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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. Sagittal T1 image showing the pathognomonic central callosal "holes" (microinfarcts) of SS. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve.
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Figure 4: MRI brain. Sagittal T1 image showing the pathognomonic central callosal "holes" (microinfarcts) of SS. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve.

Mentions: The patient has now been followed for 36 months (156 weeks). Within two days after onset of treatment her headache and vomiting resolved, and her encephalopathy began to improve. Her encephalopathy continued to steadily improve thereafter and has never relapsed. Repeat neuropsychological evaluation at week 41 revealed a full scale IQ of 122 and a return to pre-morbid estimates in many domains. Fifty two weeks after onset of treatment she scored in the 98th percentile on her college entrance examination. Repeat MRI at 60 weeks revealed marked resolution of the previously noted white matter lesions. Her corpus callosum was thinned and showed the typical residual callosal "holes" of SS (Figure 4).


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. Sagittal T1 image showing the pathognomonic central callosal "holes" (microinfarcts) of SS. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve.
© Copyright Policy - open-access
Related In: Results  -  Collection

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

Figure 4: MRI brain. Sagittal T1 image showing the pathognomonic central callosal "holes" (microinfarcts) of SS. These residual "holes" (and sometimes, "spokes") develop as the acute callosal changes resolve.
Mentions: The patient has now been followed for 36 months (156 weeks). Within two days after onset of treatment her headache and vomiting resolved, and her encephalopathy began to improve. Her encephalopathy continued to steadily improve thereafter and has never relapsed. Repeat neuropsychological evaluation at week 41 revealed a full scale IQ of 122 and a return to pre-morbid estimates in many domains. Fifty two weeks after onset of treatment she scored in the 98th percentile on her college entrance examination. Repeat MRI at 60 weeks revealed marked resolution of the previously noted white matter lesions. Her corpus callosum was thinned and showed the typical residual callosal "holes" of SS (Figure 4).

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