Vanishing white matter (VWM) disease: management
Evidence-based neurology checklist on vanishing white matter (vwm) disease: management: Magnetic resonance imaging (MRI) This shows a diffuse leukoencephalopathy It involves the cerebrum and cerebellum There is early involvement of the corpus callosum inner rim The signal intensity approaches CSF…
Magnetic resonance imaging (MRI)
- This shows a diffuse leukoencephalopathy
- It involves the cerebrum and cerebellum
- There is early involvement of the corpus callosum inner rim
- The signal intensity approaches CSF intensity
- There may be extensive cystic degeneration and cerebral atrophy
- Restricted diffusion is seen in the relatively spared areas
Magnetic resonance spectroscopy (MRS)
Preventative treatments of attacks
Acute treatments of attacks
Chronic disease management
Investigational treatments
Synonym
References
- van der Knaap MS, Barth PG, Gabreëls FJ, et al. A new leukoencephalopathy with vanishing white matter. Neurology 1997; 48:845-855.
- Labauge P, Horzinski L, Ayrignac X, et al. Natural history of adult-onset eIF2B-related disorders: a multi-centric survey of 16 cases. Brain 2009; 132:2161-2169.
- Bektaş G, Yeşil G, Özkan MU, Yıldız EP, Uzunhan TA, Çalışkan M. Vanishing white matter disease with a novel EIF2B5 mutation: a 10-year follow-up. Clin Neurol Neurosurg 2018; 171:190-193.
- Herwerth M, Schwaiger BJ, Kreiser K, Hemmer B, Ilg R. Adult-onset vanishing white matter disease as differential diagnosis of primary progressive multiple sclerosis: a case report. Mult Scler 2015; 21:666-668.
- van der Lei HD, Steenweg ME, Bugiani M, et al. Restricted diffusion in vanishing white matter. Arch Neurol 2012; 69:723-727.
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