Dementia associated with scleromyxoedema reversed by high-dose intravenous immunoglobulin

Area of Science:

• Dermatology
• Neurology
• Immunology

Background:

• Scleromyxoedema is a rare connective tissue disease characterized by dermal deposition of acid mucopolysaccharides.
• While primarily cutaneous, it can involve cardiovascular, renal, rheumatological, and neurological systems.
• Neurological involvement affects approximately 15% of patients, presenting as psychosis, convulsions, or encephalopathy.

Observation:

• A patient with scleromyxoedema presented with severe chronic cognitive impairment and dementia, scoring 8/30 on the Folstein Mini Mental State test.
• This patient exhibited novel central nervous system manifestations not typically associated with scleromyxoedema.

Findings:

• Treatment with high-dose intravenous immunoglobulin (IVIg) led to rapid improvement in the patient's cognitive function within one week.
• Full cognitive restoration was observed at two months post-treatment, with the Folstein Mini Mental State test score increasing to 27/30.

Implications:

• High-dose IVIg may be an effective therapeutic option for neurological and cognitive manifestations of scleromyxoedema.
• This case highlights the potential for IVIg to reverse severe cognitive decline in patients with scleromyxoedema.
• Further research is warranted to explore the efficacy of IVIg in managing central nervous system involvement in scleromyxoedema.