Familial Parkinsonism, Dementia, and Lewy body disease: Study of family G

Melody A. Denson, Zbigniew K Wszolek, Ronald F. Pfeiffer, Elzbieta K. Wszolek, Tanya M. Paschall, Rodney D. McComb

Research output: Contribution to journalArticle

47 Citations (Scopus)

Abstract

Genetic influences are thought by many to play an important role in the cause of Parkinson's disease. We studied two closely intermarried families (Family G) whose ancestors immigrated to the United States from Russia. We investigated this family clinically, genealogically, and pathologically. Our pedigree contained 102 members spanning six generations, with 10 affected individuals and 1 affected spouse. Detailed telephone interviews were conducted with affected individuals, with their spouses, and with their at- risk siblings. Medical records of deceased and living affected patients were collected. Physical examinations were performed on 7 at-risk and 5 affected persons. Typical levodopa-responsive parkinsonism with bradykinesia, rigidity, resting tremor, and impaired postural reflexes was seen in 4 members, dementia was present in 3, and 3 had both dementia and parkinsonism. An autopsy completed on 1 individual, our index case, demonstrated Lewy bodies in the brainstem and neocortex and ubiquitin-positive neuritic degeneration in the CA2-3 region of the hippocampus, consistent with the limbic (transitional) form of Lewy body disease. This family is distinct both clinically and pathologically from several previously reported parkinsonian kindreds.

Original languageEnglish (US)
Pages (from-to)638-643
Number of pages6
JournalAnnals of Neurology
Volume42
Issue number4
DOIs
StatePublished - Oct 1997
Externally publishedYes

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Lewy Body Disease
Parkinsonian Disorders
Dementia
Spouses
Lewy Bodies
Hypokinesia
Russia
Neocortex
Levodopa
Tremor
Pedigree
Ubiquitin
Brain Stem
Physical Examination
Medical Records
Parkinson Disease
Reflex
Siblings
Autopsy
Hippocampus

ASJC Scopus subject areas

  • Neuroscience(all)

Cite this

Denson, M. A., Wszolek, Z. K., Pfeiffer, R. F., Wszolek, E. K., Paschall, T. M., & McComb, R. D. (1997). Familial Parkinsonism, Dementia, and Lewy body disease: Study of family G. Annals of Neurology, 42(4), 638-643. https://doi.org/10.1002/ana.410420415

Familial Parkinsonism, Dementia, and Lewy body disease : Study of family G. / Denson, Melody A.; Wszolek, Zbigniew K; Pfeiffer, Ronald F.; Wszolek, Elzbieta K.; Paschall, Tanya M.; McComb, Rodney D.

In: Annals of Neurology, Vol. 42, No. 4, 10.1997, p. 638-643.

Research output: Contribution to journalArticle

Denson, MA, Wszolek, ZK, Pfeiffer, RF, Wszolek, EK, Paschall, TM & McComb, RD 1997, 'Familial Parkinsonism, Dementia, and Lewy body disease: Study of family G', Annals of Neurology, vol. 42, no. 4, pp. 638-643. https://doi.org/10.1002/ana.410420415
Denson, Melody A. ; Wszolek, Zbigniew K ; Pfeiffer, Ronald F. ; Wszolek, Elzbieta K. ; Paschall, Tanya M. ; McComb, Rodney D. / Familial Parkinsonism, Dementia, and Lewy body disease : Study of family G. In: Annals of Neurology. 1997 ; Vol. 42, No. 4. pp. 638-643.
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