0
January 1987, Vol 44, No. 1 >
Article | January 1987

Predictability of Phenotype in Huntington's Disease

P. Michael Conneally, PhD; Lindsay A. Farrer, PhD
[+] Author Affiliations

Accepted for publication Aug 13, 1986.

Read in part at the annual meeting of the American Society of Human Genetics, Salt Lake City, Oct 11, 1985.

Reprint requests to Department of Human Genetics, Yale University School of Medicine, 333 Cedar St, New Haven, CT 06510 (Dr Farrer).


Arch Neurol. 1987;44(1):109-113. doi:10.1001/archneur.1987.00520130083023
Text Size: A A A
Published online
Article
References
Comments

• Huntington's disease (HD) is an autosomal dominant disorder with variable age at onset and variable symptoms. Results from an analysis of questionnaire data on ages at onset and death, sex of the affected parent, and motor disorder in 624 patients gave no evidence of discrete phenotypes, as suggested in the literature. The tendency for muscular rigidity (instead of chorea), an accelerated natural history and paternal transmission, each of which is often associated with the juvenile-onset form of HD, is inversely related to age at onset in the affected child. The most parsimonious explanation for clinical variability in HD is that all HD types are part of a continuum, although expression of the juvenile form may be partly determined by a maternally transmitted factor. Evidence for accelerated aging in HD and correlations between age at onset and death in patients with HD and longevity in their unaffected relatives warrant further investigation into the relationship between aging and phenotypic expression of HD.

REFERENCES

1 +
Hayden MR: Huntington's Chorea . New York, Springer-Verlag NY Inc, 1981;.
2 +
Bruyn GW:  Huntington's chorea: Historical, clinical and laboratory synopsis , in Bruyn GW, Vinken PR (eds): Handbook of Clinical Neurology . New York, John Wiley & Sons Inc, 1968;, vol 6, pp 298-378.
3 +
Edmonds C:  Huntington's chorea, dysphagia and death . Med J Aust 1966;;2:273-274.
4 +
Heathfield KWG:  Huntington's chorea: Investigation into prevalence of this disease in the area covered by the North East Metropolitan Hospital Board . Brain 1967;;90:203-232.
5 +
Leigh RJ, Newman SA, Folstein SE, et al:  Abnormal ocular motor control in Huntington's disease . Neurology 1983;;33:1268-1275.
6 +
Hayden MR: Huntington's Chorea in South Africa , dissertation, Cape Town, South Africa, 1979;.
7 +
Martin JP: The Basal Ganglia and Posture . London, Pitman Books, 1967;.
8 +
Lieberman A, Neophytides A, Casson I, et al:  Huntington's disease . NY State J Med 1979;;79:1188-1190.
9 +
Fisher JM, Kennedy KL, Caine ED, et al:  Dementia in Huntington's disease: A crosssectional analysis of intellectual decline , in Mayeux R, Rosen WG (eds): Advances in Neurology . New York, Raven Press, 1983;, vol 38: The Dementias, pp 229-238.
10 +
Folstein SE, Abbott MH, Chase GA, et al:  The association of affective disorder with Huntington's disease in a case series and in families . Psychol Med 1983;;13:537-542.
11 +
Dewhurst K, Oliver JE, McKnight AL:  Socio-psychiatric consequences of Huntington's disease . Br J Psychiatry 1970;;116:255-258.
12 +
Caine ED, Shoulson I:  Psychiatric syndromes in Huntington's disease . Am J Psychiatry 1983;;140:728-733.
13 +
Stevens DL: Huntington's Chorea: A Demographic, Genetic and Clinical Study, MD thesis, London, 1976.
14 +
Sanberg PR, Fibiger HC, Mark RF:  Body weight and dietary factors in Huntington's disease patients compared with matched controls . Med J Aust 1981;;1:407-409.
15 +
Davenport CB, Muncey EB:  Huntington's chorea in relation to heredity and eugenics . Am J Insan 1916;;73:195-222.
16 +
Myrianthopoulos NC:  Review article: Huntington's chorea . J Med Genet 1966;;3:298-314.
17 +
Chandler JH, Reed TE, DeJong RN:  Huntington's chorea in Michigan: III. Clinical observations . Neurology 1960;;10:148-153.
18 +
Von Kehrer FA:  Der Erbveitstanz (Huntingische Chorea): Allgemeiner und klinischer Teil , in Gutt A (ed): Handbuch der Erbkrankheiten . Stuttgart, West Germany, Georg Thieme Verlag, vol 3, 1940;.
19 +
Bittenbender JB, Quadfasel FA:  Rigid and akinetic forms of Huntington's chorea . Arch Neurol 1962;;7:275-288.
20 +
Stevens DL:  The classification of variants of Huntington's chorea , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, 1973;, vol 1: Huntington's Disease, pp 57-64.
21 +
Hamilton AS:  A report of 27 cases of chronic progressive chorea . Am J Insan 1908;;64:403-475.
22 +
Westphal C:  Über eine dem Bilde der cerebrospinalen grauen Degeneration ähnliche Erkrankung des zentralen Nervensystems ohne anatomischen Befund, nebst einigen bemerkunger über paradoxe Kontraktion . Arch Psych Nervenkr 1883;;14:87-96, 767-773.
23 +
Bell J:  Huntington's chorea , in Fisher RA (ed): The Treasury of Human Inheritance . New York, Cambridge University Press, 1934;, vol 4, pp 1-77.
24 +
Bird TD, Omenn GS:  Monozygotic twins with Huntington's chorea in a family expressing the rigid variant . Neurology 1975;;25:1126-1129.
25 +
Heathfield KWG:  Huntington's chorea: A centenary review . Postgrad Med J 1973;;49:32-45.
26 +
Brackenridge CJ:  The relation of type of initial symptoms and line of transmission to age at onset and death in Huntington's disease . Clin Genet 1971;;2:287-297.
27 +
Brackenridge CJ:  Relation of parental age to rigidity in Huntington's disease . J Med Genet 1974;;11:136-140.
28 +
Lyon JW:  Chronic hereditary chorea . Am Med Times 1863;;7:289-290.
29 +
Merritt AD, Conneally PM, Rahman NF, et al:  Juvenile Huntington's chorea , in Barbeau A, Brunette J (eds): Progress in Neurogenetics . Princeton, NJ, Exerpta Medica, 1969;, pp 645-650.
30 +
Haslam RHA, Curry B, Johns R:  Infantile Huntington's disease . Can J Neurol Sci 1983;;10:200-203.
31 +
Hansotia P, Cleeland CS, Chun RWM:  Juvenile Huntington's chorea . Neurology 1968;;18:217-224.
32 +
Pericak-Vance MA: Genetic Linkage Studies in Huntington's Disease , dissertation, Indiana University, Bloomington, 1978;.
33 +
Boehnke M, Conneally PM, Lange K:  Two models for a maternal factor in the inheritance of Huntington's disease . Am J Hum Genet 1983;;35:845-860.
34 +
Myers RH, Goldman D, Bird ED, et al:  Maternal transmission in Huntington's disease . Lancet 1983;;1:208-210.
35 +
Myers RH, Cupples LA, Schoenfeld M, et al:  Maternal factors in onset of Huntington's disease . Am J Hum Genet 1985;;37:511-523.
36 +
Farrer LA, Conneally PM:  A genetic model for age at onset in Huntington's disease . Am J Hum Genet 1985;;37:350-357.
37 +
Finch CE:  The relationship of aging changes in the basal ganglia to manifestations of Huntington's chorea . Ann Neurol 1980;;7:406-410.
38 +
Ross RA, Judd AB, Pickel VM, et al:  Strain-dependent variations in number of mid-brain dopaminergic neurones . Nature 1976;;264:654-656.
39 +
Severson JA, Randall PK, Finch CE:  Genotypic influences on striatal dopaminergic regulation in mice . Brain Res 1981;;210:201-215.
40 +
Finch CE:  Rodent models for aging processes in the human brain , in Corkin S, Davis SL, Growdin JH, et al (eds): Aging . New York, Raven Press, 1982;, vol 19: Alzheimer's Disease: A Report of Progress, pp 249-257.
41 +
Farrer LA, Conneally PM, Yu PL:  The natural history of Huntington's disease: Possible role of 'aging genes.' Am J Med Genet 1984;;18:115-123.
42 +
Haldane JBS:  The relative importance of principal and modifying genes in determining some human diseases . J Genet 1941;;41:149-157.
43 +
Conneally PM, Gersting JM, Wexler NS:  Huntington's disease: A research roster of HD patients and their families, abstracted . Am J Hum Genet 1980;;32:103A.
44 +
Turner TH:  Huntington's chorea without dementia: A problem case . Br J Psychiatry 1985;;146:548-550.
45 +
Brackenridge CJ:  A genetic and statistical study of some sex-related factors in Huntington's disease . Clin Genet 1971;;2:267-286.
46 +
Brackenridge CJ:  The relation of sex of affected parent to the age at onset of Huntington's disease . J Med Genet 1973;;10:333-336.
47 +
Brackenridge CJ:  The relation of birth order to age at onset of Huntington's disease . Hum Hered 1972;;22:584-587.
48 +
Brackenridge CJ:  The relation of some features of Huntington's disease to the age at onset of symptoms in parents . Clin Genet 1972;;3:477-482.
49 +
Brackenridge CJ:  Parental factors associated with rigidity in Huntington's disease . J Med Genet 1980;;17:112-114.
50 +
Van Dijk JG, Van der Velde EA, Roos RAC, et al:  Juvenile Huntington's disease . Hum Genet 1986;;73:235-239.
51 +
Brackenridge CJ, Chamberlin M:  The relation of the sex of choreic and rigid subjects to the age at onset of Huntington's disease . Clin Genet 1974;;5:248-253.
52 +
Brackenridge CJ:  Prognostic factors of Huntington's disease of early onset . Eur Neurol 1982;;21:112-116.
53 +
Farrer LA: Genetic and Anthropometric Studies of Aging in Huntington Disease , dissertation, Indiana University, Bloomington, 1985;.
54 +
Brackenridge CJ:  Factors influencing dementia and epilepsy in Huntington's disease of early onset . Acta Neurol Scand 1980;;62:305-311.
55 +
Bird MT, Paulson G:  The rigid form of Huntington's chorea . Neurology 1971;;21:271-276.
56 +
Liss L, Paulson G, Sommer A:  Rigid form of Huntington's chorea: A clinopathologic study of three cases , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, 1973;, vol 1: Huntington's Disease, pp 405-424.
57 +
Markham C:  Huntington's chorea in childhood , in Barbeau A, Brunette J (eds): Progress in Neurogenetics . Princeton, NJ, Exerpta Medica, 1969;, pp 651-660.
58 +
Oliver JE, Dewhurst K:  Childhood and adolescent forms of Huntington's disease . Neurology 1969;;32:455-459.
59 +
Brackenridge CJ, Teltscher B:  Estimation of the age at onset of Huntington's disease from factors associated with the affected parent . J Med Genet 1975;;12:64-69.
60 +
Jervis G:  Huntington's chorea in childhood . Arch Neurol 1963;;9:244-257.
61 +
Folstein SE, Abbott MH, Franz ML, et al:  Phenotypic heterogeneity in Huntington's disease . J Neurogenet 1985;;1:175-184.
62 +
Gusella JF, Wexler NS, Conneally PM, et al:  A polymorphic DNA marker genetically linked to Huntington's disease . Nature 1983;;306:234-238.
63 +
Hayden MR, Soles JA, Ward RH:  Age of onset in siblings of persons with juvenile Huntington's disease . Clin Genet 1985;;28:100-105.
64 +
Faught E, Falgout JC, Leli DA:  Late-onset variant of Huntington's chorea . South Med J 1983;;76:1266-1270.
65 +
Myers RH, Sax DS, Schoenfeld M, et al:  Late onset of Huntington's disease . J Neurol Neurosurg Psychiatry 1985;;48:530-534.
66 +
Martin GM:  Genetic syndromes in man with a potential relevance to the pathobiology of aging , in Bergsma D, Lenz W (eds): Birth Defects . New York, Alan R Liss Inc, 1978;, vol 14, pp 5-39.
67 +
Burch PRJ:  Genetic and pathogenetic implications of the age incidence of Huntington's chorea , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, vol 1: Huntington's Disease, 1973;, pp 179-190.
68 +
Podolsky S, Leopold NA, Sax DS:  Increased frequency of diabetes mellitus in patients with Huntington's chorea . Lancet 1972;;1:1356-1358.
69 +
 National Diabetes Data Group: Classification and diagnosis of diabetes mellitus and other categories of glucose intolerance . Diabetes 1979;;28:1039-1057.
70 +
Farrer LA:  Diabetes mellitus in Huntington's disease . Clin Genet 1985;;27:62-67.
71 +
Sanberg PR, Fibiger HC:  Body weight, feeding, and drinking behaviors in rats with kainic acid-induced lesions with striatal neurons, with a note on body weight symptomatology in Huntington's disease . Exp Neurol 1979;;66:444-466.
72 +
Farrer LA, Meaney FJ:  Anthropometric assessment of Huntington's disease patients and families . Am J Phys Anthropol 1985;;67:185-194.
73 +
Tellez-Nagel I, Johnson AB, Terry AD:  Studies on brain biopsies of patients with Huntington's chorea . J Neuropathol Exp Neurol 1974;;33:308-332.
74 +
Brizzee KR:  Aging changes in relation to diseases of the nervous system , in Ordy JM, Brizzee KR (eds): Neurobiology of Aging . New York, Plenum Publishing Corp, 1975;.
75 +
Oepen H:  Paroxysmale Storungen bei der Huntingtonschen Chorea . Arch Psych Nervenkr 1963;;204:245-261.
76 +
Stahl SM, Faull KF, Barchas JD, et al:  CSF monoamine metabolites in movement disorders and normal aging . Arch Neurol 1985;;42:166-169.
77 +
Stahl SM, Thiemann S, Faull KF, et al:  Neurochemistry of dopamine in Huntington's dementia and normal aging . Arch Gen Psychiatry 1986;;43:161-164.

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Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Hayden MR: Huntington's Chorea . New York, Springer-Verlag NY Inc, 1981;.
2 +
Bruyn GW:  Huntington's chorea: Historical, clinical and laboratory synopsis , in Bruyn GW, Vinken PR (eds): Handbook of Clinical Neurology . New York, John Wiley & Sons Inc, 1968;, vol 6, pp 298-378.
3 +
Edmonds C:  Huntington's chorea, dysphagia and death . Med J Aust 1966;;2:273-274.
4 +
Heathfield KWG:  Huntington's chorea: Investigation into prevalence of this disease in the area covered by the North East Metropolitan Hospital Board . Brain 1967;;90:203-232.
5 +
Leigh RJ, Newman SA, Folstein SE, et al:  Abnormal ocular motor control in Huntington's disease . Neurology 1983;;33:1268-1275.
6 +
Hayden MR: Huntington's Chorea in South Africa , dissertation, Cape Town, South Africa, 1979;.
7 +
Martin JP: The Basal Ganglia and Posture . London, Pitman Books, 1967;.
8 +
Lieberman A, Neophytides A, Casson I, et al:  Huntington's disease . NY State J Med 1979;;79:1188-1190.
9 +
Fisher JM, Kennedy KL, Caine ED, et al:  Dementia in Huntington's disease: A crosssectional analysis of intellectual decline , in Mayeux R, Rosen WG (eds): Advances in Neurology . New York, Raven Press, 1983;, vol 38: The Dementias, pp 229-238.
10 +
Folstein SE, Abbott MH, Chase GA, et al:  The association of affective disorder with Huntington's disease in a case series and in families . Psychol Med 1983;;13:537-542.
11 +
Dewhurst K, Oliver JE, McKnight AL:  Socio-psychiatric consequences of Huntington's disease . Br J Psychiatry 1970;;116:255-258.
12 +
Caine ED, Shoulson I:  Psychiatric syndromes in Huntington's disease . Am J Psychiatry 1983;;140:728-733.
13 +
Stevens DL: Huntington's Chorea: A Demographic, Genetic and Clinical Study, MD thesis, London, 1976.
14 +
Sanberg PR, Fibiger HC, Mark RF:  Body weight and dietary factors in Huntington's disease patients compared with matched controls . Med J Aust 1981;;1:407-409.
15 +
Davenport CB, Muncey EB:  Huntington's chorea in relation to heredity and eugenics . Am J Insan 1916;;73:195-222.
16 +
Myrianthopoulos NC:  Review article: Huntington's chorea . J Med Genet 1966;;3:298-314.
17 +
Chandler JH, Reed TE, DeJong RN:  Huntington's chorea in Michigan: III. Clinical observations . Neurology 1960;;10:148-153.
18 +
Von Kehrer FA:  Der Erbveitstanz (Huntingische Chorea): Allgemeiner und klinischer Teil , in Gutt A (ed): Handbuch der Erbkrankheiten . Stuttgart, West Germany, Georg Thieme Verlag, vol 3, 1940;.
19 +
Bittenbender JB, Quadfasel FA:  Rigid and akinetic forms of Huntington's chorea . Arch Neurol 1962;;7:275-288.
20 +
Stevens DL:  The classification of variants of Huntington's chorea , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, 1973;, vol 1: Huntington's Disease, pp 57-64.
21 +
Hamilton AS:  A report of 27 cases of chronic progressive chorea . Am J Insan 1908;;64:403-475.
22 +
Westphal C:  Über eine dem Bilde der cerebrospinalen grauen Degeneration ähnliche Erkrankung des zentralen Nervensystems ohne anatomischen Befund, nebst einigen bemerkunger über paradoxe Kontraktion . Arch Psych Nervenkr 1883;;14:87-96, 767-773.
23 +
Bell J:  Huntington's chorea , in Fisher RA (ed): The Treasury of Human Inheritance . New York, Cambridge University Press, 1934;, vol 4, pp 1-77.
24 +
Bird TD, Omenn GS:  Monozygotic twins with Huntington's chorea in a family expressing the rigid variant . Neurology 1975;;25:1126-1129.
25 +
Heathfield KWG:  Huntington's chorea: A centenary review . Postgrad Med J 1973;;49:32-45.
26 +
Brackenridge CJ:  The relation of type of initial symptoms and line of transmission to age at onset and death in Huntington's disease . Clin Genet 1971;;2:287-297.
27 +
Brackenridge CJ:  Relation of parental age to rigidity in Huntington's disease . J Med Genet 1974;;11:136-140.
28 +
Lyon JW:  Chronic hereditary chorea . Am Med Times 1863;;7:289-290.
29 +
Merritt AD, Conneally PM, Rahman NF, et al:  Juvenile Huntington's chorea , in Barbeau A, Brunette J (eds): Progress in Neurogenetics . Princeton, NJ, Exerpta Medica, 1969;, pp 645-650.
30 +
Haslam RHA, Curry B, Johns R:  Infantile Huntington's disease . Can J Neurol Sci 1983;;10:200-203.
31 +
Hansotia P, Cleeland CS, Chun RWM:  Juvenile Huntington's chorea . Neurology 1968;;18:217-224.
32 +
Pericak-Vance MA: Genetic Linkage Studies in Huntington's Disease , dissertation, Indiana University, Bloomington, 1978;.
33 +
Boehnke M, Conneally PM, Lange K:  Two models for a maternal factor in the inheritance of Huntington's disease . Am J Hum Genet 1983;;35:845-860.
34 +
Myers RH, Goldman D, Bird ED, et al:  Maternal transmission in Huntington's disease . Lancet 1983;;1:208-210.
35 +
Myers RH, Cupples LA, Schoenfeld M, et al:  Maternal factors in onset of Huntington's disease . Am J Hum Genet 1985;;37:511-523.
36 +
Farrer LA, Conneally PM:  A genetic model for age at onset in Huntington's disease . Am J Hum Genet 1985;;37:350-357.
37 +
Finch CE:  The relationship of aging changes in the basal ganglia to manifestations of Huntington's chorea . Ann Neurol 1980;;7:406-410.
38 +
Ross RA, Judd AB, Pickel VM, et al:  Strain-dependent variations in number of mid-brain dopaminergic neurones . Nature 1976;;264:654-656.
39 +
Severson JA, Randall PK, Finch CE:  Genotypic influences on striatal dopaminergic regulation in mice . Brain Res 1981;;210:201-215.
40 +
Finch CE:  Rodent models for aging processes in the human brain , in Corkin S, Davis SL, Growdin JH, et al (eds): Aging . New York, Raven Press, 1982;, vol 19: Alzheimer's Disease: A Report of Progress, pp 249-257.
41 +
Farrer LA, Conneally PM, Yu PL:  The natural history of Huntington's disease: Possible role of 'aging genes.' Am J Med Genet 1984;;18:115-123.
42 +
Haldane JBS:  The relative importance of principal and modifying genes in determining some human diseases . J Genet 1941;;41:149-157.
43 +
Conneally PM, Gersting JM, Wexler NS:  Huntington's disease: A research roster of HD patients and their families, abstracted . Am J Hum Genet 1980;;32:103A.
44 +
Turner TH:  Huntington's chorea without dementia: A problem case . Br J Psychiatry 1985;;146:548-550.
45 +
Brackenridge CJ:  A genetic and statistical study of some sex-related factors in Huntington's disease . Clin Genet 1971;;2:267-286.
46 +
Brackenridge CJ:  The relation of sex of affected parent to the age at onset of Huntington's disease . J Med Genet 1973;;10:333-336.
47 +
Brackenridge CJ:  The relation of birth order to age at onset of Huntington's disease . Hum Hered 1972;;22:584-587.
48 +
Brackenridge CJ:  The relation of some features of Huntington's disease to the age at onset of symptoms in parents . Clin Genet 1972;;3:477-482.
49 +
Brackenridge CJ:  Parental factors associated with rigidity in Huntington's disease . J Med Genet 1980;;17:112-114.
50 +
Van Dijk JG, Van der Velde EA, Roos RAC, et al:  Juvenile Huntington's disease . Hum Genet 1986;;73:235-239.
51 +
Brackenridge CJ, Chamberlin M:  The relation of the sex of choreic and rigid subjects to the age at onset of Huntington's disease . Clin Genet 1974;;5:248-253.
52 +
Brackenridge CJ:  Prognostic factors of Huntington's disease of early onset . Eur Neurol 1982;;21:112-116.
53 +
Farrer LA: Genetic and Anthropometric Studies of Aging in Huntington Disease , dissertation, Indiana University, Bloomington, 1985;.
54 +
Brackenridge CJ:  Factors influencing dementia and epilepsy in Huntington's disease of early onset . Acta Neurol Scand 1980;;62:305-311.
55 +
Bird MT, Paulson G:  The rigid form of Huntington's chorea . Neurology 1971;;21:271-276.
56 +
Liss L, Paulson G, Sommer A:  Rigid form of Huntington's chorea: A clinopathologic study of three cases , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, 1973;, vol 1: Huntington's Disease, pp 405-424.
57 +
Markham C:  Huntington's chorea in childhood , in Barbeau A, Brunette J (eds): Progress in Neurogenetics . Princeton, NJ, Exerpta Medica, 1969;, pp 651-660.
58 +
Oliver JE, Dewhurst K:  Childhood and adolescent forms of Huntington's disease . Neurology 1969;;32:455-459.
59 +
Brackenridge CJ, Teltscher B:  Estimation of the age at onset of Huntington's disease from factors associated with the affected parent . J Med Genet 1975;;12:64-69.
60 +
Jervis G:  Huntington's chorea in childhood . Arch Neurol 1963;;9:244-257.
61 +
Folstein SE, Abbott MH, Franz ML, et al:  Phenotypic heterogeneity in Huntington's disease . J Neurogenet 1985;;1:175-184.
62 +
Gusella JF, Wexler NS, Conneally PM, et al:  A polymorphic DNA marker genetically linked to Huntington's disease . Nature 1983;;306:234-238.
63 +
Hayden MR, Soles JA, Ward RH:  Age of onset in siblings of persons with juvenile Huntington's disease . Clin Genet 1985;;28:100-105.
64 +
Faught E, Falgout JC, Leli DA:  Late-onset variant of Huntington's chorea . South Med J 1983;;76:1266-1270.
65 +
Myers RH, Sax DS, Schoenfeld M, et al:  Late onset of Huntington's disease . J Neurol Neurosurg Psychiatry 1985;;48:530-534.
66 +
Martin GM:  Genetic syndromes in man with a potential relevance to the pathobiology of aging , in Bergsma D, Lenz W (eds): Birth Defects . New York, Alan R Liss Inc, 1978;, vol 14, pp 5-39.
67 +
Burch PRJ:  Genetic and pathogenetic implications of the age incidence of Huntington's chorea , in Barbeau A, Chase TN, Paulson GW (eds): Advances in Neurology . New York, Raven Press, vol 1: Huntington's Disease, 1973;, pp 179-190.
68 +
Podolsky S, Leopold NA, Sax DS:  Increased frequency of diabetes mellitus in patients with Huntington's chorea . Lancet 1972;;1:1356-1358.
69 +
 National Diabetes Data Group: Classification and diagnosis of diabetes mellitus and other categories of glucose intolerance . Diabetes 1979;;28:1039-1057.
70 +
Farrer LA:  Diabetes mellitus in Huntington's disease . Clin Genet 1985;;27:62-67.
71 +
Sanberg PR, Fibiger HC:  Body weight, feeding, and drinking behaviors in rats with kainic acid-induced lesions with striatal neurons, with a note on body weight symptomatology in Huntington's disease . Exp Neurol 1979;;66:444-466.
72 +
Farrer LA, Meaney FJ:  Anthropometric assessment of Huntington's disease patients and families . Am J Phys Anthropol 1985;;67:185-194.
73 +
Tellez-Nagel I, Johnson AB, Terry AD:  Studies on brain biopsies of patients with Huntington's chorea . J Neuropathol Exp Neurol 1974;;33:308-332.
74 +
Brizzee KR:  Aging changes in relation to diseases of the nervous system , in Ordy JM, Brizzee KR (eds): Neurobiology of Aging . New York, Plenum Publishing Corp, 1975;.
75 +
Oepen H:  Paroxysmale Storungen bei der Huntingtonschen Chorea . Arch Psych Nervenkr 1963;;204:245-261.
76 +
Stahl SM, Faull KF, Barchas JD, et al:  CSF monoamine metabolites in movement disorders and normal aging . Arch Neurol 1985;;42:166-169.
77 +
Stahl SM, Thiemann S, Faull KF, et al:  Neurochemistry of dopamine in Huntington's dementia and normal aging . Arch Gen Psychiatry 1986;;43:161-164.

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