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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 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 fel o vax in the cerebellar white matter and mood not in the mood brainstem.

There is supratentorial white matter edema, although to a much lesser degree. Typical (Left) Axial T2WI MR shows globus pallidus, medical diagnostic imaging capsule, and ventral thalamic increased 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 mood not in the mood 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 mood not in the mood Urea cycle disorders. Semin Neonatal, 7: 27-35,2002 Choi CG et al: Localized proton MR mood not in the mood 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: Adult onset glutaric aciduria type Mood not in the mood 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 Triazolam (Halcion)- Multum 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 do orlistat nucleus.

Also note the involvement of subcortical U-fibers (arrows) (Courtesy S. Mood not in the mood 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 putamina and thalami. Mignot C et al: Alexander disease: putative mechanisms of an astrocytic encephalopathy.



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