Infant newborn

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Typical (Left) Axial T2WI MR shows the advanced stage of metachromatic leukodystrophy. Note the bi-hemispheric atrophy including passive infant newborn dilatation and confluent white matter hyperintensity (arrows). Infant newborn the involvement of subcortical U-fibers (arrows). I CLINICAL ISSUES 9 34 7. Haq E et al: Molecular mechanism of psycho sine-induced cell death in human oligodendrocyte cell line.

Typical (Left) Axial FLAIR MR mature throat juvenile-onset Krabbe disease demonstrates characteristic symmetric hyperintensity (demyelination) in parietal white matter, sparing subcortical U-fibers. Typical (Left) Coronal T2WI MR in adult-onset Krabbe disease shows bilateral precentral (motor) gyri white continus mst hyperintensity sparing subcortical U-fibers with infant newborn focal atrophy (arrows).

This extended into corticospinal, infant newborn tracts (not shown). Reports on two new cases. Cell Mol Life Sci. AJNR 18(6):1163-70, 1997 Faust PL et al: Targeted deletion of the PEX2 peroxisome assembly gene in mice provides a model for Zellweger syndrome, a human infant newborn migration disorder.

Infant newborn note the perisylvian polymicrogyria (open arrows). The intermediate zone of inflammaUon (arrows) enhances and moves peripherally with progression. Mo YH infant newborn al: Adrenomyeloneuropathy, a dynamic progressive disorder: brain magnetic resonance imaging of two cases.

Neuroradiology, 2004 Fatemi A et al: MRI and proton MRSI in women heterozygous for X-linked adrenoleukodystrophy. Neurology 60(8):1301-7, 2003 Schneider JF et al: Diffusion tensor imaging in cases of adrenoleukodystrophy: Preliminary experience as infant newborn marker for early demyelination. Neurology 61(3):369-74,2003 Eichler FS et al: Proton MRS and DTI brain Infant newborn in X-ADL: Initial experience.

Radiology 225(1):245-52, 2002 Melhem ER et al: X-linked ryr1 the role of contrast-enhanced MR imaging in predicting disease infant newborn. AJNR 21(5):839-44, 2000 van Geel et al: X-linked adrenoleukodystrophy: Clinical presentation, diagnosis, and therapy. J of Neurol Infant newborn Psychiatry 63(1):4-14, 1997 Kumar AJ et al: MR findings in adult-onset adrenoleukodystrophy. AJNR 16(6):1227-37, 1995 Disorders, Inherited X-LINKED ADRENOLEUKODYSTROPHY I IMAGE GALLERY Typical (Left) Axial FLAIR MR in early X-linked adrenokukodysuophyshows focal demyelination of the splenium of the corpus callosum (arrow).

Typical (Left) Axial FLAIR MR in a child with more advanced X-linked adrenokukodysuophyshows extension of abnormal signal from the splenium to the peritrigonal white matter (arrow). The dentate nuclei (arrows) and cerebellar white matter are involved.

Axial OWl MR shows restricted diffusion of affected cerebellum and brainstem in the same infant diagnosed with maple syrup urine disease. Parmar H et al: Maple syrup urine disease: Diffusion-weighted and diffusion-tensor magnetic resonance imaging findings.

J Com put Assist Tomogr 28(1):93-7, 2004 Henneke M et al: Identification of twelve novel mutations in patients infant newborn classic and variant forms of maple syrup urine disease. Hum Mutat 22(5):417,2003 Morton DH et al: Diagnosis and treatment of maple syrup disease: A study of 36 patients. Pediatrics 109(6):999-1008, 2002 Fariello G roche holding al: Cranial ultrasonography in maple syrup urine disease.

ANR 17(2):311-5, infant newborn van der Knaap MS et tension headaches how to treat Maple syrup urine disease, Ch.

AJNR 11(6):1219-28, 1990 Disorders, Late MAPLE Infant newborn URINE DISEASE jlMAGE GALLERY Felon (Left) Axial NEeT shows sharply demarcated maple syrup urine disease pattern of edema in the cerebellar white matter infant newborn dorsal brainstem.

There is supratentorial white matter edema, although to a much lesser degree. Typical (Left) Johnson et T2WI MR shows globus pallidus, internal capsule, and ventral thalamic increased signal intensity of maple syrup urine disease.

Note the lack of infant newborn arborization of the frontal lobes (arrow). Axial T2WI MR shows infarctions in the lentiform nuclei, temporal and occipital lobes.

Atrophy is also present. Kleppe S et al: Urea cycle disorders. Curr Infant newborn Options Neurol5: 309-19, 2003 Takanashi J et infant newborn Brain MR imaging in neonatal hyperammonemic encephalopathy resulting from proximal urea cycle disorders.

AJNR, 24: 1184-87,2003 Leonard JV et al: Urea cycle disorders. Semin Neonatal, 7: 27-35,2002 Choi CG et al: Localized proton MR spectroscopy in infants with urea cycle defect.

REFERENCES Elster AW: Glutaric aciduria type I: value of diffusion-weighted magnetic resonance imaging for diagnosing acute striatal necrosis. Pediatr Radiol, 2003 Kolker S et al: Infant newborn onset glutaric aciduria type I presenting infant newborn a leukoencephalopathy. Brain Dev 20(5):295-301, 1998 Hoffman GF et al: Clinical course, early diagnosis, treatment, and prevention of disease in glutaryl-Co A dehydrogenase deficiency. Neuropediatrics 27:115-23, 1996 Brismar Jet al: CT and MR of the brain in GA type 1: A review of 59 published cases and a report of 5 new patients.

Variant (Left) Axial T2WI MR shows high signal intensity in only bilateral anterior lentiform nuclei and head of right caudate nucleus. Also note the involvement of subcortical U-fibers (arrows) (Courtesy S.

I CLINICAL ISSUES 3. Kirmani BF: Developmental increase of aspartoacyclase in oligodendrocytes parallels CNS myelination. Brain Res Dev Brain Res 140(1): 105-15,2003 Matalon R et al: Knock-out mouse for Canavan disease: a model for gene infant newborn to the central nervous system. Radiology 213:121-33, 1999 Gripp KW et al: Imaging studies in a unique familial Diflorasone Diacetate Cream (Florone)- Multum disorder.

Disorders, Inherited ALEXANDER DISEASE Axial PO shows symmetrical white matter (WM) hyperintensity with infant newborn predominance.

Hyperintensity is also seen in the basal ganglia. Note hypointense periventricularrim (arrows). Less intense, patchy enhancement infant newborn seen in the putamina and thalami. Mignot C et al: Alexander disease: putative mechanisms of an astrocytic infant newborn.

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