Neuropathology of prodromal Lewy body disease

Movement Disorders - Tập 29 Số 3 - Trang 410-415 - 2014
Álex Iranzo1,2,3, Ellen Gelpí4,3, Eduard Tolosa1,2, José Luís Molinuevo2, Mónica Serradell2, Carles Gaig1,2, Joan Santamaría1,2
1Centro de Investigación Biomédica en Red sobre Enfermedades Neurodegenerativas (CIBERNED), Barcelona, Spain
2Neurology Service Hospital Clínic de Barcelona, Institut d'Investigacións Biomédiques August Pi I Sunyer (IDIBAPS) Barcelona Spain
3The first two authors contributed equally to this work.
4Neurological Tissue Bank, Biobanc-Hospital Clinic, IDIBAPS, Barcelona, Spain

Tóm tắt

ABSTRACTBackground

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

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