Hypertrophic Olivary Degeneration Does Not Reduce Essential Tremor

Elkouzi, Ahmad; Kattah, Jorge C.; Elble, Rodger J.

doi:10.1002/mdc3.12275

Cited by 16 publications

(11 citation statements)

References 10 publications

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“…This case is similar in some respects to that reported by Elkouzi and colleagues [ 24 ]. Their patient had longstanding ET and then in later life developed degeneration of the ION.…”

Section: Discussionsupporting

confidence: 91%

“…The two cases differ in the sense that the types of changes in the ION were not the same – the published cases had pseudo-hypertrophic olivary degeneration [ 24 ] and ours had olivary degeneration of indeterminate cause. Also, our case came to postmortem, whereas the olivary changes in the published case were noted on brain imaging alone [ 24 ]. As discussed by Elkouzi and colleagues [ 24 ], these cases argue against the ION hypothesis of ET.…”

Section: Discussionmentioning

confidence: 78%

“…Recently, Elkouzi and colleagues [ 24 ] reported a patient with longstanding ET who developed hypertrophic olivary degeneration later in life, characterized clinically by ataxia and palatal tremor and by olivary pseudo-hypertrophy on magnetic resonance imaging. His ET tremor did not lessen despite the onset of this second syndrome of olivary degeneration and, based on this observation, the authors concluded that the ION was not the source of tremor in their patient [ 24 ]. We now report a case with longstanding, severe familial ET.…”

Section: Introductionmentioning

confidence: 99%

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Inferior Olivary nucleus degeneration does not lessen tremor in essential tremor

et al. 2018

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BackgroundIn traditional models of essential tremor, the inferior olivary nucleus was posited to play a central role as the pacemaker for the tremor. However, recent data call this disease model into question.Case presentationOur patient had progressive, long-standing, familial essential tremor. Upper limb tremor began at age 10 and worsened over time. It continued to worsen during the nine-year period he was enrolled in our brain donation program (age 85 – 94 years), during which time the tremor moved from the moderate to severe range on examination. On postmortem examination at age 94, there were degenerative changes in the cerebellar cortex, as have been described in the essential tremor literature. Additionally, there was marked degeneration of the inferior olivary nucleus, which was presumed to be of more recent onset. Such degeneration has not been previously described in essential tremor postmortems. Despite the presence of this degeneration, the patient’s tremor not only persisted but it continued to worsen during the final decade of his life.ConclusionsAlthough the pathophysiology of essential tremor is not completely understood, evidence such as this suggests that the inferior olivary nucleus does not play a critical role in the generation of tremor in these patients.

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“…This case is similar in some respects to that reported by Elkouzi and colleagues [ 24 ]. Their patient had longstanding ET and then in later life developed degeneration of the ION.…”

Section: Discussionsupporting

confidence: 91%

Section: Discussionmentioning

confidence: 78%

Section: Introductionmentioning

confidence: 99%

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Inferior Olivary nucleus degeneration does not lessen tremor in essential tremor

et al. 2018

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“…A recently published case report similarly suggests that the ION is not the source of tremor in ET. 61 In that report, development of hypertrophic olivary degeneration in a patient with longstanding ET of 20 years’ duration did not alter the nature of the tremor. Although the patient developed gait imbalance and palatal tremor, the fact that there was no change in the pattern of upper limb tremor in the presence of a progressively degenerating olivary nucleus, argues against the notion that the ION plays a crucial role in the genesis of tremor in ET.…”

Section: Methodsmentioning

confidence: 80%

The Olivary Hypothesis of Essential Tremor: Time to Lay this Model to Rest?

Louis

Lenka

2017

Tremor and Other Hyperkinetic Movements

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“…However, studies also showed that the inferior olive might not be involved in ET, 41 , 42 and postural tremor could be generated by gabaergic dysfunction of the cerebellar dentate nucleus and thalamus within the cerebellothalamocortical circuit. 43 , 44 Interestingly, the thalamus is preferentially affected in SCA2 patients, which could explain the higher occurrence of postural tremor in SCA2.…”

Section: Discussionmentioning

confidence: 99%