Creutzfeldt-Jakob’s disease: case report with emphasis on the electroencephalographic features

Abstract

Creuzfeldt-Jakob’s Disease is a rare neurodegenerative disorder that is included among the transmissible spongiform encephalopathies. The clinical features are those of a rapid progressive dementia with mioclonic jerks, which progresses to death in less than one year. We report the case of a 79 years old woman, with initial complaints of vertigo, visual and gait compromise, with an initial brain MRI, EEG and CSF that had no specific features of CJD. After 12 days she was again admitted to the hospital with a worsening of her symptoms, along with global spasticity, inability to walk and a further deterioration of speech and swallowing. A new EEG disclosed periodic triphasic complexes, typical of the disease. CSF had a positive 14-3-3 protein. She later evolved with mioclonic jerks, coma, multiple infectious complications and, ultimately, death 45 days after her second admission. We focus on the role of the EEG as an adjuctive eletrophysiological tool for the presumptive in vivo diagnosis of the disease.

Doença de Creutzfeldt-Jakob, Variante de Heidenhain. Relato de Caso e Revisão do Tema

“…Um marcador de que deve ser solicitado em todo caso suspeito de DCJ é a proteína 14.3.3, com sensibilidade de cerca de 90%. Outro exame complementar que auxilia no diagnóstico é o EEG, com seu achado típico (complexo ponta-onda periódico) presente em 2/3 dos pacientes 4,5,8,11 .Devem ser investigadas outras possibilidades diagnósticas juntamente com a da DCJ, sendo necessária a exclusão de doenças paraneoplásicas e encefalites autoimunes, os principais diagnósticos diferenciais da doença.…”