Lower brainstem dysfunction in an infant with persistent primitive trigeminal artery.
A 6-month-old boy with persistent primitive trigeminal artery (PPTA) presented with stridor, dysphagia, delayed motor development and postural neck and shoulder dystonia. Magnetic resonance imaging/angiography and ultrasonography revealed PPTA, with flow from the dilated basilar artery to the right internal carotid artery, lower brainstem compression by the dilated basilar artery, and cerebellar vermis hypoplasia. Evoked potentials showed lower pons and medulla oblongata functional disruption. These lesions may be related to vascular etiology in the lower brainstem or to congenital malformation syndrome involving infratentorial structures. The relationship of this condition to Möbius syndrome is discussed.
Okanishi
Tohru
T
Division of Child Neurology, Institute of Neurological Sciences, Faculty of Medicine, Tottori University, 36-1 Nishi-cho, Yonogo 683-8504, Japan.
Saito
Yoshiaki
Y
Miki
Shiho
S
Nagaishi
Jun-Ichi
J
Hanaki
Keiichi
K
Tomita
Yutaka
Y
Fukuda
Chisako
C
Fujii
Shinya
S
Fujiwara
Kazunori
K
Kawamoto
Katsuyuki
K
Hata
Fumiko
F
Maegaki
Yoshihiro
Y
Ohno
Kousaku
K
D000015:Abnormalities, Multiple / Q000503:physiopathology; D001927:Brain Diseases / Q000209:etiology* / Q000503:physiopathology*; D001933:Brain Stem / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology*; D002536:Cerebral Arteries / Q000002:abnormalities* / Q000000981:diagnostic imaging / Q000473:pathology; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000473:pathology / Q000503:physiopathology; D016057:Evoked Potentials, Auditory, Brain Stem / Q000502:physiology; D006801:Humans; D007223:Infant; D018810:Magnetic Resonance Angiography; D008279:Magnetic Resonance Imaging; D008297:Male; D014463:Ultrasonography