Demetrius M. Maraganore, M.D.

Demetrius M. Maraganore, M.D.

Demetrius M. Maraganore, M.D.

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Personal Bio

Treatment Philosophy

It is my core value, and the core value of our Department of Neurology, that the needs of the patient come first. I believe that as the leaders in our field, it is our responsibility to provide tomorrow's healthcare today.

Personal Interests

My personal interests are to develop methods to predict, prevent, and halt neurological diseases, through the application of molecular prognostic and therapeutic approaches; and to lead the region's pre-eminent providers of neurological care.

Conditions & Procedures

Conditions

Parkinsonism, Parkinson's Disease (PD), Tremors

General Information

Gender

Male

Affiliation

NorthShore Medical Group

Academic Rank

Clinical Professor

Languages

English, Greek, Italian

Board Certified

Neurology

Clinical Service

Education, Training & Fellowships

Medical School

Northwestern Feinberg School of Medicine, 1985

Internship

Mayo School of Graduate Medical Education, 1986

Residency

Mayo School of Graduate Medical Education, 1989

Fellowship

National Hospital for Neurology and Neurosurgery, 1990

Locations

A

NorthShore Medical Group

2180 Pfingsten Rd.
Suite 2000
Glenview, IL 60026
847.570.2570 847.657.5708 fax This location is wheelchair accessible.

Insurance

Every effort has been made to ensure the accuracy of the information in this directory. However, some changes may occur between updates. Please check with your provider to ensure that he or she participates in your health plan.

Aetna HMO/PPO/POS
BCBS HMOI
BCBS PPO *except Blue Choice IL
Beechstreet PPO
CCN PPO
CIGNA Choice Fund
CIGNA Choice Fund PPO
CIGNA EPO
CIGNA Network
CIGNA Network Open Access
CIGNA POS
CIGNA POS Open Access
CIGNA PPO
CIGNA:Open Access Plus
Community Care Partners
First Health PPO
Galaxy PPO
Great West POS
Great West PPO
Healthcares Finest Network PPO
Humana Choice Care PPO
Humana IPA--HMO
Humana POS
Humana PPO
Land of Lincoln
Medicare
Multiplan Admar PPO
Multiplan Formost PPO
Multiplan Health Network PPO
Multiplan Wellmark PPO
NorthShore Employee Network I (EPO Option)
NorthShore Employee Network II (EPO Plus & CDHP)
PHCS PPO
Preferred Plan PPO
Railroad Medicare - Cook County
Railroad Medicare - Lake County
UHC *except Core & Navigate
Unicare PPO

Publications

  • Global investigation and meta-analysis of the C9orf72 (G4C2)n repeat in Parkinson disease.

    Neurology 2014 Oct 17

    Authors: Theuns J,
    Abstract
    The objective of this study is to clarify the role of (G4C2)n expansions in the etiology of Parkinson disease (PD) in the worldwide multicenter Genetic Epidemiology of Parkinson's Disease (GEO-PD) cohort.
    C9orf72 (G4C2)n repeats were assessed in a GEO-PD cohort of 7,494 patients diagnosed with PD and 5,886 neurologically healthy control individuals ascertained in Europe, Asia, North America, and Australia.
    A pathogenic (G4C2)n>60 expansion was detected in only 4 patients with PD (4/7,232; 0.055%), all with a positive family history of neurodegenerative dementia, amyotrophic lateral sclerosis, or atypical parkinsonism, while no carriers were detected with typical sporadic or familial PD. Meta-analysis revealed a small increase in risk of PD with an increasing number of (G4C2)n repeats; however, we could not detect a robust association between the C9orf72 (G4C2)n repeat and PD, and the population attributable risk was low.
    Together, these findings indicate that expansions in C9orf72 do not have a major role in the pathogenesis of PD. Testing for C9orf72 repeat expansions should only be considered in patients with PD who have overt symptoms of frontotemporal lobar degeneration/amyotrophic lateral sclerosis or apparent family history of neurodegenerative dementia or motor neuron disease.
    PMID: 25326098 [PubMed - as supplied by publisher]
  • Does α-synuclein have a dual and opposing effect in preclinical vs. clinical Parkinson's disease?

    Parkinsonism & related disorders 2014 Jun

    Authors: Markopoulou K,
    Abstract
    α-Synuclein gene (SNCA) multiplications cause familial parkinsonism and allele-length polymorphisms within the SNCA dinucleotide repeat REP1 increase the risk for developing Parkinson's disease (PD). Since SNCA multiplications increase SNCA expression, and REP1 genotypes that increase the risk of developing PD show increased SNCA expression in cell-culture systems, animal models, and human blood and brain, PD therapies seek to reduce SNCA expression. We conducted an observational study of 1098 PD cases to test the hypothesis that REP1 genotypes correlated with reduced SNCA expression are associated with better motor and cognitive outcomes. We evaluated the association of REP1 genotypes with survival free of Hoehn and Yahr stages 4 or 5 (motor outcome) and of Modified Telephone Interview for Cognitive Status score ≤27 or Alzheimer's Disease Dementia Screening Interview score ≥2 (cognitive outcome). Median disease duration at baseline was 3.3 years and median lag time from baseline to follow-up was 7.8 years. Paradoxically, REP1 genotypes associated with increased risk of developing PD and increased SNCA expression were associated with better motor (HR = 0.87, p = 0.046, covariate-adjusted age-scale analysis; HR = 0.85, p = 0.020, covariate-adjusted time-scale analysis) and cognitive outcomes (HR = 0.90, p = 0.12, covariate-adjusted age-scale analysis; HR = 0.85, p = 0.023, covariate-adjusted time-scale analysis). Our findings raise the possibility that SNCA has a dual, opposing, and time-dependent role. This may have implications for the development of therapies that target SNCA expression.
    PMID: 24656894 [PubMed - as supplied by publisher]
  • Alpha-synuclein repeat variants and survival in Parkinson's disease.

    Movement disorders : official journal of the Movement Disorder Society 2014 Jul

    Authors: Chung SJ,
    Abstract
    To determine whether α-synuclein dinucleotide repeat (REP1) genotypes are associated with survival in Parkinson's disease (PD).
    Investigators from the Genetic Epidemiology of Parkinson's Disease Consortium provided REP1 genotypes and baseline and follow-up clinical data for cases. The primary outcome was time to death. Cox proportional hazards regression models were used to assess the association of REP1 genotypes with survival.
    Twenty-one sites contributed data for 6,154 cases. There was no significant association between α-synuclein REP1 genotypes and survival in PD. However, there was a significant association between REP1 genotypes and age at onset of PD (hazard ratio: 1.06; 95% confidence interval: 1.01-1.10; P value = 0.01).
    In our large consortium study, α-synuclein REP1 genotypes were not associated with survival in PD. Further studies of α-synuclein's role in disease progression and long-term outcomes are needed.
    PMID: 24578302 [PubMed - as supplied by publisher]
  • Protective effect of LRRK2 p.R1398H on risk of Parkinson's disease is independent of MAPT and SNCA variants.

    Neurobiology of aging 2014 Jan

    Authors: Heckman MG,
    Abstract
    The best validated susceptibility variants for Parkinson's disease are located in the α-synuclein (SNCA) and microtubule-associated protein tau (MAPT) genes. Recently, a protective p.N551K-R1398H-K1423K haplotype in the leucine-rich repeat kinase 2 (LRRK2) gene was identified, with p.R1398H appearing to be the most likely functional variant. To date, the consistency of the protective effect of LRRK2 p.R1398H across MAPT and SNCA variant genotypes has not been assessed. To address this, we examined 4 SNCA variants (rs181489, rs356219, rs11931074, and rs2583988), the MAPT H1-haplotype-defining variant rs1052553, and LRRK2 p.R1398H (rs7133914) in Caucasian (n = 10,322) and Asian (n = 2289) series. There was no evidence of an interaction of LRRK2 p.R1398H with MAPT or SNCA variants (all p ≥ 0.10); the protective effect of p.R1398H was observed at similar magnitude across MAPT and SNCA genotypes, and the risk effects of MAPT and SNCA variants were observed consistently for LRRK2 p.R1398H genotypes. Our results indicate that the association of LRRK2 p.R1398H with Parkinson's disease is independent of SNCA and MAPT variants, and vice versa, in Caucasian and Asian populations.
    PMID: 23962496 [PubMed - as supplied by publisher]
  • Population-specific frequencies for LRRK2 susceptibility variants in the Genetic Epidemiology of Parkinson's Disease (GEO-PD) Consortium.

    Movement disorders : official journal of the Movement Disorder Society 2013 Oct

    Authors: Heckman MG,
    Abstract
    Variants within the leucine-rich repeat kinase 2 gene are recognized as the most frequent genetic cause of Parkinson's disease. Leucine-rich repeat kinase 2 variation related to disease susceptibility displays many features that reflect the nature of complex, late-onset sporadic disorders like Parkinson's disease.
    The Genetic Epidemiology of Parkinson's Disease Consortium recently performed the largest genetic association study for variants in the leucine-rich repeat kinase 2 gene across 23 different sites in 15 countries.
    Herein, we detail the allele frequencies for the novel risk factors (p.A419V and p.M1646T) and the protective haplotype (p.N551K-R1398H-K1423K) nominated in the original publication. Simple population allele frequencies not only can provide insight into the clinical relevance of specific variants but also can help genetically define patient groups.
    Establishing individual patient-based genomic susceptibility profiles that incorporate both risk factors and protective factors will determine future diagnostic and treatment strategies.
    PMID: 23913756 [PubMed - as supplied by publisher]
  • Genetic susceptibility loci, environmental exposures, and Parkinson's disease: a case-control study of gene-environment interactions.

    Parkinsonism & related disorders 2013 Jun

    Authors: Chung SJ,
    Abstract
    Prior studies causally linked mutations in SNCA, MAPT, and LRRK2 genes with familial Parkinsonism. Genome-wide association studies have demonstrated association of single nucleotide polymorphisms (SNPs) in those three genes with sporadic Parkinson's disease (PD) susceptibility worldwide. Here we investigated the interactions between SNPs in those three susceptibility genes and environmental exposures (pesticides application, tobacco smoking, coffee drinking, and alcohol drinking) also associated with PD susceptibility.
    Pairwise interactions between environmental exposures and 18 variants (16 SNPs and two variable number tandem repeats, or "VNTRs") in SNCA, MAPT and LRRK2, were investigated using data from 1098 PD cases from the upper Midwest, USA and 1098 matched controls. Environmental exposures were assessed using a validated telephone interview script.
    Five pairwise interactions had uncorrected P-values < 0.05. These included pairings of pesticides × SNCA rs3775423 or MAPT rs4792891, coffee drinking × MAPT H1/H2 haplotype or MAPT rs16940806, and alcohol drinking × MAPT rs2435211. None of these interactions remained significant after Bonferroni correction. Secondary analyses in strata defined by type of control (sibling or unrelated), sex, or age at onset of the case also did not identify significant interactions after Bonferroni correction.
    This study documented limited pairwise interactions between established genetic and environmental risk factors for PD; however, the associations were not significant after correction for multiple testing.
    PMID: 23507417 [PubMed - as supplied by publisher]
  • A multi-centre clinico-genetic analysis of the VPS35 gene in Parkinson disease indicates reduced penetrance for disease-associated variants.

    Journal of medical genetics 2012 Nov

    Authors: Sharma M,
    Abstract
    Two recent studies identified a mutation (p.Asp620Asn) in the vacuolar protein sorting 35 gene as a cause for an autosomal dominant form of Parkinson disease . Although additional missense variants were described, their pathogenic role yet remains inconclusive.
    We performed the largest multi-center study to ascertain the frequency and pathogenicity of the reported vacuolar protein sorting 35 gene variants in more than 15,000 individuals worldwide. p.Asp620Asn was detected in 5 familial and 2 sporadic PD cases and not in healthy controls, p.Leu774Met in 6 cases and 1 control, p.Gly51Ser in 3 cases and 2 controls. Overall analyses did not reveal any significant increased risk for p.Leu774Met and p.Gly51Ser in our cohort.
    Our study apart from identifying the p.Asp620Asn variant in familial cases also identified it in idiopathic Parkinson disease cases, and thus provides genetic evidence for a role of p.Asp620Asn in Parkinson disease in different populations worldwide.
    PMID: 23125461 [PubMed - as supplied by publisher]
  • Large-scale replication and heterogeneity in Parkinson disease genetic loci.

    Neurology 2012 Aug 14

    Authors: Sharma M,
    Abstract
    Eleven genetic loci have reached genome-wide significance in a recent meta-analysis of genome-wide association studies in Parkinson disease (PD) based on populations of Caucasian descent. The extent to which these genetic effects are consistent across different populations is unknown.
    Investigators from the Genetic Epidemiology of Parkinson's Disease Consortium were invited to participate in the study. A total of 11 SNPs were genotyped in 8,750 cases and 8,955 controls. Fixed as well as random effects models were used to provide the summary risk estimates for these variants. We evaluated between-study heterogeneity and heterogeneity between populations of different ancestry.
    In the overall analysis, single nucleotide polymorphisms (SNPs) in 9 loci showed significant associations with protective per-allele odds ratios of 0.78-0.87 (LAMP3, BST1, and MAPT) and susceptibility per-allele odds ratios of 1.14-1.43 (STK39, GAK, SNCA, LRRK2, SYT11, and HIP1R). For 5 of the 9 replicated SNPs there was nominally significant between-site heterogeneity in the effect sizes (I(2) estimates ranged from 39% to 48%). Subgroup analysis by ethnicity showed significantly stronger effects for the BST1 (rs11724635) in Asian vs Caucasian populations and similar effects for SNCA, LRRK2, LAMP3, HIP1R, and STK39 in Asian and Caucasian populations, while MAPT rs2942168 and SYT11 rs34372695 were monomorphic in the Asian population, highlighting the role of population-specific heterogeneity in PD.
    Our study allows insight to understand the distribution of newly identified genetic factors contributing to PD and shows that large-scale evaluation in diverse populations is important to understand the role of population-specific heterogeneity.
    PMID: 22786590 [PubMed - as supplied by publisher]
  • Genomic determinants of motor and cognitive outcomes in Parkinson's disease.

    Parkinsonism & related disorders 2012 Aug

    Authors: Chung SJ,
    Abstract
    Little is known regarding genetic factors associated with motor or cognitive outcomes in Parkinson's disease (PD).
    To identify common genetic variants associated with motor and cognitive outcomes in PD.
    The sample consisted of 443 PD cases included in the first genome-wide association study (GWAS) of PD. Methods included telephone interview assessments of motor and cognitive outcomes, a median 9 years following the initial clinical assessments. Analyses included Cox proportional hazard models to study the association of 198,345 single nucleotide polymorphisms (SNPs) with survival free of Hoehn and Yahr stage ≥ 4 (motor outcome), and either TICS-M ≤ 27 or AD-8 ≥ 2 (cognitive outcomes).
    The SNP rs10958605 in the C8orf4 gene had the smallest p value in analyses of the motor outcome (HR = 1.81; 95% CI = 1.42-2.31; p = 1.51 × 10(-6)). The SNP rs6482992 in the CLRN3 gene had the smallest p value in analyses of the cognitive outcome (HR = 2.03, 95% CI 1.47-2.79, p = 4.08 × 10(-6)). However, no SNP associations were significant after Bonferroni correction. The C8orf4 gene had small p values for both motor and cognitive outcomes, highlighting inflammation as a possible pathogenesis mechanism for progression in PD.
    This study suggests that common variants in several genes may be associated with motor and cognitive outcomes in PD, with biological plausibility.
    PMID: 22658654 [PubMed - as supplied by publisher]
  • Mutations in CIZ1 cause adult onset primary cervical dystonia.

    Annals of neurology 2012 Apr

    Authors: Xiao J,
    Abstract
    Primary dystonia is usually of adult onset, can be familial, and frequently involves the cervical musculature. Our goal was to identify the causal mutation in a family with adult onset, primary cervical dystonia.
    Linkage and haplotype analyses were combined with solution-based whole-exome capture and massively parallel sequencing in a large Caucasian pedigree with adult onset, primary cervical dystonia to identify a cosegregating mutation. High-throughput screening and Sanger sequencing were completed in 308 Caucasians with familial or sporadic adult onset cervical dystonia and matching controls for sequence variants in this mutant gene.
    Exome sequencing led to the identification of an exonic splicing enhancer mutation in exon 7 of CIZ1 (c.790A>G, p.S264G), which encodes CIZ1, Cip1-interacting zinc finger protein 1. CIZ1 is a p21(Cip1/Waf1) -interacting zinc finger protein expressed in brain and involved in DNA synthesis and cell-cycle control. Using a minigene assay, we showed that c.790A>G altered CIZ1 splicing patterns. The p.S264G mutation also altered the nuclear localization of CIZ1. Screening in subjects with adult-onset cervical dystonia identified 2 additional CIZ1 missense mutations (p.P47S and p.R672M).
    Mutations in CIZ1 may cause adult onset, primary cervical dystonia, possibly by precipitating neurodevelopmental abnormalities that manifest in adults and/or G1/S cell-cycle dysregulation in the mature central nervous system.
    PMID: 22447717 [PubMed - as supplied by publisher]

In the News

Jul 2014

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Featured Videos

Drs. Demetrius Maraganore and Julian Bailes discuss the innovative research being done at the NorthShore Neurological Institute.

Drs. Demetrius Maraganore and Julian Bailes discuss the innovative research being done at the NorthShore Neurological Institute.

NorthShore Neurological Institute - Treatments: Doctors Bailes, Alleva & Maraganore (Neurology)

NorthShore Neurological Institute - Treatments: Doctors Bailes, Alleva & Maraganore (Neurology)

NorthShore Neurological Institute - Centers & Programs: Doctors Bailes, Alleva & Maraganore (Neurology)

NorthShore Neurological Institute - Centers & Programs: Doctors Bailes, Alleva & Maraganore (Neurology)

NorthShore Neurological Institute: Doctors Bailes, Alleva & Maraganore (Neurology)

NorthShore Neurological Institute: Doctors Bailes, Alleva & Maraganore (Neurology)

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