[Neuropathology of tauopathies and synucleinopathies, and neuroanatomy of sleep disorders: meeting the challenge].
Abnormalities of tau and alpha-synuclein have been described in a variety of neurodegenerative diseases often associated with sleep disorders. Neuropathological descriptions concerning these diseases are rapidly expanding, and they become difficult to summarise. On the other hand, the human neuroanatomy of sleep remains an ill defined issue. Main tauopathies are Alzheimer's disease, progressive supranuclear palsy, cortico-basal degeneration, argyrophilic grain disease, Pick disease and fronto-temporal degeneration with Parkinsonism associated with chromosome 17. In contrast to Alzheimer's disease, where abnormal tau containing cells are mainly neurones, in the other disorders, both neurones and glial cells are affected. The presynaptic protein alpha-synuclein is a major constituent of Lewy-type lesions in Parkinson disease and in dementia with Lewy bodies. Alpha-synuclein is also found in neurones and glia of Multi System Atrophy. This led to group these disorders into the still ill defined group of synucleinopathies. The lesions of tauopathies and synucleinopathies are presented, and their distribution in the most common disorders is described, distinguishing when possible neuronal loss and neuropathological markers. Recent data show that their extension is far larger than previously assumed and that they involve a variety of areas possibly involved in sleep regulation. Sleep disorders have been described in various tauopathies and synucleinopathies. However, no detailed clinico-pathological reports concerning the distribution of affected and spared areas in patients studied by polysomnography are available. Furthermore, the similarities of sleep disorders associated with different diseases, the interindividual variability, the frequently associated disorders, and the difficulties in quantifying neuronal loss make any clinicopathological correlation uncertain. The knowledge of sleep neuroanatomy is mainly based on animal studies. The few data concerning the structures of human brain areas involved in sleep organisation are recalled. Several systems known to be acting in sleep physiology are usually affected by tauopathies and synucleinopathies, but the pattern of their involvement in sleep pathology remains highly conjectural. The neuropathology of sleep disorders in tauopathies and synucleinopathies is a still uncultivated field.
Hauw
J-J
JJ
Laboratoire de Neuropathologie Raymond Escourolle, Groupe Hospitalier Pitié-Salpêtrière, Paris. jean-jacques.hauw@psl.ap-hop-paris.fr
Hausser-Hauw
C
C
Duyckaerts
C
C
D000328:Adult; D000368:Aged; D001923:Brain Chemistry; D006801:Humans; D016631:Lewy Bodies / Q000737:chemistry; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000172:deficiency / Q000502:physiology*; D019636:Neurodegenerative Diseases / Q000150:complications / Q000378:metabolism* / Q000473:pathology; D012893:Sleep Wake Disorders / Q000209:etiology / Q000378:metabolism* / Q000473:pathology; D051843:Synucleins; D024801:Tauopathies / Q000150:complications / Q000378:metabolism / Q000473:pathology; D051844:alpha-Synuclein; D016875:tau Proteins / Q000172:deficiency / Q000502:physiology*
D009419:Nerve Tissue Proteins; C497604:SNCA protein, human; D051843:Synucleins; D051844:alpha-Synuclein; D016875:tau Proteins