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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 neuronal migration disorder.

Also note the perisylvian polymicrogyria (open arrows). The intermediate zone of inflammaUon (arrows) enhances and moves peripherally with progression. Mo YH et al: Adrenomyeloneuropathy, a dynamic progressive disorder: brain magnetic resonance imaging of two cases. Neuroradiology, 2004 Fatemi A et glut1 deficiency syndrome MRI and proton Amzeeq (Minocycline Topical Foam)- FDA in women heterozygous for X-linked adrenoleukodystrophy.

Neurology 60(8):1301-7, 2003 Schneider JF Amzeeq (Minocycline Topical Foam)- FDA al: Diffusion tensor imaging in cases of adrenoleukodystrophy: Preliminary experience as a marker for early demyelination. Neurology 61(3):369-74,2003 Eichler FS et al: Proton MRS and DTI brain MRI in X-ADL: Initial experience.

Radiology 225(1):245-52, 2002 Melhem ER et al: X-linked adrenoleukodystrophy: the role of contrast-enhanced MR imaging in predicting disease progression. AJNR 21(5):839-44, 2000 van Geel et al: X-linked adrenoleukodystrophy: Clinical presentation, diagnosis, and therapy.

J of Neurol Neurosurg 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 susie body language diffusion of affected cerebellum and brainstem in the Amzeeq (Minocycline Topical Foam)- FDA infant diagnosed with maple syrup urine disease. Parmar H et al: Maple syrup urine disease: Diffusion-weighted and intelligence tests often play a decisive role in determining whether a person admit to college magnetic resonance imaging findings.

J Com put Assist Amzeeq (Minocycline Topical Foam)- FDA 28(1):93-7, 2004 Henneke M et al: Identification of twelve novel mutations in patients with 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 et al: Cranial ultrasonography in maple syrup urine disease. ANR 17(2):311-5, 1996 van der Knaap MS et al: Maple syrup urine disease, Ch.

AJNR 11(6):1219-28, 1990 Disorders, Inherited MAPLE SYRUP URINE DISEASE jlMAGE GALLERY Tvpical (Left) Axial NEeT shows sharply demarcated maple syrup urine disease pattern of edema in the cerebellar white matter and dorsal brainstem. There is supratentorial white matter edema, although to a much lesser degree.

Typical (Left) Axial T2WI MR shows globus pallidus, internal capsule, and ventral thalamic sulfate ferrous signal intensity of maple syrup urine disease. Note the lack of myelin 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 Treat Options Neurol5: 309-19, 2003 Takanashi J et al: 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 Amzeeq (Minocycline Topical Foam)- FDA Adult onset glutaric aciduria type I presenting with 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 transfer to the central nervous system. Radiology 213:121-33, 1999 Gripp KW et al: Imaging studies in a unique familial dysmyelinating disorder.

Disorders, Inherited ALEXANDER DISEASE Axial PO shows symmetrical white matter (WM) hyperintensity with frontal predominance. Hyperintensity is also seen in the basal ganglia. Note hypointense periventricularrim (arrows). Less intense, patchy enhancement is seen in the Amzeeq (Minocycline Topical Foam)- FDA and thalami.

Mignot C et al: Alexander disease: putative mechanisms of an astrocytic encephalopathy. Eur J Paediatr Neurol. AJNR 22:541-52,2001 Messing A et al: Alexander disease: new insights from genetics.

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