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|Year : 2001 | Volume
| Issue : 2 | Page : 162-5
Trigeminal schwannoma associated with pathological laughter and crying.
Virani MJ, Jain S
Department of Neurosurgery, Jaslok Hospital and Research Centre, Mumbai, 400 026, India.
Department of Neurosurgery, Jaslok Hospital and Research Centre, Mumbai, 400 026, India.
A 46 year old man with trigeminal schwannoma displayed symptoms of ataxia with pathological laughter and crying. The tumour developed in the cerebellopontine angle, compressing the pontomesencephalic structures backward, extending in the posterior parasellar region and Meckel's cave. No recurrence of laughter and crying attacks were noted after total removal of the tumour. Theories of mechanism of pathological laughter and crying reported in the literature are reviewed.
|How to cite this article:|
Virani M J, Jain S. Trigeminal schwannoma associated with pathological laughter and crying. Neurol India 2001;49:162
Laughing and crying are termed pathological when the behaviour is continuous and inappropriate. Forced laughing and crying is a rare neurological symptom with a specific differential diagnosis. The absence of coexisting emotional changes always signify a pathological substrate in the brain, either diffuse or focal. Though cases of pathological laughter and crying have been documented in the literature, their diffuse nature precludes useful topographic analysis and clinicoanatomical correlation. Pathophysiology of pathological laughter and crying can, at present, only be explained by a pathoanatomical stand point. A case of trigeminal schwannoma presenting with pathological laughter in combination with crying is reported and relevant literature reviewed.
A 46 year old right-handed man presented with spontaneous uncontrollable laughter with inappropriate crying, giddiness, weakness of left side of face and ataxia of left hand of 8 months duration. The patient was aware that his labile emotion was not normal and had once sought psychiatric help. The medical, neurological and psychiatric histories were otherwise unremarkable. On examination, there was no evidence of a generalised emotional or intellectual disturbances. There was left facial lower motor neuron weakness, with impaired pinprick over right V1-V2 division. The left hand showed finger nose ataxia and moderate dysdiadochokinesia. Romberg's test was positive. Eye movements were normal but coarse nystagmus was present on looking to the right. There were no cerebellar signs, long tract signs or cranial nerve deficit. He was getting brief spells of sudden onset laughter and crying. The patient could not stop these episodes and there was nothing funny either in his mind or in the enviornment. There was no impairment of consciousness during these attacks.
Results of standard laboratory tests and chest X-ray were normal. EEG revealed no epileptiform discharges. MRI brain showed a fairly large to oval, predominantly cystic, peripherally enhancing (with Gadolinium-DTPA) space occupying lesion [Figure - 1] measuring 3.7x3.2x2.4 cms in size, in the right cerebellopontine angle region, anteriorly seen extending into the middle cranial fossa in the posterior parasellar region and Meckel's cave on the right side.
Tumour was hypointense on T1WI and revealed two hyperintense specks represented as fat within the tumour. It was hyperintense on T2WI. A fluid-debris level was also seen within it, suggestive of cystic/necrotic changes. Seventh and eighth nerve complex was spared. There was marked displacement of the pontomesencephalic structures and aqueduct. Superior portion of the 4th ventricle was also compressed. Cerebellum and cerebral hemisphere were free of lesion. In view of the neurological findings and MRI scan, a trigeminal nerve tumour was suspected.
He was operated in November 1992, under general anaesthesia and a right retromastoid supracerebellar approach was employed to excise the tumour. Histological examination, confirmed trigeminal schwannoma [Figure - 2]. The postoperative recovery was uneventful, with complete resolution of unprovoked laughter and crying spells on the same day. The left facial weakness improved completely in 7 days but the impaired pin-prick over right Vl-V2 division remained. The long term follow up MRI scan [Figure - 3] did not reveal any residual lesion in the right cerebellopontine angle or in the region of Meckel's cave. A follow up visit 7years after surgery showed no recurrence of abnormal laughter and crying. Apart from vague pain over the right side of face in Vl-V2 division, his entire neurological examination had been normal. In particular, appropriate laughter and crying never recurred.
Uncontrollable attacks of laughter or crying, generally devoid of emotional content and precipitated by minor affective or neutral events, may be observed in patients with lesions in diverse areas in the cortex, diencephalon, and brain stem, but in most instances, lesions involving systems affecting motor function (pyramidal tract or extrapyramidal fibre lesions) and an interruption of the brain stem are observed., The precise mechanism and neuroanatomical substrates of pathological laughter and crying (PLC) remain uncertain. Recognised causes include subcortical and brain stem infarction (due to vascular occlusion or basilar ischaemia,,, tumours in and around the upper brain stem and multiple sclerosis. Isolated cases have been described in which the condition resulted from cerebellar haemorrhage, insecticide exposure and traumatic brain injury. Among cases with patho-logical laughter and crying from distortion of upper brain stem by extrinsic or intrinsic tumour pontine glioma and clival chordoma,,,, are prominent causes. Though, petroclival meningioma, acoustic neurinoma have also been reported, Cairns was the first to report night terrors in a patient with a brain stem tumour. Since then, 15 cases of brain stem tumours,, associated with inappropriate laughter and/or crying have been documented in the literature in addition to our case. Two major supranuclear pathways control the pontomedullary mechanisms of facial and other movements required in laughing and crying, i.e. corticobulbar pathway and frontopontomedullary connection for emotional expression. Wilson in 1924 assumed that the second pathway is involuntarily inhibited by the first. Unilateral involvement of this anterior pathway leaves the opposite side of the face under volitional control but paretic during laughing, smiling and crying (facial emotional paralysis). Poeck concluded that supranuclear motor pathways are always involved with loss of a control mechanism somewhere in the brainstem, between thalamus and medulla.
An analysis of cases of PLC reported in the literature highlight four main features : a) in almost all the cases some degree of compression of upper brain stem structures was found; b) as per Wilson's hypothesis and Poeck's conclusion, the control center for laughter and crying exists somewhere in the brain stem; c) cases reported by Isono et al and Matsuoka et al presented with oniric behaviour during stage I REM, which was also correlated by experiments on cats; d) the pathological laughter and crying spells were found to be different from the gelastic seizures, which are accompanied by an abrupt sympathetic system activation, probably due to the direct paroxymal activation of limbic and paralimbic structures or other autonomic centers of the hypothalamus and medulla. It appears that lesions in many areas of the brain stem cause abnormal emotional activity at various levels. e.g. involvement of pontomesencephalic structures, posterior diencephalic or limbic region, and anterior hypothalamic, frontal,or temporal areas. Specific nuclei in pons and medulla, include locus ceruleus, nucleus pontis oralis, nucleus pontis caudalis, raphe nuclei and nucleus of the solitary tract. Similarly, importance of raphe nuclei has been implicated in serotonergic transmission, which plays an important role in mechanism of pathological crying. The serotonergic raphe nuclei in the brain stem give rise to long projections to the limbic forebrain structures. The principle ascending fibres arise from serotonin cell bodies located in the dorsal nucleus of the raphe and in the superior central nucleus. The major ascending pathway from the rostral raphe nuclei joins the medial forebrain bundle in the lateral hypothalamus. Fibres leaving this main ascending bundle enter the substantia nigra, the intrathalamic nuclei, the stria terminalis, the septum, and the internal capsule., Cases have been described in literature in which PLC was immediately and permanently reversed with serotonin uptake inhibitor therapy.,,,
We conclude that any tumour in the region of CP angle, compressing the pontomesencephalic structures, may have an unusual presentation without any obvious brain stem sign or symptoms. lt appears that in our case tumour had obviously compressed these structures especially in the pons, such as locus ceruleus and raphe nuclei which had resulted in and produced pathological laughter and pathological crying through a supranuclear disconnection, eliminating cortical and limbic influences. The lack of stereotype in the patient's laughter unaltered cognition, normal EEG and eventual localisation, argue against the spells being seizures. Any laughter and crying which is inappropriate and can not be explained, should be evaluated in detail to rule out pontine lesion.
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