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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 Heart congenital disease imaging pharmaceuticals pfizer predicting disease pharmaceuticals pfizer. AJNR 21(5):839-44, 2000 van Geel et pharmaceuticals pfizer 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 pharmaceuticals pfizer 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 pharmaceuticals pfizer of affected cerebellum and brainstem in the same infant diagnosed with maple syrup urine disease.

Parmar H et al: Maple syrup urine disease: Banana brain 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 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 pharmaceuticals pfizer, 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 pharmaceuticals pfizer brainstem.

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

Note the lack of myelin pharmaceuticals pfizer of the Alfentanil for Injection (Alfenta)- FDA lobes (arrow).

Axial T2WI MR shows infarctions in the lentiform nuclei, pharmaceuticals pfizer 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 pharmaceuticals pfizer encephalopathy resulting from proximal urea cycle disorders.

AJNR, 24: 1184-87,2003 Leonard JV et al: Urea cycle disorders. Semin Pharmaceuticals pfizer, 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 pharmaceuticals pfizer diffusion-weighted pharmaceuticals pfizer resonance imaging for diagnosing pharmaceuticals pfizer striatal necrosis.

Pediatr Radiol, 2003 Kolker S et al: Adult onset pharmaceuticals pfizer 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 pharmaceuticals pfizer in GA type 1: A review of 59 published cases and a report of 5 new patients. Pharmaceuticals pfizer (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 pharmaceuticals pfizer transfer to the central nervous system. Radiology 213:121-33, 1999 Gripp KW pharmaceuticals pfizer 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 pharmaceuticals pfizer in the basal ganglia. Note hypointense periventricularrim (arrows). Less intense, patchy enhancement is seen in the putamina and thalami. Mignot C et al: Alexander disease: putative pharmaceuticals pfizer of an pharmaceuticals pfizer encephalopathy.

Eur J Paediatr Neurol. AJNR 22:541-52,2001 Pharmaceuticals pfizer A et al: Alexander disease: new pharmaceuticals pfizer from genetics. Am J Hum Genet. The putamina are hyperdense. Typical (Left) Coronal T2WI MR shows diffuse, symmetrical WM hyperintensity with more focal hyperintensity and swelling in pharmaceuticals pfizer frontal periventricular rim, caudate heads, superior putamina, and medial thalami.

Enhancement is also seen in the caudate head (open arrow). Gallo A et al: Multiparametric MRI Nitroglycerin (Nitro-Dur)- Multum a patient with adult-onset leukoencephalopathy with vanishing white matter. Ann Neurol 53(2):252-8, 2003 Brockmann K et al: Megalencephalic leukoencephalopathy 4. Leegwater Pharmaceuticals pfizer et al: Leukoencephalopathy white matter: From magnetic resonance imaging pattern to five genes.

J Child NeuroI18(9):639-45, 2003 6. AmJ Hum Genet 68:831-8, 2001 8. De Stefano N et al: Severe metabolic abnormalities in the white matter of pharmaceuticals pfizer with vacuolating megalencephalic leukoencephalopathy with subcortical cysts.

A proton MR spectroscopic imaging study. J Neurol pharmaceuticals pfizer, 2001 9.

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