Headache in Arnold-Chiari malformation type I – clinical cases and literature review


  • Ilyana Aleksandrova UHATNP "St.Naum"; Medical university – Sofia
  • A. Asenova UHATNP "St.Naum"; Medical university – Sofia
  • N. Topalov UHATNP "St.Naum"; Medical university – Sofia
  • V. Bojinova UHATNP „St. Naum”


Chiari type I malformation, occipital headache, Valsalva maneuver, idiopathic intracranial hypertension


Arnold Chiari type I malformation is characterized by elongation and protrusion of the cerebellar tonsils by more than 5 mm through foramen magnum. Headache is the most common initial clinical manifestation.
We present two cases of patients with headache with herniation of the cerebellar tonsils through foramen magnum. The first patient reports brief episodes of occipital headache typical for Chiari malformation, worsened by cough, as well as episodes of migraine headache with occipital or frontal localization.
In the second patient with headache the fundoscopy revealed papilledema and MRI showed mild protrusion (2.5 mm) of the cerebellar tonsils in foramen magnum. Chiari type I malformation can be asymptomatic or presents with a wide spectrum of neurologic symptoms, of which headache is the most common. Although brief episodes of headache with occipital/suboccipital localization, provoked by cough and other Valsalva-like maneuvers, are the hallmark of Chiari type I malformation, comorbidity with forms of primary headache is possible. The presence of bilateral papilledema without ventricular dilatation in a patient with cerebellar herniation makes the differential diagnosis with idiopathic intracranial hypertension very difficult and requires follow-up with MRI.


Abu-Arafeh, I., Campbell, E. Headache, Chiari malformation type 1 and treatment options. Arch Dis Child, 2017, 102, 3, 210-211.

Aitken, L., Lindan, C., Sidney, S., Gupta, N., Barkovich A. et.al. Chiari type I malformation in pediatric population. Pediatric Neurology, 2009, 40, 449-454.

Alperin, N., Loftus, J., Oliu, C., Bagci, A., Lee, S., Ertl-Wagner, B. et al. Imaging-Based Features of Headaches in Chiari Malformation Type I. Neurosurgery, 2015, 77, 1, 96-103.

Banik, R., Lin, D., Miller, N. Prevalence of Chiari I malformation and cerebellar ectopia in patients with pseudotumor cerebri. J Neurol Sci, 2006, 15, 247, 1, 71-75.

Beretta, E., Vetrano, I., Curone, M., Chiapparini, L., Furlanetto, M., Bussone, G., Valentini, L. Chiari malformation-related headache: outcome after surgical treatment. Neurol Sci, 2017, 38, Suppl 1, 95-98.

Bezuidenhout, A., Chang, Y., Heilman, C., Bhadelia, R. Headache in Chiari Malformation. Neuroimaging Clin N Am, 2019, 29, 2, 243-253.

Caldarelli, M., Di Rocco, C. Diagnosis of Chiari I malformation and related syringomyelia: radiological and neurophysiological studies. Childs Nerv Syst, 2004, 20, 332.

Curone, M., Valentini, L., Vetrano, I., Beretta, E., Furlanetto, M., Chiapparini, L. et al. Chiari malformation type 1-related headache: the importance of a multidisciplinary study. Neurol Sci, 2017, 38, Suppl 1, 91-93.

Headache Classification Committee of the International Headache Society (IHS). The International Classification of Headache Disorders, 3rd edition (beta version). Cephalalgia. 2013, 33, 9, 629-808.

Istek, S. Chiari type 1 malformation in a pseudotumour cerebri patient: is it an acquired or congenital Chiari malformation? BMJ Case Rep, 2014, 4, bcr2013201845.

Mehta, A., Chilakamarri, P.,, Zubair, A., Kuruvilla, D. Chiari Headache. Curr Pain Headache Rep, 2018, 14, 22, 7, 49.

Novegno, F., Caldarelli, M., Massa, A., Chieffo, D., Massimi, L., Pettorini, B. et al. The natural history of the Chiari Type I anomaly. J Neurosurg Pediatr, 2008, 2, 3, 179-187.

Olszewski, A., Proctor, M. Headache, Chiari I malformation and foramen magnum decompression. Curr Opin Pediatr, 2018, 30, 6, 786-790.

Pascual, J., Oterino,. A, Berciano, J. Headache in type I Chiari malformation. Neurology, 1992, 42, 8, 1519-1521.

Raza-Knight, S., Mankad, K., Prabhakar, P., Thompson, D. Headache outcomes in children undergoing foramen magnum decompression for Chiari I malformation. Arch Dis Child, 2017, 102, 3, 238-243.

Schwedt, T., Guo, Y., Rothner, A. “Benign” imaging abnormalities in children and adolescents with headache. Headache, 2006, 46, 387-398.

Toldo, I., Tangari, M., Mardari, R., Perissinotto, E., Sartori, S., Gatta, M. et al. Headache in children with Chiari I malformation. Headache, 2014, 54, 5, 899-908.

Vaphiades, M., Braswell, R.. Resolution of Chiari I malformation following acetazolamide therapy. Semin Ophthalmol, 2007, 22, 1, 9-11.

Victorio, M., Khoury, C. Headache and Chiari I Malformation in Children and Adolescents. Semin Pediatr Neurol, 2016, 23, 1, 35-39.

Williams, B. Cough headache due to craniospinal pressure dissociation. Arch Neurol, 1980, 37, 4, 226-230.



How to Cite

Aleksandrova, I., A. Asenova, N. Topalov, and V. Bojinova. “Headache in Arnold-Chiari Malformation Type I – Clinical Cases and Literature Review”. Cephalgia, vol. 24, no. 1, Dec. 2022, pp. 21-27, https://glavobolie.org/journal/index.php/ce/article/view/57.