4. Dandy WE, Blackfan K D Internal hydrocephalus: an experimental, clinical and pathological study. Am J Dis Child
84061182,1914
5. de h n g e SA, de Vlieger M: Hydrocephalus associated with raised venous pressure. Dev Med Child Neurol 12:Suppl
22~28-32,1970 6. Ellis RWB: Internal hydrocephalus following cerebral thrombosis, in an infant. Proc R Soc Med 30768-772, 1937 7. Emery JL, Zachary RB: Hydrocephalus associated with obliteration of the longitudinal sinus. AMA Arch Dis Child 3 1:288292,1956 8. Epstein R, Hochwald GM, Ransohoff J: Neonatal hydrocephalus treated by compressive head wrapping. Lancet
1~634-636, 1973 9. Ford FR,Murphy EL Increased intracranial pressure. A clinical analysis of causes and characteristics of several types. Bull Johns Hoplcins Hosp 64,369-398,1939 10. Gardner WJ: Otitic sinus thrombosis causing intracranial hypertension. Arch Otolaryngol 30:253-268,1939 11. Gibson JB, Taylor AR, Richardson AE: Congenital meriovenous fistula with an aneurysm of the greaz cerebral vein and hydrocephalus treated surgically. J Neurol Neurosurg Psychiatry 22:224-228,1959 12. Gills JP, Kapp JP, Odom GL: Benign intracranial hypertension: pseudorumor from obstruction of dural sinuses. Arch Ophthalmol 78~592-595,1967 13. Greer M. Benign intracranial hypertension: I. Mastoiditis and lateral obstruction. Neurology (Minneap) 12:472-476.1962 14. Haar FL, Miller CA: Hydrocephalus resulting from superior vena cava thrombosis in an infant. Case report. J Neurosurg
42:597-601,1975 15. Hooper R: Hydrocephalus and obstruction of the superior vena cava in infancy. Pediatrics 28:792-799,1961 16. Janeway R, Kelly D L Papilledema and hydrocephalus associated with recurrent polyneuriris. Arch Neurol 15:507514, 1966 17. Kind ME: Hydrocephalus and the dural venous sinuses. J Neurosurg 19195-201. 1962 18. Ray BS,Dunbar HS: Thrombosisof the dural venous sinuses as a cause of "pseudotumor cerebri." Ann Surg 134:376-386, 1951 19. Russell DS: Observations on the Pathology of Hydrocephalus (Special Report Series, Medical Research Council, No 265). London. H i s Majesty's Stationery Office, 1349 20. Stewan DR, Johnson DG, Myers GG: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J Pediatr Surg 10:771-777,1975
Sagittal Sinus Thrombosis as a Lomplication of Regional Enteritis Bruce Sigsbee, MD, and David A. Rottenberg, M D
A y o u n g woman with r e g i o n a l enteritis and a history of recurrent thrombophlebitis and p u l m o n a r y embolism developed sagittal sinus t h r o m b o s i s during an acute exacerbation of her illness. Repeated attempts to d o c u ment a hypercoagulable state were unsuccessful. Sigsbee B, Rottenbcrg DA: Sagittal sinus thrombosis as a complication of regional enteritis. Ann Neurol 3A50-452, 1978
Neurological complications of regional enteritis, which include tetany, seizures, peripheral neuropa t h y , m y o p a t h y , and subacute combined degeneration of the spinal cord, are usually associated with intestinal malabsorption, metabolic imbalance, or steroid therapy. This paper describes a heretofore unreported complication, widespread cortical venous thrombosis and sagittal sinus occlusion, in a y o u n g woman with regional enteritis and a history of recurrent thrombophlebitis and pulmonary embolism. Case Report A 30-year-old right-handed woman with regional enteritis and a history of classic migraine wils admirted to Memorial Sloan-Kettering Cancer Center (MSKCC) for evaluation of a right hemiparesis. Approximately ten days prior to admission she complained of bifrontal headache associated with intermittent "dizziness." This headache, unlike her usual predominantly right-sided migraine headaches, was nor preceded o r accompanied by visual symptoms o r by focal sensory or motor phenomena. O n e day prior to admission she awoke with blurred vision in the right eye and right-sided numbness; shortly thereafter she had a severe, throbbing left hemicranial headache. Later that afternoon she experienced difficulty using h e r right arm, and by evening she was unable to walk without assistance. W h e n she awoke the next morning, the headache was sall present and her right leg was completely paralyzed, whereupon she was brought to
MSKCC. From the time the patient was 10 years old, s h e had suffered from occasional severe hemicranial headaches pre-
From the Departments of Neurology, Memorial Sloan-Kcttering Cancer Center and Cornell University Medical College, New York, NY. Accepted for publication Nov 23. 1977. Address reprint requests to Dr Rottenbcrg, 1275 York Ave, New York, NY 10021.
450 0364-5 134/7810003-05l6$01.00 @ 1978 by Bruce Sigsbee
ceded by teichopsia and contralateral paresthesias. In 1961, when she was 14 years old, regional enteritis developed. Subsequent flareups of the disease were associated with erythema nodosum and arthritis and were complicated on two occasions by small bowel obstruction. During the four to five weeks prior to admission, and in spite of daily doses of triamcinolone, she experienced an exacerbation of the enteritis associated with mild malabsorption. Twice in 1963, again in 1970, and in September, 1976 (approximately six months prior to admission), the patient had deep vein thrombophlebitis; pulmonary embolization was documented in 1963. Each of these thromboembolic episodes was temporally associated with an exacerbation of the regional enteritis, and on each occasion she was taking pharmacological doses of corticosteroids. On admission the patient appeared chronically ill. Her temperature was 38.4"C. She was lethargic, emotionally labile, intermittently confused, and uncooperative, but not dysphasic. The optic fundi were normal, and spontaneous venous pulsations were present bilaterally. Her visual fields could not be adequately evaluated. Except for mild weakness of the right lower face, no cranial nerve abnormalities were recorded. The right upper and lower extremities were Transfemoral lejt internal carotid arteriogram: venous phase in lateralprojection. There is complete nonjilling of the superior sagittal sinus; extensive anastomotic venous rollaterals over the cerebral convexity drain through a large vein of Labbi into the basal venous sinuses.
hyporeflexic and hypotonic; both plantar responses were flexor. The right leg was externally rotated and plegic, whereas the right arm was only moderately paretic. Pinprick was promptly perceived bilaterally. Her level of consciousness and responsiveness fluctuated markedly from day to day. On the third hospital day she became difficult to arouse, and a lumbar puncture was performed; the opening pressure was 390 mm CSF, and the fluid (clear and colorless) contained no cells, 62 mg protein per deciliter, and 58 mg glucose per deciliter. Serial electroencephalograms were disorganized and slow with intermittent delta activity over the left temporooccipital region. A CAT scan with and without intravenous contrast material was normal. Cerebral angiography revealed widespread cortical vein thrombosis and sagittal sinus occlusion (Figure). The following coagulation studies were normal on at least two occasions: prothrombin time, partial thromboplastin time, thrombin time, reptilase time, fibrinogen concentration, fibrin split products, factor V and factor VIII nctivity, and platelet count. Antithrombin I11 activity was not measured. No pathogens were grown from the urine, sputum, or blood. When, on the ninth hospital day, deep vein thrombophlebitis developed in the left arm, the patient was started on a regimen of sodium warfarin. She improved slowly and was discharged on the twenty-third hospital day with slight residual right lower extremity weakness. Lumbar puncture was repeated one day prior to discharge: the opening pressure was 215 mm CSF, and the fluid contained 1
Brief Communication: Sigsbee and Rottenberg: Sagittal Sinus Thrombosis 45 1
mononuclear cell per cubic millimeter, 17 mg protein per deciliter, and 50 mg glucose per deciliter. Discussion Sagittal sinus thrombosis has not been previously reported as a complication of regional enteritis. This seems somewhat surprising when one considers that cerebral venous thrombosis is a well-known if infrequent complication of ulcerative colitis [2] and that cerebral arterial occlusions have been reported in patients with regional enteritis [ S ] . The association of systemic thromboembolism with inflammatory bowel disease (IBD)’ was recognized in 1936 by Bargen and Barker [l], who postulated that thrombi that form in small rectal veins adjacent to mucosal ulcers propagate through branches of the hypogastric veins to reach more proximal venous channels; they suggested that thrombus propagation was associated with blood hypercoagulability. More recently, thrombocytosis and prolongation of the plasma prothrombin time have been reported in patients with IBD. Lee et al [4] concluded that “the blood of patients with active and extensive ulcerative colitis may be hypercoagulable as a result of acceleration of the early stages of coagulation.” Hypercoagulability per se does not explain the occurrence of cerebral venous thrombosis in our patient with regional enteritis, inasmuch as repeated attempts to document a coagulopathy were unsuccessful and the platelet count remained within normal limits. Although corticosteroids have been shown to inhibit reactive fibrinolysis in experimental animals [3], and our patient was taking pharmacological doses of triamcinolone at the time of admission, one can only speculate on the relationship between the corticosteroid therapy and the pathogenesis of her sagittal sinus thrombosis. Two additional mechanisms may have contributed to the production of widespread cerebral venous thrombosis. Microthrombi originating in pelvic and femoral veins may have become dislodged and found their way into the cerebral venous circulation by way of Batson’s vertebral plexus; such microthrombi might then have served as a nidus for secondary thrombus formation. Skinhoj and Paulson [6] reported reduced regional blood flow during the prodromal phase of a migraine attack, and Wessler [7] provided experimental support for the clinical observation that retarded blood flow predisposes to intravascular thrombosis. It may be that our patient’s prodromal headache was, in fact, migrainous and that the associated venous stasis-in the setting of a hyper‘Inflammatory bowel disease refers to ulcerative colitis and regional enteriris.
452
Annals of Ncurology
Vol 3 No 5 May 1978
coagulable state-was sufficient to initiate thrombus formation in cortical veins and dural sinuses. When a patient with active regional enteritis complains of unusually severe headache and neurological examination reveals focal signs, or signs of increased intracranial pressure, or both, the possibility of sagittal sinus thrombosis should be considered and appropriate diagnostic studies performed without delay. A history of thromboembolism or laboratory evidence of abnormal blood coagulability further supports this diagnosis and increases the need for prompt diagnostic evaluation and treatment. Dr Ronenberg is recipient of Teacher Investigator Award No. 1 KO7 NS 00286-01 from the National Institute of Neurological and Communicative Disorders and Stroke.
References 1. Bargen JA, Barker NW: Extensive arterial and venous thrombosis complicating chronic ulcerative colitis. Arch Intern Med 58:17-31, 1936 2. Borda IT, Southern RF, Brown W F Cercbral venous thrombosis in ulcerative colitis. Gastroenterology 64:116-119, 1973 3. Gerria WBJ. Prakke EM. van der Meer J, et al: Corticosteroids and experimental intravascular coagulation. Scand J Haematol 13:5-10, 1974 4. Lee JCL, Spinell JA, Saver WG, et al: Hypercoagulability associated with chronic ulcerative colitis: changes in blood coagulation factors. Gastroenterology 54:76-85, 1968 5. Silverstein A. Present D H : Cerebrovascular occlusions in relatively young patients with regional enteritis. JAMA 215:976977, 1971 6. Skinhe] E, Paulson OB: Regional blood flow in internal carotid distribution during migraine attack. Br Med J 3:569-570,1969 7. Wessler S: Thrombosis in the presence of vascular stasis. Am J Med 33:648-666. 1962
84061182,1914
5. de h n g e SA, de Vlieger M: Hydrocephalus associated with raised venous pressure. Dev Med Child Neurol 12:Suppl
22~28-32,1970 6. Ellis RWB: Internal hydrocephalus following cerebral thrombosis, in an infant. Proc R Soc Med 30768-772, 1937 7. Emery JL, Zachary RB: Hydrocephalus associated with obliteration of the longitudinal sinus. AMA Arch Dis Child 3 1:288292,1956 8. Epstein R, Hochwald GM, Ransohoff J: Neonatal hydrocephalus treated by compressive head wrapping. Lancet
1~634-636, 1973 9. Ford FR,Murphy EL Increased intracranial pressure. A clinical analysis of causes and characteristics of several types. Bull Johns Hoplcins Hosp 64,369-398,1939 10. Gardner WJ: Otitic sinus thrombosis causing intracranial hypertension. Arch Otolaryngol 30:253-268,1939 11. Gibson JB, Taylor AR, Richardson AE: Congenital meriovenous fistula with an aneurysm of the greaz cerebral vein and hydrocephalus treated surgically. J Neurol Neurosurg Psychiatry 22:224-228,1959 12. Gills JP, Kapp JP, Odom GL: Benign intracranial hypertension: pseudorumor from obstruction of dural sinuses. Arch Ophthalmol 78~592-595,1967 13. Greer M. Benign intracranial hypertension: I. Mastoiditis and lateral obstruction. Neurology (Minneap) 12:472-476.1962 14. Haar FL, Miller CA: Hydrocephalus resulting from superior vena cava thrombosis in an infant. Case report. J Neurosurg
42:597-601,1975 15. Hooper R: Hydrocephalus and obstruction of the superior vena cava in infancy. Pediatrics 28:792-799,1961 16. Janeway R, Kelly D L Papilledema and hydrocephalus associated with recurrent polyneuriris. Arch Neurol 15:507514, 1966 17. Kind ME: Hydrocephalus and the dural venous sinuses. J Neurosurg 19195-201. 1962 18. Ray BS,Dunbar HS: Thrombosisof the dural venous sinuses as a cause of "pseudotumor cerebri." Ann Surg 134:376-386, 1951 19. Russell DS: Observations on the Pathology of Hydrocephalus (Special Report Series, Medical Research Council, No 265). London. H i s Majesty's Stationery Office, 1349 20. Stewan DR, Johnson DG, Myers GG: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J Pediatr Surg 10:771-777,1975
Sagittal Sinus Thrombosis as a Lomplication of Regional Enteritis Bruce Sigsbee, MD, and David A. Rottenberg, M D
A y o u n g woman with r e g i o n a l enteritis and a history of recurrent thrombophlebitis and p u l m o n a r y embolism developed sagittal sinus t h r o m b o s i s during an acute exacerbation of her illness. Repeated attempts to d o c u ment a hypercoagulable state were unsuccessful. Sigsbee B, Rottenbcrg DA: Sagittal sinus thrombosis as a complication of regional enteritis. Ann Neurol 3A50-452, 1978
Neurological complications of regional enteritis, which include tetany, seizures, peripheral neuropa t h y , m y o p a t h y , and subacute combined degeneration of the spinal cord, are usually associated with intestinal malabsorption, metabolic imbalance, or steroid therapy. This paper describes a heretofore unreported complication, widespread cortical venous thrombosis and sagittal sinus occlusion, in a y o u n g woman with regional enteritis and a history of recurrent thrombophlebitis and pulmonary embolism. Case Report A 30-year-old right-handed woman with regional enteritis and a history of classic migraine wils admirted to Memorial Sloan-Kettering Cancer Center (MSKCC) for evaluation of a right hemiparesis. Approximately ten days prior to admission she complained of bifrontal headache associated with intermittent "dizziness." This headache, unlike her usual predominantly right-sided migraine headaches, was nor preceded o r accompanied by visual symptoms o r by focal sensory or motor phenomena. O n e day prior to admission she awoke with blurred vision in the right eye and right-sided numbness; shortly thereafter she had a severe, throbbing left hemicranial headache. Later that afternoon she experienced difficulty using h e r right arm, and by evening she was unable to walk without assistance. W h e n she awoke the next morning, the headache was sall present and her right leg was completely paralyzed, whereupon she was brought to
MSKCC. From the time the patient was 10 years old, s h e had suffered from occasional severe hemicranial headaches pre-
From the Departments of Neurology, Memorial Sloan-Kcttering Cancer Center and Cornell University Medical College, New York, NY. Accepted for publication Nov 23. 1977. Address reprint requests to Dr Rottenbcrg, 1275 York Ave, New York, NY 10021.
450 0364-5 134/7810003-05l6$01.00 @ 1978 by Bruce Sigsbee
ceded by teichopsia and contralateral paresthesias. In 1961, when she was 14 years old, regional enteritis developed. Subsequent flareups of the disease were associated with erythema nodosum and arthritis and were complicated on two occasions by small bowel obstruction. During the four to five weeks prior to admission, and in spite of daily doses of triamcinolone, she experienced an exacerbation of the enteritis associated with mild malabsorption. Twice in 1963, again in 1970, and in September, 1976 (approximately six months prior to admission), the patient had deep vein thrombophlebitis; pulmonary embolization was documented in 1963. Each of these thromboembolic episodes was temporally associated with an exacerbation of the regional enteritis, and on each occasion she was taking pharmacological doses of corticosteroids. On admission the patient appeared chronically ill. Her temperature was 38.4"C. She was lethargic, emotionally labile, intermittently confused, and uncooperative, but not dysphasic. The optic fundi were normal, and spontaneous venous pulsations were present bilaterally. Her visual fields could not be adequately evaluated. Except for mild weakness of the right lower face, no cranial nerve abnormalities were recorded. The right upper and lower extremities were Transfemoral lejt internal carotid arteriogram: venous phase in lateralprojection. There is complete nonjilling of the superior sagittal sinus; extensive anastomotic venous rollaterals over the cerebral convexity drain through a large vein of Labbi into the basal venous sinuses.
hyporeflexic and hypotonic; both plantar responses were flexor. The right leg was externally rotated and plegic, whereas the right arm was only moderately paretic. Pinprick was promptly perceived bilaterally. Her level of consciousness and responsiveness fluctuated markedly from day to day. On the third hospital day she became difficult to arouse, and a lumbar puncture was performed; the opening pressure was 390 mm CSF, and the fluid (clear and colorless) contained no cells, 62 mg protein per deciliter, and 58 mg glucose per deciliter. Serial electroencephalograms were disorganized and slow with intermittent delta activity over the left temporooccipital region. A CAT scan with and without intravenous contrast material was normal. Cerebral angiography revealed widespread cortical vein thrombosis and sagittal sinus occlusion (Figure). The following coagulation studies were normal on at least two occasions: prothrombin time, partial thromboplastin time, thrombin time, reptilase time, fibrinogen concentration, fibrin split products, factor V and factor VIII nctivity, and platelet count. Antithrombin I11 activity was not measured. No pathogens were grown from the urine, sputum, or blood. When, on the ninth hospital day, deep vein thrombophlebitis developed in the left arm, the patient was started on a regimen of sodium warfarin. She improved slowly and was discharged on the twenty-third hospital day with slight residual right lower extremity weakness. Lumbar puncture was repeated one day prior to discharge: the opening pressure was 215 mm CSF, and the fluid contained 1
Brief Communication: Sigsbee and Rottenberg: Sagittal Sinus Thrombosis 45 1
mononuclear cell per cubic millimeter, 17 mg protein per deciliter, and 50 mg glucose per deciliter. Discussion Sagittal sinus thrombosis has not been previously reported as a complication of regional enteritis. This seems somewhat surprising when one considers that cerebral venous thrombosis is a well-known if infrequent complication of ulcerative colitis [2] and that cerebral arterial occlusions have been reported in patients with regional enteritis [ S ] . The association of systemic thromboembolism with inflammatory bowel disease (IBD)’ was recognized in 1936 by Bargen and Barker [l], who postulated that thrombi that form in small rectal veins adjacent to mucosal ulcers propagate through branches of the hypogastric veins to reach more proximal venous channels; they suggested that thrombus propagation was associated with blood hypercoagulability. More recently, thrombocytosis and prolongation of the plasma prothrombin time have been reported in patients with IBD. Lee et al [4] concluded that “the blood of patients with active and extensive ulcerative colitis may be hypercoagulable as a result of acceleration of the early stages of coagulation.” Hypercoagulability per se does not explain the occurrence of cerebral venous thrombosis in our patient with regional enteritis, inasmuch as repeated attempts to document a coagulopathy were unsuccessful and the platelet count remained within normal limits. Although corticosteroids have been shown to inhibit reactive fibrinolysis in experimental animals [3], and our patient was taking pharmacological doses of triamcinolone at the time of admission, one can only speculate on the relationship between the corticosteroid therapy and the pathogenesis of her sagittal sinus thrombosis. Two additional mechanisms may have contributed to the production of widespread cerebral venous thrombosis. Microthrombi originating in pelvic and femoral veins may have become dislodged and found their way into the cerebral venous circulation by way of Batson’s vertebral plexus; such microthrombi might then have served as a nidus for secondary thrombus formation. Skinhoj and Paulson [6] reported reduced regional blood flow during the prodromal phase of a migraine attack, and Wessler [7] provided experimental support for the clinical observation that retarded blood flow predisposes to intravascular thrombosis. It may be that our patient’s prodromal headache was, in fact, migrainous and that the associated venous stasis-in the setting of a hyper‘Inflammatory bowel disease refers to ulcerative colitis and regional enteriris.
452
Annals of Ncurology
Vol 3 No 5 May 1978
coagulable state-was sufficient to initiate thrombus formation in cortical veins and dural sinuses. When a patient with active regional enteritis complains of unusually severe headache and neurological examination reveals focal signs, or signs of increased intracranial pressure, or both, the possibility of sagittal sinus thrombosis should be considered and appropriate diagnostic studies performed without delay. A history of thromboembolism or laboratory evidence of abnormal blood coagulability further supports this diagnosis and increases the need for prompt diagnostic evaluation and treatment. Dr Ronenberg is recipient of Teacher Investigator Award No. 1 KO7 NS 00286-01 from the National Institute of Neurological and Communicative Disorders and Stroke.
References 1. Bargen JA, Barker NW: Extensive arterial and venous thrombosis complicating chronic ulcerative colitis. Arch Intern Med 58:17-31, 1936 2. Borda IT, Southern RF, Brown W F Cercbral venous thrombosis in ulcerative colitis. Gastroenterology 64:116-119, 1973 3. Gerria WBJ. Prakke EM. van der Meer J, et al: Corticosteroids and experimental intravascular coagulation. Scand J Haematol 13:5-10, 1974 4. Lee JCL, Spinell JA, Saver WG, et al: Hypercoagulability associated with chronic ulcerative colitis: changes in blood coagulation factors. Gastroenterology 54:76-85, 1968 5. Silverstein A. Present D H : Cerebrovascular occlusions in relatively young patients with regional enteritis. JAMA 215:976977, 1971 6. Skinhe] E, Paulson OB: Regional blood flow in internal carotid distribution during migraine attack. Br Med J 3:569-570,1969 7. Wessler S: Thrombosis in the presence of vascular stasis. Am J Med 33:648-666. 1962
