First case of progressive solitary sclerosis with relapsing attacks: A 49-year-old woman

This case highlights a rare form of solitary sclerosis with a relapsing pattern, contrasting with the typical single attack and nonrelapsing nature of the disease. Despite the lack of new lesions on MRI, This case demonstrates that, despite the rarity of this variant, a relapsing form of solitary sclerosis can occur and should be considered a differential diagnosis.

Progressive solitary sclerosis is characterized by isolated demyelinating damage to the central nervous system in the spinal cord and brainstem, leading to progressive motor impairment. We describe the case of a 49-year-old woman who suffered several recurrent attacks of right hemiparesis over time. The patient initially responded well to methylprednisolone pulse therapy without maintenance therapy. However, subsequent episodes resulted in mild residual symptoms and the progression of her condition. Clinical examination revealed normal cranial nerve function, decreased sensation in the right limbs, and abnormal signal findings on MRI of the cervical spine. Laboratory tests, vasculitis screening, cerebrospinal fluid (CSF) analysis, and brain/spinal cord angiography were all within normal limits. Based on these findings and the patient's clinical presentation, a diagnosis of progressive solitary sclerosis with relapsing attacks was made. Rituximab treatment was initiated with administration of a first dose of 1000 mg, followed by a second dose 6 months later.