High‐Frequency Palatal Tremor and Stimulus‐Sensitive Leg Myoclonus with Degeneration of Inferior Olivary Nuclei in Celiac Disease

Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration involving the dento-rubro-olivary pathway and presents clinically as palatal tremor. We present a 48 year old male patient who developed Holmes' tremor and bilateral HOD five months after brainstem hemorrhage. The severe rest tremor was refractory to pharmacotherapy and botulinum toxin injections, but was markedly reduced after thalamotomy. Magnetic resonance imaging permitted visualization of HOD, which appeared as a characteristic high signal intensity in the inferior olivary nuclei on T2- and proton-density-weighted images. Enlargement of the inferior olivary nuclei was also noted. Palatal tremor was absent in that moment and appears about two months later. The delayed-onset between insult and tremor following structural lesions of the brain suggest that compensatory or secondary changes in nervous system function must contribute to tremor genesis. The literature and imaging findings of this uncommon condition are reviewed.

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The Dentato-Rubro-Olivary Tract: Clinical Dimension of This Anatomical Pathway

Case Reports in Otolaryngology ◽

10.1155/2013/934386 ◽

2013 ◽

Vol 2013 ◽

pp. 1-3 ◽

Cited By ~ 2

Author(s):

Fadil Khoyratty ◽

Thomas Wilson

Keyword(s):

Red Nucleus ◽

Inferior Olivary Nucleus ◽

Superior Cerebellar Peduncle ◽

Palatal Tremor ◽

History Of ◽

Brachium Conjunctivum ◽

Central Tegmental Tract ◽

Cerebellar Peduncle ◽

Inferior Olivary ◽

Olivary Nucleus

Symptomatic palatal tremor is potentially the result of a lesion in the triangle of Guillain-Mollaret (1931) and is associated with hypertrophic olivary degeneration (HOD) which has characteristic MR findings. The triangle is defined by dentate efferents ascending through the superior cerebellar peduncle and crossing in the decussation of the brachium conjunctivum inferior to the red nucleus, to finaliy reach the inferior olivary nucleus (ION) via the central tegmental tract. The triangle is completed by ION decussating efferents terminating on the original dentate nucleus via the inferior cerebellar peduncle. We can demonstrate the anatomy of this anatomical triangle using a clinical case of palatal tremor presenting with bilateral subjective pulsatile tinnitus along with the pathognomonic MR findings previously described. The hyperintense T2 signal in these patients may be permanent, but the hypertrophied olive normally regresses after 4 years. The temporal relationship between the evolution of the histopathology and the development of the palatal tremor remains unknown as does the natural history of the tremor. Botox injection at the level of tensor and levator veli palatini insertion have been used to treat patients with disabling tremor synchronous tinnitus. A lesion involving the triangle can have a quite varied clinical expression.

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Progressive ataxia and palatal tremor: T1-weighted with magnetization transfer pulse hyperintensity in the inferior olivary nucleus

Arquivos de Neuro-Psiquiatria ◽

10.1590/0004-282x20130014 ◽

2013 ◽

Vol 71 (4) ◽

pp. 264-265 ◽

Cited By ~ 3

Author(s):

James Henrique Yared ◽

Bruno Siqueira Campos Lopes ◽

Ricardo Mendes Rogério ◽

Lázaro Luis Faria do Amaral ◽

Nelson Fortes Paes Diniz Ferreira

Keyword(s):

Magnetization Transfer ◽

Inferior Olivary Nucleus ◽

Palatal Tremor ◽

Progressive Ataxia ◽

Inferior Olivary ◽

Olivary Nucleus

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Palatal Tremor Revisited: Disorder with Nosological Diversity and Etiological Heterogeneity

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/cjn.2017.273 ◽

2017 ◽

Vol 45 (2) ◽

pp. 243-247

Author(s):

Madhu Nagappa ◽

Parayil S. Bindu ◽

Sanjib Sinha ◽

Rose D. Bharath ◽

Mangalore Sandhya ◽

...

Keyword(s):

Magnetic Resonance Imaging ◽

Magnetic Resonance ◽

Case Series ◽

Inferior Olivary Nucleus ◽

Imaging Features ◽

Resonance Imaging ◽

Palatal Tremor ◽

Inferior Olivary ◽

Etiological Heterogeneity ◽

Olivary Nucleus

AbstractThis case series aimed to describe clinicoradiological, electromyographic, and etiological spectra in palatal tremor (essential=1; symptomatic=26). Patients with symptomatic palatal tremor had 2 to 10 Hz arrhythmic electromyographic bursts, a spectrum of changes in inferior olivary nucleus, with/without lesions in Guillain Mollaret triangle, and varied etiologies (genetic=9, vascular=6, trauma=3, infections=3). Exome sequencing showed variations in POLG, WDR81, NDUFS8, TENM4, and EEF2. Clinical phenotypes of patients with POLG, WDR81, and NDUFS8 variations were consistent with that described in literature. We highlight salient magnetic resonance imaging features, electrophysiological observations, and diverse etiologies in a large cohort of palatal tremor.

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