Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by the association of severe headaches with or without additional neurological symptoms and a 'string and beads' appearance on cerebral arteries, which resolves spontaneously in 1-3 months. We present the clinical, neuroimaging and outcome data of 67 consecutive patients prospectively diagnosed over 3 years in our institution with an angiographically confirmed RCVS. There were 43 females and 24 males with a mean age of 42 years (19-70). RCVS was spontaneous in 37% of patients and secondary in the 63% others, to postpartum in 5 and to exposure to various vasoactive substances in 37, mainly cannabis, selective serotonin-recapture inhibitors and nasal decongestants. The main pattern of presentation (94% of patients) was multiple thunderclap headaches recurring over a mean period of 1 week. In 51 patients (76%), headaches resumed the clinical presentation. Various complications were observed, with different time courses. Cortical subarachnoid haemorrhage (cSAH) (22%), intracerebral haemorrhage (6%), seizures (3%) and reversible posterior leukoencephalopathy (9%) were early complications, occurring mainly within the first week. Ischaemic events, including TIAs (16%) and cerebral infarction (4%), occurred significantly later than haemorrhagic events, mainly during the second week. Significant sex differences were observed: women were older, had more frequent single-drug exposure and a higher rate of stroke and cSAH. Sixty-one patients were treated by nimodipine: 36% had recurrent headaches, 7% TIAs and one multiple infarcts. The different time courses of thunderclap headaches, vasoconstriction and strokes suggest that the responsible vasospastic disorder starts distally and progresses towards medium sized and large arteries. No relapse was observed during the 16 +/- 12.4 months of follow-up. Our data suggest that RCVS is more frequent than previously thought, is more often secondary particularly to vasoactive substances, and should be considered in patients with recurrent thunderclap headaches, cSAH or cryptogenic strokes with severe headaches.

Background and Purpose-Reversible cerebral vasoconstriction syndrome (RCVS), characterized by severe headaches and reversible constriction of cerebral arteries, may be associated with ischemic and hemorrhagic strokes. The aim of this study was to describe the frequency, patterns, and risk factors of intracranial hemorrhages in RCVS. Methods-We analyzed prospective data on 89 consecutive patients with RCVS, of which 8 were postpartum and 46 used vasoactive substances. Standard bivariate and multivariate statistical tests were applied to compare patients with and without hemorrhage. Results-Thirty patients (34%), of which 5 were postpartum and 12 used vasoactive substances, developed at least 1 type of intracranial hemorrhage, including cortical subarachnoid (nϭ27), intracerebral (nϭ11), and subdural hemorrhage (nϭ2

Thirty patients with a typical orthostatic headache were treated by early lumbar epidural blood patch (EBP) without previously performing lumbar puncture or identifying a CSF leak and with or without typical MRI changes. A complete cure was obtained in 77% of patients after one (57%) or two (20%) EBPs. Spontaneous intracranial hypotension with typical orthostatic headache can be diagnosed without lumbar puncture and can be cured by early EBP in a majority of patients.

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The association of RCVS and CeAD was found in 12% of our patients with RCVS and 7% of our patients with CeAD. Underlying mechanisms are unknown. In practice, our results point to the need for a systematic study of both cervical and intracranial arteries in the 2 conditions.

Background and Purpose-Recently COL4A1, a gene encoding the type IV collagen ␣1 chain, has been found to be involved in families with autosomal-dominant porencephaly and infantile hemiparesis. In addition to neonatal stroke, some family members had experienced, during adulthood, spontaneous intracerebral hemorrhages (ICHs) and leukoencephalopathy, suggestive of underlying small-vessel disease of the brain. We now report a patient with sporadic, recurrent ICHs and a novel COL4A1 mutation. Methods-We performed a clinical and genetic study of a 25-year-old-patient with an 8-year history of recurrent ICHs. Key Words: intracerebral hemorrhage Ⅲ genetics Ⅲ white matter Ⅲ young adults I nfantile hemiparesis (congenital hemiplegia, or hemiparetic cerebral palsy) is considered the consequence of perinatal stroke. The prevalence of perinatal stroke for infants Ͻ30 days of age is estimated to be 1 in 4000 live births, with approximately two thirds ischemic and one third hemorrhagic. 1 The underlying mechanisms of infantile hemiparesis are heterogeneous. Intrauterine growth restriction, preeclampsia, chorioamnionitis, systemic infection, birth asphyxia, prolonged rupture of membranes, cord abnormalities, and cardiac and coagulation disorders have all been reported as risk factors. 1-3 However, infantile hemiparesis can occur after normal pregnancy and normal birth. Families with infantile hemiparesis and autosomal-dominant porencephaly have also been reported. 4 Recently, a new mouse mutant that develops fatal perinatal hemorrhage and porencephaly has been identified with a mutation in Col4a1, a gene that encodes the type IV collagen ␣1 chain. 5 Mutant Col4a1 mice have structural alterations of the vascular basement membrane in the brain and other tissues. Recent studies have shown that mutations in COL4A1 on chromosome 13q34 are involved in human families with infantile hemiparesis and porencephaly. [5][6][7] Interestingly, some mutant mice had, depending on their genetic background, a highly tortuous retinal vasculature in addition to perinatal hemorrhage and porencephaly. 8 This retinal phenotype has been previously associated with familial infantile hemiparesis in a French family that was shown to have a COL4A1 mutation. 8,9 COL4A1 also plays a role in intracranial hemorrhage (ICH) during adulthood in the mouse and in humans. 8,10 Herein we describe a novel COL4A1 mutation in a young patient with a history of infantile hemiparesis who experienced, during adulthood, sporadic, recurrent ICHs associated with diffuse white-matter abnormalities. Results-This Subjects and MethodsA 25-year-old male with infantile hemiparesis and recurrent ICHs and his 45-year-old mother were examined. Family history was obtained from the mother. Genomic DNA was extracted from white blood cells of the proband and his mother. Each of the 52 coding exons of the COL4A1 gene was amplified (oligonucleotides and conditions available on request from E.T. Results Clinical DataThe proband is a 25-year-old man with no remarkable perinatal event except ...

Spinal epidural haematomas (SEH) are rare; most are caused by trauma, anticoagulant therapy, vascular anomalies, hypertension, blood dyscrasias, epidural anaesthesia or, rarely, spinal surgery. We report 11 cases and review the literature (16 cases). The clinical picture is that of acute spinal cord compression. MRI characteristics are quite specific. On sagittal sections, the SEH appears as a biconvex mass, dorsal to the thecal sac, clearly outlined and with tapering superior and inferior margins. The dura mater is seen as curvilinear low signal separating the haematoma from the cord. Within 24 h of onset, the haematoma is isointense with the cord on T1-weighted images and heterogeneous on T2-weighted images. Later, it gives high signal on both T1- and T2-weighted images. Differential diagnosis must include subdural haematoma, epidural neoplasm and abscess. Complete neurological recovery rapidly follows laminectomy and removal of the clot. In three of our cases, the haematoma resolved spontaneously. MRI is the best examination for diagnostic and follow-up.