Enfermedad de Creutzfeldt-Jakob: experiencia de 5 años en un hospital terciario de Chile / Creutzfeldt-Jakob disease, experience in 17 patients

Background: Creutzfeldt-Jakob disease (CJD) is a prion affection that typically produces a rapidly progressive dementia with different neurologic and extra-neurologic manifestations. Aim: To characterize clinical, imaging and electroencephalography findings in patients with a probable CJD. Patients and Methods: A case series study of patients admitted in the Neurology department at a public hospital, between 2014 and 2019. Demographic, clinical, imaging, and electroencephalographic data of patients with probable CJD were analyzed. Results: Seventeen patients aged 63 ± 11 years (53% women) with a probable CJD were gathered. The incidence was 4.7 cases/year per million inhabitants. Twenty four percent of patients had a family history of CJD. The median time between the onset of symptoms and the hospital admission was three months with a survival of four months. The most common clinical manifestations were an amnesic syndrome in 88%, myoclonus in 76%, frontal syndrome and ataxia in 71%. Brain MRI was abnormal in all patients. The preponderant finding was the involvement of the caudate nucleus in 82% of cases. In the EEG, 94% of patients had abnormalities. All had a theta-delta slowing as a base rhythm. The pseudo-periodic pattern was observed in the 29% and status epilepticus in 18%. Conclusions: In this group of patients we observed the heterogeneity of the clinical manifestations of the disease, the frequent imaging and electroencephalographic alterations and the short evolution time leading to death.

Afasia como debut de estado hiperosmolar hiperglicémico. Caso clínico / Aphasia as the presenting symptom of a hyperglycemic hyperosmolar state. Case report

Neurological manifestations such as seizures, disorders of consciousness and abnormal movements such as hemichorea and hemiballismus can be the presenting symptoms of hyperglycemic hyperosmolar states. Exceptionally, focal signs as hemiparesis or aphasia are described. We report a 66-year-old man, presenting with nonfluent aphasia and right subtle hemiparesis. The computed tomography, computed tomography angiography and brain magnetic resonance did not show acute ischemic lesions or obstruction of arterial vessels. The initial laboratory evaluation disclosed a blood glucose of 936 mg/dL, a plasma osmolality of 331 mOsm/Kg, and positive plasma ketones. After the treatment of hyperglycemia and hyperosmolality, focal symptoms subsided.