Abstract: Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular disease, accounting for 1% of all strokes occurring when a blood clot forms in any of the venous sinuses of the brain. CVST commonly presents as severe headache, seizures, focal neurological deficit, nausea and vomiting. Presentation of CVST in the form of multifocal intracerebral haemorrhage (ICH) is an extremely rare occurrence. We report a case of 29-year-old male presenting with multifocal ICH and seizures with underlying CVST. The finding of multifocal ICH was incidentally found on Brain radio imaging and further workup revealed underlying CVST. This case report underscores the importance of Brain radio imaging in an otherwise normal patient presenting with seizure disorder. CVST as an aetiology for multifocal ICH is a very rare yet significant phenomenon. 

KEYWORDS: Multifocal Intracerebral Haemorrhage (ICH), Cerebral venous sinus thrombosis (CVST), Seizures, Metabolic Syndrome. 

INTRODUCTION

Cerebral venous sinus thrombosis (CVST) is a rare cerebrovascular disease, accounting for 1% of all strokes, but with a mortality rate as high as 10%. There are many causes and risk factors of CVST, such as pregnancy, oral contraceptive, infection, trauma, central nervous system tumour, metabolic syndrome and coagulopathies. Initial presentation of CVST as intracerebral haemorrhage (ICH) is rare and further as multifocal ICH is rarest.¹ Few common clinical features of CVST may be in form of Headache, giddiness, nausea, vomiting, seizure and focal neurological deficit out of which headache is the commonest presentation found in up to 77% in some studies.²

Despite the advances in the recognition of CVT in recent years, the diagnosis and treatment may be difficult because of the diversity of underlying risk factors.³

The reported incidence of spontaneous solitary ICH is CSVT is around 33%, however multifocal (at 2 or more sites within 48 hours) ICH are much more uncommon.⁴

Hence, we are reporting a case of multifocal intracerebral haemorrhage in a patient with underlying Cerebral venous sinus thrombosis (CVST).

CASE REPORT

A 29 years old male taxi driver by occupation admitted to medical ward with history of first episode of abnormal body movements in the form of generalized tonic clonic type with frothing from mouth and loss of consciousness which remained for 20 minutes. There was a history of post ictal confusion and headache which persisted for 2 hours. He had no significant history of Alcohol intake and head injury. Past history and family history were unremarkable.

General physical examination at the time of admission revealed that he was drowsy, not oriented to time place & person with GCS E₃V₂M₅. His blood pressure was 160/104 mm of Hg and rest of the vitals were normal. After recovery from post ictal confusion state, patient was examined thoroughly and found to have a height of 199 cm weighing 100 kg. His BMI was 25.25 kg/m².

His waist circumference was 118 cm and hip circumference was 96 cm, with waist: hip ratio of 1.23. CNS examination revealed no focal neurological deficit.  

So, a clinical diagnosis of seizure disorder with hypertension was suspected and an urgent NCCT head was ordered.  

The NCCT head showed multiple hyperdense focal areas in right frontal and parietal lobes suggestive of multifocal ICH. The patient was subjected to further extensive workup.

His hemogram showed a haemoglobin level of 14.4 g% with Erythrocyte sedimentation rate (ESR) of 25. The TLC and Platelet counts were within normal limits. Other routine investigations like Renal function test (RFT), Liver function test (LFT), Serum electrolytes and urine examination were within normal limits. His fever profile consisting of MP QBC, Dengue and Scrub Typhus came out negative.  His C-reactive protein (CRP) level was elevated to 14.74 mg/L. His coagulation profile consisting of aPTT, PT-INR, BT and CT were normal.  Fasting lipid profile revealed an elevated Triglyceride level of 183 mg/dL and fasting blood sugar level was 114 mg/dL. His serum homocysteine level was 24.66 mimol/L (Normal reference range 5.46-16.2)

A diagnosis of Metabolic syndrome or Syndrome X was also established.

X-ray Chest and electrocardiogram were normal. USG Abdomen showed fatty liver.

Considering the atypical presentation and the nature of bleed, further radio imaging of the Brain in the form of MR Brain, MR Angiogram and MR Venogram were ordered.

MR Brain showed multiple acute intraparenchymal haemorrhages in cortical and subcortical aspect of right frontal and parietal lobes with mild peri-lesional oedema. Largest measuring 2.8*1.5 cm.

MR Venography revealed acute cerebral venous thrombosis in superior sagittal sinus, right transverse sinus and right sigmoid sinus. On post contrast study filling defect was noted.  

MR Angiography appeared normal.

Patient was treated with anti-epileptic medications for seizure control. Parenteral anticoagulant Enoxaparin was given for full 5 days with oral Warfarin overlap. Patient was discharged after satisfactory management and asked to follow up at Medical OPD with INR report.

DISCUSSION

CVST occurs when a blood clot forms in any of the venous sinuses of the brain. Approximately 5 people per million are affected by CVST and it accounts for approximately 1% of all stroke events. There may be a lot of risk factors of CVST, such as Female gender, pregnancy, oral contraceptive, infection, trauma, central nervous system tumour, metabolic syndrome and coagulopathies.⁶ CSVT is extremely rare in young adult males. The probable risk factor in our patient may be metabolic syndrome. Although CVST is observed with increasing frequency in daily practice and has a variety of nonspecific clinical symptoms, its presentation with an associated ICH via CT and magnetic resonance imaging (MRI) is infrequent. In patients with CVST, spontaneous intracranial haemorrhage accounts for 30% to 40% of ICH.⁷ CVST-induced ICH includes simple cerebral haemorrhage and venous infarction haemorrhage. Furthermore, the distribution of venous infarction haemorrhages typically does not conform to the normal distribution of simple cerebral haemorrhage; the hematoma usually occurs closer to the surface of the brain, with a large area of low density around the focal point. Intramedullary or subcortical meniscus haemorrhage is the earliest manifestation of CVST combined with ICH, and the "zebra striated” haemorrhage is also a common characteristic of this combination. Increased venous and capillary pressure results in diapedesis of red blood cells and subsequent rupture of small vessels. Therefore, ICH may be an extension of this sequence of events. In patients with lobar ICH of otherwise unclear origin or with cerebral infarction that crosses typical arterial boundaries, imaging of the cerebral venous system should be performed.

Multiple simultaneous ICH is an uncommon event and have been observed in only 2% of haemorrhagic strokes.⁴ Yen et al.⁸ reported an incidence of 0.8% for simultaneous multiple ICH among all intracranial haemorrhages. However, in simultaneous multiple ICHs, it is most commonly detected in the bilateral thalami and then the putamen.⁹ Due to the small number of reported cases, the underlying pathology is still not known. Reported causative factors include hypertension, multiple micro bleeding, cerebral amyloid angiopathy, vasculitis, administration of intravenous tissue plasminogen activator, asphyxiation, deep cerebral vein thrombosis, and neoplasm; these causative factors are similar to those for single spontaneous ICH.^10,11.

CONCLUSION

Cerebral venous sinus thrombosis (CVST)is an uncommon disorder in young adult males.  A CVST presenting with multifocal haemorrhages on radio imaging and seizure episode is very rare. Like other risk factors, Metabolic syndrome is a significant cause for CVST. This case report underlies the importance of an urgent radio imaging in an otherwise normal patient presenting with seizure disorder. CVST as an aetiology for multifocal ICH is a very rare yet significant phenomenon.

Dr. Mahesh Dave¹, Dr. Aniruddha Burli², Dr. Nagaraj², Dr. Ayush Agarwal²

¹Senior Professor and Unit Head, ²Junior Resident.                                                    

Department of General Medicine, RNT Medical College, Udaipur, Rajasthan (India). 

REFERENCES

1. Saposnik G, Barinagarrementeria F, Brown RD Jr, et al. Diagnosis and management of cerebral venous thrombosis: a statement for healthcare professionals from the American Heart Association/American Stroke Association. Stroke 2011; 42:1158–92
2. Lurlaro S, Beghi E, et. al. Does headache represent a clinical marker in early diagnosis of cerebral venous thrombosis? A prospective multicentric study. NeurolSci 2004;25: S298-S299.
3. Stam J. Thrombosis of the cerebral veins and sinuses. N Engl J Med 2005; 352:1791–8.
4. Van Asch CJ, Luitse MJ et. al. Incidence, case fatality, and functional outcome of intracerebral haemorrhage over time, according to age, sex, and ethnic origin: a systematic review and meta-analysis. Lancet Neurol. 2010 Feb;9(2):167-76.
5. Jin-Suk Seo, Taek-Kyun Nam, Jeong-Taik Kwon, Yong-Sook Park. Journal of Cerebrovascular and Endovascular Neurosurgery ISSN 2234-8565, EISSN 2287-3139.
6. Jing Sun, MD, PhDa , Zhidong He, MDb , Guangxian Nan, MD, PhD. Cerebral venous sinus thrombosis presenting with multifocal intracerebral haemorrhage and subarachnoid haemorrhage. Medicine (2018) 97:50(e13476)
7. Minami K, Iida M, Iida H. Case report: central venous catheterization via internal jugular vein with associated formation of perioperative venous thrombosis during surgery in the prone position. J Anesth 2012;26: 464–6
8. Tanno Yen, Ono J, Suda S, Karasudani H, Yamakami I, Isobe K, et al. [Simultaneous, multiple hypertensive intracerebral hematomas: report of 5 cases and review of literature]. No shinkeiGeka. 1989 Mar;17(3):223-8. Japanese.
9. Chanda A, Nanda A. Multiple cavernomas of brain presenting with simultaneous hemorrhage in two lesions: a case report. Surg Neurol. 2002 May;57(5):340-4; discussion 344-5.
10. Kase CS, Pessin MS, Zivin JA, del Zoppo GJ, Furlan AJ, Buckley JW, et al. Intracranial hemorrhage after coronary thrombolysis with tissue plasminogen activator. Am J Med. 1992 Apr;92(4):384-90.
11. Ooneda G, Yoshida Y, Suzuki K, Sekiguchi T. Morphogenesis of plasmatic arterionecrosis as the cause of hypertensive intracerebral hemorrhage. VirchowsArchiv A PatholPathol Anat. 1973 Oct;361(1):31-8.