Sjogren-Larsson syndrome: management
Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .
Sjogren-Larsson syndrome: management. Arrest of myelination, Periventricular white matter signal abnormalities. Fully referenced neurology checklist.
Magnetic resonance imaging (MRI) brain: features
- Arrest of myelination
- Periventricular white matter signal abnormalities
- Mild ventricular enlargement
- Magnetic resonance spectroscopy (MRS): this shows abnormal lipid peaks
Electroencephalogram (EEG)
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Treatment of ichthyosis
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Potential treatments
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References
- Willemsen MA, IJlst L, Steijlen PM, et al. Clinical, biochemical and molecular genetic characteristics of 19 patients with the Sjögren-Larsson syndrome. Brain 2001; 124:1426-1437.
- Moghaddam FR, Safar F, Asheghan M, Soltani ZR, Zade FD. Sjögren-Larsson syndrome in two brothers: a case report. Cases J 2009; 2:8434.
- Nagappa M, Bindu PS, Chiplunkar S, et al. Child Neurology: Sjögren-Larsson syndrome. Neurology 2017; 88:e1-e4.
