Atypical presentation of isolated deep medullary vein thrombosis as acute onset neurological deficit in an adult: a case report
Dev Kansara1
, Harsh Shah1
, Charanjeet Singh2, Sushant Chaudhary3, Meher Ursekar1, Nishant Gupta4
Contributions: (I) Conception and design: D Kansara, H Shah; (II) Administrative support: N Gupta, M Ursekar; (III) Provision of study materials or patients: M Ursekar; (IV) Collection and assembly of data: D Kansara, H Shah; (V) Data analysis and interpretation: C Singh, S Chaudhary; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors.
Background: Cerebral venous sinus thrombosis is a rare, but well-recognized condition, often accompanied by thrombosis of the deep medullary veins (DMVs). Isolated DMV thrombosis, though rarer, has been documented as a cause of cerebral intraparenchymal hemorrhage in newborns. Literature on isolated superior striate vein thrombosis, a type of DMV thrombosis, in adults is however limited. Recognizing isolated DMV thrombosis as a cause of focal parenchymal hemorrhage in adults is important as it has implications for further management. We present a case report of an adult female with imaging findings suggestive of isolated superior striate vein thrombosis. Our case is unique as our patient did not report any headache.
Case Description: A 58-year-old female presented with left-sided weakness and slurring of speech. Known co-morbidities included stage 3 chronic kidney disease (CKD), hyperhomocysteinemia and hypertension controlled on daily oral antihypertensive medication. Initial magnetic resonance imaging (MRI) revealed a focal hemorrhage in the right corona radiata, with atypical features for a primary hemorrhage and a diagnosis of isolated DMV thrombosis was made. Conservative management was decided upon and follow up computed tomography (CT) showed expected temporal evolution with partial resolution of the thrombosis. The patient showed complete clinical resolution in 2–3 days post-ictus and remained so even at a 6-month follow-up with no residual or new symptoms.
Conclusions: The report emphasizes the need for heightened clinical and radiological awareness of this rare condition, which can mimic more common pathologies and is often underdiagnosed. The findings also underscore the importance of accurate diagnosis to avoid mismanagement and suggest that conservative management is effective, though standardized treatment protocols remain undefined.
Keywords: Thrombosis; case report; deep medullary vein (DMV); cerebral vein; conservative management
Received: 26 August 2024; Accepted: 06 March 2025; Published online: 11 June 2025.
doi: 10.21037/jxym-24-47
Highlight box
Key findings
• This case report presents a rare instance of isolated superior striate vein thrombosis, a subtype of deep medullary vein thrombosis (DMVT), in a 58-year-old woman. Our case highlights the need for knowledge and reasonable suspicion of this entity as, clinical presentations can vary. Our case presented with an acute onset focal neurological deficit and without headache, which is a unique point. Imaging showed linear hyperdensities within the right corona radiata on non-contrast computed tomography and susceptibility on magnetic resonance imaging, indicative of venous thrombosis. The patient was managed conservatively, with follow-up imaging showing no progression and a reduction in associated edema.
What is known and what is new?
• Isolated DMVT, especially in the superior striate vein, is a rare but known cause of focal cerebral intraparenchymal hemorrhage, primarily seen in neonates and children. Handful of cases have been reported in adults.
• However, in addition to contributing towards the limited literature of isolated DMVT in adults, this case highlights the incidence of atypical presentations and the fact that presentations can be highly variable—emphasizing the importance of reasonable suspicion and recognizing characteristic imaging findings even more.
What is the implication, and what should change now?
• This report highlights the rarity of isolated superior striate vein thrombosis in adults, emphasizing the need for awareness among clinicians and radiologists. It also suggests the potential for conservative management in cases without progressive symptoms or significant recanalization, pending further research to establish treatment protocols.
Introduction
Background
Cerebral venous drainage is ramified into a superficial venous system and a deep system of veins. The superficial veins ultimately then drain into the deeper venous system which in turn drains into the major deep neck veins—the internal jugular veins. The superficial system consists mainly of the superficial cortical veins and the sagittal sinus, whereas the deep system consists of rest of the dural venous sinuses as well as few deep cerebral veins (1). These include the subependymal veins and the deep medullary veins (DMVs). This anatomy is depicted in more detail in Figure 1.
Thrombosis of the cerebral venous sinuses is a well-documented entity, which is more common in children however this may or may not be accompanied by deep cerebral vein thrombosis (2). Isolated deep cerebral venous thrombosis in children is rarer (incidence not known) (2), and is even more rare in adults with only one case report published in literature, that too in recent times (3).
Etiology in neonates and children is multifactorial including maternal conditions including, but not limited to, pre-eclampsia, diabetes or chorioamnionitis as well as perinatal causes such as birth asphyxia, sepsis, cardiac defects, and inherited prothrombotic states (4). Etiopathogenesis, as with thrombosis anywhere in the body, involves any or all of the conditions in Virchow’s triad—hypercoagulability, alteration in blood flow, vessel wall injury/endothelial damage (5). Hence, we could argue that any of the traditional risk factors for venous thrombosis would apply here as well, some of which include lifestyle factors such as smoking, oral contraception and even genetic factors (for example Factor V Leiden). However, since literature in this area is limited, definitive etiological factors for isolated deep cerebral venous thrombosis in adults are not well known.
Occlusion of venous outflow leads to venous congestion, engorgement and pooling of interstitial fluid within tissue leading to vasogenic edema. Also, backpressure transferred to the capillaries causes reduction in perfusion pressure gradient with resultant ischemia and infarction, which in turn can lead to cytotoxic edema in the brain. Increased capillary pressure can also lead to their rupture, leading to petechial hemorrhage and in later stages venule and even venous rupture leading to macrohemorrhage (6).
Clinically, adults may present with transient neurodeficits (“stroke-like episodes”) or headaches ranging in severity from the more typical “thunder-clap headache” to even no headache (2).
Brain magnetic resonance (MR) imaging is the gold-standard modality for diagnosis of deep cerebral venous thrombosis in neonates and children (7-9). In a meta-analysis performed by Pin et al. regarding deep cerebral venous thrombosis in children, thrombosis was visualized as T2-hypointense linear fan shaped structures (10). Diffusion-weighted MR imaging is sensitive for detection of the vasogenic and cytotoxic edema developing from deep cerebral venous thrombosis and subsequent venous infarction (11). Haemorrhage, ranging from petechial haemorrhages to larger hematomas was another important collateral imaging finding found. It is feasible to assume that similar findings would be expected in the adult population.
Rationale and knowledge gap
Literature regarding isolated superior striate vein thrombosis, which is a subtype of DMV thrombosis, in adults is sparse, with only a handful of cases being reported (3). Previous case report describes two women, aged 20 and 39 years, experiencing transient neurological symptoms and headaches. Imaging studies using computed tomography (CT) and magnetic resonance imaging (MRI) confirmed a diagnosis of isolated superior striate vein thrombosis, with long-term imaging follow-up conducted. Both cases showed favorable outcomes with conservative management that did not involve anticoagulation. Thrombophilia screenings were negative, and both individuals were active smokers using oral contraceptives. We present a case report of an adult lady with acute onset neurological deficit and characteristic imaging findings of isolated superior striate vein thrombosis. Our case is unique clinically as the patient in our cause reported only transient neurological symptoms, but no headache.
Objective
This knowledge gap of the variety of ways in which isolated DMV thrombosis may present is what we aim to fill to an extent, by our case report. Hence, this case report’s main objective is to highlight the atypical presentations of isolated DMV thrombosis to alert clinicians to keep this differential diagnosis along with its key characteristic imaging findings in mind so as to prevent misdiagnosis and its consequences in terms of management. We present this case in accordance with the CARE reporting checklist (available at https://jxym.amegroups.com/article/view/10.21037/jxym-24-47/rc).
Case presentation
All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal. A 58-year-old lady presented to the emergency room with acute-onset left sided weakness of grip, having difficulty to wear her clothes after she woke up in the morning. Her relatives also noted slurring of speech. Known co-morbidities included stage 3 chronic kidney disease, hyperhomocysteinemia and hypertension controlled on daily oral antihypertensive medication. Initial blood pressure assessment on arrival showed a reading of 170/100 mmHg. Other vital signs were normal. On examination, the only positive findings were mild dysarthria and a left upper motor neuron facial palsy.
With high suspicion for acute hypertensive intraparenchymal hemorrhage, an initial plain MRI of the brain was performed. On the susceptibility-weighted (Sw) images (Figure 2A), two linear foci of susceptibility, oriented obliquely with respect to the right lateral ventricle wall were observed within an area which had features suggestive of acute focal hemorrhage. These corresponded to hyperintensities with hypointense rims on T1-w and linear hypointensities with hyperintense rims on T2-w images and T2-FLAIR images (Figure 2B,2C). On the diffusion-weighted imaging (DWI) (Figure 2D), linear hypointensities surrounded by rims of increased diffusion were observed typical for hemorrhage. Corresponding changes were also seen on the apparent diffusion coefficient (ADC) maps (Figure 2E). Given the location and morphology of the focal hemorrhage, a suspicion for isolated superior striate vein thrombosis was raised.
To obtain further information, non-contrast CT of the brain (Figure 2F,2G) was later performed which demonstrated two hyperdense streaks (corresponding to the areas of susceptibility mentioned above), within the right corona radiata, located just beyond the margin of the right lateral ventricle, which, on coronal views were seen to be located at the superior-lateral angle of the right lateral ventricle with mild surrounding edema. Given the location and morphology, a suspicion for isolated superior striate vein thrombosis was raised. Findings were hence confirmed to be due to isolated superior striate vein thrombosis.
The patient reported feeling back to normal with 100 percent return in function and improvement in symptoms within 2–3 days of the initial event. A decision was made to manage the patient conservatively in view of the imaging findings which were more characteristic of DMVT than a hypertensive bleed. A follow up CT (Figure 2H) 10 days later showed reduction in the CT attenuation of the previously seen linear hyperdensities along with reduction in the perilesional edema which was now minimal in keeping with the expected temporal evolution of acute venous thrombosis.
She then visited the neurology clinic for follow-up and on examination motor power had returned to normal levels. There was no residual neurological deficit and a full recovery was confirmed. She was then advised regular clinical follow up and asked to return if clinically worsened. No active management was carried out. Telephonic contact was made and she confirmed feeling absolutely fine with no residual motor deficit and no return of new symptoms in the interim.
No adverse events or unanticipated events occurred during the period of this study.
Imaging findings are shown in Figure 2.
iMDT discussion
We presented a case of a 58-year-old lady who came to the emergency room with an acute onset focal stroke-like episode. She had no history of similar episodes in the past and no underlying neurological disorder. Such clinical scenarios are fairly commonly in neurological practice in the given epidemiological subset of the patient and the differential diagnosis is generally broad, especially if there is no known underlying neurological disorder. Hence, it is common clinical practice to characterize these events as a “stroke”. The patient is a known case of CKD (stage 3) and hyperhomocysteinemia which are known risk factors for venous thrombosis (12,13). Important to note is that she did not report any headache and absence of this clinical finding should not rule out the possibility of a deep vein thrombosis which is evident from our case.
To evaluate the nature of the event that caused the acute onset neurological deficit, the neurologist often turns towards imaging to determine the cause (14) which is of utmost importance to decide further management. Initial MRI showed linear streaks of susceptibility in the right corona radiata with surrounding vasogenic edema (secondary to venous hypertension and congestion). Due to clinical suspicion and characteristic imaging findings, a diagnosis of isolated superior striate vein thrombosis was made. No evidence of significant venous infarction, however, was observed. Other deep cerebral veins including the subependymal veins were spared. By far, the most common scenario to occur in a patient with the given clinical findings, viz. acute onset neurological deficit in a known hypertensive patient would be a primary acute hypertensive intraparenchymal hemorrhage in the brain (15), and it would be prudent on the side of both the neurologist and radiologist to consider the same. This bias is not only due to the sheer incidence of hypertensive hemorrhage (15) and how common it is clinically encountered in the hypertensive cohort, but also due to lack of awareness towards other entities like isolated DMV thrombosis which may present in a similar fashion.
Due to this bias, adequate clinical suspicion, knowledge of characteristic imaging findings and awareness of this entity was of utmost importance in clinching the diagnosis. Otherwise, these findings could have been easily mis-diagnosed as primary hemorrhage, although they appear atypical in location and morphology—in the location of the superior striate veins (this can be correlated with relevant anatomy of the deep venous system as shown in Figure 1). A CT was then done which showed hyperdense streaks corresponding to the areas of susceptibility on the MRI. Follow up CT imaging after 10 days showed expected temporal evolution of the thrombosis with decrease in the attenuation of the hyperdense streaks without evidence of significant recanalization and decrease in the surrounding vasogenic edema. There was no evidence of progression of thrombosis. The patient went on to have a full clinical recovery within 2–3 days on initial onset and never developed any complications or re-occurrence of symptoms on a 6-month follow-up period.
Isolated DMV thrombosis has been reported in adults before, although only in a handful of cases, which showcases its rarity. Mazini et al. (3) have reported cases of two women, aged 20 and 39 respectively with typical risk factors for thrombosis including smoking and oral contraception who presented with severe headache and focal neurological deficits. It is important to note that even our patient had risk factors that would lead to hypercoagulable state including CKD and hyperhomocysteinemia. However, it is stressed here that our patient did not develop any headaches. This is the additional unique piece of information that our study brings into picture that has not been reported elsewhere. This is significant because it is important to create, at least within one’s mind a mental image of the varieties in which an entity may present, for adequate clinical and radiological suspicion to develop, which is vital for making an accurate diagnosis. Given the limited literature on this topic, this information may not be abundant and our study aims to add to the same. On imaging, there were typical features of thrombosis of the superior striate vein in both patients including linear hyperdense structures on CT extending from the corona radiate to the subependymal region and corresponding diffusion restriction and susceptibility artefacts on the respective MR sequences. One woman also developed a small punctate hemorrhage in the putamen. On follow up, there was absence of thrombus progression, reduction of edema and reduction of thrombus conspicuity on Sw-MR imaging. These findings and progression is similar to what was encountered in our study suggestive of the typical imaging features and non-progressive nature of the condition. However, there was no evidence of significant recanalization in both cases. Given the uncomplicated clinical course and absence of progression on follow up, in addition to a paucity of literature, no decision for antithrombotic therapy was made and both patients were left to follow up. The outcomes were similar in our patient as well with no significant recanalization and no clinical deterioration. Both patients in their study, however, made the decision to stop smoking and discontinue usage of oral contraceptives, and there was no recurrence of the thrombosis in an 8-year follow up period.
A simple comparison between our case and existing cases has been provided in Table 1.
Table 1
| Parameter | Our case | Mazini et al. | |
|---|---|---|---|
| Case 1 | Case 2 | ||
| Age | 58 years | 20 years | 39 years |
| Sex | Female | Female | Female |
| Underlying comorbidities | Hypertension, stage 3 CKD, hyperhomocysteinemia | Active smoker, on oral contraception | Active smoker, on oral contraception, dyslipidemia |
| Previous medical history | None | None | None |
| Clinical presentations | Acute-onset left sided weakness of grip, no headache | Thunderclap headache associated with acute but transient left-sided hemiparesis and dysarthria resolving over a 30 min period | Severe headache, transient dysarthria, left leg and face weakness, and left arm paresthesia |
| Imaging findings | CT: linear hyperdensities in the corona radiata oriented along the SSVs | CT: linear hyperdensities in the corona radiata oriented along the SSVs | CT: linear hyperdensities in the corona radiata oriented along the SSVs |
| MRI: topographically matching linear hyperintensities showed restricted diffusion and susceptibility artifact on SWI consistent with thrombus | MRI: topographically matching linear hyperintensities showed restricted diffusion and susceptibility artifact on SWI consistent with thrombus | MRI: topographically matching linear hyperintensities showed restricted diffusion and susceptibility artifact on SWI consistent with thrombus | |
| Follow up imaging revealed absence of thrombus progression, resolution of the edema and residual thrombus (absence of recanalization) | Follow up imaging revealed absence of thrombus progression, resolution of the edema and residual thrombus (absence of recanalization) | Follow up imaging revealed absence of thrombus progression, resolution of the edema and residual thrombus (absence of recanalization) | |
| Management and prognosis | Fully recovered in 2–3 days post-ictus | Neurological deficit recovered over a 30 min period but headache over a few weeks | Neurological deficit recovered in 3 days but headache over a few weeks |
CKD, chronic kidney disease; CT, computed tomography; MRI, magnetic resonance imaging; SSVs, superior striate veins; SWI, susceptibility-weighted imaging.
The strengths of our study in terms of adding to limited knowledge of the variety of presentations of isolated DMV thrombosis in adults and its consequences in terms of making an accurate diagnosis have already been adequately highlighted. An obvious limitation of our study is that we have reported only a single case, hence it is difficult to draw a larger picture in terms of the common presentations for this entity. A further limitation is a lack of even more long-term follow-up imaging to confirm if the thrombosis persisted on long-term follow up, viz. 6 months, a year and so on. Quite apparently, more thorough case reports and other literature are needed to further understand this entity more fully.
Conclusions
In conclusion, the clinician as well as radiologist must be vary of this rare but known clinical entity of isolated thrombosis of the superior striate vein (a DMV) in adults in the setting of risk factors for venous thrombosis, clinical signs and symptoms such as a “stroke-like episode” or headache accompanied with classical radiological findings as described herein. However, the clinician and radiologist must also know be aware of the variety of clinical presentations for isolated DMV thrombosis. This is where case reports like ours strengthen knowledge and create awareness of albeit rare, but important clinical presentations of clinico-pathological entities like isolated DMV thrombosis, which if not known, or for which there is not an adequate degree of suspicion, would more likely than not be missed. This can have all the diagnostic, management-related and prognostic repercussions of a misdiagnosis. Although, treatment protocols for isolated DMV thrombosis have not yet been defined due to lack of studies, and conservative management may be performed in most cases.
Acknowledgments
None.
Footnote
Reporting Checklist: The authors have completed the CARE reporting checklist. Available at https://jxym.amegroups.com/article/view/10.21037/jxym-24-47/rc
Peer Review File: Available at https://jxym.amegroups.com/article/view/10.21037/jxym-24-47/prf
Funding: None.
Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://jxym.amegroups.com/article/view/10.21037/jxym-24-47/coif). The authors have no conflicts of interest to declare.
Ethical Statement: The authors are accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved. All procedures performed in this study were in accordance with the ethical standards of the institutional and/or national research committee(s) and with the Helsinki Declaration and its subsequent amendments. Written informed consent was obtained from the patient for the publication of this case report and accompanying images. A copy of the written consent is available for review by the editorial office of this journal.
Open Access Statement: This is an Open Access article distributed in accordance with the Creative Commons Attribution-NonCommercial-NoDerivs 4.0 International License (CC BY-NC-ND 4.0), which permits the non-commercial replication and distribution of the article with the strict proviso that no changes or edits are made and the original work is properly cited (including links to both the formal publication through the relevant DOI and the license). See: https://creativecommons.org/licenses/by-nc-nd/4.0/.
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Cite this article as: Kansara D, Shah H, Singh C, Chaudhary S, Ursekar M, Gupta N. Atypical presentation of isolated deep medullary vein thrombosis as acute onset neurological deficit in an adult: a case report. J Xiangya Med 2025;10:7.
