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NIMHANS, Bengaluru (CJD registry, neuropathology and brain bank)

Bengaluru neuroscience institute that hosts India's longest-running CJD registry (69 cases in the 1968 to 1997 period, 85 recorded to 2005) and built an in-house CSF 14-3-3 assay. RT-QuIC and PRNP testing are not known to be offered there.

Key facts

  • NIMHANS (Bengaluru) CJD registry reported 69 cases in 30 years (Shankar et al.); sporadic CJD is the most common form in India and probably under-reported; familial CJD very rare; none of the 2011 to 2015 patients had histopathology or CSF 14-3-3.
  • Lists NIMHANS neuropathology work by S. K. Shankar and Anita Mahadevan including prion disease pathobiology.
  • The CJD registry at NIMHANS recorded 69 cases from across India over 1968 to 1997, and an eastern India series states that 85 CJD cases were recorded in the national registry to September 2005.
  • NIMHANS researchers developed an in-house dot blot assay for CSF 14-3-3 using antibodies to a recombinant core protein: about a ten-fold difference in CSF levels between CJD cases (N=50) and disease controls (N=70), described as economical and sensitive.
  • The authors report that increased awareness had sent more possible and probable CJD cases to the registry seeking diagnosis and a tailored care plan.
  • Independent Indian reviews state that RT-QuIC is not available in India to the authors' knowledge and that prion protein genotyping facilities are lacking.
  • A tertiary-centre series (December 2011 to October 2015) of probable CJD cases had no brain or tonsil histology and no CSF 14-3-3, showing how often diagnosis relies on MRI and EEG alone; flupirtine was used for symptom control.
  • An eastern India series of probable sporadic CJD reports eight patients dying within a mean of 4.56 months from onset.

Timeline

  1. 1968Registry period begins.
  2. 1997End of the 69-case registry period (1968 to 1997).
  3. 2005Registry records 85 CJD cases (as cited in 2013).
  4. 2016Dot blot 14-3-3 assay published and tertiary-centre CJD series reported.
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