Sjogren-Larsson syndrome associated with the dandy-walker malformation

Report of a case

D. P. Fivenson, A. W. Lucky, S. Iannoccone

Research output: Contribution to journalArticle

9 Citations (Scopus)

Abstract

The Sjogren-Larsson syndrome is defined as the association of ichthyosis, spastic diplegia or tetraplegia, and mental retardation. Although most cases described have come from the Swedish county of Vasterbotten, isolated reports from the United States and elsewhere do exist. Numerous neurologic abnormalities have been reported with this syndrome, including speech defects, seizures, and pyramidal tract disorders. Abnormal ophthalmologic findings are not rare. Previous studies with computerized tomographic (CT) scans have revealed no abnormalities of cranial anatomy. We report a 6-year-old boyf with Sjogren-Larsson syndrome who had a Dandy-Walker malformation as demonstrated by diagnostic imaging by CT and magnetic resonance imaging scans. This case illustrates that developmental defects of the central nervous system can also occur in association with Sjogren-Larsson syndrome.

Original languageEnglish (US)
Pages (from-to)312-315
Number of pages4
JournalPediatric Dermatology
Volume6
Issue number4
StatePublished - 1989

Fingerprint

Sjogren-Larsson Syndrome
Dandy-Walker Syndrome
Nervous System Malformations
Ichthyosis
Pyramidal Tracts
Quadriplegia
Cerebral Palsy
Diagnostic Imaging
Intellectual Disability
Anatomy
Seizures
Central Nervous System
Magnetic Resonance Imaging

ASJC Scopus subject areas

  • Dermatology
  • Pediatrics, Perinatology, and Child Health

Cite this

Sjogren-Larsson syndrome associated with the dandy-walker malformation : Report of a case. / Fivenson, D. P.; Lucky, A. W.; Iannoccone, S.

In: Pediatric Dermatology, Vol. 6, No. 4, 1989, p. 312-315.

Research output: Contribution to journalArticle

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