Baló concentric sclerosis

Baló concentric sclerosis (BCS), also known as Baló disease or concentric periaxial leukoencephalopathy, is a rare and aggressive demyelinating disorder of the central nervous system. It is considered a variant of multiple sclerosis (MS) and is characterized by the distinctive formation of alternating rings of demyelinated and myelinated white matter in the brain, creating a concentric or onion-like pattern visible on MRI or at pathological examination. The disease primarily affects the brain's white matter, though lesions can also occur in the brainstem and spinal cord. Clinical presentation is variable but often includes rapidly progressive neurological symptoms such as headache, cognitive dysfunction, behavioral changes, aphasia (difficulty with language), seizures, hemiparesis (weakness on one side of the body), and visual disturbances. Some patients experience a fulminant, monophasic course leading to severe disability or death within weeks to months, while others may follow a relapsing-remitting course more similar to typical MS. The disease most commonly affects young to middle-aged adults, with cases reported across diverse ethnic backgrounds, though it has been noted with particular frequency in populations from the Philippines and China. There is no established standard treatment for Baló concentric sclerosis due to its rarity. Management strategies are largely extrapolated from multiple sclerosis treatment protocols and typically include high-dose intravenous corticosteroids during acute episodes. Plasma exchange (plasmapheresis) has been used in steroid-refractory cases with some reported benefit. Immunosuppressive agents and disease-modifying therapies used in MS, such as mitoxantrone or natalizumab, have been tried in individual cases. Prognosis is highly variable; while historically considered a rapidly fatal condition, improved neuroimaging has led to recognition of milder cases, and some patients experience partial or even substantial recovery.

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