CASE REPORT

Pseudobulbar Palsy Caused bya Large Petroclival Meningioma:Report ofTwo CasesWilliam E. McCormick, M.D.1 and Joung H. Lee, M.D.'

ABSTRACT

Two patients sought treatment for symptoms of bulbar motor dysfunc-tion and the marked emotional lability that characterizes pseudobulbar palsy(PBP). Magnetic resonance (MR) imaging showed large petroclival masses withsevere compression and displacement of the brainstem. A suboccipital craniec-tomy with a transpetrosal, transtentorial approach to the tumor was performed inboth patients. One patient required a second-stage surgery to resect an extensionof the tumor into the cavernous sinus. In both patients pathological evaluationconfirmed the diagnosis of syncytial meningioma. After surgery, PBP resolved inboth patients. Large posterior fossa tumors should be included in the differentialdiagnosis of patients with a clinical picture of bulbar motor dysfunction coupledwith emotional lability.

KEYWORDS: Meningioma, pathological laughter, pseudobulbar palsy, tumor

In 1837 Magnus reported the first case ofpseudobulbar palsy (PBP) in a patient who hadsuffered multiple infarcts.1 The term pseudobulbarpalsy, however, was not coined until 1877 whenLepine used it to differentiate lower cranial nervepalsies caused by supranuclear lesions from cranialnerve palsies caused by lesions of the bulbarnuclei.2 PBP is characterized by dysarthia, dyspha-gia, facial and tongue weakness, and emotional la-bility. It is caused by bilateral dysfunction of the

corticobulbar tracts, which exert supranuclear con-trol over the brainstem motor nuclei that, in turn,control mastication, speech, and deglutition.3 Animportant difference from primary disease of thebulbar motor nuclei is the spontaneous, involun-tary laughing or crying associated with PBP. In ad-dition, the jaw jerk reflex typically becomes exag-gerated in PBP. Unlike lower motor neuron bulbarparalysis, there is no atrophy or fasciculations ofthe affected muscles.

Skull Base, volume 12, number 2,2002. Address for correspondence and reprint requests: Joung H. Lee, M.D., Section of Skull Base Surgery,Department of Neurosurgery/S80, Cleveland Clinic Foundation, 9500 Euclid Avenue, Cleveland, OH 44195. E-mail: leej@ccf.org.'Department of Neurosurgery, The Cleveland Clinic Foundation, Cleveland, Ohio. Copyright C 2002 byThieme Medical Publishers, Inc.,333 Seventh Avenue, New York, NY 10001, USA. Tel: +1(212) 584-4662. 1531-5010,p;2002,12,02,067,072,ftx,en;sbsOO266x.

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PBP is commonly described in conditionscausing bilateral corticobulbar lesions such as amyo-trophic lateral sclerosis, lacunar infarcts, or de-myelinating conditions such as multiple sclerosis.4One of the abnormal expressions of emotion some-times described in patients with PBP is pathologi-cal laughter, which is characterized by involuntary,uncontrollable, inappropriate laughter. Pathologicallaughter alone has been reported in association withmultiple tumors of the posterior fossa, particularlythose involving the pons and midbrain. Both extra-axial tumors5-12 and intra-axial tumors13-16 of theposterior fossa have been associated with this con-dition. Unlike pathological laughter, PBP in associ-ation with posterior fossa neoplasms is extremelyrare. We present two patients with reversible PBPassociated with large petroclival meningiomas.

ILLUSTRATIVE CASES

transpetrosal, transtentorial approach about 60% ofthe tumor was resected, leaving the extension inthe cavernous sinus and petrous apex. Three weekslater, she underwent a left frontotemporal cra-niotomy to resect the residual tumor (Fig. 1). Post-operatively, the patient developed delayed onsetcerebrospinal fluid rhinorrhea, which was treatedby repacking the temporal bone with a muscle andfat graft. Histological examination identified a be-nign syncytial meningioma.

The patient was in excellent condition aftersurgery. Her preoperative subjective swallowing dif-ficulties, emotional outbursts, and cerebellar ataxiaresolved completely. She exhibited mild hypesthesiain the left Vl and V2 distribution and left-sidedhearing loss. Imaging studies revealed no evidenceof tumor until 5 years after surgery. At that timeMRI showed a small recurrence in the petroclivalregion. She has no new complaints and is scheduledto undergo Gamma KnifeTM (Elekta, Stockholm,Sweden) treatment.

Case 1

A 37-year-old woman had several months' historyof spontaneous laughing spells and episodes ofuncontrollable crying. These emotional outburstswere embarrassing and distressing to her. She alsoreported progressive dysphagia as well as difficul-ties with balance and gait. On neurological exami-nation, she exhibited mild dysmetria in the leftupper and lower extremities. Her cranial nerve ex-

amination was unremarkable and a gag reflex was

present. Her extremity reflexes were brisk bilater-ally and plantar responses were extensor. She alsohad a moderately ataxic gait. Magnetic resonance

imaging (MRI) revealed a large, left-sided mass inthe petroclival region that extended into the cav-

ernous sinus (Fig. 1). The brainstem was severelycompressed and displaced.

Because the tumor extended into the cav-

ernous sinus from the petroclival region, the pa-

tient underwent two staged operations. First, sheunderwent a suboccipital craniectomy. Through a

Case 2

A 57-year-old woman sought treatment for a 10-month history of progressive dysarthria, dysphagia,and frequent episodes of emotional lability. Heremotional lability was characterized by frequentoutbursts of inappropriate laughing and cryingspells. She reported right-sided weakness, gait dif-ficulties, and the recent onset of urinary incon-tinence. On admission, she exhibited a sensorydeficit in the distribution of the left 5th cranialnerve and subtle left-sided weakness. Her gag reflexwas decreased and her speech was dysarthric. Mildright hemiparesis and right hemisensory deficitwere noted and her plantar responses were extensorbilaterally. MRI of the brain revealed a 4- x 3-X 2.5-cm mass in the left petroclival region withmarked mass effect on the pons and midbrain. Mildhydrocephalus was also present. Audiometry re-vealed mild left sensorineural hearing loss at 30decibels.

PSEUDOBULBAR PALSY AND PETROCLIVAL MENINGIOMA/McCORMICK, LEE 69

Figure 1 Upper: PreoperativeTI-weighted, gadolinium-enhanced MRIs of Case 1, showing a large, left-sided petrocli-val meningioma extending into the cavernous sinus and compressing the brainstem severely. Lower: Immediate post-operative, gadolinium-enhanced MRIs showing a small residual tumor. The brainstem is completely decompressed.

A retrolabyrinthine transpetrosal, transten-torial approach was performed. Intraoperatively, alarge vascular tumor with an indistinct interfacewith the brainstem was encountered. A thin rim oftumor was left adherent to the midbrain and lateralpetrous wall. Histological examination identified abenign syncytial meningioma.

Postoperatively, the patient made a good re-covery. Her emotional lability resolved immedi-ately and her dysarthria, dysphagia, and gait diffi-culty improved gradually. One year after surgery,she exhibited mild residual left-sided V2 and V3sensory loss as well as mild right hemisensory loss.Follow-up imaging has revealed no evidence ofprogression of the residual tumor.

DISCUSSION

Multiple posterior fossa neoplasms have been re-ported in association with disordered emotional ex-pression, typically in the form ofpathological laugh-ter. Bhatjwale et al.5 described four patients withmassive trigeminal neuromas whose principal symp-tom was pathological laughter. Pathological laughteralso has been associated with posterior fossa menin-giomas,6,7,10,11 epidermoid tumors,13 trochlear nerveneurinomas,2 and clival chordomas.8,12 Intrinsicbrain tumors associated with pathological laughterinclude pontine glioblastomas multiforme,4 hypo-thalamic glioblastomas multiforme,15 and metastaticmelanomas of the pons.16 Like PBP, pathological

70 SKULL BASE: AN INTERDISCIPLINARY APPROACH/VOLUME 12, NUMBER 2 2002

laughter is most often associated with degenerative,vascular, and demyelinating processes.4 Poeckl7 re-viewed the published pathological anatomy in threecases of pathological laughter and found that thesupranuclear motor pathways are involved bilater-ally, always in the brainstem between the thalamusand medulla. In PBP, which more aptly describesthe clinical presentation of our two cases, the bilat-eral lesions can involve not only the pontine and/ormesencephalic levels but also the thalamus at thecapsular and cortical levels.

It is extremely rare for tumors of the poste-rior fossa to manifest with PBP. Tsuzaka et al.18 re-ported one patient with a primary lymphoma ofthe central nervous system who presented withPBP. This patient had a lesion on the right cerebel-lum as well as multiple lesions in the left frontallobe and basal ganglia. PBP also has been reportedin children who underwent posterior fossa surgeryfor large midline vermian or fourth ventricular tu-mors.19 In these cases, PBP was attributed to re-traction pressure that caused edema to track alongfiber pathways. Likewise, Hattori et al.20 reported apatient with PBP who had an arteriovenous mal-formation involving the thalamus and midbrain.This patient, however, also had Moyamoya diseasewith areas of diffuse cerebral infarcts and atrophy.

There are at least three possible causes forthe PBP in our two patients with posterior fossatumors, namely compression, ischemia, and de-myelination. The relatively large size of the neo-plasms in our two patients may have caused com-pressive dysfunction of the pontomesencephalicareas that contain the corticobulbar tracts bilater-ally. Compression and ischemia are likely interre-lated in that compressive mechanical injury leadsto compromise ofblood flow through small vessels.The portions of the brainstem made ischemic bycompression may remain in an ischemic penumbraand may have significant potential for recoveryafter decompression. The acute resolution of symp-toms in our patients after surgery supports such atheory. Furthermore, the absence of abnormalityon pre- and postoperative MRI argues against in-farction or demyelination as responsible for the

PBP in these two patients. We suggest that largecompressive posterior fossa tumors should be in-cluded in the differential diagnosis ofpatients witha clinical picture of bulbar motor dysfunction cou-pled with emotional lability.

REFERENCES

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2. Lepine R. Note sur la paralysie glosso-labiee cerebralea forme pseudo-bulbaire. Rev Mens Med Chir 1877;1:909-922

3. Mitsumoto H. Disorders of upper and lower motor neu-rons. In: Bradley WG, Daroff RB, Fenichel GM, eds. Neu-rology in Clinical Practice, Vol. 1. 2nd ed. Boston: Butter-worth-Heinemann, 1996:261-263

4. Adams RD, Victor M, Ropper AH. The limbic lobes andthe neurology of emotion. In: Adams RD, Victor M, Rop-per AH, eds. Principles of Neurology. 6th ed. New York:McGraw-Hill, 1997:508-521

5. Bhatjiwale MG, Nadkarni TD, Desai KI, Goel A. Patho-logical laughter as a presenting symptom of massivetrigeminal neuromas: report of four cases. Neurosurgery2000:47:469-472

6. Cantu RC, Drew JH. Pathological laughing and crying as-sociated with a tumor ventral to the pons. J Neurosurg1966;24:1024-1026

7. Matsuoka S, Aragaki Y, Numaguchi K. A case of an-gioblastic meningioma with pathological laughter: withspecial reference to laughter in brain tumor. Neurol MedChir (Tokyo) 1977;17:195-201

8. Matsuoka S, Yokota A, Yasukouchi H, et al. Clival chor-doma associated with pathological laughter. J Neurosurg1993;79:428-433

9. Nadkarni T, Goel A. Trochlear nerve neurinoma presentingas pathological laughter. Br J Neurosurg 1999;13: 212-213

10. Osumi Y, Yamador A, Tamaki N. A case of ventrally situ-ated brainstem meningioma associated with forced laugh-ter. Rinsho Shinkeigaku 1976;16:715-720

11. Shafqat S, Elkind MS, Chiocca EA, Takeoka M, Ko-roshetz WJ. Petroclival meningioma presenting withpathological laughter. Neurology 1998;50:1918-1919

12. Stevenson GC, Stoney RJ, Perkins RK, Adams JE. A trans-cervical transclival approach to the ventral surface of thebrainstem for removal of a clivus chordoma. J Neurosurg1966;24:544-554

13. Achari AN, Colover J. Posterior fossa tumors with patho-logical laughter. JAMA 1976;235:1469-1471

14. Cairns H. Mental disorders with tumours of the pons.Folia Psychiatr NeurolJpn 1957;53:193-203

15. Ironside R. Disorders of laughter due to brain lesions.Brain 1956;79:589-609

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16. Stern WE, Brown WJ. Pathological laughter. J Neurosurg1957;14:129-139

17. Poeck K. Pathological laughter and crying. In: Vinken PJ,Bruyn GW, Klawans HL, eds. Handbook of Clinical Neu-rology, Vol 45. Amsterdam: North-Holland; 1985

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Commentary

Certainly pathological laughter has previ-ously been reported in association with differenttypes of tumors in the posterior fossa. However,pseudobulbar palsy (PBP) associated with poste-rior fossa tumors as the initial manifestation is veryrare. PBP tends to be associated with neurodegen-erative and demyelinating disorders, which aremostly irreversible pathological processes.

If diagnosed properly, PBP associated withresectable tumors of the posterior fossa is re-versible. This potential emphasizes the importanceof imaging studies in patients with PBP. However,new posterior fossa lesions in patients already diag-

nosed with neurodegenerative or demyelinatingdisorders cannot be excluded. In such cases, the ap-pearance of new PBP symptoms warrants reimag-ing. This caveat is especially important for neurolo-gists, who usually follow these patients.

Typically, petroclival meningiomas manifestwith symptoms related to the trigeminal, vestibulo-cochlear, and glossopharyngeal nerves and withgait ataxia and dementia due to hydrocephalus.PBP and hemiparesis are late symptoms, associatedwith large or giant tumors. If a patient has no cra-nial nerve symptoms, PBP may be the initial symp-tom, especially in patients in rural areas or other-wise stoic individuals.

Laligam N. Sekhar, M.D.'Ramin Rak, M.D.'

Commentary

XVell-articulated and referenced, this casereport presents a rare and seldom recognized sys-tem complex associated with petroclival menin-giomas. The authors are to be commended for theirexcellent presentation.

Myles L. Pensak, M.D., F.A.C.S.2

Skull Base, volume 12, number 2, 2002. 'Mid-Atlantic Brain & Spine Institutes, Annandale, Virginia. Copyright C 2002 by ThiemeMedical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel: +1(212) 584-4662. 1531-5010,p;2002,12,02,071,071,ftx,en;sbsOO267x.

Skull Base, volume 12, number 2, 2002. 2Department of Otolaryngology, University of Cincinnati, Cincinnati, Ohio. Copyright C 2002 byThieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA. Tel: +1(212) 584-4662. 1531-5010,p;2002,12,02,071,071,ftx,en;sbsOO268x.