Hypertrophic Olivary Degeneration: Neurosurgical Perspective and Literature Review

Önen, Mehmet Reşid; Moore, Kelli; Çıkla, Ulaş; Üçer, Melih; Schmidt, Bradley T.; Field, Aaron S.; Başkaya, Mustafa K.

doi:10.1016/j.wneu.2018.01.150

Cited by 31 publications

(26 citation statements)

References 28 publications

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“…A lesion within the functional brainstem loop of the dentato-rubro-olivary pathway (DROP) leads to secondary trans-synaptic neurodegeneration of the inferior olivary nucleus, a condition called "hypertrophic olivary degeneration" (HOD) [1][2][3][4][5]. The DROP, or so-called Guillain-Mollaret triangle (GMT), consists of three anatomic structures: the medullar inferior olivary nucleus (ION), the mesencephalic red nucleus, and the contralateral dentate nucleus of the cerebellum [5][6][7][8]. Even though the underlying pathology is not yet completely understood, it is agreed that disinhibition of the ION leads to its degenerative hypertrophy and a change in the modulation of cerebellar functioning [1,9,10].…”

Section: Introductionmentioning

confidence: 99%

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

Schaller‐Paule

Foerch

Kluge

et al. 2019

JCM

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(1) Background: A lesion within the dentato-rubro-olivary pathway (DROP) in the posterior fossa can cause secondary neurodegeneration of the inferior olivary nucleus: so-called hypertrophic olivary degeneration (HOD). The clinical syndrome of HOD occurs slowly over months and may be overlooked in progressive neuro-oncological diseases. Posterior fossa tumors are often located near these strategic structures. The goal of this study was to analyze the systematics of HOD occurrence in neuro-oncological patients. (2) Methods: The neuroradiological database of the university healthcare center was scanned for HOD-related terms from 2010 to 2019. After excluding patients with other causes of HOD, 12 datasets from neuro-oncological patients were analyzed under predetermined criteria. (3) Results: Patients received multimodal tumor treatments including neurosurgery, radiotherapy, and chemotherapy. HOD occurred both unilaterally (left n = 4; right n = 5) and bilaterally (n = 3). Though the mass effect of posterior fossa tumors had already affected strategic structures of the DROP, none of the patients showed signs of HOD on MRI until therapeutic measures including neurosurgery affecting the DROP were applied. HOD was visible on MRI within a median of 6 months after the neurosurgical intervention. In 67%, the presumed underlying surgical lesion in the DROP lay in the contralateral dentate nucleus. (4) Conclusion: In a selected cohort of neuro-oncological patients, therapeutic lesions within the DROP were associated with HOD occurrence.

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Section: Introductionmentioning

confidence: 99%

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

Schaller‐Paule

Foerch

Kluge

et al. 2019

JCM

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“…HOD is an unusual type of neuronal degeneration in that it results in neuronal hypertrophy, as opposed to atrophy, which is the most common type of neuronal degeneration seen in other synaptic circuits [9,11,12]. Multiple factors that affect the dentato-rubro-olivary tract have been identified as causes of HOD, including hemorrhage, neoplasms, trauma, demyelination, inflammation, radiation damage, surgical manipulation, and idiopathic nature [13][14][15][16][17][18][19].…”

Section: Discussionmentioning

confidence: 99%

“…The pathophysiologic mechanism for these abnormal movements is believed to be secondary to the loss of inhibitory signals -the same mechanism that promotes hypertrophy [20]. Most cases of HOD are managed with observation, given there is no specific treatment developed at this point [11]. Gabapentin has been proposed as a palliative option for the treatment of the ocular nystagmus associated with HOD.…”

Section: Discussionmentioning

confidence: 99%

Temporal Evolution of Hypertrophic Olivary Degeneration in a Pediatric Patient: A Case Report and Review of Literature

Torres

Vicenty-Padilla

Cay-Martínez

et al. 2020

Cureus

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Hypertrophic olivary degeneration (HOD) is a rare type of neuronal degeneration seen after interruption of the dentato-rubro-olivary tract also known as the Guillain-Mollaret triangle (GMT). It is associated with hypertrophic changes of the inferior olive. Commonly reported in adults, this lesion presents with ataxia and oculopalatal myoclonus. Up to date, few cases have been published in the literature that refer to pediatric cases. This diagnosis is particularly important in the setting of brainstem tumor surgery as it should not be confused for tumor recurrence or metastasis, in turn avoiding unwarranted surgical intervention. We present the case of a 15-year-old male who underwent resection of a left superior cerebellar peduncle (SCP) pilocytic astrocytoma. On follow-up, magnetic resonance imaging (MRI) demonstrated evidence of mild residual tumor as well as progressive engorgement of the inferior olivary nucleus (ION). The patient was clinically asymptomatic and has since been observed expectantly without any issues. We were able to pinpoint the most probable location of injury in our patient's GMT. HOD remains a somewhat obscure entity. Its presentation may be early and not accompanied by significant neurologic findings, in contrast to what has been previously reported. Particularly in neoplastic cases, it may represent a diagnostic challenge and could be easily confused for tumor recurrence. A multidisciplinary approach for this entity, as with other pathologies, is of particular importance. Its proper recognition will result in the best outcomes for the patient.

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“…The most characteristic finding shown on the MRI of the head of patients with SPT and sporadic PAPT is the HOD of the medulla [ 10 11 23 24 25 26 27 28 29 30 32 33 35 69 ]. As mentioned before, the HOD is a transsynaptic degeneration of the inferior olivary nucleus of the medulla because of a lesion within the boundary of the GM triangle [ 24 25 26 69 ].…”

Section: Investigations (Table 4)mentioning

confidence: 99%

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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Hypertrophic Olivary Degeneration: Neurosurgical Perspective and Literature Review

Cited by 31 publications

References 28 publications

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis

Temporal Evolution of Hypertrophic Olivary Degeneration in a Pediatric Patient: A Case Report and Review of Literature

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

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