MASA Syndrome

Alternative Names

  • Mental Retardation, Aphasia, Shuffling Gait, and Adducted Thumbs
  • Clasped Thumb and Mental Retardation
  • Thumb, Congenital Clasped, with Mental Retardation
  • Adducted Thumb with Mental Retardation
  • Gareis-Mason Syndrome
  • Spastic Paraplegia Type 1
  • SPG1
  • Crash Syndrome
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WHO-ICD-10 version:2010

Congenital malformations, deformations and chromosomal abnormalities

Congenital malformations of the nervous system

OMIM Number

303350

Mode of Inheritance

X-linked recessive

Gene Map Locus

Xq28

Description

MASA syndrome is a rare neurological disorder with an X-linked recessive mode of inheritance. It is characterized by mental retardation, adducted thumbs, shuffling gait, and aphasia. Other symptoms and physical findings include abnormal widening of cavities within the brain and accumulation of excessive cerebrospinal fluid in the hydrocephalus, mild short stature, abnormally increased inward curvature of the lower spine (exaggerated lumbar lordosis), and other skeletal abnormalities. Brain imaging provides evidence of dilated cerebral ventricles, hypoplasia or agenesis of the corticospinal tract, stenosis of the aqueduct of Sylvius, and agenesis of the corpus callosum.

Epidemiology in the Arab World

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Subject IDCountrySexFamily HistoryParental ConsanguinityHPO TermsVariantZygosityMode of InheritanceReferenceRemarks
303350.1United Arab EmiratesMaleNoYes Global developmental delay; Intellectu...NM_001278116.2:c.604G>THemizygousX-linkedSztriha et al. 2000
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