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White matter diseases

White matter diseases. Nguyễn Thị Ánh Hồng. White matter diseases: Dysmyelinating ( primary abnormality of myelin formation) processes Demyelinating ( secondary destruction of normal myelin) Demyelinating processes: Primary and secondary Primary demyelinating disorders: autoimmune ( MS)

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White matter diseases

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  1. White matter diseases NguyễnThịÁnhHồng

  2. White matter diseases: • Dysmyelinating ( primary abnormality of myelin formation) processes • Demyelinating ( secondary destruction of normal myelin) • Demyelinating processes: Primary and secondary • Primary demyelinating disorders: autoimmune ( MS) • Secondary demyelinating disorders: infectious, vascular and toxic – metabolic categories

  3. Classification and characterization of periventricular and deep white matter hyperintensities on MRI • PWMHs: • continuous with the margins of each lateral ventricle, • distance from the ventricle surface: 3 - 13mm • Juxtaventricular WMHs: < 3mm. • Deep and juxtacortical WMHs: 4mm from the corticomedullary junction

  4. White matter diseases: Causes • Autoimmune White Matter Diseases • Multiple Sclerosis • NeuromyelitisOptica • Acute Disseminated Encephalomyelitis ADEM • Acute Hemorrhagic Leukoencephalitis • Infectious White Matter Diseases • Progressive Multifocal Leukoencephalopathy PMLE • HIV Encephalopathy • Lyme Disease • Vascular White Matter Diseases: Small-Vessel Disease • Small-Vessel Disease Related to Arteriolosclerosis • Cerebral Amyloid Angiopathy • Cerebral Autosomal-dominant Arteriopathy with Subcortical Infarcts and Leukoencephalopathy CADASIL • Primary Angiitis of the Central Nervous System • Susac Syndrome • Systemic Lupus Erythematosus • Toxic-Metabolic White Matter Diseases • Osmotic Myelinolysis • Treatment-related Leukoencephalopathies • Posterior Reversible Encephalopathy Syndrome • Ethanol-related Diseases • Illicit Drugs: Heroin Inhalation

  5. Autoimmune White Matter Diseases: Multiple Sclerosis (MS) • MS is a primary demyelinating disease of unknown etiology (autoimmune category), perivenular inflammation/demyelination, manifesting as periventricular, juxtacortical, infratentorial, and spinal cord lesions at MRI. • MS: nontraumatic neurologic disability in young adults. The prevalence: 15- 250/ 100 000. F > M (2:1). Clinical onset: 20–40 years, peak at 30 years. • MRI: the most important paraclinical tool for diagnosing and monitoring MS. McDonald criteria: • Dissemination in space: WMHs at periventricular, juxtacortical, infratentorial, and spinal cord; • Dissemination in time: both contrast-enhancing and nonenhancing lesions or development of new lesions at follow-up MRI. New WMHs + contrast-enhancing: inflammatory activity

  6. Periventricular & juxtacortical WMHs ( dissemination in space) enhancing & nonenhancing lesions ( dissemination in time). Dawson fingers: periventricular lesions perpendicular to the ependymal surface .

  7. Autoimmune White Matter Diseases: NeuromyelitisOptica NMO • NMO (Devic disease) is a demyelinating disease of autoimmune etiology induced by NMO–immunoglobulin G • 0.5 - 4.4 per 100 000, more common in Asian, Indian, and African. F: M = 6,5:1 The age of onset varies widely. • The optic neuritis can be unilateral or bilateral.

  8. hyperintensity and swelling of the right optic nerve hyperintensity involving the medulla oblongata and cervical cord

  9. Autoimmune White Matter Diseases: Acute Disseminated Encephalomyelitis ADEM • ADEM: • 0,4/10000 person – years, • children ( <15 years old), • within 2 weeks after an infection (50%–75%: upper respiratory) or a vaccination. • M = F • Clinical: encephalopathy, fever, headache, seizure, multiple focal deficits, and meningeal signs. • ADEM: • multiple and bilateral/ both brain and spine, both white matter and gray matter. • ADEM lesions: • round and large/ the deep gray matter nuclei, thalamus, and brainstem. • Subcortical WMHs and U-fiber involvement are frequently observed. Corpus callosum involvement in ADEM can sometimes be seen

  10. 12F. T2 FLAIR, bilateral lesions: deep gray matter nuclei & periventricular-subcortical WM 28F. T2 FLAIR, multiple scattered foci of microhemorrhage at the corticosubcortical interface

  11. Infectious White Matter Diseases:Progressive Multifocal Leukoencephalopathy PML • PML is an infection related to reactivation of John Cunningham (JC) virus in the context of cellular immunodeficiency (eg, HIV infection/AIDS, lymphoproliferative diseases, or transplantation). • Imagings: • asymmetric lesions with peripheral white matter and subcortical U-fibers • Little mass effect • No significant contrast enhancement. • PML may also involve the corpus callosum.

  12. PML 65W - Axial T2WI shows a large asymmetric confluent WMH, with involvement of the subcortical U-fibers and without mass effect. - Coronal CE: the frontal WM (left > right), extension to the subcortical U-fibers & corpus callosum. Note the sparing of the gray matter (typical) and an adjacent rim of contrast enhancement (atypical).

  13. Infectious White Matter Diseases:HIV Encephalopathy • HIV encephalopathy is also known as AIDS dementia complex (ADC). • HIV encephalopathy occurs in 15%–20% of AIDS patients and is a major cause of morbidity. • Lower CD4 cell count, longer disease duration, and older age are at higher risk. • Diffuse and marked periventricular demyelination, neuronal loss, astroglial proliferation, and vacuolar changes are observed. • MRI: diffuse bilateral and symmetric periventricular WMH, central WM, No mass effect or enhancement.

  14. 27M, Axial T2 FLAIR: symmetric periventricular white matter lesions, some prominence of ventricles.

  15. Infectious White Matter Diseases:Diffuse Cytomegalovirus (CMV) encephalitis • Both clinical and radiological features of CMV encephalitis mimic HIVE. • Patients with CMV encephalitis have had acquired immunodeficiency syndrome (AIDS) for a longer duration • Rapidly progressive dementia, lower CD4 counts and shorter survival period than HIVE patients

  16. Infectious White Matter Diseases:CNS Tuberculosis • Clinical: mild headache, low grade fever, seizures, focal deficits. 50% cases not have pulmonary tuberculosis. • MRI: meningeal enhancement, hydrocephalus, focal parenchymal enhancements

  17. Vascular White Matter Diseases: Small-Vessel Disease • Small-vessel disease is the leading cause of white matter disease • 6 types: • Arteriolosclerosis (type 1); • Cerebral amyloid angiopathy (type 2); • Inherited vasculopathies (type 3) ( CADASIL, MELAS, Fabry disease); • Inflammatory vasculitides (type 4) ( primary angiitis of the central nervous system [PACNS], Susac syndrome, systemic lupus erythematosus [SLE], Sjögren syndrome); • Venous collagenosis (type 5); • And other (type 6) ( radiation therapy).

  18. Vascular White Matter Diseases: Small-Vessel Diseases:Arteriolosclerosis • Vacular risk factors: hypertension, diabetes, smoking, cholesterol… • Small vessel diseases: • Lacunar ischemic • Hemorrhage stroke • Microbleeds • Perriventricular spaces • Brain atrophy

  19. Vascular White Matter Diseases: Small-Vessel Diseases:Cerebral Amyloid Angiopathy • Cerebral amyloid angiopathy is a vasculopathy caused by β-amyloid deposition in the media and adventitia of cortical and leptomeningeal arteries • The occipital and frontal regions more frequently. • After the age of 55 years, prevalence 10%–40% , > 80% of patients with Alzheimer disease. M=F. • Peripheral micro- and macrohemorrhages, atraumatic convexal subarachnoid hemorrhage, superficial siderosis, and WMH are common radiologic manifestations.

  20. Cerebral amyloid angiopathy 77M. Axial SWI (susceptibility-weighted image): microbleeds at the corticosubcortical interface.

  21. Vascular White Matter Diseases: Small-Vessel Diseases:Cerebral Autosomal-dominant Arteriopathy with Subcortical Infarcts and LeukoencephalopathyCADASIL • CADASIL is an inherited nonarteriosclerotic and amyloid-negative small-vessel disease with autosomal-dominant transmission, NOTCH3 gene on chromosome 19. • Prevalence: 3-4/ 100 000, M=F, onset: 3rd and 4th decades. • Clinical: migraines, recurrent ischemic strokes, and progressive cognitive impairment in young adults. CADASIL is the most frequent hereditary cause of subcortical vascular dementia. • The radiologic findings: • The end of the 3rd decade, WMHs in the temporal pole • The 4th decade, the WMHs progress to the posterior temporal, frontal, and parietal regions, the basal nuclei and thalami. Subcortical U-fibers can be involved, and subcortical lacunar infarcts become a common finding. Involvement of the external capsule and corpus callosum, which are other important markers for CADASIL • The 5th decade, microbleeds develop • 50–60 years, extensive WMHs, lacunes, and

  22. CADASIL, 65M Axial T2 FLAIR: confluent periventricular WMHs, with typical involvement of the external capsules.

  23. Vascular White Matter Diseases: Small-Vessel Diseases:Susac Syndrome • Rare, young adults (20–40 years), F:M = 2:1. Susac syndrome: vasculitis produced by antiendothelial antibodies to specific neural vessels the cochlea, retina, and brain microinfarcts. • Clinical: hearing loss (sensorineural), visual loss, and encephalopathy. • Ophthalmoscopy is a cornerstone of the diagnosis, branch retinal artery occlusion with retinal infarcts, arterial hyperfluorescence, and leakage of fluorescein. • MRI: multiple microinfarcts, +/- diffusion restriction and contrast enhancement. • Leptomeningeal enhancement: 1/3 of patients, • Parenchymal enhancement: 2/3. • Corpus callosum involvement is the rule ( central region of the corpus callosum is more frequently

  24. Susac syndrome, 23M. Axial T2FLAIR: hyperintense lesions involving the periventricular white and deep gray matter (a) and corpus callosum (c), with restricted diffusion on DWI (b)

  25. Vascular White Matter Diseases: Small-Vessel Diseases:Systemic Lupus Erythematosus • Up to 50% of patients with lupus develop neuropsychiatric SLE (NPSLE), a main cause of morbidity, and the cause of death in approximately 19% of patients with SLE. • MRI: Hyperintensity at white & gray matter. Small foci of contrast enhancement & restricted diffusion were also present Neuropsychiatric lupus, 36F T2FLAIR: extensive hyperintensity at white & gray matter of the right anterior temporal lobe and right midbrain.

  26. Toxic-Metabolic White Matter Diseases:Osmotic Myelinolysis • Osmotic myelinolysis occurs with osmotic stress, rapid hyponatremia, alcohol abuse or anorexia. • Disorientation, vomiting, spastic quadriplegia, unresponsiveness, and cranial nerve palsies are classic symptoms. • 65% involves the pons and is typically central with sparing of the periphery, the thalami, basal nuclei, and subcortical WM. In the acute phase, diffusion restriction can be present. 67M, rapid hyponatremia T2FLAIR: extensive central pontine hyperintensity sparing the periphery.

  27. Toxic-Metabolic White Matter Diseases:Treatment-related Leukoencephalopathies • Treatment-related Leukoencephalopathies • Many therapies are associated with brain changes. • More commonly: methotrexate, several chemotherapies (fluorouracil, cytarabine), and antiepileptics (vigabatrin). • Methotrexate Leukoencephalopathy. • High doses of methotrexate. • Frontoparietal WM and centrum semiovales, periventricular and deep WM, no contrast enhancement or mass effect, atrophy. 7M, high dose methotrexate

  28. Toxic-Metabolic White Matter Diseases:Posterior Reversible Encephalopathy Syndrome PRES • Hypertensive crisis, preeclampsia/eclampsia, or cytotoxic drugs. • PRES: cortical and subcortical white matter areas. • bilateral areas of T2 hyperintense edema, more frequently involving the parieto-occipital and posterior frontal regions. 26F, Preeclampsia

  29. Toxic-Metabolic White Matter Diseases:Ethanol-related Diseases • Wernicke Encephalopathy. • Vitamin B1 deficiency • Classic symptoms: ataxia, nystagmus, ophthalmoplegia, severe memory impairment. • Typical locations: the mammillary bodies, periaqueductal gray matter, medial thalami, and tectal plate, with signal intensity anomaly preceding atrophic changes. • Marchiafava-Bignami Disease: • Chronic alcoholism. • Confusion and seizures. • the middle layers of the body of the corpus callosum • Type A, extensive lesions, swelling of the corpus callosum, and poor outcome; • Type B, smaller lesions, without callosal swelling, with better outcome. Diffusion restriction of the corpus callosum in the acute phase

  30. Toxic-Metabolic White Matter Diseases:Illicit Drugs: Heroin Inhalation Heroin-induced leukoencephalopathy in 26M. Axial T2FLAIR: - bilateral of posterior WM, - the posterior arms of the internal capsule, - the splenium of the corpus callosum.

  31. Toxic-Metabolic White Matter Diseases: • Delayed Hypoxic Ischemic Injury: • poisoning and recovery after cardiac arrest. • Carbon Monoxide Leukoencephalopathy • The globipallidi are typically involved, • periventricular white matter injury can be a delayed manifestation • Radiation Leukoencephalopathy • 1/3 of patients, whole-brain radiation therapy and/or high doses (30–40 Gy). • Most often, risk factors: large target areas + chemotherapy. Months - years after the radiation therapy. More severe damage: necrotic changes with contrast enhancement.

  32. Conclusion • A systematic approach combining imaging, clinical, and laboratory data is crucial for making the correct diagnosis in white matter disorders. • Understanding the pathologic substrate is fundamental for understanding the radiologic manifestations.

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