Subarachnoid hemorrhage in isolated cortical vein thrombosis: are presentation of an unusual condition

Hemorragia subaracnóide na trombose isolada de veia cortical: apresentação rara de uma condição incomum

LETTER

Subarachnoid hemorrhage in isolated cortical vein thrombosis: are presentation of an unusual condition

Hemorragia subaracnóide na trombose isolada de veia cortical: apresentação rara de uma condição incomum

Leonardo Kayat Bittencourt^I; Fernando Palma-Filho^II; Romeu Côrtes Domingues^III; Emerson Leandro Gasparetto^IV

^IClínica de Diagnóstico por Imagem (CDPI), Rio de Janeiro RJ, Brazil: Médico Radiologista, CDPI e Multi-Imagem, Rio de Janeiro RJ, Brazil. Pós-Graduando em Radiologia, Universidade Federal do Rio de Janeiro (UFRJ), Rio de Janeiro RJ, Brazil

^IIClínica de Diagnóstico por Imagem (CDPI), Rio de Janeiro RJ, Brazil: Médico Radiologista, CDPI e Multi-Imagem

^IIIClínica de Diagnóstico por Imagem (CDPI), Rio de Janeiro RJ, Brazil: Médico Radiologista e Diretor Médico, CDPI e Multi-Imagem

^IVClínica de Diagnóstico por Imagem (CDPI), Rio de Janeiro RJ, Brazil: Médico Radiologista, CDPI e Multi-Imagem, Professor Adjunto do Departamento de Radiologia, UFRJ

Cerebral vein thrombosis (CVT) is a difficult diagnosis to establish, due to its widely variable clinical manifestations¹. Among those, subarachnoid hemorrhage (SAH) is regarded as one of the rarest presentations and of relatively recent recognition^2,3. Isolated cortical vein thrombosis (ICoVT), i.e. without concomitant venous sinus thrombosis, is an extremely rare presentation of CVT, being mainly reported as sparse case reports or small series of cases^1,4,5 .

We describe a case of ICoVT presenting with SAH, including clinical features, MR imaging findings and imaging follow-up.

CASE

A 31 year-old female patient presented with a 12-day history of worsening "thunderclap" pulsatile headache since she underwent an ankle surgery with spinal anesthesia. The symptomatology was then attributable to the procedure, until the patient developed syncope and transient right hemiparesis on the seventh day post-surgery. The patient reported use of oral contraceptives and denied family history of coagulopathies or deep vein thrombosis.

She underwent an MR imaging scan and fluid-attenuated inversion-recovery (FLAIR) sequence showed linear hyperintensities filling in the cortical sulci of the parietal lobes bilaterally, suggesting SAH (Fig 1A). Mild hyperintense lesions on FLAIR were also seen on the adjacent gyri (Fig 1B). In addition, tubular serpiginous structures, hyperintense on T1-weighted images and converging to the superior sagittal sinus (SSS) were noted adjacent to the areas suspected of SAH (Fig 1C,D). An MR venography showed no evidence of underlying sinus venous thrombosis (Fig 2A,B). The findings were compatible with a diagnosis of isolated cortical venous thrombosis associated with subarachnoid hemorrhage.

The patient was managed conservatively, with withdrawal of the oral contraceptives, physical rest, and anticoagulation measures. The symptoms improved, and the follow-up MR imaging 10 days later showed resolution of the aforementioned findings (Fig 3).

DISCUSSION

Cerebral vein thrombosis accounts for 1-2% of strokes in young adults⁴. There are more than 100 etiologic factors associated with the condition⁶, among which the use of oral contraceptives, recent surgery, coagulopathies, dehydration and malignancy are the most prevalent. The clinical manifestations are highly variable, most frequently being presented as headache (95%), seizures (47%), focal motor deficits (43%), papilledema (41%), altered consciousness state (39%), intracranial hypertension (20%), or coma (15%)⁷.

Subarachnoid hemorrhage is regarded as an extremely rare manifestation of CVT, being found in the literature only through isolated case reports^3,4. Worthy of mention, though, is one series with 32 patients that reported 50% of them with more than 100 erythrocytes per cubic millimeter of CSF, although no MR imaging scans were available, and only two patients presented with sudden onset headache⁸. The exact cause of its association with CVT is object of speculation, with most of the theories suggesting rupture of small cortical veins secondary to hemorrhagic infarction or to venous hypertension⁹. The main distribution is along the cortical sulci at the convexities, with typical sparing of basal cisterns³.

In patients with CVT, the dural sinuses are affected in as much as 98% of the time⁵, being the involvement of the cortical veins usually secondary to retrograde propagation of the primary thrombus. Isolated cortical vein thrombosis is rather unusual, and also confined to case reports or small series¹. The clinical manifestations and risk factors are not well established, but reviewed cases suggest that they are analogous to dural sinus thrombosis. When symptomatology is mild, the diagnosis may be often missed or underestimated, due to frequent anatomical variations in cortical vein distribution.

The concomitance of ICoVT and SAH is even rarer, and to our knowledge there are only 3 reports describing a total of 5 cases of this association^4,10,11. Another article mentioned a case of ICoVT where the CT revealed "thin hyperdensities between frontal sulci", but this finding was interpreted as "cortical venous stasis and dilatation"⁵.

We reported a case of ICoVT associated with SAH in a post-operative female patient in use of oral contraceptives. The presenting complaint consisted of "thunderclap" headache, and there were transient focal deficits during the evolution of the case. Once the imaging diagnosis was established and proper therapeutic measures were taken, rapid resolution of the symptoms was achieved. Our case is in accordance with the available reports in the literature regarding the risk factors¹³, symptomatology and outcomes^4,11, and provides another example of this rare association.

In conclusion, SAH is a readily recognizable condition, most frequently urging invasive procedures or surgical measures, while ICoVT is related to a more subtle imaging finding, generally requiring only medical treatment. In the setting of SAH, CVT can be suspected whenever the cortical sulci are affected and the basal cisterns are spared. The imaging diagnosis is based on FLAIR, T1-weighted images and MR venography findings. Care should be taken to evaluate the cortical veins, for there may be thrombosis even though the sinuses are pervious. This case is thus an example of an unusual manifestation of a common finding - SAH - helping to establish the diagnosis of a rare condition - ICoVT.

Received 3 March 2009, received in final form 1 July 2009. Accepted 18 July 2009.

Dr. Leonardo Kayat Bittencourt - Rua Cinco de Julho 142 / 1101 - 22051-030 Rio de Janeiro RJ - Brasil. E-mail: [e-mail]

• 1. Boukobza M, Crassard I, Bousser MG, Chabriat H. MR Imaging features of isolated cortical vein thrombosis: diagnosis and follow-up. AJNR 2009;30:344-348.
• 2. Sztajzel R, Coeytaux A, Dehdashti AR, Delavelle J, Sinnreich M. Subarachnoid hemorrhage: a rare presentation of cerebral venous thrombosis. Headache 2001;41:889-892.
• 3. Oppenheim C, Domingo V, Gauvrit JY, et al. Subarachnoid hemorrhage as the initial presentation of dural sinus thrombosis. AJNR 2005;26:614-617.
• 4. Chang R, Friedman DP. Isolated cortical venous thrombosis presenting as subarachnoid hemorrhage: a report of three cases. AJNR 2004;25: 1676-1679.
• 5. Miranda H, Mellado P, Sandoval R, Huete L. [Isolated cortical thrombosis: report of two patients] - original in Spanish. Rev Med Chile 2007; 135:1313-1317.
• 6. Bousser MG. Cerebral venous thrombosis: nothing, heparin or local thrombolysis. Stroke 1999;30:481-483.
• 7. Kimber J. Cerebral venous sinus thrombosis. QJM 2002;95:137-142.
• 8. de Bruin S, de Haan R, Stam J. Clinical features and prognostic factors of cerebral venous sinus thrombosis in a prospective series of 59 patients. J Neurol Neurosurg Psychiatry 2001;70:105-108.
• 9. Sakaki T, Matsuyama T, Nagata K, Nakase H, Hirabayashi H, Morimoto T. Delayed intracerebral haemorrhage after intracranial surgery. J Clin Neurosci 1999;6:54-57.
• 10. Spitzer C, Mull M, Rohde V, Kosinski CM. Non-traumatic cortical subarachnoid haemorrhage: diagnostic work-up and aetiological background. Neuroradiology 2005;47:525-531.
• 11. Wang YF, Fuh JL, Lirng JF, Chang FC, Wang SJ. Spontaneous intracranial hypotension with isolated cortical vein thrombosis and subarachnoid haemorrhage. Cephalalgia 2007;27:1413-1417.
• 12. Leach JL, Strub WM, Gaskill-Shipley MF. Cerebral venous thrombus signal intensity and susceptibility effects on gradient recalled-echo MR imaging. AJNR 2007; 28:940-945.
• 13. Kimber J. Cerebral venous sinus thrombosis. QJM 2002;95:137-142.

Publication Dates

• Publication in this collection
  16 Dec 2009
• Date of issue
  Dec 2009