Creutzfeldt–Jakob disease or CJD, (Hans Gerhard Creutzfeldt, 1885 - 1964, German psychiatrist and Alfons Maria Jakob, 1884 - 1931, German psychiatrist) (also called subacute spongiform encephalopathy), is a rare, well characterized clinicopathological entity defined by a rapidly progressing dementia associated with pathognomonic vacuolation in the cortex giving a microscopic appearance of spongiform change. Both humans and animals may be affected.
The overall incidence is estimated to be as low as 1 in 1,000,000 people, with no discernible pattern of exposure in the patient population. The aetiopathology of the disease is still obscure. Cases of transmission by corneal transplantation and deep electrode implants have been reported. The similarities with another disease confined to a tribe of the Eastern highlands of Papua New Guinea (Kuru) transmitted by cannibalism have led to he suggestion that the disease is transmitted through proteinaceous infectious particles containing no nucleic acids (prions).
Clinically the disease may be suspected in the case of a rapidly progressive dementia associated with upper motor neuron dysfunction and myoclonus. Characteristic EEG changes, such as periodic synchronous discharge, aid the diagnosis, despite nonspecific findings for this disease. The definite diagnosis of Creutzfeldt -Jakob disease is based on histopathological findings: typical changes are vacuolation and loss of neurons with hypertrophy and proliferation of glial cells, most pronounced in the cerebral cortex. The pathological lesions are typically most severe in or even confined to grey matter, at least early in the course of the disease. Loss of myelin appears to be secondary to degeneration of neurons. There is usually no inflammation.
Neuroimaging studies provide supporting information for the diagnosis. CT scan does not usually show any abnormality except for brain atrophy. MR imaging is more sensitive than CT scan in detecting parenchymal changes, displaying focal abnormal T2 hyperintensities in the cerebral cortex and basal ganglia that probably reflects areas of gliosis. Involvement of the cerebral white matter has been reported by a few authors. In general, basal ganglia abnormalities occur earlier, though most of the abnormalities on MR are seen in the late stages of Creutzfeldt - Jakob disease.
The disease is invariably fatal with an average duration of a few months although survivals as long as several years have been reported.
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