Yamazaki K, Katayama S, Hirata K, Ishihara T, Odaka M
Department of Neurology, Dokkyo University School of Medicine.
Rinsho Shinkeigaku. 1994 Oct;34(10):1046-51.
The patient was a 26-year-old male who was noted to have asymmetry on the face, upper limbs, and trunk, and who showed no pursuit eye movements and no changes in the facial expression from birth. The patient developed transient global amnesia and was hospitalized. Neurological examination established atrophy of the right face, and left trunk involving the upper limb, and bilateral oculomotor and abducens nerve palsy. In auditory brainstem response, waves III to V were bilaterally obscured, and the blink reflex disclosed disturbances of peripheral facial nerve and the brainstem, suggesting developmental abnormalities of the facial motor nucleus. Contralateral R1 was also recorded in the blink reflex, and crossed trigeminofacial pathway was suspected. Möbius syndrome is associated with various muscle and skeletal abnormalities, but the coexistence of crossed total hemiatrophy has not been reported. We think that our case might be the first case of Möbius syndrome with total hemiatrophy.
