AJDRAJNR - American Journal of Neuroradiology

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Diffusion-Weighted and Conventional MR Imaging Findings of Neuroaxonal Dystrophy

R. Nuri Senera

a From the Department of Radiology, Ege University Hospital, Bornova, Izmir, Turkey



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FIG 1. Images obtained in a 5-year-old girl. (Her sibling, a 9-year-old girl, was similarly affected.)

A, T2-weighted image reveals characteristic hyperintensity of the cerebellum. The dentate nuclei have higher signal intensity.

B, Diffusion-weighted image obtained at a b value of 1000 s/mm2 appears normal.

C, ADC map (section identical to that in B) is abnormal. ADC values in the cerebellum are high and higher in the dentate nuclei (2.12 x 10–3 mm2/s) than the surrounding parenchyma (1.67 x 10–3 mm2/s); these all indicate elevated diffusion rates.

D, T2-weighted image reveals hyperintense lesions in the corticospinal tract.



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FIG 2. Images obtained in a 19-month-old male patient.

A, FLAIR image reveals characteristic hyperintensity of the cerebellum.

B, Diffusion-weighted image obtained at a b value of 1000 s/mm2 reveals hyperintensities in the folia (restricted diffusion).

C, FLAIR image reveals hyperintensities in the genu and splenium of the corpus callosum.

D, Diffusion-weighted image obtained at a b value of 1000 s/mm2 reveals hyperintensity of the splenium, indicating restricted diffusion. (The genu was similarly involved.)



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FIG 3. Image obtained in a 6-year-old boy.

A, FLAIR image reveals characteristic hyperintensity of the cerebellum.

B, ADC map reveals an increased ADC value (1.41 x 10–3 mm2/s) in the cerebellum, indicating elevated diffusion compared with a normal ADC value (0.88 x 10–3 mm2/s) in the occipital lobe. (Diffusion-weighted image obtained at a b value of 1000 s/mm2 appeared normal.)



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FIG 4. Images obtained in a 5-year-old boy. ADC map reveals an increased ADC value (1.47 x 10–3 mm2/s) in the cerebellum compared with normal values from the pons (0.95 x 10–3 mm2/s) and temporal lobe (0.94 x 10–3 mm2/s).



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FIG 5. Images obtained in a 5-year-old girl.

A, Sagittal FLAIR image reveals characteristic hyperintensity of the vermis.

B, ADC map reveals an increased ADC value in the cerebellum (1.61 x 10–3 mm2/s) compared with the normal value from the right temporal lobe (0.75 x 10–3 mm2/s).