Hypo: inc T2, nl T1 Dys/Demye: inc T2, decr T1

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Presentation transcript:

Hypo: inc T2, nl T1 Dys/Demye: inc T2, decr T1 Leukodystrophy 2013 Hypo: inc T2, nl T1 Dys/Demye: inc T2, decr T1

Confluent Within confluent: Diffuse (can be end stage of everything, so what’s diffuse in the beginning MLC (megalencencephaluc with subcortical cysts) Vanishing White matter disease (YOU have to have low signal on FLAIR) LAMA2: Frontal Classic is Alexanders Often has contrast

Parietal - occital ALD – onset in childhood Has contrast enhancement TEMPORAL LOBE Aicardiai Syndrome - calcifications Cogenital CMV

Leukodystrophy MRI T2 hyperintensity abnormality needed for dx Findings on other sequences needed (T1 and FLAIR) Pattern varies according to dx

White matter maturation What you seen at maturity is noted by 8 mo on T1 What you see at maturity is noted by 24 mo on T2 Schiffman and der Knaap Neurology 2009

MRI technique to ddx White matter T2 hyperintensive with T1 isointense = Hypomyelination (T1 looks like young infant pattern) PMP (PLP-1) PMD-like GJA12 Salla disease T2 hyperintense with T1 hypointense = demyelination X linked ALD ABCD1 MLD LBSL Many others

4H Syndrome Certain patterns of hypomyelination Steemweg Nraom 2010133:2971

False hypomyelination occurs in certain phases Some delayed myelination plus multifocal lesions can occur Hypomyelination is NOT delayed myelination Vaurs-Barriere et al. 2009

Hyperintense and hypointense T1 Confluent vs. multifocal Patterns Diffuse Periventricular predominance Subcortical Large assymmetric Cerebellar

Multifocal can be progressive or stataic Static 18q minus syndrome Sjogren Larsson RNAase T2 deficient Cogenital CMV

Multifocal PML Brucellosis Vasculopathies (Cadasil, Fabry, susac) Mitochondrial LBSL Callagen IV A1 defect

Frontal predominance Infantile Alexander Infantile MLD

Brainstem involvement/Cerebellum Alexander or LBSL Leigh Wilson Peroxisomal

Special MRI features Cystic white matter degeneration CACH/VWM Mitochondrail Enlarged perivascular Mucopolysaccharidoses Other chromosomal abnormaliteis Lowe syndrome

Special features indicate specific entities Cortical dysplasia Cortical lesions Basal ganglia Contrast enhancement MS, infection, ADEM,vasulitis, malignace Mitochondrial disorders X-ALD Leurkoencephalopathy with califications and cysts LCC

Calcifications Cockayne Syndrome

Cortical L-2 hydroxyglutaric aciduria Canavans Kearns-Sayre

Periventricualr MLD (beware MS) Adult polyglucosan body disease (always thin cord) Krabbe LBSL Later onset neuronal degneral (neuronal ceroid lipfuscinosis)

Patterns Diffuse from the beginning Micro-bleeds CACH/VMM Acquired and genetic vasculopathy Collagen IV A1 defect, amyloid,

Periventricular MLD and Krabbe MLD has involvement of corpus callosum Krabbe more likely has atrophy

Mitochondrial disorders Frequently the dendate are involved

Large assymemetric lesions HDLS: hereditary diffuse leukoencephalopathy with spheroids – MARKED FRONTAL ATROPHY CRMSS

BRAIN STEM Predomindance Peroxismal disorders LBSL – entire cord and brainstem Adult polyglucosan body disease CEREBELLUM CTX ADLD

Multifocal Progressive vs. static vs. prominent perivascular spaces Lowe – enlarged perivascular spaces “PTEN” autism and microcephalphy

Delayed myelination- can be neuronal disorders SOX10 related disorders MCT8 related disorders Other neuronal disorders The white matter improves but the patient does not!