Cerebral venous sinus thrombosis is presenting as unusual bilateral blindness: A rare association case report

Special Issue Article

Dr. Jamelah Ahmed


    The Journal of Neuropsychiatry 2020 Vol.4No.1     Cerebral venous sinus thrombosis is presenting as unusual bilateral blindness: A rare association case report Dr. Jamelah Ahmed Alghalbi1, Dr. Mohamad Almoataz Selim2, Dr. Noor Omar Bamohammed3, Dr. Nedaa Bantan4 1 Deputy Head, Department of Neurology, Consultant Neurologist/Consultant Strokologist, King Abdullah Joint Medical Complex KAMCJ, Jeddah, Saudi Arabia 2 Assistant Consultant, Department of Neurology, Alazhar University Cairo, King Abdullah joint Medical Complex KAMCJ, Jeddah, Saudi Arabia 3.  General Physician, Department of Gastrology, King Abdullah joint Medical Complex KAMCJ, Jeddah, Saudi Arabia 4. General Physician, Department of Neurology, King Abdullah joint Medical Complex KAMCJ, Jeddah, Saudi Arabia     Abstract Cerebral venous sinus thrombosis (CVST) is uncommon yet perilous condition. CVST symptoms are mainly severe headaches but other complications may occur, like weakness on one side of the body, difficulty in speaking or seizures and changes in mental status However, we are reporting a cerebral venous sinus thrombosis case of a 44 years old Caucasian female, that was admitted to conservative therapy for an onset of severe, ongoing headache and blindness that affected both eyes. The patient was improved upon hospitalization and prompt anticoagulation therapy. Despite rare vision impairment was reported, our case report shows an unusual association of bilateral blindness and CVST that was not described before in the medical literature. Keywords: cerebral venous sinus thrombosis, superior sagittal sinus, bilateral blindness, computed tomography Introduction Cerebral venous sinus thrombosis (CVST) is a distinct cerebrovascular disorder. The symptoms and complications occurring are highly variable. Women are more prone to CVST than men. About two thirds of adult patients are women. Mostly women who have had recent headaches after starting oral contraception or women who have had seizures after giving birth in the obstetrical ward may have sinus thrombosis [1]. The clinical presentation of CVST is varied and may include headache, vomiting, seizures, unexplained changes in status, and a depressed level of consciousness [2]. In this article, we report a case of a patient who developed an unusual bilateral blindness accompanied to cerebral venous sinus thrombosis. It is hoped that the presentation of this case will draw attention to this unexpected rare and devastating symptom and that this condition will put into consideration. Hence, educating clinicians and increasing their awareness of the unusual symptom that resulted due toCVST.   Case Presentation A 44 yrs old female patient, with no chronic medical disease was presented to the hospital with chief complaints of 1 Month history of headache (in the right side of the head), otitis media . Patient described the headache as, “the worst headache of her life.” Patient stated the headache was in the occipital and frontal areas, and characterized the pain as a stabbing pain. She described the headache was different from her previous migraine headaches. The headaches are pulsating in nature; sometime last for one day but others wake her up from sleep. The symptoms are associated with tinnitus, neck spasm, photopia, and light-headiness. No nausea or vomiting was associated to thecase. Early morning the day she got presented to the hospital, the patient started to experience bilateral loss of vision and confusion. There was no history of nausea, vomiting, seizure, fever or bleeding from anywhere. There is no family history of blood disorder, The patient has primary infertility but hasn’t received any hormonal therapy in the last 2 years. The patient is also considered moker (The patient has history of substanveabuse .surgical history 4 tubal fertility surgeries (last surgery was 5 years ago). Physical examination revealed normal vital signs and the Neurological cranial nerves were intact, kernig sign and brudzinski sign were negative except for right pronator drift. CT of the head and CT brain showed delta sign picture of cerebral sinus thrombosis.CTV and CTA    showed    thrombosis    of     superior     sagittal  sinus thrombosis (SSST), right transverse and right sigmoid sinuses. MR Venogram Brain and MR Angio Head stated that no acute infarction was found. However, left cerebellar hemisphere tiny foci of sub acute infarction were noted.MR venogram and MR angio stated that there was no acute infarction. However, a tiny foci ofsubacute infarction in left cerebellar hemisphere was found. MRV shows no visualization of venous flow in superior sagittal sinus which may indicate venous sinus thrombosis and an attenuated right transverse sinus flow. Lab results stated that D- dimer was marginally raised. However, papilledema was noted on physical exam. No sign of toxicity was reported. The patient was placed on IV fluids, oxygen, and tramadol for pain, heprin dose that was adjusted according to the patient’s body weight. The coagulation profile was followed up with aPTT (activated prothrombin time) 2-3 target. Then warfarin was added until INR was 2-3. Omeprazole and paracetmaol was also presicribed. Over the course of hospitalization, patient’s Blindness was improved she was able to read and visual acuity 20/20.   Discussion (CVST) is a rare and potentially serious type of cerebro- vascular disease that is more predominant in females. It is estimated that CVST occurs in five individuals for every million people [3]. If not treated in the correct way, mortality rate would be 4.3% of CVST patients in the acute phase [4]. Despite that hereditary thrombophilia, intracranial, local infections, pregnancy, purperium, postoperative state, and the use of oral contraceptives or hormonal therapy are considered to be main risk factors of CVST, less than 20% of the cases are of no known clear risk factors [5]. One of the most common symptoms of CVST is severe headache. In many cases the headache is the only symptom of CVST. Each 9 out of 10 individuals with sinus thrombosis have a headache; that may develop suddenly or tends to worsen over the period of several days [6]. Other symptoms may include seizures that are more common in women who develop sinus thrombosis, postpartum, difficulty to move one or more limbs, weakness on one side of the body, speech difficulties, mental status alteration and depressed level of consciousness. Superior sagittal sinus alone or in combination with lateral sinus is the most common site of CVST. Ophthalmological manifestations of CVST are not common and are mainly the consequence of elevated intracranial pressure (ICP). Papilledema is manifested sometimes asymmetrically in the 2 eyes and rarely in 1 eye only [7]. Computer Tomography scan of brain is the initial   The Journal of Neuropsychiatry 2020 Vol.4No.1     tool for investigation. Delta sign in CT is classical of CVST, it usually presents with non specific changes like brain oedema or elevated intracranial pressure. (MRI) of brain is much more sensitive than CT in this condition and often demonstrates the thrombus, unless the scan is obtained during the early few days or months afterwards. CT venography or MR venography could if available for the diagnosis, staging and follow-up [8]. MR angiography or MR venography are among some modalities for imagingtheanatomy of cerebral venous system with further precision [9]. The main pillar of managing CVST in patients with no contra-indications for anti-coagulation (AC) is either by body weight adjusted subcutaneous low molecular weight heparin (LMWH) or activated prothrombin time (aPTT) adjusted intravenous heparin [10]. The rationale of anticoagulant therapy in CVST is to avoid thrombus extension and to favour spontaneous thrombus resolution. When the level of awareness is adjusted to normal, therapy is switched to oral anticoagulants aiming to keep the INR between 2 to 3. Oral AC is prescribed for 3 months if CVST was secondary to a transient risk factor or 6–12 months in patients with idiopathic CVST [11]. Conventional modalities in order to decrease intracranial pressure are indicated in papilloedema and threatened vision. Acetazolamide might be considered if the papilloedema is persistant. In few patients, vision continues to deteriorate despite taking acetazolamide. In these cases, shunting procedures (lumboperitoneal, ventriculoperitoneal shunts or optic nerve fenestration) should be considered. With immediate anticoagulation therapy and long-term follow-up, survival rate increases even if some degree of neuro-deficit remains in the survivingpatients. In the present case there was an acute blindness in the causation of CVST. Unusual bilateral blindness occurred as a consequence of papilloedema that improved upon hospitalization. It is probably related to superior sagittal sinus thrombosis (SSS) that caused optic disc inflammation and elevated Intracranial pressure. Severe headaches were treated with tramadol to alleviate pain but it was not fully eliminated. The patient had tubal fertility 4 times and had hormonal therapy which might be the reason behind the development of the CVST. The limb weakness in the patient may be contributed to Cerebral venous sinus thrombosis, affecting superior sagittal sinus. The ongoing pain and persistent blindness need further investigations to manage the unexpected complication ofCVST. Conclusion Our case is unique in the way that cerebral venous sinus thrombosis is presented with unusual bilateral blindness that was probably due to Superior sagittal sinus thrombosis with improvement of symptoms on prompt therapy with anticoagulants. It is evident from the discussion that, although symptoms of CVST are known, an unexpected unique new                         symptom might appear that needs to be considered. Further investigations and research are still required in the pathophysiology and management of CVST to ensure the finest possible treatment for patients. References 1.          Stam J. Thrombosis of the cerebral veins and sinuses. The New England journal of medicine. 2005; 352(17):1791-1798. https://doi.org/10.1056/NEJMra042354 2.          Ferro       JM,         Canhão                 P,             Bousser  MG,        Stam      J, Barinagarrementeria F. Cerebral vein and dural sinus thrombosis                          in             elderly    patients. Stroke. 2005; 36(9):1927-32. DOI:10.1161/01.STR.0000177894.05495.54. PMID 16 100024. 3.          Cerebral venous thrombosis: influence of risk factors and imaging findings on prognosis. Appenzeller S, Zeller CB, Annichino-Bizzachi JM, Costallat LT, Deus-     Silva L, Voetsch B, Faria AV, Zanardi VA, Damasceno BP, Cendes F Clin Neurol Neurosurg. 2005; 107(5):371-8. 4.          Prognosis of cerebral vein and dural sinus thrombosis: results of the International Study on Cerebral Vein and Dural Sinus Thrombosis (ISCVT). Ferro JM, Canhão P, Stam J, Bousser MG, Barinagarrementeria F, ISCVT Investigators. Stroke. 2004; 35(3):664-70. 5.          Khatri IA, Al Kawi A, Ilyas A et al. Unusual causes of cerebral venous thrombosis. J Pak Med Assoc. 2006; 56:501-506. 6.          Stam J. Thrombosis of the cerebral veins and sinuses. The New England journal of medicine. 2005; 352(17):1791-1798. https://doi.org/10.1056/NEJMra042354 7.          IC Papilledema. The vexing issues. Trobe JD. J Neuroophthalmol. 2011; 31(2):175-86. 8.          MR. staging of acute duralsinus  thrombosis: correlation with venous pressure measurements and implications for treatment and prognosis. Tsai FY, Wang AM, Matovich VB, Lavin M, Berberian B, Simonson TM, Yuh WT AJNR Am J Neuroradiol. 1995; 16(5):1021-9. 9.          Gadolinium-enhanced MR. of chronic dural sinus thrombosis. Dormont D, Sag K, Biondi A, Wechsler B, Marsault C AJNR Am J Neuroradiol. 1995; 16(6):1347- 52. 10.        EFNS Guideline on the treatment of cerebral venous and sinus thrombosis in adult patients. EFNS Guidelines. European Journal of Neurology. 2010; 17:1229-1235. 11.        EFNS guideline on the treatment of cerebral venous and sinus thrombosis. Einhäupl K, Bousser MG, de Bruijn SF, Ferro JM, Martinelli I, Masuhr F, Stam J Eur J Neurol. 2006; 13(6):553-9. Bottom Note: This abstract has been taken from the conference world neuroscience dated on 27th May 2020         ISSN2471-8548                    The Journal of Neuropsychiatry         Volume4,Issue1           Page-8

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