Table of ContentsView AllTable of ContentsTypesSymptomsCausesDiagnosisTreatmentPrognosis
Table of ContentsView All
View All
Table of Contents
Types
Symptoms
Causes
Diagnosis
Treatment
Prognosis
White matter disease is a group of conditions that specifically affect the areas of thecentral nervous system(CNS) defined aswhite matter. Some of these conditions, such as multiple sclerosis (MS), affect only the white matter of the CNS (brain and spinal cord), and some conditions, such as vascular dementia, may also affect other areas of the brain.
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What Is White Matter?
The white matter of the CNS appears white when viewed microscopically after preparation with some stains. White matter is heavily myelinated, meaning it is enclosed in a myelin sheath.Myelinis a type of fatty protective coating over the nerves that helps to facilitate the speed of nerve signals in nerve pathways throughout the nervous system.
White matter is located in the deep, inner regions of the brain and the outer regions of the spinal cord.
By contrast, the gray matter appears gray when viewed microscopically after some types of staining. Gray matter is located on the outer regions of the brain and the central areas of the spinal cord.
Types of White Matter Disease
Areas AffectedSome of the conditions that cause white matter disease do not involve other areas of the CNS. However, many white matter diseases have the potential to affect other areas of the CNS in addition to white matter and may manifest as white matter disease or as more extensive CNS disorders.
Areas Affected
Some of the conditions that cause white matter disease do not involve other areas of the CNS. However, many white matter diseases have the potential to affect other areas of the CNS in addition to white matter and may manifest as white matter disease or as more extensive CNS disorders.
White Matter Disease Symptoms
The symptoms of white matter disease can be either permanent or intermittent, depending on the disease. The symptoms corresponded to the affected areas of CNS involvement.
Many nerve pathways travel throughout the white matter in the CNS. So, relatively small areas of white matter disease can cause substantial weakness or sensory changes. Extensive white matter disease can also cause nonspecific symptoms, such as dementia and vision changes.
Symptoms of white matter disease may include combinations of the following:
Most white matter diseases do not cause all of these symptoms. Rather, they each produce a pattern of symptoms that are characteristic of that particular disease.
White matter disease can occur due to degeneration,demyelination, inflammation, infection, drug toxicity, nutritional deficiency, or any condition that causes damage to the white matter of the CNS.
Many of the conditions affecting white matter are associated with inflammation.In fact, vascular dementia, which occurs due to strokes, is associated with extensive vascular disease affecting the small blood vessels of the brain. Risk factors include smoking, uncontrolled high blood pressure (hypertension), unhealthy fat and cholesterol levels, and untreated diabetes—all of which contribute to inflammation of the blood vessels.
White matter disease is diagnosed through a process that involves taking a medical history, performing a physical examination, and reviewing imaging tests blood samples or samples ofcerebrospinal fluid(CSF).
The key features of the medical history include a description and timing of the symptoms, and any other associated symptoms, such as a fever.
The physical examination helps determine whether the condition is mainly a neurological condition or a systemic disease (one that affects the entire body). Your medical provider will examine the appearance of your skin, ask you to identify painful areas, check for weakness or sensory changes, and check reflexes and coordination.
Diagnostic testing may include:
Some white matter diseases can be reversed or controlled with treatment. However, some white matter diseases can only be treated symptomatically.
Immune suppression: Several different white matter diseases can be treated with anti-inflammatory medications, such as steroids, immunoglobulins, orplasmapheresis(removing blood plasma). These treatments are used for MS exacerbations and ADEM. While they are sometimes used to manage acute stages of HIV encephalopathy or PML, the underlying infections can worsen with immunosuppression.
Additionally, MS is also treated withdisease-modifying therapy (DMT), which helps prevent exacerbations.
The prognosis of white matter disease varies. MS is not a fatal disease. By managing the MS and co-occurring conditions,life expectancymay be only slightly shorter.
Most infectious causes of white matter disease can be treated with medication, resulting in improvement and good survival. However, HIV encephalopathy and PML are associated with a very poor prognosis and short life expectancy.
Vascular dementia generally is associated with a reduced life expectancy after diagnosis.This is because vascular dementia develops due to factors that can also increase the risk of stroke and heart disease.
Summary
White matter diseases affect the white matter in the CNS. Some white matter diseases only involve white matter, such as MS. Some white matter diseases can also involve other parts of the CNS.
There’s a wide range of white matter diseases. The timing, causes, symptoms, and treatments vary. Most of these diseases are associated with inflammation, but there are also other underlying causes with each of these conditions. Some white matter diseases can be treated effectively, but others are permanent, such as vascular dementia.
7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Hemachudha P, Rattanawong W, Pongpitakmetha T, Phuenpathom W.Fluorouracil-induced leukoencephalopathy mimicking neuroleptic malignant syndrome: a case report.J Med Case Rep.2023;17(1):86. doi:10.1186/s13256-023-03814-3Shih YC, Tseng WI, Montaser-Kouhsari L.Recent advances in using diffusion tensor imaging to study white matter alterations in Parkinson’s disease: a mini review.Front Aging Neurosci. 2023 Feb 22;14:1018017. doi:10.3389/fnagi.2022.1018017Zhang W, Zhou X, Yin J, et al.YKL-40 as a novel biomarker related to white matter damage and cognitive impairment in patients with cerebral small vessel disease.Brain Res.2023;1807:148318. doi:10.1016/j.brainres.2023.148318Sarbu N, Shih RY, Jones RV, Horkayne-Szakaly I, Oleaga L, Smirniotopoulos JG.White matter diseases with radiologic-pathologic correlation.Radiographics.2016;36(5):1426-1447. doi:10.1148/rg.2016160031Gertje EC, Janelidze S, van Westen D, Cullen N, Stomrud E, Palmqvist S, Hansson O, Mattsson-Carlgren N.Associations between CSF markers of inflammation, White matter lesions, and cognitive decline in individuals without dementia.Neurology.2023 Mar 7:10.1212/WNL.0000000000207113. doi:10.1212/WNL.0000000000207113Graf LM, Rosenkranz SC, Hölzemer A, et al.Clinical presentation and disease course of 37 consecutive cases of progressive multifocal leukoencephalopathy (PML) at a German tertiary-care hospital: a retrospective observational study.Front Neurol. 2021;12:632535. doi:10.3389/fneur.2021.632535British Heart Foundation.Vascular dementia: your questions answered.
7 Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Hemachudha P, Rattanawong W, Pongpitakmetha T, Phuenpathom W.Fluorouracil-induced leukoencephalopathy mimicking neuroleptic malignant syndrome: a case report.J Med Case Rep.2023;17(1):86. doi:10.1186/s13256-023-03814-3Shih YC, Tseng WI, Montaser-Kouhsari L.Recent advances in using diffusion tensor imaging to study white matter alterations in Parkinson’s disease: a mini review.Front Aging Neurosci. 2023 Feb 22;14:1018017. doi:10.3389/fnagi.2022.1018017Zhang W, Zhou X, Yin J, et al.YKL-40 as a novel biomarker related to white matter damage and cognitive impairment in patients with cerebral small vessel disease.Brain Res.2023;1807:148318. doi:10.1016/j.brainres.2023.148318Sarbu N, Shih RY, Jones RV, Horkayne-Szakaly I, Oleaga L, Smirniotopoulos JG.White matter diseases with radiologic-pathologic correlation.Radiographics.2016;36(5):1426-1447. doi:10.1148/rg.2016160031Gertje EC, Janelidze S, van Westen D, Cullen N, Stomrud E, Palmqvist S, Hansson O, Mattsson-Carlgren N.Associations between CSF markers of inflammation, White matter lesions, and cognitive decline in individuals without dementia.Neurology.2023 Mar 7:10.1212/WNL.0000000000207113. doi:10.1212/WNL.0000000000207113Graf LM, Rosenkranz SC, Hölzemer A, et al.Clinical presentation and disease course of 37 consecutive cases of progressive multifocal leukoencephalopathy (PML) at a German tertiary-care hospital: a retrospective observational study.Front Neurol. 2021;12:632535. doi:10.3389/fneur.2021.632535British Heart Foundation.Vascular dementia: your questions answered.
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
Hemachudha P, Rattanawong W, Pongpitakmetha T, Phuenpathom W.Fluorouracil-induced leukoencephalopathy mimicking neuroleptic malignant syndrome: a case report.J Med Case Rep.2023;17(1):86. doi:10.1186/s13256-023-03814-3Shih YC, Tseng WI, Montaser-Kouhsari L.Recent advances in using diffusion tensor imaging to study white matter alterations in Parkinson’s disease: a mini review.Front Aging Neurosci. 2023 Feb 22;14:1018017. doi:10.3389/fnagi.2022.1018017Zhang W, Zhou X, Yin J, et al.YKL-40 as a novel biomarker related to white matter damage and cognitive impairment in patients with cerebral small vessel disease.Brain Res.2023;1807:148318. doi:10.1016/j.brainres.2023.148318Sarbu N, Shih RY, Jones RV, Horkayne-Szakaly I, Oleaga L, Smirniotopoulos JG.White matter diseases with radiologic-pathologic correlation.Radiographics.2016;36(5):1426-1447. doi:10.1148/rg.2016160031Gertje EC, Janelidze S, van Westen D, Cullen N, Stomrud E, Palmqvist S, Hansson O, Mattsson-Carlgren N.Associations between CSF markers of inflammation, White matter lesions, and cognitive decline in individuals without dementia.Neurology.2023 Mar 7:10.1212/WNL.0000000000207113. doi:10.1212/WNL.0000000000207113Graf LM, Rosenkranz SC, Hölzemer A, et al.Clinical presentation and disease course of 37 consecutive cases of progressive multifocal leukoencephalopathy (PML) at a German tertiary-care hospital: a retrospective observational study.Front Neurol. 2021;12:632535. doi:10.3389/fneur.2021.632535British Heart Foundation.Vascular dementia: your questions answered.
Hemachudha P, Rattanawong W, Pongpitakmetha T, Phuenpathom W.Fluorouracil-induced leukoencephalopathy mimicking neuroleptic malignant syndrome: a case report.J Med Case Rep.2023;17(1):86. doi:10.1186/s13256-023-03814-3
Shih YC, Tseng WI, Montaser-Kouhsari L.Recent advances in using diffusion tensor imaging to study white matter alterations in Parkinson’s disease: a mini review.Front Aging Neurosci. 2023 Feb 22;14:1018017. doi:10.3389/fnagi.2022.1018017
Zhang W, Zhou X, Yin J, et al.YKL-40 as a novel biomarker related to white matter damage and cognitive impairment in patients with cerebral small vessel disease.Brain Res.2023;1807:148318. doi:10.1016/j.brainres.2023.148318
Sarbu N, Shih RY, Jones RV, Horkayne-Szakaly I, Oleaga L, Smirniotopoulos JG.White matter diseases with radiologic-pathologic correlation.Radiographics.2016;36(5):1426-1447. doi:10.1148/rg.2016160031
Gertje EC, Janelidze S, van Westen D, Cullen N, Stomrud E, Palmqvist S, Hansson O, Mattsson-Carlgren N.Associations between CSF markers of inflammation, White matter lesions, and cognitive decline in individuals without dementia.Neurology.2023 Mar 7:10.1212/WNL.0000000000207113. doi:10.1212/WNL.0000000000207113
Graf LM, Rosenkranz SC, Hölzemer A, et al.Clinical presentation and disease course of 37 consecutive cases of progressive multifocal leukoencephalopathy (PML) at a German tertiary-care hospital: a retrospective observational study.Front Neurol. 2021;12:632535. doi:10.3389/fneur.2021.632535
British Heart Foundation.Vascular dementia: your questions answered.
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