Dean Shibata, M.D. Department of Radiology, University of Washington. White Matter, Metabolic, and Degenerative Disease.

Transcription

1 White Matter, Metabolic, and Degenerative Disease UW Radiology Review 2015 Dean Shibata, M.D Neuroradiology Department of Radiology, University of Washington MS (Multiple Sclerosis) ADEM (Acute Disseminated Encephalomyelitis) CPM (Central Pontine Myelinolysis) PRES (Posterior Reversible Encephalopathy Syndrome) PML (Progressive Multifocal Leukoencephalopathy) 1

2 WM Dz: Classific by Mechanism Primary demyelinating disease Multiple Sclerosis Secondary demyelinating disease Allergic Viral Vascular Metabolic Toxic WM Dz: Classific by Mechanism Allergic (immunologic) Viral Acute disseminated encephalomyelitis - ADEM HIV-associated encephalitis Prog multifocal leukoencephalopathy - PML Subacute sclerosing Panencephalitis y SSPE Vascular Binswanger s disease (WM sml vessel dz) Postanoxic encephalopathy Toxic 2

3 WM Dz: Classific by Mechanism Toxic Radiation Marchiafava-Bignami Disease (Corpus Collosum) Disseminated necrotizing leukoencephalopathy Drugs (chemorx, metamphetamine, cocaine) Toxins (triethyl, tin, lead) Metabolic central pontine myelinolysis (osmotic) - CPM Traumatic: Diffuse axonal shear injury - DAI WM Dz: Classific by Mechanism Dysmyelinating Disease Adrenoleukodystrophy Males, posterior, Pons/Medulla, Gad+ Metachromatic Leukodystrophy (MLD) cerebellum, spares subcortical U fibers and BG Krabbe s Disease Ca++ BG Alexander Disease Big head, Anterior Canavan s Disease Big head, NAA, stem/bg Pelizaeus-Merzbacher Disease (PMD) tigroid WM Toxins (solvents, tin, lead) 3

4 Metachromatic leukodystrophy. Tigroid WM (Also Pelizaeus- Merzbacher) Cheon J et al. Radiographics 2002;22: by Radiological Society of North America Metachromatic leukodystrophy Leopard Skin WM Cheon J et al. Radiographics 2002;22: by Radiological Society of North America 4

5 Hallervorden-Spatz Pantothenate kinase-associated neurodegeneration (PKAN) Guillerman R P Radiology 2000;217: by Radiological Society of North America Barkovich 1995 Canavan s 5

6 Which one is Adrenoleukodystrophy? 1 2 Adrenoleukodystrophy? 1. Post Dz 2. Ant Dz 6

7 ALD Alexander WM Small Vessel Dz Risk factors Hypertension APOE4 Responds to Rx Predicts future clinical strokes Correlates with cognitive decline Clinical Reporting variable 7

8 Clinical Correlations: Risk for Stroke WMH grade Strokes/1000 Person Years (Number at Risk) MRI Infarct absent MRI Infarct present <= (1005) 7.2 (170) (753) 14.6 (269) (414) 31.0 (222) (116) 34.1 (124) >= (92) 21.4 (913) CHS: Kuller LH, et al, Stroke 2004 Number of Reports x 1x (risk for future stroke) 2x 3x Severity of White Matter Disease in Clinical Reports 4x 4x 4x White Matter Grade (CHS Standard Scale) Severe Moderate Mild Minimal None No Mention 4x 4x 8

9 1 White Matter 2 Dz Standards Number of Reports Severity of White Matter Disease in Clinical Reports Severe Moderate Mild Minimal None No Mention White Matter Grade (CHS Standard Scale) 9

10 Severity of White Matter Disease in Clinical Reports Severity of White Matter Disease in Clinical Reports 100% Clinical Report Description 80% 60% 40% 20% 0% Severe Moderate Mild Minimal None No Mention White Matter Grade Multiple Sclerosis Variants: Acute - death in 10 months Devics = Neuromyelitis optica Balo s Disease - Concentric Sclerosis 85 % have ovoid perivent lesions (Dawson s fingers) 50 to 90 % w definite MS have CC lesions 10% of adults have PF lesions MS lesions generally lack mass effect 10

11 44 year old numbness legs and feet 35 year old R body numbness R leg weakness Dawson s Fingers Flame shaped periventricular 11

12 21 year old optic neuritis 29 year old trouble controlling R side body Target Sign 42 year old scattered parethesias 41 year old blurred vision FLAIR Sagittal 12

13 Corpus Collosum Punched out lesions on T1W 13

14 DDx: Corpus Callosum Lesions Tumors: GBM, Lymphoma, Lipoma Demyelinating Dz: MS, Marchiafava- Bignami, PML Infarct ( rare, dual arterial supply) Multiple Sclerosis: Which one is False? 1) Most lesions occur at the periventricular white matter and corpus collosum 2) Atrophy of the corpus collosum is not seen 3) Enhancement of the optic nerves may be seen 4) Spinal cord lesions may mimic neoplasm with cord enlargement 14

15 Multiple Sclerosis: which is false? 1. Periventricular white matter and corpus collosum lesions 2. No corpus collosum atrophy 3. Optic nerve enhancement 4. Spinal cord lesions may mimic neoplasm Tumefactive MS 15

16 PreGad PostGad Tumefactive MS Which one is Multiple Sclerosis? 24 year old lethargy p respiratory illness 24 year old scattered parethesias

17 Which is Multiple Sclerosis? year old lethargy p respiratory illness 24 year old scattered parethesias Histoplasmosis MS 17

18 Which one is Multiple Sclerosis? 23 year old vague parethesias 39 year old headaches 1 2 Which one is Multiple Sclerosis?

19 23 year old vague parethesias 39 year old headaches MS Mets ADEM: Acute Disseminated Encephalomyelitis Monophasic but often variable enhancement Relatively little mass effect Deep grey (thalami) common May have hemorrhage Subcortical (ADEM) Vs calloseptal (MS) 19

20 11 year old lethargy hemiparesis 3 year old rapidly mental status ADEM ADEM 20

21 6 year old Bilat arm and leg numbness 5 year old drowsiness and seizure ADEM 3 year old level of conciousness 9 year old 9 days of L hemiparesis ADEM 21

22 ADEM: Which one is False? 1) It is a viral infection 2) It is more common in children than adults 3) Optic neuritis can be seen 4) Long-term f/u may be need to r/o MS 5) Both cerebral and cerebellar WM may be involved ADEM: Which is False? 1. Viral Infection 2. Children > Adults 3. Optic Neuritis 4. F/U imaging to R/O MS 5. Cerebral & Cerebellar WM 22

23 CPM: Central Pontine Myelinolysis Transverse pontine fibers most severely involved Vs corticospinal tract Extra PM in 50% >75% EtOH or hyponatremia correction DWI bright early, may enhance Variable resolution Spastic quadriparesis, pseudobulbar palsy CPM 23

24 CPM CPM: Which is NOT in DDX? 1) Pontine Infarct 2) Multiple sclerosis 3) Lyme Disease 4) Brainstem Glioma 5) Marchiafava-Bignami Disease 24

25 CPM: Which is not in DDx? 1. Pontine infarct 2. Multiple Sclerosis 3. Lyme Disease 4. Brainstem Glioma 5. Marchiafava- Bignami Disease? Diagnosis Normal Dina A. Jacobs et al. Neurology 2003;61:969 25

26 Brain stem disease: 1. CPM 2. Multiple System Atrophy 3. Wilson s Disease 4. ADEM 5. Olivary Pontine Degenerative Wilsons Normal 26

27 Wilson s Disease AKA: Hepatolenticular Degeneration Autosomal recessive: Copper accumulation Hand weakness, dysarthria, pseudo- Parkinsons High T2: Lentiform nuclei and midbrain Red nucleus and substania nigra surrounded by high T2 signal Midbrain: Panda sign Wilson s Gourmet 27

28 Wilson s? Diagnosis Normal 28

29 Brain stem disease: 1. CPM 2. Multiple System Atrophy 3. Huntington s 4. ADEM 5. Progressive supranuclear palsy Multisystem Atrophy Normal 29

30 Multisystem Atrophy (MSA) Sporadic neurodegenerative disease Synucleinopathy: alpha-synuclein metabolism Autonomic (Shy-Drager), Striaonigral, Olivopontocerebellar High T2: pontocerebellar tracts Pons: Hot Cross Bun sign Multisystem Atrophy Hot cross bun 30

31 ? Diagnosis Normal Brain stem disease: 1. CPM 2. Multiple System Atrophy 3. Huntington s 4. Progressive supranuclear palsy 5. Olivary Pontine Degenerative 31

32 Progressive supranuclear palsy Normal Progressive supranuclear palsy Hummingbird sign 32

33 Progressive Supranuclear Palsy (PSP) AKA: Steele-Richardson-Olszewski syndrome Sporadic neurodegenerative disease Cognitive decline, abnormal eye movements Midbrain atrophy ( Hummingbird sign ) High T2: Pontine tegmentum, midbrain tectum Reversible Post Leukoencephalopathy Syndrome (RPLS or PRES) Most often caused by abrupt changes in blood pressure, seizures, or certain immunosuppressive medications. Vasogenic edema predom in WM related to loss of autoregulatory ability Overall prognosis is good. DWI negative (Vs acute infarct cytotoxic edema) Usually in occipital, parietal, and temporal areas 33

34 48 year old post seizure 2 months later PRES Hypertensive Encephalopathy 34

35 Hypertensive Encephalopathy 58 yo cardiac Tx w Seizure Which one is PRES? 45 yo w impaired vision

36 Which one is PRES? yo cardiac Tx w Seizure 45 yo w impaired vision PRES PML 36

37 PML: Progressive Multifocal Leukoencephalopathy JC papovavirus Most commonly HIV or other Immunocompromised Classically: WM geographic lesions without enhancement or mass effect Formerly endstage HIV w poor prognosis PML 37

38 PML PML 38

39 PML: Which is FALSE? 1) It is caused by the JC virus 2) It is more common in males than females 3) CT frequently shows ringlike enhancement 4) Predilection for the parietal and occipital lobes 5) The spinal cord is rarely involved PML: Which is False? 1. JC Virus 2. Males > Females 3. Ring Enhances 4. Parietal/Occipital 5. Spinal Cord rarely 39

40 Signs: Tigroid WM: Metachromatic leukodystrophy Eye of the Tiger: Hallervorden-Spatz (PKAN) Panda sign: Wilsons Hot Cross Bun: Multiple system atrophy Hummingbird Sign: Progressive Supranuclear palsy Metachromatic leukodystrophy (MLD). Tigroid WM Cheon J et al. Radiographics 2002;22: by Radiological Society of North America 40

41 Hallervorden-Spatz Pantothenate kinase-associated neurodegeneration (PKAN) Guillerman R P Radiology 2000;217: by Radiological Society of North America Progressive supranuclear palsy Hummingbird sign 41

42 Wilson s Panda Multisystem Atrophy Hot cross bun 42

43 ?? Duck face sign CPM?? Crocodile sign 43