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Zhao et al. Developments in auxiliary examination of CJD

are still the gold standards for CJD diagnosis. The method which could reverse the disease course does
classic pathological features of CJD are spongiform not exist currently. Prior reports indicate that intensive
degeneration, astrocytes gliosis and nerve cell life-sustaining treatments, such as assisted breathing
loss. But the percentage of pathological diagnosis with ventilator and nutritional support, can prolong
among CJD patients is very low. Some surgical and the patients’ lives, but could not prevent death. [68]
pathology departments are not active to do autopsy The causative agent, prions, are not completely
or biopsy because of the infectious and incurable inactivated by exposure to high temperature,
characteristic. And the sterilization methods for ultraviolet and ionizing radiation, or chemicals that
contaminated instruments have not been promoted are effective against common viruses. [69] In order to
in many countries. Furthermore, many people refuse prevent transmission of this devastating disease and
biopsy partly due to fear of invasive examination or reduce the risk to public health, earlier and accurate
refuse autopsy because of traditional cultural beliefs. clinic diagnosis of CJD are critically important.
Less invasive biopsy of the olfactory mucosa or Auxiliary examination plays a significant role in CJD
skeletal muscle and other novel ultrasensitive seeding diagnosis. EEG is most widely used as the traditional
assays based on the amplified detection of PrP, such examination method in clinic and DWI has the highest
as real-time quaking-induced conversion, have been sensitivity and specificity. The value of 14-3-3 protein
developed, but have not been extensively used in testing remains controversial. The use of MRS for CJD
clinic. [61,62] Thus the diagnosis of CJD based on clinical diagnosis in clinic is limited. Detection of tau protein,
manifestations and non-invasive examinations (14- DTI and PET have considerable potential in the
3-3 protein testing, EEG, DWI, PET, etc.) remains differential diagnosis of different rapidly progressive
significant.
dementia, especially in the situation of overlapping
The clinical diagnostic criteria for CJD were clinical manifestations. In addition, DWI and DTI may
first proposed in 1979, using a combination of be useful to assess pathological changes of CJD.
clinical features and EEG. [63] 14-3-3 protein test The abnormal results of auxiliary examination, such
was assessed in subsequent years and added as the region of PSWCs, the abnormal hyperintensity
to the diagnostic criteria later. The World Health area of DWI and the low metabolism area of PET,
Organization (WHO) formulated detailed diagnostic are consistent with the symptoms and the area of
guidelines in 1998, including clinic symptom, PSWCs pathologic change. Multiple combined auxiliary
of EEG, 14-3-3 protein and exclusion diagnosis. examination methods may increase the accuracy
According to the diagnostic guidelines of WHO, of diagnosis and differential diagnosis among CJD
14-3-3 protein can provide a possible-to-probable patients. Further investigation into potential auxiliary
diagnosis. [31,64] Abnormal findings on MRI were examination methods for earlier and more accurate
mentioned at that time but without high attention. In diagnosis of CJD are certainly needed.
the manual for surveillance of human transmissible
spongiform encephalopathies 2003, WHO described Authors’ contributions
the diagnostic criteria for the four CJD subtypes Concept and design: J.T. Zhang
(sCJD, gCJD, iCJD and vCJD, separately) in more Data analysis, manuscript preparation and editing: W.
detail. And the bilateral symmetrical pulvinar high Zhao
signal on MRI brain scan was incorporated into Literature search: J.J. Jiang
the diagnostic criteria for vCJD. [30] As research
has progressed, MRI has played an increasingly Financial support and sponsorship
significant role in CJD diagnosis. [40,60,65,66] The clinical This study received financial aid from the first batch
criteria for the diagnosis of CJD have been revised of fund that supports clinical and scientific research
to include abnormal hyperintensities on DWI based approved by Chinese People’s Liberation Army General
in part on key studies (Zerr et al., [18] Centers for Hospital in 2016. Project No.: 2016FC-CXYY-1002.
Disease Control and Prevention [67] ). Vitali et al. [41] also
proposed detailed MRI diagnostic criteria. Although Conflicts of interest
DTI and PET have received much attention, they are There are no conflicts of interest.
not readily used in diagnosis. In fact, PET is more
often used for the exclusion diagnosis. Patient consent
No patients were involved.
CONCLUSION
Ethics approval
CJD is infective, untreatable and fatal. The treatment Not applicable.
Neuroimmunology and Neuroinflammation ¦ Volume 4 ¦ July 21, 2017 141