page_title: Sjögren–Larsson syndrome
section_title: Signs and symptoms
hierarchical_section_title: Sjögren–Larsson syndrome / Signs and symptoms
caption_attribution_description: English: T2-weighted MR images of a 25 yo Sjögren-Larsson syndrome patient. MRI revealed dysmyelination in the deep periventricular white matter and reduced brain volume in frontal lobe
context_page_description: Sjögren–Larsson syndrome is a rare autosomal recessive form of ichthyosis with neurological symptoms. It can be identified by a triad of medical disorders. The first is ichthyosis, which is a buildup of skin to form a scale-like covering that causes dry skin and other problems. The second identifier is paraplegia which is characterized by leg spasms. The final identifier is intellectual delay.
  The gene of SLS is found on chromosome 17. In order for a child to receive SLS both parents must be carriers of the SLS gene. If they are carriers their child has a ¼ chance of getting the disease. In 1957 Sjögren and Larsson proposed that the Swedes with the disease all descended from a common ancestor 600 years ago. Today only 30–40 persons in Sweden have this disease.
context_section_description: Dry and scaly skin similar to all other ichtyosiforms (types of ichthyosis).
  Neurological problems – this can often cause mild paralysis in the legs
  Mild to moderate intellectual disability.
  Often associated ocular features, which include pigmentary changes in the retina.
  The usual presentation of crystalline maculopathy is from the age of 1-2 years onwards.
caption: T2-weighted MRI images of a 25 year-old male with Sjögren–Larsson syndrome revealing dysmyelination in the deep periventricular white matter and reduced brain volume in the frontal lobe.
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