Pathophysiology of idiopathic dystonia: Findings from genetic animal models

Progress in Neurobiology

Volumn 54, Issue 6, 1998, Pages 633-677

  Richter, Angelika ^a   Löscher, Wolfgang ^a  

^a UNIVERSITY OF VETERINARY MEDICINE HANNOVER   (Germany)

Author keywords

[No Author keywords available]

Indexed keywords

BASAL GANGLION; CHOREOATHETOSIS; DYSTONIA; GENETIC MODEL; IDIOPATHIC DISEASE; MOTOR DYSFUNCTION; NEUROPATHOLOGY; NONHUMAN; PATHOPHYSIOLOGY; PRIORITY JOURNAL; REVIEW; ANIMAL; BRAIN; DISEASE MODEL; ELECTROPHYSIOLOGY; GENETICS; HAMSTER; HUMAN; PATHOLOGY; RAT; RAT STRAIN; SPINAL CORD;

ANIMALS; BRAIN; CRICETINAE; DISEASE MODELS, ANIMAL; DYSTONIA; ELECTROPHYSIOLOGY; HUMANS; RATS; RATS, MUTANT STRAINS; SPINAL CORD;

EID: 0032040365     PISSN: 03010082     EISSN: None     Source Type: Journal    
DOI: 10.1016/S0301-0082(97)00089-0     Document Type: Article

References (256)

1. Adams, L. M. and Foote, S. L. (1988) Possible involvement of brain noradrenergic neurons in dystonia. In: Dystonia 2. Advances In Neurology. pp. 313-333, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
2. Adler, C. H., Wrabetz, L., Brin, M. F. and Hurtig, H. (1994) Cerebellar ataxia, dystonia, and tremor within a family: variable phenotypes of a single genetic disorder? Mov. Disord. 9, 155-160.
3. Afifi, A. K. (1994) Basal ganglia: Functional anatomy and physiology. Part 1. J. Child Neurol. 9, 249-260.
4. Albin, R. L., Young, A. B. and Penny, J. B. (1989) The functional anatomy of basal ganglia disorders. Trends Neurosci. 12, 366-375.
5. Angelini, L., Rumi, V., Lamperti, E. and Nardocci, N. (1988) Transient paroxysmal dystonia in infancy. Neuropediatrics 19, 171-174.
6. Aston-Jones, G., Shipley, M. T. and Grzanna, R. (1995) The locus coeruleus, A5 and A7 noradrenergic cell groups. In: The rat nervous system. 2nd ed., Ed. G. Paxinos. pp. 183-206. Academic Press: San Diego.
7. Barragan, L. A., Delhaye-Bouchaud, N. and Laget, P. (1985) Drug-induced activation of the inferior olivary nucleus in young rabbits. Differential effects of harmaline and quipazine. Neuropharmacolgy 24, 645-654.
8. Battista, A. F., Goldstein, M., Miyamoto, T. and Matsumoto, Y. (1976) Effects of centrally acting drugs on experimental torticollis in the monkey. In: Dystonia. Advances in Neurology, pp. 329-340, Eds R. Eldrige and S. Fahn. Raven Press: New York.
9. Beales, M., Lorden, J. F., Walz, E. and Oltmans, G. A. (1990) Quantitative autoradiography reveals selective changes in cerebellar GABA receptors of the rat mutant dystonic. J. Neurosci. 10, 1874-1885.
10. Bertrand, C. M. and Lenz, F. A. (1995) Surgical treatment of dystonias. In: Handbook of dystonia, pp. 329-345, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
11. Bhatia, K. P. and Marsden, C. D. (1994) The behavioural and motor consequences of focal lesions of the basal ganglia in man. Brain 117, 859-876.
12. Boyce, S., Clarke, C. E., Luquin, R., Peggs, D., Robertson, R. G., Mitchell, I. J., Sambrook, M. A. and Crossman, A. R. (1990a) Induction of chorea and dystonia in parkinsonian primates. Movement Dis. 5, 3-7.
13. Boyce, S., Rupniak, N. M., Steventon, M. J. and Iversen, S. D. (1990b) Characterization of dyskinesias induced by L-dopa in MPTP-treated squirrel monkeys. Psychopharmacology 102, 21-27.
14. Bowen, W. D., Walker, J. M., Yashar, A. G., Matsumoto, R. R., Walker, F. O. and Lorden, J. F. (1988) Altered haloperidol-sensitive sigma receptors in the genetically dystonic (dt) rat. Eur. J. Pharmacol. 147, 153-154.
15. Brand, M. P., Heales, S. J. R., Land, J. M. and Clark, J. B. (1995) Tetrahydrobiopterin deficiency and brain nitric oxide synthase in the hph-1 mouse. J. Inher. Metab. Dis. 18, 33-39.
16. Brent, P. J. (1993) Behavioural effect of pretreatment with opioid antagonists and sigma binding site ligands on the abnormal motor response produced by the kappa opioid agonist U50,488H in guinea-pigs. Neuropharmacology 32, 751-760.
17. Bressman, S. B., Hunt, A. L., Heiman, G. A., Brin, M. F., Burke, R. E., Fahn, S., Trugman, J. M., de Leon, D., Kramer, P. L., Wilhelmsen, K. C. and Nygaard, T. G. (1994a) Exclusion of the DYT1 locus in a non-Jewish family with early-onset dystonia. Movement Dis. 9, 626-632.
18. Bressman, S. B., Heiman, G. A., Nygaard, T. G., Ozelius, L. J., Hunt, A. L., Brin, M. F., Gordon, M. F., Moskowitz, C. B., de Leon, D., Burke, R. E., Fahn, S., Risch, N. J., Breakefield, X. O. and Kramer, P. L. (1994b) A study of idiopathic torsion dystonia in a non-Jewish family: evidence for genetic heterogeneity. Neurology 44, 283-287.
19. Brown, A., Bernier, G., Mathieu, M., Rossant, J. and Kothary, R. (1995) The mouse dystonia musculorum gene is a neural isoform of bullous pemphigoid antigen 1. Nature Genet. 10, 301-306.
20. Brown, A., Copeland, N. G., Gilbert, D. J., Jenkins, N. A., Rossant, J. and Kothary, R. (1994a) The genomic structure of an insertional mutation in the dystonia musculorum locus. Genomics 20, 371-376.
21. Brown, A., Lemieux, N., Rossant, J. and Kothary, R. (1994b) Human homolog of a mouse sequence from the dystonia musculorum locus is on chromosome 6p12. Mammalian Genome 5, 434-437.
22. Brown, L. L. and Lorden, J. F. (1989) Regional cerebral glucose utilization reveals widespread abnormalities in the motor system of the rat mutant dystonic. J Neurosci 9, 4033-4041.
23. Burgunder, J.-M., Ewald, U., Richter, A. and Löscher, W. (1996) Expression of neuropeptides genes in the genetically dystonic hamster. Movement Dis. 11(suppl. 1), 50.
24. Burke, R. E. and Fahn, S. (1988) An evaluation of sustained postural abnormalities in rats induced by intracerebro-ventricular injection of chlorpromazine methiodide or somatostatin as models of dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 493-500, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
25. Calne, D. B. and Lang, A. E. (1988) Secondary dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 9-33, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
26. Campbell, R. and Peterson, A. C. (1992) An intrinsic neuronal defect operates in dystonia musculorum: A study of dt/dt ↔ + / + chimeras. Neuron 9, 693-703.
27. Carpenter, M. B. (1956) A study of the red nucleus in the rhesus monkey: anatomical degeneration and physiological effects resulting from localized lesions in the red nucleus. J. Comp. Neurol. 105, 195-249.
28. Ceballos-Baumann, A. O. (1996) Dystonien. In: Bewegungsstörungen in der Neurologie. pp. 89-140, Eds B. Conrad and A. O. Ceballos-Baumann. Thieme Verlag: Stuttgart/New York.
29. Ceballos-Baumann, A. O., Passingham, R. E., Marsden, C. D. and Brooks, D. J. (1995) Motor reorganization in acquired hemidystonia. Ann. Neurol. 37, 746-757.
30. Chamberlain, S., Shaw, J., Rowland, A., Wallis, J., South, S., Nakamura, Y., von Gabain, A., Farrall, M. and Williamson, R. (1988) Mapping of mutation causing Friedreich's ataxia to human chromosome 9. Nature 334, 248-250.
31. Changeux, J. P. and Mikoshiba, K. (1978) Genetic and epigenetic factors regulating synapses formation in vertebrate cerebellum and neuromuscular junction. Maturation of the nervous system. Prog. Brain Res. 48, 43-64.
32. Cherubini, E., Gaiarsa, J. L. and Ben-Ari, Y. (1991) GABA: an excitatory transmitter in early postnatal life. Trends Neurosci. 14, 515-519.
33. Chesselet, M.-F. and Delfs, J. M. (1996) Basal ganglia and movement disorders: an update. Trends Neurosci. 19, 417-422.
34. Christoph, G. R., Leonzio, R. J. and Wilcox, K. S. (1986) Stimulation of the lateral habenula inhibits dopamine-containing neurons in the substantia nigra and ventral tegmental area of the rat. J. Neurosci. 6, 613-619.
35. Chu, N.-S., Huang, C.-C., Lu C.-S. and Calne, D. B. (1995) Dystonia caused by toxins. In: Handbook of dystonia, pp. 241-265, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
36. Collinge, J., Pycock, C. J. and Taberner, P. V. (1983) Studies on the interaction between cerebral 5-hydroxytryptamine and gamma-aminobutyric acid in the mode of action of diazepam in the rat. Br. J. Pharmacol. 79, 637-643.
37. Consroe, P., Musty, R. and Conti, L. (1988) Effects of cannabidiol in animal models of neurological dysfunctions. In: Marijuana: an International Research Report, Proceedings of the Melbourne Symposium on Cannabis, pp. 147-152, Eds G. Chester, P. Consroe and R. Musty. Australian Government Publishing Service: Canberra.
38. Consroe, P., Sandyk, R. and Snider, S. R. (1986) Open label evaluation of cannabidiol in dystonic movement disorders. Int. J. Neurosci. 30, 277-282.
39. Corey, A. and Koller, W. (1983) Phenytoin-induced dystonia [Letter]. Ann. Neurol. 14, 92.
40. Crossman, A. R. (1987) Primate models of dyskinesia: The experimental approach to the study of basal ganglia-related involuntary movement disorders. Neurosciene 21, 1-40.
41. DeLong, M. R. (1990) Primate models of movements disorders of basal ganglia origin. Trends Neurosci. 13, 281-285.
42. Demirkiran, M. and Jankovic, J. (1995) Paroxysmal diskinesias: clinical features and classification. Ann. Neurol. 38, 571-579.
43. de Yebenes, J. G., Russell, N. and Fahn, S. (1988a) Intracerebral infusion of norepinephrine produces dystonia in the rat. Neurology (Suppl. 1) 38, 207.
44. de Yebenes, J. G., Vazquez, A., Martinez, A., Mena, M. A., del Rio, R. M., de Filipe, C. and del Rio, J. (1988b) Biochemical findings in symptomatic dystonias. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 167-175, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
45. Duane, D. D. (1988) Spasmodic torticollis. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 473-492, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
46. Duchen, L. W., Strich, S. J. and Falconer, D. S. (1964) Clinical and pathological studies of an hereditary neuropathy in mice (dystonia musculorum). Brain 87, 367-378.
47. Ebert, U., Gernert, M., Löscher, W. and Richter, A. (1996) Abnormal c-fos expression in the lateral habenula during dystonic attacks in a hamster model of idiopathic dystonia. Brain Res. 728, 125-129.
48. Eidelberg, D., Moeller, J. R., Ishikawa, T., Dhawan, V., Spetsieris, P., Przedborski, S. and Fahn, S. (1995) The metabolic topography of idiopathic torsion dystonia. Brain 118, 1473-1484.
49. Fahn, S. (1984) The varied clinical expressions of dystonia. Neurologic Clinics 2, 541-554.
50. Fahn, S. (1988) Concept and classification of dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 1-8, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
51. Fahn, S. (1994) The paroxysmal dyskinesias. In: Movement Disorders 3, pp. 311-345, Eds C. D. Marsden and S. Fahn. Butterworth-Heinemann: Oxford.
52. Fahn, S. (1995) Medical treatment of dystonia. In: Handbook of dystonia, pp. 317-328, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
53. Fahn, S., Marsden, C. D. and Calne, D. B. (1987) Classification and investigation of dystonia. In: Movement disorders 2, pp. 332-358, Eds C. D. Marsden and S. Fahn. Butterworths: London.
54. Fink, J. K., Rainier, S., Wilkowski, J., Jones, S. M., Kume, A., Hedera, P., Albin, R., Mathay, J., Girbach, L., Varvil, T., Otterud, B. and Leppert, M. (1996) Paroxysmal dystonic choreoathetosis: tight linkage to chromosome 2q. Am. J. Hum. Genet. 59, 140-145.
55. Fisher, J. E. Jr. and Iturrian, W. B. (1984) The spontaneous seizures genotype sz in hamsters as a disease model of benzodiazepine receptor deficits. Soc. Neurosci. Abstr. 10, 1065.
56. Fisher, J. E. Jr. and Iturrian, W. B. (1985) Benzodiazepine receptor involvement in the postictal refractory period of the spontaneous seizure prone hamster. Soc. Neurosci. Abstr. 11, 272.
57. Fisher, J. E. Jr. (1986) Role of endogenous benzodiazepine receptor ligands in aberrant neurogenesis producing susceptibility. Ph.D. thesis, Emory University, Athens (Georgia).
58. Flaherty, A. W. and Graybiel, A. M. (1994) Anatomy of the basal ganglia. In: Movement Disorders 3, pp. 3-27, Eds C. D. Marsden and S. Fahn. Butterworth-Heinemann: Oxford.
59. Fletcher, N. A., Stell, R., Harding, A. E. and Marsden, C. D. (1988) Degenerative cerebellar ataxia and focal dystonia. Mov. Disord. 4, 336-342.
60. Fouad, G., Servidei, S., Durcan, S., Bertini, E. and Ptacek, L. J. (1996) A gene for familial paroxysmal dyskinesia (FPD1) maps to chromosome 2q. Am. J. Hum. Genet. 59, 135-139.
61. Fredow, G. and Löscher, W. (1991) Effects of pharmacological manipulation of GABAergic neurotransmission in a new mutant hamster model of paroxysmal dystonia. Europ. J. Pharmacol. 192, 207-219.
62. Frost, M. D., Ritter, F. J., Hoskin, C., Mims, J. and Espe-Lillo, J. (1996) Movement disorder associated with lamotrigine treatment in children and adolescents. Epilepsia 37(Suppl. 5), 112.
63. Fu, J. J. and Lorden, J. F. (1995) Inhibition of deep cerebellar nuclei neurons in dystonic rats. Soc. Neurosci. Abstr. 21, 2081.
64. Fu, J. J. and Lorden, J. F. (1996) Responsiveness of cerebellar nuclei neurons in the dystonic rat to excitatory amino acids. Soc. Neurosci. Abstr. 22, 1649.
65. Fu, Y. S., Tseng, G. F. and Yin, H. S. (1996) Extrinsic inhibitory innervation to rubral neurons in rat brain-stem sclices. Exp. Neurol. 137, 142-150.
66. Garthwaite, J. and Garthwaite, G. (1989) Cellular origins of cyclic GMP responses to excitatory amino acid receptor agonists in rat cerebellum in vitro. J. Neurosci. 48, 29-39.
67. Gerfen, C. R. (1992) The neostriatal mosaic: Multiple levels of compartimental organization. Trends Neurosci. 15, 133-139.
68. Gernert, M. and Löscher, W. (1997) A subconvulsive dose of PTZ elevates the neural firing rate of substantia nigra pars reticulata neurons in dystonic but not in non-dystonic hamsters. In: Göttingen Neurobiology Report 1995. Proceedings of the 23rd Göttingen Neurobiology Conference, Vol. 2, p. 970, Eds N. Elsner and H. Wässle. Thieme: Stuttgart.
69. sz hamsters with paroxysmal dystonia: an EEG power spectrum analysis of depth electrode recordings. Brain Res. 760, 102-108.
70. Gernert, M., Richter, A., Rundfeldt, C. and Löscher, W. Quantitative EEG analysis of depth electrode recordings from striatum and globus pallidus of mutant hamsters with paroxysmal dystonia. Movement Dis., in press.
71. Gernert, M., Richter, A. and Löscher, W. (1997b) The spontaneous firing rate of dorsomedial but not ventromedial striatal neurons is elevated in dystonic hamsters. Soc. Neurosci. Abstr. 23.
72. Gilman, S., Junck, L., Young, A. B., Hichwa, R. D., Markel, D. S., Koeppe, R. A. and Ehrenkaufer, R. L. E. (1988) Cerebral metabolic activity in idiopathic dystonia studied with positron emission tomography. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 231-236, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
73. Giron, L. T., Koller, W. C. and Tanner, C. M. (1995) Epidemiology of dystonic disorders. In: Handbook of dystonia, pp. 103-114, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
74. Green, M. C. (1981) Genetic variants and strains of the laboratory mouse, p.64 Gustav Fischer: Stuttgart.
75. Hallett, M. (1993) Physiology of basal ganglia disorders: an overview. Can. J. Neurol. Sci. 20, 177-183.
76. Hanker, J. S. and Peach, R. (1976) Histochemical and ultrastructural studies on primary sensory neurons in mice with dystonia musculorum. I. Acetylcholinesterase and lysosomal hydrolases. Neuropathol. Appl. Neurobiol. 2, 79-97.
77. Hedreen, J. C., Zweig, R. M., DeLong, M. R., Whitehouse, P. J. and Price, D. L. (1988) Primary dystonias: A review of the pathology and suggestions for new directions of study. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 123-132, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
78. Herkenham, M., Lynn, A. B., de Costa, B. R. and Richfield, E. K. (1991) Neuronal localisation of cannabinoids in the basal ganglia of the rat. Brain Res. 547, 267-274.
79. Herkenham, M. and Nauta, W. J. H. (1977) Afferent connections of the habenular nuclei in the rat. A horseradish peroxidase study, with a note on the fiber-of-passage problem. J. Comp. Neurol. 173, 123-146.
80. Herkenham, M. and Nauta, W. J. H. (1979) Efferent connections of the habenular nuclei in the rat. J. Comp. Neurol. 231, 19-48.
81. Holstege, G. (1995) The basic, somatic and emotional components of the motor system in mammals. In: The rat nervous system. 2nd ed, Ed. G. Paxinos. pp. 137-154. Academic Press: San Diego.
82. Holstege, G., Blök, B. F. M. (1995) Brain stem systems involved in the blink reflex, feeding mechanisms, and micturition. In: The rat nervous system. 2nd ed, Ed. G. Paxinos. pp. 183-214. Academic Press: San Diego.
83. Hornykiewicz, O., Kish, S., Becker, L. E., Farley, I. and Shannak, K. (1986) Brain neurotransmitters in dystonia musculorum deformans. N. Engl. J. Med. 315, 347-353.
84. Hyland, K. and Bola, F. (1989) Tetrahydrobiopterin and biogenic amine status of the hph-1 mouse mutant. Biol. Chem. Hoppe Seyler (Abstr.) 370, 387.
85. Hyland, K., Gunasekera, R. S., Engle, T. and Arnold, L. A. (1996) Tetrahydrobiopterin and biogenic amine metabolism in the hph-1 mouse. J. Neurochem. 67, 752-759.
86. Ichinose, H., Ohye, T., Takahashi, E., Seki, N., Hori, T., Sagawa, M., Nomura, Y., Endo, K., Tanaka, H., Tsuji, S., Fujita, K. and Nagatsu, T. (1994) Hereditary progressive dystonia with marked diurnal fluctuation caused by mutations in the GTP cyclohydrolase I gene. Nature Genet. 8, 236-241.
87. Ikeda, M., Mikuni, M., Nishikawa, T. and Takahashi, K. (1989) A neurochemical study of a new mutant mouse presenting myoclonus-like involuntary movements: a possible model of spontaneous serotonergic hyperactivity. Brain Res. 495, 337-348.
88. Ikoma, K., Samii, A., Mercuri, B., Wassermann, E. M. and Hallett, M. (1996) Abnormal cortical motor excitability in dystonia. Neurology 46, 1371-1376.
89. 400. Neurosci. Res. 8, 189-201.
90. Inoue, Y., Matsumura, Y., Inoue, K., Ichikawa, R. and Takayama, C. (1993) Abnormal synaptic architecture in the cerebellar cortex of a new dystonic mutant mouse Wriggle Mouse Sagami. Neurosci. Res. 16, 39-48.
91. Ishikawa, K., Shibanoki, S., Kubo, T., Kogure, M., Imamura, Y., Osawa, N., Ohmura, M. and Mikoshiba, K. (1989) Functional difference in monoamine transmitters in the behaviorally abnormal mutant (wriggle mouse sagami). Neurosci. Lett. 103, 343-348.
92. Jackson, A. and Crossman, A. R. (1984) Experimental choreoathetosis produced by injection of the gamma-aminobutyric acid antagonist into the lentiform nucleus in the monkey. Neurosci. Lett. 46, 41-45.
93. Jacquet, Y. (1988) A dystonia-like syndrome after neuropeptide (MSH/ACTH) stimulation of the rat locus ceruleus. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 299-311, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
94. Jankovic, J. and Shale, H. (1989) Dystonia in musicians. Semin. Neurol. 9, 131-135.
95. Jankovic, J., Svendsen, C. N. and Bird, E. D. (1987) Brain neurotransmitters in dystonia. N. Engl. J. Med. 316, 278-279.
96. Janota, I. (1972) Ultrastructural studies of an hereditary sensory neuropathy in mice (dystonia musculorum). Brain 95, 529-536.
97. Kaakkola, S. and Teräväinen, H. (1990) Animal models of parkinsonism. Pharmacol. Toxicol. 67, 95-100.
98. Kagamihara, Y., Ohi, K. and Tanaka, R. (1993) Disorders in reciprocal inhibition upon initiation of voluntary ankle dorsiflexion movement in cerebellar motor disorders. In: Role of the cerebellum and basal ganglia in voluntary movements. pp. 33-40, Eds M. Mano, I. Hamada and M. R. DeLong. Excerpta Medica: Amsterdam.
99. Kalen, P., Strecker, R. E., Rosengren, E. and Björklund, A. (1989) Regulation of striatal serotonin release by the lateral habenuladorsal raphe pathway in the rat as demonstrated by in vivo microdialysis: Role of excitatory amino acid and GABA. Brain Res. 492, 187-202.
100. Karbe, H., Holthoff, V. A., Rudolf, J., Herholz, K. and Heiss, W. D. (1992) Positron emission tomography demonstrates frontal cortex and basal ganglia hypometabolism in dystonia. Neurology 42, 1540-1544.
101. Kerrick, J. M., Kelly, B. J., Maister, B. H., Graves, N. N. and Leppik, I. E. (1995) Involuntary movement disorders associated with felbamate. Neurology 45, 185.
102. Khalifa, A. E. and Iturrian, W. B. (1993) Triphasic relapse in dystonic hamster during pregnancy as a rigorous neurochemical model for movement disorder. Soc. Neurosci. Abstr. 19, 1626.
103. Kim, J. J. and Thompson, R. F. (1997) Cerebellar circuits and synaptic mechanisms involved in classical eyeblink conditioning. Trends Neurosci. 20, 177-181.
104. Knappskog, P. M., Flatmark, T., Mallet, J., Ludecke, B. and Bartholome, K. (1995) Recessively inherited L-DOPA-responsive dystonia caused by a point mutation (Q381K) in the tyrosine hydroxylase gene. Hum. Mol. Genet. 4, 1209-1212.
105. Kokubun, T. (1991) A morphological study on the brain in Wriggle Mouse Sagami, a new neurological mutant with dystonic disorders, with special reference to the abnormality of the cerebellum. Hokkaido Igaku Zasshi 66, 333-347.
106. Korczyn, A. D. and Inzelberg, R. (1993) Dystonia. Curr. Opin. Neurol. Neurosurg. 6, 350-357.
107. Kothary, R., Clapoff, S., Brown, A., Campbell, R., Peterson, A. and Rossant, J. (1988) A transgene containing lacZ inserted into the dystonia locus is expressed in the neural tube. Nature 335, 435-437.
108. Kramer, P. L., Bressman, S. B., Fahn, S., Ozelius, L. and Breakefield, X. O. (1995) The genetics of dystonia. In: Handbook of dystonia, pp. 43-58, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
109. Kramer, P. L., de Leon, D., Ozelius, L., Risch, N., Bressman, S. B., Brin, M. F., Schuback, D. E., Burke, R. E., Kwiatkowski, D. J., Shale, H., Gusella, J. F., Breakfield, X. O. and Fahn, S. (1990) Dystonia gene in Ashkenazi Jewish population is located on chromosome 9q32-34. Ann. Neurol. 27, 114-120.
110. Kramer, P. L., Heiman, G. A., Gasser, T., Ozelius, L. J., de Leon, D., Brin, M. F., Burke, R. E., Hewett, J., Hunt, A. L., Moskowitz, C., Nygaard, T. G., Wilhelmsen, K. C., Fahn, S., Breakefield, X. O., Risch, N. J. and Bressman, S. B. (1994) The DYT1 gene on 9q34 is responsible for most cases of early linb-onset idiopathic torsion dystonia in non-Jews. Am. J. Hum. Genet. 55, 468-475.
111. Lalonde, R., Joyal, C. C. and Botez, M. I. (1994) Exploration and motor coordination in dystonia musculorum mutant mice. Physiol. Behav. 56, 277-280.
112. Lamarre, Y. and Mercier, L. A. (1971) Neurophysiological studies of harmaline-induced tremor in the cat. Can. J. Physiol. Pharmacol. 49, 1049-1058.
113. Lauterbach, E. C. (1995) Bipolar disorders, dystonia, and compulsion after dysfunction of the cerebellum, dentatorubrothalamic tract, and substantia nigra. Biol. Psych. 40, 726-730.
114. LeDoux, M. S. and Lorden, J. F. (1996) Single-unit activity of cerebellar Purkinje cells in the awake genetically dystonic rat. Soc. Neurosci. Abstr. 22, 1629.
115. LeDoux, M. S., Lorden, J. F. and Ervin, J. (1993) Cerebellectomy eliminates the motor syndrome of the genetically dystonic rat. Exp. Neurol. 120, 302-310.
116. LeDoux, M. S., Lorden, J. F. and Ervin, J. (1994) Inferior olive serotonin and norepinephrine levels during development in the genetically dystonic rat. Brain Res. Bull. 33, 299-305.
117. LeDoux, M. S., Lorden, J. F. and Hurst, D. C. (1995a) Single-unit activity of cerebellar nuclear cells in the awake genetically dystonic rat. Soc. Neurosci. Abstr. 21, 271.
118. LeDoux, M. S., Lorden, J. F. and Meinzen-Derr, J. (1995b) Selective elimination of cerebellar output in the genetically dystonic rat. Brain Res. 697, 91-103.
119. Lee, J. (1994) Persistent dystonia associated with carbamazepine therapy: a case report. N.Z. Med. J. 107, 360.
120. Lee, M. S. and Marsden, C. D. (1994) Movement disorders following lesions of the thalamus or subthalamic region. Movement Dis. 9, 493-507.
121. Leenders, K. L., Quinn, N., Frackowiak, R. S. J. and Marsden, C. D. (1988) Brain dopaminergic system studied in patients with dystonia using positron emission tomography. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 243-247, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
122. Lehericy, S., Vidailhet, M., Dormont, D., Pierot, L., Chiras, J., Mazetti, P., Marsault, C. and Agid, Y. (1996) Striatopallidal and thalamic dystonia. A magnetic resonance imaging anatomoclinical study. Arch. Neurol. 53, 241-250.
123. Leonard, B. E. and Faherty, C. (1996) SSRIs and movement disorders: is serotonin the culprit? Human Psychopharmacol. 11, 75-82.
124. Leube, B., Rudnicki, D., Ratzlaff, T., Kessler, K. R., Benecke, R. and Auburger, G. (1996) Idiopathic torsion dystonia: Assignment of a gene to chromosome 18q in a German family with adult onset, autosomal dominant inheritance and purely focal distribution. Hum. Mol. Genet. 5, 1673-1677.
125. LeWitt, P. A. (1995) Dystonia caused by drugs. In: Handbook of dystonia, pp. 227-240, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
126. Löscher, W., Annies, R. and Richter, A. (1994) Marked regional disturbances in brain metabolism of monoaminergic neurotransmitters in the genetically dystonic hamster. Brain Res. 658, 199-208.
127. Löscher, W., Blanke, T., Richter, A. and Hoppen, H.-O. (1995) Gonadal sex hormones and dystonia: experimental studies in genetically dystonic hamsters. Movement Dis. 10, 92-102.
128. Löscher, W., Fisher, J. E. Jr., Schmidt, D., Fredow, G., Hönack, D. and Iturrian, W. B. (1989) The sz mutant hamster: a genetic model of epilepsy or of paroxysmal dystonia? Movement Dis. 4, 219-232.
129. Löscher, W. and Fredow, G. (1992) Effects of pharmacological manipulation of dopaminergic and cholinergic neurotransmission. Eur. J. Pharmacol. 213, 31-39.
130. Löscher, W. and Hörstermann, D. (1992) Abnormalities in amino acid neurotransmitters in discrete brain regions of genetically dystonic hamsters. J. Neurochem. 59, 689-694.
131. Löscher, W. and Richter, A. (1993) The glycine/NMDA receptor ligand (+)-HA-966 but not D-cycloserine exert antidystonic efficacy in a genetic animal model of dystonia. Europ. J. Pharmacol. 239, 245-247.
132. IA receptor antagonist (+)-WAY-100135 aggravates dystonic movements in a mutant hamster model. Europ. J. Pharmacol. 255, 235-238.
133. Löscher, W. and Schmidt, D. (1988) Which animal models should be used in the search for new antiepileptic drugs? A proposal based on experimental and clinical considerations Epilepsy Res. 2, 145-181.
134. Löscher, W. and Schmidt, D. (1994) Strategies in antiepileptic drug development: is rational drug design superior to random screening and structural variation? Epilepsy Res. 17, 93-134.
135. Lorden, J. F. (1995) Animal models of dystonia. In: Handbook of dystonia, pp. 5-42, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
136. Lorden, J. F., Lutes, J., Michaela, V. L. and Ervin, J. (1992) Abnormal cerebellar output in rats with an inherited movement disorder. Exp. Neurol. 118, 95-104.
137. Lorden, J. F., McKeon, T. W., Baker, H. J., Cox, N. and Walkley, S. U. (1984) Characterization of the rat mutant dystonic (dt): a new animal model of dystonia musculorum deformans. J. Neurosci. 4, 1925-1932.
138. Lorden, J. F., Oltmans, G. A., McKeon, T. W., Lutes, J. and Beales, M. (1985) Decreased cerebellar 3′5′-cyclic guanosine monophosphate levels and insensitivity to harmaline in the genetically dystonic rat (dt). J. Neurosci. 5, 2618-2625.
139. Lorden, J. F., Oltmans, G. A., Stratton, S. E. and Mays, L. E. (1988) Neuropharmacological correlates of the motor syndrome of the genetically dystonic (dt) rat. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 277-297, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
140. Lorden, J. F. and Thorstad, M. H. (1994) Effects of GABA blockade in the inferior olive on the activity of Purkinje cells in the genetically dystonic (dt) rat. Soc. Neurosci. Abstr. 20, 22.
141. Lutes, J., Lorden, J. F., Davis, B. J. and Oltmans, G. A. (1992) GABA levels and GAD immunoreactivity in the deep cerebellar nuclei of rats with altered olivo-cerebellar function. Brain Res. Bull. 28, 329-336.
142. Lutes, J., Lorden, J. F. and Oltmans, G. A. (1985) Effcets of clonidine on the motor syndrome of the genetically dystonic rat. Neurosci. Abstr. 11, 670.
143. Manyam, B. V., Ferraro, T. N. and Hare, T. A. (1988) Evaluation of amino acid neurotransmitters, related compounds, and precursors in dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 203-209, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
144. Marsden, C. D. (1981) Treatment of torsion dystonia. In: Disorders of Movements. Current Status of Modern Therapy, Vol. 8, pp. 81-?? Ed. A. Barbeau. Lippincott: Philadelphia.
145. Marsden, C. D. (1988) Investigation of dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 35-44, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
146. Marsden, C. D., Harrison, M. J. G. and Bundey, S. (1976) Natural history of idiopathic torsion dystonia. Advances in Neurology 14, 177-187.
147. Marsden, C. D., Obeso, J. A., Zarranz, J. J. and Lang, A. E. (1985) The anatomical basis of symptomatic hemidystonia. Brain 109, 463-483.
148. Marsden, C. D. and Quinn, N. P. (1990) The dystonias. Br. Med. J. 300, 139-144.
149. Matsui, K., Mukoyama, M., Adachi, K. and Ando, K. (1987) Fundamental study in ataxic mice (Wriggle Mouse Sagami). Experim. Animals (Tokyo) 36, 185-189.
150. McDonald, J. D. and Bode, V. C. (1988) Hyperphenyalaninaemia in the hph-1 mouse mutant. Pediatr. Res. 23, 63-67.
151. McDonald, J. D., Cotton, R. G. H., Jennings, I., Ledley, F. D., Woo, S. L. C. and Bode, V. C. (1988) Biochemical defect of the hph-1 mouse mutant is a deficiency in GTP-cyclohydrolase activity. J. Neurochem. 50, 655-657.
152. McGeer, E. G. and McGeer, P. L. (1988) The dystonias. Canad. J. Neurol. Sci. 15, 447-483.
153. McGeer, E. G. and McGeer, P. L. (1995) Pathology of dystonias. Handbook of dystonia, pp. 77-102, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
154. McKeon, T. W., Lorden, J. F., Beales, M. and Oltman, G. A. (1986) Alterations in the noradrenergic projection to the cerebellum of the dystonic (dt) rat. Brain Res. 366, 89-97.
155. McKeon, T. W., Lorden, J. F., Oltmans, G. A., Beales, M. and Walkley, S. U. (1984) Decreased catalepsy response to haloperidol in the genetically dystonic (dt) rat. Brain Res. 308, 89-96.
156. McLellan, D. L. (1982) Cerebellar and deep brain stimulation in movement disorders. In: Movement disorders, pp. 334-344, Eds C. D. Marsden and S. Fahn. Butterworth: London.
157. Messer, A. (1982) Amino acid changes in the mouse mutant dystonia musculorum similar to those in Friedreich's ataxia. Can. J. Neurol. Sci. 9, 185-188.
158. ALB) mouse. Life Sci. 25, 2217-2221.
159. Messer, A. and Strominger, N. L. (1980) An allele of the mouse mutant dystonia musculorum exhibits lesions in the red nucleus and striatum. Neuroscience 5, 543-549.
160. Michela, V. L., Stratton, S. E. and Lorden, J. F. (1990) Enhanced sensitivity to quipazine in the genetically dystonic rat (dt). Pharmacol. Biochem. Behav. 37, 129-133.
161. Mink, J. W. (1996) The basal ganglia: Focused selection and inhibition of competing motor programs. Progress Neurobiol. 50, 381-425.
162. Mitchell, I. J., Boyce, C. E., Sambrook, M. A. and Crossman, A. R. (1992) A 2-deoxyglucose uptake study of the effects of dopamine agonists on the parkinsonian primate brain. Implications for the neural mechanisms that mediate dopamine agonist-induced dyskinesia. Brain 115, 809-824.
163. Mitchell, I. J., Brotchie, J. M., Graham, W. C., Page, R. D., Robertson, R. G., Sambrook, M. A. and Crossman, A. R. (1991) Advances in the understanding of neural mechanisms in movement disorders. In: The basal ganglia III. pp. 607-616, Eds G. Bernardi, M. B. Carpenter, G. Di Chiara, M. Morelli and P. Stanzione. Plenum Press: New York.
164. Mitchell, I. J., Clarke, C. E., Boyce, C. E., Robertson, R. G., Peggs, D., Sambrook, M. A. and Crossman, A. R. (1989) Neural mechanisms underlying parkinsonian symptoms based upon regional uptake of 2-deoxyglucose in monkeys exposed to 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine. Neuroscience 32, 213-226.
165. Mitchell, I. J., Luquin, R., Boyce, C. E., Robertson, R. G., Sambrook, M. A. and Crossman, A. R. (1990) Neural mechanisms of dystonia: evidence from a 2-deoxyglucose uptake study in a primate model of dopamine agonist-induced dystonia. Movement Dis. 5, 49-54.
166. Möhler, H. (1992) GABAergic synaptic transmission. Regulation by drugs. Arzneimittelforschung 42, 211-214.
167. Morgan, J. I. and Curran, T. (1991) Stimulus-transcription coupling in the nervous system: Involvement of the inducible protooncogenes fos and jun. Annu. Rev. Neurosci. 14, 421-451.
168. Mori, S., Homma, Y. and Nakajima, K. (1995) Locomotion and posture: Modulation by upper neurons. In: Age-related dopamine-dependent disorders. Monogr. Neural Sci. pp. 170-177, Eds Segawa, M. and Nomura, Y. Karger: Basel.
169. Moss, T. H. (1981) Segmental demyelination in the peripheral nerves of mice affected by a hereditary neuropathy (dystonia musculorum). Acta Neuropath. 53, 51-56.
170. Müller, U. and Kupke, K. G. (1990) The genetics of primary torsion dystonia. Hum. Genet. 84, 107-115.
171. Muramatsu, S., Yoshida, M. and Nakamura, S. (1990) Electrophysiological study of dyskinesia produced by microinjection of picrotoxin into the striatum of the rat. Neurosci. Res. 7, 369-380.
172. Naudon, L., Clavel, N., Delfs, J. M., Lorden, J. F. and Chesselet, X. (1996) Differential expression of glutamic acid decarboxylase mRNA in cerebellar purkinje cells and deep cerebellar nuclei of the genetically dystonic rat. Soc. Neurosci. Abstr. 22, 1649.
173. Nobrega, J. N. (1992) Brain metabolic mapping and behaviour: assessing the effects of early development experiments in adult animals. In: Advances in metabolic mapping techniques for brain imaging of behavioural and learning functions, pp. 125-149, Eds F. Gonzales-Lima. Kluwer:Amsterdam.
174. Nobrega, J. N., Richter, A., Burnham, W. M. and Löscher, W. (1995) Alterations in the brain GABAA/benzodiazepine receptor-chloride ionophore complex in a genetic model for generalized dystonia: a quantitative autoradiographic analysis. Neuroscience 64, 229-239.
175. Nobrega, J. N., Richter, A., Jiwa, D., Raymond, R. and Löscher, W. (1997) Alterations in N-methyl-D-aspartate receptor binding in dystonic hamster brains. Brain Res. 744, 161-165.
176. Nobrega, J. N., Richter, A., Tozman, N., Jiwa, D. and Löscher, W. (1996) Quantitative autoradiography reveals regionally selective changes in dopamine D1 and D2 receptor binding in the genetically dystonic hamster. Neuroscience 71, 927-936.
177. Nobrega, J. N., Richter, A., Jiwa, D., Raymond, R. and Löscher, W. Regional alterations in neuronal activity in dystonic hamster brain determined by quantitative CO histochemistry. Neuroscience (in press).
178. Nutt, J. G., Muenter, M. D., Aronson, A., Kurland, L. T. and Melton, L. J. (1988) Epidemiology of focal and generalised dystonia in Rochester, Minnesota. Movement Dis. 3, 188-194.
179. Nygaard, T. G. (1995) Dopa-responsive dystonia. In: Handbook of dystonia, pp. 213-226, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
180. Nygaard, T. G., Wilhelmsen, K. C., Risch, N. J., Brown, D. L., Trugman, J. M., Gilliam, T. C., Fahn, S. and Weeks, D. E. (1993) Linkage mapping of dopa-responsive dystonia (DRD) to chromosome 14q. Nature Genetics 5, 386-391.
181. Obeso, J. A. and Gimenez-Roldan, S. (1988) Clinicopathological correlation in symptomatic dystonia. In: Dystonia 2. Advances in neurology, Vol. 50, pp. 113-122, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
182. O'Brien, G. A., Oltmans, G. A. and Lorden, J. F. (1990) Decreased quisqualate-sensitive glutamate binding in the cerebellar cortex of the dystonic rat. Soc. Neurosci. Abstr. 16, 1191.
183. Oltmans, G. A., Beales, M. and Lorden, J. F. (1986) Glutamic acid decarboxylase activity in micropunches of the deep cerebellar nuclei of the genetically dystonic (dt) rat. Brain Res. 385, 148-151.
184. Oltmans, G. A., Beales, M., Lorden, J. F. and Gordon, J. H. (1984) Alterations in the cerebellar glutamic acid decarboxylase (GAD) activity in a genetic model of torsion dystonia (rat). Exp. Neurol. 85, 216-222.
185. Osawa, N., Ohmura, M., Ishihara, Y. and Nakano, T. (1988) Observation and management of ″Wriggle Mouse Sagami″ with hereditary extrapyramidal syndrome. Kitasato Med. 18, 441-446.
186. 18F-Dopa uptake and normal glucose metabolism in idiopathic dystonia syndrome. J. Neurolog. Sci. 111, 195-199.
187. Ozelius, L., Kramer, P. L., Moskowitz, C. B., Kwiatkowski, D. J., Brin, M. F., Bressman, S. B., Schuback, D. E., Falk, C. T., Risch, N., deLeon, D., Burke, R. E., Haines, J., Gusella, J. F., Fahn, S. and Breakefield, X. O. (1989) Human gene for torsion dystonia located on chromosome 9q32-q34. Neuron 2, 1427-1434.
188. Page, R. D., Sambrook, M. A. and Crossman, A. R. (1993) Thalamotomy for the alleviation of levodopa-induced dyskinesia: experimental studies in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine-treated parkinsonian monkey. Neuroscience 55, 147-165.
189. Parent, A. and Hazrati, L.-N. (1995a) Functional anatomy of the basal ganglia. I. The cortico-basal ganglia-thalamo-cortical loop. Brain Res. Rev. 20, 91-127.
190. Parent, A. and Hazrati, L.-N. (1995b) Functional anatomy of the basal ganglia. II. The place of subthalamic nucleus and external pallidum in basal ganglia circuitry. Brain Res. Rev. 20, 91-127.
191. 18F]spiperone binding in putamen in idiopathic focal dystonia. J. Neurosci. 17, 843-850.
192. Pertwee, R. G. (1988) Drugs which stimulate or facilitate central GABAergic transmission interact synergistically with delta-9-tetrahydrocannabinol to produce marked catalepsy in mice. Neuropharmacology 27, 1265-1270.
193. Pickel, V. M., Joh, T. H. and Reis, D. J. (1977) A serotonergic innervation of noradrenergic neurons in nucleus locus coeruleus: demonstration by immunocytochemical localization of the transmitter specific enzymes tyrosine and tryptophan hydroxylase. Brain Res. 131, 197-214.
194. 18]F-Dopa uptake in familial idiopathic dystonia. Brain 116, 1191-1199.
195. Porter, R. H. P., Greene, J. G., Higgins, D. S. and Greenamyre, J. T. (1994) Polysynaptic regulation of glutamate receptors and mitochondrial enzyme activities in the basal ganglia of rats with unilateral dopamine depletion. J. Neurosci. 14, 7192-7199.
196. Pratt, D. G., Möhler, H., Richter, A. and Löscher, W. (1995) Regionally selective and age-dependent alterations in benzodiazepine receptor binding in the genetically dystonic hamster. J Neurochem 64, 2153-2158.
197. Price, J. L. (1995) Thalamus. In: The rat nervous system. 2nd ed, Ed. G. Paxinos. pp. 629-645. Academic Press: San Diego.
198. Reilly, J. A., Hallett, M., Cohn, L. G., Tarkka, I. M. and Dang, N. (1992) The N30 component of somatosensory evoked potentials in patients with dystonia. Electroenceph. Clin. Neurophysiol. 84, 243-247.
199. Richter, A., Brotchie, J. M., Löscher, W. and Crossman, A. R. (1995) 2-Deoxyglucose uptake study in mutant dystonic hamsters: abnormalities in the motor system. In: Göttingen Neurobiology Report 1995. Proceedings of the 23rd Göttingen Neurobiology Conference. p. 852, Eds N. Elsner and R. Menzel. Thieme: Stuttgart.
200. 3H]-2-deoxyglucose uptake study in mutant dynstonic hamsters: abnormalities in discrete brain regions of the motor system. Movement Dis., in press.
201. Richter, A., Fredow, G. and Löscher, W. (1991) Antidystonic effects of the NMDA receptor antagonists memantine, MK-801 and CGP 37849 in a hamster model of paroxysmal dystonia. Neurosci. Lett. 133, 57-60.
202. Richter, A., Gernert, M. and Löscher, W. (1997) Prodystonic effects of riluzole in an animal model of idiopathic dystonia related to decreased total power in the red nucleus? Europ. J. Pharmacol. 332, 133-142.
203. sz). Eur. J. Neurosci. Suppl. 5, 223.
204. Richter, A. and Löscher, W. (1993a) Alterations in pharmacological sensitivity of GABAergic but not dopaminergic and glutamatergic systems during ontogenesis in dystonic mutant hamsters. Europ. J. Pharmacol. 231, 111-119.
205. Richter, A. and Löscher, W. (1993b) The atypical neuroleptic, clozapine, exerts antidystonic activity in a mutant hamster model. Comparison with haloperidol. Eur. J. Pharmacol. 242, 309-312.
206. Richter, A. and Löscher, W. (1993c) The kappa opioid receptor agonist U50,488H exerts antidystonic activity in a mutant hamster model of generalized dystonia. Europ. J. Pharmacol. 236, 289-294.
207. Richter, A. and Löscher, W. (1994a) Neuroleptics and movement disorders. Effects of classical and atypical neuroleptics, administered alone or in combination with NMDA antagonists, in a genetic hamster model of generalized dystonia. Naunyn Schmiedeberg's Arch. Pharmacol. 350 (suppl.), 16.
208. Richter, A. and Löscher, W. (1994b) (+)-WIN-55,212-2, a novel cannabinoid receptor agonist, exerts antidystonic effects in mutant dystonic hamsters. Europ. J. Pharmacol. 264, 371-377.
209. Richter, A. and Löscher, W. (1995) Behavioural response to pharmacological manipulation of serotonin receptors in the genetically dystonic hamster. Pharmacol. Biochem. Behav. 52, 655-665.
210. 2+ channel antagonists in a hamster model of idiopathic dystonia. Eur. J. Pharmacol. 300, 197-202.
211. Richter, A. and Löscher, W. (1997a) Dextrorphan, but not dextromethorphan, exerts antidystonic effects in mutant dystonic hamsters. Brain Res. 745, 336-338.
212. Richter, A. and Löscher, W. (1997b) SR 141716A antagonises antidystonic and cataleptic effects of the cannabinoid receptor agonist (+)-WIN 55,212-2 in genetically dystonic hamsters. Naunyn Schmiedeberg's Arch. Pharmacol. 355(suppl.), 91.
213. Richter, A. and Löscher, W. (1997c) MK-801 potentiates antidystonic effects of clozapine but not of haloperidol in mutant dystonic hamsters. Brain Res., 769, 296-302.
214. Richter, A., Löscher, W., Baran, H. and Gramer, M. (1996a) Increased levels of kynurenic acid in brains of genetically dystonic hamsters. Devel. Brain Res. 92, 111-116.
215. Richter, A., Löscher, W. and Fredow, G. (1990) Paradoxical enhancement of severity of dystonic disturbances in mutant Syrian golden hamsters during chronic treatment with phenobarbital. Naunyn-Schmiedeberg's Arch. Pharmacol. 342 (suppl.), R32.
216. Richter, A., Löscher, W. and Löschmann, P.-A. (1993) The AMPA receptor antagonist NBQX exerts antidystonic effects in an animal model of idiopathic dystonia. Eur. J. Pharmacol. 231, 287-291.
217. Richter, A., Löschmann, P.-A. and Löscher, W. (1994) The novel antiepileptic drug lamotrigine exerts prodystonic effects in a mutant hamster model of generalized dystonia. Eur. J. Pharmacol. 264, 345-351.
218. Richter, A., Löschmann, P.-A. and Löscher, W. (1996b) Antidystonic effects of inhibitors of nitric oxide synthase in genetically dystonic hamsters. Soc. Neurosci. Abstr. 22, 1649.
219. Riker, D. K., Messer, A. and Roth, R. H. (1981) Increased noradrenergic metabolism in the cerebellum of the mouse mutant dystonia musculorum. J. Neurochem. 37, 649-654.
220. Rothwell, J. C. (1995) The physiology of dystonia. In: Handbook of dystonia, pp. 59-76, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
221. Rothwell, J. C. and Obeso, J. A. (1987) The anatomical and physiological basis of torsion dystonia. In: Movement Disorders 2, pp. 313-331, Ed. Marsden, C. D. and S. Fahn. Butterworths: London.
222. Rouiller, E. M., Liang, F., Babalian, A., Moret, V. and Wiesendanger, M. (1994) Cerebellothalamocortical and pallidothalamocortical projections to the primary and supplementary motor cortical areas: a multiple tracing study in macaque monkeys. J. Compar. Neurol. 345, 185-213.
223. Ruigrok, T. J. H. and Cella, F. (1995) Precerebellar nuclei and red nucleus. In: The rat nervous system, pp. 277-308. 2nd ed, Ed. G. Paxinos. Academic Press: San Diego.
224. Sasaki, K. and Gemba, H. (1993) Cerebro-cerebellar interactions: For fast and stable timing of voluntary movement. In: Role of the cerebellum and basal ganglia in voluntary movements, pp. 40-50, Eds M. Mano, I. Hamada and M. R. DeLong. Excerpta Medica: Amsterdam.
225. 18F]-Dopa positron emission tomography. Ann. Neurol. 30, 24-30.
226. Schmied, A., Amalric, M., Dormont, D. and Farin, D. (1990) GABAergic mechanisms in the cat red nucleus: effects of intracerebral microinjections of muscimol or bicuculline in a conditioned motor task. Exp. Brain Res. 81, 523-532.
227. Segawa, M. and Nomura, Y. (1995) Hereditary progressive dystonia with marked diurnal fluctuation and dopa-responsive dystonia: pathognomonic clinical features. In: Age-related dopamine-dependent disorders. Monogr. Neural Sci, pp. 10-24, Eds Segawa, M. and Y. Nomura. Karger: Basel.
228. Sharp, F. R., Sagar, S. M. and Swanson, R. A. (1993) Metabolic mapping with cellular resolution: c-fos vs. 2-deoxyglucose. Crit. Rev. Neurobiol. 7, 205-228.
229. Sheehy, M. P., Rothwell, J. C. and Marsden, C. D. (1988) Writer's cramp. In: Dystonia 2. Advances in Neurology, Vol. 50, pp. 457-472, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
230. - ion channels modulated by multiple drug-binding sites. Trends Pharmacol. Sci. 13, 446-450.
231. Singewald, N., Kaehler, S., Hemeida, R. and Philippu, A. (1997) Release of serotonin in the rat locus coeruleus: Effects of cardiovascular, stressful and noxios stimuli. Europ. J. Neurosci. 9, 556-562.
232. Sokoloff, L. (1981) Localization of functional activity in the central nervous system by measurement of glucose utilization with radioactive deoxyglucose. J. Cereb. Blood Flow Metabol. 1, 7-36.
233. Sotelo, C. and Guenet, J. L. (1988) Pathologic changes in the CNS of dystonia musculorum mutant mouse: an animal model for human spinocerebellar ataxia. Neuroscience 27, 403-424.
234. Spinella, G. M. and Sheridan, P. H. (1994) Research opportunities in dystonia. Neurology 44, 1177-1179.
235. A receptors. Science 269, 977-981.
236. Stanley, E., Messer, A. and Strominger, N. L. (1983) Effects of age and strain differences on the red nucleus of the mouse mutant dystonia musculorum. Anat. Rec. 206, 313-318.
237. Stewart, B. R., Rupniak, N. M. J., Jenner, P. and Marsden, C. D. (1988) Animal models of neuroleptic-induced acute dystonia. In: Dystonia 2. Advances in Neurology, Vol. 50, pp. 343-359, Eds S. Fahn, C. D. Marsden and D. B. Calne. Raven Press: New York.
238. Stone, M. R., Vant Groenewout, J. I., Truong, D. D. and Matsumoto, R. R. (1994) Histamine dysfunction in the red nucleus can produce torsional dystonia which is relieved by diphenhydramine, pyrilamine or cimetidine. Soc. Neurosci. Abstr. 20, 1277.
239. Stone, T. W. (1993) Neuropharmacology of quinolinic and kynurenic acids. Pharmacol. Rev. 45, 309-379.
240. Stratton, S. E. and Lorden, J. F. (1991) Effect of harmaline on cells of the inferior olive in the absence of tremor: differential response of genetically dystonic and harmaline-tolerant rats. Neuroscience 41, 543-549.
241. Stratton, S. E., Lorden, J. F., Mays, L. E. and Oltmans, G. A. (1988) Spontaneous and harmaline-stimulated Purkinje cell activity in rats with a genetic movement disorder. J. Neurosci. 8, 3327-3336.
242. Tarlov, E. (1969) The postural effects of lesions of the vestibular nuclei: A note on species differences among primates. J. Neurosurg. 31, 187-195.
243. Thach, W. T., Mink, J. W., Goodkin, H. P. and Keating, J. G. (1993) Combining versus gating motor programs: Differential roles for cerebellum and basal ganglia? In: Role of the cerebellum and basal ganglia in voluntary movements. pp. 235-245, Eds M. Mano, I. Hamada and M. R. DeLong. Excerpta Medica: Amsterdam.
244. Tsui, J. K. C. (1995) Applications of botulinum toxin: Cervical and limb dystonia. In: Handbook of dystonia, pp. 415-429, Eds J. K. C. Tsui and D. B. Calne. Marcel Dekker: New York.
245. Vaamonde, J., Narbona, J., Weiser, R., Garcia, M. A., Brannan, T. and Obeso, J. A. (1994) Dystonic storms: a practical management problem. Clin. Pharmacol. 17, 344-347.
246. Vila, M., Levy, R., Herrero, M.-T., Faucheux, B., Obeso, J. A., Agid, Y. and Hirsch, E. C. (1996) Metabolic activity of the basal ganglia in parkinsonian syndromes in human and non-human primates: a cytochrome oxidase histochemistry study. Neuroscience 71, 903-912.
247. Wahnschaffe, U., Fredow, G., Heintz, P. and Löscher, W. (1990) Neuropathological studies in a mutant hamster model of paroxysmal dystonia. Movement Dis. 5, 286-293.
248. Walker, J. M., Bowen, W. D., Walker, F. O., Matsumoto, R. R., De Costa, B. and Rice, K. C. (1990) Sigma receptors: Biology and function. Pharmacol. Rev. 42, 355-402.
249. Weissman, A. D., McCann, D. J., Lorden, J. F. and Su, T.-P. (1993) An absence of changes in sigma receptor subtypes in the brains of genetically dystonic (dt) rats. Eur. J. Pharmacol. 250, 329-332.
250. Wichmann, T. and DeLong, M. R. (1996) Functional and pathophysiological models of the basal ganglia. Curr. Opin. Neurobiol. 6, 751-758.
251. Wieland, S. and Lucki, I. (1991) Altered behavioural responses mediated by serotonin receptors in the genetically dystonia (dt) rat. Brain Res. Bull. 26, 11-16.
252. Wong-Riley, M. T. T. (1989) Cytochrome oxidase: an endogenous metabolic marker for neuronal activity. Trends Neurosci. 12, 94-101.
253. Yamada, H., Fujimoto, K.-I. and Yoshida, M. (1995) Neuronal mechanism underlying dystonia induced by bicuculline injection into the putamen of the cat. Brain Res. 677, 333-336.
254. Yoon, C. H., Peterson, J. S. and Corrow, D. (1976) Spontaneous seizures: a new mutation in Syrian golden hamsters. J. Heredity 67, 115-116.
255. Yoshida, M., Nagatsuka, Y., Muramatsu, S. and Niijima, K. (1991) Differential roles of the caudate nucleus and putamen in motor behavior of the cat as investigated by local injection of GABA antagonists. Neurosci. Res. 10, 34-51.
256. Zervas, N. T. (1977) Long-term review of dentatectomy in dystonia musculorum deformans and cerebral palsy. Acta Neurochir. Suppl. 24, 49-51.