Get access

Neuropathology of prodromal Lewy body disease

Authors

  • Alex Iranzo MD,

    Corresponding author
    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    2. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
    • Correspondence to: Dr. Alex Iranzo de Riquer, Neurology Service, Hospital Clinic de Barcelona, C/Villarroel 170, Barcelona 08036, Spain; airanzo@clinic.ub.es

    Search for more papers by this author
    • The first two authors contributed equally to this work.

  • Ellen Gelpi MD,

    1. Neurological Tissue Bank, Biobanc-Hospital Clinic, Barcelona, Spain
    Search for more papers by this author
    • The first two authors contributed equally to this work.

  • Eduard Tolosa MD,

    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    2. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
    Search for more papers by this author
  • José Luis Molinuevo MD,

    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    Search for more papers by this author
  • Mónica Serradell BSc,

    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    Search for more papers by this author
  • Carles Gaig MD,

    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    2. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
    Search for more papers by this author
  • Joan Santamaria MD

    1. Neurology Service, Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS), Barcelona, Spain
    2. Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
    Search for more papers by this author

  • Funding agencies: This study was supported in part by the Beca de Recerca Clínica de l'Acadèmia de Ciéncies Médiques de Catalunya, 2011.

  • Relevant conflicts of interest/financial disclosures: Nothing to report.

  • Full financial disclosures and author roles may be found in the online version of this article.

ABSTRACT

Background

Current evidence suggests that there is a prodromal stage in Parkinson disease characterized by a variety of nonmotor symptoms.

Methods and Results

A 69-year-old man presented to our sleep center with isolated rapid eye movement sleep behavior disorder. During a 10-year follow-up period, longitudinal clinical and laboratory assessments indicated the development of hyposmia, depression, mild cognitive impairment, and constipation. Parkinsonism was absent, but dopamine transporter imaging showed subclinical substantia nigra damage. Postmortem examination demonstrated neuronal loss and Lewy body pathology in the peripheral autonomic nervous system (eg, cardiac and myenteric plexus), olfactory bulb, medulla, pons, substantia nigra pars compacta (estimated cell loss, 20%-30%), nucleus basalis of Meynert, and amygdala, sparing the neocortex.

Conclusions

Our observations indicate that nonmotor symptoms plus widespread peripheral and central nervous system pathological changes occur before parkinsonism and dementia onset in diseases associated with Lewy pathology. The current diagnostic criteria for Parkinson's disease miss these patients, who present only with nonmotor symptoms. © 2014 International Parkinson and Movement Disorder Society

Ancillary