scholarly journals Neuro-Behçet: Pons Involvement with Longitudinal Extension to Midbrain and Hypertrophic Olivary Degeneration

2015 ◽  
Vol 7 (2) ◽  
pp. 148-151 ◽  
Author(s):  
Emilio Franco-Macías ◽  
Florinda Roldán-Lora ◽  
Paula Martínez-Agregado ◽  
Nuria Cerdá-Fuertes ◽  
Francisco Moniche

A 21-year-old right-handed man developed progressive dysarthria and gait disturbance over 4 months (associated with intermittent hiccups). During that time, he also suffered from uveitis. A physical examination showed pseudobulbar and pyramidal signs and genital and oral ulcers. A brain MRI revealed an extensive lesion mainly located in the ventral pons, with an extension upwards to the midbrain. The inferior olivary nucleus also showed hyperintensity. After the treatment with intravenous methylprednisolone and pulses of cyclophosphamide, he improved. As observed on his MRI, his lesions also improved, except for an increase of the inferior olivary nucleus, consistent with hypertrophic olivary degeneration. Neuro-Behçet tropism for ventral brainstem explains the usual presentation with pyramidal syndrome. Hypertrophic olivary degeneration due to pons involvement could explain the hiccup attacks in a few known cases.

2015 ◽  
Vol 5 ◽  
pp. 8 ◽  
Author(s):  
Radha Sarawagi ◽  
Aravind Murugesan

Hypertrophic olivary degeneration (HOD) is seen following lesions in the Guillain–Mollaret triangle. This is unique because the inferior olivary nucleus hypertrophies following degeneration unlike the typical atrophy seen in other structures. We report two cases of HOD in two different clinical scenarios.


2017 ◽  
Vol 31 (2) ◽  
pp. 182-185 ◽  
Author(s):  
Silvia Lana ◽  
Chiara Ganazzoli ◽  
Girolamo Crisi

Hypertrophic olivary degeneration (HOD) is a rare trans-synaptic neuronal degeneration of the inferior olivary nucleus caused by an injury to the dentato-rubro-olivary connection, also known as Guillain-Mollaret triangle. It leads to hypertrophy of the affected nucleus rather than atrophy and is characterized by hyperintensity on T2-weighted images. Unilateral and bilateral cases are described. We present a case of a 70-year-old patient affected by a tumor inside the fourth ventricle who suffered from diplopia and right seventh cranial nerve palsy. He underwent surgery and developed left seventh cranial nerve palsy. Three months after resection, magnetic resonance imaging revealed the appearance of bilateral HOD. This is the first report of bilateral HOD occurrence after surgical bilateral damage of the rubro-olivary fibers running in central tegmental tracts.


2021 ◽  
Vol 16 ◽  
pp. 263310552110074
Author(s):  
Fardad Behzadi ◽  
Peter J Fiester ◽  
Dinesh Rao

Hypertrophic olivary degeneration is a rare condition caused by a lesion in the Guillain-Mollaret triangle which leads to trans-synaptic degeneration resulting in the degenerative hypertrophy of the inferior olivary nucleus. This condition presents clinically with palatal tremor but can also produce ocular myoclonus or cerebellar signs. While any lesion that occurs within the Guillian-Mollaret triangle and results in the deafferentation of the inferior olive can lead to hypertrophic olivary degeneration, the most common etiologies include ischemic and hemorrhagic stroke, vascular malformation, neoplasm, and iatrogenic injury related to surgery. We report a series of 7 patients who presented with this condition bilaterally on MRI imaging, including 1 case which represents the first report of toxoplasmosis leading to the development of bilateral hypertrophic olivary degeneration and only the third reported case, unilateral or bilateral, related to an infectious etiology.


2014 ◽  
Vol 13 (4) ◽  
pp. 408-413 ◽  
Author(s):  
Gunes Orman ◽  
Thangamadhan Bosemani ◽  
George I. Jallo ◽  
Thierry A. G. M. Huisman ◽  
Andrea Poretti

Hypertrophic olivary degeneration (HOD) is a dynamic process caused by disruptive lesions affecting components of the Guillain-Mollaret triangle (GMT). The authors applied diffusion tensor imaging (DTI) to investigate longitudinal changes of the GMT components in a child with HOD after neurosurgery for a midbrain tumor. Diffusion tensor imaging data were acquired on a 1.5-T MRI scanner using a balanced pair of diffusion gradients along 20 noncollinear directions 1 day and 3, 6, and 9 months after surgery. Measurements from regions of interest (ROIs) were sampled in the affected inferior olivary nucleus, ipsilateral red nucleus, and contralateral superior and inferior cerebellar peduncles and dentate nucleus. For each ROI, fractional anisotropy and the mean, axial, and radial diffusivities were calculated. In the affected inferior olivary nucleus, the authors found a decrease in fractional anisotropy and an increase in mean, axial, and radial diffusivities 3 months after surgery, while 3 months later fractional anisotropy increased and diffusivities decreased. For all other GMT components, changes in DTI scalars were less pronounced, and fractional anisotropy mildly decreased over time. A detailed analysis of longitudinal DTI scalars in the various GMT components may shed light on a better understanding of the dynamic complex histopathological processes occurring in pediatric HOD over time.


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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