White Matter and Neurodegenerative Diseases (2022)

Demyelinating disease can be divided into four main categories on the basis of etiology: (1) primary, (2) ischemic, (3) infectious, and (4) toxic and metabolic (Table 7.1).


Primary Demyelination


Multiple sclerosis (MS) is the classic example of a primary demyelinating disease. MS is a disease characterized by immune dysfunction with the production of abnormal immunoglobulins and T cells, which are activated against myelin and mediate the damage associated with the disease. It is a chronic, relapsing, often disabling disease affecting more than a quarter of a million people in the United States alone. The age of onset is between 20 and 40 years, with only 10% of cases presenting in individuals older than 50 years. There is a female predominance of almost two to one. Although several environmental factors have been associated with MS, such as higher geographic latitudes and upper socioeconomic status, the etiology of MS remains unclear.


Establishing a diagnosis of MS is challenging, because no specific examination, laboratory test, or physical finding, taken in isolation, is unequivocally diagnostic or pathognomonic of this disorder. At the same time, diagnosing a patient with MS is portentous, as there are significant implications on many aspects of their life, including eligibility for health insurance. However, establishing the diagnosis is important because promising therapies are available, including β-interferon and antineoplastic drugs. These agents suppress the activity of the T cells, B cells, and macrophages that are thought to lead the attack on the myelin sheath.


The classic clinical definition of MS is multiple CNS lesions separated in both time and space. Patients may present with virtually any neurologic deficit, but they most commonly present with limb weakness, paresthesia, vertigo, and visual or urinary disturbances. Important characteristics of MS symptoms are their multiplicity and tendency to vary over time. The clinical course of MS is characterized by unpredictable relapses and remissions of symptoms. The diagnosis can be supported with clinical studies, which include visual, somatosensory, or motor-evoked potentials and analysis of CSF for oligoclonal banding, immunoglobulin G index, and presence of myelin basic protein. Histopathologically, active MS lesions represent areas of selective destruction of myelin sheaths and perivenular inflammation, with relative sparing of the underlying axons. These lesions may occur throughout the white matter of the CNS, including the spinal cord. The inflammatory demyelination interrupts nerve conduction and nerve function, producing the symptoms of MS. Note that histopathologically, the inflammation is a key differentiating feature between MS and other white matter conditions, such as osmotic myelinolysis (central pontine and extrapontine myelinolysis) and posterior reversible encephalopathy syndrome (PRES), which lack inflammatory changes. MR is the most sensitive indicator in the detection of MS plaques, but imaging findings alone should never be considered diagnostic.
In clinically confirmed cases of MS, MR typically demonstrates lesions in more than 90% of cases. This compares with far less than 50% for CT and 70% to 85% for laboratory tests such as brain stem–evoked potentials and CSF oligoclonal bands. Nevertheless, the ultimate diagnosis rests with the careful combination of clinical symptoms, history, and clinical testing, including MR imaging.









Table 7.1 Classification of White Matter Diseases













Primary demyelination
Multiple sclerosis
Ischemic demyelination
Deep white matter infarcts
Lacunar infarcts
Vasculitis (including sarcoidosis and lupus)
Dissection
Thromboembolic infarcts
Migrainous ischemia
Moyamoya disease
Postanoxia
Infection-related demyelination
Progressive multifocal leukoencephalopathy
HIV encephalopathy
Acute disseminated encephalomyelitis
Subacute sclerosing panencephalitis
Lyme disease
Neurosyphilis
Toxic and metabolic demyelination
Central pontine myelinolysis
Marchiafava–Bignami disease
Wernicke–Korsakoff syndrome
Radiation injury
Necrotizing leukoencephalopathy
Dysmyelination (inherited white matter disease)
Metachromatic leukodystrophy
Adrenal leukodystrophy
Leigh disease
Alexander disease

A variety of T2WI techniques have been described for optimizing the detection of white matter lesions, with fluid-attenuated inversion recovery (FLAIR) sequences leading the way. As the name suggests, FLAIR imaging has the advantage of providing heavy T2 weighting while suppressing signal from CSF. As such, FLAIR images provide improved lesion conspicuity of periventricular lesions, which may otherwise be obscured by the bright signal of CSF on FSE T2WIs. Comparative studies have demonstrated that FLAIR imaging provides the best visualization of supratentorial white matter lesions. However, the FLAIR sequence may have mild limitations when imaging the posterior fossa and spine, partly because of pulsation artifacts. In these anatomic regions, both proton density and short tau inversion recovery (STIR) imaging are valuable.


MS plaques are typically round or ovoid, with a periventricular or juxtacortical location (Fig. 7.1). Lesions are bright on T2WIs, reflecting active inflammation or chronic scarring, and only a fraction of MS plaques will demonstrate contrast enhancement. Enhancing lesions are indicative of acute lesions with active demyelination and disruption of the blood–brain barrier. In older lesions, without residual inflammatory reaction, abnormal high signal on T2WIs persists, reflecting residual scarring. Within the CNS, cells can mount only a limited response to neuronal injury. This scarring typically manifests as a focal proliferation of astroglia at the site of injury, termed “gliosis.” In severe cases of MS, actual loss of neuronal tissue may occur and the white matter lesions may actually have dark signal on T1WIs, often referred to as the “dark lesions” of MS. These lesions are prognostically significant because they reflect actual loss of underlying neuronal tissue rather than simple demyelination and are in keeping with a more advanced stage of this disease. Additionally, in chronic cases of MS, there is diffuse loss of deep cerebral white matter, with associated thinning of the corpus callosum and ex vacuo ventriculomegaly.

(Video) White and Gray Matter Damage Increases the Risk of Neurodegenerative Diseases


Although many white matter lesions are nonspecific in nature, the pattern suggestive of MS includes lesions that are periependymal, abutting the ependymal surface, as well as lesions involving the posterior fossa structures, other than for the central pons. The pons is excluded because most lesions in this location are either ischemic in nature or the result of osmotic demyelination, discussed later in this chapter. The periventricular lesions suggestive of MS are often ovoid and aligned perpendicular to the long axis of the ventricles. This pattern is the result of the alignment of the lesions along the perivenular spaces. Additional characteristic features include lesions along the callosal septal interface, as well as lesions that are confluent in nature and greater than 6 mm in diameter with a periventricular location.


In addition to the periventricular white matter, the cerebellar and cerebral peduncles as well as the corpus callosum, medulla, and spinal cord can be involved in MS. Ischemic changes are rare in these locations; as a result, if periventricular lesions are accompanied by lesions in any of these areas, this dramatically increases the specificity for the diagnosis of MS. The pons is excluded from this list of posterior fossa structures due to its proclivity for small vessel ischemic injury. In contrast, because ischemic changes rarely involve the medulla and cerebellar/cerebral peduncles, the presence of lesions in these areas is a useful differential diagnostic factor in suggesting MS. This is particularly important in patients older than 50 years, because it is difficult to decide whether multifocal white matter lesions are the result of ischemia or a demyelinating process. Additional concepts for making this distinction are discussed in the next subsection.


Although the periependymal lesions and posterior fossa location of white matter lesions describe above are certainly suggestive of MS, these findings are not diagnostic of MS as numerous other conditions outlined below, such as lupus, antiphospholipid syndrome, and other angiopathic conditions may be the cause. It is a disservice to both the patient and the referring clinician for the radiologist to constantly parrot a differential list, which includes MS in every patient who may have a few punctate white matter foci. With the quality of modern-day MR imaging and the exquisite sequences such as thin section 3D volume FLAIR, a significant number of all MR scans will reveal white matter lesions, even in the young, 0 to 40 years of age. In this age group, studies have revealed white matter lesions in over 50%. It is important to note that these ubiquitous lesions are often punctate measuring on the order of 1 to 2 mm, and quite different from the periependymal lesions of MS. In contrast to MS lesions, these hyperintense foci are typically located within the subcortical and deep white matter, often clustered in the frontal lobes and associated with perivascular spaces (Fig. 7.2). In fact these punctate foci may simply represent mild gliosis associated with the perivascular space. It should also be pointed out that these punctate foci of hyperintensity are not associated with traumatic etiology. Several poorly controlled studies in the early days of MR created a lore in this regard, which has since been disproven. In this regard, Chapter 3 highlights the characteristic imaging features of
diffuse axonal injury including microbleeds as hallmarks of traumatic pathology.






White Matter and Neurodegenerative Diseases (1)

Figure 7.1. Multiple Sclerosis. Coronal and sagittal fluid-attenuated inversion recovery images (A,B), coronal postcontrast fat saturation T1WI (C), and axial diffusion-weighted image (D). A 26-year-old woman with multiple sclerosis (MS) and a recent flare-up in clinical symptoms demonstrates numerous patchy white matter lesions scattered throughout the subcortical and deep cerebral white matter. Note how many of these lesions have a characteristic flame shaped configuration with a periependymal or juxtacortical location (arrows). Although the periventricular lesions are very suggestive of MS, these lesions are not in and of themselves diagnostic of MS and must be correlated with clinical examination and other clinical studies (visual, somatosensory, or motor-evoked potentials, and analysis of CSF for oligoclonal banding and immunoglobulin G index) before confirming a diagnosis of MS. These lesions may be indistinguishable from other demyelinating conditions, such as acute disseminated encephalomyelitis, and autoimmune/connective tissue disorders such as systemic lupus erythematosus. Note the associated contrast enhancement and restrictive diffusion evident on postcontrast image (C) and the diffusion-weighted image (D), are in keeping with active foci of demyelination.


MS lesions may also present as a large, conglomerate, deep white matter mass that can be mistaken for a neoplasm (Fig. 7.3). These lesions are referred to as tumefactive MS or tumefactive demyelinating lesions (TDL) and differentiation from malignancy may be challenging, with lesions not uncommonly making it to biopsy before the correct diagnosis is established. A useful imaging finding that often differentiates these conglomerate MS plaques from neoplasms is that they often demonstrate a peripheral crescentic rim of contrast enhancement, which represents the advancing leading edge of active demyelination. Detecting this pattern of enhancement and searching carefully for other more characteristic periventricular or posterior fossa lesions are essential clues in distinguishing TDL from neoplasm.


The spinal cord may also be involved with MS, and whenever a focal abnormality of the spinal cord is detected, a demyelinating MS plaque must be in the differential diagnosis. Demyelinating plaques may have mild mass effect as well as contrast enhancement, thus mimicking a neoplasm. The majority of spinal cord MS lesions (70% to 80%) will have associated plaques in the brain. In the setting of a cord lesion, performing an MR scan of the head may confirm the diagnosis, thus avoiding a spinal cord biopsy (see Chapter 10).


Ischemic Demyelination

(Video) week 6white matter neurodegenerative diseases


Age-Related Demyelination. Small-vessel ischemic changes within the deep cerebral white matter are seen with such frequency in middle age (>50 years) that they are considered a normal part of aging. This represents an arteriosclerotic vasculopathy of the penetrating cerebral arteries. The deep white matter is more susceptible to ischemic injury than gray
matter, because it is supplied by long, small-caliber penetrating end arteries, without significant collateral supply. In contrast, cortical gray matter, as well as parts of the brain stem such as the midbrain and medulla, have robust collateral blood supply, thus minimizing the risk of ischemia. The deep penetrating vessels supplying the white matter become narrowed by arteriosclerosis and lipohyalin deposits. The result is the formation of small ischemic lesions, primarily involving the deep cerebral and periventricular white matter as well as the basal ganglia (Fig. 7.4). The cortex, subcortical “U” fibers, central corpus callosum, medulla, midbrain, and cerebellar peduncles are usually spared because of their dual blood supply, which decreases their vulnerability to hypoperfusion. As previously described, if lesions are identified in these locations, a cause other than ischemia should be entertained.






White Matter and Neurodegenerative Diseases (2)

Figure 7.2. Collage: Punctate White Matter Foci without Underlying Disease. Series of patients (ages 3, 12, 17, and 21) presenting with various benign symptoms including vertigo, headache without a history of migraines, trauma, or vascular risk factors. These tiny punctate foci have been reported in over 50% of young patients and potentially reflect small foci of nonspecific gliosis associated with perivascular spaces.


Histologically, areas of infarction demonstrate axonal atrophy with diminished myelin. Early neuropathologists noted the areas of paleness associated with these changes and coined the term “myelin pallor.” These white matter changes have received many names over the years, including leukoaraiosis, microangiopathic leukoencephalopathy, and subcortical arteriosclerotic encephalopathy. None of these terms are very satisfying, as they do not accurately reflect all the changes observed histologically and overstate the clinical significance of these lesions. A more appropriate term may simply be “age-related white matter changes.” These small ischemic white matter lesions are often asymptomatic, and clinical correlation is always required before a diagnosis of subcortical arteriosclerotic encephalopathy or multi-infarct dementia (Binswanger disease) is made. The white matter infarcts just described differ from lacunar infarcts. Lacunae refer to small infarcts (5 to 10mm) occurring within the basal ganglia, typically the upper two-thirds of the putamina. Both lacunar and deep white matter infarcts have similar etiologies and are the result of disease involving the deep penetrating arteries.


Differentiating white matter lesions related to ischemic changes from MS lesions can be difficult, especially in the older patient. This is important because 10% of patients who present with MS are older than 50 years of age. Clinical testing and history are helpful. Additionally, deep white matter infarcts tend to spare the subcortical arcuate fibers and the corpus callosum, both of which can be involved with MS. Involvement of the callosal–septal interface is quite specific for MS.


Nonspecific punctuate white matter lesions (small bright lesions on T2WIs) are more prominent in any patient with a vasculopathy, whether related to atherosclerosis (age, hypertension, diabetes, hyperlipidemia, coronary artery disease); hypercoagulable conditions; vasculitis (lupus, sarcoid, polyarteritis nodosa, Behçet syndrome); or drug-related vasculopathy. In younger individuals with punctuate white matter lesions, if a definable pathology exists, hypercoagulable states, as well as embolic and vasculitic etiologies, figure prominently
(Figs. 7.5 to 7.8). Hypercoagulable conditions include a diverse set of diseases with the common theme of increased risk of microvascular thrombotic disease. Serum testing can be used to evaluate for the presence of these disease conditions, which include homocystinemia, antiphospholipid syndrome, Factor V Leiden, prothrombin gene mutation, and deficiencies of natural proteins that prevent clotting (the anticoagulant proteins such as antithrombin, protein C, and protein S deficiencies). A classic case presentation is that of a young adult female with prior miscarriages presenting with headaches/migraines and ischemic white matter changes. These findings are suggestive of antiphospholipid syndrome (aka phospholipid antibody syndrome), where circulating antiphospholipid antibodies (cardiolipin or lupus anticoagulant antibodies) lead to a hypercoagulable state with resultant white matter and ischemic changes.






White Matter and Neurodegenerative Diseases (3)

Figure 7.3. Tumefactive Demyelinating Lesion (TDL). Axial T2WI (A), DWI (B), coronal postcontrast T1WI (C), and fluid-attenuated inversion recovery image (D). Images from a 30-year-old woman presenting with transient bouts of right hemiparesis, as well as depression and fatigue. Images reveal a large left parietal mass with a peripheral rim of restricted diffusion and enhancement (arrowheads). This lesion could be mistaken for a neoplasm or atypical progressive multifocal leukoencephalopathy and undergo biopsy. The diagnosis of tumefactive MS was confirmed with paraclinical testing, including evoked potentials and CSF oligoclonal bands.


In the young adult population presenting with small white matter lesions, in addition to hypercoagulable conditions and migrainous ischemia, consider cardiogenic embolic etiologies. An echocardiogram plays an important role in the evaluation


of a potential patent foramen ovale or valvular vegetation. In many normal children and young adults, subcortical lesions and periventricular hyperintensities are common; they are reported to be present in these locations in 5% and 75%, respectively, of the young normal population. Commonly these punctuate foci of white matter T2 hyperintensity will have no known etiology despite evaluation for all the conditions outlined earlier. In this setting, these lesions may simply reflect a small focus of gliosis associated with normal perivascular space or simply the gliotic residue of a remote unspecified insult, such as an immune-mediated postviral condition. A radiologist can do considerable disservice to both patient and doctor by suggesting these punctate foci are potentially MS or posttraumatic in nature.






White Matter and Neurodegenerative Diseases (4)

Figure 7.4. Ischemic Demyelination. This 72-year-old woman presented with forgetfulness. Axial fast spin–echo T2WI reveals diffuse patchy lesions throughout the subcortical and deep white matter. These lesions are in keeping with ischemic demyelination of the deep white matter, with several old lacunar infarcts (arrow) of the basal ganglia. Note the ex vacuo ventriculomegaly resulting from loss of deep cerebral white matter.

(Video) Silent Brain Infarcts and White Matter Disease





White Matter and Neurodegenerative Diseases (5)

Figure 7.5. Antiphospholipid Antibody Syndrome. This 32-year-old woman presented with headaches and a history of several miscarriages. A, B. T2-weighted images demonstrate scattered focal subcortical and deep white matter lesions. Although these lesions are nonspecific, serum testing revealed elevated circulating pathogenic immunoglobulins/antibodies specifically targeting DNA and other nuclear constituents collectively termed antibodies to nuclear antigens, for example, lupus anticoagulants and anticardiolipin antibodies. This represents an immune complex disease referred to as antiphospholipid antibody syndrome.






White Matter and Neurodegenerative Diseases (6)

Figure 7.6. Lupus Cerebritis. A 38-year-old woman presented with cognitive deficits and history of a connective tissue disorder. The T1WI demonstrates numerous dark periventricular lesions with striking loss of deep white matter and associated ex vacuo ventriculomegaly. These dark lesions represent underlying axonal loss with neuronal dropout, reflecting a more severe stage of white matter disease. These findings are characteristic of any severe or long-standing white matter disease such as chronic MS, or as in this case, chronic lupus cerebritis.






White Matter and Neurodegenerative Diseases (7)

Figure 7.7. Moyamoya Disease. A 6-year-old boy presents with episodes of focal motor weakness. T2WI (not shown) showed multiple scattered subcortical white matter hyperintensities. MR angiography (A) and conventional angiography (B) reveal marked stenosis of the supraclinoid internal carotid vasculature (arrow), with a dramatic proliferation of tiny collateral vessels (arrowheads) presenting as a “puff of smoke” (the literal Japanese translation of moyamoya). The cause of this vascular disorder is unknown but can be treated with various external to internal vascular bypass surgeries such as encephaloduroarteriosynangiosis. MR angiography plays a useful role in assessing the patency of these shunts once surgically completed.






White Matter and Neurodegenerative Diseases (8)

Figure 7.8. Drug-Induced Vasculopathy. Axial fluid-attenuated inversion recovery image (A), diffusion-weighted image (B), and angiography (C) in a 43-year-old female who presented with headache, confusion, and weakness. Significant signal abnormalities are noted involving the cortex and subcortical white matter of the high frontoparietal convexities (arrows in A) with associated restricted diffusion (arrowheads in B). Catheter angiography reveals considerable vascular beading (arrows in C). Drug-induced vasculopathy is most commonly seen with methamphetamine and sympathomimetic drugs. Both angiography and brain biopsy each have about 30% false positive rates.






White Matter and Neurodegenerative Diseases (9)

Figure 7.9. Ependymitis Granularis (Normal Finding). A,B. Axial fluid-attenuated inversion recovery images in a 42-year-old man presenting with headaches. The periventricular hyperintensity noted about the tips of the frontal and occipital ventricular horns is a normal finding (white arrows). These areas of periependymal hyperintensity may be exacerbated by any process that results in underlying white matter disease. Note the circular artifact located within the left basal ganglia; it is related to magnetic susceptibility artifact from the patient’s orthodontic braces (red arrowheads). One should be aware of artifacts that may mimic pathologic lesions, especially flow and magnetic susceptibility artifacts that can give rise to lesions that are not necessarily contiguous to the cause of the artifact. Incidental note is made of a small focus of subcortical hyperintensity along the left temporoparietal lobe related to a site of posttraumatic gliosis (red arrow in B).


Ependymitis granularis is a normal anatomic finding that may mimic pathology. Ependymitis granularis consists of an area of high signal on a T2WI along the tips of the frontal horns (Fig. 7.9). These foci of signal range in width from several millimeters to a centimeter. Histologic studies of this subependymal area reveal a loose network of axons with low myelin count. This porous ependyma allows transependymal flow of CSF, resulting in a focal area of T2 prolongation. Unfortunately, this entity has been given a name that sounds more like a disease entity than a simple histologic observation. Similarly, with the use of FLAIR imaging, a region of periventricular T2 hyperintensity can be noted about the ventricular trigones as a normal finding. With age, prominent periventricular T2 hyperintensity may be noted along the entire length of the lateral ventricles as a normal finding, and this may be referred to as senescent periventricular hyperintensity or periventricular halo.


Prominent perivascular spaces can also mimic deep white matter or lacunar infarcts. As blood vessels penetrate into the brain parenchyma, they are enveloped by CSF and a thin sheath of pia. These CSF-filled perivascular clefts are called Virchow–Robin spaces and present as punctate foci of high signal on T2WIs (Fig. 7.10). They are typically located in the centrum semiovale (high cerebral hemispheric white matter) and the lower basal ganglia at the level of the anterior commissure, where the lenticulostriate arteries enter the brain parenchyma. These perivascular spaces are typically 1 to 2 mm in diameter but can be considerably larger. They can be seen as a normal variant at any age but become more prominent with increasing age as atrophy occurs.


An important means for differentiating a periventricular space from a parenchymal lesion is the use of the proton density-weighted (first-echo T2W) or FLAIR images. On the proton density-weighted sequence, CSF has similar signal intensity as white matter. A perivascular space is composed of CSF and will parallel CSF signal intensity on all sequences (i.e., isointense to brain parenchyma on proton density sequences). In contrast, ischemic lesions, unless cavitated with cystic change, will be bright on the proton density sequence as a result of the presence of associated gliosis. Both a deep infarct and a perivascular space will be bright on the second-echo T2WI, but only the infarct will remain bright on the first-echo image. Similarly, on a FLAIR image, because fluid signal is attenuated, only true parenchymal lesions with gliosis will yield abnormal signal. On occasion, however, a small amount of persistent T2 hyperintensity can be associated with perivascular spaces on the proton density or FLAIR sequences, and this may account for many of the incidental punctate foci of hyperintensities noted in the young. An additional differentiating feature between giant perivascular spaces and lacunae is location. Lacunar infarcts tend to occur in the upper two-thirds of the corpus striatum because they reflect end-arteriole infarcts in the distal vascular distribution. In contrast, periventricular spaces are typically smaller, bilateral, and often

symmetric within the inferior third of the striatum, where the vessels enter the anterior perforated substance.

(Video) Neurodegenerative Disorders Part I - Dementia, Alzheimer's, MND, MS

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Aug 29, 2016 | Posted by drzezo in GENERAL RADIOLOGY | Comments Off on White Matter and Neurodegenerative Diseases

FAQs

What diseases cause white matter disease? ›

White matter disease may develop with conditions associated with aging, such as stroke, but it can also affect young people due to conditions such as cerebral adrenoleukodystrophy and multiple sclerosis (MS). Read on to learn more about white matter disease and its symptoms, causes, and prognosis.

Can MRI detect neurodegenerative disease? ›

Routine use of high field MRI has greatly contributed to the clinical diagnosis of neurodegenerative disorders, because MRI enables to visualize degenerative process showing either atrophy of the specific areas or degeneration of specific structures.

Is white matter related to dementia? ›

Originally, white matter disease was considered a normal, age-related change. But over the last decade, medical experts have come to understand that the presence of large areas of disease in the white matter of the brain are associated with cognitive decline and dementia in patients.

Does white matter on brain mean Alzheimer's? ›

Background: White matter lesions (WML) are a risk factor for Alzheimer's disease.

What diseases cause white matter on the brain? ›

White matter disease is an umbrella term for damage to your brain's white matter caused by reduced blood flow to the tissue.
...
Risk factors for cardiovascular disease include:
  • High blood pressure (hypertension).
  • High cholesterol (hyperlipidemia, dyslipidemia).
  • Smoking.
  • Poorly managed diabetes.
4 May 2022

What does it mean when your MRI shows white matter? ›

White matter lesions (WMLs) are areas of abnormal myelination in the brain. These lesions are best visualized as hyperintensities on T2 weighted and FLAIR (Fluid-attenuated inversion recovery) sequences of magnetic resonance imaging. They are considered a marker of small vessel disease.

How is neurodegeneration measured? ›

Neuronal dysfunction and altered connectivity of distinct brain networks are thought to occur early in the course of neurodegenerative diseases and can be measured indirectly with functional magnetic resonance imaging (fMRI).

What causes CBD disease? ›

What causes CBD? CBD occurs when brain cells in certain parts of the brain are damaged as a result of a build-up of a protein called tau. The surface of the brain (cortex) is affected, as well as a deep part of the brain called the basal ganglia.

What is iron deposition brain? ›

Aceruloplasminemia is a rare genetic disorder characterized by the abnormal accumulation of iron in the brain and various internal organs. Affected individuals develop neurological symptoms including cognitive impairment and movement disorders. Degeneration of the retina and diabetes may also occur.

What does it mean when you have white matter on the brain? ›

White matter disease is the wearing away of tissue in the largest and deepest part of your brain that has a number of causes, including aging. This tissue contains millions of nerve fibers, or axons, that connect other parts of the brain and spinal cord and signal your nerves to talk to one another.

Does white matter disease cause fatigue? ›

Brain white matter (WM), and more specifically neuronal connectivity, is thought to perform a crucial role in the central processing of fatigue [1]. In diseases of the WM, such as multiple sclerosis (MS), persisting fatigue is a common disabling complication [2].

Can white matter disease cause personality changes? ›

Now a study has shown that white matter hyperintensities are also found in frontotemporal dementia. Frontotemporal dementia, which often affects people under the age of 65, mainly results in changes in personality, behavior and problems with language rather than memory.

Which type of dementia is associated with white matter lesions? ›

White matter lesions increase the risk of poststroke dementia and, together with lacunar infarcts, are considered the primary type of brain lesions in subcortical ischemic vascular dementia.

Can white matter disease be reversed? ›

White matter disease doesn't have a cure, but there are treatments that can help manage your symptoms. The primary treatment is physical therapy. Physical therapy can help with any balance and walking difficulties you may develop.

Is white matter disease the same as MS? ›

“In general, white matter disease causes acute MS symptoms, like numbness and weakness," Stone says. "Gray matter disease causes progressive symptoms, like fatigue and memory loss. These higher brain functions are called cognitive functions. Most MS disability actually comes from cognitive dysfunction."

Should I be concerned about white matter in the brain? ›

The presence of white matter hyperintensities has been correlated with a higher risk of stroke, which can lead to vascular dementia. White matter hyperintensities are often referred to as white matter disease. Initially, white matter disease was thought to simply be related to aging.

What does white matter signal abnormalities mean? ›

White matter lesions, quantified as 'white matter signal abnormalities' (WMSA) on neuroimaging, are common incidental findings on brain images of older adults. This tissue damage is linked to cerebrovascular dysfunction and is associated with cognitive decline.

Should I worry about white matter hyperintensities? ›

White matter hyperintensities proliferate as the brain ages and are associated with increased risk for cognitive decline as well as Alzheimer's disease and related dementias.

Do most older people have white matter disease? ›

White matter lesions are often found on MR scans of elderly people, they are attributed to degenerative changes of long penetrating arteries. 1-6 Reported prevalence ranges from 5% to 90%, depending on study design, study population, and rating scales.

At what age does the average human have the most white matter? ›

It starts and ends with roughly the same amount of white matter and peaks between ages 30 and 50. But each of the 24 regions changes a different amount. Some parts of the brain, like those that control movement, are long, flat arcs, staying relatively stable throughout life.

Does everyone have white matter in their brain? ›

“Gray matter” is only one of two types of brain tissue; the other “white matter” is rarely mentioned. Yet white matter makes up half the human brain and has not been thought to be important in cognition or learning outside the context of pathology.

How do you know if you have a neurodegenerative disease? ›

Some of the more common symptoms of neurodegenerative diseases include: memory loss. forgetfulness. apathy.

What manifestations of cognitive impairment are primarily characteristic of delirium? ›

Manifestations of delirium include cognitive impairment with reduced awareness, reversed sleep/wake cycle, and distorted thinking and perception.

What are neuroimaging biomarkers? ›

Neuroimaging biomarkers use brain imaging techniques in order to image the morphology (e.g., MRI), the function (e.g. fMRI), the microenvironment (e.g., perfusion MRI), the metabolism (e.g., PET-FDG), or the molecular content (e.g., MR spectroscopy) of the brain and its lesions (Boland.

What are the first symptoms of corticobasal degeneration? ›

Initial symptoms include stiffness; shaky, slow or clumsy movements; and difficulty with speech and comprehension. Other symptoms include: Balance Difficulty walking and balancing. Memory Short-term memory problems, such as repeating questions or misplacing objects.

What is the most common degenerative brain disorder? ›

Alzheimer's disease and Parkinson's disease are the most common neurodegenerative diseases. In the United States, as many as 6.2 million people may have Alzheimer's disease, according to a report from the Alzheimer's Disease Association in 2022.

What is the rarest brain disease? ›

Creutzfeldt-Jakob disease (CJD) is an extremely rare, degenerative brain disorder. It affects about one in every million people per year worldwide.

Can too much iron cause dementia? ›

High iron levels appear to push the progress of the disease, accelerating cognitive decline. People with a build-up of both amyloid and iron are highly likely to develop dementia.

What are the symptoms of iron on the brain? ›

Symptoms
  • Dementia.
  • Difficulty speaking.
  • Difficulty swallowing.
  • Muscle problems such as rigidity or involuntary muscle contractions (dystonia)
  • Seizures.
  • Tremor.
  • Vision loss, such as from retinitis pigmentosa.
  • Weakness.
2 Aug 2020

What is Ferroportin disease? ›

Ferroportin disease, also known as hemochromatosis type 4, is a rare genetic disorder characterized by the abnormal accumulation of iron in the body. Ferroportin disease is caused by mutations of the SLC40A1 gene. The specific symptoms associated with ferroportin disease can vary greatly from one person to another.

Does white matter disease cause headaches? ›

Patients with extensive white matter hyperintensities are likely to have tension-type headaches or to have headaches develop during middle age, according to results published in Cephalagia. Currently, there are no established treatments or strategies for managing white matter hyperintensities.

Is white matter disease rare? ›

The prevalence of leukoencephalopathy with vanishing white matter is unknown. Although it is a rare disorder, it is believed to be one of the most common inherited diseases that affect the white matter.

Is it normal to have white matter hyperintensities on the brain in your 40's? ›

White matter hyperintensity (WMH) is common in healthy adults in their 60s and can be seen as early as in their 30s and 40s.

What is the life expectancy of someone diagnosed with white matter disease? ›

In general, the prognosis is grave, with the majority of patients dying after a few years. However, some die only after several months, and some manage to survive for several decades [6].

What vitamins help with white matter disease? ›

Vitamin D, Folate, and Cobalamin Serum Concentrations Are Related to Brain Volume and White Matter Integrity in Urban Adults.

Does white matter change with age? ›

Many studies have shown that with increasing age, white matter FA decreases, including such white matter areas as the corpus callosum, deep frontal, inferior frontal, medial orbital, fornix, anterior limb of internal capsule, external capsule, anterior cingulum, inferior longitudinal fasciculus, and cerebellar tracts ( ...

Does anxiety cause white matter? ›

Conclusions: Non-clinical individuals with high anxiety already have white matter alterations in the thalamus-cortical circuit and some emotion-related areas that were widely reported in anxiety-related disorders. The altered white matter may be a vulnerability marker in individuals at high risk of clinical anxiety.

Can stress cause white matter? ›

Increased exposures to stressful events are associated with a corresponding increase in the progression of white matter hyperintensities.

Is white matter disease always progressive? ›

White matter disease is a progressive disorder caused by age-related decline in the part of the nerves (the white matter) that connect different areas of brain to each other and to the spinal cord. This disorder can result in memory loss, imbalance and can lead to problems with mobility in older age.

Is white matter disease a precursor to dementia? ›

Originally, white matter disease was considered a normal, age-related change. But over the last decade, medical experts have come to understand that the presence of large areas of disease in the white matter of the brain are associated with cognitive decline and dementia in patients.

Does white matter in the brain mean dementia? ›

White matter disease is strongly linked to cardiovascular disease risk factors, and researchers believe that white matter disease is a biomarker (medical sign) of the lifelong risk of stroke, dementia and disability.

Does white matter mean dementia? ›

White matter has a legitimate position in the study of dementia. The neuropathology of white matter disorders is typically diffuse or widespread, thus disrupting many networks simultaneously and producing a multi-domain syndrome that merits the term dementia.

What foods are good for white matter? ›

February 15, 2012 — A diet rich in vegetables, legumes, fruits, and olive oil and low in dairy, red meat, and processed meat appears to lower white matter hyperintensity volume, new work from the Northern Manhattan Study shows.

Does alcohol cause white matter disease? ›

In contrast, human neuroimaging studies have generally found that alcohol is associated with deleterious changes in the brain including global and regional brain shrinkage and white matter damage, with frontal lobes being particularly affected (Oscar-Berman and Marinkovic, 2007; Sullivan et al., 2010).

Does white matter disease cause vision problems? ›

An MRI of the Brain usually demonstrates white matter lesions seen in typical locations (demyelinating plaques). Sudden onset of double vision is another finding in MS. In this condition the patient usually complains of double vision with both eyes open and the separation of the images is usually horizontal.

What diseases cause white matter on the brain? ›

White matter disease may develop with conditions associated with aging, such as stroke, but it can also affect young people due to conditions such as cerebral adrenoleukodystrophy and multiple sclerosis (MS). Read on to learn more about white matter disease and its symptoms, causes, and prognosis.

What does it mean when you have white matter on a brain MRI? ›

White matter lesions are among the most common incidental findings—which means the lesions have no clinical significance—on brain scans of people of any age. They may also reflect a mixture of inflammation, swelling, and damage to the myelin.

What causes increased white matter in the brain? ›

Many different diseases, injuries, and toxins can cause changes in your white matter. Doctors point to the same blood vessel problems that lead to heart trouble or strokes: Long-term high blood pressure. Ongoing blood vessel inflammation.

How do you know if you have white matter disease? ›

Advances in medical imaging have made white matter disease easier to spot. A magnetic resonance imaging (MRI) test, which takes pictures of the inside of your brain, can show any damage. Changes to white matter will show up super-bright white (your doctor may call this "hyperintense") on an MRI scan.

Is white matter disease the same as MS? ›

“In general, white matter disease causes acute MS symptoms, like numbness and weakness," Stone says. "Gray matter disease causes progressive symptoms, like fatigue and memory loss. These higher brain functions are called cognitive functions. Most MS disability actually comes from cognitive dysfunction."

How serious is white matter disease? ›

The life expectancy after a diagnosis of white matter disease depends on the speed it progresses and the severity of any other conditions it may cause, like stroke and dementia. White matter disease is believed to be a factor in both strokes and dementia. However, more research must be done for further confirmation.

What is the life expectancy of someone diagnosed with white matter disease? ›

In general, the prognosis is grave, with the majority of patients dying after a few years. However, some die only after several months, and some manage to survive for several decades [6].

Can you reverse white matter disease? ›

While there is no known cure for white matter disease, treatments can help to manage the symptoms. Controlling the risk factors associated with heart disease can help decrease the progression of the disease.

What vitamins help with white matter disease? ›

Vitamin D, Folate, and Cobalamin Serum Concentrations Are Related to Brain Volume and White Matter Integrity in Urban Adults.

Can white matter in the brain be repaired? ›

White matter injuries are very serious, but, depending on the type and extent of the injury, extensive recovery may occur. As long as the neuron cell bodies remain healthy, axons can regrow and slowly repair themselves.

Does white matter in brain mean multiple sclerosis? ›

White matter lesions (WML) are a frequent neuroradiological finding in brain MRI with a large number of underlying causes [1]. Two of the most common etiologies are multiple sclerosis (MS) and vascular disorders causing small vessel disease (SVD), each with distinct and characteristic features [2, 3].

Can stress cause white matter? ›

Increased exposures to stressful events are associated with a corresponding increase in the progression of white matter hyperintensities.

What does white matter on the brain mean? ›

White matter is found in the deeper tissues of the brain (subcortical). It contains nerve fibers (axons), which are extensions of nerve cells (neurons). Many of these nerve fibers are surrounded by a type of sheath or covering called myelin. Myelin gives the white matter its color.

What is another name for white matter disease? ›

Description. Leukoencephalopathy with vanishing white matter is a progressive disorder that mainly affects the brain and spinal cord (central nervous system). This disorder causes deterioration of the central nervous system's white matter, which consists of nerve fibers covered by myelin.

Can white matter disease cause personality changes? ›

Now a study has shown that white matter hyperintensities are also found in frontotemporal dementia. Frontotemporal dementia, which often affects people under the age of 65, mainly results in changes in personality, behavior and problems with language rather than memory.

Does alcohol cause white matter disease? ›

In contrast, human neuroimaging studies have generally found that alcohol is associated with deleterious changes in the brain including global and regional brain shrinkage and white matter damage, with frontal lobes being particularly affected (Oscar-Berman and Marinkovic, 2007; Sullivan et al., 2010).

At what age does the average human have the most white matter? ›

It starts and ends with roughly the same amount of white matter and peaks between ages 30 and 50. But each of the 24 regions changes a different amount. Some parts of the brain, like those that control movement, are long, flat arcs, staying relatively stable throughout life.

What foods are good for white matter? ›

February 15, 2012 — A diet rich in vegetables, legumes, fruits, and olive oil and low in dairy, red meat, and processed meat appears to lower white matter hyperintensity volume, new work from the Northern Manhattan Study shows.

Does everyone have white matter in the brain? ›

“Gray matter” is only one of two types of brain tissue; the other “white matter” is rarely mentioned. Yet white matter makes up half the human brain and has not been thought to be important in cognition or learning outside the context of pathology.

Videos

1. Tackling a rare brain disease that affects children
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2. Imaging of White matter I - Prof Dr. Mamdouh Mahfouz (In Arabic)
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3. Clinicopathological classification of neurodegenerative diseases
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4. Neuro imaging: white matter diseases
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5. Neurodegenerative Disorders - Sphingolipidoses Mucopolysaccharidoses [EXPLAINED]
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6. 1-inherited white matter disorders (leukodystrophy)
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