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1.
Rev Clin Esp ; 208(4): 193-6, 2008 Apr.
Artigo em Espanhol | MEDLINE | ID: mdl-18381004

RESUMO

Creutzfeldt-Jakob disease is a prion spongiform encephalopathy that causes a rapidly progressive dementia whose neuropathological features are neuronal loss, proliferation of glial cells, and the presence of small vacuoles, which produces a spongiform appearance. Two cases detected in the year 2005 in the <> Hospital are presented. The first one is the case of a man who presented with intense malaise and weight loss, developing dementia in 4 months. The second one was a woman who presented with diplopia, headache and gait disturbances who developed severe neurological impairment with dementia in three months. In both cases the 14.3.3 protein in the cerebrospinal fluid was positive and the EEG showed a characteristic pattern. The genetic studies were negative and both were homozygous for methionine at codon 129. Given that the incidence of this disease in Castilla la Mancha is 0.6 cases/100000 inhabitants/year, the detection of 2 cases in less than a year in Talavera de la Reina (whose population is 150,000) surprised us, leading us to intensify surveillance.


Assuntos
Síndrome de Creutzfeldt-Jakob/diagnóstico , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Espanha
2.
Rev. clín. esp. (Ed. impr.) ; 208(4): 193-196, abr. 2008. ilus, tab
Artigo em Es | IBECS | ID: ibc-63894

RESUMO

La enfermedad de Creutzfeldt-Jakob (ECJ) es una encefalopatía espongiforme producida por priones que provoca una demencia progresiva cuyas características neuropatológicas son la pérdida neuronal, la proliferación de células gliales y las vacuolas, lo que origina el aspecto de esponja. Se presentan dos casos detectados en el año 2005 en el Hospital Nuestra Señora del Prado. El primero es el de un hombre que comenzó con intensa astenia y cuadro constitucional, desarrollando una demencia en 4 meses; y el segundo caso es el de una mujer que comenzó con diplopía, cefalea y alteraciones de la marcha, desarrollando un deterioro neurológico con demencia en tres meses. En ambos casos la proteína 14.3.3 del líquido cefalorraquídeo fue positiva y el electroencefalograma mostró un patrón característico. Los estudios genéticos fueron negativos, y ambos presentaban polimorfismo del codón 129, siendo homocigotos para la metionina. Nos ha sorprendido la aparición de dos casos en menos de un año en Talavera de la Reina y nos ha hecho aumentar la vigilancia


Creutzfeldt-Jakob disease is a prion spongiform encephalopathy that causes a rapidly progressive dementia whose neuropathological features are neuronal loss, proliferation of glial cells, and the presence of small vacuoles, which produces a spongiform appearance. Two cases detected in the year 2005 in the «Nuestra Señora del Prado» Hospital are presented. The first one is the case of a man who presented with intense malaise and weight loss, developing dementia in 4 months. The second one was a woman who presented with diplopia, headache and gait disturbances who developed severe neurological impairment with dementia in three months. In both cases the 14.3.3 protein in the cerebrospinal fluid was positive and the EEG showed a characteristic pattern. The genetic studies were negative and both were homozygous for methionine at codon 129. Given that the incidence of this disease in Castilla la Mancha is 0.6 cases/100000 inhabitants/year, the detection of 2 cases in less than a year in Talavera de la Reina (whose population is 150,000) surprised us, leading us to intensify surveillance


Assuntos
Humanos , Masculino , Feminino , Pessoa de Meia-Idade , Idoso , Síndrome de Creutzfeldt-Jakob/diagnóstico , Síndrome de Creutzfeldt-Jakob/fisiopatologia , Doenças Priônicas/fisiopatologia , Metionina/genética , Mioclonia/etiologia
11.
Aten Primaria ; 10(8): 907-9, 1992 Nov 15.
Artigo em Espanhol | MEDLINE | ID: mdl-1457713

RESUMO

OBJECTIVE: To analyse certain factors which contribute to the existing backlog in specialised neurological care, such as: where the patient was referred from, the reason for the appointment, tests carried out and the final diagnosis. We used a random sample of patients. DESIGN: Retrospective study of patients referred from Primary Care, who were attended as out-patients in the Neurology clinic during 1991. SITE. Health Area 2 of the Autonomous Community of Madrid. MAIN MEASUREMENTS AND RESULTS: The average number of patients per doctor sent from each one of the seven Centres studied was 7.69, with outliers of between 2 and 21, with a standard deviation of 3.95 and a rate of 51.31%. The proportion of new patients to patients having follow-up checks was 2 to 3, respectively. The differences between the number of patients sent from the different centres were found in the less serious pathologies. CONCLUSIONS: We suggest that the criteria used by Primary Care professionals and specialists should be strengthened and unified. This was one of the factors which weighed most on the quality and backlog of care.


Assuntos
Doenças do Sistema Nervoso/terapia , Seguimentos , Humanos , Doenças do Sistema Nervoso/diagnóstico , Pacientes Ambulatoriais , Atenção Primária à Saúde , Encaminhamento e Consulta , Espanha , Fatores de Tempo
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