Rinsho Shinkeigaku (Clinical Neurology)

Case Report

Posterior leukoencephalopathy syndrome presenting with bilateral ataxie optique: a case report

Kazuo Yamashiro, M.D.1), Makiko Kunoki, M.D.2), Yoshiharu Miura, M.D.3), Junji Tomiyama, M.D.4), Hideki Mochizuki, M.D.1) and Yoshikuni Mizuno, M.D.1)

1)Department of Neurology, Juntendo University School of Medicine
2)Department of Internal Medicine, Juntendo University School of Medicine
3)Department of Neurology, Metropolitan Bokutoh Hospital
4)Department of Internal Medicine, Metropolitan Bokutoh Hospital

We report a 45 years old right-handed woman who developed acute respiratory distress syndrome (ARDS) after the operation for rupture of an ovarian cyst. One week after the onset of ARDS, she presented visual disturbance and Gerstmann syndrome. MRI T2-weighted images demonstrated abnormal high intensity lesions involving the gray and white matter of the occipital lobes bilaterally extending to the parietal lobes. Based on these findings, she was diagnosed as having posterior leukoencephalopathy syndrome (PLES). Her neurologic symptoms gradually improved, however, she started to complain of difficulty in grasping a cup placed on her peripheral visual fields. Neurological examination revealed no visual disturbance, weakness or cerebellar ataxia. She could easily reach objects presented in the central visual field, however, she could not grasp objects presented peripheral visual fields, while she was looking straight ahead. This disorder was observed both in the right and left visual field, whether she used the right hand or the left hand. We thought she had ataxie optique of Garcin. She was noted to have bilateral both direct and crossed ataxie optique. Ataxie optique is characterized by disturbance in reaching objects presented in the peripheral visual field. The underlying pathophysiologic mechanism is believed to be disconnection of the fibers between the primary visual area and the angular gyrus at the parieto-occipital junction. Crossed ataxie optique consisting of difficulty in reaching objects presented in the contralateral visual field believed to be caused by disconnection of crossed pathways of the corpus callosum. Ataxie optique in our patient can be explained by disconnection of both direct and crossed fibers.
Ataxie optique is sometimes unrecognized by the patient. Our patient suggests that ataxie optique may well be a symptom of PLES.

(CLINICA NEUROL, 45: 13－17, 2005)
key words: ataxie optique, posterior leukoencephalopathy syndrome, Gerstmann syndrome

(Received: 27-Dec-03)