Isolated Area Postrema Syndrome Preceding the Diagnosis of Giant Cell Arteritis: A Case Report.

OBJECTIVE: We report a case of biopsy-proven giant cell arteritis after an initial presentation of area postrema syndrome. METHODS: A 65-year-old man was evaluated using MRI, temporal artery biopsy, and ultrasound. RESULTS: The patient presented with refractory nausea, vomiting, and hiccups that caused weight loss without any other neurologic or clinical symptoms. His MRI scan 15 days later revealed a hyperintense sign on the area postrema with no abnormal diffusion or contrast enhancement, compatible with isolated area postrema syndrome. An extensive workup for inflammation and other etiologies including neuromyelitis optica spectrum disorder (NMOSD), myelin oligodendrocyte glycoprotein antibody disorder, and multiple sclerosis (MS) showed negative results. The patient responded to treatment with methylprednisolone. Two months after the initial clinical manifestation, the patient developed fatigue, headache, and scalp tenderness. He was diagnosed with giant cell arteritis after ultrasonography and biopsy were performed. He responded well to oral glucocorticoids and had only 1 relapse during tapering. He has not had arteritic ischemic optic neuropathy or any new episodes of area postrema syndrome. DISCUSSION: This case demonstrates the importance of expanding the differential diagnosis in patients with area postrema syndrome and no other signs of NMOSD.

Plasma exchange in inflammatory demyelinating disorders of the central nervous system: reasonable use in the clinical practice.

Plasma exchange (PLEX) is a therapeutic apheresis modality in which the plasma is separated from inflammatory factors such as circulating autoreactive immunoglobulins, the complement system, and cytokines, and its therapeutic effect is based on the removal of these mediators of pathological processes. Plasma exchange is well established for various neurological disorders, and it is applied successfully in central nervous system inflammatory demyelinating diseases (CNS-IDD). It mainly modulates the humoral immune system; thus, it has a greater theoretical effect in diseases with prominent humoral mechanisms, such as neuromyelitis optica (NMO). However, it also has a proven therapeutic effect in multiple sclerosis (MS) attacks. Several studies have suggested that patients with severe attacks of CNS-IDD have poor response to steroid therapy but show clinical improvement after the PLEX treatment. Currently, PLEX is generally established only as a rescue therapy for steroid unresponsive relapses. However, there are still research gaps in the literature regarding plasma volume, number of sessions, and how early the apheresis treatment needs to started. Thus, in the present article, we summarize the clinical studies and meta-analyses, especially about MS and NMO, outlining clinical data regarding the experience with therapeutic PLEX in severe attacks of CNS-IDD, the clinical improvement rates, the prognostic factors of a favorable response, and highlighting the likely role of the early apheresis treatment. Further, we have gathered this evidence and suggested a protocol for the treatment of CNS-IDD with PLEX in the routine clinical practice.

Plasmaférese (PLEX) é um procedimento em que o plasma é separado de fatores inflamatórios como imunoglobulinas autorreativas circulantes, sistema complemento e citocinas, e seu efeito terapêutico se baseia na remoção desses mediadores de processos patológicos. A PLEX está bem estabelecida no tratamento de diversos distúrbios neurológicos, e é utilizada com sucesso em surtos de doenças desmielinizantes inflamatórias do sistema nervoso central (CNS-IDD). A PLEX modula principalmente o sistema imunológico humoral; assim, tem efeito teórico maior em doenças com mecanismos patológicos humorais proeminentes, como a neuromielite óptica (NMO). No entanto tem também efeito terapêutico comprovado em surtos de esclerose múltipla (EM). Estudos sugerem que a corticoterapia é pouco eficaz em pacientes com surtos graves de CNS-IDD, e que estes apresentam melhora clínica após o tratamento com PLEX. Atualmente, a PLEX está geralmente estabelecida apenas como terapia de resgate para surtos não responsivos a corticosteroides. No entanto, há lacunas na literatura sobre a quantidade de troca de volume plasmático, o número de sessões, e o tempo de início da aférese terapêutica. Dessa forma, resumimos neste artigo estudos clínicos e metanálises, especialmente sobre EM e NMO, e delineamos os dados clínicos sobre a experiência com o uso de PLEX em surtos graves de CNS-IDD, as taxas de melhora clínica, os fatores prognósticos para uma resposta favorável, e destacamos o provável papel do tratamento precoce nestes casos. Em um segundo momento, reunimos essas evidências em uma sugestão de protocolo de tratamento de CNS-IDD com PLEX na prática clínica rotineira.

Plasma exchange in inflammatory demyelinating disorders of the central nervous system: reasonable use in the clinical practice / Plasmaférese em doenças inflamatórias desmielinizantes do sistema nervoso central: uso coerente na prática clínica

Abstract Plasma exchange (PLEX) is a therapeutic apheresis modality in which the plasma is separated from inflammatory factors such as circulating autoreactive immunoglobulins, the complement system, and cytokines, and its therapeutic effect is based on the removal of these mediators of pathological processes. Plasma exchange is well established for various neurological disorders, and it is applied successfully in central nervous system inflammatory demyelinating diseases (CNS-IDD). It mainly modulates the humoral immune system; thus, it has a greater theoretical effect in diseases with prominent humoral mechanisms, such as neuromyelitis optica (NMO). However, it also has a proven therapeutic effect in multiple sclerosis (MS) attacks. Several studies have suggested that patients with severe attacks of CNS-IDD have poor response to steroid therapy but show clinical improvement after the PLEX treatment. Currently, PLEX is generally established only as a rescue therapy for steroid unresponsive relapses. However, there are still research gaps in the literature regarding plasma volume, number of sessions, and how early the apheresis treatment needs to started. Thus, in the present article, we summarize the clinical studies and meta-analyses, especially about MS and NMO, outlining clinical data regarding the experience with therapeutic PLEX in severe attacks of CNS-IDD, the clinical improvement rates, the prognostic factors of a favorable response, and highlighting the likely role of the early apheresis treatment. Further, we have gathered this evidence and suggested a protocol for the treatment of CNS-IDD with PLEX in the routine clinical practice.

Resumo Plasmaférese (PLEX) é um procedimento em que o plasma é separado de fatores inflamatórios como imunoglobulinas autorreativas circulantes, sistema complemento e citocinas, e seu efeito terapêutico se baseia na remoção desses mediadores de processos patológicos. A PLEX está bem estabelecida no tratamento de diversos distúrbios neurológicos, e é utilizada com sucesso em surtos de doenças desmielinizantes inflamatórias do sistema nervoso central (CNS-IDD). A PLEX modula principalmente o sistema imunológico humoral; assim, tem efeito teórico maior em doenças com mecanismos patológicos humorais proeminentes, como a neuromielite óptica (NMO). No entanto tem também efeito terapêutico comprovado em surtos de esclerose múltipla (EM). Estudos sugerem que a corticoterapia é pouco eficaz em pacientes com surtos graves de CNS-IDD, e que estes apresentam melhora clínica após o tratamento com PLEX. Atualmente, a PLEX está geralmente estabelecida apenas como terapia de resgate para surtos não responsivos a corticosteroides. No entanto, há lacunas na literatura sobre a quantidade de troca de volume plasmático, o número de sessões, e o tempo de início da aférese terapêutica. Dessa forma, resumimos neste artigo estudos clínicos e metanálises, especialmente sobre EM e NMO, e delineamos os dados clínicos sobre a experiência com o uso de PLEX em surtos graves de CNS-IDD, as taxas de melhora clínica, os fatores prognósticos para uma resposta favorável, e destacamos o provável papel do tratamento precoce nestes casos. Em um segundo momento, reunimos essas evidências em uma sugestão de protocolo de tratamento de CNS-IDD com PLEX na prática clínica rotineira.

Diffuse spinal cord metastasis after resection of SMARCB1 sinonasal carcinoma manifesting with a right foot drop-a case report.

INTRODUCTION: The sinonasal carcinoma are rare tumors of the head and neck. The undifferentiated sinonasal carcinoma subtypes are constantly being explored and new mutations, with different prognosis markers and biological behaviors are being described. The SMARCB1 negative sinonasal carcinoma subtypes have been recently described with few reports of leptomeningeal and spinal cord invasion. CASE PRESENTATION: This study presents the case of a 59-year-old woman, with no previous disease, presenting initially with epistaxis that evolved to cranial nerve deficits and a left eye complete oftalmoplegia. After diagnostic investigation, she had a diagnosis of a left ethmoid sinus sinonasal carcinoma. Following resection of the tumor, she evolved with a right foot drop that eventually has been linked to diffuse spinal cord impairment. The histopathological diagnosis confirmed a SMARCB1 negative sinonasal carcinoma. Due to the diffuse metastasis, she underwent palliative care and died eight months after the surgery. DISCUSSION: Spinal cord metastasis may manifest with different clinical signs. Our case shows a rare manifestation of SMARCB1-deficient sinonasal carcinoma, a new subtype of sinonasal carcinoma, summarizing the importance of a high grade of suspicion of spinal cord invasion on these patients. SMARCB1 sinonasal carcinomas are rare new tumors of the head and neck, whose biological behaviors are yet to be explored. To the best of our knowledge, this is one of the few case reports describing simultaneous spread of this tumor to the central nervous system and spinal cord.

An atypical case of neurotoxoplasmosis in immunocompetent patient.

Toxoplasmosis is an infection caused by Toxoplasma gondii, an intracellular protozoan that is often associated with immunocompromised patients and is rare in immunocompetent. A 60-year-old man was admitted with a history of 2 days of headache and right-sided weakness. There was no history of fever, surgeries, or any other comorbid illness. Cerebrospinal fluid showed just mild pleocytosis with 15 cells/mm3, predominantly lymphomononuclear. MRI showed Peripheral enhancing lesion with central diffusion restriction and perivascular enhancing lesion with restricted diffusion with vasogenic edema and leptomeningeal enhancement in the white matter. Viral serologies, tumor markers, protein electrophoresis were normal. The patient was submitted to brain biopsy, revealing necrotic brain parenchyma with predominantly acute inflammation, with diffuse encephalitis pattern, and cysts with bradyzoites (cystozoites) of Toxoplasma gondii in the brain parenchyma. The central nervous system infection by Toxoplasma gondii can present as meningoencephalitis during primary infection in an immunocompetent, although it is rare. Central nervous system lymphoma is the main differential diagnosis of neurotoxoplasmosis by imaging, especially in our case.

Breast milk supplementation and preterm infant development after hospital discharge: a randomized clinical trial / Suplementação do leite materno e desenvolvimento de lactentes pré-termo após alta hospitalar: ensaio clínico randomizado

Abstract Objectives: To assess the effect of maternal breast milk supplementation on the development of exclusively breast-fed very low birth weight preterm infants at 12 months of corrected age. Methods: A randomized clinical trial with 53 infants followed-up after discharge from the neonatal unit until a corrected gestational age of 12 months. Newborns in the intervention group were breastfed exclusively with maternal milk and received 2 g of a multinutrient supplement (Pré-Nan®, Nestlé, Vevey, Switzerland) added to expressed breast milk twice a day until a corrected age of 4–6 months. The control group was exclusively breastfed without supplementation. After monthly follow-up, developmental assessment was performed using the Bayley III Scale. Results: There was no statistically significant difference on the Bayley III Scale between the intervention and control groups in any of the assessed domains: motor, cognitive, and communication. However, scores in the three domains were always higher in the group that received the supplement. There were a similar number of cases of developmental delay in both groups: seven (28%) in the group that received the supplement and nine (33.3%) in the group that was exclusively breastfed. Conclusions: The results failed to show an association between post-discharge multinutrient supplementation and development in the assessed infants.

Resumo Objetivos: Avaliar o efeito da suplementação do aleitamento materno exclusivo com aditivo multicomponente no desenvolvimento de lactentes nascidos pré-termo de muito baixo peso aos 12 meses de idade gestacional corrigida. Método: Ensaio clínico randomizado com 53 lactentes, acompanhados da alta hospitalar na Unidade Neonatal até o 12° mês de idade gestacional corrigida. Aqueles alocados no grupo intervenção permaneciam em aleitamento materno exclusivo e recebiam dois gramas de suplemento multicomponente em pó (Pré-Nan®, Nestlé, Vevey, Suíça), adicionados ao leite ordenhado duas vezes ao dia, por quatro a seis meses de idade gestacional corrigida. O grupo controle permanecia em aleitamento materno exclusivo sem suplementação. Após acompanhamento mensal, foi feita avaliação do desenvolvimento por meio da Escala de Bayley III. Resultados: Na comparação do desenvolvimento pela Escala de Bayley III entre os grupos intervenção e controle, não houve diferença estatística significativa nos domínios estudados: motor, cognitivo e linguagem. Porém, os valores dos escores foram sempre maiores no grupo intervenção do que no grupo controle nos três domínios. O atraso de desenvolvimento se distribuiu de forma similar nos grupos: sete casos (28%) no grupo intervenção e nove (33,3%) no grupo controle. Conclusões: Os resultados não mostraram associação entre suplementação multicomponente pós-alta e desenvolvimento dos lactentes analisados pela Escala de Bayley III.

Breast milk supplementation and preterm infant development after hospital discharge: a randomized clinical trial.

OBJECTIVES: To assess the effect of maternal breast milk supplementation on the development of exclusively breast-fed very low birth weight preterm infants at 12 months of corrected age. METHODS: A randomized clinical trial with 53 infants followed-up after discharge from the neonatal unit until a corrected gestational age of 12 months. Newborns in the intervention group were breastfed exclusively with maternal milk and received 2g of a multinutrient supplement (Pré-Nan(®), Nestlé, Vevey, Switzerland) added to expressed breast milk twice a day until a corrected age of 4-6 months. The control group was exclusively breastfed without supplementation. After monthly follow-up, developmental assessment was performed using the Bayley III Scale. RESULTS: There was no statistically significant difference on the Bayley III Scale between the intervention and control groups in any of the assessed domains: motor, cognitive, and communication. However, scores in the three domains were always higher in the group that received the supplement. There were a similar number of cases of developmental delay in both groups: seven (28%) in the group that received the supplement and nine (33.3%) in the group that was exclusively breastfed. CONCLUSIONS: The results failed to show an association between post-discharge multinutrient supplementation and development in the assessed infants.

Sarcoma sinovial cervical / Neck Synovial Sarcoma

Introdução: O sarcoma sinovial (SS) é uma neoplasia rara e agressiva, sendo a região da cabeça e pescoço envolvida em 5% a 10% dos casos. A apresentação clínica é uma massa indolor de crescimento progressivo. As metástases ocorrem em 10% a 15% dos casos, principalmente por via hematogênica, para pulmões, linfonodos e medula óssea. O tratamento inclui exérese cirúrgica ampla e radioterapia. Ainda não existem dados que comprovem a eficácia da quimioterapia neste tipo de tumor; seu principal benefício consistiria na prevenção de metástases à distancia. A sobrevida em 5 anos varia de 40% a 50%. Objetivo: Relatar um caso de sarcoma sinovial cervical em mulher de 21 anos. Resultados: Paciente com história de tumor cervical de crescimento rápido há 05 meses, inicialmente indolor. Ao exame a lesão apresentava consistência fibroelástica, superfície lisa, aderida a planos profundos, comprometendo os níveis II, III, IV e V à direita e dor à palpação. Tomografia Computadorizada evidenciando grande tumor homogêneo com efeito de massa nos níveis II a V à direita e ocupando espaço parafaríngeo. Punção aspirativa (PAAF) sugestivo de sarcoma. Foi submetida à ressecção do tumor cervical e quimioterapia adjuvante. O anatomopatológico da lesão, com estudo imunohistoquímico, foi compatível com sarcoma sinovial cervical. Está no o 3º ano de seguimento pós-operatório e encontra-se sem sinais de lesão residual ou recidiva. Conclusão: o sarcoma sinovial cervical é uma neoplasia rara e agressiva que demanda ressecção cirúrgica ampla.

Introduction: The synovial sarcoma (SS) is a rare and aggressive neoplasm, with the head and neck involved in 5% to 10% of cases. The clinical presentation is a painless mass with progressive growth. Metastases occur in 10% to 15% of cases, mainly hematogenic to lungs, lymph nodes and bone marrow. Treatment includes wide surgical excision and radiotherapy. There are no data to prove the effectiveness of chemotherapy in this tumor type, but its main benefit would be the prevention of distant metastases. The 5-year survival ranges from 40% to 50%. Objective: To report a case of synovial sarcoma of the neck in a 21 years old female. Results: female with history of cervical tumor of rapid growth for 05 months, initially painless. On examination the lesion presented fibroelastic consistency, smooth surface, adhered to deep planes, compromising levels II, III, IV and V to the right. Computed tomography revealed a large homogenous tumor with mass effect on levels II to V and occupying the right parapharyngeal space. Needle aspiration were suggestive of sarcoma. Underwent resection of the cervical tumor and adjuvant chemotherapy. Histopathological examination of the lesion with immunohistochemical study was consistent with cervical synovial sarcoma. She is in the 3rd year of postoperative follow-up and found no signs of residual lesion or recurrence. Conclusion: synovial sarcoma of the neck is a rare and aggressive neoplasm that requires wide surgical resection.

Estenose faríngea: benefícios do laser de CO2 / Pharyngeal stenosis: the benefits of CO2 laser

Introdução: O tratamento endo-oral com laser de CO2 pararessecção de estenose faringoesofágica apresenta-se como umaopção terapêutica viável na resolução dos casos sem sucessoapós tratamentos convencionais com dilatações. Os autoresapresentam um caso de estenose faringoesofágica tratadocirurgicamente com sessões de laser de CO2 e discorrem sobreresultados funcionais e qualidade de vida no pós-operatóriodo paciente. Objetivo: Descrever a tendência e os benefíciosintrínsecos de tal técnica cirúrgica por via endo-oral para ressecçãode estenose faringoesofágica ocorrida após laringectomia totalcom esvaziamento cervical e radioterapia adjuvante. Relato deCaso: Paciente do gênero feminino, 52 anos, com diagnósticode carcinoma espinocelular de laringe, estádio T4N1M0,submetido a laringectomia total, com esvaziamento cervicalradical modificado e radioterapia adjuvante. Evoluiu com graveestenose faringoesofágica mesmo após tentativas de dilataçõescom sondas, sem sucesso. Foi submetido à ressecção cirúrgicacom laser de CO2 em quatro sessões, com intervalo de 30 diasentre as mesmas. Após ressecção da estenose faringoesofágica,a paciente apresentou resultados funcionais satisfatórios. Evoluiucom boa deglutição para dieta pastosa (aproximadamente1200 ml por dia) até sólidos e com ganho ponderal satisfatório.Comentários finais: O presente trabalho demonstra que o usodo laser de CO2 é válido na ressecção de estenose faríngeaou faringoesofágica por ser um método conservador, queproporciona bom resultado terapêutico e poucas complicaçõespós-operatórias.