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CASE REPORT: SUBACUTE SCLEROSING PANENCEPHALITIS
Last modified: 2014-04-03
Abstract
SUMMARY : Subacute sclerosing panencephalitis (PES) is a progressive inflammatory disease of the central nervous system related persistent infection and abnormal measles virus (Paramyxovirus), presenting high mortality and poor prognosis1. It affects primarily children and young adults, in 50 % of cases, infection history Paramyxovirus below 2 years of age2; being age a risk factor for ESP, due to immaturity of the imunne system3. The incidence is 1-4:100.000 cases1,7. The disease progresses slowly with progressively divided in 4 stages2.
OBJECTIVE : Present a case diagnosed with Subacute Sclerosing Subacute and compare the data collected with the current scientific literature.
CASE REPORT : Patient, 16 years old female with history of social isolation and difficulty in school since October 2011; evolved with changing behavior, frequent falls, myoclonus, difficulty speaking and perform their daily activities. Reported Measles box with 1 year of age without complications. Neurological examination presents poor contact lack of understanding; Walking with broad-based, with momentary stoppages due to periodic myoclonus associated with facial expression. The EEG showed characteristic for this disease stroke, as well as antibodies and hypergammaglobulinemia in the CSF.
CONCLUSION : The diagnosis is made by the previous history of Measles virus infection, presence of complexes Radermacker and anti-measles antibodies in the blood and cerebrospinal fluid; histopathological findings in brain biopsy or autópsia5. It is a disease with fatal course, and no treatment.
OBJECTIVE : Present a case diagnosed with Subacute Sclerosing Subacute and compare the data collected with the current scientific literature.
CASE REPORT : Patient, 16 years old female with history of social isolation and difficulty in school since October 2011; evolved with changing behavior, frequent falls, myoclonus, difficulty speaking and perform their daily activities. Reported Measles box with 1 year of age without complications. Neurological examination presents poor contact lack of understanding; Walking with broad-based, with momentary stoppages due to periodic myoclonus associated with facial expression. The EEG showed characteristic for this disease stroke, as well as antibodies and hypergammaglobulinemia in the CSF.
CONCLUSION : The diagnosis is made by the previous history of Measles virus infection, presence of complexes Radermacker and anti-measles antibodies in the blood and cerebrospinal fluid; histopathological findings in brain biopsy or autópsia5. It is a disease with fatal course, and no treatment.
Keywords
measles; panencephalitis; myoclonus
References
1- M M Cruzeiro; T C Vale; L A Pires; G M Franco. Panencefalite esclerosante subaguda: relato de caso atípico. Arq. Neuro-Psiquiatr. v.65 n.4a São Paulo dez. 2007
2- S Pinto; M. M. Campos; M. T. L. Pimenta. Panencefalite Esclerosante Subaguda: de novo uma velha doença. Acta Pediatr Port 2006; 2(37): 62-4
3- Y Abe,K Hashimoto, M Watanabe, S Ohara, M Sato, Y Kawasaki, Y Hashimoto, M Hosoyaa. Characteristics of Viruses Derived from Nude Mice with Persistent Measles Virus Infection. J Virol. 2013 April; 87(8): 4170–4175.
4- A Aggarwal, MD. Subacute Sclerosing Panencephalitis Presenting as Mania. The Journal of Neuropsychiatry and Clinical Neurosciences 2011;23:E15-E16.
5- A. Diament. Neuroviroses. Neurologia Infantil 5a edição 2010; 53: 1020-22
6- MK Simmonds, DWG Brown, L Jin. Measles viral load may reflect SSPE disease progression. Virol J. 2006; 3: 49.
7- KJ Almeida, SMD Brucki, MIS Duarte, CAG P, S Rosemberg, R Nitrini. Basal ganglia lesions in subacute sclerosing panencephalitis. Dement Neuropsychol 2012 December;6(4):286-289.
8- S Martins, L Ribeiro, M Fonte, R Chorão. Caso electroencefalográfico. Nascer e Crescer v.20 n.1 Porto mar. 2011.
2- S Pinto; M. M. Campos; M. T. L. Pimenta. Panencefalite Esclerosante Subaguda: de novo uma velha doença. Acta Pediatr Port 2006; 2(37): 62-4
3- Y Abe,K Hashimoto, M Watanabe, S Ohara, M Sato, Y Kawasaki, Y Hashimoto, M Hosoyaa. Characteristics of Viruses Derived from Nude Mice with Persistent Measles Virus Infection. J Virol. 2013 April; 87(8): 4170–4175.
4- A Aggarwal, MD. Subacute Sclerosing Panencephalitis Presenting as Mania. The Journal of Neuropsychiatry and Clinical Neurosciences 2011;23:E15-E16.
5- A. Diament. Neuroviroses. Neurologia Infantil 5a edição 2010; 53: 1020-22
6- MK Simmonds, DWG Brown, L Jin. Measles viral load may reflect SSPE disease progression. Virol J. 2006; 3: 49.
7- KJ Almeida, SMD Brucki, MIS Duarte, CAG P, S Rosemberg, R Nitrini. Basal ganglia lesions in subacute sclerosing panencephalitis. Dement Neuropsychol 2012 December;6(4):286-289.
8- S Martins, L Ribeiro, M Fonte, R Chorão. Caso electroencefalográfico. Nascer e Crescer v.20 n.1 Porto mar. 2011.
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