"MRI "
"T1: irregular concentric isointense and hypointense areas 13"
"T2: irregular concentric areas alternating iso/hypointense and hyperintense signal, with central hyperintensity 4,9,13"
"T1 C+ (Gd): typically a peripheral ring of enhancement in the area of active demyelination 9,13"
"DWI: some restricted diffusion in the outer ring 7,13"
"MR spectroscopy: may have an increase in the choline to NAA ratio 13"
"Although first described by the Austrian neurologist Otto Marburg (1874-1948) in 1909 13, it is named after József Baló (1895–1979), a Hungarian neuropathologist who subsequently published the unusual case and subsequent death of a young law student who presented with fulminantly deteriorating, focal neurological symptoms in 1928 11. "Leuko-encephalitis periaxialis concentrica" was the term he proposed for the concentric focus of grey and white layers in the white matter that he found during autopsy 2,11."
"Although first described by the Austrian neurologist Otto Marburg (1874-1948) in 1909 13, it is named after József Baló (1895–1979), a Hungarian neuropathologist who subsequently published the unusual case and subsequent death of a young law student who presented with fulminantly deteriorating, focal neurological symptoms in 1928 11. "Leuko-encephalitis periaxialis concentrica" was the term he proposed for the concentric focus of grey and white layers in the white matter that he found during autopsy 2,11."
"MR spectroscopy: may have an increase in the choline to NAA ratio 13"
"Rarely, Baló-like lesions have been reported in other diseases, including NMO spectrum disorder, CADASIL, and progressive multifocal leukoencephalopathy 12,13."
"ADEM"
"Acute presentation is typically treated with corticosteroids, and some, if not many, patients recover 12,13. In those who do not respond, a variety of second-line therapies have been tried with no consensus or therapeutic trials to guide treatment 13. Options include plasma exchange, intravenous immunoglobulin, cyclophosphamide, and haematopoietic stem cell transplantation 12,13. Whether long-term immunosuppression should be offered to patients is a subject of conjecture 13."
"The prognosis of Baló concentric sclerosis is variably reported 13. Generally, it is described as having a rapidly progressive course without remissions or relapses. Although in a group of patients it can be fatal, this is not always the case, with some, if not many, patients making excellent recovery 1,4,6,8,12,13."
"Marburg variant of MS"
Expected headings
"MRI "
"Baló concentric sclerosis, or simply Baló disease, is a rare and usually severe monophasic demyelinating disease, considered a variant of multiple sclerosis, appearing as a rounded lesion with alternating layers of high and low signal intensity on MRI, giving it a characteristic 'bullseye' or 'onion bulb' appearance 1,9,12. Whether there is a clear distinction among multiple sclerosis, tumefactive demyelination, and Baló concentric sclerosis remains unclear 12."
"The presentation is non-specific and often mimics mass lesions such as brain tumours, with specific signs and symptoms related to the size and location of the lesions, including headache, seizures, focal neurological deficits, and cognitive changes 12,13."
"Classically, Baló concentric sclerosis is described as an acute monophasic illness, most closely resembling acute Marburg type multiple sclerosis, with rapid progression and sometimes a fulminant course 4,8,9,12. Some patients, however, present with more limited acute self-limiting disease, or even a relapsing-remitting clinical course, more similar to typical multiple sclerosis 12."
"The prognosis of Baló concentric sclerosis is variably reported 13. Generally, it is described as having a rapidly progressive course without remissions or relapses. Although in a group of patients it can be fatal, this is not always the case, with some, if not many, patients making excellent recovery 1,4,6,8,12,13."
"Baló concentric sclerosis is a rare disorder most commonly encountered in young adults, with an average age of 34 years 12,13. There is a female preponderance 13. It is more common among East Asian populations, in particular among patients of southern Han Chinese, Filipino, and Taiwanese descent 13."
"The characteristic feature of Baló concentric sclerosis is the development of multiple alternating bands of demyelinated and myelinated white matter, which is seen as concentric rings or irregular stripes of high or low signal depending on the sequence, giving it a characteristic 'bullseye' or 'onion bulb' appearance 9,13. These lesions are typically present in the cerebral white matter, often sparing subcortical U-fibres and cortical grey matter 13. There is typically minimal perilesional oedema 13."
"high-grade glioma (e.g. glioblastoma)"