Rare cause of cerebral venous sinus thrombosis:Spontaneous intracranial hypotension syndrome:A case report

BACKGROUND Spontaneous intracranial hypotension syndrome is a relatively uncommon neurological disorder of unknown etiology with a good prognosis.Cerebral venous sinus thrombosis is a specific type of cerebrovascular disease caused by multiple etiologies of cerebral venous sinus or vein thrombosis that obstructs cerebral venous return and is associated with impaired cerebrospinal fluid absorption;this entity is rarely seen clinically.Spontaneous intracranial hypotension syndrome is one of the causes of cerebral venous sinus thrombosis,and the probability of their combined occurrence is only 1%-2%.As such,it is easily overlooked clinically,thus increasing the difficulty of diagnosis and treatment.CASE SUMMARY A 29-year-old young woman presented with postural headache.Lumbar puncture suggested a pressure of 50 mmH2O(normal 80 mmH2O-180 mmH2O),and magnetic resonance imaging cerebral venography suggested thrombosis of the supratentorial sinus.These findings were considered indicative of cerebral venous sinus thrombosis due to spontaneous intracranial hypotension syndrome after ruling out immunological causes,tumor,infection,abnormal coagulation mechanism,and hypercoagulable state,etc.She was treated with rehydration and low-molecular heparin anticoagulation for 15 d,and follow-up magnetic resonance imaging cerebral venography suggested resolution of the thrombus.The patient had complete improvement of her headache symptoms.CONCLUSION Spontaneous intracranial hypotension syndrome is one of the rare causes of cerebral venous sinus thrombosis,which is frequently misdiagnosed or missed and deserves consideration by clinicians during differential diagnosis.Dehydration should be avoided in such patients,and early rehydration and anticoagulation therapy are effective treatment options.

Management of an intracranial hypotension patient with diplopia as the primary symptom:A case report

BACKGROUND Intracranial hypotension(IH)is a disorder involving cerebrospinal fluid(CSF)hypovolemia due to spontaneous or traumatic spinal CSF leakage and is easily being misdiagnosed or missed,especially in these patients without the prototypical manifestation of an orthostatic headache.At present,the management of IH with both cranial nerve VI palsy and bilateral subdural hematomas(SDHs)is still unclear.CASE SUMMARY A 67-year-old male Chinese patient complained of diplopia on the left side for one and a half mo.Computed tomography revealed bilateral SDHs and a midline shift.However,neurotrophic drugs were not effective,and 3 d after admission,he developed a non-orthostatic headache and neck stiffness.Enhanced magnetic resonance imaging revealed dural enhancement as an additional feature,and IH was suspected.Magnetic resonance myelography was then adopted and showed CSF leakage at multiple sites in the spine,confirming the diagnosis of having IH.The patient fully recovered following multiple targeted epidural blood patch(EBP)procedures.CONCLUSION IH is a rare disease,and to the best of our knowledge,IH with diplopia as its initial and primary symptom has never been reported.In this study,we also elucidated that it could be safe and effective to treat IH patients with associated cranial nerve VI palsy and bilateral SDHs using repeated EBP therapy.

Epidural blood patch for spontaneous intracranial hypotension with subdural hematoma: A case report and review of literature

BACKGROUND Cerebrospinal fluid(CSF)leakage at C1/2 in spontaneous intracranial hypotension(SIH)is rare.Subdural hematoma(SDH),a serious complication of SIH,may lead to neurological deficits.This report presents a case of SDH after spontaneous C1/2 CSF leakage,which was treated with a targeted epidural blood patch(EBP).CASE SUMMARY A 60-year-old man with no history of trauma was admitted to our hospital with orthostatic headache,nausea,and vomiting.Brain computed tomography imaging revealed bilateral,subacute to chronic SDH.Brain magnetic resonance imaging(MRI)findings were SDH with dural enhancement in the bilateral cerebral convexity and posterior fossa and mild sagging,suggesting SIH.Although the patient underwent burr hole trephination,the patient’s orthostatic headache was aggravated.MR myelography led to a suspicion of CSF leakage at C1/2.Therefore,we performed a targeted cervical EBP using an epidural catheter under fluoroscopic guidance.At 5 d after EBP,a follow-up MR myelography revealed a decrease in the interval size of the CSF collected.Although his symptoms improved,the patient still complained of headaches;therefore,we repeated the targeted cervical EBP 6 d after the initial EBP.Subsequently,his headache had almost disappeared on the 8th day after the repeated EBP.CONCLUSION Targeted EBP is an effective treatment for SDH in patients with SIH due to CSF leakage at C1/2.

Recurrent Intracranial Hypotension Complicated with Subdural Hematoma—A Case Report and a Case Series Review

Intracranial hypotension has variable clinical manifestations;subdural hematoma is one of the complications of intracranial hypotension with the reported incidence ranging from 16% to 57%. The author would like to share a case of subdural hematoma caused by recurrent intracranial hypotension with different cerebrospinal fluid (CSF) leakage site and to review a case series of intracranial hypotension treated in the author’s hospital (Kaohsiung Veterans General Hospital). A 44-year-old male having the past history of intracranial hypotension was treated in our Neurology division one month previous to this admission, who was sent to our emergency room (ER) due to severe orthostatic headache with nausea and vomiting. Computed tomography (CT) scan of brain at ER showed bilateral subdural hematoma, more on the left side with mass effect. Both surgical removal of the subdural hematoma and epidural blood patch were performed and he had a good outcome. Orthostatic headache is a specific symptom sign of intracranial hypotension. Epidural blood patch is effective to manage intracranial hypotension either the leakage site of CSF is detected or not. In case of non-traumatic subdural hematoma, intracranial hypotension should be kept in mind.

Factors affecting cerebrospinal fluid opening pressure in patients with spontaneous intracranial hypotension

Objective： Spontaneous intracranial hypotension （SIH） is recognized far more commonly than ever before. Though usually characterized by low cerebrospinal fluid （CSF） pressure, some patients with SIH are observed to have normal pressure values. In this study, we aimed to confirm the proportion of patients with normal CSF opening pressure （CSF OP） and explore the factors affecting CSF OP in SIH patients. Methods： We retrospectively reviewed 206 consecutive SIH patients and analyzed their clinical and imaging variables （including demographic data, body mass index （BMI）, duration of symptoms, and brain imaging findings）. Univariate and multiVariate analyses were performed to identify the potential factors affecting CSF OP. Results： In a total of 114 （55.3%） cases the CSF OP was 〈60 mmH2O （1 mmH2O=9.80665 Pa）, in 90 （43.7%） cases it was between 60 and 200 mmH2O, and in 2 （1.0%） cases it was 〉200 mmH2O. Univadate analysis showed that the duration of symptoms （P〈0.001）, BMI （P〈0.001）, and age （P=0.024） were positively correlated with CSF OP. However, multivariate analysis suggested that only the duration of symptoms （P〈0.001） and BMI （P〈0.001） were strongly correlated with CSF OP. A relatively high R2 of 0.681 was obtained for the multivariate model. Conclusions： Our study indicated that in patients without a low CSF OP, a diagnosis of SIH should not be excluded. BMI and the duration of symptoms can influence CSF OP in SIH patients, and other potential factors need further investigation.

Rapid resolution of subdural hematoma after targeted epidural blood patch treatment in patients with spontaneous intracranial hypotension

Background Subdural hematoma （SDH） is a common complication of spontaneous intracranial hypotension （SIH）.To date,the management of SDH caused by SIH remains controversial.In this paper,we reviewed the clinical course of SDH in patients with SIH,and discuss the underlying mechanism and attributing factors for rapid resolution of subdural hematomas after epidural blood patch （EBP） surgery.Methods We retrospectively reviewed a cohort of seventy-eight SIH patients diagnosed and treated with targeted EBP in our neurology center.Patients who received early CT/MRI follow-up after EBP operation were included.Results A series of four cases of SIH complicated with SDHs were evaluated.Early follow-up neuroimages of these patients revealed that SDHs could be partially or totally absorbed just two to four days after targeted epidural blood patch treatment.Conclusion Targeted epidural blood patch can result in rapid hematoma regression and good recovery in some patients with a combination of SDH and SIH.

Spontaneous intracranial hypotension complicated with cerebral venous thrombosis and subdural effusion:a case report

Spontaneous intracranial hypotension treatment can be complicated by concomitant cerebral venous thrombosis and subdural hematoma.A 48 years old male,presenting orthostatic headache and neck pain for 1 month displayed sagittal sinus thrombosis and bilateral subdural effusions,as well as extradural fluid collection at T3-T8 level,upon magnetic resonance imaging.Cerebrospinal fluid opening pressure was 50 mmH2O,and a leak was confirmed at C2-C3 level by computed tomography(CT)myelogram.The presence of subdural hematoma precluded anticoagulation treatments.An autologous epidural blood patch at C2-C3 level under CT guidance improved the patient’s condition,remaining free of residual symptoms or recurrence at six-month follow-up.

Epidural Blood Patches Performed with Miethke Sensor Reservoir for Continuous Intracranial Pressure Monitoring

An epidural blood patch (EBP) is a procedure performed by injecting autologous blood into a patient’s epidural space, usually at the site of a suspected CSF leak. It is typically performed in patients with characteristic postural headaches due to low intracranial pressure. We report a case of a young female with an implanted Miethke Sensor Reservoir, which was used for continuous intracranial pressure (ICP) monitoring during a two-level epidural blood patch. ICP increased only with thoracic injection, suggesting thoracic EBP may have greater efficacy than lumbar EBP in treating SIH and PDPH when the site of CSF leak is unknown.

Antithrombin gene Arg197Stop mutation-associated venous sinus thrombosis in a Chinese family

This study sought to elucidate the genetic correlation of cerebral venous sinus thrombosis caused by a hereditary antithrombin deficiency in a Chinese family, at the genetic and protein levels. A nonsense mutation from C to T on locus 6431 in exon 3B of the antithrombin gene was observed, leading to an arginine （CGA） to stop codon （TGA） change in the protein. This is the first report of this mutation in China. Ineffective heparin therapy in the propositus patient is associated with a lack of heparin binding sites after antithrombin gene mutation. Characteristic low intracranial pressure in the acute phase might be specific to this patient with cerebral venous sinus thrombosis.

Paradoxical herniation after decompressive craniectomy provoked by mannitol:A case report

BACKGROUND Paradoxical transtentorial herniation is a rare but life-threatening complication of cerebrospinal fluid drainage in patients with large decompressive craniectomy.However,paradoxical transtentorial herniation after rapid intravenous infusion of mannitol has not been reported yet.CASE SUMMARY A 48-year-old male suffered from a right temporal vascular malformation with hemorrhage.In a coma,the patient was given emergency vascular malformation resection,hematoma removal,and the right decompressive craniectomy.The patient woke up on the 1st d after the operation and was given 50 g of 20% mannitol intravenously every 8 h without cerebrospinal fluid drainage.On the morning of the 7th postoperative day,after 50 g of 20% mannitol infusion in the Fowler’s position,the neurological function of the patient continued to deteriorate,and the right pupils dilated to 4 mm and the left to 2 mm.Additionally,computed tomography revealed an increasing midline shift and transtentorial herniation.The patient was placed in a supine position and given 0.9% saline intravenously.A few hours later,the patient was fully awake with purposeful movements on his right side and normal communication.CONCLUSION Paradoxical herniation may occur,although rarely,after infusing high-dose mannitol intravenously in the Fowler’s position in the case of a large craniectomy defect.An attempt should be made to place the patient in the supine position because this simple maneuver may be life-saving.Do not use high-dose mannitol when the flap is severely sunken.

Postpartum posterior reversible encephalopathy syndrome secondary to preeclampsia and cerebrospinal fluid leakage:A case report and literature review

BACKGROUND Postpartum posterior reversible encephalopathy syndrome(PRES) is not uncommon.Its mechanisms and risk factors are not clear.CASE SUMMARY A 28-year-old woman underwent cesarean section but had inadvertent dural puncture during epidural anesthesia.To manage the symptoms of intracranial hypotension,crystalloid fluid was infused.However,the patient developed postpartum preeclampsia and PRES.The patient was treated with diazepam and dehydration therapy.The signs of cerebral lesions on magnetic resonance imaging disappeared on postpartum day 7.CONCLUSION Postpartum preeclampsia and PRES can develop concomitantly.Treating postdural puncture headaches with infusion of crystalloid fluid may precipitate the development of PRES.

Dural enhancement detected by magnetic resonance imaging reflecting the underlying causes of cerebral venous sinus thrombosis

Dural enhancement detected by magnetic resonance imaging is a common finding in patients with cerebral venous sinus thrombosis （CVST） and is usually interpreted as a change secondary to CVST. We report two cases of CVST with intense and diffuse dural enhancement that resulted from pachymeningitis in one patient and spontaneous intracranial hypotension in another. Pachymeningitis and spontaneous intracranial hypotension were also determined to be the underlying causes of CVST. The clinical data of these two patients are described. In patients with CVST, dural enhancement is not always a secondary change to CVST. It can be a manifestation of the underlying causes of CVST. When diffuse and intense dural enhancement is revealed, sufficient ancillary tests are warranted to rule out other potential patholooical chanoes of the dura mater those can result in CVST.