Brain Involvement in Salla Disease
Pirkko Sonninen
,a,
Taina Auttia,
Tarja Varhoa,
Mirja Hämäläinena and
Raili Raininkoa
a From the Department of Diagnostic Radiology, University Hospital of Turku, Finland (P.S.); the Department of Radiology, University Central Hospital of Helsinki, Finland (T.A.); the Department of Medical Genetics, University of Turku, Finland (T.V.); the Department of Child Neurology, University Hospital of Helsinki, Finland (M.H.); and the Department of Diagnostic Radiology, University Hospital, Uppsala, Sweden (R.R.).
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FIG 1. Case 8: 7-year-old boy with clinically mild type of Salla disease.
A–C, At the age of 27 months the corpus callosum is very thin. T2-weighted (2500/90) spin-echo MR images show high signal intensity in the cerebral white matter (A) and somewhat high signal intensity in the internal capsules (B) as a sign of hypomyelination. Myelin was better seen in the internal capsules on T1-weighted image (C).
D and E, At the age of 7 years, T2-weighted images show myelination of the internal capsules had progressed (D), and myelin was also seen above the atria (E).
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FIG 2. Case 6: 6-year-old girl with conventional type of Salla disease. MR image shows homogeneous high signal intensity in the cerebral white matter, including the internal capsules.
FIG 3. Case 11: 15-year-old boy with clinically severe type of Salla disease. MR image shows extremely high signal intensity around the lateral ventricles and moderate atrophic changes.
FIG 4. Case 13: 20-year-old man with clinically severe type of Salla disease. MR image shows very high signal intensity in the entire cerebral white matter.
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FIG 5. A and B, Ratios between signal intensity in the deep nuclei and in the peripheral cerebral white matter with 1.5-T equipment (A) and with 1.0-T equipment (B). Solid circles indicate patients; open circles, control subjects
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FIG 6. A and B, Signal intensity in the deep nuclei and peripheral cerebral white matter expressed as a percentage of the intensity of CSF signal in the tips of the frontal horns. Examinations with 1.5-T equipment (A) and with 1.0-T equipment (B). The number of subjects measured varies in the figures, because in two patients and in four control subjects the frontal horns were very small or the sections containing the structures of interest were not optimally located in the sections. Solid circles indicate patients; open circles, control subjects
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