Evaluation of neuroinflammation in X-linked adrenoleukodystrophy

We present findings of ¹¹C-[R]-PK11195 positron emission tomography in a child with X-linked adrenoleukodystrophy. ¹¹C-[R]- PK11195 is a radioligand with a high and specific affinity for peripheral benzodiazepine receptors, expressed by activated microglia in cases of neuroinflammation, and therefore it is applicable to the in vivo detection of neuroinflammation with positron emission tomography. ¹¹C-[R]-PK11195 positron emission tomography demonstrated increased tracer binding in the occipital, parietal, and posterior temporal white matter, in the genu of the corpus callosum, the bilateral posterior thalami, most of the posterior limb of the internal capsule, the bilateral cerebral peduncles, and the brainstem, indicating underlying neuroinflammation. The rest of the brain, including the cerebral cortices and cerebellum, exhibited minimal ¹¹C-[R]-PK11195 binding. Our findings indicate significant neuroinflammation associated with white matter destruction in X-linked adrenoleukodystrophy, which can be visualized in vivo with an ¹¹C-[R]-PK11195 positron emission tomography scan. ¹¹C-[R]-PK11195 positron emission tomography may also help evaluate the inflammatory burden and follow-up of the disease evolution. This technique may be particularly useful for evaluating treatment response, which is not easy with other imaging modalities, after white matter is significantly and extensively damaged.