[Type I neurofibromatosis and megadolichobasilar artery].
Intracranial vascular malformations are rare but tend to appear more frequently than usual in patients with type I neurofibromatosis (NFI). Aneurysms of the basilar artery have been described four times so far. We report two cases of 51- and 62-year-old patients with type I neurofibromatosis who showed long fusiform dilation of the basilar artery. Clinically both patients presented with locked-in syndrome and died 15 and 11 days after admission. The diagnosis was confirmed by autopsy. These are the first published cases of locked-in syndrome following thrombosis of a megadolichobasilar artery in association with neurofibromatosis I. Our results show that cerebral vascular malformations are found more frequently than random chance would predict in patients with NF I.
Roth
C
C
Neurologische Klinik, Institut für Pathologie, Klinikum Kassel. roth99@web.de
Gaiser
T
T
Nagelmeier
I
I
Rüschoff
J
J
Ferbert
A
A
D001488:Basilar Artery / Q000473:pathology; D020526:Brain Stem Infarctions / Q000175:diagnosis / Q000235:genetics; D002533:Cerebral Angiography; D002869:Chromosome Aberrations; D017809:Fatal Outcome; D006801:Humans; D002532:Intracranial Aneurysm / Q000175:diagnosis* / Q000235:genetics; D018810:Magnetic Resonance Angiography; D008297:Male; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000175:diagnosis* / Q000235:genetics; D009460:Neurologic Examination; D011149:Pons / Q000473:pathology; D014057:Tomography, X-Ray Computed; D014715:Vertebrobasilar Insufficiency / Q000175:diagnosis* / Q000235:genetics