a sensitive level and loss of sphincter control. After a stationary evolution with imaging and infectious studies within normal limits, a diagnosis of a functional neurological disorder was suspected; thus, the Spinal Injuries Center (SIC) test was performed, showing positive results. Treatment and evolution: A multidisciplinary intervention was carried out, including the neurology, psychiatry and psychology rehabilitation. Interventions amied towards joint movements, muscle strengthening, electrical stimulation and psychotherapy were used. Eventually, the patient's complete recovery was achieved before hospital discharge, in addition to a satisfactory integration into social and family activities, with a outpatient follow-up requirement. Conclusions: This case reflects the importance of a detailed neurological analysis, knowledge of different semiology tools and the diagnostic challenge of functional neurological disorders. The intervention of a multidisciplinary team favors multidimensional approaches and favorable clinical results.

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ABSTRACT Post-thoracotomy paraplegia after non-aortic surgery is an extremely uncommon complication. A 56-year-old woman presented with a 1-year history of progressive shortness of breath. Computed tomography revealed a locally advanced posterior mediastinal mass involving the ribs and the left neural foramina. Tumor excision with a left pneumonectomy was performed. Post-resection, bleeding was noted in the vicinity of the T4-T5 vertebral body, and the bleeding point was packed with oxidized cellulose gauze (Surgicel®). Postoperatively, the patient complained of bilateral leg numbness extending up to the T5 level, with bilateral paraplegia. An urgent laminectomy was performed, and we noted that the spinal cord was compressed by two masses of Surgicel® with blood clots measuring 1.5 × 1.5cm at T4 and T5 levels. The paraplegia did not improve despite the removal of the mass, sufficient decompression, and aggressive postoperative physiotherapy. Surgeons operating in fields close to the intervertebral foramen should be aware of the possible threat to the adjacent spinal canal as helpful hemostatic agents can become a preventable threat.

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ABSTRACT Spinal cord ischemia due to decreased cord perfusion is a devastating complication in patients with thoracoabdominal dissection following frozen elephant trunk (FET) repair surgery. However, rare occurrence of spinal cord ischemia leading to paraplegia after long-term follow-up of FET repair has been reported. Here, we describe a case of spinal cord ischemia resulting in paraplegia nine years after hybrid total arch repair with FET. Cerebrospinal fluid drainage and serial treatment were utilized to decrease intraspinal pressure and increase blood flow to the spinal cord. Three months after the onset of paraplegia and with treatment and rehabilitation, the patient recovered to walk.

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Abstract Objective: To describe the speech pattern of patients with hereditary Spastic Paraplegia type 4 (SPG4) and correlated it with their clinical data. Methods: Cross-sectional study was carried out in two university hospitals in Brazil. Two groups participated in the study: the case group (n = 28) with a confirmed genetic diagnosis for SPG4 and a control group (n = 17) matched for sex and age. The speech assessment of both groups included: speech task recording, acoustic analysis, and auditory-perceptual analysis. In addition, disease severity was assessed with the Spastic Paraplegia Rating Scale (SPRS). Results: In the auditory-perceptual analysis, 53.5% (n = 15) of individuals with SPG4 were dysarthric, with mild to moderate changes in the subsystems of phonation and articulation. On acoustic analysis, SPG4 subjects' performances were worse in measurements related to breathing (maximum phonation time) and articulation (speech rate, articulation rate). The articulation variables (speech rate, articulation rate) are related to the age of onset of the first motor symptom. Conclusion: Dysarthria in SPG4 is frequent and mild, and it did not evolve in conjunction with more advanced motor diseases. This data suggest that diagnosed patients should be screened and referred for speech therapy evaluation and those pathophysiological mechanisms of speech involvement may differ from the length-dependent degeneration of the corticospinal tract.

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ABSTRACT Introduction: Hereditary spastic paraplegia (HSP) is the term for a group of neurological disorders characterized by progressive spasticity and muscle weakness in the lower limbs. Its etiology is genetic and has been associated with mutations in more than 60 genes. HSP is rare and may be useful in the differential diagnosis of cerebral palsy. Case presentation: 16-year-old male with a diagnosis of HSP due to mutation of the NIPAi gene:c.316G>A (p. Gly106arg), which corresponds to HSP type 6 (SPG6). The patient presented with clinical signs of progressive upper motor neuron syndrome in the lower limbs, such as spasticity, hyperreflexia and paraparesis, associated with focal onset seizures diagnosed at age 11 and successfully treated with valproic acid. Spasticity treatment was complex and included oral baclofen, intraoperative botulinum toxin, physical therapy, and multilevel orthopedic surgery for the management of musculoskeletal deformities. Conclusion: This is a rare case of complex HSP, associated with epilepsy, due to the mutation of the NIPAi gene (SPG6), the most common pathogenic variant within this type of mutation. The present case demonstrates the importance of making an early diagnosis of GSP6 to perform timely interventions in these patients, prevent complications, and avoid a higher level of disability.

RESUMEN Introducción. La paraplejía espástica hereditaria (PEH) es un grupo de trastornos neurológicos caracterizados por espasticidad progresiva y debilidad muscular de miembros inferiores. Su etiología es genética y se ha asociado con mutaciones en más de 60 genes. La PEH es poco frecuente y puede ser útil en el diagnóstico diferencial de la parálisis cerebral. Presentación de caso. Adolescente masculino de 16 años con diagnóstico de PEH por mutación del gen NIPAi: c. 316G>A (p. Gly106arg), correspondiente a una PEH tipo 6 (SPG6). El paciente presentó signos clínicos de síndrome de motoneurona superior progresivos en miembros inferiores como espasticidad, hiperreflexia y paraparesia, asociados a epilepsia de inicio focal diagnosticada a los 11 años y tratada satisfactoriamente con ácido valproico. El manejo de la espasticidad fue complejo e incluyó baclofeno oral, toxina botulínica intraoperatoria, terapia física y cirugía ortopédica multinivel para manejo de deformidades musculoesqueléticas en miembros inferiores. Conclusión. El presente caso demuestra la importancia de realizar un diagnóstico temprano de la SPG6 (variante más común de la PEH) para realizar intervenciones oportunas en estos pacientes, prevenir complicaciones y evitar un mayor nivel de discapacidad.