Leukoaraiosis

Last updated
Axial T2 FLAIR sequence MR image of a middle-aged man with leukoaraiosis. Leukoaraiosis.jpg
Axial T2 FLAIR sequence MR image of a middle-aged man with leukoaraiosis.
MRI image: Leukoaraiosis in a 90-year-old patient with cerebral atrophy. Leukoaraiosis 1.jpg
MRI image: Leukoaraiosis in a 90-year-old patient with cerebral atrophy.
Head CT showing periventricular white matter lesions. Periventricular white matter lesions (annotated).jpg
Head CT showing periventricular white matter lesions.

Leukoaraiosis is a particular abnormal change in appearance of white matter near the lateral ventricles. It is often seen in aged individuals, but sometimes in young adults. [1] [2] On MRI, leukoaraiosis changes appear as white matter hyperintensities (WMHs) in T2 FLAIR images. [3] [4] On CT scans, leukoaraiosis appears as hypodense periventricular white-matter lesions. [5]

Contents

The term "leukoaraiosis" was coined in 1986 [6] [7] by Hachinski, Potter, and Merskey as a descriptive term for rarefaction ("araiosis") of the white matter, showing up as decreased density on CT and increased signal intensity on T2/FLAIR sequences (white matter hyperintensities) performed as part of MRI brain scans.

These white matter changes are also commonly referred to as periventricular white matter disease, or white matter hyperintensities (WMH), due to their bright white appearance on T2 MRI scans. Many patients can have leukoaraiosis without any associated clinical abnormality. However, underlying vascular mechanisms are suspected to be the cause of the imaging findings. Hypertension, smoking, diabetes, [3] hyperhomocysteinemia, and heart diseases are all risk factors for leukoaraiosis.

Leukoaraiosis has been reported to be an initial stage of Binswanger's disease but this evolution does not always happen.

Causes

The blue arrows indicate leucoaraiosis. In the left image these may well represent transependymal CSF diapedesis due to normal pressure hydrocephalus, which in turn is suggested by the narrowed superior CSF spaces and acute callosal angle. The unilateral occurrence of these alterations in right image suggests they are probably due to vascular encephalopathy. Normal pressure hydrocephalus versus atrophy.jpg
The blue arrows indicate leucoaraiosis. In the left image these may well represent transependymal CSF diapedesis due to normal pressure hydrocephalus, which in turn is suggested by the narrowed superior CSF spaces and acute callosal angle. The unilateral occurrence of these alterations in right image suggests they are probably due to vascular encephalopathy.

White matter hyperintensities can be caused by a variety of factors, including ischemia, micro-hemorrhages, gliosis, damage to small blood vessel walls, breaches of the barrier between the cerebrospinal fluid and the brain, or loss and deformation of the myelin sheath. [8] [9] Multiple small vessel infarcts in the subcortical white matter can cause the condition, often the result of chronic hypertension leading to lipohyalinosis of the small vessels. Patients may develop cognitive impairment and dementia. [10] [11]

Special cases

See also

Related Research Articles

<span class="mw-page-title-main">White matter</span> Areas of myelinated axons in the brain

White matter refers to areas of the central nervous system (CNS) that are mainly made up of myelinated axons, also called tracts. Long thought to be passive tissue, white matter affects learning and brain functions, modulating the distribution of action potentials, acting as a relay and coordinating communication between different brain regions.

Vascular dementia (VaD) is dementia caused by problems in the blood supply to the brain, resulting from a cerebrovascular disease. Restricted blood supply (ischemia) leads to cell and tissue death in the affected region, known as an infarct. The three types of vascular dementia are subcortical vascular dementia, multi-infarct dementia, and stroke related dementia. Subcortical vascular dementia is brought about by damage to the small blood vessels in the brain. Multi-infarct dementia is brought about by a series of mini-strokes where many regions have been affected. The third type is stroke related where more serious damage may result. Such damage leads to varying levels of cognitive decline. When caused by mini-strokes, the decline in cognition is gradual. When due to a stroke, the cognitive decline can be traced back to the event.

<span class="mw-page-title-main">Binswanger's disease</span> Medical condition

Binswanger's disease, also known as subcortical leukoencephalopathy and subcortical arteriosclerotic encephalopathy, is a form of small-vessel vascular dementia caused by damage to the white brain matter. White matter atrophy can be caused by many circumstances including chronic hypertension as well as old age. This disease is characterized by loss of memory and intellectual function and by changes in mood. These changes encompass what are known as executive functions of the brain. It usually presents between 54 and 66 years of age, and the first symptoms are usually mental deterioration or stroke.

<span class="mw-page-title-main">Alexander disease</span> Rare genetic disorder of the white matter of the brain

Alexander disease is a very rare autosomal dominant leukodystrophy, which are neurological conditions caused by anomalies in the myelin which protects nerve fibers in the brain. The most common type is the infantile form that usually begins during the first two years of life. Symptoms include mental and physical developmental delays, followed by the loss of developmental milestones, an abnormal increase in head size and seizures. The juvenile form of Alexander disease has an onset between the ages of 2 and 13 years. These children may have excessive vomiting, difficulty swallowing and speaking, poor coordination, and loss of motor control. Adult-onset forms of Alexander disease are less common. The symptoms sometimes mimic those of Parkinson’s disease or multiple sclerosis, or may present primarily as a psychiatric disorder.

<span class="mw-page-title-main">CADASIL</span> Medical condition

CADASIL or CADASIL syndrome, involving cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, is the most common form of hereditary stroke disorder, and is thought to be caused by mutations of the Notch 3 gene on chromosome 19. The disease belongs to a family of disorders called the leukodystrophies. The most common clinical manifestations are migraine headaches and transient ischemic attacks or strokes, which usually occur between 40 and 50 years of age, although MRI is able to detect signs of the disease years prior to clinical manifestation of disease.

<span class="mw-page-title-main">Cerebral amyloid angiopathy</span> Disease of blood vessels of the brain

Cerebral amyloid angiopathy (CAA) is a form of angiopathy in which amyloid beta peptide deposits in the walls of small to medium blood vessels of the central nervous system and meninges. The term congophilic is sometimes used because the presence of the abnormal aggregations of amyloid can be demonstrated by microscopic examination of brain tissue after staining with Congo red. The amyloid material is only found in the brain and as such the disease is not related to other forms of amyloidosis.

<span class="mw-page-title-main">Perivascular space</span>

A perivascular space, also known as a Virchow–Robin space, is a fluid-filled space surrounding certain blood vessels in several organs, including the brain, potentially having an immunological function, but more broadly a dispersive role for neural and blood-derived messengers. The brain pia mater is reflected from the surface of the brain onto the surface of blood vessels in the subarachnoid space. In the brain, perivascular cuffs are regions of leukocyte aggregation in the perivascular spaces, usually found in patients with viral encephalitis.

<span class="mw-page-title-main">Central canal</span> Cerebrospinal fluid-filled space around the spinal cord

The central canal is the cerebrospinal fluid-filled space that runs through the spinal cord. The central canal lies below and is connected to the ventricular system of the brain, from which it receives cerebrospinal fluid, and shares the same ependymal lining. The central canal helps to transport nutrients to the spinal cord as well as protect it by cushioning the impact of a force when the spine is affected.

<span class="mw-page-title-main">Lesional demyelinations of the central nervous system</span>

Multiple sclerosis and other demyelinating diseases of the central nervous system (CNS) produce lesions and glial scars or scleroses. They present different shapes and histological findings according to the underlying condition that produces them.

Toxic leukoencephalopathy is a rare condition that is characterized by progressive damage (-pathy) to white matter (-leuko-) in the brain (-encephalo-), particularly myelin, due to causes such as exposure to substance use, environmental toxins, or chemotherapeutic drugs. The prevalence of this disease is infrequent and often goes unreported, especially in cases resulting from substance use. Magnetic resonance imaging (MRI) is a popular method to study and diagnose the disease. However, even with technological advances, the exact mechanism and underlying pathophysiology of toxic leukoencephalopathy remains unknown and is thought to vary between sources of toxicity. The clinical severity of toxic leukoencephalopathy also varies among patients, exposure time, concentration, and purity of the toxic agent. Some reversibility of the condition has been seen in many cases when the toxic agent is removed.

Perfusion is the passage of fluid through the lymphatic system or blood vessels to an organ or a tissue. The practice of perfusion scanning is the process by which this perfusion can be observed, recorded and quantified. The term perfusion scanning encompasses a wide range of medical imaging modalities.

<span class="mw-page-title-main">Susceptibility weighted imaging</span>

Susceptibility weighted imaging (SWI), originally called BOLD venographic imaging, is an MRI sequence that is exquisitely sensitive to venous blood, hemorrhage and iron storage. SWI uses a fully flow compensated, long echo, gradient recalled echo (GRE) pulse sequence to acquire images. This method exploits the susceptibility differences between tissues and uses the phase image to detect these differences. The magnitude and phase data are combined to produce an enhanced contrast magnitude image. The imaging of venous blood with SWI is a blood-oxygen-level dependent (BOLD) technique which is why it was referred to as BOLD venography. Due to its sensitivity to venous blood SWI is commonly used in traumatic brain injuries (TBI) and for high resolution brain venographies but has many other clinical applications. SWI is offered as a clinical package by Philips and Siemens but can be run on any manufacturer’s machine at field strengths of 1.0 T, 1.5 T, 3.0 T and higher.

<span class="mw-page-title-main">Hyperintensity</span> High intensity on MRI brain scans

A hyperintensity or T2 hyperintensity is an area of high intensity on types of magnetic resonance imaging (MRI) scans of the brain of a human or of another mammal that reflect lesions produced largely by demyelination and axonal loss. These small regions of high intensity are observed on T2 weighted MRI images within cerebral white matter or subcortical gray matter. The volume and frequency is strongly associated with increasing age. They are also seen in a number of neurological disorders and psychiatric illnesses. For example, deep white matter hyperintensities are 2.5 to 3 times more likely to occur in bipolar disorder and major depressive disorder than control subjects. WMH volume, calculated as a potential diagnostic measure, has been shown to correlate to certain cognitive factors. Hyperintensities appear as "bright signals" on an MRI image and the term "bright signal" is occasionally used as a synonym for a hyperintensity.

<span class="mw-page-title-main">Cerebral atherosclerosis</span> Medical condition

Cerebral atherosclerosis is a type of atherosclerosis where build-up of plaque in the blood vessels of the brain occurs. Some of the main components of the plaques are connective tissue, extracellular matrix, including collagen, proteoglycans, fibronectin, and elastic fibers; crystalline cholesterol, cholesteryl esters, and phospholipids; cells such as monocyte derived macrophages, T-lymphocytes, and smooth muscle cells. The plaque that builds up can lead to further complications such as stroke, as the plaque disrupts blood flow within the intracranial arterioles. This causes the downstream sections of the brain that would normally be supplied by the blocked artery to suffer from ischemia. Diagnosis of the disease is normally done through imaging technology such as angiograms or magnetic resonance imaging. The risk of cerebral atherosclerosis and its associated diseases appears to increase with increasing age; however there are numerous factors that can be controlled in attempt to lessen risk.

<span class="mw-page-title-main">Pathology of multiple sclerosis</span> Pathologic overview

Multiple sclerosis (MS) can be pathologically defined as the presence of distributed glial scars (scleroses) in the central nervous system that must show dissemination in time (DIT) and in space (DIS) to be considered MS lesions.

<span class="mw-page-title-main">Vladimir Hachinski</span> Canadian clinical neuroscientist

Vladimir Hachinski is a Canadian clinical neuroscientist and researcher based at the Schulich School of Medicine and Dentistry at Western University. He is also a Senior Scientist at London's Robarts Research Institute. His research pertains in the greatest part to stroke and dementia, the interactions between them and their joint prevention. He and John W. Norris helped to establish the world's first successful stroke unit at Sunnybrook Hospital in Toronto, and, by extension, helped cement stroke units as the standard of care for stroke patients everywhere. He discovered that the control of the heart by the brain is asymmetric, the fight/flight (sympathetic) response being controlled by the right hemisphere and the rest and digest (parasympathetic) response being controlled by the left hemisphere and damage to one key component can lead to heart irregularities and sudden death. This discovery has added fundamental knowledge to how the brain controls the heart and blood pressure and lays the foundation for helping prevent sudden death.

<span class="mw-page-title-main">MRI sequence</span>

An MRI sequence in magnetic resonance imaging (MRI) is a particular setting of pulse sequences and pulsed field gradients, resulting in a particular image appearance.

Gradient echo is a magnetic resonance imaging (MRI) sequence that has wide variety of applications, from magnetic resonance angiography to perfusion MRI and diffusion MRI. Rapid imaging acquisition allows it to be applied to 2D and 3D MRI imaging. Gradient echo uses magnetic gradients to generate a signal, instead of using 180 degrees radiofrequency pulse like spin echo; thus leading to faster image acquisition time.

Radiologically isolated syndrome (RIS) is a clinical situation in which a person has white matter lesions suggestive of multiple sclerosis (MS), as shown on an MRI scan that was done for reasons unrelated to MS symptoms. The nerve lesions in these people show dissemination in space with an otherwise normal neurological examination and without historical accounts of typical MS symptoms.

Inversion recovery is an MRI sequence that provides high contrast between tissue and lesion. It can be used to provide high T1 weighted image, high T2 weighted image, and to suppress the signals from fat, blood, or cerebrospinal fluid (CSF).

References

  1. Putaala J., Kurkinen M., Tarvos V., Salonen O., Kaste M., Tatlisumak T. (2009). "Silent brain infarcts and leukoaraiosis in young adults with first-ever ischemic stroke". Neurology. 72 (21): 1823–1829. doi:10.1212/WNL.0b013e3181a711df. PMID   19470964. S2CID   593328.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  2. 1 2 Aik Kah, Tan (2018). "CuRRL Syndrome: A Case Series" (PDF). Acta Scientific Ophthalmology. 1 (3): 9–13.
  3. 1 2 Habes M, Erus G, Toledo JB, Zhang T, Bryan N, Launer LJ, Rosseel Y, Janowitz D, Doshi J, Van der Auwera S, von Sarnowski B, Hegenscheid K, Hosten N, Homuth G, Völzke H, Schminke U, Hoffmann W, Grabe H, Davatzikos C (2016). "White matter hyperintensities and imaging patterns of brain ageing in the general population". Brain . 139 (Pt 4): 1164–79. doi:10.1093/brain/aww008. PMC   5006227 . PMID   26912649.
  4. Yan, Shenqiang; Wan, Jinping; Zhang, Xuting; Tong, Lusha; Zhao, Song; Sun, Jianzhong; Lin, Yuehan; Shen, Chunhong; Lou, Min (2014). "Increased Visibility of Deep Medullary Veins in Leukoaraiosis: A 3-T MRI Study". Frontiers in Aging Neuroscience. 6: 144. doi: 10.3389/fnagi.2014.00144 . PMC   4074703 . PMID   25071553.
  5. Kobari M, Meyer JS, Ichijo M, Oravez WT (1990). "Leukoaraiosis: correlation of MR and CT findings with blood flow, atrophy, and cognition". AJNR Am J Neuroradiol. 11 (2): 273–81. PMC   8334682 . PMID   2107711.
  6. Hachinski, VC; Potter, P; Merskey, H (1986). "Leuko-araiosis: An ancient term for a new problem". The Canadian Journal of Neurological Sciences. 13 (4 Suppl): 533–34. doi: 10.1017/S0317167100037264 . PMID   3791068. S2CID   38151019.
  7. Hachinski, V. C.; Potter, P.; Merskey, H. (1987). "Leuko-Araiosis". Archives of Neurology. 44 (1): 21–23. doi:10.1001/archneur.1987.00520130013009. PMID   3800716.
  8. Raz N, Yang Y, Dahle CL, Land S (2012). "Volume of white matter hyperintensities in healthy adults: contribution of age, vascular risk factors, and inflammation-related genetic variants". Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease . 1822 (3): 361–69. doi:10.1016/j.bbadis.2011.08.007. PMC   3245802 . PMID   21889590.
  9. Lin, Jing; Wang, Dilong; Lan, Linfang; Fan, Yuhua (2017). "Multiple Factors Involved in the Pathogenesis of White Matter Lesions". BioMed Research International. 2017: 9372050. doi: 10.1155/2017/9372050 . ISSN   2314-6141. PMC   5339523 . PMID   28316994.
  10. Fauci, Anthony S.; Braunwald, Eugene; Weiner, Charles; Kasper, Dennis L.; Hauser, Stephen L.; Longo, Dan L.; Jameson, J. Larry; Loscalzo, Joseph (2008). Harrison's Principles of Internal Medicine (17th ed.). New York: McGraw-Hill. ISBN   978-0-07-149619-3.[ page needed ]
  11. Hu, He-Ying; Ou, Ya-Nan; Shen, Xue-Ning; Qu, Yi; Ma, Ya-Hui; Wang, Zuo-Teng; Dong, Qiang; Tan, Lan; Yu, Jin-Tai (January 2021). "White matter hyperintensities and risks of cognitive impairment and dementia: A systematic review and meta-analysis of 36 prospective studies". Neuroscience and Biobehavioral Reviews. 120: 16–27. doi:10.1016/j.neubiorev.2020.11.007. ISSN   1873-7528. PMID   33188821. S2CID   226301188.
  12. O'Sullivan M, Morris RG, Huckstep B, Jones DK, Williams SCR, Markus HS (2004). "Diffusion tensor MRI correlates with executive dysfunction in patients with ischaemic leukoaraiosis". J Neurol Neurosurg Psychiatry. 75 (3): 441–47. doi:10.1136/jnnp.2003.014910. PMC   1738975 . PMID   14966162.{{cite journal}}: CS1 maint: multiple names: authors list (link)
  13. Maldjian JA, Whitlow CT, Saha BN, Kota G, Vandergriff C, Davenport EM, Divers J, Freedman BI, Bowden DW. "Automated White Matter Total Lesion Volume Segmentation in Diabetes". AJNR Am J Neuroradiol. 2013 Jul 18

Further reading