Abstract
Huntington’s disease (HD) is a hereditary autosomally-dominant transmitted neurodegenerative disease. Clinically, HD is characterized by a hyperkinetic movement disorder, the so-called chorea, and progressive cognitive deterioration, i.e., dementia. Furthermore, HD may also lead to psychiatric symptoms such as depression, delinquency, alcoholism, or schizophreniform psychosis. These manifestations can precede the onset of chorea by several years and represent a considerable burden to the patient and his family. Woody Guthrie, the famous American musician, was affected by HD; the fatal consequences of this disease on his life and that of his family have been described in his biographies (Klein 1980).
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References
Andrews TC, Weeks RA, Turjanski N et al (1999) Huntington’s disease progression. PET and clinical observations. Brain 122:2353–2363
Araujo DM, Cheney SR, Tatsukawa KJ et al (2000) Deficits in striatal dopamine D-2 receptors and energy metabolism detected by in vivo MicroPET imaging in a rat model of Huntington’s disease. Exp Neurol 166:287–297
Aylward EH, Brandt J, Codori AM et al (1994) Reduced basal ganglia volume associated with the gene of Huntington’s disease in asymptomatic at-risk persons. Neurology 44:823–828
Beal MF (2000) Energetics in the pathogenesis of neurode-generative diseases. Trends Neurosci 23:298–304
Beal MF, Hantraye P (2001) Novel therapies in the search for a cure for Huntington’s disease. Proc Natl Acad Sci USA 98:3–4
Burke JR, Enghild JJ, Martin ME et al (1996) Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. Nat Med 2:347–350
Dezutter NA, Dom RJ, de Groot TJ et al (1999) 99mTc-MAMA-chrysamine G, a probe for beta-amyloid protein of Alzheimer’s disease. Eur J Nucl Med 26:1392–1399
Grafton ST, Maziotta JC, Pahl JJ et al (1990) A comparison of neurological, metabolic, structural, and genetic evaluation in persons at risk of Huntingtons disease. Ann Neurol 28:614–621
Hayden MR, Martin WR, Stoessl AJ et al (1986) Positron emission tomography in the early diagnosis of Huntington’s disease. Neurology 36:888–894
Huntington G (1872) On chorea. Med Surg Rep 26:317–321
Klein J (1980) Woody Guthrie: a life. Ballantine Books, New York
Koroshetz WJ, Jenkins BG, Rosen BR et al (1997) Energy metabolism defects in Huntington’s disease and effects of coenzyme Q10. Ann Neurol 41:160–165
Kuhl DE, Phelps ME, Markham CH et al (1982) Cerebral metabolism and atrophy in Huntington’s disease determined by 18FDG and computed tomographic scan. Ann Neurol 12:425–434
Kunig G, Leenders KL, Sanchez-Pernaute R et al (2000) Benzodiazepine receptor binding in Huntington’s disease: [HC]flumazenil uptake measured using positron emission tomography. Ann Neurol 47:644–648
Kuwert T, Lange HW, Langen KJ et al (1990) Cortical and subcortical glucose consumption measured by PET in patients with Huntington’s disease. Brain 113:1405–1423
Kuwert T, Lange HW, Boecker H et al (1993) Striatal glucose consumption in chorea-free subjects at risk of Huntington’s disease. J Neurol 241:31–36
Kuwert T, Bartenstein P, Grünwald F et al (1998) Clinical significance of positron emission tomography in neuro-medicine. A position paper on the results of an interdisciplinary consensus conference. Nervenarzt 69:1045–1060
Lange HW (1976) Morphometric studies of the neuropathological changes in choreatic diseases. J Neurol Sci 28:401–425
Leenders KL, Frackowiack RS, Quinn N et al (1986) Brain energy metabolism and dopaminergic function in Huntington’s disease measured in vivo using positron emission tomography. Mov Dis 1:69–77
Ludolph AC, Seelig M, Ludolph AG et al (1992) ATP deficits and neuronal degeneration induced by 3-Nitropropionic acid. Ann NY Acad Sci 11:300–302
Mazziotta JC, Phelps ME, Pahl JJ et al (1987) Reduced cerebral glucose metabolism in asymptomatic subjects at risk for Huntington’s disease. N Engl J Med 316:357–362
McGowan DP, van Roon-Mom W, Holloway H et al (2000) Amyloid-like inclusions in Huntington’s disease. Neuroscience 100:677–680
Paulson HL, Fishbeck KH (1996) Trinucleotide repeats in neurogenetic disorders. Annu Rev Neurosci 19:79–107
Phelps ME (2000) PET: the merging of biology and imaging into molecular imaging. J Nucl Med 41:661–681
Schlaug A, Hefter H, Engelbrecht V et al (1996) Neurological impairment and recovery in Wilson’s disease: evidence from PET and MRI. J Neurol Sci 136:129–139
Tabrizi SJ, Cleeter MW, Xuereb J et al (1999) Biochemical abnormalities and excitotoxicity in Huntington’s disease brain. Ann Neurol 45:25–32
The Huntington’s Disease Collaborative Research Group (1993) A novel gene containing a trinudeotide repeat that is expanded and unstable on Huntington’s disease chromosomes. Cell 72:971–983
Tobin AJ, Signer ER (2000) Huntington’s disease: the challenge for cell biologists. Trends Cell Biol 10:531–536
Trottier Y, Bianclana V, Mandel JL et al (1994) Instability of CAG repeats in Huntington’s disease: relation to parental transmission and age of onset. J Med Genet 31:377–382
Vonsattel JP, Myers RH, Stevens TJ et al (1985) Neuropathological classification of Huntington’s disease. J Neuropathol Exp Neurol 44:559–577
Wexler A (1996) Mapping fate—a memoir of family, risk, and genetic research. Random House, Times Books, New York
Zeron MM, Chen N, Moshaver A et al (2001) Mutant huntingtin enhances excitotoxic cell death. Mol Cell Neurosci 17:41–53
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Kuwert, T. (2003). Imaging of Cerebral Metabolism in Huntington’s Disease. In: Feinendegen, L.E., Shreeve, W.W., Eckelman, W.C., Bahk, YW., Wagner, H.N. (eds) Molecular Nuclear Medicine. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-55539-8_19
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DOI: https://doi.org/10.1007/978-3-642-55539-8_19
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