"Fou rire prodromique" as the presentation of pontine ischaemia secondary to vertebrobasilar stenosis

Abstract

Pathological laughter is associated with a wide range of neuropsychiatric disorders. "Fou rire prodromique" is one of its forms and was first described by Féré in 1903.1 It can be literally translated as "prodrome of crazy laughter" and is defined as pathological laughter preceding a major apopletic event.2 It has been reported rarely and its mechanisms are still not fully understood.This type of pathological laughter has been reported as the presentation of bilateral thalamic hemorrhages, 3 strokes in the right lenticular nucleus/internal capsule, 4 and left capsule/lenticular nucleus/ insula due to left internal carotid occlusion, 5 6 left capsule/ thalamus, 7 or left putamen/internal capsule. 2Other reports described pathological laughter after hemiparesis in pontine strokes or heralding left temporal ischaemia. 8-12We present the first description of pathological laughter as the actual initial symptom of pontine stroke secondary to severe vertebrobasilar artery stenosis. This report has been published in abstract form. 13Case report A 65 year old right handed man suddenly developed uncontrollable, persistent, unemotional laughter which lasted for almost an hour. He felt exhausted because of the laughter and then developed right hand and facial paresis that resolved almost completely on arrival at the emergency room.He had hypertension for 30 years and was taking 60 mg nadolol every day. He denied smoking and used to drink a few beers. He was in good health, except for occasional nonpositional dizziness for 2 weeks and one spell of vertigo with an increaase in his blood pressure before his admission. He denied dysphagia, diplopia, amaurosis, headache, dysphonia, dysarthria, hearing loss, loss of consciousness, and head or neck trauma.He was admitted to a local hospital where a head CT was reported "negative". He continued to have brief spells of laughter alternating with crying but with no paresis. Next day, a neurologist documented normal examination and a preliminary diagnosis of left hemispheric transient ischaemic attacks versus gelastic epilepsy or anxiety was made. A few hours later, he developed right brachial-facial paresis. Brain MRI/MRA showed small infarcts in the midlateral pons (ventrotegmental junction at the level of the pontomesencephalic junction) and left cerebellar hemisphere (fig 1). Occlusion of the left vertebral artery and narrowing of the right vertebral and basilar arteries was also seen. Intravenous heparin was started.His condition deteriorated and cerebral angiography was performed. Neurological examination disclosed dysarthria, bilateral, reactive, 2 mm pupils, desconjugated gaze, gaze evoked upbeat nystagmus, decreased corneal reflex on the left, preserved gag reflex, right hemiparesis (2/5 in the upper limb, 4/5 in the lower limb), decreased tone, brisk reflexes on the right, right Babinski's sign, and decreased pinprick and touch on the right.

Pathological Laughter, Multiple Sclerosis, Behavioural Abnormality

“…P athological laughter (Fou rire prodromique) is an uncommon clinical manifestation of lesion involving cerebro-brainstem-cerebellar pathways which control the laughter [1]. Existence of laughing centre has been hypothecated in the basal pons.…”

A Note about ‘Fou Rire Prodromique’

“…Another argument to use a mechanism-based classification is the fact that in most published cases, the initial phenomenology evolved to different manifestations. For example, our patient had 'classic' FRP, followed hours later by laughter and crying [10]. This probably illustrates the effects of ischemic spreading and different degrees of ischemic activation, which was first observed in animal studies using electrical stimulation [2,10].…”

“…Terms like 'spasmodic laughter', 'emotionalism', and 'sham mirth' have been used to describe and differentiate the phenomenology. However, as we have recently proposed [10,12], a new classification based on the disease mechanism is warranted to better understand and treat the disorders of laughter and crying. For example, we have recently proposed that FRP might result from ischemic-induced ephaptic activation of white matter tracts leading to activation or disinhibition of nearby structures [10].…”

“…However, in several quoted references, other neurological deficits preceded laughter: in Wali [3], transient weakness preceded laughter by a few days; in Lago et al [4], blindness preceded concomitant laughter and weakness; in Carel et al [5], initial right hemiplegia preceded laughter; in Couderq et al [6], right hemiparesis and dysarthria preceded laughter, and in Tei and Sakamoto [7], right hemiparesis might have preceded laughter. In addition, other cases of laughter preceding acute neurological events [8][9][10] were not included in the literature review.…”

Pathological Laughter as an Unusual Manifestation of Acute Stroke

“…However, the clinical significance of the acute-onset type remains controversial. A recent case report suggested that the acute-onset type of pathological laughter might be a symptom of acute stroke [6] . We report a patient who experienced fluctuating pathological laughter simultaneously with neurological deficits following recurrent bilateral pontine infarcts, which supports the hypothesis that 'the acute-onset type of pathological laughter is one of the symptoms of acute stroke'.…”