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Subcortical Dementia Neuropsychology, Neuropsychiatry, and Pathophysiology

Published online by Cambridge University Press:  29 January 2018

J. L. Cummings
J. L. Cummings*
Affiliation:
Neurobehavior Unit, 256b (691/B111), West Los Angeles Veterans Administration Medical Center (Brentwood Division), 11301 Wilshire Boulevard, Los Angeles, California 90073, USA
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Abstract

Subcortical dementia refers to a clinical syndrome characterised by slowing of cognition, memory disturbances, difficulty with complex intellectual tasks such as strategy generation and problem solving, visuospatial abnormalities, and disturbances of mood and affect. The syndrome was first described by Kinnier Wilson, but further progress in development of the concept has occurred only within the past ten years. Subcortical dementia occurs in degenerative extrapyramidal disorders and has also been identified in inflammatory, infectious, and vascular conditions. Histologic, metabolic, and neurochemical investigations implicate dysfunction primarily of subcortical neurotransmitter systems and subcortical structures or subcortical-frontal connections in the genesis of the syndrome. Subcortical dementia contrasts neuropsychologically and anatomically with disorders such as dementia of the Alzheimer type that affect primarily the cerebral cortex. The clinical characteristics of subcortical dementia reflect the interruption of fundamental functions (motivation, mood, timing, arousal) mediated by phylogenetically and ontogenetically early maturing structures.

Information

Type
Review Article
Information
The British Journal of Psychiatry , Volume 149 , Issue 6 , December 1986 , pp. 682 - 697
Copyright
Copyright © 1986 The Royal College of Psychiatrists 

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References

Albert, M. L.(1978) Subcortical dementia. In Alzheimer's Disease: Senile Dementia and Related Disorders (eds R. Katzman, R. D. Terry, & K. L. Bick). New York: Raven Press.Google Scholar
Albert, M. L., Feldman, R. G. & Willis, A. L.(1974) The ‘subcortical dementia’ of progressive supranuclear palsy. Journal of Neurology, Neurosurgery” and Psychiatry, 37, 121–130.CrossRefGoogle ScholarPubMed
Albert, M. S., Butters, N. & Brandt, J.(1981) Patterns of remote memory in amnesic and demented patients. Archives of Neurology, 38, 495–500.CrossRefGoogle ScholarPubMed
Anden, N.-E., Dalstrom, A., Fuxe, K., Larsson, K., Olson, L. & Ungerstedt, U.(1966) Ascending monoamine neurons to the diencephalon. Acta Physiologica Scandinavia, 67, 313–326.CrossRefGoogle Scholar
Arendt, T., Bigl, V., Arendt, A. & Tennstedt, A.(1983) Loss of neurons in the nucleus basalis of Meynert in Alzheimer's disease, paralysis, agitans, and Korsakoff's disease. Acta Neuropathologica, 61, 101–108.Google Scholar
Arregui, A., Bennett, J. P. Jr., Bird, E. D., Yamamura, H. I., Iversen, L. L. & Snyder, S. H.(1977) Huntington's chorea: selective depletion of activity of angiotensin converting enzyme in the corpus striatum. Annals of Neurology, 2, 294–298.Google Scholar
Bachman, D. L. & Albert, M. L.(1984) The dopaminergic syndromes of dementia. In Brain Pathology (eds G. Pilleri & F. Tagliavini). Bern, Switzerland: Brain Anatomy Institute.Google Scholar
Ball, M. J.(1984) The morphological basis of dementia in Parkinson's disease. Canadian Journal of Neurological Science, 11, 180–184.Google Scholar
Ballard, P. A., Tetrad, J. W., & Langston, W.(1985) Permanent human parkinsonism due to l-methyl-4-phenyl- 1,2,3,6- tetrahydropyridine (MPTP): seven cases. Neurology, 35, 949–956.CrossRefGoogle Scholar
Bales, K. A. & Tomoeda, C. K.(1983) Confrontation naming impairment in dementia. Brain and Language, 19, 98–114.Google Scholar
Beardsley, J. V. & Puletti, F.(1971) Personality (MMPI) and cognitive (WAIS) changes after levodopa treatment. Archives of Neurology, 25, 145–150.CrossRefGoogle ScholarPubMed
Benson, D. F. & Cummings, J. L.(1985) Subcortical dementia. In Brain Pathology, (eds G. Pilleri & F. Tagliavini). Bern, Switzerland: Brain Anatomy Institute.Google Scholar
Benson, D. F. & Cummings, J. L., Kuhl, D. E., Hawkins, R. A., Phelps, M. E., Cummings, J. L. & Tsai, S. Y.(1983) The fluoro-deoxyglucose 18F scan in Alzheimer's disease and multi-infarct dementia. Archives of Neurology, 40, 711–714.CrossRefGoogle Scholar
Bes, A., Guell, A., Fabre, N., Dupui, Ph, Victor, G. & Gerand, G.(1983) Cerebral blood flow studied by xenon-133 inhalation technique in parkinsonism: loss of hyperfrontal pattern. Journal of Cerebral Blood Flow and Metabolism, 3, 33–37.Google Scholar
Bird, E. D. & Iversen, L. L.(1974) Huntington's chorea: postmortum measurement of glutamic acid decarboxylase, choline acetyltransferase and dopamine in basal ganglia. Brain, 97. 457–472.Google Scholar
Bissette, G., Nemeroff, C. B., Decker, M. W., Kizer, J. S., Agid, Y. & Javoy-Agid, F.(1985) Alterations in regional brain concentrations of neurotensin and bombesin in Parkinson's disease. Annals of Neurology, 17, 324–328.Google Scholar
Boll, T. J., Heaton, R. & Reitan, R. M., (1974) Neuropsychological and emotional correlates of Huntington's chorea. Journal of Nervous and Mental Disease, 158, 61–69.CrossRefGoogle ScholarPubMed
Boller, F., Mizutani, T., Roessmann, U. & Gambetti, P.(1980) Parkinson disease, dementia, and Alzheimer disease: clinicopathological correlations. Annals of Neurology, 7, 329–335.Google Scholar
Boller, F., Mizutani, T., Roessmann, U. & Gambetti, P., Passafiume, D., Keefe, N. C., Rogers, K., Morrow, L. & Kim, Y.(1984) Visuospatial impairment in Parkinson's disease. Archives of Neurology, 41, 485–490.Google Scholar
Bowen, F. P., Kamienny, R. S., Burns, M. M. & Yahr, M. D.(1975) Parkinsonism: effects of levodopa treatment on concept formation. Neurology, 25, 701–704.Google Scholar
Brouwers, P., Cox, C., Martin, A., Chase, T. & Fedio, P.(1984) Differential perceptual-spatial impairment in Huntington's and Alzheimer's dementias. Archives of Neurology, 41, 1073–1076.CrossRefGoogle ScholarPubMed
Brown, G. L. & Wilson, W. P.(1972) Parkinsonism and depression. Southern Medical Journal, 65, 540–545.Google Scholar
Burns, R. S., Chiueh, C. C., Markey, S. P., Ebert, M. H., Jacobowitz, D. M. & Kopin, I. J.(1983) A primate model of parkinsonism: selective destruction of dopaminergic neurons in pars compacta of the substantia nigra by N-methyl-4-phenyl-1.2.3.6- tetrahydropyridine. Proceedings of the National Academy of Science, 80, 4546–4550.CrossRefGoogle ScholarPubMed
Butters, N., Tarlow, S., Cermak, L. S. & Sax, D.(1976) A comparison of the information processing deficits of patients with Huntington's chorea and Korsakoff's syndrome. Cortex, 12, 134–144.Google Scholar
Butters, N., Tarlow, S., Cermak, L. S. & Sax, D. Sax, D., Montgomery, K. & Tarlow, S.(1978) Comparison of the neuropsychological deficits associated with early and advanced Huntington's disease. Archives of Neurology, 35, 585–589.CrossRefGoogle ScholarPubMed
Caine, E. D., Ebert, M. H. & Weingartner, H.(1977). An outline for the analysis of dementia. Neurology, 27, 1087–1092.Google Scholar
Caine, E. D., Ebert, M. H. & Weingartner, H., & Shoulson, I.(1983) Psychiatric syndromes in Huntington's disease. American Journal of Psychiatry, 140, 728–733.Google ScholarPubMed
Carter, H. R. & Sukavajana, C.(1956) Familial cerebello-olivary degeneration with late development of rigidity and dementia. Neurology, 6, 876–884.CrossRefGoogle ScholarPubMed
Celesia, G. G. & Wanamaker, W. M.(1972) Psychiatric disturbances in Parkinson's disease. Diseases of the Nervous System, 33, 577–583.Google ScholarPubMed
Chandler, J. H. & Bebin, J.(1956) Hereditary cerebellar ataxia. Neurology, 6, 187–195.CrossRefGoogle ScholarPubMed
Clark, A. W., Parhead, L. M., Folstein, S. E., Whitehouse, P. J., Hedreen, J. C., Price, D. L. & Chase, G. A.(1983) The nucleus basalis in Huntington's disease. Neurology, 33, 1262–1267.CrossRefGoogle ScholarPubMed
CORSELLIS, J. A. N.(1976) Ageing and the dementias. In Greenfield's Neuropathology (eds. W. Blackwood & J. A. N. Corsellis). Chicago: Yearbook Medical Publishers.Google Scholar
Crow, T. J., Johnstone, E. C. & McClelland, H. A.(1976) The coincidence of schizophrenia and parkinsonism: some neurochemical implications. Psychological Medicine, 6, 227–233.Google Scholar
Cummings, J. L.(1985a) Psychosomatic aspects of movement disorders. In Interface Between Neurology and Psychiatry (ed. M. R. Trimble). Karger: New York.Google Scholar
Cummings, J. L.(1985b) Organic delusions: phenomenology, anatomical correlations, and review. British Journal of Psychiatry, 146, 184–197.Google Scholar
Cummings, J. L.(1985c) Clinical Neuropsychiatry, New York: Grune & Stratton.Google Scholar
Cummings, J. L. & Benson, D. F.(1983) Dementia: A Clinical Approach. Boston: Butterworths.Google Scholar
Cummings, J. L. & Benson, D. F.(1984) Subcortical dementia: review of an emerging concept. Archives of Neurology, 41, 874–879.Google Scholar
Cummings, J. L. & Benson, D. F.(1986). Dementia of the Alzheimer type: an inventory of diagnostic clinical features. Journal of the American Geriatrics Society, 34, 12–19.Google Scholar
Cummings, J. L. & Benson, D. F., Gosenfeld, L., Houlihan, J. & McCaffrey, T.(1983) Neuropsychiatric manifestations of idiopathic calcification of the basal ganglia: case report and review. Biological Psychiatry, 18, 591–601.Google Scholar
Cummings, J. L. & Benson, D. F., Gosenfeld, L., Houlihan, J. & McCaffrey, T. & Mendez, M. F.(1984) Secondary mania with focal cerebrovascular lesions. American Journal of Psychiatry, 141, 1084–1087.Google Scholar
Cummings, J. L. & Benson, D. F., Gosenfeld, L., Houlihan, J. & McCaffrey, T. & Mendez, M. F., Benson, D. F., Hill, M. A. & Read, S.(1985) Aphasia in dementia of the Alzheimer type. Neurology, 35, 394–397.CrossRefGoogle ScholarPubMed
Dania, G. & Hilron, R. C.(1975) Judgement of the visual vertical and horizontal in patients with parkinsonism. Neurology, 25, 43–47.Google Scholar
D'Antona, R., Baron, J. C., Samson, Y., Serdari, M., Viader, F., Acid, Y. & Gambier, J.(1985) Frontal cortex hypometabolism detected by positron tomography in patients with progressive supranuclear palsy. Brain, 108, 785–799.Google Scholar
Davies, D. L.(1949) Psychiatric changes associated with Friedreich's ataxia. Journal of Neurology, Neurosurgery, and Psychiatry, 12, 246–250.Google Scholar
Davies, G. C., Williams, A. C., Markey, S. P., Ebert, M. H., Caine, E. D., Reichert, C. M. & Kopin, I. J.(1979) Chronic parkinsonism secondary to intravenous injection of mepiridine analogues. Psychiatry Research, 1, 249–254.Google Scholar
Davies, P. H., Bergeron, C. & McLachlan, D. R.(1985) Atypical presentation of progressive supranuclear palsy. Annals of Neurology, 17, 337–343.Google Scholar
Den, H. Artog Jager, W. A. & Bethlelm, J.(1960) The distribution of Lewy bodies in the central and autonomic nervous systems in idiopathic paralysis agitans. Journal of Neurology, Neurosurgery, and Psychiatry, 23, 283–290.Google Scholar
Dewhurst, K., Oliver, J., Trick, K. L. K. & McKnight, A. L.(1969) Neuropsychiatric aspects of Huntington's disease. Confinia Neurologica, 31, 258–268.CrossRefGoogle ScholarPubMed
Direnfeld, L. K., Albert, M. L., Volicer, L., Langlais, P. J., Marquis, J. & Kaplan, E.(1984) Parkinson's disease: the possible relationship of laterality to dementia and neurochemical findings. Archives of Neurology, 41, 935–941.Google Scholar
Dom, R., Malfroid, M. & Baro, F.(1976) Neuropathology of Huntington's chorea. Studies of the ventrobasal complex of the thalamus. Neurology, 26, 64–68.CrossRefGoogle ScholarPubMed
Emson, P. C., Arregui, A., Clement-Jones, V., Sandberg, B. E. B. & Rossor, M.(1980a) Regional distribution of methionine-enkephalin and substance P-like immunoreactivity in normal human brain and in Huntington's disease. Brain Research, 199, 147–160.Google Scholar
Emson, P. C., Arregui, A., Clement-Jones, V., Sandberg, B. E. B. & Rossor, M., Rehfeld, J. F., Langevin, H. & Rossor, M.(1980b) Reduction in cholecystokinin-like immunoreactivity in the basal ganglia in Huntington's disease. Brain Research, 198, 497–500.CrossRefGoogle ScholarPubMed
Enna, S. J., Bird, E. D., Bennett, J. P. Jr., Bylund, D. B., Yamamura, H. L., Iversen, L. L. & Snyder, S. H.(1976) Huntington's chorea: changes in neurotransmitter receptors in brain. New England Journal of Medicine, 294, 1305–1309.CrossRefGoogle Scholar
Epelbaum, J., Ruberg, M., Moyse, E., Javoy-Agid, F., Dubois, B. & Agid, Y.(1983) Somatostatin and dementia in Parkinson's disease. Brain Research, 278, 376–379.Google Scholar
Evarts, E. V., Teravainen, H. & Calne, D. B.(1981) Reaction time in Parkinson's disease. Brain, 104, 167–186.Google Scholar
Fairweather, D. S.(1947) Psychiatric aspects of the post-encephalitic syndrome. Journal of Mental Science, 93, 201–254.Google Scholar
Fisk, J. D., Goodale, M. A., Burkhart, G. & Barnett, H. J. M.(1982) Progressive supranuclear palsy: the relationship between ocular motor dysfunction and psychological test performance. Neurology, 32, 698–705.Google Scholar
Foster, N. L., Chase, T. N., Mansi, L., Brooks, R., Fedio, P., Patronas, N. T. & Di Chiro, G. (1984) Cortical abnormalities in Alzheimer's disease. Annals of Neurology, 16, 649–654.Google Scholar
Freedman, M., Rivoria, P., Butters, N., Sax, D. S. & Feldman, R. G.(1984) Retrograde amnesia in Parkinson's disease. Canadian Journal of Neurological Science, 11, 297–301.Google Scholar
Gainotti, G., Caltagirone, C., Masullo, C. & Miceli, G.(1980) Patterns of neuropsychologic impairment in various diagnostic groups of dementia. In Aging of the Brain and Dementia (eds. L. Amaducci, A. N. Davison, & P. Antuono). New York: Raven Press.Google Scholar
Gaspar, P. & Gray, F.(1984) Dementia in idiopathic Parkinson's disease. Acta Neuropathologica, 64, 43–52.Google Scholar
Garnett, E. S., Nahmias, C. & Firnau, G.(1984) Central dopaminergic pathways in hemiparkinsonism examined by positron emission tomography. Canadian Journal of Neurological Science, 11, 174–179.Google Scholar
Globus, M., Mildworf, B. & Melamed, E.(1985) Cerebral blood flow and cognitive impairment in Parkinson's disease. Neurology, 35, 1135–1139.CrossRefGoogle ScholarPubMed
Goldstein, N. P., Ewart, J. C., Randall, R. V. & Gross, J. B.(1968) Psychiatric aspects of Wilson's disease (hepatolenticular degeneration): results of psychometric tests during long-term therapy. American Journal of Psychiatry, 124, 1555–1561.Google Scholar
Goodman, R. M., Hall, C. L. Jr., Terango, L., Perine, G. A. Jr. & Roberts, P. L.(1966) Huntington's chorea. A multidisciplinary study of affected parents and first generation offspring. Archives of Neurology, 15, 345–355.Google Scholar
Graff-Radford, N. R., Eslinger, P. J., Damasio, A. R. & Yamada, T.(1984) Nonhemorrhagic infarction of the thalamus: behavioral, anatomic, and physiologic correlates. Neurology, 34, 14–23.Google Scholar
Graybiel, A. M. & Ragsdale, C. W. Jr.(1979) Fiber connections of the basal ganglia. Progress in Brain Research, 51, 239–283.Google Scholar
Greenfield, J. G.(1954) The Spinocerebellar Degenerations, Oxford: Blackwell Scientific Publications.Google Scholar
Graybiel, A. M. & Ragsdale, C. W. & Bosanquet, F. D.(1953) The brainstem lesions in parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 16, 223–226.Google Scholar
Guberman, A. & Stuss, D.(1983) The syndrome of bilateral paramedian thalamic infarction. Neurology, 33, 540–546.Google Scholar
Hakim, A. M. & Mathieson, G.(1979) Dementia in Parkinson disease: a neuropathologic study. Neurology, 29, 1209–1214.Google Scholar
Halgin, R., Riklan, M. & Misiak, H.(1977) Levodopa, parkinsonism, and recent memory. Journal of Nervous and Mental Disease, 164, 268–272.Google Scholar
Hart, R. P., Kwentus, J. A., Leshner, R. T. & Frazier, R.(1985) Information processing speed in Friedreich's ataxia. Armais of Neurology, 17, 612–614.Google Scholar
Heston, L. L.(1981) Genetic studies of dementia: with emphasis on Parkinson disease and Alzheimer's neuropathology. In The Epidemiology of Dementia (eds J. A. Mortimer & L. M. Schuman). New York; Oxford University Press.Google Scholar
Hier, D. B., Thomas, C. & Shindler, A. G.(1983) A case of subcortical dementia due to sarcoidosis of the hypothalamus and fornices. Brain and Cognition, 2, 189–198.Google Scholar
Hoehn, M. M., Crowley, T. J. & Rutledge, C. O.(1976) Dopamine correlates of neurological and psychological status in untreated parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 39. 941–951.Google Scholar
Horn, S.(1974) Some psychological factors in parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 37, 27–31.Google Scholar
Horne, D. J. Del, (1971) Performance on delayed response tasks by patients with parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 34, 192–194.Google Scholar
Hornykiewicz, O.(1973) Parkinson's disease: from brain homogenate to treatment. Federation Proceedings, 32, 183–190.Google Scholar
Iversen, S. D.(1984) Recent advances in the anatomy and chemistry of the limbic system. In Psychopharmacology of the Limbic System (eds M. R. Trimble & E. Zarifian). New York: Oxford University Press.Google Scholar
Jackson, J. A. & Immerman, S. L.(1919) A case of pseudosclerosis associated with a psychosis. Journal of Nervous and Mental Disease, 49, 5–13.Google Scholar
Jackson, J. A. & Immerman, S. L., Jankovic, J. & Ford, J.(1983) Progressive supranuclear palsy: clinical features and response to treatment in 16 patients. Annals of Neurology, 13, 273–278.Google Scholar
Janati, A. & Appel, A. R.(1984) Psychiatric aspects of progressive supranuclear palsy. Journal of Nervous and Mental Disease, 172, 85–89.Google Scholar
Jasper, H.(1949) Diffuse projection systems: the integrative action of the thalamic reticular system. Electroencephalography and Clinical Neurophysiology, 1, 403–420.Google Scholar
Javoy-Agid, F. & Agid, Y.(1980) Is the mesocortical dopaminergic system involved in Parkinson disease? Neurology, 30. 1326–1330.CrossRefGoogle ScholarPubMed
Javoy-Agid, F. & Agid, Y., Taquet, H., Ploska, A., Cherif-Zahar, C., Ruberg, M. & Agid, Y.(1981) Distribution of catecholamines in the ventral mesencephalon of human brain with special reference to Parkinson's disease. Journal of Neurochemistry, 36, 2101–2105.Google Scholar
Javoy-Agid, F. & Agid, Y., Taquet, H., Ploska, A., Cherif-Zahar, C., Ruberg, M. & Agid, Y., Ruberg, M., Pique, L., Bertagna, X., Taquet, H., Studler, J. M., Cesselin, F., Epelbaum, J. & Agid, Y.(1984) Biochemistry of the hypothalamus in Parkinson's disease. Neurology, 34. 672–675.Google Scholar
Johnson, T. N., Rosvold, H. E. & Mishkin, M.(1968) Projections from behaviorally-defined sections of the prefrontal cortex to the basal ganglia, septum, and diencephalon of the monkey. Experimental Neurology, 21, 20–34.Google Scholar
Josiassen, R. C., Curry, L., Roemer, R. A., DeBease, C. & Mancall, E. L.(1982) Patterns of intellectual defícit in Huntington's disease. Journal of Clinical Neuropsychology, 4, 173–183.Google Scholar
Katz, A., Naseem, A., Horoupian, D. S., Rothner, A. D. & Davies, P.(1984) Familial multisystem atrophy with possible thalamic dementia. Neurology, 34, 1213–1217.CrossRefGoogle ScholarPubMed
Keddie, K. M. G.(1969) Hereditary ataxia presumed to be of the Menzel type, complicated by paranoid psychosis, in a mother and two sons. Journal of Neurology, Neurosurgery and Psychiatry, 32. 82–87.Google Scholar
Kemp, J. M. & Powell, T. P. S.(1970) The cortico-striate projection of the monkey. Brain, 93, 525–546.Google Scholar
Kimura, D., Barnett, H. J. M. & Burkhart, G.(1981) The psychological test pattern in progressive supranuclear palsy. Neuropsychologia, 2, 301–306.Google Scholar
Kish, S. J., Chang, L. J., Mirchandani, L., Shannak, K. & Hornykiewicz, O.(1985) Progressive supranuclear palsy: relationship between extrapyramidal disturbances, dementia, and brain neurotransmitter markers. Annals of Neurology, 18, 530–536.Google Scholar
Knehr, C. A. & Bearn, A. G.(1956) Psychological impairment in Wilson's disease. Journal of Nervous and Mental Disease, 124, 251–255.Google Scholar
Krieg, W. J. S.(1966) Thalamic afferents to the frontal lobe: of the macaque. Journal of Neurology, 5, 317–329.Google Scholar
Kuhl, D. E., Phelps, M. E., Markham, C. H., Metter, E. J., Riege, W. H. & Winter, J.(1982) Cerebral metabolism and atrophy in Huntington's disease determined by 18FDG and computed tomographic scan. Annals of Neurology, 12, 425–434.Google Scholar
Kuhl, D. E., Phelps, M. E., Markham, C. H., Metter, E. J., Riege, W. H. & Winter, J., Metter, E. J. & Riege, W. H.(1984) Patterns of local cerebral glucose utilization determined in Parkinson's disease by the 18F fluorodeoxyglucose method. Annals of Neurology, 15, 411–424.Google ScholarPubMed
Lavy, S., Malamed, E., Cooper, G. Bentin, S. & Rinot, Y.(1979) Regional cerebral blood flow in patients with Parkinson's disease. Archives of Neurology, 36, 344–348.Google Scholar
Lees, A. J. & Smith, E.(1983) Cognitive deficits in the early stages of Parkinson's disease. Brain, 106, 257–270.Google Scholar
Lloyd, K. G. & Hornykiewicz, O.(1973) L-glutamic acid decarboxylase in Parkinson's disease: effect of L-dopa therapy. Nature, 243, 521–523.Google Scholar
Lloyd, K. G. & Hornykiewicz, O. Mohler, H., Heitz, Ph. & Bartholini, G.(1975) Distribution of choline acetyltransferase and glutamate decarboxylase within the substantia nigra and in other brain regions from control and parkinsonian patients. Journal of Neurochemistry, 25, 789–795.Google Scholar
Maher, E. R., Smith, E. M. & Lees, A. J.(1985) Cognitive deficits in the Steele-Richardson-Olszewski syndrome (progressive supranuclear palsy). Journal of Neurology, Neurosurgery, and Psychiatry, 48, 1234–1239.Google Scholar
Malamud, N.(1967) Psychiatric disorders with intracranial tumors of the limbic system. Archives of Neurology, 17, 113–123.Google Scholar
Mann, J. J., Stanley, M., Kaplan, R. D., Sweeney, J. & Neophytidies, A.(1983) Central catecholamine metabolism in vivo and the cognitive and motor deficits in Parkinson's disease. Journal of Neurology, Neurosurgery, and Psychiatry, 34, 209–218.Google Scholar
Marsh, G. G., Markham, C. M. & Ansel, R.(1971) Levodopa's awakening effect on patients with parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 34, 209–218.Google Scholar
Martin, W. R. W., Beckman, J. H., Calne, D. B., Adam, M. J., Harrop, R., Rogers, J. G., Ruth, T. J., Sayre, C. I. & Pate, B. D.(1984) Cerebral glucose metabolism in Parkinson's disease. Canadian Journal of Neurological Science, 11, 169–173.Google Scholar
Martone, M., Butters, N., Payne, M., Becker, J. T. & Sax, D. S.(1984) Dissociations between skill learning and verbal recognition in amnesia and dementia. Archives of Neurology, 41, 965–970.Google Scholar
Matison, R., Mayeux, R., Rosen, J. & Fahn, S.(1982) “Tip-of- the-tongue” phenomenon in Parkinson's disease. Neurology, 32, 567–570.Google Scholar
Mayeux, R.(1983) Emotional changes associated with basal ganglia disorders. In Neuropsychology of Human Emotion (eds K. M. Heilman & P. Satz). New York: The Guildford Press.Google Scholar
Mayeux, R., Stern, Y., Rosen, J. & Leventhal, J.(1981) Depression, intelleaual impairment, and Parkinson's disease. Neurology, 31, 645–650.Google Scholar
Mayeux, R., Stern, Y., Rosen, J. & Leventhal, J. & Benson, D. F. (1983) Is ‘subcortical dementia’ a recognizeable clinical entity? Annals of Neurology, 14, 278–283.Google Scholar
Mayeux, R., Stern, Y., Rosen, J. & Leventhal, J. Benson, D. F., Cote, L. & Williams, B. W.(1984) Altered serotonin metabolism in depressed patients with Parkinson's disease. Neurology, 34, 642–646.Google Scholar
McGeer, P. L. & McGeer, E. G.(1971) Cholinergic enzyme systems in Parkinson's disease. Archives of Neurology, 25, 265–268.Google Scholar
McGeer, P. L. & McGeer, E. G.(1976) Enzymes associated with the metabolism of catecholamines, acetylcholine and GABA in human controls and patients with Parkinson's disease and Huntington's chorea. Journal of Neurochemistry, 26, 65–76.Google Scholar
McGeer, P. L. & McGeer, E. G. Wada, J. A. & Jung, E.(1971) Effects of globus pallidus lesions and Parkinson's disease on brain glutamic acid decarboxylase. Brain Research, 32, 425–431.Google Scholar
McHugh, P. R. & Folstein, M. F.(1975) Psychiatric syndromes of Huntington's chorea: a clinical and phenomenologic study. In Psychiatric Aspects of Neurologic Disease (eds D. F. Benson & D. Blumer). New York: Grune & Stratton.Google Scholar
Mesuiam, M-M., Mufson, E. J., Levey, A. I. & Wainer, B. H.(1983) Cholinergic innervation of cortex by the basal forebrain: cytochemistry and cortical connections of the septal area, diagonal band nuclei, nucleus basalis (substantia innominata), and hypothalamus in the rhesus monkey. Journal of Comparative Neurology, 214, 170–197.Google Scholar
Mindham, R. H. S.(1970) Psychiatric symptoms in parkinsonism. Journal of Neurology, Neurosurgery, and Psychiatry, 33, 188–191.Google Scholar
Monfort, J. C., Javoy-Agid, F., Hauw, J. J., Dubois, B. & Agid, Y.(1985) Brain glutamate decarboxylase in Parkinson's disease with particular reference to a premortem severity index. Brain, 108, 301–313.Google Scholar
Mortimer, J. A., Pirozzolo, F. J., Hansch, E. C. & Webster, D. D.(1982) Relationship of motor symptoms to intellectual deficits in Parkinson's disease. Neurology, 32, 133–137.Google Scholar
Nakano, H. & Hirano, A.(1984) Parkinson's disease: neuron loss in the nucleus basalis without concomitant Alzheimer's disease. Annals of Neurology, 15, 415–418.Google Scholar
Nauta, W. J. H.(1982) Limbic innervation of the striatum. In Gilles de la Tourette Syndrome (eds A. J. Friedhoff & T. N. Chase). New York: Raven Press.Google Scholar
Nauta, W. J. H. & Domesick, V. B.(1978) Crossroads of limbic and striatal circuitry: hypothalamonigral connections. In Limbic Mechanisms (eds K. E. Livingston & O. Hornykiewicz). New York: Plenum Press.Google Scholar
Nauta, W. J. H. & Domesick, V. B.(1981) Ramifications of the limbic system. In Psychiatry and the Biology of the Human Brain (ed. S. Matthysse). New York: Elsevier/North-Holland.Google Scholar
Oppenheimer, D. R.(1976) Diseases of the basal ganglia, cerebellum and motor neurons. In Greenfield's Neuropathology (eds W. Blackwood & J. A. N. Corsellis). Chicago: Yearbook Medical Publishers.Google Scholar
Pandey, R. S., Sreenivas, K. N., Patih, N. M. & Swamy, H. S.(1981) Dopamine-B-hydroxylase in a patient with Wilson's disease and mania. American Journal of Psychiatry, 138, 1628–1629.Google Scholar
Penny, J. B. Jr. & Young, A. B.(1982) Quantitative autoradiography of neurotransmitter receptors in Huntington's disease. Neurology, 32, 1391–1395.Google Scholar
Perry, R. H., Tomlinson, B. E., Candy, J. M., Blessed, G., Foster, J. F., Bloxham, C. A. & Perry, E. R.(1983) Cortical cholinergic deficit in mentally impaired parkinsonian patients. Lancet, 2, 789–790.Google Scholar
Perry, T. L., Hansen, S. & Kloster, M.(1973) Huntington's chorea: deficiency of γ-aminobutyric acid in brain. New England Journal of Medicine, 288, 337–342.Google Scholar
Pilleri, G., Pietrini, V., Tagliavini, F., Trabattoni, F. & Lechi, A. (1981) “Thalamic” dementia in herpes encephalitis: clinicopathological report. Acta Neuropathologica, (Suppl) 7, 156–159.Google Scholar
Pirozzolo, F. J., Hansch, E. C., Mortimer, J. A., Webster, D. D. & Kuskowski, M. A.(1982) Dementia in Parkinson disease: a neuropsychological analysis. Brain and Cognition, 1, 71–83.Google Scholar
Price, K. S., Farley, I. J. & Hornykiewicz, O.(1978) Neurochemistry of Parkinson's disease: relation between striatal and limbic dopamine. Advances in Biochemical Psychopharmacology, 19, 293–300.Google Scholar
Proctor, F., Riklan, M., Cooper, I. S. & Teuber, H-L(1964) Judgement of visual and postural vertical by parkinsonian patients. Neurology, 14, 287–293.Google Scholar
Rafal, R. D. & Grimm, R. J.(1981) Progressive supranuclear palsy: functional analysis of the response to methysergide and antiparkinsonian agents. Neurology, 31, 1507–1518.Google Scholar
Rafal, R. D. & Grimm, R. J., Posner, M. L., Walker, J. A. & Friedreich, F. J.(1984) Cognition and the basal ganglia. Brain, 107, 1083–1094.Google Scholar
Reitan, R. M. & Boll, T. J.(1971) Intellectual and cognitive functions in Parkinson's disease. Journal of Consulting and Clinical Psychology, 37, 364–369.Google Scholar
Robins, A. H.(1976) Depression in patients with parkinsonism. British Journal of Psychiatry, 128, 141–145.Google Scholar
Rosenbaum, D.(1941) Psychosis with Huntington's chorea. Psychiatric Quarterly, 15, 93–99.Google Scholar
Rosenberg, C., Nadleman, K. & Starr, A.(1985) Cognitive evoked potentials (P300) in early Huntington's disease. Archives of Neurology, 42, 984–987.Google Scholar
Ruberg, M., Ploska, A., Javoy-Agid, F. & Agid, Y.(1982) Muscarinic binding and choline acetyltransferase activity in parkinsonian subjects with reference to dementia. Brain Research, 232, 129–139.Google Scholar
Ruberg, M., Ploska, A., Javoy-Agid, F. & Agid, Y., Javoy-Agid, F., Hirsch, E., Scatton, B., L'Heureux, R., Hauw, J. J., Duyckaerts, C., Gray, F., Morel-Maroger, A., Rascol, A., Serdaru, M. & Agid, Y.(1985) Dopaminergic and cholinergic lesions in progressive supranuclear palsy. Annals of Neurology, 18, 523–529.Google Scholar
Sax, D. S., O'Donnell, B., Butters, N., Menzer, L., Montgomery, K. & Kayne, H. L.(1983) Computed tomographic, neurologic and neuropsychological correlates of Huntington's disease. International Journal of Neuroscience, 18, 21–36.Google Scholar
Scatton, B., Rouquier, E., Javoy-Agid, J. & Agid, Y.(1982) Dopamine deficiency in the cerebral cortex in Parkinson disease. Neurology, 32, 1039–1040.Google Scholar
Scatton, B., Rouquier, E., Javoy-Agid, J. & Agid, Y., Javoy-Agid, F., Rouquier, E., Dubois, B. & Agid, Y.(1983) Reduction of cortical dopamine, noradrenalin, serotonin, and their metabolites in Parkinson's disease. Brain Research, 275, 321–328.Google Scholar
Schoenfeld, M., Myers, R. H., Cupples, L. A., Berkman, B., Sax, D. S. & Clark, E.(1984) Increased rate of suicide among patients with Huntington's disease. Journal of Neurology, Neurosurgery, and Psychiatry, 47, 1283–1287.Google Scholar
Schulman, S.(1957) Bilateral symmetrical degeneration of the thalamus. Journal of Neuropathology and Experimental Neurology, 16, 446–470.Google Scholar
Shepherd, M.(1955) Report of a family suffering from Friedreich's disease, peroneal muscular atrophy, and schizophrenia. Journal of Neurology, Neurosurgery, and Psychiatry, 18, 297–304.Google Scholar
Smith, W. T.(1976) Intoxications, poisons and related metabolic disorders. In Greenfield's Neuropathology (eds W. Blackwood & J. A. N. Corsellis). Chicago: Yearbook Medical Publishers.Google Scholar
Steele, J. C., Richardson, J. C. & Olszewski, J.(1964) Progressive supranuclear palsy. Archives of Neurology, 10, 333–359.Google Scholar
Stern, K.(1939) Severe dementia associated with bilateral symmetrical degeneration of the thalamus. Brain, 62, 157–171.Google Scholar
Stern, Y. & Langston, J. W.(1985) Intellectual changes in patients with MPTP-induced parkinsonism. Neurology, 35, 1506–1509.Google Scholar
Stern, Y. & Langston, J. W., Mayeux, R. & Cote, L.(1984) Reaction time and vigilance in Parkinson's disease. Archives of Neurology, 41, 1086–1089.Google Scholar
Studler, J. M., Javoy-Agid, F., Cesselin, F., Legrand, J. C. & Agid, Y.(1982) CCK-8-immunoreactivity in human brain: selective decrease in the substantia nigra from parkinsonian patients. Brain Research, 243, 176–179.Google Scholar
Tagliavini, F., Pilleri, G., Bouras, C. & Constantinidis, J.(1984) The basal nucleus of Meynert in patients with progressive supranuclear palsy. Neuroscience Letters, 44, 37–42.Google Scholar
Talland, G. A. & Schwab, R. S.(1964) Performance with multiple sets in Parkinson's disease. Neuropsychologia, 2, 45–53.CrossRefGoogle Scholar
Taquet, H., Javoy-Agid, F., Cesselin, F., Hamon, M., Legrand, J. C. & Agid, Y.(1982) Microphotography of methionine- enkephalin, dopamine and noradrenalin in the vental mesencephalon of human control and parkinsonian brains. Brain Research, 235, 303–314.Google Scholar
Trauner, D. A.(1985) Olivopontocerebellar atrophy with dementia, blindness, and chorea. Archives of Neurology, 42, 757–758.Google Scholar
Trimble, M. R. & Cummings, J. L.(1981) Neuropsychiatric disturbances following brainstem lesions. British Journal of Psychiatry, 138, 56–59.Google Scholar
Tweedy, J. R., Langer, K. G. & McDowell, F. H.(1982) The effect of semantic relations on the memory deficit associated with Parkinson's disease. Journal of Clinical Neuropsychology, 4, 235–247.Google Scholar
Villardita, C., Smirni, P. & Zappala, G.(1983) Visual neglect in Parkinson's disease. Archives of Neurology, 40, 737–739.Google Scholar
Walker, A. E.(1937) The thalamus in relation to the cerebral cortex. Journal of Nervous and Mental Disease, 85, 249–259.Google Scholar
Warburton, J. W.(1967) Memory disturbance and the Parkinson syndrome. British Journal of Medical Psychology, 40, 169–171.Google Scholar
Wastek, G. J. & Yamamura, H. I.(1978) Biochemical characterization of the muscarinic cholinergic receptor in human brain: alterations in Huntington's disease. Molecular Pharmacology, 14, 768–780.Google Scholar
Whitehouse, P. J. Price, D. L., Clark, A. W., Coyle, J. T. & Delong, M. R.(1981) Alzheimer disease: evidence for selective loss of cholinergic neurons in the nucleus basalis. Annals of Neurology, 10, 122–126.Google Scholar
Whitehouse, P. J. Price, D. L., Clark, A. W., Coyle, J. T. & Delong, M. R., Hedreen, J. C., White, C. L. III & Price, P. L.(1983) Basal forebrain neurons in the dementia of Parkinson disease. Annals of Neurology, 13, 243–248.Google Scholar
Whitehouse, P. J. Price, D. L., Clark, A. W., Coyle, J. T. & Delong, M. R., Hedreen, J. C., White, C. L. III & Price, P. L., Trifiletti, R. R., Jones, B. E., Folstein, S., Price, D. L., Snyder, S. H. & Kuhar, M. J.(1985) Neurotransmitter receptor alterations in Huntington's disease: autoradiographic and homogenate studies with special reference to benzodiazepine receptor complexes. Annals of Neurology, 18, 202–210.Google Scholar
Wilson, R. S., Kaszniak, A. W., Klawans, H. L. & Garron, D. C.(1980) High speed memory scanning in parkinsonism. Cortex, 16. 67–72.Google Scholar
Wilson, S. A. K.(1912) Progressive lenticular degeneration: a familial nervous disease associated with cirrhosis of the liver. Brain, 34, 295–509.Google Scholar
Yakovlev, P. I.(1948) Motility, behavior, and the brain. Journal of Nervous and Mental Disease, 107, 313–325.Google Scholar
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