The stroke syndrome of cortical vein thrombosis K. Jacobs, MD; T. Moulin, MD; J. Bogousslavsky, MD; F. Woimant, MD; I. Dehaene, MD; L. Tatu, MD; G. Besson, MD; E. Assouline, MD; and J. Casselman, MD Article abstract-Cortical vein thrombosis without sinus involvement is rarely diagnosed, although it may commonly be overlooked. We report four cases of cerebral venous thrombosis limited to the cortical veins. The diagnosis was made on surgical intervention in one patient and by angiography in three patients. Together with a survey of the published cases, the clinical and neuroimaging patterns of our patients allow delineation of several features suggestive of cortical venous stroke. Focal or generalized seizures followed by hemiparesis, aphasia, hemianopia, or other focal neurologic dysfunction in the absence of signs of increased intracranial pressure should suggest this possibility. Neuroimaging (CT, MRI) shows an ischemic lesion that does not follow the boundary of arterial territories and often has a hemorrhagic component, without signs of venous sinus thrombosis. Conventional angiography demonstrates no arterial occlusion but may show cortical vein thrombosis corresponding to the infarct, although these may also be nonspecific findings. The role of MR angiography, which is well-established in sinus thrombosis, remains to be assessed in patients with brain ischemia due to isolated cortical vein occlusion. NEUROLOGY 1996;47:376-382 Cortical vein thrombosis has seldom been reported.lS2 Clinical diagnosis is difficult, and in many cases, it was not made before pathologic ~erification.~,~ More- over, the term cortical vein thrombosis is sometimes used, confusingly, as a general term for any type of venous thrombosis affecting the brain, including si- nus thrombosis. We studied four patients with iso- lated cortical vein thrombosis whose neurologic fea- tures and brain imaging suggest that clinical diagnosis is feasible and should probably be evoked more often. Patient reports. Patient 1 (Brugge). A 38-year-old woman presented with flu-like symptoms, transient blurred vision, and migrating paresthesias 6 months prior to admission in October 1992. Brain MRI, which had been performed in May 1992, was normal. The patient was hos- pitalized because of progressive numbness and generalized subjective weakness. Physical examination, brain CT, and CSF analysis were normal. During hospitalization, she de- veloped simple partial seizures, with jerking of the left arm and abdominal wall. Carbamazepine was adminis- tered. EEG, short latency somatosensory, visual, and brainstem auditory evoked potentials were normal. EEG after sleep deprivation contained intermittent delta and theta waves in the right frontotemporal region. Brain MRI (figure lA, B) showed a small nodular area in the right parietal region that was hypointense on TI-weighted im- ages and hyperintense on T,-weighted images. This area did not enhance after the administration of gadolinium. A slight enhancement was present, however, in the meninges overlying the lesion (figure 1C). Angiography showed non- specific signs of a space-occupying lesion in the right pari- etal region. Neither vein occlusion nor collateral venous circulation was reported. Coagulation parameters (includ- ing antithrombin 111, protein C, protein S, and lupus anti- coagulant), Borrelia and syphilis serology, angiotensin con- verting enzyme, rheumatoid factor, and antinuclear antibodies were normal. Control MRI of the brain, per- formed just before the surgical procedure, showed menin- geal enhancement after the administration of gadolinium. At surgery (November 24, 19921, a yellowish area was seen in the right postrolandic cortex. In the center of this area, there was a thrombosed cortical vein, previously emptying into the superior sagittal sinus (figure 1D). Pathologic ex- amination showed a parenchymatous edema, an old ve- nous thrombus partially replaced by fibrous tissue, and signs of recanalization. The postoperative evolution was favorable. Anticoagulants were not given. One year after hospital discharge, the patient developed spondylarthritis, and was found to be HLA-B27 positive. A 42-year-old, right-handed woman complained of leg pain. Radiculography was per- formed. After 24 hours the patient complained of an in- creasingly severe headache. Six days later, she developed intermittent dysarthria. The following day she had a tonic- clonic epileptic seizure. Neurologic examination showed paresis of the right face and arm, visual extinction on double simultaneous stimulation in the right visual field, and global aphasia. Personal history was negative, except for the use of oral contraceptives until 2 years before ad- mission. Coagulation parameters (including protein C, pro- tein s, antithrombin I11 and antiphospholipid antibodies) and serologic tests (Borrelia, syphilis, and HIV) all proved Patient 2 (Besanqon). From the Departments of Neurology (Drs. Jacobs and Dehaene) and Radiology (Dr. Casselman), Algemeen Ziekenhuis St.-Jan, Brugge, Belgium; the Department of Neurology (Drs. Moulin and Tatu), Centre Hospitalier Universitaire Jean Minjoz, BesanGon, France; the Department of Neurology ( Drs. Bogousslavsky and Besson), Centre Hospitalier Universitaire Vaudois, Lausanne, Switzerland; and the Departments of Neurology (Dr. Woimant) and Radiology (Dr. Assouline), Hopital Lariboisihre, Pans, France. Received November 6, 1995. Accepted in final form February 2, 1996. Address correspondence and reprint requests to Dr. Julien Bogousslavsky, Department of Neurology CHUV, CH-1011 Lausanne, Switzerland. 376 Copyright 0 1996 by the American Academy of Neurology