Arnold-Chiari malformation can present with internuclear ophthalmopleg
ia, sixth nerve paralysis, and transient or variable wave-form nystagm
us. The differential diagnosis of a fluctuating ocular deviation shoul
d include Arnold-Chiari malformation as well as myasthenia gravis and
multiple sclerosis. Downward displacement of the cerebellum can even s
imulate cerebellar atrophy on computed tomography. The presence of ske
letal, lower motor neuron, and lower bulbar signs may alert the clinic
ian. Once the etiology is suspected, magnetic resonance imaging (to in
clude the foramen magnum) will determine the diagnosis. Clinical manag
ement should be individually tailored. In our experience, one patient
required foramen magnum decompression. A recession resection with adju
stable sutures was performed in two patients.