Skip to main content

Advertisement

Log in

Posterior Fossa Syndrome After Cerebellar Stroke

  • Original Paper
  • Published:
The Cerebellum Aims and scope Submit manuscript

Abstract

Posterior fossa syndrome (PFS) due to vascular etiology is rare in children and adults. To the best of our knowledge, PFS due to cerebellar stroke has only been reported in patients who also underwent surgical treatment of the underlying vascular cause. We report longitudinal clinical, neurocognitive and neuroradiological findings in a 71-year-old right-handed patient who developed PFS following a right cerebellar haemorrhage that was not surgically evacuated. During follow-up, functional neuroimaging was conducted by means of quantified Tc-99m-ECD SPECT studies. After a 10-day period of akinetic mutism, the clinical picture developed into cerebellar cognitive affective syndrome (CCAS) with reversion to a previously learnt accent, consistent with neurogenic foreign accent syndrome (FAS). No psychometric evidence for dementia was found. Quantified Tc-99m-ECD SPECT studies consistently disclosed perfusional deficits in the anatomoclinically suspected but structurally intact bilateral prefrontal brain regions. Since no surgical treatment of the cerebellar haematoma was performed, this case report is presumably the first description of pure, “non-surgical vascular PFS”. In addition, reversion to a previously learnt accent which represents a subtype of FAS has never been reported after cerebellar damage. The combination of this unique constellation of poststroke neurobehavioural changes reflected on SPECT shows that the cerebellum is crucially implicated in the modulation of neurocognitive and affective processes. A decrease of excitatory impulses from the lesioned cerebellum to the structurally intact supratentorial network subserving cognitive, behavioural and affective processes constitutes the likely pathophysiological mechanism underlying PFS and CCAS in this patient.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1

Similar content being viewed by others

References

  1. Schmahmann JD, Sherman JC. The cerebellar cognitive–affective syndrome. Brain. 1998;121:561–79.

    Article  PubMed  Google Scholar 

  2. Schmahmann JD. Disorders of the cerebellum: ataxia, dysmetria of thought, and the cognitive affective syndrome. J Neuropsychiatry Clin Neurosci. 2004;16:367–78.

    Article  PubMed  Google Scholar 

  3. Pollack IF, Polinko P, Albright AL, Towbin R, Fitz C. Mutism and pseudobulbar symptoms after resection of posterior fossa tumors in children: incidence and pathophysiology. Neurosurgery. 1995;37:885–93.

    Article  PubMed  CAS  Google Scholar 

  4. Catsman-Berrevoets CE, Aarsen FK. The spectrum of neurobehavioural deficits in the posterior fossa syndrome in children after cerebellar tumour surgery. Cortex. 2010;46:933–46.

    Article  PubMed  Google Scholar 

  5. Mariën P, De Smet HJ, Wijgerde E, Verhoeven J, Crols R, De Deyn PP. The posterior fossa syndrome in adults: a new case and comprehensive survey of the literature. Cortex. 2013;49:284–300.

    Article  PubMed  Google Scholar 

  6. Dunwoody GW, Alsagoff ZS, Yuan SY. Cerebellar mutism with subsequent dysarthria in an adult: case report. Br J Neurosurg. 1997;11:161–3.

    Article  PubMed  CAS  Google Scholar 

  7. Coplin WM, Kim DK, Kliot M, Bird TD. Mutism in an adult following hypertensive cerebellar hemorrhage: nosological discussion and illustrative case. Brain Lang. 1997;59:473–93.

    Article  PubMed  CAS  Google Scholar 

  8. Idiaquez J, Fadic R, Mathias CJ. Transient orthostatic hypertension after partial cerebellar resection. Clin Auton Res. 2011;21:57–9.

    Article  PubMed  Google Scholar 

  9. De Smet HJ, Mariën P. Posterior fossa syndrome in an adult patient following surgical evacuation of an intracerebellar haematoma. Cerebellum. 2012;11:587–92.

    Article  PubMed  Google Scholar 

  10. Verhoeven J, Mariën P. Neurogenic foreign accent syndrome: articulatory setting, segments and prosody in a Dutch speaker. J Neurolinguistics. 2011;23:599–614.

    Article  Google Scholar 

  11. Mariën P, Verhoeven J, Engelborghs S, Rooker S, Pickut BA, De Deyn PP. A role for the cerebellum in motor speech planning: evidence from foreign accent syndrome. Clin Neurol Neurosurg. 2006;108:518–22.

    Article  PubMed  Google Scholar 

  12. Cohen DA, Kurowski K, Steven MS, Blumstein SE, Pascual-Leone A. Paradoxical facilitation: the resolution of foreign accent syndrome after cerebellar stroke. Neurology. 2009;73:566–7.

    Article  PubMed  CAS  Google Scholar 

  13. Folstein MF, Folstein SE, McHugh PR. Mini-mental state: a practical method for grading the cognitive state of patients for the clinician. J Psychiatr Res. 1975;12:189–98.

    Article  PubMed  CAS  Google Scholar 

  14. Wechsler D. Wechsler Adult Intelligence Scale—III (WAIS-III). London: The Psychological Corporation; 1997.

    Google Scholar 

  15. Randolph C. Repeatable Battery for the Assessment of Neuropsychological Status (RBANS). San Antonio: Psychological Corporation; 1998.

    Google Scholar 

  16. Raven JC. Progressive Matrices: a perceptual test of intelligence. London: Lewis; 1938.

    Google Scholar 

  17. Mariën P, Mampaey E, Vervaet A, Saerens J, De Deyn PP. Normative data for the Boston Naming Test in native Dutch speaking elderly. Brain Lang. 1998;65:447–67.

    Article  PubMed  Google Scholar 

  18. Reitan RM. Validity of the trail making test as an indicator of organic brain damage. Percept Mot Skills. 1958;8:271–6.

    Google Scholar 

  19. Golden CJ. Stroop Color and Word Test: a manual for clinical and experimental uses. Chicago: Stoelting; 1978.

    Google Scholar 

  20. Heaton RK, Chelune GJ, Talley JL, Kay GG, Curtiss G. Wisconsin Card Sorting Test: revised and expanded. Lutz: Psychological Assessment Resources; 1993.

    Google Scholar 

  21. Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: a frontal assessment battery at bedside. Neurology. 2000;55:1621–6.

    Article  PubMed  CAS  Google Scholar 

  22. Luria AR. Human brain and psychological processes. New York: Harper & Row; 1966.

    Google Scholar 

  23. De Deyn PP, Engelborghs S, Saerens J, Goeman J, Mariën P, Maertens K, et al. The Middelheim Frontality Score: a behavioural assessment scale that discriminates frontotemporal dementia from Alzheimer’s disease. Int J Geriatr Psychiatry. 2005;20:70–9.

    Article  PubMed  Google Scholar 

  24. Bohlhalter S, Goldfine A, Matteson S, Garraux G, Hanakawa T, Kansaku K, et al. Neural correlates of tic generation in Tourette syndrome: an event-related functional MRI study. Brain. 2006;129:2029–37.

    Article  PubMed  CAS  Google Scholar 

  25. Müller-Vahl KR, Kaufmann J, Grosskreutz J, Dengler R, Emrich HM, Peschel T. Prefrontal and anterior cingulate cortex abnormalities in Tourette syndrome: evidence from voxel-based morphometry and magnetization transfer imaging. BMC Neurosci. 2009;10:47. doi:10.1186/1471-2202-10-47.

    Article  PubMed  Google Scholar 

  26. Mariën P, Engelborghs S, Fabbro F, De Deyn PP. The lateralized linguistic cerebellum: a review and new hypothesis. Brain Lang. 2001;79:580–600.

    Article  PubMed  Google Scholar 

  27. Eidelberg D, Moeller JR, Antonini A, Kazumata K, Dhawan V, Budman C, et al. The metabolic anatomy of Tourette’s syndrome. Neurology. 1997;48:927–34.

    Article  PubMed  CAS  Google Scholar 

  28. Miller NG, Reddick WE, Kocak M, Glass JO, Löbel U, Morris B, et al. Cerebellocerebral diaschisis is the likely mechanism of postsurgical posterior fossa syndrome in pediatric patients with midline cerebellar tumors. AJNR Am J Neuroradiol. 2010;31:288–94.

    Article  PubMed  CAS  Google Scholar 

  29. Lagrèze HL, Levine RL, Pedula KL, Nickles RJ, Sunderland JS, Rowe BR. Contralateral flow reduction in unilateral stroke: evidence for transhemispheric diaschisis. Stroke. 1987;18:882–6.

    Article  PubMed  Google Scholar 

  30. Andrews RJ. Transhemispheric diaschisis: a review and comment. Stroke. 1991;22:943–9.

    Article  PubMed  CAS  Google Scholar 

  31. Lane R, Krikbride V, Hughes P, Jones B, Costa D. Diagnosis of herpes simplex encephalitis by single photon emission tomography. Lancet. 1989;1:778–9.

    Article  PubMed  CAS  Google Scholar 

  32. Conti F, Manzoni T. The neurotransmitters and postsynaptic actions of callosally projecting neurons. Behav Brain Res. 1994;64:37–53.

    Article  PubMed  CAS  Google Scholar 

  33. Iglesias S, Marchal G, Rioux P, Beaudouin V, Hauttement AJ, de la Sayette V, et al. Do changes in oxygen metabolism in the unaffected cerebral hemisphere underlie early neurological recovery after stroke? A positron emission tomography study. Stroke. 1996;27:1192–9.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgements

The authors wish to thank Mrs Inge Bats for preparing the figure and Mrs Ellen Wouters for the editorial assistance.

Conflict of Interest

The authors of the manuscript Peter Mariën, Lieven Verslegers, Maarten Moens, Guido Dua, Piet Herregods, and Jo Verhoeven explicitly disclose no conflicts of interests.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Peter Mariën.

Additional information

Peter Mariën and Lieven Verslegers contributed equally to the manuscript.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Mariën, P., Verslegers, L., Moens, M. et al. Posterior Fossa Syndrome After Cerebellar Stroke. Cerebellum 12, 686–691 (2013). https://doi.org/10.1007/s12311-013-0478-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12311-013-0478-7

Keywords

Navigation