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Letter

Nature Genetics 36, 1008–1013 (1 September 2004) | doi:10.1038/ng1419

Abnormal cerebellar development and axonal decussation due to mutations in AHI1 in Joubert syndrome

Russell J Ferland , Wafaa Eyaid , Randall V Collura , Laura D Tully , R Sean Hill , Doha Al-Nouri , Ahmed Al-Rumayyan , Meral Topcu , Generoso Gascon , Adria Bodell , Yin Yao Shugart , Maryellen Ruvolo & Christopher A Walsh

Joubert syndrome is a congenital brain malformation of the cerebellar vermis and brainstem with abnormalities of axonal decussation (crossing in the brain) affecting the corticospinal tract and superior cerebellar peduncles. Individuals with Joubert syndrome have motor and behavioral abnormalities, including an inability to walk due to severe clumsiness and 'mirror' movements, and cognitive and behavioral disturbances.