Quinacrine treatment trial for sporadic Creutzfeldt-Jakob disease.

Objective: To determine whether oral quinacrine increases survival in sporadic Creutzfeldt-Jakob disease (sCJD).

Methods: This NIH/National Institute on Aging-funded, double-blinded, placebo-controlled, stratified randomization treatment trial was conducted at the University of California, San Francisco from February 2005 through May 2009 (ClinicalTrials.gov, NCT00183092).

Differential diagnosis of Jakob-Creutzfeldt disease.

Objectives: To identify the misdiagnoses of patients with sporadic Jakob-Creutzfeldt disease (sCJD) during the course of their disease and determine which medical specialties saw patients with sCJD prior to the correct diagnosis being made and at what point in the disease course a correct diagnosis was made.

Design: Retrospective medical record review.

Setting: A specialty referral center of a tertiary academic medical center.

Voltage-gated potassium channel autoimmunity mimicking creutzfeldt-jakob disease.

Background: Rapidly progressive dementia has a variety of causes, including Creutzfeldt-Jakob disease (CJD) and neuronal voltage-gated potassium channel (VGKC) autoantibody-associated encephalopathy.

Objective: To describe patients thought initially to have CJD but found subsequently to have immunotherapy-responsive VGKC autoimmunity.

Design: Observational, prospective case series.

Rapidly progressive dementia.

In contrast with more common dementing conditions that typically develop over years, rapidly progressive dementias can develop subacutely over months, weeks, or even days and be quickly fatal. Because many rapidly progressive dementias are treatable, it is paramount to evaluate and diagnose these patients quickly. This review summarizes recent advances in the understanding of the major categories of RPD and outlines efficient approaches to the diagnosis of the various neurodegenerative, toxic-metabolic, infectious, autoimmune, neoplastic, and other conditions that may progress rapidly.

Creutzfeldt-Jakob disease in recipients of corneal transplants.

Purpose: Creutzfeldt-Jakob disease (CJD) transmission has been documented to occur from the use of corneal grafts. We report 4 cases of CJD with a history of corneal transplantation and assess the frequency of coincidental CJD among corneal transplant recipients.

Methods: Medical records and eye bank documents were reviewed.

A case of enteroviral meningoencephalitis presenting as rapidly progressive dementia.

Background: A 70-year-old immunocompetent male presented to a memory disorders clinic with a 7-month illness that had begun with somatic complaints including transient right temporal head pain, left buttock pain, and right conjunctival injection. About 3 months after the first signs of illness, the patient had begun to develop insidious cognitive and behavioral decline, which progressed most rapidly in the 2 months before presentation. An assessment completed during hospitalization for intermittent fevers and confusion had not revealed an infectious etiology, although mild pleocytosis in the cerebrospinal fluid had been noted.

Rapidly progressive dementia.

Rapidly progressive dementias (RPDs) are neurologic conditions that develop subacutely over weeks to months or, rarely, acutely over days. In contrast to most dementing conditions that take years to progress to death, RPD quickly can be fatal. It is critical to evaluate patients who have RPD without delay, usually in a hospital setting, as they may have a treatable condition.

Anti-GAD antibody cerebellar ataxia mimicking Creutzfeldt-Jakob disease.

In a patient with a rapidly progressive neurological condition with ataxia and cognitive complaints, Creutzfeldt-Jakob disease (CJD) is often high in the differential, particularly when there is an elevated CSF 14-3-3 protein level. We present a case of anti-glutamic acid decarboxylase antibody (anti-GAD65) positive cerebellar ataxia associated with cognitive complaints and elevated CSF 14-3-3 protein.