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Imaging Features of Fabry Disease

Olivier Lidove1, Isabelle Klein2, Jean-Daniel Lelièvre1, Philippa Lavallée3, Jean-Michel Serfaty2, Emmanuel Dupuis4, Thomas Papo1 and Jean-Pierre Laissy2

1 Department of Internal Medicine, Hôpital Bichat Claude-Bernard, 46 rue Henri Huchard, 75722 Paris, Cedex 18, France.
2 Department of Radiology, Hôpital Bichat Claude-Bernard, 75722 Paris, Cedex 18, France.
3 Department of Neurology, Hôpital Bichat Claude-Bernard, 75722 Paris, Cedex 18, France.
4 Department of Nephrology, Hôpital Bichat Claude-Bernard, 75722 Paris, Cedex 18, France.


Figure 1
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  		Fig. 1A —30-year-old man with pontine and left deep gray nucleus involvement. Midsagittal T1-weighted images (1.5-T system, 2D gradient-refocused echo; TR/TE, 24/9; flip angle, 40°; slice thickness, 8 mm). (A) shows nodular pontine hyposignal (short arrow), which displays slight hypersignal on corresponding axial (B) and coronal (C) T2-weighted images (2D fast spin-echo; 4,400/126; slice thickness, 6-7 mm) (short arrows). CSF-like hyperintensity is also seen in left putamen (long arrow, C).

 

Figure 2
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  		Fig. 1B —30-year-old man with pontine and left deep gray nucleus involvement. Midsagittal T1-weighted images (1.5-T system, 2D gradient-refocused echo; TR/TE, 24/9; flip angle, 40°; slice thickness, 8 mm). (A) shows nodular pontine hyposignal (short arrow), which displays slight hypersignal on corresponding axial (B) and coronal (C) T2-weighted images (2D fast spin-echo; 4,400/126; slice thickness, 6-7 mm) (short arrows). CSF-like hyperintensity is also seen in left putamen (long arrow, C).

 

Figure 3
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  		Fig. 1C —30-year-old man with pontine and left deep gray nucleus involvement. Midsagittal T1-weighted images (1.5-T system, 2D gradient-refocused echo; TR/TE, 24/9; flip angle, 40°; slice thickness, 8 mm). (A) shows nodular pontine hyposignal (short arrow), which displays slight hypersignal on corresponding axial (B) and coronal (C) T2-weighted images (2D fast spin-echo; 4,400/126; slice thickness, 6-7 mm) (short arrows). CSF-like hyperintensity is also seen in left putamen (long arrow, C).

 

Figure 4
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  		Fig. 2 —52-year-old woman with periventricular hyperintense nodules on FLAIR imaging (1.5-T system; TR/TE, 9,000/146; inversion time, 2,250 msec; slice thickness, 5 mm). Nodular pattern, although nonspecific, should suggest disease in nonhypertensive patient and is related to cerebral vasculopathy involving long perforating arteries.

 

Figure 5
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  		Fig. 3A —Deep gray matter involvement seen at various levels in different patients on T1-weighted images (1.5-T system, 2D spin echo; TR/TE, 520/10; slice thickness, 5 mm). In every patient, abnormalities are seen as increased signal. Thalamus involvement is obvious in 40-year-old patient.

 

Figure 6
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  		Fig. 3B —Deep gray matter involvement seen at various levels in different patients on T1-weighted images (1.5-T system, 2D spin echo; TR/TE, 520/10; slice thickness, 5 mm). In every patient, abnormalities are seen as increased signal. Bilateral substantia nigra involvement is seen (arrows) in 38-year-old man.

 

Figure 7
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  		Fig. 4A —40-year-old man without hypertension Hypertrophic cardiomyopathy is seen on short-axis cine MRI views (1.5-T system, 2D steady-state free precession; TR/TE, 3.6/1.5; slice thickness, 8 mm) in diastole (A) and systole (B) and in four-chamber cine MRI views in diastole (C) and systole (D). Ventricular cavity is virtually absent in systole because of concentric hypertrophy of myocardial fibers.

 

Figure 8
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  		Fig. 4B —40-year-old man without hypertension Hypertrophic cardiomyopathy is seen on short-axis cine MRI views (1.5-T system, 2D steady-state free precession; TR/TE, 3.6/1.5; slice thickness, 8 mm) in diastole (A) and systole (B) and in four-chamber cine MRI views in diastole (C) and systole (D). Ventricular cavity is virtually absent in systole because of concentric hypertrophy of myocardial fibers.

 

Figure 9
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  		Fig. 4C —40-year-old man without hypertension Hypertrophic cardiomyopathy is seen on short-axis cine MRI views (1.5-T system, 2D steady-state free precession; TR/TE, 3.6/1.5; slice thickness, 8 mm) in diastole (A) and systole (B) and in four-chamber cine MRI views in diastole (C) and systole (D). Ventricular cavity is virtually absent in systole because of concentric hypertrophy of myocardial fibers.

 

Figure 10
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  		Fig. 4D —40-year-old man without hypertension Hypertrophic cardiomyopathy is seen on short-axis cine MRI views (1.5-T system, 2D steady-state free precession; TR/TE, 3.6/1.5; slice thickness, 8 mm) in diastole (A) and systole (B) and in four-chamber cine MRI views in diastole (C) and systole (D). Ventricular cavity is virtually absent in systole because of concentric hypertrophy of myocardial fibers.

 

Figure 11
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  		Fig. 5 —Cardiac involvement in 38-year-old man. Late-enhancement T1-weighted cardiac image in short axis (1.5-T system, 3D inversion recovery T1-weighted multishot gradient echo; TR/TE, 3.9/1.4; flip angle, 25°; inversion-recovery prepulse delay, 200 msec) shows band of hyperenhancement assumed to be related to myocardial fibrosis in upper part of septum (large arrows) and subepicardial nodules in inferior wall (small arrows). Cine MRI image at same level (not shown) displayed normal segmental contraction.

 

Figure 12
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  		Fig. 6A —Cardiac involvement in 42-year-old woman. Late-enhancement T1-weighted cardiac images in short axis (same parameters as in Fig. 5) show nodular transmural hyperenhancement in anterior wall (arrow, A) and several patchy, slightly hyperenhancing nodules in inferolateral wall of left ventricle (arrows, B).

 

Figure 13
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  		Fig. 6B —Cardiac involvement in 42-year-old woman. Late-enhancement T1-weighted cardiac images in short axis (same parameters as in Fig. 5) show nodular transmural hyperenhancement in anterior wall (arrow, A) and several patchy, slightly hyperenhancing nodules in inferolateral wall of left ventricle (arrows, B).

 

Figure 14
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  		Fig. 7A —MRI follow-up of hypertrophic cardiomyopathy in 43-year-old man. Short-axis cine MRI views (same parameters as in Figs. 4A, 4B, 4C, and 4D) in diastole (A) and systole (B) at middle portion of left ventricle (LV) before initiation of enzyme replacement therapy. Left ventricle myocardial mass is estimated at 136 g/m2. End-diastolic and end-systolic LV thicknesses are 13 mm and 28 mm, respectively.

 

Figure 15
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  		Fig. 7B —MRI follow-up of hypertrophic cardiomyopathy in 43-year-old man. Short-axis cine MRI views (same parameters as in Figs. 4A, 4B, 4C, and 4D) in diastole (A) and systole (B) at middle portion of left ventricle (LV) before initiation of enzyme replacement therapy. Left ventricle myocardial mass is estimated at 136 g/m2. End-diastolic and end-systolic LV thicknesses are 13 mm and 28 mm, respectively.

 

Figure 16
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  		Fig. 7C —MRI follow-up of hypertrophic cardiomyopathy in 43-year-old man. Corresponding cine MRI images 6 months later show decrease in LV hypertrophy, with LV mass estimated at 115 g/m2, with end-diastolic and end-systolic LV thicknesses of 12 mm and 26 mm, respectively.

 

Figure 17
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  		Fig. 7D —MRI follow-up of hypertrophic cardiomyopathy in 43-year-old man. Corresponding cine MRI images 6 months later show decrease in LV hypertrophy, with LV mass estimated at 115 g/m2, with end-diastolic and end-systolic LV thicknesses of 12 mm and 26 mm, respectively.

 

Figure 18
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  		Fig. 8 —40-year-old man. Cardiac CT image of aortic valve leaflet shows thickening with calcifications.

 

Figure 19
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  		Fig. 9A —40-year-old man on hemodialysis for 6 years. Sonograms show bilateral kidney involvement. Long-axis diameter of right (A) and left kidney (not shown) is nearly normal, with normal external contours. Corticosinusal thickness seems normal. Both kidneys contain cysts and appear hyperechogenic.

 

Figure 20
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  		Fig. 9B —40-year-old man on hemodialysis for 6 years. Sonograms show bilateral kidney involvement. Noncontrast CT shows some degree of renal atrophy and confirms presence of multiple cysts, some of them displaying peripheral high attenuation values (short arrows). Some dense calcifications are present along right renal sinus.

 

Figure 21
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  		Fig. 10 —Bronchial thickening in both lower lobes (arrows) in nonsmoking 40-year-old man.

 

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