PAULO EURIPEDES MARCHIORI
Projetos de Pesquisa
Unidades Organizacionais
Departamento de Neurologia, Faculdade de Medicina - Docente
LIM/45 - Laboratório de Fisiopatologia Neurocirúrgica, Hospital das Clínicas, Faculdade de Medicina
LIM/45 - Laboratório de Fisiopatologia Neurocirúrgica, Hospital das Clínicas, Faculdade de Medicina
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- Neuroinfection survey at a neurological ward in a Brazilian tertiary teaching hospital(2011) MARCHIORI, Paulo E.; LINO, Angelina M. M.; MACHADO, Luis R.; PEDALINI, Livia M.; BOULOS, Marcos; SCAFF, MilbertoOBJECTIVES: This study was undertaken to characterize the neuroinfection profile in a tertiary neurological ward. INTRODUCTION: Neuroinfection is a worldwide concern and bacterial meningitis, tetanus and cerebral malaria have been reported as the commonest causes in developing countries. METHODS: From 1999 to 2007, all patients admitted to the Neurology Ward of Hospital das Clinicas, Sao Paulo University School of Medicine because of neuroinfection had their medical records reviewed. Age, gender, immunological status, neurological syndrome at presentation, infectious agent and clinical outcome were recorded. RESULTS: Three hundred and seventy four cases of neuroinfectious diseases accounted for 4.2% of ward admissions and the identification of infectious agent was successful in 81% of cases. Mean age was 40.5 +/- 13.4 years, 63.8% were male, 19.7% were immunocompromised patients and meningoencephalitis was the most common clinical presentation despite infectious agent. Viruses and bacteria were equally responsible for 29.4% of neuroinfectious diseases; parasitic, fungal and prion infections accounted for 28%, 9.6% and 3.5% respectively. Human immunodeficiency virus (HIV), herpes simplex virus 1 (HSV1), Mycobacterium tuberculosis, Treponema pallidum, Taenia solium, Schistosoma mansoni, Cryptococcus neoformans and Histoplasma capsulatum were the more common infectious pathogens in the patients. Infection mortality rate was 14.2%, of which 62.3% occurred in immunocompetent patients. CONCLUSION: Our institution appeared to share some results with developed and developing countries. Comparison with literature may be considered as quality control to health assistance.
- Cor pulmonale in a patient with Brown-Vialetto-Van Laere syndrome: A case report(2011) SILVA-JUNIOR, Francisco Pereira da; MOURA, Rafael de Deus; ROSEMBERG, Sergio; MARCHIORI, Paulo Euripedes; CASTRO, Luiz Henrique MartinsBrown-Vialetto-Van Laere syndrome (BVVLS) is a rare neurological disease characterized by sensorineural hearing loss and multiple cranial nerve palsies, usually involving the VIIth and IXth to XIIth cranial nerves. We describe the clinical and pathological features of a 33-year-old woman with BVVLS. The patient developed progressive exertional dyspnea, with clinical and laboratory findings of right-sided heart failure and pulmonary hypertension. She developed status epilepticus in the setting of cardiac deterioration and respiratory infection, and died of cardiogenic and septic shock. Autopsy disclosed bilateral neuronal loss and gliosis in the inferior colliculi, locus coeruleus and facial and vestibular nuclei. Cor pulmonale is a complication of hypoventilation-induced hypoxia and hypercapnia and had not yet been reported in BVVLS.
- Psychogenic non-epileptic seizures and psychoanalytical treatment: results(2014) SANTOS, Niraldo de Oliveira; BENUTE, Glaucia Rosana Guerra; SANTIAG, Alessandra; MARCHIORI, Paulo Euripedes; LUCIA, Mara Cristina Souza deBackground: the occurrence of psychogenic non-epileptic seizures (PNES) is estimated to be between 2 to 33 cases in every 100,000 inhabitants. The number of patients with PNES reaches 19% of those treated as epileptics. Patients with PNES are treated as if they had intractable epilepsy, with unsatisfactory results even after medication treatment is used to its maximum. The aim of this study is to present the effects of individual psychoanalytical treatment in patients with PNES, assessing its impact in the evolution of the clinical picture and its association with sex, time of disease, social, psychological and professional harm, as well as going through with treatment. Methods: The case base was composed of 37 patients with PNES. The diagnosis was reached with video-EEG monitoring. Psychoanalytical treatment was carried out through 12 months of weekly sessions timed for around 50-minutes each, in a total of 48 individual sessions. Results: This study found a high rate of success in the treatment of PNES patients. 29.7% (n=11) of patients had cessation or cure of symptoms and 51.4% (n=19) had a decrease in the number of episodes. There is an association between cessation or decrease in the number of episodes and sex (p<0.01), religion (p<0.01) and concluding treatment (p<0.01). Conclusion: Individual psychoanalytical treatment applied to patients with PNES is considered effective and can be an essential form of assistance for the reduction or cessation of episodes.
- Unilateral central retinal artery occlusion as the sole presenting sign of Susac syndrome in a young man: case report(2013) APOSTOLOS-PEREIRA, Samira Luiza dos; KARA-JOSE, Lucia B. Passos; MARCHIORI, Paulo Euripedes; MONTEIRO, Mario Luiz RibeiroWe report the case of a 24-year-old man presenting with sudden visual loss in the left eye from a central retinal artery occlusion. An extensive clinical investigation revealed no etiology. Three weeks later, however, the patient developed hearing loss followed by encephalopathy and multiple branch retinal artery occlusions in the right eye. Fluorescein angiography confirmed retinal vascular occlusions with no sign of vasculitis. The neurological examination revealed a diffuse encephalopathy while the MRI scan disclosed several small areas of infarcts in the brain. Bilateral sensorineural hearing loss was confirmed on audiometry. The patient was diagnosed with Susac syndrome and treated with methylprednisolone and cyclophosphamide, resulting in slight improvement and stabilization. This case shows that Susac syndrome may be diagnosed late due to the absence at onset of one or more of the symptoms of the classic triad (encephalopathy, multiple branch retinal artery occlusions and hearing loss). This case also serves to emphasize that Susac syndrome should be considered in the differential diagnosis of central retinal artery occlusion, even in apparently healthy young men.
- Voiding Dysfunction in Patients With Neuromyelitis Optica Spectrum Disorders(2016) CARVALHO, Fabricio Leite de; GOMES, Cristiano Mendes; APOSTOLOS-PEREIRA, Samira L.; BESSA JR., Jose; PINHEIRO, Marcello; MARCHIORI, Paulo E.; BRUSCHINI, Homero; SROUGI, Miguel; CALLEGARO, DagobertoAims: We assessed the lower urinary tract symptoms (LUTS) and urodynamic findings in patients with neuromyelitis optica spectrum disorders (NMO-SD), a recently defined neurological disease. Methods: We prospectively evaluated seven men and 23 women (mean age 41.1 +/- 13.5 years) with an established diagnosis of NMO-SD who were invited to participate irrespective of the presence of LUTS. Neurological evaluation was assessed with the Expanded Disability Status Scale (EDSS) and LUTS were evaluated with the Overactive Bladder questionnaire (OAB-V8) and the International Prostate Symptom Score (I-PSS). All patients underwent videourodynamics, transabdominal urinary tract sonography, urine culture, and serum creatinine levels. Results: The mean time of disease duration was 33.8 +/- 30.8 months. Neurological evaluation showed a mean EDSS score of 5.3 +/- 1.8. The most frequent videourodynamic findings were detrusor-sphincter dyssynergia (DSD) and detrusor overactivity (DO) in 11 (36.6%) patients, DSD without DO in seven (23.3%) and DO without DSD in six (20.0%) patients. Voiding dysfunction assessed by I-PSS and OAB-V8 increased with the degree of neurological impairment (P = 0.018; r = 0.42 and P = 0.006; r = 0.48 respectively). Patients with DSD had higher I-PSS (18.5 +/- 11.4 vs 7.0 +/- 9.2; P = 0.029) and OAB-V8 scores (22.8 +/- 15.8vs 9.1 +/- 7.8; P = 0.008), and worse neurological impairment (mean EDSS 5.9 +/- 1.8 vs 4.5 +/- 1.5; P = 0.027). Conclusions: Most patients with NMO-SD have LUTS and voiding dysfunction, with DSD and DO as the main urodynamic findings. The severity of the neurological disease is a predictive factor for the occurrence of voiding dysfunction and detrusor-sphincter dyssynergia. (C) 2014 Wiley Periodicals, Inc.
- Diagnostic accuracy of concentric needle jitter in myasthenia: Prospective study(2017) MACHADO, Flavia C. N.; KOUYOUMDJIAN, Joao A.; MARCHIORI, Paulo E.Introduction: The aim of this study was to estimate jitter parameters in the orbicularis oculi muscle using a concentric needle electrode (CNE) in patients with myasthenia gravis (MG) and to determine its diagnostic accuracy for jitter analysis (CNEMG-jitter). Methods: CNEMG-jitter was performed in 20 healthy subjects and 33MG patients using the voluntary contraction technique. Receiver operating characteristic (ROC) curves were constructed to determine cut-off points with the best sensitivity/specificity combination for jitter analysis. Results: CNEMG-jitter yielded high positivity rates for ocular MG (92.3%) and generalized MG (100%). The ROC curve cut-off point that provided the highest sensitivity without false positives was 24.7 s for mean jitter and 33.1 s for the 18th highest value. Sensitivity was 93.9% for both parameters. Diagnostic accuracy of CNEMG-jitter was>96%. Conclusions: CNEMG-jitter yielded high sensitivity and specificity rates. Our reference values were lower than previously published values, possibly due to less technical variation between the different recordings. Muscle Nerve55: 190-194, 2017
- Multiple endocrine neoplasia type 1 presenting as refractory epilepsy and polyneuropathy - A case report(2012) PAIVA, Anderson Rodrigues Brandao de; CASTRO, Luiz Henrique Martins; RODRIGUES JR., Waldyr; PASSARELLI, Valmir; JORGE, Carmen Lisa; BROTTO, Mario Wilson I.; HIRATA, Maria Teresa Alves; MARCHIORI, Paulo EuripedesHypoglycemia is a well recognized cause of acute symptomatic seizures. The fact that hypoglycemia can cause peripheral neuropathy is less appreciated. We describe a case of insulinoma associated peripheral neuropathy. A 17 year-old previously healthy man was referred for investigation of refractory epilepsy. A history of recurrent seizures, slowly progressive weakness of his feet and hands, and weight gain was obtained. Physical examination showed signs of a chronic sensory-motor polyneuropathy. He was diagnosed with insulinoma and primary hyperparathyroidism, characterizing multiple endocrine neoplasia, type 1 syndrome. Cases of insulinoma associated peripheral neuropathy are very rare. The more characteristic clinical picture appears to be distal weakness, worse in the intrinsic hand and feet muscles, and no or mild sensory signs. Peripheral nervous system symptoms may not completely resolve, despite removal of the cause of hyperinsulinism/hypoglycemia and full reversion of central nervous system symptoms. Mechanisms underlying hypoglycemic neuropathy are still poorly understood.
- Serial and prolonged EEG monitoring in anti-N-Methyl-D-Aspartate receptor encephalitis(2014) SILVA-JUNIOR, Francisco Pereira da; CASTRO, Luiz Henrique Martins; ANDRADE, Joaquina Queiroz; BASTOS, Carla Guimaraes; MOREIRA, Camila Hobi; VALERIO, Rosa Maria Figueiredo; JORGE, Carmen Lisa; MARCHIORI, Paulo Euripedes; NITRINI, Ricardo; GARZON, ElianaObjective: To describe serial electroencephalographic (EEG) findings of three patients with anti-NMDAR encephalitis. Methods: Three women (age 15-34 years) with confirmed anti-NMDAR encephalitis underwent serial EEG recordings. Continuous EEG for 72 h was performed in one case and 3-day video-EEG monitoring was obtained in two cases. Results: Generalized rhythmic delta activity (GRDA) was found in all patients. GRDA persisted for hours, but was not continuous on a 24-h EEG recording, disclosed no frequency, voltage or field evolution, and was not seen on the first EEG of two patients. Extreme delta brush was noted in two patients who presented more severe disease. One patient presented seizures, which were electrographically and clinically different from the GRDA pattern and from dyskinetic movements. Conclusions: Serial or continuous EEG may be necessary to detect GRDA in anti-NMDAR patients. To avoid unnecessary treatment, this pattern should not be interpreted as indicative of ictal activity, unless there is evidence of its ictal nature. Significance: Our findings may contribute to the diagnosis of anti-NMDAR encephalitis in cases with characteristic clinical picture.
- Endodermal cyst of the third cranial nerve(2012) FIGUEIREDO, Eberval G.; STERMAN NETO, Hugo; MARCHIORI, Paulo E.; ROSEMBERG, Sergio; TEIXEIRA, Manoel J.
- Clinical variability of early-onset congenital myasthenic syndrome due to biallelic RAPSN mutations in Brazil(2018) ESTEPHAN, Eduardo de Paula; ZAMBON, Antonio Alberto; MARCHIORI, Paulo Euripedes; SILVA, Andre Macedo Serafim da; CALDAS, Vitor Marques; MORENO, Cristiane Araujo Martins; REED, Umbertina Conti; HORVATH, Rita; TOPF, Ana; LOCHMUELLER, Hanns; ZANOTELI, EdmarMutations in RAPSN are an important cause of congenital myasthenic syndrome (CMS), leading to endplate acetylcholine receptor deficiency. We present three RAPSN earlyonset CMS patients (from a Brazilian cohort of 61 CMS patients). Patient 1 and patient 2 harbor the mutation p.N88K in homozygosity, while patient 3 harbors p.N88K in compound heterozygosity with another pathogenic variant (p.V165M; c.493G > A). At onset, patient 3 presented with more severe symptoms compared to the other two, showing generalized weakness and repeated episodes of respiratory failure in the first years of life. During adolescence, she became gradually less symptomatic and does not require medication anymore, presenting better long-term outcomes than patients 1 and 2. This case series illustrates the variability of RAPSN earlyonset CMS, with patient 3, despite severe onset, revealing an almost complete reversal of myasthenic symptoms, not limited to apneic episodes. Moreover, it suggests that RAPSN CMS may be underdiagnosed in nonEuropean countries.