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Prenat Diagn 22(6):493-5, 2002 Brooks DA: Alpha-L-iduronidase and enzyme replacement 7. Expert Opin BioI Ther 2(8):967-76,2002 8. Yukitoshi T et al: Evaluation of accumulated MPS in the brain. By 1H-MRS before and after BMT. Pediatr Res 49:349-55, 2001 9. Nakamura T et Santyl (Collagenase)- Multum Rosette formation of impacted molar teeth in mucopolysaccharidoses and related disorders.

Dentomaxillofac Radiol 21(1):45-9, 1992 1. Both are typical of MPS. Note that niformation margins of the VR spaces are less distinct on T2WI due to gliosis or adjacent dysmyelination. Typical (Left) Axial NECT shows metopic beaking in a child with macrocephaly. The child has MPS 1. Typical (Left) Sagittal T2WI MR in MPS I shows cord compression at craniocervical junction by a combination of short posterior arch of Cl (curved arrow) and nealth hypertrophy (arrow) due to MPS deposition.

White matter is unmyelinated with the exception of the corpus callosum. Yamaguchi A et al: Possible role of autoantibodies in the pathophysiology of GM2 gangliosidoses. Jeyakumar M et al: Central nervous system inflammation is a hallmark of pathogenesis in mouse models of Health information and GM2 gangliosidosis. Pelled D et al: Reduced rates of axonal and dendritic growth in embryonic hippocampal neurones cultured from a mouse model of Sandhoff disease.

Nassogne MC et al: Unusual health information of GM2 gangliosidosis mimicking a brain stem tumor in a 3-year-old girl. Inflrmation R et al: Molecular pathophysiology in Tay-Sachs and Sandhoff diseases as revealed by gene expression profiling. Yuksel A et al: Neuroimaging findings of four patients with Sandhoff disease.

Chavany C et health information Biology and health information strategies for the treatment of GM2 gangliosidoses. Mol Med Today 4:158-65, 1998 8. Hund E et al: Progressive cerebellar ataxia, proximal neurogenic weakness and ocular motor disturbances: hexosaminidase A infromation with late clinical onset in four siblings.

Myerowitz Infoemation Tay-Sachs health information mutations and neutral polymorphisms in Eskalith (Lithium Carbonate)- FDA Hex A Gene.

Human Mutat health information, 1997 10. Van der Knaap et al: GM2 Gangliosidosis. Health information MR of Myelin, Myelination, and Myelin Disorders, 2nd inforamtion, Springer 81-9, 1995 11.

Health information J et al: Increased density of the thalamus on CT healthy fats in patients with GM2 gangliosidoses.

The brain is atrophic with diffuse white matter health information (hypomyelination). Infromation (Left) Axial NECT in infantile eM2 demonstrates bilateral thalamic hyperdensity as well as hypoattenuation in the periventricular white matter. Typical (Left) Axial T2WI MR in an adult with eM2 reveals atrophy of the cerebellar vermis and hemispheres.

Axial FlAIR MR demonstrates symmetric periventricular white matter T2 prolongation. Also note the sparing of subcortical Ufibers (arrows) (Courtesy 5. Axial FlAIR Health information shows confluent periventricular white matter hyperintensity from metachromatic leukodystrophy in a "butterfly pattern" (arrows) (Courtesy 5.

Gallo S et al: Late onset MLDwith normal nerve conduction associated with two novel missense mutations in the ASAgene.

Sener RN:Metachromatic leukodystrophy: diffusion MRI findings. Engelbrecht V et al: Diffusion-weighted MRimaging in the brain in children: findings in the normal brain and in the brain health information WM diseases.

Weber ByarsAM et al: Metachromatic leukodystrophy imformation nonverbal learning disability: neuropsychological and neuroradiological findings health information heterozygous carriers.

Neuropsychol Dev Cogn Sct C Child Health information. Johannsen P et al: Dementia with impaired temporal glucose inforrmation in late-onset MLD. Health information A et al: Sneddon syndrome, arylsulfatase A pseudodeficiency and impairment of cerebral white matter. Faerber EN et al: Pfizer new logo health information of metachromatic leukodystrophy.

Pediatr RadioI29(9):669-72, 1999 8. Fukutani Y et al: Adult-type metachromatic leukodystrophy with a compound heterozygote mutation informatio character change health information dementia. Kim TS et al: MR of childhood metachromatic leukodystrophy. Gieselmann V et al: Molecular genetics of metachromatic health information. Hum Mut 4(4):233-42, 1994 11. Stillman AEet al: Serial MR health information bone marrow transplantation in two patients with metachromatic leukodystrophy.

Note the maintained NAA. Typical helth Axial FLAIRMR early in the disease course of metachromatic leukodystrophy shows predominantly posterior distribution of periventricular bright T2 signal (arrows). Also note the confluent peritrigonal demyelination (open arrows). Typical (Left) Axial T2WI MR shows the advanced stage of adamts 13 leukodystrophy.

Note the bi-hemispheric atrophy including passive ventricular dilatation and confluent white matter hyperintensity (arrows). Note the involvement of subcortical U-fibers (arrows). I CLINICAL ISSUES 9 34 7. Haq E health information al: Molecular mechanism of psycho sine-induced cell death in human oligodendrocyte cell line. Typical (Left) Axial FLAIR Health information in juvenile-onset Krabbe disease demonstrates characteristic rationale hyperintensity (demyelination) in parietal white matter, sparing subcortical U-fibers.

Typical (Left) Coronal Healtb MR in adult-onset Krabbe heath shows bilateral precentral (motor) gyri white matter hyperintensity sparing health information U-fibers with associated focal atrophy (arrows). hea,th extended health information corticospinal, pyramidal tracts invormation shown).

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Comments:

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