Demyelinating Diseases of the Brain
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1 Department of Radiology University of California San Diego Demyelinating Diseases of the Brain John R. Hesselink, M.D.
2 T1-Weighted Images
3 Normal White Matter Contents Axons with envelope of myelin Neuroglia - Astrocytes & Oligodendrocytes Vascular structures Interstitial space
4 CNS Myelin Produced & maintained by oligodendrocytes Functions as insulator of axons Facilitates transmission of neural impulses Relatively short T1 and T2 relaxation times Demyelinating disease Less myelin & more water Prolongation of both T1 and T2
5 T2-Weighted Image
6 White Matter Diseases Effects of aging Multiple sclerosis Infection & Inflammation Radiation injury Toxic & Metabolic disorders Hereditary disorders
7 History: 26 y/o female with migraine headaches 462
8 {Page 2} Dx: Normal VR spaces
9 History: 59 y/o woman with frequent falls Dx: Large VR space 688
10 History: 77 y/o man with hearing loss 212
11 Dx: VR spaces {Page 2}
12 History: 55 y/o man with cognitive decline 530
13 Dx: Etat crible {Page 2}
14 History: 89 y.o. man with multiple stroke-like episodes 120
15 {Page 3} Dx: FLAIR - VR's and DWMI
16 White Matter Ischemia Pathophysiology Functional significance Associated with aging What do we call it? MR findings Differential diagnosis
17 Deep White Matter Ischemia Imaging Features Subcortical & periventricular location Hyperintense on T2 & FLAIR images Mildly hypointense on T1 images No enhancement Sparing of subcortical U-fibers & corpus callosum
18 History: 62 y.o. male with history of poorly controlled hypertension 153
19 Hypertension {Page 2}
20 {Page 3}
21 Dx: FLAIR - DWMI {Page 4}
22 History: 76 y.o. male with vertebral-basilar TIA's 119
23 Vertebral-basilar TIA's {Page 2}
24 TIA's {Page 3} Dx: FLAIR & Diffusion - DWMI
25 History: 79 y/o man with left leg weakness 208
26 Leg weakness {Page 2} Dx: Brain metastases - Bladder CA
27 Deep White Matter Ischemia Periventricular Disease 529
28 {Page 2} Dx: DWMI, VR's & lacunes
29 History: 55 y/o female with motor weakness, double vision, & emotional lability 272
30 Motor weakness {Page 2}
31 {Page 3} Dx: Chronic multiple sclerosis; Incidental sebaceous cyst left posterior scalp
32 Multiple Sclerosis Clinical Features Chronic inflammatory disease of myelin Patients between 20 and 50 years old Etiology unknown, but probably a post-viral autoimmune reaction Acute, transient attacks of neurologic dysfunction Dx by history, physical exam, lab & imaging
33 History: 35 y/o man with bilateral facial pain 363
34 {Page 2}
35 History: 50 y/o woman with a right hemiparesis 291
36 {Page 2}
37 {Page 3} Dx: MS subcallosal striations
38 History: 28 y/o woman referred for brain tumor 440
39 {Page 2}
40 441 History: 45 y/o woman with arm & leg weakness, sensory changes, & bilateral visual loss
41 {Page 2} Dx: Neuromyelitis optica (Devic's disease)
42 History: 25 y/o woman with right sided weakness 398
43 {Page 2} Dx: Balo's concentric sclerosis
44 History: 23 y/o man with intermittent and migratory numbness & paresthesias for several months
45 Dx: Chronic-progressive MS {Page 2}
46 Differential Features WM Ischemia Multiple Sclerosis Age > 60 years years Corpus callosum Subcortical U- fibers No No Yes Yes Enhance No Yes (acute)
47 History: 62 y.o. man with episodes of weakness which was poorly localized 30
48 Episodes of weakness {Page 2}
49 {Page 3} Dx: Multiple sclerosis
50 History: 53 y/o woman with 4 days of headache, fatigue, ataxia and poor memory 674
51 {Page 2}
52 {Page 3}
53 {Page 4} Dx: Metastasis Adeno CA Lung & chronic MS
54 White Matter Diseases Infectious & Inflammatory Disorders Progressive multifocal leukoencephalopathy Postinfectious encephalomyelitis HIV encephalitis
55 History: 31 y/o male with multiple neurological deficits 407
56 {Page 2}
57 {Page 3} Dx: AIDS - PML
58 History: 48 y/o HIV+ man with fever, mutism, ataxia & dysphagia 288
59 Ataxia & dysphagia {Page 2} Dx: PML - Progressive multifocal leukoencephalopathy Gd+
60 History: 8 year old boy with 1-2 week history of left eye ptosis, limited EOMs, and ataxia. C-53-56
61 Dx: ADEM {Page 2}
62 Acute Disseminated Encephalomyelitis (ADEM) A post-infectious encephalitis Immune-mediated reaction Viral agents: measles, chickenpox or vaccination for rabies or smallpox Acute demyelinating disease Mostly a monophasic illness
63 725 History: 67 y/o woman with progressively altered mental status over several days
64 Dx: Probable ADEM {Page 2} Bronchitis 3 weeks earlier
65 History: 61 y/o woman with diabetes & hypertension presented with aphasia 255
66 Aphasia {Page 2}
67 {Page 3} Dx: Post-Infectious encephalitis (Acute disseminated encephalomyelitis)
68 History: 35 y/o male with fever & ataxia 511
69 {Page 2} Dx: HIV encephalitis
70 Radiation Injury Imaging Features Latent period of 6-8 months Symmetric white matter involvement Initial sparing of corpus callosum and subcortical U-fibers Scalloped outer margins Progression over 2-3 years
71 History: 51 y/o woman with a breast mass
72 {Page 2} 4 months post radiation
73 {Page 3} 14 months post radiation Dx: Radiation change
74 History: 52 y/o women had radiation therapy for a glioma 1 year ago 190
75 S/P radiation for glioma {Page 2} Dx: Radiation injury
76 White Matter Diseases Acquired Toxic & Metabolic Disorders Effects of chemotherapeutic agents Immunosuppressant therapy (PRES) Central pontine myelinolysis Organic Compounds and Drugs
77 Neurotoxic Chemotherapeutic Agents Methotrexate, cis-platin, ARA-C, BCNU, Thiotepa Acute effects: Develop during therapy Transient diffuse WM hyperintensity Delayed effects: Onset within few weeks or months Patchy or confluent WM pattern Little mass effect or enhancement Calcification in children Synergistic effect of chemo & radiation
78 History: 67 y/o female with lung CA Dx: Toxic encephalopathy - Chemo: carboplat & taxol O 9-10
79 History: 19 y/o woman with systemic lupus erythematosus, candida pneumonia & increasing confusion 321
80 Treated with cytoxan 2 weeks earlier {Page 2}
81 {Page 3} Dx: Toxic encephalopathy from cytoxan therapy
82 History: 47 y/o woman with myelodysplasia developed seizures and mental confusion 396
83 {Page 2} s/p bone marrow transplantation
84 Dx: Cyclosporine toxicity {Page 3}
85 Immunosuppressant Therapy Cyclosporines most widely used Neurotoxic effects in 20% High signal lesions on T2WIs Predilection for occipital white matter Gray matter damage also occurs White matter injury often reversible
86 History: 30 y/o man with headache, left HH, & left HP 718
87 {Page 2} Renal transplant rejection while on cyclosporine; Bp = 220/120
88 Dx: PRES (Cyclosporine & acute hypertension) {Page 3} Tx: anti-hypertensives & steroids 2 months later
89 History: 53 y/o man admitted with liver failure & hyponatremia 469
90 {Page 2} Dx: Central pontine myelinolysis
91 History: 25 y/o male found comatose with decerebrate posturing 721
92 {Page 2}
93 {Page 3} Dx: Toxic encephalopathy Benzodiazepine & Heroine
94 Metabolic Diseases Inherited disorders Specific enzyme deficit Early infancy or childhood Prominent white matter involvement, but gray matter also affected
95 History: 14 y.o. boy with recent onset of disorientation and confusion 38
96 Disorientation & confusion {Page 2}
97 {Page 3} Dx: Adrenoleukodystrophy
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