An optic neuritis associated with Arnold-Chiari malformation

K.P. Pavlyuchenko, I.G. Zykov, N.I. Chizhevskaya


The introduction. Arnold-Chiari malformation associated with an optic neuritis has not been reported in the literature. Type I Arnold-Chiari malformation consists on the caudal displacement of cerebellar tonsils through the foramen magnum. It is often asymptomatic, although it may display symptoms as a result of cerebellum, brainstem, high cervical spinal cord or the lower cranial nerve, involvement.
Diagnostically, the fundus picture of an optic neuritis may be confused with a few other entities.
Papilledema, retrobulbar neuritis, central retinal vein occlusion and anterior ischemic optic neuropathy are the close mimickers funduscopically. Differentiation, however, is not difficult by performing detailed ophthalmic, nervous system and general examination. For early revealing the etiology of an optic neuritis, great value has Magnetic resonance imaging of the brain.
Materials and methods. A 40-year-old female presented with a progressive decrease in vision in the left eye for two weeks. There was no fever or other symptoms at the onset of this problem.
Results and discussion. On examination, the best-corrected visual acuity was 1,0 in the right eye and 0,04 in the left eye. The left optic disc was with blurred margins. The veins were dilated and tortuous. Central fields of this patient revealed a central scotoma in the left eye and normal right eye fields. MRI of the brain revealed Arnold– Chiari I malformation. A general physical examination of the patient was also normal.
The vision improved to 0,8 and the optic nerve swelling had decreased after a treatment of two weeks .
Eight months later, the fundus became normal with the return of visual acuity to 1,0. No neurological symptoms supervened at any time. The patient remained asymptomatic during a follow-up period of the next one year.
Conclusions. Arnold– Chiari I malformation-associated optic neuritis is rare. However, it is important that ophthalmologists differentiate ACM-associated optic neuritis from multiple sclerosis-associated optic neuritis because of the different treatment. Nuclear magnetic resonance imaging studies are important for early diagnosis and prompt treatment in these patients.


optic neuritis, Arnold – Chiari malformation


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