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

2013 ◽  
Vol 71 (4) ◽  
pp. 264-265 ◽  
Author(s):  
James Henrique Yared ◽  
Bruno Siqueira Campos Lopes ◽  
Ricardo Mendes Rogério ◽  
Lázaro Luis Faria do Amaral ◽  
Nelson Fortes Paes Diniz Ferreira
2020 ◽  
Vol 79 (6) ◽  
pp. 641-646
Author(s):  
Matthew R McCord ◽  
Eileen H Bigio ◽  
Kwok Ling Kam ◽  
Victoria Fischer ◽  
Farres Obeidin ◽  
...  

Abstract Spinocerebellar ataxia type 3 (SCA3), also known by the eponym Machado-Joseph disease, is an autosomal dominant CAG trinucleotide (polyglutamine) repeat disease that presents in young- to middle-aged adults. SCA3 was first described in Azorean individuals and has interesting epidemiological patterns. It is characterized clinically by progressive ataxia and neuropathologically by progressive degenerative changes in the spinal cord and cerebellum, along with degeneration of the cortex and basal ganglia. Here, we describe the clinical and neuropathologic features in a case of SCA3 with unique findings, including involvement of the inferior olivary nucleus and cerebellar Purkinje cell layer, which are classically spared in the disease. We also discuss research into the disease mechanisms of SCA3 and the potential for therapeutic intervention.


2013 ◽  
Vol 2013 ◽  
pp. 1-3 ◽  
Author(s):  
Fadil Khoyratty ◽  
Thomas Wilson

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.


Author(s):  
Madhu Nagappa ◽  
Parayil S. Bindu ◽  
Sanjib Sinha ◽  
Rose D. Bharath ◽  
Mangalore Sandhya ◽  
...  

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.


1985 ◽  
Vol 24 (7) ◽  
pp. 645-654 ◽  
Author(s):  
L.A. Barragan ◽  
N. Delhaye-Bouchaud ◽  
P. Laget

2018 ◽  
Vol 5 (1) ◽  
Author(s):  
Elan D. Louis ◽  
Daniel Trujillo Diaz ◽  
Sheng-Han Kuo ◽  
Shi-Rui Gan ◽  
Etty P. Cortes ◽  
...  

2008 ◽  
Vol 104 (5) ◽  
pp. 1351-1358 ◽  
Author(s):  
Jianguo Zhuang ◽  
Fadi Xu ◽  
Donald T. Frazier

Several studies have demonstrated that cerebellar deep nuclei, particularly the rostral fastigial nucleus (FNr), are involved in respiratory modulation. These nuclei receive inputs from the contralateral caudal inferior olivary nuclei of the medulla. The objectives of this study were to determine whether electrical and chemical activation of the vicinity of the caudal inferior olivary nuclei (vIOc) affected respiration and, if true, whether the FNr was involved in the vIOc stimulation-evoked ventilatory responses. Experiments were conducted in 30 anesthetized and spontaneously breathing rats. Our results showed that 1) electrical (25 or 100 μA at 10 or 20 Hz for 10 s) and chemical (1 or 100 mM, 25–50 nl N-methyl-d-aspartate) stimulation of the vIOc augmented ventilation predominantly via increasing tidal volume; 2) the responses to the electrical stimulation were almost eliminated by lesion of the contralateral FNr via microinjection of ibotenic acid; and 3) the respiratory responses to electrical stimulation in the vicinity of the rostral IO were 65–70% smaller compared with that evoked by vIOc stimulation. These findings strongly suggest that vIOc neurons play a significant role in modulation of respiratory activity, largely depending on their projections to the FNr.


1993 ◽  
Vol 70 (5) ◽  
pp. 2181-2186 ◽  
Author(s):  
I. Lampl ◽  
Y. Yarom

1. Subthreshold membrane potential oscillations have been observed in different types of CNS neurons. In this in vitro study, we examined the possible role of these oscillations by analyzing the responses of neurons from the inferior olivary nucleus to a combined stimulation of sine wave and synaptic potentials. 2. A nonlinear summation of the sine wave and the synaptic potential occurred in olivary neurons; a superlinear summation occurred when the synaptic potential was elicited at the trough of the sine wave or during the rising phase. On the other hand, a less than linear summation occurred when the synaptic potentials were evoked during the falling phase of the wave. 3. Significant changes in the delay of the synaptic responses were observed. As a result of these changes, the maximum amplitude of the response occurred at the peak of the sine wave, regardless of the exact time of stimulation. The output of the neuron was therefore synchronized with the sine wave and depended only partly on the input phase. 4. These data demonstrate that neurons from the inferior olivary nucleus are capable of operating as accurate synchronizing devices. Moreover, by affecting the delay line, they act as a logic gate that ensures that the information will be added to the system only at given times.


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