Variant Creutzfeldt-Jacob Disease: A Review of 150 Cases

Caronna, John J.
July 2011
Neurology Alert;Jul2011, Vol. 29 Issue 11, p82
IN THE MID-1990S, VARIANT CREUTZFELDT-JAKOB DISEASE (vCJD) was recognized as an example of bovine-to-human spread of bovine spongiform encephalopathy (mad cow disease) by ingestion of meat products infected with the pathogenic prion protein. At present, more than 200 probable cases of vCJD have been identified in 11 countries. In all patients, infection occurred either during residence in the UK or through contact with meat products, animals, or animal products exported from the UK. Four cases of vCJD transmitted by blood transfusion have been reported. The authors undertook a retrospective study of the first 150 cases of vCJD identified in the UK between 1995 and 2005. They systematically analyzed symptoms, signs, and the diagnostic process with the aim of achieving an earlier diagnosis in future cases of vCJD. In this series, individuals with vCJD rarely sought medical attention early in the clinical course. The median time from onset to first medical contact was 2.5 months (mean 3.4 months). Early clinical features of vCJD were subtle and of insidious onset. Non-specific psychiatric symptoms -- anxiety, irritability, social withdrawal, agitation, and insomnia -- dominated the early clinical course. Neurologic features developed early in only a minority of patients, were usually non-specific symptoms such as painful dysesthesias, and were not associated with an abnormal neurological examination. Patients with neurological features or a combination of neurological and psychiatric symptoms presented earlier to a medical practitioner, in comparison with those who had only psychiatric features: neurological onset, 2 months; psychiatric onset, 3.3 months; and combined neurologic and psychiatric symptoms, 1.7 months. At first medical contact, vCJD was not considered as a possible diagnosis in any patient. The mean time from onset to neurological referral was 7.4 months. In 147 of 150 cases, the mean time from onset to suspected diagnosis of vCJD was 8.9 months and to confirmed diagnosis by current diagnostic criteria (see article) was 10.5 months. In two older patients aged 68 and 74 years not diagnosed in life, vCJD was identified at autopsy. In one case detailed information was not available. Thirty-eight patients diagnosed within 6 months of onset had both a more rapidly progressive course and earlier objective neurologic signs, which probably led to the earlier diagnosis. Brain MRI supported the clinical diagnosis of vCJD in 143 of 150 patients. The majority of the negative MRI scans did not include the fluid attenuated inversion recovery (FLAIR) sequence that is most sensitive for identifying hyperintensity of the pulvinar and thalamus that is characteristic of vCJD. The authors conclude that achieving an early diagnosis of vCJD remains a challenge because of the insidious onset of illness, and the non-specific and mainly psychiatric early clinical features in the majority of cases.



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