des secousses interparoxystiques dans l'epilepsie partielle continue, et interpretation de cette dissociation. Rev Neurol 1922;1:61-3. 25. Radermecker J, Van Bogaert 1. Sur l'epilepsie partielle continue (Kojewnikoft). Moruztsschr Psychiatr Neurol 1949; 118:273-87. 26. Juul-Jensen P, Denny-Brown D. Epilepsia partialis continua. A clinical, electroencephalographic, and neuropathological study of nine cases. Arch Neurol 1966;15:563-78. 27. Denny-Brown D, Yanagisawa N. Dystonia resulting from lesions of the basal ganglia in infant macaques. Trans Am Neurol Assoc 1972;97: 105-8. 28. Morimoto T, Oguni H, Awaya Y, Hayakawa T, Fukuyama Y. A case of a rapidly progressive central nervous system disorder manifesting as a pallidal posture and ocular motor apraxia. Brain Dev (Tokyo) 1985 ;7:449-53. 29. Aicardi 1. Some disorders of the central grey matter in children: clinical and radiological diagnosis. Qin Exp Neurol 1985;21:1-25. 30. Aicardi J, Gordon N, Hagberg B. Holes in the brain. Dev Med Child Neurol 1985 ;27:249-52. 31. Kretzschmar K, Ludwig B, Kramer G, Collmann H, Kazner E. Bilateral lesions of the putamina. Neuroradiology 1986;28: 87-91. A 32. Campistol J, Fernandez-Alvarez E, Ruscalleda J. CT appearance of low attenuation areas in basal ganglia in childhood: report of 23 cases. Comput Radiol 1987; 11:229-35. 33. Savoiardo M, Passerini A, D'Incerti 1. Neuroradiology of basal ganglia diseases in children and adolescents. In: Angelini L, Lanzi G, Balottin V, Nardocci N, eds. Extrapyramidal disorders in childhood. Amsterdam: Elsevier Science Publishers BV, 1987: 151-67. 34. Peters ACB, Vielvoye GJ, Versteeg J, Bots GTAM, Lindeman 1. ECHO 25 focal encephalitis and subacute hemichorea. Neurology 1979;29:676-81. 35. Hattori H, Kawamori J, Takao T, et al. Computed tomography in postinfluenzal encephalitis. Brain Dev (Tokyo) 1983; 5:564-7. 36. Charney EB, Orecchio EJ, Zimmermann RA, Berman PH. Computerized tomography in infantile encephalitis. Am J Dis Child 1979; 133:803-5. 37. Ochi J, Okuno T, Venoyama Y, Narita H, Mikawa H. Symmetrical low density areas in bilateral thalami in an infant with measles encephalitis. Comput RadioI1986;10:137-9. 38. Leheup BP, Feillet F, Roland J, Andre JL, Pierson M. Lesions des noyaux gris centraux au cours des oreillons. Evolution c1inique et neuroradiologique d'un cas. Rev Neurol (Paris) 1987; 143:301-3. Cerebral Venous Thrombosis Associated with Recurrent Epilepsy-like Attacks Takashi Ichiyama, MD, Sadataka Houdou, MD, Yutaka Tomita, MD, Kumiko Yoshioka, MD and Kousaku Ohno, MD A 9-year-old girl with recurrent episodes of unconsciousness, with or without convulsions, was reported. The episodes, which have been observed 9 times over the last 6 years, were characterized by generalized or hemi-convulsions, associated features of headache and vomiting, disturbance of consciousness lasting for a few days, slow activity on EEG continuing for a few weeks after the episodes and no prophylactic effect of anticonvulsants. Repeated EEG examinations revealed no epileptic discharges. Cerebral angiography showed corkscrew-like veins in the frontal region. Cerebral venous thrombosis is strongly suggested as the underlying cause of her episodes. Key words: Cerebral venous thrombosis, cerebral angiography, corkscrew-like vein, epilepsy. Ichiyama T, Houdou S, Tomita Y, Yoshioka K, Ohno K. Cerebral venous thrombosis associated with recurrent epilepsy-like attacks. Brain Dev 1989;11:326-8 Cerebral venous thrombosis is a relatively uncommon diagnosis in children. Its clinical features are variable, depending on the extent and site of the occlusion [1] . We report a 9-year-old girl with recurrent episodes of headache, vomiting, disturbance of consciousness and convulsions over the last 6 years, who was initially treated for epilepsy. CASE REPORT The girl, born in May 1979, was the product of an uncomplicated full-term pregnancy and uneventful delivery. Her parents were healthy and unrelated. In September 1982, at age three, she suddenly had a generalized clonic convulsion lasting 20 minutes. Therefore, she was admitted to a hospital. She became responsive two hours later, but then vomited several times and complained of headache. Neurological examinations disclosed somnolence, confusion, bilateral Babinski's reflexes, nuchal rigidity and Kernig's sign. Complete blood cell counts, serum chemistry results, serum electrolytes, and blood sugar and ammonia were all within normal range. A lumbar puncture revealed an opening pressure of 170 mmH 2 0. The CSF glucose level was 43 mg/dl, the protein level 24 mg/dl and the WBC count 9/mm 3 • EEG showed diffuse high voltage delta activity. She was treated with phenobarbital (PB) and glycerol. She became alert three days later and the neurological symptoms disappeared. EEG on day 7 still showed diffuse and intermittent high voltage delta activity, but it had become almost normal one month later. In January 1984, she vomited several times and then had a generalized clonic convulsion. She was placed on PB therapy for epilepsy. High voltage 3 Hz activity in the left hemisphere was noticed on EEG performed 35 days after the episode. In September 1984, she vomited on 4-5 consecutive days and became unconscious. She became alert one day later. Valproic acid (VPA) was added to the PB therapy. In February 1985, January and March 1986, July and November 1987, and April 1988 she became unconscious and then, except for in two episodes, had generalized or right hemi-convulsions lasting 15-30 minutes. In each episode, a somnolent and confused state with vomiting and/or headache continued for several hours to three days. EEG performed 11 days after the last episode showed 1-2 Hz activity without alpha waves in the left hemisphere (Fig 1). In June 1988, she was admitted to our hospital for detailed examinations (No 88-8600). She showed no abnormality on physical or neurological examination. Serum electrolytes, immunoglobulins, serum and urinary amino acids, and urinary organic acids were all within normal range. Serologic tests for syphilis, antinuclear antibody, and anticardiolipin IgG and IgM antibodies were all negative. The results of coagulation studies, including on plate- lets (260,000/mm 3 ), bleeding time (4 min; normal, 2-5 min), prothrombin time (123%), activated partial thromboplastin time (35 sec; control, 28-39 sec), fibrinogen (154 mg/dl), antithrombin III (93%), beta-thromboglobulin (52 ng/ml; normal, < 52 ng/ml) and platelet factor 4 (14.3 ng/ml; normal, <20 ng/ml), were normal. A lumbar puncture revealed a normal opening pressure. The CSF was acellular with normal concentrations of glucose, protein, _,"'&pi .....................".,____---. .~-"'-.v.-.....,....-'-""" ~ .~~~~~~~~~~~~~~ ~~'~'---"'""'--~u ~.-~~~~~~--~~~~~ ,"'"rrJ'I\J\. ;.f'vw-J ,~~,,~~~~~~~~~~~ '-o1,r"''''v''../\. ''''\.,/\ 1'4 -------------------------~ Fig 1 EEG 11 days after an attack shows 1-2 Hz slow activity without alpha waves, the so-called lazy phenomenon, in the left hemisphere. From the Division of Child Neurology, Institute of Neurological Science, Tottori University School of Medicine, Yonago (TI, SH, YT, KO); Department of Pediatrics, Shimane Medical University, Shimane (KY). Received for pUblication: March 27,1989. Accepted for publication: June 15, 1989. Correspondence address: Dr. Takashi Ichiyama, Division of Child Neurology, Institute of Neurological Science, Tottori University School of Medicine, Nishi-machi 86, Yonago 683, Japan. Fig 2 Cerebral angiography. Left internal carotid angiogram, venous phase. lateral projection. At the frontal convexity, corkscrew..like cortical veins (arrows), and poor filling of the anterior portion of the superior sagittal sinus and deep veins can be seen. (F: frontal) Ichiyama et al: Cerebral venous thrombosis 327 lactate and pyruvate. EEG of the left hemisphere revealed poor background alpha wave formation in the awake state and poor spindle formation in sleep, but neither abnormal slow waves nor seizure activities were noticed. Also, a rebuild-up phenomenon after hyperventilation was not observed. CT scanning and MRI revealed slight cortical atrophy in the frontal region, but an "empty delta sign" on enhanced CT was not noted. Cerebral angiography (CAG) showed corkscrew-like veins in the frontal region, with dominancy on the left, and ftlling of the anterior portion of the superior sagittal sinus, deep medullary veins and deep veins was poor (Fig 2). These findings are features of sagittal sinus thrombosis. We diagnosed her episodes as being closely related to cerebral venou~ thrombosis. Although we could not detect any abnormalities suggesting coagulopathy as an underlying cause of the cerebral venous thrombosis, we started aspirin therapy (3 mg/ kg/day) in combination with PB and VPA therapy. She has remained well for 11 months after discharge without a recurrence of the symptoms. DISCUSSION The patient's recurrent episodes, which have been observed 9 times over the last 6 years, can be summarized as follows: 1) disturbance of consciousness lasting 1-3 days, 2) generalized or right hemi-convulsions (in several episodes there were no convulsions), 3) associated signs of headache and vomiting, and 4) abnormal EEG with slow activity lasting for a few weeks after the episodes. It hardly seems likely she had epilepsy, because epileptic discharges were not detected in extensive EEG examinations. The slow activity observed on EEG after the episodes lasted too long for it to be epileptic postictal slow activity. Also, the features of the clinical attacks were not typical epileptic ones. Such episodes may be observed in patients with metabolic or vascular disorders. Metabolic errors were excluded by many examinations, such as blood chemistry, amino acid and organic acid analysis, etc. The corkscrew-like veins and poor filling of the anterior portion of the sagittal sinus observed on CAG are consistent with sagittal sinus thrombosis [1,2]. Both the prolonged disturbance of consciousness and the abnormal slow activity on EEG lasting for a few weeks strongly suggest a long-lasting functional brain disturbance due to a vascular abnormality. Vomiting and headache would reflect increased intracranial pressure accompanying the cerebral venous thrombosis and, practically, a lumbar puncture, only once carried out at the time of an attack, showed an elevated opening pressure. The most important pathophysiological question may be whether or not each episode was due to the persistence of the pathological state causing repeated venous occlusion and recanalization. Patients with recurrent attacks consisting of headache, vomiting and disturbance of 328 Brain & Development, Vol 11, No 5,1989 consciousness have rarely been reported [3, 4]. Cases with cerebral venous thrombosis have been associated with trauma, infectious diseases, pregnancy, use of oral contraceptives, and hematologic, gastrointestinal, cardiac and metabolic disorders, including immunological ones [5-8]. So far as examined, we could not detect any underlying abnormalities that explained the venous thrombosis. In our case, high voltage slow activity after the initial episode was observed in both hemispheres on EEG, and afterwards the area in which high voltage slow activity was observed was localized to the left hemisphere. It is possible to speculate that in each of her episodes an identical vein was occluded through an unknown mechanism and recanalized immediately without permanent functional deficits but with temporary functional disturbances, as suggested by. the abnormal slow activity. With the repeated occlusion and recanalization, collateral circulation may have developed gradually and therefore the extent of the area functionally affected by the occlusion may have been limited. In a case of recurrent epileptic episodes with a prolonged confusional state and slow activity on EEG, the possibility of cerebral venous thrombosis should be examined carefully. ACKNOWLEDGMENTS We are deeply indebted to Prof. K. Takeshita for the vast amount of advice, Dr. Y. Tanaka (Sanin Rousai Hospital) for his skillful help with the CAG and Dr. K. Ishii (Tohoku University) for the critical comments as to the CAG findings. REFERENCES 1. Ya§argil MG, Damur M. Thrombosis of the cerebral veins and dural sinuses. In: Newton TH, Dotts DS, eds. Radiology of the skull and brain. St Louis: Mosby, 1974:2375-400. 2. Shinohara Y, Hamaguchi K. Cerebral venous and sinus thrombosis with temporal lobe epilepsy (in Japanese). Rinsho Shinkeigaku (Tokyo) 1973;13:718-25. 3. Takashima S, Goto F, Fukuuchi Y, Koto A, Ikeda Y. Superior sagittal sinus thrombosis as a complication of idiopathic thrombocytopenic purpura (in Japanese). Rinsho Shinkeigaku (Tokyo) 1988;28:812-8. 4. Nishimura RN, Stepanek D, Howieson J, Hammerstad J. Internal cerebral vein thrombosis. Arch Neurol 1982;39: 439-40. 5. Imai WK, Everhart FR, Sanders JM. Cerebral venous sinus thrombosis: report of a case and review of the literature. Pediatrics 1982;70:965-70. 6. Schwab FJ, Peyster RG, Brill CB. CT of cerebral sinus thrombosis in a child with homocystinuria. Pediatr Radiol 1987;17:244-5. 7. Kaplan RE, Springate JE, Feld LG, Cohen ME. Pseudotumor cerebri associated with cerebral venous sinus thrombosis, internal jugular vein thrombosis and systemic lupus erythematosus. J Pediatr 1985 ;107:266-8. 8. Haan J, Caekebeke JFV, Van Der Meer FJM, Wintzen AR. Cerebral venous thrombosis as presenting sign of myeloproliferative disorders. J Neurol Neurosurg Psychiatry 1988;51: 1219-20.