Incidental Lewy body disease in a patient with REM sleep behavior disorder

Abstract

We studied an 84-year-old man with a 20-year history of nocturnal violent behavior during sleep, but no other clinically evident neuropsychiatric disorders. Polysomnographic investigations confirmed that he suffered from REM sleep behavior disorder (RBD). Histopathologic examination revealed he had Lewy body disease with a marked decrease of pigmented neurons in the locus ceruleus and substantia nigra. These histologic findings represent the first documented evidence of a loss of brainstem monoaminergic neurons in clinically idiopathic RBD and suggest that Lewy body disease might provide an explanation for idiopathic RBD in the aged.

Insights into REM sleep behavior disorder pathophysiology in brainstem-predominant Lewy body disease

“…The few cases which have been studied with magnetic resonance imaging (MRI) have shown lesions in the dorsal pons [10,11], yet the specific neuronal networks involved in human RBD pathogenesis have not been identified with certainty. Although ubiquitin and α-synuclein immunocytochemistry were not used in the case reported by Uchiyama et al [4], and the consensus criteria for the clinical and pathologic diagnoses of dementia with Lewy bodies (DLB) and LBD had not yet been published, the distribution of LBs suggests that their case would now be classified as brainstem-predominant LBD [6,7]. Due to the severe neuronal loss and gliosis in the SN and LC in their case, the authors postulated that degeneration of brainstem monoaminergic neurons explained RBD [4].…”

“…Although ubiquitin and α-synuclein immunocytochemistry were not used in the case reported by Uchiyama et al [4], and the consensus criteria for the clinical and pathologic diagnoses of dementia with Lewy bodies (DLB) and LBD had not yet been published, the distribution of LBs suggests that their case would now be classified as brainstem-predominant LBD [6,7]. Due to the severe neuronal loss and gliosis in the SN and LC in their case, the authors postulated that degeneration of brainstem monoaminergic neurons explained RBD [4]. Additional cases of idiopathic RBD with neuropathologic examination are needed to confirm or refute this hypothesis.…”

“…There is ample data from animal and human studies that dysfunction of brainstem structures and networks are likely involved, but which specific neuronal networks are involved in RBD pathogenesis has not yet been determined [1,3]. To date, fewer than 25 cases of polysomnographically (PSG) proven RBD cases have undergone neuropathologic examination [3], and only one case of idiopathic RBD has undergone autopsy, in which "incidental Lewy body disease" was found [4]. We report herein another case of idiopathic RBD associated with Lewy body disease (LBD), but with degenerative changes topographically different from those previously reported.…”

Premotor Symptoms and Early Diagnosis of Parkinson's Disease

“…There are data that suggest that rapid eye movement (REM) sleep behavior disorder (RBD) is a synucleinopathy [54][55][56]. There is a high occurrence of RBD in patients with PD, up to 60% in some studies [57,58], and up to 80% in DLB patients [59] [60][61][62], with Postuma et al [61] also finding deficits in color discrimination compared with a control population.…”

Combinations of Markers Provide Clues to the Underlying Neurodegenerative Disorder in REM Sleep Behavior Disorder

“…In PD, approximately 60% of the nigrostrial neurons of the SN are degenerated before patients fulfill the clinical criteria of PD (Fearnkey et al, 1991). In some cases of PD, the patient appears to develop cortical disease before the motor signs of "stage 3" disease, whereas iRBD patients with ILBD could be diagnosed after longstanding disease with no evidence of motor or cognitive abnormalities (Uchiyama et al, 1995;. If www.intechopen.com progression of syncleinopathies is not universal, it is essential to understand why.…”