Essential Tremor Associated With Focal Nonnigral Lewy Bodies

Abstract

Background: Essential tremor is one of the most common neurological diseases. Its links with Parkinson disease (PD) are often debated. There have been few published postmortem studies. Objective: To study our first case of essential tremor through the recently established Essential Tremor Centralized Brain Repository. Design: Report of a case of a patient with a diagnosis of severe essential tremor for 46 years who exhibited no signs of parkinsonism.

Computerised analysis of writing and drawing by essential tremor phenotype

“…Loss of Purkinje cells, suggested by some18 19 but not other studies,20 is more contentious. Then there are a minority of ET brains in which brainstem Lewy bodies are present 19 21. The neuropathology of ET is consistent with the heterogeneity of the ET syndrome and could explain impaired motor control.…”

Computerised analysis of writing and drawing by essential tremor phenotype

“…Loss of Purkinje cells, suggested by some18 19 but not other studies,20 is more contentious. Then there are a minority of ET brains in which brainstem Lewy bodies are present 19 21. The neuropathology of ET is consistent with the heterogeneity of the ET syndrome and could explain impaired motor control.…”

An order in Lewy body disorders: Retrograde degeneration in hyperbranching axons as a fundamental structural template accounting for focal/multifocal Lewy body disease

“…Another example (Fig. E) represents an atypical gradient of αS lesions accentuated in LC (dmX < LC > SN), which has been reported in cases of essential tremor . Similarly, initiation of αS lesions in their distal axons may spread to the soma of LC neurons, which may then spread to dmX and SN neurons.…”

Essential tremor: the most common form of cerebellar degeneration?

“…Cerebellar degeneration can result in ET but other pathophysiological mechanisms are possible and are likely. Thus, as noted above, in a subset of ET cases (8 of 33 in one reported series), the main pathology was brainstem Lewy bodies [144,154], and in a recent report, the spectrum of observable pathology in the cerebellum in a small proportion of ET cases overlapped with that seen in controls [45]. Whether degeneration may be present in other cerebellar outflow pathways in some ET cases remains to be explored.…”

Computerised analysis of writing and drawing by essential tremor phenotype

“…Loss of Purkinje cells, suggested by some18 19 but not other studies,20 is more contentious. Then there are a minority of ET brains in which brainstem Lewy bodies are present 19 21. The neuropathology of ET is consistent with the heterogeneity of the ET syndrome and could explain impaired motor control.…”

An order in Lewy body disorders: Retrograde degeneration in hyperbranching axons as a fundamental structural template accounting for focal/multifocal Lewy body disease

“…Another example (Fig. E) represents an atypical gradient of αS lesions accentuated in LC (dmX < LC > SN), which has been reported in cases of essential tremor . Similarly, initiation of αS lesions in their distal axons may spread to the soma of LC neurons, which may then spread to dmX and SN neurons.…”

Essential tremor: the most common form of cerebellar degeneration?

“…Cerebellar degeneration can result in ET but other pathophysiological mechanisms are possible and are likely. Thus, as noted above, in a subset of ET cases (8 of 33 in one reported series), the main pathology was brainstem Lewy bodies [144,154], and in a recent report, the spectrum of observable pathology in the cerebellum in a small proportion of ET cases overlapped with that seen in controls [45]. Whether degeneration may be present in other cerebellar outflow pathways in some ET cases remains to be explored.…”

Computerised analysis of writing and drawing by essential tremor phenotype

“…Loss of Purkinje cells, suggested by some18 19 but not other studies,20 is more contentious. Then there are a minority of ET brains in which brainstem Lewy bodies are present 19 21. The neuropathology of ET is consistent with the heterogeneity of the ET syndrome and could explain impaired motor control.…”

An order in Lewy body disorders: Retrograde degeneration in hyperbranching axons as a fundamental structural template accounting for focal/multifocal Lewy body disease

“…Another example (Fig. E) represents an atypical gradient of αS lesions accentuated in LC (dmX < LC > SN), which has been reported in cases of essential tremor . Similarly, initiation of αS lesions in their distal axons may spread to the soma of LC neurons, which may then spread to dmX and SN neurons.…”

Essential tremor: the most common form of cerebellar degeneration?

“…Cerebellar degeneration can result in ET but other pathophysiological mechanisms are possible and are likely. Thus, as noted above, in a subset of ET cases (8 of 33 in one reported series), the main pathology was brainstem Lewy bodies [144,154], and in a recent report, the spectrum of observable pathology in the cerebellum in a small proportion of ET cases overlapped with that seen in controls [45]. Whether degeneration may be present in other cerebellar outflow pathways in some ET cases remains to be explored.…”