Manifestations Of Creutzfeldt-Jakob Disease Research Articles

Alice in Wonderland Syndrome as a Manifestation of Creutzfeldt-Jakob Disease.

Alice in Wonderland Syndrome as a Manifestation of Creutzfeldt-Jakob Disease.

Psychoses as the presenting manifestation of Creutzfeldt-Jakob disease in an elderly male

The differential diagnosis of acute psychosis includes delirium, dementia and primary psychiatric disorders. A comprehensive medical evaluation is crucial to make the proper diagnosis. We report the case of a healthy elderly man who presents with acute psychosis. We describe our diagnostic evaluation based on the patient's history and physical examination, which identified Creutzfeldt-Jakob disease. Avoiding diagnostic error in patients who face such a rapidly progressive and catastrophic illness is paramount.

Diffusion-weighted magnetic resonance imaging in diagnosis of Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD), a rare disease, is uncharacterized by computed tomography (CT) and magnetic resonance imaging (MRI). This study was aimed to evaluate the diffusion-weighted MRI (DWI) manifestations of CJD and to discuss their diagnostic value. The findings of T(1)-weighted MRI (T(1)WI), T(2)-weighted MRI (T(2)WI), DWI and post-contrast MRI in 5 patients (3 patients with biopsy-proven CJD and 2 patients with clinically-proven CJD) were retrospectively analyzed in this study. Four out of the 5 patients had cerebral atrophy of various degrees. One patient showed symmetric high signal intensity at the bilateral globus pallidus and the head of the caudate nucleus, with very high signal in the cerebral cortex on the DWI. This patient only had symmetric slightly high signal at the bilateral globus pallidus and putamen on T(2)WI. One patient had high signal intensity at the basal ganglia and cerebral cortex on DWI, but abnormal T(2) signal intensity at the bilateral paraventricular white matter on MRI. Two patients presented with widely gyri-like high signal intensity at the cortex on DWI, but routine MRI showed bilateral paraventricular long T(2) signal intensity in 1 patient and no abnormal findings in another. No abnormalities were shown by both routine MRI and DWI in the last patient. DWI is more sensitive than its conventional counterpart in the depiction of CJD. DWI is more sensitive to detect cortical abnormal signal intensity in CJD not detected by T(2)WI.

The psychiatric manifestation of Creutzfeldt-Jakob disease.

Creutzfeldt-Jakob disease (CJD) is considered to be very rare in the population, and the psychiatric manifestation of the disease even rarer with only one report in the past few years in Taiwan. To clarify whether the psychiatric manifestation of CJD is really rare or whether it is neglected in Taiwan, the authors reviewed the discharge notes of patients who had been admitted to a neurological unit in the past 15 years and conducted a chart review of the patients of CJD supported by the clinical courses, EEG finding and brain biopsies. An inquiry was made by telephoning their families to follow up their condition after discharge. Five of the 8 cases with CJD had psychiatric symptoms including changes of mood, thought, behavior and perception during their course of illness. Four cases had been sent to the psychiatric unit and received treatment under several kinds of psychiatric diagnoses. Two patients had been admitted to the psychiatric unit and one had received electroconvulsive treatment. Two of the patients had been suspected to be the victims of neuroleptic malignant syndrome. It is likely that it is psychiatrists who will meet CJD patients first in the early stages of disease. CJD should be kept in mind and EEGs with detailed neurological checkups should be completed, if the cognitive functions of the patients with unusual neurological symptoms deteriorate quickly and their psychiatric symptoms fail to respond to any treatment.

Homonymous Field Defect as the First Manifestation of Creutzfeldt-Jakob Disease

Purpose Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder. Methods We examined three patients who initially complained of a nonspecific, insidious visual disturbance. Results The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease. Conclusion At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.

Homonymous Field Defect as the First Manifestation of Creutzfeldt-Jakob Disease

Although in the early stage of Creutzfeldt-Jakob disease most patients have obvious dementia, we found that the disease can be diagnosed in patients solely on the basis of a visual system disorder. We examined three patients who initially complained of a nonspecific, insidious visual disturbance. The three patients were found to have Creutzfeldt-Jakob disease, confirmed by histopathologic analysis. Each patient had a homonymous hemianopsia on the first neuro-ophthalmologic examination. The initial neurologic, neuroimaging, and electrophysiologic examinations were not conclusive. The subsequent rapid deterioration in the neurologic status, including dementia and typical electroencephalographic changes, was suggestive of Creutzfeldt-Jakob disease. At onset of Creutzfeldt-Jakob disease, patients may have a homonymous hemianopsia despite normal results of magnetic resonance imaging of the brain and neurologic examination.

Creutzfeldt-Jakob Disease with Mixed Transcortical Aphasia: Insights into Echolalia

Aphasia is a common manifestation of Creutzfeldt-Jakob disease (CJD), and investigation of the linguistic disorders of CJD patients may provide insights into the neurobiological mechanisms of language and aphasia. We report an autopsy-confirmed case of CJD in which the presenting symptom was change in language abilities. The patient ultimately evidenced mixed transcortical aphasia (MTA) with echolalia. Disruption of frontal-subcortical circuits with environmental dependency accounts for the symptoms in MTA, including intact repetition and echolalia. Observation in this patient and a review of the literature suggest that frontal-subcortical circuit dysfunction may contribute to the syndrome of echolalia. This hypothesis offers an alternative explanation to “isolation” of the speech area as the cause of MTA.