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BMJ Case Rep. 2010; 2010: bcr10.2009.2325.
Published online 2010 February 8. doi:  10.1136/bcr.10.2009.2325
PMCID: PMC3030288
Copyright 2010 BMJ Publishing Group Ltd
Findings that shed new light on the possible pathogenesis of a disease or an adverse effect
Dolichoectasia and multifocal simultaneous intracranial haemorrhages
Jose Gutierrez, David Adams, Leticia Tornes, Richard Isaacson, and Clinton B Wright
University of Miami/Leonard M Miller School of Medicine, Department of Neurology, 1387, 1120 NW 14th Street, Miami, FL 33136, USA
Correspondence to Jose Gutierrez, drjosegc/at/hotmail.com
Abstract
Dolichoectasia is found primarily in aged individual with atherosclerotic disease. It presents with brain stem compression and stroke, which could be ischaemic or haemorrhagic. Even if severe atherosclerosis is thought to play a pivotal role, new evidence suggest that the internal elastic laminae is disrupted, intracranially and extracranially, so multiorganic involvement with various clinical presentations can occur. We present a rare association of multiorganic dolichoectasia debuting with multiple intracranial haemorrhages. A 79-year-old woman presented with altered mental status and left hemiplegia. The work up demonstrated brain arteries dolichoectasia and an abdominal aortic aneurysm with multiple site intraparenchymal haemorrhages. In this case, the presentation of multiple site dolichoectasia and multiple haemorrhages is probably linked to a systemic “media disease” that affected the media of small, medium and large arteries rather than a coincidental finding.
Background
Dolichoectasia is a disease that has been neglected in the stroke field; over the last 100 years there has been an average of 100 papers published on the topic, with most of them being case reports and surgical series. This case is a reminder of this disease and supports the general idea that dolichoectasia or dilatatory arteriopathy is a multiorganic disease and its presence could alert us to the possibility of multisystemic symptoms. The multiple haemorrhages, the coexistence of abdominal aortic aneurysm (AAA) and the anterior and posterior intracranial dolichoectasia have not been reported in the past.
A 79-year-old woman presented to the emergency department after 24 h of confusion and lethargy followed by a left hemiparesis. The patient’s previous medical history was remarkable for hypertension, unclassified dementia, abdominal aortic aneurysm, and placement of an on-demand pacemaker 3 years before admission. She was taking aspirin, a β-blocker, an angiotensin converting enzyme (ACE) inhibitor, and an acetylcholinesterase inhibitor for her dementia. On physical examination she was afebrile with a blood pressure of 113/97 mm Hg and a regular pulse of 101 beats/min. She was lethargic, not following commands, but blinked to threat. Left facial weakness was noted. She moved all extremities; however, the left side moved significantly less so than the right. Deep tendon reflexes were 3+ symmetrically, and a Babinski sign was present on the left.
The blood workup showed pre-renal azotaemia and vitamin B12 deficiency, but was otherwise unremarkable. Urinalysis suggested infection, which was confirmed with a urine culture that grew Escherichia coli.
The echocardiogram and the Doppler study of the neck vessels were normal. The brain computed tomography (CT) scan showed multifocal small haemorrhages with a larger right subcortical haematoma, and multifocal dolichoectasia (fig 1). A brain CT angiogram corroborated the dolichoectasia and an abdominal CT angiogram demonstrated the aortic aneurysm (fig 2). Arterial diameters were measured on CT angiogram source images: basilar artery (arrow)=11.1 mm; right intracranial carotid artery (left arrowhead)= 5.3 mm; left intracranial carotid artery (right arrowhead) = 7.3 mm; and abdominal aortic aneurysm (arrow) with a diameter of 77 mm.
Figure 1
Figure 1
Non-contrasted brain computed tomography (CT). (A) Large and dilated basilar artery (arrow) and carotid arteries (arrowhead). (B) Haemorrhagic lesions, one on the right caudate nucleus (left arrow) and the left thalamus (right arrow). (C) Right centrum (more ...)
Figure 2
Figure 2
Brain and abdomen CT angiography. (A) Anterior and posterior circulation dilatatory arteriopathy (dolichoectasia). (B) Basilar artery in the posterior fossa (arrow). (C) Posterior circulation. (D) Middle cerebral arteries and the anterior cerebral arteries. (more ...)
Cerebral amyloid angiopathy (CAA) was a possibility based on age, location of the bleeding and the number of lesions.1 The lack of magnetic resonance imaging (MRI) data prevented us from investigating further for old haemorrhagic lesions to support this diagnosis. Other conditions were entertained. A cerebro-retinal vasculopathy such as hereditary endotheliopathy with retinopathy, nephropathy, and stroke (HERNS) was a possibility, but the age of the patient and the lack of retinal involvement made this unlikely.2 Endocarditis, brain metastases and multiple embolic strokes were also considered; however, the lack of clinical and ancillary findings plus the atypical location and appearance of the haemorrhages led us away from these conditions.
CAA is characterised by amyloid peptide deposition in the media of small and medium size brain arteries and often presents as multiple simultaneous haemorrhages.3 In the absence of other demonstrable causes of haemorrhages in this patient, our case would be considered “probable CAA.”4 However, the presence of multifocal dolichoectasia persuaded us to consider the latter as a likely proximate cause of the haemorrhages.
Outcome and follow-up
The patient slowly regained alertness and started interacting with her family members. The acute renal insufficiency remitted with hydration, she received vitamin B12 supplementation, and the urine infection was treated with antibiotics. A follow-up brain CT did not show any new haemorrhages. She was discharged to a nursing home.
Even though there is no known association of CAA and dolichoectasia,5 they both share a common pathology: they both are “media diseases”. “Intracranial dolichoectasia” (also known as “fusiform aneurysm” or “dilatatory arteriopathy”) is characterised by disruption of the internal elastic lamina (IEL) of large vessels,5 most commonly affecting the vertebrobasilar system6 and, if symptomatic, usually presents with compressive symptoms of the brain stem or stroke.7 Most of the strokes are ischaemic, but up to 18% of patients can develop either parenchymal or subarachnoid haemorrhages.8
Dolichoectasia also has associations with abdominal aortic aneurysm, carotid artery enlargement, and coronary artery disease,7,9 as well as small vessel disease of the brain (lacunar infarcts, leukoaraiosis, and état criblé). This suggests that the media of small arteries might be affected similarly to that of large arteries.5–7,9 These associations support the idea that a systemic media dysfunction exists in dolichoectasia. Some animal studies have shown that artery wall shear stress is a probable factor involved in the abnormal dilatation of the artery. The augmented dynamic forces would activate the endothelium with a cascade of complex mechanical and biochemical signals that lead to a media transformation and probably deformation.10
The degree of dolichoectasia of the anterior and posterior vessels in our patient is suggestive of an advanced disease when compared to other patients6,7 and, as implied by others, age and hypertension are key factors involved in this disease.5–9 It is interesting that on admission our patient did not have significantly elevated blood pressure as might be expected for intracranial bleeding, and no gross evidence of atherosclerosis in large vessels was found.
We believe that dolichoectasia is a marker of arterial disease, that the small arteries are as affected as the medium or large arteries, and that the bleeding in this case could have happened in the context of a weakened small artery rather than just having been a coincidental finding. The acute trigger could have been the urinary infection, minor trauma, and mild diastolic pressure elevation in combination with an antiplatelet agent, or even an elevated homocysteine level secondary to vitamin B12 deficiency, but always in the context of an abnormally fragile artery wall.
To our knowledge, this is the first case report of severe anterior and posterior intracranial dolichoectasia presenting with simultaneous multiple parenchymal haemorrhages in a patient with hypertension, dementia and an abdominal aortic aneurysm. Larger and systematic studies are needed to better characterise this “systemic dilatatory arteriopathy”.
Learning points
  • Intracranial dolichoectasia is a differential diagnosis in patients with intracranial haemorrhage.
  • Intracranial dolichoectasia can coexist with other large artery disease—that is, aortic aneurysm, carotid ectasia, hepatic artery ectasia, etc.
  • Intracranial dolichoectasia can coexist with small artery disease—that is, lacunar infarcts, leukoaraiosis and état cribble.
  • Dolichoectasia of the brain arteries should be looked for in every brain imaging study since mounting evidence suggests that this arterial deformation is pathological rather that an indolent incidental finding.
Footnotes
Competing interests: None.
Patient consent: Patient/guardian consent was obtained for publication.
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