Cerebellar cognitive affective syndrome related to chiari malformation: a case report

Abstract

Numerous studies have showed that the cerebellum has a role in processing higher brain functions besides motor coordination. Various types of congenital lesions of cerebellum are associated with neuropsychological impairments and behavioral changes. In Chiari malformation, the cerebellar structures have been stuck in posterior fossa. In this paper a case of Chiari malformation, presenting with affective instability and behavioral disinhibition was described.

Keywords

• cerebellum, affect, behavior, Chiari malformation

Chiari malformasyonuna bağlı serebellar kognitif affektif sendrom: Bir olgu sunumu

Öz

Serebellumun, motor hareketlerin koordinasyonunun yanı sıra, yüksek beyin fonksiyonlarının gerçekleşmesinde rolü olduğu birçok çalışmada gösterilmiştir. Serebellumun konjenital lezyonları çeşitli nöropsikolojik bozukluklar ve davranış değişiklikleri ile ilişkilendirilmiştir. Chiari malformasyonunda, serebellar yapılar posterior fossa içinde sıkışmış halde bulunurlar. Bu yazıda duygusal instabilite ve davranışsal disinhibisyon belirtileri ile giden bir Chiari malformasyonu olgusu sunulmuştur.

Anahtar Kelimeler

• serebellum, afekt, davranış, Chiari malformasyonu

References

1. Holmes G. A form of familial degeneration of the cerebellum. Brain 1907;30:466-88.
2. Strick PL, Dum RP, Fiez JA. Cerebellum and nonmotor function. Annu Rev Neurosci 2009;32:413-34.
3. Alexander MP, Gillingham S, Schweizer T, Stuss DT. Cognitive impairments due to focal cerebellar injuries in adults. Cortex 2012;48:980-90.
4. Leggio MG, Tedesco AM, Chiricozzo FR, Clausi S, Orsini A, Molinari M. Cognitive sequencing impairments in patients with focal or atrophiccerebellar damage. Brain 2008;131:1332-43.
5. Wallesch CW, Horn A. Long-term effects of cerebellar pathology on cognitive functions. Brain Cogn 1990;14:19-25.
6. Fiez JA, Raichle ME. Linguistic processing. Schmahmann JD, editör. The cerebellum and cognition. International Review of Neurobiology içinde. San Diego: Academic Press;1997; s. 233-354.
7. De Smet HJ, Mariën P. Posterior fossa syndrome in an adult patient following surgical evacuation of an intracerebellar haematoma. Cerebellum 2012;11:587-92.
8. Levisohn L, Cronin-Golomb A, Schmahmann JD. Neuropsychological sequelae of cerebellar tumors in children. Soc Neurosci Abstr. 1997;23:496.

9. Longcamp M, Anton JL, Roth M, Velay JL. Visual presentation of single letters activates a premotor area involved in writing. NeuroImage. 2003;19:1492-500.
10. Schmahmann JD. From movement to thought: anatomic substrates of the cerebellar contribution to cognitive processing. Hum Brain Mapp 1996;4:74-98.
11. Schmahmann JD, Sherman JC. The cerebellar cognitive affective syndrome. Brain 1998;121:561-79.
12. Bolduc M, Limperopoulos C. Neurodevelopmental outcomes in children with cerebellar malformation: a systematic review. Dev Med Child Neurol. 2008;51:256-67.
13. Riva D, Giorgi C. The cerebellum contributes to higher functions during development. Brain 2000;123:1051-61.
14. Daniel PM, Strich SJ. Some observations on the congenital deformity of the central nervous system known as the ArnoldChiari malformation. J Neurol Neurosurg Psychiatry. 1958;17:255
15. Çayköylü A, Deniz O, Aygül A. Depresif yakınmalarla başvuran bir Arnold-Chiari Tip I olgusu. AÜTD 2001;33:83-5.
16. Iwabuchi K, Miyauchi T, Kyuuma Y, et al. A sudden-death in a case of Arnold Chiari malformation (type I) with sleep apnea. No-To Shinkei 1985;37:575-81.
17. Chisholm BT, Velamoor R, Chandarana PC, Cochrane DK. Anxiety disorder in a case of Arnold Chiari malformation. J Psychiatr Neurosci 1993;18:67-8.
18. The Ataxia Neuropharmacology Committee of the World Federation of Neurology. International Cooperative Ataxia Rating Scale for pharmacological assessment of the cerebellar syndrome. J Neurol Sci 1997;145:205-11.
19. Neary D, Snowden J, Mann D. Frontotemporal Dementia. Lancet, Neurol 2005;4:771-80.
20. Mesulam MM. Principles of behavioral neurology. Philadelphia: FA Davis; 1985.
21. Baillieux H, De Smet HJ, Dobbeleir A, Paquier P F, De Deyn PP, Mariën P. Cognitive and affective disturbances following focal cerebellar damage in adults: A neuropsychological and SPECT study. Cortex 2010; 46:869-79.
22. Desmond JE. Cerebellar involvement in cognitive function: Evidence from neuroimaging. International Review of Psychiatry 2001;13:283-94.
23. Stoodley CJ, Valera EM, Schmahmann JD. Functional topography of the cerebellum for motor and cognitive tasks: An fMRI study. NeuroImage 2012;59:1560-70.