Progressive ataxia and palatal tremor: Two autopsy cases of a novel tauopathy

Gao, Andrew; Faust-Socher, Achinoam; Almurshed, Maryam; Bigio, Marc R. Del; Lang, Anthony E.; Muñoz, David G.

doi:10.1002/mds.27074

Cited by 20 publications

(27 citation statements)

References 39 publications

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“…This retrograde degeneration might cause secondary (deafferentation type) hypertrophic degeneration in other olivary neurons potentially through the loss of axon collaterals. Recently, more widespread 3R (repeat) and 4R tau deposition has been reported in the postmortem study of two patients with PAPT [7]. The current histopathological association of the sporadic PAPT with tau deposition points towards a primary neurodegenerative process [7,28].…”

Section: Pathogenesis Of Progressive Ataxia and Palatal Tremor (Papt)mentioning

confidence: 92%

Palatal Tremor – Pathophysiology, Clinical Features, Investigations, Management and Future Challenges

Bhattacharjee

2020

Tremor and Other Hyperkinetic Movements

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Background: Palatal tremor is involuntary, rhythmic and oscillatory movement of the soft palate. Palatal tremor can be classified into three subtypes; essential, symptomatic and palatal tremor associated with progressive ataxia. Methods: A thorough Pubmed search was conducted to look for the original articles, reviews, letters to editor, case reports, and teaching neuroimages, with the keywords "essential", "symptomatic palatal tremor", "myoclonus", "ataxia", "hypertrophic", "olivary" and "degeneration". Results: Essential palatal tremor is due to contraction of the tensor veli palatini muscle, supplied by the 5 th cranial nerve. Symptomatic palatal tremor occurs due to the contraction of the levator veli palatini muscle, supplied by the 9 th and 10 th cranial nerves. Essential palatal tremor is idiopathic, while symptomatic palatal tremor occurs due to infarction, bleed or tumor within the Guillain-Mollaret triangle. Progressive ataxia and palatal tremor can be familial or idiopathic. Symptomatic palatal tremor and sporadic progressive ataxia with palatal tremor show signal changes in inferior olive of medulla in magnetic resonance imaging. The treatment options available for essential palatal tremor are clonazepam, lamotrigine, sodium valproate, flunarizine and botulinum toxin. The treatment of symptomatic palatal tremor involves the treatment of the underlying cause. Discussion: Further studies are required to understand the cause and pathophysiology of Essential palatal tremor and progressive ataxia and palatal tremor. Similarly, the link between tauopathy and palatal tremor associated progressive ataxia needs to be explored further. Oscillopsia and progressive ataxia are more debilitating than palatal tremor and needs new treatment approaches.

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Section: Pathogenesis Of Progressive Ataxia and Palatal Tremor (Papt)mentioning

confidence: 92%

Palatal Tremor – Pathophysiology, Clinical Features, Investigations, Management and Future Challenges

Bhattacharjee

2020

Tremor and Other Hyperkinetic Movements

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“…Its distribution differed, but generally comprised somatodendritic inclusions in the inferior olives, nigra, locus coeruleus, red nucleus, and thalami alongside cortical and/or hippocampal tau pathology that was interpreted as an incidental finding related to aging. 26,27 Globular Glial Tauopathies (GGT)…”

Section: Idiopathic/environmentally Mediated Disorders Progressive Atmentioning

confidence: 99%

Some New and Unexpected Tauopathies in Movement Disorders

Mulroy

Jaunmuktane

Balint

et al. 2020

Movement Disord Clin Pract

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“…Another syndrome termed progressive ataxia and palatal tremor (PAPT) has also been described; in this presumed neurodegenerative syndrome, a progressive ataxia and palatal/oculopalatal tremor develops in patients without history of a structural brainstem or cerebellar lesion. [119][120][121] Box 6: Chronic Vestibular Syndrome…”

Section: Oculopalatal Tremormentioning

confidence: 99%

Vestibular Disorders: Pearls and Pitfalls

Choi

Gold

2019

Semin Neurol

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Dizziness and vertigo are symptoms that commonly lead patients to seek neurologic or emergency care. Because symptoms are often vague and imprecise, a systematic approach is essential. By categorizing vestibular disorders based on the timing, triggers, and duration of symptoms, as well as emphasizing focused ocular motor and vestibular examinations, the majority of vestibular diagnoses can be made at the bedside. This paper will discuss the pearls and pitfalls in the history and examination of the most common acute, episodic, and chronic vestibular disorders.

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Progressive ataxia and palatal tremor: Two autopsy cases of a novel tauopathy

Cited by 20 publications

References 39 publications

Palatal Tremor – Pathophysiology, Clinical Features, Investigations, Management and Future Challenges

Palatal Tremor – Pathophysiology, Clinical Features, Investigations, Management and Future Challenges

Some New and Unexpected Tauopathies in Movement Disorders

Vestibular Disorders: Pearls and Pitfalls

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