Cerebral blood flow in dystonia due to pantothenate kinase-associated neurodegeneration

Abstract

Background and purpose: The aim of this study was to look for deviations of cerebral perfusion in patients suffering from pantothenate kinase-associated neurodegeneration, where the globus pallidus is affected by severe accumulation of iron.

Material and methods: Under resting conditions, cerebral blood flow was measured by the magnetic resonance imaging technique of arterial spin labelling in cortical areas and basal ganglia in eight pantothenate kinase-associated neurodegeneration patients and 14 healthy age-matched control subjects and correlated to T2* time of these areas and - in patients - to clinical parameters.

Results: Despite highly significant differences of T2* time of the globus pallidus (20 vs 39 ms, p < 0.001), perfusion values of this nucleus were nearly identical in both groups (32 ± 3.3 vs 31 ± 4.0 ml/min/100 g) as well as in total brain gray matter (both 62 ± 6.7 resp. ±10.3 ml/min/100 g), putamen (41 ± 5.4 vs 40 ± 6.1 ml/min/100 g), in selected cortical regions, and the cerebellum. Correlations between perfusion and T2* time to clinical data did not reach significance (p > 0.05).

Conclusion: The absence of any obvious deviations of perfusion in the group of patients during a resting condition does not support the view that (non-functional) vascular pathology is a major pathogenic factor in pantothenate kinase-associated neurodegeneration in the younger age group. The findings underline the value of the arterial spin technique to measure cerebral blood flow in areas of disturbed susceptibility.

Keywords: Pantothenate kinase-associated neurodegeneration; cerebral blood flow; globus pallidus; tiger’s eye.

Figure 1.

Original (not co-registered) T2W images showing the “tiger’s eye” (left) and cerebral blood flow (CBF) maps with regions of interest (ROIs) covering putamen (middle) and globus pallidus (right) in axial (upper row) and coronal projection (lower row).

Figure 2.

Correlation of cerebral blood flow (CBF) of gray matter (GM) (upper image), putamen (middle image), and globus pallidus (lower image) to age in patients (squares, solid lines) and controls (crosses, broken lines).