J Neurol Surg B Skull Base 2024; 85(02): 189-201
DOI: 10.1055/a-2028-6373
Original Article

Emotional Lability as a Symptom of Extra-axial Posterior Fossa Tumors: a Case–Control Review of Neuroanatomy and Patient-Reported Quality of Life

1   Division of Neurosurgery, University of British Columbia Faculty of Medicine, Vancouver, Canada
2   University of Alberta Faculty of Medicine, Edmonton, Canada
,
Peter Gooderham
1   Division of Neurosurgery, University of British Columbia Faculty of Medicine, Vancouver, Canada
,
Ryojo Akagami
1   Division of Neurosurgery, University of British Columbia Faculty of Medicine, Vancouver, Canada
› Author Affiliations

Abstract

Introduction Emotional lability (EL), the uncontrollable and unmotivated expression of emotion, is a rare and distressing symptom of brainstem compression. In published case reports, EL from an extra-axial posterior fossa tumor was alleviated by tumor resection. The primary aim herein was to radiographically establish the degree of compression from mass lesions onto brainstem structures. Secondarily, we compared changes in patient-reported quality of life (QOL) pre- and postoperatively.

Methods A retrospective review of posterior fossa tumors treated between 2002 and 2018 at Vancouver General Hospital revealed 11 patients with confirmed EL. Each case was matched to three controls. A lateral brainstem compression scale characterized mass effect at the level of the medulla, pons, and midbrain in preoperative axial T2-weighted fluid-attenuated inversion recovery magnetic resonance imaging (FLAIR MRI) scans. Compression and clinical variables were compared between patient groups. Short Form-36 version 1 health surveys were retrospectively obtained from patient charts to compare pre- versus postoperative changes in survey scores between EL and control patients.

Results EL symptoms ceased postoperatively for all EL patients. EL tumors exert greater compression onto the pons (p = 0.03) and EL patients more commonly have cerebellar findings preoperatively (p = 0.003). Patients with EL-causing tumors experienced greater improvement postoperatively in “Health Change” (p = 0.05), which was maintained over time.

Conclusion Findings suggest that compression onto the pons inhibits control over involuntary, stereotyped expression of emotion. This adds to evidence that EL may be attributed to cerebellum deafferentation from cortical and limbic structures through the basis pontis, leading to impaired modulation of emotional response. QOL results augment benefits of offering patients EL-alleviating tumor resection surgery.



Publication History

Received: 11 November 2022

Accepted: 29 January 2023

Accepted Manuscript online:
04 February 2023

Article published online:
10 March 2023

© 2023. Thieme. All rights reserved.

Georg Thieme Verlag KG
Rüdigerstraße 14, 70469 Stuttgart, Germany

 
  • References

  • 1 Rapalino O, Smirniotopoulos JG. Extra-axial brain tumors. Handb Clin Neurol 2016; 135: 275-291
  • 2 Nicholls JG, Paton JF. Brainstem: neural networks vital for life. Philos Trans R Soc Lond B Biol Sci 2009; 364 (1529): 2447-2451
  • 3 Ringer A, Bohinski R. Chiari I Malformation & Syringomyelia. Mayfield Clinic Health Information; 2018
  • 4 Parvizi J, Anderson SW, Martin CO, Damasio H, Damasio AR. Pathological laughter and crying: a link to the cerebellum. Brain 2001; 124 (Pt 9): 1708-1719
  • 5 LeDoux JE. Emotion circuits in the brain. Annu Rev Neurosci 2000; 23 (01) 155-184
  • 6 Virani MJ, Jain S. Trigeminal schwannoma associated with pathological laughter and crying. Neurol India 2001; 49 (02) 162-165
  • 7 Meininger V. Treatment of emotional lability in ALS. Lancet Neurol 2005; 4 (02) 70
  • 8 Ghaffar O, Chamelian L, Feinstein A. Neuroanatomy of pseudobulbar affect : a quantitative MRI study in multiple sclerosis. J Neurol 2008; 255 (03) 406-412
  • 9 Arciniegas DB, Wortzel HS. Emotional and behavioral dyscontrol after traumatic brain injury. Psychiatr Clin North Am 2014; 37 (01) 31-53
  • 10 House A, Dennis M, Molyneux A, Warlow C, Hawton K. Emotionalism after stroke. BMJ 1989; 298 (6679): 991-994
  • 11 Rapalino O, Smirniotopoulos JG. Extra-axial brain tumors. In: Masdeu JC, González RG. eds. Handbook of Clinical Neurology. Vol. 135. Elsevier; 2016: 275-291
  • 12 Cheng T-J, Ke DS, Tsai T-C. Petroclival meningioma presenting with pathological laughter: report of a case and review of the literature. Acta Neurol Taiwan 2003; 12 (04) 187-190
  • 13 Muzumdar D, Agrahar P, Desai K, Goel A. Pathological laughter as a presenting symptom of petroclival meningioma–case report. Neurol Med Chir (Tokyo) 2001; 41 (10) 505-507
  • 14 McCormick WE, Lee JH. Pseudobulbar palsy caused by a large petroclival meningioma: report of two cases. Skull Base 2002; 12 (02) 67-71
  • 15 Cantu RC, Drew JH. Pathological laughing and crying associated with a tumor ventral to the pons. Case report. J Neurosurg 1966; 24 (06) 1024-1026
  • 16 Tsutsumi S, Hatashita S, Kadota Y, Abe K, Ueno H. Tentorial meningioma associated with pathological laughter–case report. Neurol Med Chir (Tokyo) 2000; 40 (05) 272-274
  • 17 Hassan I, Sahjpaul R, Tan JE, Moien Afshari F. Pseudobulbar affect due to skull base meningioma resolving after temporal lobectomy for epilepsy. Can J Neurol Sci 2018; 45 (04) 485-486
  • 18 Bhatjiwale MG, Nadkarni TD, Desai KI, Goel A. Pathological laughter as a presenting symptom of massive trigeminal neuromas: report of four cases. Neurosurgery 2000; 47 (02) 469-471 , discussion 471–472
  • 19 Nadkarni TD, Goel A. Trochlear nerve neurinoma presenting as pathological laughter. Br J Neurosurg 1999; 13 (02) 212-213
  • 20 Matsuoka S, Yokota A, Yasukouchi H. et al. Clival chordoma associated with pathological laughter. Case report. J Neurosurg 1993; 79 (03) 428-433
  • 21 Stevenson GC, Stoney RJ, Perkins RK, Adams JE. A transcervical transclival approach to the ventral surface of the brain stem for removal of a clivus chordoma. J Neurosurg 1966; 24 (02) 544-551
  • 22 Achari AN, Colover J. Posterior fossa tumors with pathological laughter. JAMA 1976; 235 (14) 1469-1471
  • 23 Lal AP, Chandy MJ. Pathological laughter and brain stem glioma. J Neurol Neurosurg Psychiatry 1992; 55 (07) 628-629
  • 24 Famularo G, Corsi FM, Minisola G, De Simone C, Nicotra GC. Cerebellar tumour presenting with pathological laughter and gelastic syncope. Eur J Neurol 2007; 14 (08) 940-943
  • 25 Hargrave DR, Mabbott DJ, Bouffet E. Pathological laughter and behavioural change in childhood pontine glioma. J Neurooncol 2006; 77 (03) 267-271
  • 26 Parvizi J, Coburn KL, Shillcutt SD, Coffey CE, Lauterbach EC, Mendez MF. Neuroanatomy of pathological laughing and crying: a report of the American Neuropsychiatric Association Committee on Research. J Neuropsychiatry Clin Neurosci 2009; 21 (01) 75-87
  • 27 Parvizi J, Joseph J, Press DZ, Schmahmann JD. Pathological laughter and crying in patients with multiple system atrophy-cerebellar type. Mov Disord 2007; 22 (06) 798-803
  • 28 Calvert T, Knapp P, House A. Psychological associations with emotionalism after stroke. J Neurol Neurosurg Psychiatry 1998; 65 (06) 928-929
  • 29 Schiffer R, Pope LE. Review of pseudobulbar affect including a novel and potential therapy. J Neuropsychiatry Clin Neurosci 2005; 17 (04) 447-454
  • 30 Gil Z, Fliss DM. Quality of life in patients with skull base tumors: current status and future challenges. Skull Base 2010; 20 (01) 11-18
  • 31 Tufarelli D, Meli A, Alesii A. et al. Quality of life after acoustic neuroma surgery. Otol Neurotol 2006; 27 (03) 403-409
  • 32 Ware Jr JE, Sherbourne CD. The MOS 36-item short-form health survey (SF-36). I. Conceptual framework and item selection. Med Care 1992; 30 (06) 473-483
  • 33 Wilson SAK. Some problems in neurology. II: Pathological laughing and crying. J Neurol Psychopathol 1924; IV: 299-333
  • 34 Shafqat S, Elkind MSV, Chiocca EA. et al. Petroclival meningioma presenting with pathological laughter. Neurology 1998; 50 (06) 1918-1919