HOD
MCID: HYP707
MIFTS: 18

Hypertrophic Olivary Degeneration (HOD)

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Hypertrophic Olivary Degeneration

MalaCards integrated aliases for Hypertrophic Olivary Degeneration:

Name: Hypertrophic Olivary Degeneration 20
Hod 20

Classifications:



Summaries for Hypertrophic Olivary Degeneration

GARD : 20 Hypertrophic olivary degeneration (HOD) is a rare neurological condition caused by degeneration in the brain stem, the structure that connects the brain to the spinal cord. Signs and symptoms include palatal tremors, lack of movement coordination ( ataxia ), Holmes tremor, vision problems, muscle weakness, and gait impairment, along with an MRI showing enlargement (hypertrophy) of the inferior olivary nucleus. The inferior olivary nucleus is the lower part of the olivary body, an oval structure located on each side of the brain stem that assists in cerebellar motor learning and functioning. HOD is considered unique because the olive initially becomes hypertrophic rather than atrophic (wasted). Over time, the olive goes through atrophy. These changes can be followed by MRI, and even though the imaging characteristics of HOD resolve, the clinical hallmarks such as palatal tremor persist. HOD is caused by lesions to the dentate-rubro-olivary pathway (or Guillian-Mollaret triangle ). Lesions may be due to bleeding, ischemia (restricted blood flow), vascular malformations, infarct (localized tissue death), tumors, and several neurological diseases, but there are cases of HOD where no cause can be identified. HOD may be classified as unilateral (affecting one side) or bilateral (affecting two sides). The condition is commonly managed with medication, but some cases may require surgery.

MalaCards based summary : Hypertrophic Olivary Degeneration, also known as hod, is related to kearns-sayre syndrome and leigh syndrome. An important gene associated with Hypertrophic Olivary Degeneration is POLG (DNA Polymerase Gamma, Catalytic Subunit). Affiliated tissues include brain, spinal cord and eye.

Related Diseases for Hypertrophic Olivary Degeneration

Diseases related to Hypertrophic Olivary Degeneration via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 75)
# Related Disease Score Top Affiliating Genes
1 kearns-sayre syndrome 29.7 SURF1 POLG
2 leigh syndrome 29.5 SURF1 POLG
3 hypopigmentation, organomegaly, and delayed myelination and development 11.4
4 tremor 10.8
5 myoclonus 10.6
6 ataxia and polyneuropathy, adult-onset 10.6
7 pontine hemorrhage 10.5
8 cavernous malformation 10.4
9 glioma susceptibility 1 10.4
10 malignant astrocytoma 10.4
11 pilocytic astrocytoma 10.4
12 mutism 10.3
13 medulloblastoma 10.2
14 alexander disease 10.2
15 wilson disease 10.2
16 stroke, ischemic 10.2
17 benign ependymoma 10.2
18 cellular ependymoma 10.2
19 pathologic nystagmus 10.2
20 wallerian degeneration 10.2
21 traumatic brain injury 10.2
22 creutzfeldt-jakob disease 10.1
23 multiple sclerosis 10.1
24 epidermoid cysts 10.1
25 ocular motor apraxia 10.1
26 sensory ataxic neuropathy, dysarthria, and ophthalmoparesis 10.1
27 spinocerebellar ataxia 20 10.1
28 diffuse large b-cell lymphoma 10.1
29 lymphoma 10.1
30 amnestic disorder 10.1
31 uveitis 10.1
32 basilar artery occlusion 10.1
33 autosomal dominant cerebellar ataxia 10.1
34 neuroendocrine tumor 10.1
35 relapsing-remitting multiple sclerosis 10.1
36 hemangioma 10.1
37 demyelinating disease 10.1
38 central nervous system lymphoma 10.1
39 cranial nerve palsy 10.1
40 myopathy 10.1
41 cavernous hemangioma 10.1
42 essential tremor 10.1
43 anterograde amnesia 10.1
44 dystonia 10.1
45 cerebrovascular disease 10.1
46 b-cell lymphoma 10.1
47 neuromyelitis optica 10.1
48 athetosis 10.1
49 chronic hiccups 10.1
50 primary central nervous system lymphoma 10.1

Graphical network of the top 20 diseases related to Hypertrophic Olivary Degeneration:



Diseases related to Hypertrophic Olivary Degeneration

Symptoms & Phenotypes for Hypertrophic Olivary Degeneration

Drugs & Therapeutics for Hypertrophic Olivary Degeneration

Search Clinical Trials , NIH Clinical Center for Hypertrophic Olivary Degeneration

Genetic Tests for Hypertrophic Olivary Degeneration

Anatomical Context for Hypertrophic Olivary Degeneration

MalaCards organs/tissues related to Hypertrophic Olivary Degeneration:

40
Brain, Spinal Cord, Eye, Thalamus, Pons

Publications for Hypertrophic Olivary Degeneration

Articles related to Hypertrophic Olivary Degeneration:

(show top 50) (show all 151)
# Title Authors PMID Year
1
Hypertrophic olivary degeneration: Case series and review of literature. 20 61
27772756 2016
2
Hypertrophic Olivary Degeneration: A Neurosurgical Point of View. 61 20
26588253 2016
3
Haploinsufficiency of POU4F1 causes an ataxia syndrome with hypotonia and intention tremor. 61
33783914 2021
4
Holmes tremor and palatal tremor in hypertrophic olivary degeneration: involvement of brainstem. 61
32851537 2021
5
Peribuccal and pharyngeal myorhythmia as a presenting symptom of hypertrophic olivary degeneration: Expert commentary. 61
33771463 2021
6
Peribuccal and pharyngeal myorhythmia as a presenting symptom of hypertrophic olivary degeneration. 61
33685822 2021
7
Association between paraneoplastic rhombencephalitis and hypertrophic olivary degeneration. 61
33452052 2021
8
A Paravermal Trans-Cerebellar Approach to the Posterior Fossa Tumor Causes Hypertrophic Olivary Degeneration by Dentate Nucleus Injury. 61
33445527 2021
9
Mollaret's triangle: An important neuroanatomical territory for all clinicians. 61
33767898 2021
10
Hypertrophic olivary degeneration concomitant with bilateral middle cerebellar peduncles Wallerian degeneration following unilateral pontine infarction. 61
33160302 2020
11
Transient Palatal Tremor and Action Induced Foot and Leg Dystonia due to Hypertrophic Olivary Degeneration: a Case Report. 61
32912540 2020
12
Temporal Evolution of Hypertrophic Olivary Degeneration in a Pediatric Patient: A Case Report and Review of Literature. 61
33240705 2020
13
Hypertrophic Olivary Degeneration and Movement Disorder in a Patient with Familial Creutzfeldt-Jakob Disease. 61
33178508 2020
14
Segmental Myorhythmia with Palatal Tremor Due to Bilateral Hypertrophic Olivary Degeneration in Wilson Disease. 61
33043080 2020
15
A meta-analysis of case studies and clinical characteristics of hypertrophic olivary degeneration secondary to brainstem infarction. 61
33070531 2020
16
Palato-pharyngo-laryngeal myoclonus with recurrent retrograde feeding tube migration after cerebellar hemorrhagic stroke: a case report and review of hypertrophic olivary degeneration. 61
32493244 2020
17
Hypertrophic olivary degeneration: a single case report. 61
31215002 2020
18
Development of Hypertrophic Olivary Degeneration following Pontine Hemorrhage. 61
32339325 2020
19
Hypertrophic Olivary Degeneration and Holmes Tremor: Case Report and Review of the Literature. 61
32084623 2020
20
Transaxonal degenerations of cerebellar connections: the value of anatomical knowledge. 61
32490959 2020
21
A Syndrome of Progressive Ataxia and Palatal Tremor (PAPT) in a Patient with Hypertrophic Olivary Degeneration. 61
32606544 2020
22
Oculopalatal tremor following sequential medullary infarcts that did not cause hypertrophic olivary degeneration. 61
32082592 2020
23
Pediatric case of persistent hiccups associated with hypertrophic olivary degeneration. 61
32419431 2020
24
Microsurgical Management of Midbrain Cavernous Malformations: Predictors of Outcome and Lesion Classification in 72 Patients. 61
30888039 2019
25
Delayed Occurrence of Hypertrophic Olivary Degeneration after Therapy of Posterior Fossa Tumors: A Single Institution Retrospective Analysis. 61
31888178 2019
26
Cerebellar atrophy with Chiari malformation: An example of trans-synaptic degeneration? 61
31447362 2019
27
Ocular Motor and Vestibular Disorders in Brainstem Disease. 61
31688322 2019
28
Hypertrophic olivary degeneration: A case report. 61
31949348 2019
29
Combined hypertrophic olivary degeneration and Wallerian degeneration of the bilateral middle cerebellar peduncles: a case report. 61
30941625 2019
30
Hypertrophic olivary degeneration: A comprehensive review focusing on etiology. 61
31026459 2019
31
Palatal tremor and hypertrophic olivary degeneration as sequelae of basilar artery occlusion. 61
31583194 2019
32
Posterior fossa syndrome and increased mean diffusivity in the olivary bodies. 61
31349230 2019
33
[Hypertrophic olivary degeneration : Cause of new neurological symptoms after stroke]. 61
30488087 2019
34
New Radiologic Findings of Hypertrophic Olivary Degeneration in 2 Patients with Brainstem Lymphoma. 61
30496930 2019
35
Hypertrophic olivary degeneration mimics relapse in neuromyelitis optica spectrum disorder. 61
30745445 2019
36
An Acute Clinical Presentation Associated with Hypertrophic Olivary Degeneration. 61
30688206 2019
37
Hypertrophic olivary degeneration in a 16-year-old girl after subtotal surgery of a brainstem pilocytic astrocytoma - a case report. 61
31019597 2019
38
Diffusion Tensor Imaging in a Case of Pontine Bleeding Showing Hypertrophic Olivary Degeneration and Cerebellar Ataxia. 61
30483105 2018
39
Hypertrophic Olivary Degeneration Following Listerial Rhombencephalitis. 61
30234466 2018
40
Eight-and-a-half syndrome caused by a pontine haemorrhage: a case report and review of the literature. 61
29243534 2018
41
MRI and Tractography in Hypertrophic Olivary Degeneration. 61
29756590 2018
42
Hypertrophic Olivary Degeneration: Neurosurgical Perspective and Literature Review. 61
29382617 2018
43
Bilateral surgical damage of the central tegmental tract resulting in bilateral hypertrophic olivary degeneration: An MRI case report. 61
28650218 2018
44
Hypertrophic olivary degeneration in children after posterior fossa surgery. An underdiagnosed condition. 61
29279964 2018
45
Unilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature. 61
29061458 2018
46
Idiopathic bilateral hypertrophic olivary degeneration. 61
29275394 2017
47
Teaching NeuroImages: Bilateral hypertrophic olivary degeneration following posterior circulation stroke. 61
29084930 2017
48
Progressive ataxia and palatal tremor: Two autopsy cases of a novel tauopathy. 61
28736850 2017
49
Hypertrophic olivary degeneration after cerebellar hemorrhage. 61
28647093 2017
50
Pontine Hemorrhage Causing Bilateral Hypertrophic Olivary Degeneration. 61
28694652 2017

Variations for Hypertrophic Olivary Degeneration

Expression for Hypertrophic Olivary Degeneration

Search GEO for disease gene expression data for Hypertrophic Olivary Degeneration.

Pathways for Hypertrophic Olivary Degeneration

GO Terms for Hypertrophic Olivary Degeneration

Sources for Hypertrophic Olivary Degeneration

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
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30 HMDB
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32 ICD10
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56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
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71 UMLS via Orphanet
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