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


Дроздовская В.С., Гончарова Н.А., Смидович Л.Г. Клинические особенности, этиология и частота поражений зрительного нерва / В.С. Дроздовская, Н.А. Гончарова, Л.Г. Смидович // Проблеми сучасної медичної науки та освіти.- 2010.- №3. – С. 35-39.

Дзяк Л.А, Зорин Н.А., Егоров В.Ф., Чередниченко Ю.В. Мальформация Арнольда-Киари: классификация, этиопатогенез, клиника, диагностика (обзор литературы) / Л.А. Дзяк, Н.А. Зорин, В.Ф. Егоров, Ю.В. Чередниченко // Український нейрохірургічний журнал. – 2001. - №1. – С.17-23.

Кански Д. Клиническая офтальмология: систематизированный подход. Пер. с англ. // Д. Кански. – М.: Логосфера. – 2006.- 744с.

Кацнельсон Л.А., Форофонова Т.И., Бунин А.Я. Сосудистые заболевания глаза / Л.А. Кацнельсон, Т.И. Форофонова, А.Я. Бунин // Москва: Медицина, 1990. – 269 с.

Трон Е.Ж. Заболевания зрительного пути / Е.Ж. Трон // Л.: Медгиз. – 1955. – 388с.

Benjamin J. Osborne, Nicolas J. Volpe Optic neuritis and risk of MS: Differential diagnosis and

management / J. Benjamin Osborne, J. Nicolas //Cleveland Clinic Journal of Medicine. - 2009. - Vol. 76, № 3. – P. 181-190.

Caranci G., Mercurio A., Altieri M., Di Piero V. Trigeminal neuralgia as the sole manifestation of an Arnold-Chiari type I malformation: case report / G. Caranci, A. Mercurio, M. Altieri, V. Di Piero //Headache. – 2008. - Vol. 48, № 4. – P. 625-7.

Dolgun H., Turkoglu E., Kertmen H., Yilmaz E.R., Sekerci Z. Chiari Type I malformation presenting with bilateral hearing loss / H. Dolgun, E. Turkoglu, H. Kertmen, E.R. Yilmaz, Z. Sekerci // J. Clin. Neurosci. - 2009. – Vol. 16, № 9. – P. 1228-1230.

Haaland Stone WJ, Ittner EA, Teitelbaum BA, Messner LV. Progressive, asymptomatic papilledema as the presenting sign of a Chiari I malformation /W.J. Haaland Stone, E.A. Ittner, B.A. Teitelbaum, L.V. Messner // Optometry. 2012 Mar 30;83(3):114-9.

Henriques-Filho P.S., Pratesi R. Sleep apnea and REM sleep behavior disorder in patients with Chiari malformations / P.S. Henriques-Filho, R. Pratesi //Arq. Neuropsiquiatr. - 2008. - Vol. 66, № 2. – P. 344-349.

Henriques Filho P.S., Pratesi R. Sleep disorder: a possible cause of attention deficit in children and adolescents with Chiari malformation type II / P.S. Henriques-Filho, R. Pratesi // Arq. Neuropsiquiatr.– 2009.–Vol.67 № 1. – P. 29-34.

Heuer G.G., Gabel B., Lemberg P.S., Sutton L.N. Chiari I malformation presenting with hearing loss: surgical treatment and literature review / G.G. Heuer, B. Gabel, P.S. Lemberg, L.N. Sutton // Childs Nerv. Syst. – 2008. – Vol. 24, № 9. – P.1063-1066.

Holsgrove D., Leach P., Herwadkar A., Gnanalingham K.K. Visual field deficit due to downward displacement of optic chiasm / D. Holsgrove, P. Leach, A. Herwadkar, K.K. Gnanalingham // Acta Neurochir. - 2009. – Vol. 151, № 8. – P. 995-997.

Jacome D. Blepharoclonus and Arnold - Chiari Malformation / D. Jacome // Acta Neurol. Scand. – 2001. – Vol. 104, № 2. – P. 113-117.

Lucchetta M., Cagnin A., Calderone M. Syringomyelia associated with Chiari I malformation /M. Lucchetta, A. Cagnin, M. Calderone // Neurol Sci. – 2009. - Vol. 30, № 6. – P. 525-526.

Milhorat T.H., Chou M.W., Trinidad E.M. Chiari I malformation redefined: clinical and radiographic findings for 364 symptomatic patients / T.H. Milhorat, M.W. Chou, E.M. Trinidad // Neurosurgery. 1999 May; 44(5):1005-17.

Miralbés Terraza S., García Oguiza A., López Pisón J., Sáenz Moreno I. Central sleep apnea syndrome as the first sign of type I Chiari malformation / S. Miralbés Terraza, A. García Oguiza, J. López Pisón, I. Sáenz Moreno // An. Pediatr. – 2008. - Vol. 68, № 3. – P. 277-281.

Nacir B., Arslan Cebeci S., Cetinkaya E., Karagoz A. Neuropathic arthropathy progressing with multiple joint involvement in the upper extremity due to syringomyelia and type I Arnold-Chiari malformation / B. Nacir, S. Arslan Cebeci, E. Cetinkaya, A. Karagoz // Rheumatol. Int. - 2009. – Vol. 26, № 2. – P. 126-131.

Obuchowska I., Mariak Z. Internuclear ophthalmoplegia--causes, symptoms and management / I. Obuchowska, Z. Mariak // Klin Oczna. – 2009. – Vol.111, № 4-6. P. 165-167.

Optic Neuritis Study Group. High- and Low-Risk Profiles for the Development of Multiple Sclerosis Within 10 Years After Optic Neuritis // Arch. Ophthalmol.– 2003.– Vol. 121. – P. 944-9.

Papanastassiou A.M., Schwartz R.B., Friedlander R.M. Chiari I malformation as a cause of trigeminal neuralgia: case report / A.M. Papanastassiou, R.B. Schwartz, R.M. Friedlander // Neurosurgery. – 2008. – Vol. 63, № 3. – P. 614-615.

Pardo Muñoz A., Reche Sainz J.A., Sanz López A., Díaz Orro B. A case of papilledema and Arnold-Chiari type I malformation / A. Pardo Muñoz, J.A. Reche Sainz, A. Sanz López, B. Díaz Orro //Arch Soc. Esp. Oftalmol. – 2002. – Vol. 77, № 8. – P. 449-453.

Pilon A., Rhee P., Newman T., Messner L. Bilateral abducens palsies and facial weakness as initial manifestations of a Chiari 1 malformation / A. Pilon, P. Rhee, T. Newman, L. Messner // Optom. Vis. Sci.–2007.– Vol. 84, № 10.– P. 936-940.

Pruszewicz A.,Wojnowski W., Jackowska J. Tinnitus as the first symptom of Arnold-Chiari-Syndrome / A. Prusze-wicz, W. Wojnowski, J. Jackowska //Otolaryngol. Pol. – 2007. – Vol. 61, № 6. – P. 998-999.

Salman M.S., Sharpe J.A., Lillakas L., Dennis M. Visual fixation in Chiari type II malformation / M.S. Salman, J.A. Sharpe, L. Lillakas, M. Dennis // J. Child Neurol. - 2009. – Vol. 24, № 2. – P.161-165.

Sami H. Erbay, Mark G. O’Callaghan , Bhadelia R. Is Lumbar Puncture Contraindicated in Patients with Chiari I Malformation? / H. Erbay Sami, Mark G. O’Callaghan, R. Bhadelia //American Journal of Neuroradiology. – 2005. – Vol. 26, № 4. – P. 365-366.

Staudenmaier C., Buncic J. R. Periodic Alternating Gaze Deviation With Dissociated Secondary Face Turn / C. Staudenmaier, J. R. Buncic // Arch Ophthalmol. – 1983. – Vol. 101, № 2. – P. 202-205.

Straus D., Foster K., Zimmerman F., Frim D. Chiari drop attacks: surgical decompression and the role of tilt table testing / D. Straus, K. Foster, F. Zimmerman, D. Frim // Pediatr Neurosurg. – 2009. – Vol. 45, № 5. – P. 384-389.

Vaphiades M.S., Braswell R. Resolution of Chiari I malformation following acetazolamide therapy / M.S. Vaphia-des, R. Braswell // Semin Ophthalmol. – 2007. – Vol. 22, № 1. – P. 9-11.

Yee R. D., Baloh R. W., Honrubia V. Episodic Vertical Oscillopsia and Downbeat Nystagmus in a Chiari Malformation / R. D. Yee, R. W. Baloh, V. Honrubia // Arch Ophthalmol. - 1984. – Vol.102, № 5. – P. 723-725.


  • There are currently no refbacks.

Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 International License.


© "Publishing House "Zaslavsky", 1997-2019


   Seo анализ сайта