Familial Neck-Tongue Syndrome


Address all correspondence to Dr. Donald W. Lewis, Division of Pediatric Neurology, Children's Hospital of the King's Daughters, 850 Southampton Avenue, Norfolk, VA 23510.


Neck-tongue syndrome is an uncommon clinical entity characterized by brief attacks of intense unilateral stabbing pain in the upper neck or occipital region upon sudden rotation of the head, accompanied by ipsilateral numbness of the tongue. Eight patients, 5 teenagers and 3 adults, with neck-tongue syndrome are presented. Each of the 5 adolescents had normal examinations and normal neuroimaging. The 3 adults were parents of the affected children and had experienced transient symptoms during their adolescence suggesting an autosomal dominant inheritance pattern.