OBJECTIVES: To describe specific aspects of the diagnostic process of transmissible spongiform @entity75 in Spain and to evaluate the 14-3-3 protein test in cerebrospinal fluid. METHODS: The annual pattern of diagnostic certainty as well as those of demand and results of biochemical and genetic studies were studied using two sets of @entity1 , those diagnosed for the 1993-1998 period, notified to a National @entity3605 Register (NCDJR), and those referred to the Tissue Bank for Neurological Research Laboratory (TBNRL). The 14-3-3 protein test was validated taking as a reference two clinical populations. RESULTS: Two-hundred and four @entity3605 cases were registered at the NCDJR: 39 out of them and 28 other @entity1 had been studied at the TBNRL. The proportion of definite @entity3605 cases decreased since 1996. Among those registered in 1997-1998, 35.5%, 36% and 20% had undergone 14-3-3 protein in LCR, histopathologic and genetic studies. The 14-3-3 test grave, for definite, @entity13895 as compared for @entity1 with other @entity74 , the following data: 12/13 sensitivity; 33/35 specificity; and 12/14 and 33/34 predictive values of positive and negative test. Two familial cases were diagnosed by identification of mutations in the TBNRL. CONCLUSIONS: The results suggest that: a) the diagnostic certainty of @entity3605 in Spain decreased due to a drop in autopsy rates; b) the 14-3-3 cerebrospinal fluid test has a high diagnostic value, and its use diffused rapid but incompletely; c) genetic studies are useful in some cases, and d) @entity3605 undereporting may be considerable. @entity3605 diagnosis and surveillance, are closely related and being consolidated in Spain.

[Diagnosis of transmissible spongiform XXXX in Spain. Population perspective].

@entity1 :: ('9606', 'Species') :: ['patients'] @entity75 :: ('MESH:D001927', 'Disease') :: ['encephalopathies'] @entity3605 :: ('MESH:D007562', 'Disease') :: ['Creutzfeldt-Jakob disease', 'Creutzfeldt-Jakob Disease'] @entity13895 :: ('MESH:C565143', 'Disease') :: ['sporadic Creutzfeldt-Jakob disease'] @entity74 :: ('MESH:D030342', 'Disease') :: ['dementing disorders']
@entity75 :: (MESH:D001927,Disease) :: ['encephalopathies']
