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Progressive multifocal leukoencephalopathy in a patient with systemic lupus erythematosus and autoimmune hepatitis. 2022
Saranya Gomathy, and
Baikuntha Panigrahi, and
Praveen Kumar Tirlangi, and
Naveet Wig, and
Megha Brijwal, and
Mehar C Sharma, and
Ajay Garg, and
Manjari Tripathi, and
Srikant Mohta, and
Ramesh Doddamani, and
Deepti Vibha, and
Rajesh Kumar Singh, and
Rajni Yadav, and
Saumya Sahu, and
Vaishali Suri, and
Kavneet Kaur, and
Madhavi Tripathi, and
Anshu Rohatgi, and
Arunmozhimaran Elavarasi
Department of Neurology, All India Institute of Medical Sciences, New Delhi, India.
Progressive multifocal leukoencephalopathy (PML) is a rare demyelinating central nervous system illness encountered in the setting of immunosuppressive conditions like human immunodeficiency virus / acquired immunodeficiency syndrome, autoimmune diseases and hematologic malignancies. We had a 54-year-old woman with systemic lupus erythematosus and coexisting autoimmune hepatitis who presented with progressive cognitive decline, right hemiparesis and ataxia who was found to have PML. She had severe CD4 lymphopenia. She was managed with low-dose prednisolone and plasma exchange after which she showed significant clinical improvement. This case highlights the diagnostic and therapeutic challenges encountered in managing a case of PML in the setting of autoimmune conditions with profound lymphopenia.
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