Glymphatic system in Pantothenase kinase associated neurodegeneration (PKAN)

Abstract

Purpose

To investigate if accumulation of iron in the globus pallidus as seen in patients suffering from Pantothenase Kinase Associated Neurodegeneration (PKAN), is related to damage of the cerebral glymphatic system.

Material and methods

In a group of 24 patients and an age-matched control group, functionality of the glymphatic system was assessed by the index of Analysis aLong the Perivascular Space (ALPS) from Diffusion Tensor Imaging data and correlated to the values of the T2∗ Times of the globus pallidus and the cerebral white matter measured by a Fast Field Echo sequence.

Results

In spite of the important reduction of the T2∗ Time of the globus pallidus, ALPS values of patients and controls were very similar and did not correlate to T2∗Time values in either group.

Conclusion

In spite of a prominent accumulation of iron in the globus pallidus of PKAN patients, which is responsible for the “eye-of-the-tiger” sign, our results are not compatible with malfunction of their glymphatic system. Obviously, increased iron content of globus pallidus alone does not necessarily indicate such sort of alteration.