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INTRODUCTION: Syringomyelia is present in 40% of pediatric patients with Chiari malformation. Typically treated with posterior fossa decompression, some cases require further intervention such as syrinx shunting. CASE REPORT: We report a 16-year-old female with Chiari type 1 malformation and syringomyelia who underwent posterior fossa decompression and subsequent free syringo-subarachnoid-peritoneal shunting. The patient developed symptoms of CSF overdrainage, and imaging indicated CSF hypotension. A distal catheter ligation temporarily improved symptoms, but eventually, a programmable ventricular shunt was necessary due to shunt dependence. CONCLUSION: This case highlights the rare complication of CSF overdrainage from syrinx shunting and the importance of shunt selection considerations.
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AIM: To describe a term newborn with acquired severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection and multisystem involvement including seizures associated to ischemic lesions in the brain. BACKGROUND: Coronavirus disease 2019 (COVID-19) is predominantly a respiratory infection, but it may affect many other systems. Most pediatric COVID-19 cases range from asymptomatic to mild-moderate disease. There are no specific clinical signs described for neonatal COVID-19 infections. In children, severe central nervous system compromise has been rarely reported. CASE DESCRIPTION: We describe a 17-day-old newborn who acquired a SARS-CoV-2 infection in a family meeting that was admitted for fever, seizures and lethargy and in whom consumption coagulopathy, ischemic lesions in the brain and cardiac involvement were documented. CONCLUSIONS: SARS-CoV-2 neonatal infection can be associated with multi-organic involvement. In our patient, significant central nervous system compromise associated to ischemic lesions and laboratory findings of consumption coagulopathy were found. CLINICAL SIGNIFICANCE: Although neonatal SARS-CoV-2 infections are infrequent, they can be associated with multi-organic involvement. Neonatologists and pediatricians should be aware of this unusual way of presentation of COVID-19 in newborn infants.
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Isquemia Encefálica/virologia , COVID-19/complicações , Doenças do Recém-Nascido/virologia , SARS-CoV-2/isolamento & purificação , Aciclovir/uso terapêutico , Antibacterianos/uso terapêutico , Antivirais/uso terapêutico , Encéfalo/diagnóstico por imagem , Isquemia Encefálica/patologia , COVID-19/patologia , Ceftriaxona/uso terapêutico , Febre , Lobo Frontal/irrigação sanguínea , Lobo Frontal/diagnóstico por imagem , Lobo Frontal/patologia , Humanos , Recém-Nascido , Doenças do Recém-Nascido/tratamento farmacológico , Doenças do Recém-Nascido/patologia , Letargia , Imageamento por Ressonância Magnética , Masculino , Nasofaringe/virologia , Convulsões , Tratamento Farmacológico da COVID-19RESUMO
The syndrome of transient headache and neurological deficits with cerebrospinal fluid lymphocytosis or pseudomigraine with temporary neurological symptoms and lymphocytic pleocytosis (HaNDL) is a syndrome consisting of recurrent headaches, reversible neurological deficit, lymphocytic pleocytosis in cerebrospinal fluid (CSF), variable duration over time and spontaneous resolution. Although several etiopathogenic mechanisms have been suggested (vascular, infectous, immunological and calcium channelopthy), its etiology remains unknown. We describe a 28 year old female, with recurrent migraine with pleocytosis, confusional syndrome and transient neurological deficit. The clinical remission was achieved within two months. Although its etiology remains unknown the differential diagnosis is discussed in order to keep in mind this syndrome.
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Linfocitose/líquido cefalorraquidiano , Transtornos de Enxaqueca/líquido cefalorraquidiano , Adulto , Afasia/diagnóstico , Diagnóstico Diferencial , Feminino , Humanos , Contagem de Linfócitos , Linfocitose/sangue , Linfocitose/etiologia , Transtornos de Enxaqueca/sangue , Transtornos de Enxaqueca/etiologia , SíndromeRESUMO
Hirayama disease is a rare neurological disorder characterized by an insidious progressive subacute unilateral or bilateral weakness of the hands and forearm muscles leading to a painless amyotrophy. The disease primarily affects young men in the second to third decades of life. It has always been described as a second motor neuron disease, thus sparing the pyramidal and sensitive pathways. It usually has a slow progression course of 3 to 5 years followed by stabilization. Since its initial description by Keyzo Hirayama in 1959, most cases have been reported in Asia, particularly Japan and India, although the disease reportedly has worldwide distribution.
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Wernicke encephalopathy is caused by thiamine deficiency. Although the clinical picture has been well established for some time, clinical diagnosis is attained in only 20% of the cases. MR imaging techniques contribute to early diagnosis of Wernicke encephalopathy. We herein report MR imaging and proton MR spectroscopic findings for a patient with clinical and biochemical features consistent with Wernicke encephalopathy. Increased lactate and typical MR imaging findings are discussed in the context of the known pathophysiology of Wernicke encephalopathy.
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Ácido Aspártico/análogos & derivados , Espectroscopia de Ressonância Magnética , Encefalopatia de Wernicke/diagnóstico , Adulto , Ácido Aspártico/análise , Encéfalo/patologia , Química Encefálica , Imagem de Difusão por Ressonância Magnética , Feminino , Humanos , Ácido Láctico/análise , Encefalopatia de Wernicke/metabolismoRESUMO
We describe a patient who showed cystic lesions in the midbrain and obstructive hydrocephalus. MR imaging features corresponded to abnormal dilatation of perivascular spaces. Similar clinical and MR imaging findings were described in previous reports of patients presenting with mesencephalo-thalamic expanding lacunae; the diagnosis of some of those cases was confirmed at postmortem examination This condition is extremely rare but should be part of a differential diagnosis when evaluating cystic abnormalities in this area of the brain.