[Periodic alternating nystagmus in familial congenital cerebellar ataxia].
We noted periodic alternating nystagmus (PAN) in two patients (mother and daughter) who were diagnosed as familial congenital cerebellar ataxia. The mother was 51-year-old and her PAN had about 4 minutes cycle. During eye closure there was a deviation of arms and trunk parallel to the direction of the slow phase of nystagmus. Vestibular stimulation during rotatory and caloric tests disclosed enhanced vestibulo-ocular reflexes, changes of direction and prolonged cycle of PAN. The patient had no visual suppression during caloric stimulation. The daughter was 14-year-old and although she had no spontaneous PAN she had latent PAN by showing changes of direction of nystagmus during the caloric stimulation. She had also no visual suppression during caloric stimulation. From these results the mechanism of PAN could be attributed to the periodic hyperfunction of vestibular nuclei caused by reduced suppression from cerebellar uvula and nodulus.
Sakakibara
R
R
Department of Neurology School of Medicine, Chiba University.
Hirayama
K
K
Takaya
Y
Y
Shinoto
H
H
Hattori
T
T
D000293:Adolescent; D002150:Caloric Tests; D002524:Cerebellar Ataxia / Q000150:complications* / Q000151:congenital / Q000235:genetics; D005260:Female; D006801:Humans; D008875:Middle Aged; D009759:Nystagmus, Pathologic / Q000209:etiology*; D010507:Periodicity; D012027:Reflex, Vestibulo-Ocular; D014726:Vestibular Nuclei / Q000503:physiopathology