Essay: White daisies on my mind (Requiem for an Alzheimer patient)C G Janson
UMDNJ-Robert Wood Johnson Medical School, Camden, NJ 08103, USA
Neurology 65:654-6. 2005
Natural history of Canavan disease revealed by proton magnetic resonance spectroscopy (1H-MRS) and diffusion-weighted MRIC G Janson
UMDNJ Robert Wood Johnson Medical School, Dept of Neurosurgery, Camden, NJ 08103, USA
Neuropediatrics 37:209-21. 2006
..The resultant model can be used to non-invasively monitor the natural history of Canavan disease or related leukodystrophies in future studies involving drug, gene therapy, or stem cell treatments...
Immortalization and functional characterization of rat arachnoid cell linesC Janson
Department of Neurosurgery, University of Minnesota, Minneapolis, MN 55455, USA
Neuroscience 177:23-34. 2011
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Bowstringing as a complication of deep brain stimulation: case reportChristopher Janson
Department of Neurosurgery, University of Minnesota, Minneapolis, Minnesota 55455, USA
Neurosurgery 66:E1205; discussion E1205. 2010
..There are no previous reports of this specific complication, which presumably has been more broadly classified under hardware-related complications...
Mild-onset presentation of Canavan's disease associated with novel G212A point mutation in aspartoacylase geneChristopher G Janson
Department of Neurology, University of Medicine and Dentistry of New Jersey Robert Wood Johnson Medical School, Camden, NJ, USA
Ann Neurol 59:428-31. 2006
..Future studies of ASPA enzyme structure and gene regulation in these subjects could lead to a better understanding of Canavan's pathophysiology and improvements in ASPA gene therapy...
Lithium citrate reduces excessive intra-cerebral N-acetyl aspartate in Canavan diseaseMitra Assadi
Robert Wood Johnson Medical School, Neurology, 3 Cooper Plaza, Suite 320, Camden, NJ 08103, USA
Eur J Paediatr Neurol 14:354-9. 2010
..Diffusion Tensor Imaging was available in two patients and suggested micro-structural improvement in the corpus callosum. The results suggest that lithium administration may be beneficial in patients with Canavan disease...
Serial 1H-MRS in GM2 gangliosidosesMitra Assadi
Division of Neurology, University of Medicine and Dentistry of New Jersey, 3 Cooper Plaza, Suite 320, Camden, NJ 08103, USA
Eur J Pediatr 167:347-52. 2008
..1H-MRS may be a valuable tool in monitoring the disease progress and response to therapy in GM2 gangliosidoses. Elevation in the mI may prove to be more sensitive than the other metabolite alterations...
Effects of AAV-2-mediated aspartoacylase gene transfer in the tremor rat model of Canavan diseaseS W J McPhee
Cell and Gene Therapy Center, UMDNJ Robert Wood Johnson Medical School, 401 Haddon Avenue, Suite 395, Camden, NJ 08103, USA
Brain Res Mol Brain Res 135:112-21. 2005
..This study provides evidence that AAV-2-mediated aspartoacylase gene transfer to the brain improves biochemical and behavioral deficits in tremor rat mutants (tm/tm) and supports the rationale of human gene transfer for Canavan disease...
Global CNS gene transfer for a childhood neurogenetic enzyme deficiency: Canavan diseaseP Leone
CNS Gene Therapy Center, Department of Neurosurgery, Thomas Jefferson University, Philadelphia, PA 19107, USA
Curr Opin Mol Ther 1:487-92. 1999
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Immune responses to AAV in a phase I study for Canavan diseaseS W J McPhee
Department of Surgery, Division of Neurosurgery, Robert Wood Johnson Medical School, University of Medicine and Dentistry of New Jersey, 401 Haddon Avenue, Camden, NJ 08103, USA
J Gene Med 8:577-88. 2006
..Long-term monitoring of subjects and expansion to phase II/III will be necessary in order to make definitive statements on safety and efficacy...