MNS: PET identifies neuroinflammation in children with NPC disease

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PET imaging with tracer C-11 PK-11195(PK) identified that children with Niemann Pick disease type C have an underlying neuroinflammation, according to a presentation at the Molecular Neuroimaging Symposium this week in Bethesda, Md.

Ajay Kumar, MD, and colleagues from the department of pediatrics and neurology at school of medicine, Wayne State University in Detroit, hypothesized that PET tracer C-11 PK-11195(PK) can be used for in vivo evaluation of activated microglial (resident macrophages of the brain and spinal cord) mediated neuroinflammation.

Niemann-Pick disease type-C (NPC) is a lysosomal storage disease caused by genetic mutations. Eight Niemann-Pick disease type-C (NPC) patients (age: 8.2 ± 3. 9 (4-16) years) and seven healthy adults (age: 27.4 ± 7.5 years) underwent dynamic PET imaging of brain after injection of 17 MBq/kg of PK in the study.

Kumar and colleagues calculated the binding potential (BP), a semi quantitative measure of the PET tracer PK, binding to its receptors in basal ganglia, thalamus and cerebellum.

BP was found to be significantly increased in the basal ganglia (0.22 vs. 0.14) and thalamus (0.42 vs. 0.3) in children with NPC compared to normal controls. This increase in BP, suggested increase in activated microglial cells in the basal ganglia and thalamus of children with Niemann Pick disease type C, suggesting underlying neuroinflammation, according to Kumar and colleagues.

Therefore, the authors concluded that PK PET may be used as a biomarker in this condition. “However, further prospective studies and neuropathologic correlations are required to confirm its value in monitoring progression and response to interventions," noted Kumar and colleagues.