Neuropediatrics 2012; 43 - PS17_09
DOI: 10.1055/s-0032-1307135

Multifocal Balo's concentric sclerosis in a 10-year-old German boy

H Losch 1, A Renneberg 1, S Pfleiderer 2
  • 1Kinderklinik Am Bürgerpark, Bremerhaven, Germany
  • 2Klinikum Bremerhaven, Radiologie, Bremerhaven, Germany

Aims: A previously healthy 10-year-old boy with multifocal Balo's concentric sclerosis is presented. Balo's concentric sclerosis is a very rare variant of multiple sclerosis with rapid procession, alternatively it may be a different entity altogether. To our knowledge only four pediatric cases have been described so far. Differentation from cerebral abcesses or neoplasms is difficult. Comparing literature cases including MRI findings and clinical presentation the diagnosis of Balo's concentric sclerosis is strongly supported. Because of the rareness of the disease therapy, duration and nature of follow-up are uncertain.

Methods: A 10-year-old-boy presented a complex-focal seizure and secondary generalization. He vomited and his mimic was asymmetric. Blood and CSF examinations yielded normal results including negative oligoclonal bands. EEG showed a focal left frontal slowing. There were trifocal lesions measuring 23 to 50mm across in MRI. Over the following days rapidly increasing neurologic symptoms developped, including dysarthria, difficulties with swallowing, facial paralysis and facial hypesthesia. MRI control showed growing of all three lesion with irregular concentric signalintensities in T2-weighted images.

Results: Considering the clinical presentation with rapid progredient neurological symptoms and multifocal huge inflammatory demyelinating lesions we initiated a therapy with high-dosed methylprednisolone over a course of five days. The boy improved quickly. Until the 12 month follow-up he remains symptomfree so far, MRI after 2 and 6 months show continuously decreasing lesions.

Conclusion: Typical clinical presentation and characteristic MRT findings led us to start a immuno-suppressive therapy without neurosurgery or biopsy, saving viable brain areas. Our patient reamins symptomfree over 1 year of follow-ups. Considering the rareness of the disease further case reports and studies are needed to establish knowledge about nature and duration of a necessary follow-up and suitable therapy.