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Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model.

Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO - Pediatr Rheumatol Online J (2008)

Bottom Line: We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear.Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM.The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA. rennebohmr@pediatrics.ohio-state.edu.

ABSTRACT
We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

No MeSH data available.


Related in: MedlinePlus

MRI brain. Axial T2 image showing multiple white matter lesions, especially in the corpus callosum. The arrows point to only two of several callosal lesions. Early in the disease, callosal lesions are usually best seen on thin section sagittal FLAIR and sagittal T1 images, with contrast.
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Figure 1: MRI brain. Axial T2 image showing multiple white matter lesions, especially in the corpus callosum. The arrows point to only two of several callosal lesions. Early in the disease, callosal lesions are usually best seen on thin section sagittal FLAIR and sagittal T1 images, with contrast.

Mentions: MRI of the brain showed numerous widespread small hyperintense white matter lesions on T2 and FLAIR in both the supratentorial and infratentorial compartments. Several of these lesions enhanced, and there was impressive leptomeningeal enhancement cloaking the cerebral and cerebellar hemispheres. The central corpus callosum was extensively involved (Figure 1). Similar lesions were present in the basal ganglia. Diffusion weighted images showed restriction in many of these lesions.


Aggressive immunosuppressive treatment of Susac's syndrome in an adolescent: using treatment of dermatomyositis as a model.

Rennebohm RM, Lubow M, Rusin J, Martin L, Grzybowski DM, Susac JO - Pediatr Rheumatol Online J (2008)

MRI brain. Axial T2 image showing multiple white matter lesions, especially in the corpus callosum. The arrows point to only two of several callosal lesions. Early in the disease, callosal lesions are usually best seen on thin section sagittal FLAIR and sagittal T1 images, with contrast.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC2267466&req=5

Figure 1: MRI brain. Axial T2 image showing multiple white matter lesions, especially in the corpus callosum. The arrows point to only two of several callosal lesions. Early in the disease, callosal lesions are usually best seen on thin section sagittal FLAIR and sagittal T1 images, with contrast.
Mentions: MRI of the brain showed numerous widespread small hyperintense white matter lesions on T2 and FLAIR in both the supratentorial and infratentorial compartments. Several of these lesions enhanced, and there was impressive leptomeningeal enhancement cloaking the cerebral and cerebellar hemispheres. The central corpus callosum was extensively involved (Figure 1). Similar lesions were present in the basal ganglia. Diffusion weighted images showed restriction in many of these lesions.

Bottom Line: We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear.Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM.The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pediatrics, Division of Pediatric Rheumatology, Ohio State University College of Medicine, Columbus, Ohio, USA. rennebohmr@pediatrics.ohio-state.edu.

ABSTRACT
We describe aggressive immunosuppressive treatment of an adolescent with Susac's syndrome (SS), a disease of the microvasculature in the brain, retina, and inner ear. Because the immunopathogenesis of SS appears to have much in common with that of juvenile dermatomyositis (JDM), the patient was treated with an approach that has been effective for severe JDM. The patient's outcome provides evidence for the importance of prompt, aggressive, and sustained immunosuppressive treatment of encephalopathic SS.

No MeSH data available.


Related in: MedlinePlus