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.

Sneddon's syndrome is characterized by livedo reticularis and multiple ischemic infarcts often associated with antiphospholipid antibodies. Intracerebral hemorrhage (ICH) is unusual in Sneddon's syndrome and has not been reported as the presenting complaint. We report a 38-year-old woman with a history of two miscarriages, Raynaud's phenomenon and livedo reticularis who presented acutely with ICH. Angiography showed prominent leptomeningeal and transdural anastomoses (pseudoangiomatosis). Anticardiolipin antibodies were positive. A right frontal brain biopsy failed to reveal vasculitis and a skin biopsy was nonspecific. MRI showed residual intracerebral hemorrhage (ICH), diffuse atrophy, multiple small white matter infarcts and leptomeningeal enhancement. This is the first report of Sneddon's syndrome presenting with an ICH. It shares features with the Divry-van Bogaert syndrome. We discuss the cause of the pseudoangiomatosis pattern and its role in the genesis of the hemorrhage and suggest that cerebral angiography should be done in every patient with Sneddon's syndrome, as it could impact therapy.

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