The incidence of Arnold–Chiari malformation in neurological practice

Arnold-Chiari malformation (ACM) is a group of congenital hindbrain malformations affecting the structural relationships between the cerebellum, brain stem, top cervical spinal cord, and bones of the skull base. In clinical practice, ACM types 0 and 1 are more common and types 2, 3, and 4 belong to rare severe (often fatal) congenital malformations.

Objective: to study gender differences in the incidence of ACM types 0 and 1 in outpatient neurological practice.

Patients and methods. A total 2039 case records of outpatients who had visited a neurologist of the Krasnoyarsk University clinic in 2008–2014 were analyzed. Neurological and neuroradiological (1.5 Tesla brain magnetic resonance imaging, phase-contrast spinal cerebral fluid flow imaging) diagnostic techniques were used. 3.4% (70/2039) of cases were randomized according to the criteria of inclusion and exception. An entire sample included 70 ACM patients (median age, 25 [17; 34] years) (30 (42.8–7.1%) men and 40 (57.2–7.1%) women).

Results. There was an increase in the incidence of ACM type 1 in the women than in the men. Conclusion. It is necessary to develop a new strategy for the prophylactic medical examination of patients with ACM to improve primary and specialized outpatient health care.

1. Milhorat TM, Nishikawa M, Kula RW, et al. Mechanisms of cerebellar tonsil herniation in patients with Chiari malformations as a guide to clinical management. Acta Neurochir (Wien). 2010 Jul;152(7):1117-27. https://doi.org/10.1007/s00701-010-0636-3. Epub 2010 May 4.

2. Fatkullina IB, Tudupova BB, Alekseeva LL. Rezul'taty sutochnogo monitoringa arterial'nogo davleniya v differentsial'noi diagnostike arterial'noi gipertenzii pri beremennosti u zhenshchin Respubliki Buryatiya. Nauchnoe obozrenie. 2009;(6):31-7. [Fatkullina IB, Tudupova BB, Alekseeva LL. The results of daily blood pressure monitoring in the differential diagnosis of hypertension in pregnancy in women of the Republic of Buryatia. Nauchnoe obozrenie. 2009;(6):31-7. (In Russ.)].

3. Hofkes SK, Iskandar BJ, Turski PA, et al. Differentiation between symptomatic Chiari I malformation and asymptomatic tonsilar ectopia by using cerebrospinal fluid flow imaging: initial estimate of imaging accuracy. Radiology. 2007 Nov;245(2):532-40. Epub 2007 Sep 21.

4. McGirt MJ, Nimjee SM, Fuchs HE, George TM. Relationship of cine phase-contrast magnetic resonance imaging with outcome after decompression for Chiari I malformations. Neurosurgery. 2006 Jul;59(1):140-6.

5. Unal M, Bagdatoglu C. Arnold-Chiari type I malformation presenting as benign paroxysmal positional vertigo in an adult patient. J Laryngol Otol. 2007 Mar;121(3):296-8. Epub 2006 Dec 14.

6. Kantimirova EA, Shnaider NA, Petrova MM, Alekseeva OV. Narusheniya sna u zhenshchin. Problemy zhenskogo zdorov'ya. 2014;9(4):57-62. [Kantimirova EA, Shnaider NA, Petrova MM, Alekseeva OV. Sleep disorders in women. Problemy zhenskogo zdorov'ya. 2014;9(4):57-62. (In Russ.)].

7. Fedirko VA. Sindromy neirovaskulyarnoi kompressii obrazovanii zadnei cherepnoi yamki v sochetanii s anomaliei Kiari. Ukrainskii neirokhirurgicheskii zhurnal. 2006;(4):74-9. [Fedirko VA. Syndromes of compression of neurovascular formations of the posterior cranial fossa in combination with Chiari malformation. Ukrainskii neirokhirurgicheskii zhurnal. 2006;(4):74-9. (In Russ.)].

8. Murray C, Seton C, Prelog K, Fitzgerald DA. Arnold-Chiari type 1 malformation presenting with sleep disordered breathing in well children. Arch Dis Child. 2006 Apr;91(4):342-3.

9. Cirignotta F, Coccagna G, Zucconi M, et al. Sleep apneas, convulsive syncopes and autonomic impairment in type I Arnold-Chiari malformation. Eur Neurol. 1991;31(1):36-40.

10. Speer MC, Enterline DS, Mehltretter L, et al. Chiari type I malformation with or without syringomyelia: prevalence and genetics. J Genet Couns. 2003 Aug;12(4):297-311. https://doi.org/10.1023/A:1023948921381.

11. Latysheva VYa, Olizarovich MV, Filyustin AE, Gurko NA. Kliniko-tomograficheskie sootnosheniya pri sindrome Arnol'da-Kiari. Mezhdunarodnyi nevrologicheskii zhurnal. 2011;(7):6-11. [Latysheva VYa, Olizarovich MV, Filyustin AE, Gurko NA. Clinical-tomographic correlation in the syndrome Arnold-Chiari. Mezhdunarodnyi nevrologicheskii zhurnal. 2011;(7):6-11. (In Russ.)].