What is the significance of leptomeningeal enhancement in Graham disease (also known as Hepatolenticular Degeneration or Wilson's Disease)?

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Last updated: February 25, 2025View editorial policy

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From the Research

Leptomeningeal enhancement is not a feature of Graham disease, as Graham disease is not a recognized medical condition in standard medical literature, but rather it is associated with Wilson's disease, a genetic disorder that leads to copper accumulation in the brain and liver.

Significance of Leptomeningeal Enhancement

Leptomeningeal enhancement on imaging studies like MRI indicates inflammation or abnormal activity in the meninges, which can be seen in various conditions including infections, autoimmune disorders, malignancies, and neurosarcoidosis.

Conditions Associated with Leptomeningeal Enhancement

Some of the conditions associated with leptomeningeal enhancement include:

  • Infections (meningitis) 1
  • Autoimmune disorders, such as MOG-IgG-associated encephalomyelitis 2
  • Malignancies (carcinomatosis) 1
  • Neurosarcoidosis
  • Leptomeningeal amyloidosis associated with transthyretin mutations 3
  • Diffuse leptomeningeal glioneuronal/neuroepithelial tumors 4

Treatment of Leptomeningeal Enhancement

Treatment depends entirely on the underlying cause.

  • For infectious causes, appropriate antimicrobial therapy is needed.
  • For inflammatory conditions, corticosteroids like dexamethasone or prednisone may be prescribed 2.
  • For malignant causes, oncology consultation and cancer-specific treatments are required 1.

Importance of Urgent Neurological Evaluation

If you're experiencing neurological symptoms with imaging showing leptomeningeal enhancement, urgent neurological evaluation is essential for proper diagnosis and treatment 2, 3. The enhancement pattern occurs because contrast agents leak into the subarachnoid space when the blood-brain barrier is compromised by disease processes.

Recent Studies

A recent study published in 2024 found that leptomeningeal enhancement in multiple sclerosis may be affected by autologous hematopoietic stem cell transplantation 5. Another study published in 2021 found that leptomeningeal amyloidosis associated with transthyretin mutations can cause cognitive decline, headache, and numbness, and can be diagnosed with MRI and genetic testing 3.

Professional Medical Disclaimer

This information is intended for healthcare professionals. Any medical decision-making should rely on clinical judgment and independently verified information. The content provided herein does not replace professional discretion and should be considered supplementary to established clinical guidelines. Healthcare providers should verify all information against primary literature and current practice standards before application in patient care. Dr.Oracle assumes no liability for clinical decisions based on this content.

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