å¡ CASE REPORT å¡ Dural Arteriovenous Malformation and Sinus Thromboses in a Patient with Prostate Cancer: An Autopsy Case Nobuo Sakurai, Yasuo Koike, Yoshio Hashizume* and Akira Takahashi A 67-year old man with prostate cancer showed Balint's syndrome, memory disturbance, anosognosia and hallucinations after having been comatose. Radiological findings indicated bilateral dural arteriovenous malformation (DAVM) and thrombosis at the bilateral transverse sinuses and superior sagittal sinus. Pathological findings showed abnormally dilated veins, diffuse neuron loss and gliosis in the parieto-occipital lobe. The chlormadinone and prostate cancer are speculated to have caused the 31: dural sinus thrombosis (Internal Medicine 1032-1037, 1992) which probably induced the DAVM. Key words: dural arteriovenous fistula, dural sinus thrombosis, superior sagittal sinus, transverse sinus, chlormadinone
Intro duction Dural arteriovenous malformations (DAVM) are in frequent lesions in which meningeal and extracranial arteries shunt blood directly into the dural sinuses. The symptoms of DAVM usually are pulsatile tinnitus and headache, depending on the site and venous drainage of the DAVM (1). Some researchers now consider DAVM to be an acquired lesion because dural sinus occlusion may precede its development 3). Here we, describe(2, a patient with prostate cancer com plicated by multiple dural sinus thrombosis and bilateral DAVM of the posterior fossa. The clinical features of this case suggest the pathogenesis of DAVM. Case Report
to his drug treatment regimen. Neurological examination on admission revealed an apathetic face, slurred speech, disturbance of short-term memory and unsteady gait. He often fell asleep, but awakened easily. His neck was supple, and his muscle strength, sensation and reflexes were unremarkable. A fundoscopic examination revealed no abnormalities. His Peripheral visual fieldblood was preserved counts were bynormal the confrontation except for method. a slight decrease of platelets to 120,000/mm3. The prothrombin time was 16.8 s (control value; 15.7 s), and the partial thromboplastin time 32.8 s (42.2 s). Serum fibrinogen was normal. The serum acid phosphatase of prostate origin was normal, but the gamma-semino-protein was elevated to 15 ng/ml. In the cerebrospinal fluid (CSF), protein was 79 mg/dl, glucose 55 mg/dl and the cell count 1/3. No tumor cells were found in the CSF. Electroencephalography His consciousness progressively deteriorated, and he showed slow background activity with sporadic theta waves. became comatose on December 7, 1986. From then on, his state of consciousness, which often was lowered after lumbar puncture, fluctuated between stupor and coma. Left hemiparesis and nuchal rigidity appeared at the end of January 1987. Steroid hormone was used to reduce elevated intracranial pressure. He recovered consciousness gradually during March. He could eat foods and respond to simple commands, but could not gaze at objects in front of him nor grasp them. Neurological findings in May showed
A 67-year-old man who was admitted to our hospital on December 2, 1986 had a 3-month history of gait disturbance, disorientation and generalized malaise. In October 1984, he had undergone a total cysterectomy because of prostate cancer. At the time of surgery, the cancer was found to have metastasized to the abdominal lymph nodes; the biopsy specimen showed poorly differentiated adenocarcinoma. He was given radiation therapy and fluorouracil after the operation. In April 1986, metastases to the cervical spine and rib were found by bone scintigraphy, and the pro gesterone derivative, chlormadinone (Prostal® ), was added From the Department of Neurology, Nagoya University School of Medicine and *the Department of Pathology, Nagoya University Hos Nagoya Received for publication August 26, 1991; Accepted for publication April 23, 1992 Reprint requests should be addressed to Dr. Nobuo Sakurai, the Department of Neurology, Nagoya University School of Medicine, 65 T Showa-ku, Nagoya 466, Japan 1032
Internal
Medicine
Vol. 31, No. 8 (August
1992)
Dural AVMand Sinus Thrombosis memory disturbance, anosognosia, jargon-like speech, confabulation, Balint's syndrome, and hallucination. He began to complain of severe headache and nuchal pain in July. His arms and legs gradually became paralyzed, and in October he became aphonic. In November, he was tetraplegic and could not eat. Cardiopulmonary arrest occurred suddenly, and he died on 15 December 1987 despite emergency resuscitation. The cervical spinogram on admission showed osteoplastic lesions at the 4th and 6th vertebra. Bone scintigraphy showed metastases at the cervical spine and left 4th rib. The initial CT scan done in November 1986 showed the absence of cortical sulci in the occipital lobe (Fig. 1A). The CT scan of January 1987 with contrast material showed areas of linear and nodular enhancement in the occipital lobe that represented enlarged cerebral veins. The CT scan done in February, when left hemiparesis was obvious, showed a subcortical hemorrhage in the right parieto-temporal lobe (Fig. IB). Contrast-enhanced CT showed abnormally enlarged veins in the temporo-parieto-occipital lobe (Fig.
1C). The CT done in June revealed mild ventricular dilata tion and a decrease in the abnormally enhanced area (Fig. ID). His last CT scan in October 1987 showed enlargement of the lateral ventricles and sulci of the frontal lobe with periventricular lucency, but there were still no sulci in the occipital lobe (Fig. IE, F). Retrograde vertebral angiography in January 1987 showed a delayed arterial phase without DAVM (Fig. 2A). The transverse sinuses could not be seen during the venous phase (Fig. 2B). A four-vessel study was made in September 1987. The bilateral occipital arteries were enlarged and had direct fistulous communication to the straight and transverse sinuses (Fig. 3A, B). The posterior half of the superior sagittal sinus (SSS) could not be seen, and the distal portions of the transverse werefrom not opacified bilaterally. Consequently, thesinuses shunt flow the DAVM drained to the cortical veins in the parieto-occipital lobe and partially to those in the temporal lobe. The blood flow passed the anterior half of the SSS and cortical veins in the retrograde direction, and drained into the cavernous sinus and collateral
Fig. 1. The initial CT scan showing the absence of cortical sulci in the occipital lobe (A). CT scan of February 1987 showing subcortica hemorrhage in the right parieto-temporal lobe (B). Enhanced CT two weeks later showing abnormally enlarged veins in the temporo-p occipital lobe (C). Enhanced CT of June 1987 showing mild ventricular enlargement and a decrease in the area of abnormally dilated v (D). The last CT scan, October 1987, showing enlarged lateral ventricles and sulci of the frontal lobe with periventricular lucency, but still are no sulci in the occipital lobe (E, F). Internal
Medicine
Vol. 31, No. 8 (August
1992)
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Sakurai et al
Fig. 2. Retrograde vertebral angiography showing a delayed arterial phase without dural arteriovenous malformation (DAVM) (A). During the late venous phase, cortical vessels in the occipital lobe (arrow) still are visible but the transverse sinuses are not
veins in the base of the skull (Fig. 3C, D). The dural branch of the right vertebral artery was connected to the dural sinus. Embolization of the DAVM was not done because of the poor prognosis of this patient. At autopsy, the dura covering the hemisphere was grossly normal. The SSS was patent anteriorly but abruptly occluded by fibrous gray tissue from the level of the parietal lobe to the venous confluence. Arteriosclerotic changes in the circle of Willis were minimal. The brain showed no signs of edema or herniation. All the surface veins of the brain were engorged and tortuous, in particular, at the parieto occipital lobes. On coronal sectioning, a small absorbed hematoma was found in the white matter of the right superior temporal gyrus. Dilated and congested veins were present deep in the sulci of the cerebral and cerebellar hemisphere. Under the light microscope, the lumen of the SSS was seen to be obliterated by a loose fibrous organized thrombus. Abnormal vascular channels appeared in the thickened dura and lumen of the SSS (Fig. 4A); the walls were arterial with smooth muscle and internal elastic lamina (Fig. 4B). The leptomeninges contained a number of en gorged veins with very thin walls or with thickened fibrous walls. The cortex of the parieto-occipital lobe was com Similar presentdilated to a moderate degree in the pressed changes by these were abnormally veins, and cortical frontal lobe, basal ganglia, thalamus, cerebellum and neuron loss with reactive gliosis was observed (Fig. 5). brainstem. Extradural metastasis of the prostate cancer was found in the upper cervical area. The spinal cord was com pressed at the 3-5 cervical level with secondary spinal infarction. Discussion No case of prostate cancer complicated by dural sinus 1034
thrombosis or with DAVM has been reported. Yazaki et al (4), however, reported decreased platelet serotonin and normal plasma serotonin in patients with untreated prostate cancer, which may indicate lasting platelet consumption in this disease (4). Disseminated intravascular coagulation sometimes occurs in patients with prostate cancer (5). Inman et al (6) showed a positive correlation between the dose of estrogen and the risk of thrombosis. Although chlormadi none is a derivative of progesterone, not estrogen, throm botic disorders have been reported in patients under chlormadinone therapy (7, 8). There was no conclusive evidence for an abnormality in the blood coagulation system in the patient's data onsometimes admission.accompany We speculate, however, Patients with DAVM venous sinus that the chlormadinone and prostate of cancer may both or have thrombosis, in particular, occlusion the transverse contributed to the formation of his sinus thrombosis. sigmoid sinuses (2, 3, 9). Most of these patients have DAVM in the posterior fossa. No case of DAVM complicated by occlusion of the SSS and bilateral transverse sinuses, except thatreports of ourhave patient, has been reported in the literature. Aforfew suggested that some DAVM are acquired and that sinus thrombosis has an important role in the pathogenesis of DAVM (2, 3). Houser et al (2) pro posed that indigenous dysplastic dural vessels present within the sinus develop further and establish direct artery-to-sinus communication during the organization of an intravenous thrombosis. The first angiography done on our patient showed occlusion of the distal portion of the bilateral transverse sinuses without DAVM. The abnormal vessels withsuggest internalthat elastic lamina present the wall induced of the SSS We the increased venousinpressure by are considered the sinus thrombosis to havecaused been indigenous the arterio-venous congenital shunt vessels. in these abnormal vessels. Internal
Medicine
Vol. 31,
No. 8 (August
1992)
Dural AVMand Sinus Thrombosis
Fig. 3. Right common carotid angiography showing the enlarged occipital artery (arrow, A, B) and direct fistulous communication to the straight sinus (SS), transverse sinuses (TS) and cerebellar vein (arrowheads, A, B). The superior sagittal sinus (SSS) is occluded from the vertex to the confluens sinuum, and the distal portion of the TS is occluded bilaterally. Deep cerebral veins were barely visible. The shunt flow from the DAVM drains into the cortical vein in the parieto-occipital lobe, then passes the SSS and cortical vein (arrows, C, D) in the retrograde direction. Eventually, the blood drains into the cavernous sinus (large arrowheads, C, D) and collateral vein (arrowheads, C, D) in the base of the skull through the sylvian vein (broad open arrows, C, D). The clinical course of our patient can be divided into three stages: The first is the period from the onset of illness to the comatose state. The absence of sulci in the occipital area on the initial CT scan indicates the thrombus was Internal
Medicine
Vol. 31, No. 8 (August
1992)
present in the transverse sinus in the very early phase of illness. The fluctuation in the patient's state of consciousness before admission indicates the subacute evolution of the thrombosis. His progressive comatose state after admission 1035
Sakurai et al
Fig. 4. Complete occlusion of the lumen of the SSS by the organized thrombus (A) (elastic Van Gieson stain, x 8.6). Abnormal vascular channels present in the lumen of the SSS have arterial walls with smooth muscle and internal elastic lamina (B) (elastic Van Gieson stain, x 34). intracranial hemodynamics and the elevated intracranial pressure explain the disturbed consciousness experienced during this stage. The degree of abnormal dilatation of his veins showed a decrease on the CT done after recovery of consciousness. This indicates an improvement in venous drainage because of the development of collateral blood flow. Clinical signs observed during the chronic dementia stage were consistent with the predominant pathological changes in the parieto occipital area. Sawada et al (10) reported a patient with sinus thrombosis and DAVM who presented the dementia syndrome. They ascribed the cause of dementia to the mass effect of dilated veins and chronic ischemia induced by Fig. 5. The cortex compressed by abnormally dilated veins in the DAVM (10). We also consider that the mass effect, chronic ischemia, and secondary neuron loss caused the neurological subarachnoidal spaces (HE stain, x 10). Epidural metastasis the 3-5th segments of the deficiencies presentofduring this stage. cervical cord and secondary disturbance of spinal circula suggests an additional thrombosis of the SSS. The rapid rise tion resulted in tetraplegia during the late stage. This lesion in venous pressure in the posterior fossa induced the DAVM probably caused respiratory arrest because the phrenic nucld which, in turn, increased the venous pressure. Venous are need located at this spinal We to be aware thatlevel. patients with prostate cancer rupture probably was the cause of the intracerebral who undergo hormone therapy are at risk of developing hemorrhage at this stage because the patient had normal sinus thrombosis. DAVM with sinus thrombosis therefore blood pressure and there were minimal arteriosclerotic should be differentiated from other diseases that cause changes in his cerebral arteries. The acute change in 1036
Internal
Medicine
Vol. 31,
No. 8 (August
1992)
Dural AVMand Sinus Thrombosis fluctuating consciousness and dementia syndrome.
vascular coagulation in the urologic patient. J Urol 116: 1, 1976. Inman WHW, Vessey MP, Westerholm B, Engelund A. Throm References boembolic disease and the steroidal content of oral contraceptives. A report to the committee on safety of drugs. Br Med J 2: 204, 1970. De Gennes JL, Turpin G, Baulac L. Accidents cardio-vascularies 1) Gaston A, Chiras J, Martin N, Meder JF, Sebag G, Dao TH. Fistules arterio-veineuses de la dure-mere cranienne. J Neuroradiol 15: 160, ischemiques au cours d'un traitement par progestatifs seuls. A propos de 3 observations. Ann Endocrinol 40: 47, 1979. 1988. 2) Houser OW, Campbell JK, Campbell RJ, Sundt TM. ArteriovenousAdam H, Schirmaer B. Nebenwirkungen der oralen Ovulations hemmer. Z Arztl Fortbild 67: 1078, 1973. malformation affecting the transverse dural venous sinus - An Handa J, Yoneda S, Handa H. Venous sinus occlusion with a dural acquired lesion. Mayo Clin Proc 54: 651, 1979. 3) Chaudhary MY, Sachdev VP, Cho SH, Weitzner I, Puljic S, Huangarteriovenous malformation of the posterior fossa. Surg Neurol 4: YP. Dural arteriovenous malformation of the major venous sinuses: 433, 1975. Sawada H, Akiguchi I, Kimura T, Fukuyama H, Kameyama M. An acquired lesion. Am J Neuroradiol 3: 13, 1982. 4) Yazaki T, Kanoh S, Inage H, et al. Studies on platelet function in A case of venous dural sinus thrombosis presenting dementia syndrome. An autopsy case. Clin Neurol 29: 318, 1989. patients with prostatic cancer. Urology 30: 60, 1987. 5) Pergament ML, Swaim WR, Blackard CE. Disseminated intra-
Internal
Medicine
Vol. 31, No. 8 (August
1992)
1037
Intro duction Dural arteriovenous malformations (DAVM) are in frequent lesions in which meningeal and extracranial arteries shunt blood directly into the dural sinuses. The symptoms of DAVM usually are pulsatile tinnitus and headache, depending on the site and venous drainage of the DAVM (1). Some researchers now consider DAVM to be an acquired lesion because dural sinus occlusion may precede its development 3). Here we, describe(2, a patient with prostate cancer com plicated by multiple dural sinus thrombosis and bilateral DAVM of the posterior fossa. The clinical features of this case suggest the pathogenesis of DAVM. Case Report
to his drug treatment regimen. Neurological examination on admission revealed an apathetic face, slurred speech, disturbance of short-term memory and unsteady gait. He often fell asleep, but awakened easily. His neck was supple, and his muscle strength, sensation and reflexes were unremarkable. A fundoscopic examination revealed no abnormalities. His Peripheral visual fieldblood was preserved counts were bynormal the confrontation except for method. a slight decrease of platelets to 120,000/mm3. The prothrombin time was 16.8 s (control value; 15.7 s), and the partial thromboplastin time 32.8 s (42.2 s). Serum fibrinogen was normal. The serum acid phosphatase of prostate origin was normal, but the gamma-semino-protein was elevated to 15 ng/ml. In the cerebrospinal fluid (CSF), protein was 79 mg/dl, glucose 55 mg/dl and the cell count 1/3. No tumor cells were found in the CSF. Electroencephalography His consciousness progressively deteriorated, and he showed slow background activity with sporadic theta waves. became comatose on December 7, 1986. From then on, his state of consciousness, which often was lowered after lumbar puncture, fluctuated between stupor and coma. Left hemiparesis and nuchal rigidity appeared at the end of January 1987. Steroid hormone was used to reduce elevated intracranial pressure. He recovered consciousness gradually during March. He could eat foods and respond to simple commands, but could not gaze at objects in front of him nor grasp them. Neurological findings in May showed
A 67-year-old man who was admitted to our hospital on December 2, 1986 had a 3-month history of gait disturbance, disorientation and generalized malaise. In October 1984, he had undergone a total cysterectomy because of prostate cancer. At the time of surgery, the cancer was found to have metastasized to the abdominal lymph nodes; the biopsy specimen showed poorly differentiated adenocarcinoma. He was given radiation therapy and fluorouracil after the operation. In April 1986, metastases to the cervical spine and rib were found by bone scintigraphy, and the pro gesterone derivative, chlormadinone (Prostal® ), was added From the Department of Neurology, Nagoya University School of Medicine and *the Department of Pathology, Nagoya University Hos Nagoya Received for publication August 26, 1991; Accepted for publication April 23, 1992 Reprint requests should be addressed to Dr. Nobuo Sakurai, the Department of Neurology, Nagoya University School of Medicine, 65 T Showa-ku, Nagoya 466, Japan 1032
Internal
Medicine
Vol. 31, No. 8 (August
1992)
Dural AVMand Sinus Thrombosis memory disturbance, anosognosia, jargon-like speech, confabulation, Balint's syndrome, and hallucination. He began to complain of severe headache and nuchal pain in July. His arms and legs gradually became paralyzed, and in October he became aphonic. In November, he was tetraplegic and could not eat. Cardiopulmonary arrest occurred suddenly, and he died on 15 December 1987 despite emergency resuscitation. The cervical spinogram on admission showed osteoplastic lesions at the 4th and 6th vertebra. Bone scintigraphy showed metastases at the cervical spine and left 4th rib. The initial CT scan done in November 1986 showed the absence of cortical sulci in the occipital lobe (Fig. 1A). The CT scan of January 1987 with contrast material showed areas of linear and nodular enhancement in the occipital lobe that represented enlarged cerebral veins. The CT scan done in February, when left hemiparesis was obvious, showed a subcortical hemorrhage in the right parieto-temporal lobe (Fig. IB). Contrast-enhanced CT showed abnormally enlarged veins in the temporo-parieto-occipital lobe (Fig.
1C). The CT done in June revealed mild ventricular dilata tion and a decrease in the abnormally enhanced area (Fig. ID). His last CT scan in October 1987 showed enlargement of the lateral ventricles and sulci of the frontal lobe with periventricular lucency, but there were still no sulci in the occipital lobe (Fig. IE, F). Retrograde vertebral angiography in January 1987 showed a delayed arterial phase without DAVM (Fig. 2A). The transverse sinuses could not be seen during the venous phase (Fig. 2B). A four-vessel study was made in September 1987. The bilateral occipital arteries were enlarged and had direct fistulous communication to the straight and transverse sinuses (Fig. 3A, B). The posterior half of the superior sagittal sinus (SSS) could not be seen, and the distal portions of the transverse werefrom not opacified bilaterally. Consequently, thesinuses shunt flow the DAVM drained to the cortical veins in the parieto-occipital lobe and partially to those in the temporal lobe. The blood flow passed the anterior half of the SSS and cortical veins in the retrograde direction, and drained into the cavernous sinus and collateral
Fig. 1. The initial CT scan showing the absence of cortical sulci in the occipital lobe (A). CT scan of February 1987 showing subcortica hemorrhage in the right parieto-temporal lobe (B). Enhanced CT two weeks later showing abnormally enlarged veins in the temporo-p occipital lobe (C). Enhanced CT of June 1987 showing mild ventricular enlargement and a decrease in the area of abnormally dilated v (D). The last CT scan, October 1987, showing enlarged lateral ventricles and sulci of the frontal lobe with periventricular lucency, but still are no sulci in the occipital lobe (E, F). Internal
Medicine
Vol. 31, No. 8 (August
1992)
1033
Sakurai et al
Fig. 2. Retrograde vertebral angiography showing a delayed arterial phase without dural arteriovenous malformation (DAVM) (A). During the late venous phase, cortical vessels in the occipital lobe (arrow) still are visible but the transverse sinuses are not
veins in the base of the skull (Fig. 3C, D). The dural branch of the right vertebral artery was connected to the dural sinus. Embolization of the DAVM was not done because of the poor prognosis of this patient. At autopsy, the dura covering the hemisphere was grossly normal. The SSS was patent anteriorly but abruptly occluded by fibrous gray tissue from the level of the parietal lobe to the venous confluence. Arteriosclerotic changes in the circle of Willis were minimal. The brain showed no signs of edema or herniation. All the surface veins of the brain were engorged and tortuous, in particular, at the parieto occipital lobes. On coronal sectioning, a small absorbed hematoma was found in the white matter of the right superior temporal gyrus. Dilated and congested veins were present deep in the sulci of the cerebral and cerebellar hemisphere. Under the light microscope, the lumen of the SSS was seen to be obliterated by a loose fibrous organized thrombus. Abnormal vascular channels appeared in the thickened dura and lumen of the SSS (Fig. 4A); the walls were arterial with smooth muscle and internal elastic lamina (Fig. 4B). The leptomeninges contained a number of en gorged veins with very thin walls or with thickened fibrous walls. The cortex of the parieto-occipital lobe was com Similar presentdilated to a moderate degree in the pressed changes by these were abnormally veins, and cortical frontal lobe, basal ganglia, thalamus, cerebellum and neuron loss with reactive gliosis was observed (Fig. 5). brainstem. Extradural metastasis of the prostate cancer was found in the upper cervical area. The spinal cord was com pressed at the 3-5 cervical level with secondary spinal infarction. Discussion No case of prostate cancer complicated by dural sinus 1034
thrombosis or with DAVM has been reported. Yazaki et al (4), however, reported decreased platelet serotonin and normal plasma serotonin in patients with untreated prostate cancer, which may indicate lasting platelet consumption in this disease (4). Disseminated intravascular coagulation sometimes occurs in patients with prostate cancer (5). Inman et al (6) showed a positive correlation between the dose of estrogen and the risk of thrombosis. Although chlormadi none is a derivative of progesterone, not estrogen, throm botic disorders have been reported in patients under chlormadinone therapy (7, 8). There was no conclusive evidence for an abnormality in the blood coagulation system in the patient's data onsometimes admission.accompany We speculate, however, Patients with DAVM venous sinus that the chlormadinone and prostate of cancer may both or have thrombosis, in particular, occlusion the transverse contributed to the formation of his sinus thrombosis. sigmoid sinuses (2, 3, 9). Most of these patients have DAVM in the posterior fossa. No case of DAVM complicated by occlusion of the SSS and bilateral transverse sinuses, except thatreports of ourhave patient, has been reported in the literature. Aforfew suggested that some DAVM are acquired and that sinus thrombosis has an important role in the pathogenesis of DAVM (2, 3). Houser et al (2) pro posed that indigenous dysplastic dural vessels present within the sinus develop further and establish direct artery-to-sinus communication during the organization of an intravenous thrombosis. The first angiography done on our patient showed occlusion of the distal portion of the bilateral transverse sinuses without DAVM. The abnormal vessels withsuggest internalthat elastic lamina present the wall induced of the SSS We the increased venousinpressure by are considered the sinus thrombosis to havecaused been indigenous the arterio-venous congenital shunt vessels. in these abnormal vessels. Internal
Medicine
Vol. 31,
No. 8 (August
1992)
Dural AVMand Sinus Thrombosis
Fig. 3. Right common carotid angiography showing the enlarged occipital artery (arrow, A, B) and direct fistulous communication to the straight sinus (SS), transverse sinuses (TS) and cerebellar vein (arrowheads, A, B). The superior sagittal sinus (SSS) is occluded from the vertex to the confluens sinuum, and the distal portion of the TS is occluded bilaterally. Deep cerebral veins were barely visible. The shunt flow from the DAVM drains into the cortical vein in the parieto-occipital lobe, then passes the SSS and cortical vein (arrows, C, D) in the retrograde direction. Eventually, the blood drains into the cavernous sinus (large arrowheads, C, D) and collateral vein (arrowheads, C, D) in the base of the skull through the sylvian vein (broad open arrows, C, D). The clinical course of our patient can be divided into three stages: The first is the period from the onset of illness to the comatose state. The absence of sulci in the occipital area on the initial CT scan indicates the thrombus was Internal
Medicine
Vol. 31, No. 8 (August
1992)
present in the transverse sinus in the very early phase of illness. The fluctuation in the patient's state of consciousness before admission indicates the subacute evolution of the thrombosis. His progressive comatose state after admission 1035
Sakurai et al
Fig. 4. Complete occlusion of the lumen of the SSS by the organized thrombus (A) (elastic Van Gieson stain, x 8.6). Abnormal vascular channels present in the lumen of the SSS have arterial walls with smooth muscle and internal elastic lamina (B) (elastic Van Gieson stain, x 34). intracranial hemodynamics and the elevated intracranial pressure explain the disturbed consciousness experienced during this stage. The degree of abnormal dilatation of his veins showed a decrease on the CT done after recovery of consciousness. This indicates an improvement in venous drainage because of the development of collateral blood flow. Clinical signs observed during the chronic dementia stage were consistent with the predominant pathological changes in the parieto occipital area. Sawada et al (10) reported a patient with sinus thrombosis and DAVM who presented the dementia syndrome. They ascribed the cause of dementia to the mass effect of dilated veins and chronic ischemia induced by Fig. 5. The cortex compressed by abnormally dilated veins in the DAVM (10). We also consider that the mass effect, chronic ischemia, and secondary neuron loss caused the neurological subarachnoidal spaces (HE stain, x 10). Epidural metastasis the 3-5th segments of the deficiencies presentofduring this stage. cervical cord and secondary disturbance of spinal circula suggests an additional thrombosis of the SSS. The rapid rise tion resulted in tetraplegia during the late stage. This lesion in venous pressure in the posterior fossa induced the DAVM probably caused respiratory arrest because the phrenic nucld which, in turn, increased the venous pressure. Venous are need located at this spinal We to be aware thatlevel. patients with prostate cancer rupture probably was the cause of the intracerebral who undergo hormone therapy are at risk of developing hemorrhage at this stage because the patient had normal sinus thrombosis. DAVM with sinus thrombosis therefore blood pressure and there were minimal arteriosclerotic should be differentiated from other diseases that cause changes in his cerebral arteries. The acute change in 1036
Internal
Medicine
Vol. 31,
No. 8 (August
1992)
Dural AVMand Sinus Thrombosis fluctuating consciousness and dementia syndrome.
vascular coagulation in the urologic patient. J Urol 116: 1, 1976. Inman WHW, Vessey MP, Westerholm B, Engelund A. Throm References boembolic disease and the steroidal content of oral contraceptives. A report to the committee on safety of drugs. Br Med J 2: 204, 1970. De Gennes JL, Turpin G, Baulac L. Accidents cardio-vascularies 1) Gaston A, Chiras J, Martin N, Meder JF, Sebag G, Dao TH. Fistules arterio-veineuses de la dure-mere cranienne. J Neuroradiol 15: 160, ischemiques au cours d'un traitement par progestatifs seuls. A propos de 3 observations. Ann Endocrinol 40: 47, 1979. 1988. 2) Houser OW, Campbell JK, Campbell RJ, Sundt TM. ArteriovenousAdam H, Schirmaer B. Nebenwirkungen der oralen Ovulations hemmer. Z Arztl Fortbild 67: 1078, 1973. malformation affecting the transverse dural venous sinus - An Handa J, Yoneda S, Handa H. Venous sinus occlusion with a dural acquired lesion. Mayo Clin Proc 54: 651, 1979. 3) Chaudhary MY, Sachdev VP, Cho SH, Weitzner I, Puljic S, Huangarteriovenous malformation of the posterior fossa. Surg Neurol 4: YP. Dural arteriovenous malformation of the major venous sinuses: 433, 1975. Sawada H, Akiguchi I, Kimura T, Fukuyama H, Kameyama M. An acquired lesion. Am J Neuroradiol 3: 13, 1982. 4) Yazaki T, Kanoh S, Inage H, et al. Studies on platelet function in A case of venous dural sinus thrombosis presenting dementia syndrome. An autopsy case. Clin Neurol 29: 318, 1989. patients with prostatic cancer. Urology 30: 60, 1987. 5) Pergament ML, Swaim WR, Blackard CE. Disseminated intra-
Internal
Medicine
Vol. 31, No. 8 (August
1992)
1037
