White matterds approach


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White matterds approach

  1. 1. Predominately Subcortical White Matter (early involvement of U-fibers) Macrocephalic Canavan Disease: Alexander Disease: Ashkenazi Jews, hypotonia (head lag), seizures, spasticity Seizures, developmental delay, spasticity WM: NEAR-COMPLETE, subcortical/deep WM: ANTERIOR-PREDOMINANT, subcortical/deep/periventricular GM: thalami, globi pallidi GM: basal ganglia MRS: ↑NAA peak ENHANCEMENT Van der Knaap Disease (Megaloencephalic Leukoencephalopathy with Cysts): Macrocephaly, seizures, ataxia, spasticity WM: Subcortical with central sparing DWI: Increased proton movement ANTERIOR & FRONTOPARIETAL SUBCORTICAL CYSTS Zellweger (Cerebrohepatorenal) Syndrome: Facial dysmorphism, mental retardation, hypotonia, hepatic dysfunction/jaundice, small renal cortical cysts WM: subcortical/deep (internal capsule) Normocephalic Vanishing White Matter Disease: Initially normal motor function with relapsing/remitting but progressive ataxia/spasticity WM: subcortical, deep, & periventricular; ISOINTENSE TO CSF Galactosemia: Kearns-Sayre Disease: Homocystinuria: Pelizaeus-Merzbacher disease: Vomiting, ↑intracranial pressure, hepatic dysfunction, hepatomegaly MITOCHONDRIAL INHERITANCE Marfanoid body habitus, osteoporosis, mental retardation Spasticity, abnormal eye/extrapyramidal movements, mental retardation WM: Subcortical & deep (anterior limb internal capslule WM: NEAR-COMPLETE subcortical & deep with “TIGROID or LEOPARD” appearance due to sparing of perivascular spaces WM: diffuse subcortical/periventricular ASSOCIATED LIVER DISEASE OPHTHALMOPLEGIA WM: subcortical with deep sparing GM: globi pallidi/thalami/dorsal medulla GM: globi pallidi DWI: restricted diffusion MRS: ↑lipid peak MRS: ↑Lactate peak/↓NAA peak POLYMICROGYRIA/PACHYGYRIA, SUBEPENDYMAL GERMINOLYTIC CYSTS IN CAUDOTHALAMIC GROOVE BASAL GANGLIA/THALAMIC CA++ GM: basal ganglia spared BILATERAL INFERIOR (vs superior in Marfan’s) LENS DISLOCATION (ECTOPIA LENTIS); INFARCTS
  2. 2. Predominately Deep White Matter (early sparing of U-fibers) Thalamic (Gray Matter) Involvement Krabbe’s Disease: 3-6 months, hypertonia, irritability WM: deep cerebral & cerebellar GM: thalami, basal ganglia, cerebellar dentate nuclei HYPERDENSE THALAMI No Thalamic Involvement Gangliosidosis GM1 & GM2 (Tay-Sachs & Sandhoff): Cherry-red macula, hypotonia, mental retardation, seizures, spasticity WM: periventricular Brainstem (Corticospinal Tract) Involvement No Brainstem (Corticospinal Tract) Involvement HYPERDENSE THALAMI X-linked Adrenoleukodystrophy: Maple Syrup Urine Disease: Metachromatic Leukodystrophy: Adolescent ♂, learning difficulty (ADHD), skin hyperpigmentation, adrenal failure, ataxia, seizures Neonate (< 1 wk) with vomiting, dystonia, & seizures 1-1.5 yrs, ↓motor/mentation WM: POSTERIOR PERITRIGONAL WITH LEADING EDGE OF ENHANCEMENT, SYMMETRIC, pons/medulla involved GM: globi pallidi WM: deep cerebellar, cerebral peduncle, globi pallidi, & dorsal pons DWI: RESTRICTION OF DIFFUSION WM: symmetric deep/periventricular with “TIGROID or LEOPARD” appearance due to sparing of perivascular spaces; POSTERIOR PREDOMINANCE; NO ENHANCEMENT Mucopolysaccharidoses (Hurler’s, Hunter’s, Morquio’s): Other: Macrocephalic, thick meninges Phenylketonuria WM: RADIATING PERIVENTRICULAR & CORPUS CALLOSAL CYSTIC AREAS (dilated perivascular spaces) Merosin-deficient m. dystrophy Lowe (Oculocerebrorenal) Disease
  3. 3. Predominately Gray Matter Deep Gray Matter Leukodystrophy Mimics Cortical Gray Matter Leigh Disease: MELAS: Other: Neuronal ceroid lipofuscinoses: MITOCHONDRIAL MITOCHONDRIAL Organic Acidopathies Respiratory failure, ataxia, visual/auditory problems, weakness Myopathy, Encephalopathy, Lactic Acidosis, Strokes Amino acid metabolic disorders Vision failure, progressive dementia, seizures BILATERAL SYMMETRIC T2/FLAIR HYPERINTENSITY & RESTRICTION OF DIFFUSION IN PUTAMINA Inflammatory Processes White Matter Injury Cerebral/cerebellar atrophy, thalami/globi pallidi involvement WM: subcortical, brainstem, deep cerebellum GM: basal ganglia Mucolipidosis type I: MIGRATING INFARCTS MC IN PARIETOOCCIPITAL CORTEX Neonatal (< 1 wk) presentation Subacute Sclerosing Panencephalitis: Acute Disseminated Encephalomyelitis: MEASLES INFECTION Multifocal punctate to large flocculent T2/FLAIR hyperintesnse WM/basal ganglia lesions, which may enhance Progressive Multifocal Leukoencephalopathy: JC PAPOVAVIRUS INFECTION 10-14 DAYS FOLLOWING VIRAL ILLNESS OR IMMUNIZATION Lyme Disease: Radiation Injury: Imaging findings simulate multiple sclerosis in a patient with skin rash, flu-like symptoms, &/or joint pain WM: sparing of subcortical Ufibers; scalloped margins DISTRIBUTION RELATES TO XRT FIELD (unless whole-brain XRT) 6-8 MONTHS FOLLOWING XRT TORCH: Microcephaly, variable (typically asymmetric) WM demyelination &/or gliosis, periventricular Ca++, subependymal cysts Periventricular Leukomalacia (WM Injury of Prematurity): Spastic diplegia, visual/cognitive impairment, associated with PROM/TORCH Mechanism: ischemic injury to watershed area WM: deep/periventricular with cystic change & volume loss Thin corpus callosum, undulating ventricular margin, enlarged ventricles
  4. 4. Axial T2WI in a child with Canavan disease shows periventricular, deep, and subcortical WM involvement, plus thalami (white arrow) and globi pallidi (white curved) involvement; very characteristic.
  5. 5. X-linked adrenoleukodystrophy with preferential involvement of descending pyramidal tract A-C. T2-weighted image shows demyelination of internal capsule, descending pyramidal tract (arrows, A) and cerebellar deep white matter (arrows, B). The peritrigonal white matter is relatively spared(C). D. On gadolinium-enhanced T1-weighted image, enhancing bilateral descending pyramidal tracts (arrows) are shown.
  6. 6. Coronal T2WI MR in another case of metachromatic leukodystrophy shows characteristic diffuse deep and periventricular white matter involvement, with sparing of the cerebellar white matter (white arrow). Coronal FLAIR MR shows bilateral and symmetric periventricular and deep white matter signal abnormality but sparing of sub-cortical U-fibers (white arrow) in child with metachromatic leukodystrophy.
  7. 7. Axial T2WI shows diffuse high signal in the centrum semiovale with sparing of the subcortical Ufibers, typical of treatment related leukoencephalopathy. Patient is s/p whole brain XRT.