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Childhood X-linked Adrenoleukodystrophy: Clinical-Pathologic Overview and MR Imaging Manifestations at Initial Evaluation and Follow-up¹

¹ From the Department of Diagnostic Radiology, Konyang University Hospital, 685 Gasuwon-dong, Seo-gu, Daejeon City 302–718, South Korea (J.H.K.); and the Department of Pediatrics, Genetic Clinics, Ajou University Hospital, Suwon, South Korea (H.J.K.). Presented as an education exhibit at the 2003 RSNA Scientific Assembly. Received May 21, 2004; revision requested September 10 and received January 3, 2005; accepted January 5. All authors have no financial relationships to disclose.

View larger version (122K): [in a new window]   Figure 1a.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (111K): [in a new window]   Figure 1b.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (125K): [in a new window]   Figure 1c.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (115K): [in a new window]   Figure 1d.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (124K): [in a new window]   Figure 1e.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (126K): [in a new window]   Figure 1f.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (126K): [in a new window]   Figure 1g.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (122K): [in a new window]   Figure 1h.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (127K): [in a new window]   Figure 1i.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (126K): [in a new window]   Figure 1j.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (123K): [in a new window]   Figure 1k.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (127K): [in a new window]   Figure 1l.  Childhood cerebral X-linked ALD in an 8-year-old boy with initial clinical findings of hearing impairment. (a–f) Initial T2-weighted MR images show confluent and symmetric bilateral hyperintense areas in the parieto-occipital deep white matter and in the splenium of the corpus callosum (a, b), increased signal intensity in the acoustic radiation (arrows in b), subtle changes in signal intensity in the brachium of the inferior colliculus (arrow in c) and lateral lemniscus (arrow in d), and involvement of the pyramidal tract in the pons and medulla oblongata (arrows in e and f). (g) Initial contrast material–enhanced T1-weighted MR image shows strong enhancement in the middle layer of the lesion (Schaumburg zone 2) (white arrow). Zones 1 (solid black arrows) and 3 (open arrow) are not enhanced. These initial MR imaging findings suggested childhood cerebral X-linked ALD, and genetic-serologic test results confirmed the diagnosis. Although the patient underwent dietary and medical treatment, hearing impairment progressed rapidly. Visual disturbance also appeared 2 months after initial evaluation, and follow-up MR imaging was performed. (h–k) Follow-up MR images demonstrate more extensive bilateral signal intensity changes in the parieto-occipital white matter (h), interval growth of the lesions involving the brachium of the inferior colliculus (arrows in i, solid white arrow in j) and the lateral lemniscus (open arrow in j, arrow in k), and signal intensity change in the right optic tract (black arrow in j). The latter finding was not demonstrated at initial MR imaging. (l) MR image obtained 6 months after initial MR imaging shows more extensive bilateral signal intensity changes in the parieto-occipital white matter, along with atrophic change in the deep white matter.

 

View larger version (127K): [in a new window]   Figure 2a.  Childhood cerebral X-linked ALD in a clinically asymptomatic 7-year-old boy. (a) On an initial brain MR image, the lesion is confined to the left parietopontine tract and to isolated white matter fibers of the corticospinal tract in the posterior limb of the internal capsule. (b) Follow-up MR image obtained 9 months later shows slight enlargement of the lesion (arrow) in the left parietopontine tract.

 

View larger version (127K): [in a new window]   Figure 2b.  Childhood cerebral X-linked ALD in a clinically asymptomatic 7-year-old boy. (a) On an initial brain MR image, the lesion is confined to the left parietopontine tract and to isolated white matter fibers of the corticospinal tract in the posterior limb of the internal capsule. (b) Follow-up MR image obtained 9 months later shows slight enlargement of the lesion (arrow) in the left parietopontine tract.

 

View larger version (134K): [in a new window]   Figure 3a.  Childhood cerebral X-linked ALD in an 8-year-old boy whose initial symptoms were similar to those of anxiety disorder. (a–c) Initial MR images demonstrate confluent hyperintense areas in the parieto-occipital white matter. The U fibers are completely spared in all areas. These findings, along with serologic testing, helped confirm the diagnosis. The patient subsequently underwent diet therapy. MR imaging performed 6 months later showed no remarkable interval change. (d, e) MR images obtained 3 years after initial diagnosis with the patient in a near-vegetative state show severe atrophic change in the cerebral hemispheres and cerebellum.

 

View larger version (128K): [in a new window]   Figure 3b.  Childhood cerebral X-linked ALD in an 8-year-old boy whose initial symptoms were similar to those of anxiety disorder. (a–c) Initial MR images demonstrate confluent hyperintense areas in the parieto-occipital white matter. The U fibers are completely spared in all areas. These findings, along with serologic testing, helped confirm the diagnosis. The patient subsequently underwent diet therapy. MR imaging performed 6 months later showed no remarkable interval change. (d, e) MR images obtained 3 years after initial diagnosis with the patient in a near-vegetative state show severe atrophic change in the cerebral hemispheres and cerebellum.

 

View larger version (115K): [in a new window]   Figure 3c.  Childhood cerebral X-linked ALD in an 8-year-old boy whose initial symptoms were similar to those of anxiety disorder. (a–c) Initial MR images demonstrate confluent hyperintense areas in the parieto-occipital white matter. The U fibers are completely spared in all areas. These findings, along with serologic testing, helped confirm the diagnosis. The patient subsequently underwent diet therapy. MR imaging performed 6 months later showed no remarkable interval change. (d, e) MR images obtained 3 years after initial diagnosis with the patient in a near-vegetative state show severe atrophic change in the cerebral hemispheres and cerebellum.

 

View larger version (136K): [in a new window]   Figure 3d.  Childhood cerebral X-linked ALD in an 8-year-old boy whose initial symptoms were similar to those of anxiety disorder. (a–c) Initial MR images demonstrate confluent hyperintense areas in the parieto-occipital white matter. The U fibers are completely spared in all areas. These findings, along with serologic testing, helped confirm the diagnosis. The patient subsequently underwent diet therapy. MR imaging performed 6 months later showed no remarkable interval change. (d, e) MR images obtained 3 years after initial diagnosis with the patient in a near-vegetative state show severe atrophic change in the cerebral hemispheres and cerebellum.

 

View larger version (119K): [in a new window]   Figure 3e.  Childhood cerebral X-linked ALD in an 8-year-old boy whose initial symptoms were similar to those of anxiety disorder. (a–c) Initial MR images demonstrate confluent hyperintense areas in the parieto-occipital white matter. The U fibers are completely spared in all areas. These findings, along with serologic testing, helped confirm the diagnosis. The patient subsequently underwent diet therapy. MR imaging performed 6 months later showed no remarkable interval change. (d, e) MR images obtained 3 years after initial diagnosis with the patient in a near-vegetative state show severe atrophic change in the cerebral hemispheres and cerebellum.

 

View larger version (131K): [in a new window]   Figure 4a.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (119K): [in a new window]   Figure 4b.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (117K): [in a new window]   Figure 4c.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (133K): [in a new window]   Figure 4d.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (127K): [in a new window]   Figure 4e.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (123K): [in a new window]   Figure 4f.  Childhood cerebral X-linked ALD in a 9-year-old boy in whom the disease had been diagnosed with serologic testing and genetic study 2 years earlier. Although the patient had a symptomatic older brother with childhood cerebral X-linked ALD, he himself remained asymptomatic throughout the 2-year follow-up period. (a–c) Initial brain MR (FLAIR) images show subtle bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (d–f) On brain MR images obtained 7 months later, the lesions in the parieto-occipital white matter seem to be aggravated. New signal intensity changes are also demonstrated in the left medial geniculate body (arrow in e), lateral lemniscus (arrowhead in f), and brachium of the inferior colliculus (arrow in f). MR imaging performed 5 months later showed no interval change.

 

View larger version (124K): [in a new window]   Figure 5a.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (108K): [in a new window]   Figure 5b.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (126K): [in a new window]   Figure 5c.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (127K): [in a new window]   Figure 5d.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (113K): [in a new window]   Figure 5e.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (133K): [in a new window]   Figure 5f.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (126K): [in a new window]   Figure 5g.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (100K): [in a new window]   Figure 5h.  Childhood cerebral X-linked ALD in a 12-year-old boy. The diagnosis was delayed because the patient’s initial symptoms were similar to those of behavioral disorder. (a–d) Initial MR images obtained 1 year after the onset of symptoms show focal bilateral signal intensity changes in the posterior limb of the internal capsule (a), involvement of the pyramidal tract in the pons (arrows in b and c) and medulla oblongata (arrows in d), and bilateral signal intensity change with mild atrophy in the middle cerebellar peduncles and adjacent cerebellum (c). Continuous medical care was advised, but the patient’s parents refused further evaluation and treatment for their son. Seven months later, the patient was admitted to the emergency department due to poor general condition and severe neurologic deterioration. (e–h) MR images show extensive bilateral lesion changes in the internal capsule (e) and upper pons (f) as well as severe atrophic change in the cerebellum and pons (g, h).

 

View larger version (101K): [in a new window]   Figure 6a.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

View larger version (114K): [in a new window]   Figure 6b.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

View larger version (130K): [in a new window]   Figure 6c.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

View larger version (126K): [in a new window]   Figure 6d.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

View larger version (138K): [in a new window]   Figure 6e.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

View larger version (131K): [in a new window]   Figure 6f.  Childhood cerebral X-linked ALD in a 7-year-old boy with an elevated plasma VLCFA level. Although the patient was asymptomatic, he had a cousin with clinically overt childhood cerebral X-linked ALD and underwent serologic testing. (a, b) Initial T2-weighted MR images of the brain show bilateral signal intensity changes in the parieto-occipital deep white matter and in the splenium of the corpus callosum. (c–e) Brain MR images obtained 1 years later demonstrate newly developed lesions in the medial geniculate body (arrows in c), brachium of the inferior colliculus (arrow in d), and lateral lemniscus (arrows in e). In spite of these brain lesions, however, the patient remained asymptomatic. (f) Brain MR image obtained 21 years after initial MR imaging shows more extensive signal intensity change in the cerebral deep white matter lesions. The patient began to show symptoms similar to those of attention deficit disorder.

 

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