Intracranial Hypotension Concurrent Presented with Pseudo-Subarachnoid Hemorrhage and Transverse Sinus Thrombosis: A Case Report

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1 NNQ Intracranial Hypotension Concurrent Presented with Pseudo-Subarachnoid Hemorrhage and Transverse Sinus Thrombosis: A Case Report Chieh-Yang Cheng 1, Che-Chuan Wang 1, Tai-Yuan Chen 2, Jinn-Rung Kuo 1,3 Departments of 1 Neurosurgery, 2 Radiology, Chi-Mei Medical Center, Yung Kang City, Tainan, Taiwan 3 Department of Biotechnology, Southern Taiwan University of Technology, Tainan, Taiwan J Taiwan Emerg Med 2010;12: ABSTRACT A 35-year-old male who presented acute on chronic onset of headache followed by vomiting and neck pain came to our emergency department in the mid-night. Initial brain CT revealed pseudo-subarachnoid hemorrhage (SAH). Misdiagnosis of cerebral aneurysm rupture with spontaneous SAH was diagnosed initially. On the next day, brain MRI showed right transverse sinus thrombosis, bilateral subdural effusion, diffuse pachymeningeal enhancement and C1-2 CSF leakage confirmed the diagnosis of intracranial hypotension. The headache improved after treatment of intravenous analgesia, hydration, administration of caffeine, and corticosteroid medication. We believe this is the first such case of intracranial hypotension concurrently presented with pseudo-sah and transverse sinus thrombosis. Keywords: headache, intracranial hypotension, sinus thrombosis, subarachnoid hemorrhage, subdural effusion INTRODUCTION The prevalence of headache in the general population is approximately 16% which causes of up to 2.7% of patient visiting to US emergency departments annually. 1,2 Among the rest, spontaneous subarachnoid hemorrhage with aneurysm rupture is a major cause of headache. However, spontaneous intracranial hypotension is rare but important and can cause persistent daily headaches. It is often misdiagnosed in the emergency department setting. 3,4 We report a case of intracranial hypotension in which the initial clinical presentation and brain CT findings mimicked spontaneous subarachnoid hemorrhage (SAH). The initial diagnosis was SAH which prompted inappropriate treatment. However, later magnetic resonance imaging (MRI) showed bilateral subdural effusion, diffuse pachymeningeal enhancement, and right transverse sinus thrombosis. We believe this is the first such case of intracranial hypotension concurrently presenting with pseudo-sah and transverse sinus thrombosis. We bring this complication of misdiagnosis to the attention of the readers because recognition of the possible pseudo-sah in intracranial hypotension is very important for providing prompt and appropriate therapy to patients. CASE REPORT A 35-year-old right-handed man, a laborer, was brought to the emergency department because of persistent occipito-frontal headache for duration of 4 weeks, which became increasingly severe and were followed by nausea and vomiting in the recent 2 days. The headache worsened in the upright position and improved when lying down. The patient s medical history was unremarkable. He had no fever or chills recently. He denied any recent trauma or medical procedure, including lumbar puncture or chiropractic. The patient s initial examination showed the following vital signs: blood pressure, 162/94 mmhg; regular pulse, 74 beats/ minute; and Received: August 16, 2010 Accepted: September 17, 2010 Address for requests and correspondence to: Jinn-Rung Kuo, Departments of Neurosurgery, Chi-Mei Medical Center, 901, Chung Hwa Road, Yung Kang, Tainan, Taiwan Tel: kuo.ox@msa.hinet.net J Taiwan Emerg Med December 2010 Vol 12 No 4

2 Intracranial hypotension appears like SAH NNR temperature, 36.8 C. The neurological examination showed clear consciousness, isochoric pupils with rapid light reflexes and no papilledema. The patient had headache and neck pain when flexing his neck. The rest of the neurological and physical examinations were unremarkable. Emergent brain computed tomography (CT) (Figure 1) showed basal cistern, tentorium, and interhemispheric hemorrhage. Under the impression of spontaneous subarachnoid hemorrhage with aneurysm rupture, the patient underwent CT-angiography. The result revealed no aneurysm, but sagging of the suprasellar cistern. He received Nimodipine, analgesia, and mannitol treatment and was transferred to the intensive care ward. Unfortunately, the symptoms persisted and worsened. On the next day, brain magnetic resonance imaging (MRI) showed bilateral thin subdural effusions (Figure 2A), post-contrast enhancement of a thickened dura on both convexities and along the tentorium, marked sagging of the brain stem (Figure 2B), and right transverse sinus thrombosis (Figure 2C), consistent with spontaneous intracranial hypotension. Venous MRI of the brain also revealed signal abnormalities consistent with right transverse sinus thrombosis (Figure 3). Screening studies for Figure 1. Computed tomography (CT) scan of brain showing increased attenuation in the basal cisterns, along the tentorium cerebelli, and along the interhemisphere cerebri resembiling subarachnoid hemorrhage. Figure 3. Venous MR angiography shows extensive right transverse sinus thrombosis (arrow). Figure 2. (A) Coronal T2-weighted MRI shows bilateral subdural effusion (arrow) (B) Coronal enhanced T1-weighted MRI shows diffuse pachymeningeal enhancement(arrow) (C) Axial enhanced T1-weightedMRI shows right transverse sinus thrombosis (arrow). J Taiwan Emerg Med December 2010 Vol 12 No 4

3 NNS Figure 4. Single-shot fast spin echo axial cervical spinal MRI shows C1-2 CSF leakage (arrow). infectious, immunological, hematological, and systemic disorders did not disclose any conventional cause of venous sinus thrombosis. Coagulation testing including prothrombin time, activated prothrombin time, and anticardiolipin antibody titer, antiphospholipid antibody titer, homocystine, proteins S and C, and antithrombin III were within normal limits. Transcranial Doppler sonography demonstrated normal flow velocity and pulsatile index of the major vessels in the basal cistern. Single-shot fast spinal echography of axial cervical spinal MRI showed C1-2 (Figure 4) cerebrospinal fluid (CSF) leakage. The patient s diagnosis was revised to intracranial hypotension at this time. We changed the patient s treatment protocol from dehydration with mannitol to aggressive hydration with normal saline. The headaches significantly improved with intensive intravenous analgesia, hydration, and administration of caffeine, corticosteroid, and bed rest with use of an abdominal binder. No blood patch treatemnt was arranged for CSF leakage in this patient. DISCUSSION The typical findings of intracranial hypotension include the classic presenting symptom of severe positional headache with normal brain CT, and MRI with gadolinium contrast typically showing diffuse enhancement of the dura mater and dural venous dilatation, and bilateral subdural effusion. Additionally, spinal radionuclide cisternography reveals CSF leakage. 1,5 In addition to the clinical presentations and imaging studies, the diagnosis of intracranial hypotension requires a CSF pressure measurement by lumbar tapping of less than 60 mmh2o. Our patient did not undergo lumbar tapping and radionuclide cisternography due to the risk of cerebral herniation and venous thrombosis. Although we could not confirm the presence of intracranial hypotension by CSF pressure measurement, the clinical presentation and MRI findings strongly suggested this diagnosis. Pseudo-SAH is defined as increasing attenuation in the basilar cisterns, along the tentorium and falx resembling subarachnoid hemorrhage without any blood in the subarachnoid space on brain CT. It is most commonly seen in patients with diffuse cerebral edema and is believed to be due to vascular engorgement. 6 In a literature review, Schievink et al reported that 4 of 40 patients with intracranial hypotension presented with pseudo-sah and effacement of the basal cistern based on brain CT study. 7 They reasoned that the increases in attenuation of the anterior and middle cerebral arteries, combined with obliteration of the basilar cistern and sylvian fissures were secondary to the brain sagging due to intracranial hypotension. Because intracranial hypotension mimics SAH on CT, it is not surprising that the possibility of misdiagnosis and inappropriate treatment is high, as we encountered with our patient. Fortunately, when the patient failed to respond to treatment, we quickly identified the correct diagnosis and changed our treatment protocol from dehydration with mannitol to aggressive hydration with normal saline, within 1 day. No sequelae occurred except that he suffered greater headache during the initial management. However, we want to emphasize that intracranial hypotension could be presented with pseudo-sah and effacement of the basal cistern based on brain CT study. Dural sinus thrombosis is rarely associated with spontaneous intracranial hypotension. 8,9 The current hypothesis explaining dural sinus thrombosis in SAH is that engorgement of the venous system due to the compensatory venous hypervolemia favors thrombosis within the dural sinuses. The increase in blood volume and slowing of circulation in the dural sinuses can lead to thrombus formation. In our patient, the features of both dural sinus thrombosis and intracranial hypotension were found on the MRI study. We believe that the sequence of events in our patient were dural sinus thrombosis followed intracranial hypotension because of the changes in the patient s headache pattern, associated with vomiting and neck pain. In conclusion, this case demonstrates an association between pseudo-sah, right transverse sinus thrombosis, and intracranial hypotension. This imaging presentation, to our knowledge, has not been previously reported. Besides demonstrating the imaging findings, we also emphasize that among patients with persistent orthostatic headache from the history, brain CT revealing effacement of the basal cistern and mannitol infusion worsening the hadache, the possibility of intracranial hypotension must be included in the differential diagnosis to provide prompt and appropriate patient therapy. Only increasing the awareness of this spontaneous form of positional headache will likely result in fewer patients in whom this diagnosis is missed J Taiwan Emerg Med December 2010 Vol 12 No 4

4 Intracranial hypotension appears like SAH NNT REFERENCES 1. Frank LR, Paxson A, Brake J. Spontaneous intracranial hypotension A case report. J Emerg Med 2005; 28: Rasmussen B, Jensen R, Schroll M, et al. Epidemiology of headache in a general population a prevalence study. J Clin Epidemio. 1991;44: Malone RE, Love JN. Spontaneous intracranial hypotension: case report and review of the literature. J Emerg Med 2007;32: Schievink WI. Misdiagnosis of spontaneous intracranial hypotension. Arch Neurol 2003;60: Wiesemann E, Berding G, Goetz F, et al. Spontaneous intracranial hypotension correlation of imaging findings with clinical features. Eur Neurol 2006;56: Given CA, Burdette JH, Elster AD, et al. Pseudo-subarachnoid hemorrhage: a potential imaging pitfall associated with diffuse cerebral edema. Am J Neuroradiol 2003;24: Schievink WI, Maya M, Tourje J, et al. Pseudo-subarachnoid hemorrhage: a CT-finding in spontaneous intracranial hypotension. Neurology 2005;65: Gutierrez E, Cuenca R, Vazquez AV, Gonzalez JL, Egido JA (2004). May intracranial hypotension be a cause of venous sinus thrombosis? Eur Neurol 51: Savoiardo M, Armenise S, Spagnolo P, et al. Dural sinus thrombosis in spontaneous intracranial hypotension. J Neurol 2006;253: J Taiwan Emerg Med December 2010 Vol 12 No 4

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