MCID: HYP707
MIFTS: 17

Hypertrophic Olivary Degeneration

Categories: Rare diseases

Aliases & Classifications for Hypertrophic Olivary Degeneration

MalaCards integrated aliases for Hypertrophic Olivary Degeneration:

Name: Hypertrophic Olivary Degeneration 53
Hod 53

Classifications:



Summaries for Hypertrophic Olivary Degeneration

NIH Rare Diseases : 53 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 pontine hemorrhage and tremor. An important gene associated with Hypertrophic Olivary Degeneration is POLG (DNA Polymerase Gamma, Catalytic Subunit). Affiliated tissues include brain, spinal cord and pons.

Related Diseases for Hypertrophic Olivary Degeneration

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

(show all 19)
# Related Disease Score Top Affiliating Genes
1 pontine hemorrhage 10.4
2 tremor 10.3
3 cavernous malformation 10.1
4 medulloblastoma 10.0
5 wilson disease 10.0
6 brain injury 10.0
7 hemangioma 10.0
8 traumatic brain injury 10.0
9 lymphoma 10.0
10 neuroendocrine tumor 10.0
11 central nervous system lymphoma 10.0
12 mutism 10.0
13 cavernous hemangioma 10.0
14 ependymoma 10.0
15 primary central nervous system lymphoma 10.0
16 myoclonus 10.0
17 mitochondrial disorders 9.3 POLG SURF1
18 leigh syndrome 9.2 POLG SURF1
19 charcot-marie-tooth disease 9.0 POLG SURF1

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:

41
Brain, Spinal Cord, Pons

Publications for Hypertrophic Olivary Degeneration

Articles related to Hypertrophic Olivary Degeneration:

(show all 42)
# Title Authors Year
1
Hypertrophic Olivary Degeneration: Neurosurgical Perspective and Literature Review. ( 29382617 )
2018
2
Hypertrophic olivary degeneration in children after posterior fossa surgery. An underdiagnosed condition. ( 29279964 )
2018
3
Unilateral Symptomatic Hypertrophic Olivary Degeneration Secondary to Midline Brainstem Cavernous Angioma: A Case Report and Review of the Literature. ( 29061458 )
2018
4
MRI and Tractography in Hypertrophic Olivary Degeneration. ( 29756590 )
2018
5
Pontine Hemorrhage Causing Bilateral Hypertrophic Olivary Degeneration. ( 28694652 )
2017
6
The dentato-rubro-olivary pathway revisited: New MR imaging observations regarding hypertrophic olivary degeneration. ( 28247932 )
2017
7
Hypertrophic olivary degeneration. ( 28754749 )
2017
8
Bilateral surgical damage of the central tegmental tract resulting in bilateral hypertrophic olivary degeneration: An MRI case report. ( 28650218 )
2017
9
Hypertrophic olivary degeneration after cerebellar hemorrhage. ( 28647093 )
2017
10
Idiopathic bilateral hypertrophic olivary degeneration. ( 29275394 )
2017
11
Hypertrophic Olivary Degeneration and Palatal or Oculopalatal Tremor. ( 28706504 )
2017
12
Teaching Neuro<i>Images</i>: Bilateral hypertrophic olivary degeneration following posterior circulation stroke. ( 29084930 )
2017
13
Hypertrophic olivary degeneration: Case series and review of literature. ( 27772756 )
2016
14
Hypertrophic olivary degeneration: A clinico-radiologic study. ( 27132500 )
2016
15
Hypertrophic olivary degeneration: unveiling the triangle of Guillain-Mollaret. ( 27191241 )
2016
16
Bilateral Hypertrophic Olivary Degeneration and Holmes Tremor without Palatal Tremor: An Unusual Association. ( 27536461 )
2016
17
Post-operative pediatric cerebellar mutism syndrome and its association with hypertrophic olivary degeneration. ( 27942473 )
2016
18
Hypertrophic Olivary Degeneration: A Neurosurgical Point of View. ( 26588253 )
2016
19
Teaching NeuroImages: hypertrophic olivary degeneration in a young man with POLG gene mutation. ( 25713120 )
2015
20
Hypertrophic Olivary Degeneration Secondary to Neuro-BehAset's Disease. ( 25801285 )
2015
21
A case of hypertrophic olivary degeneration after resection of cavernomas of the brain stem and review of the literature. ( 26504394 )
2015
22
Unusual case of posterior fossa syndrome and bilateral hypertrophic olivary degeneration after surgical removal of a large fourth ventricle ependymoma in an adult. ( 25956396 )
2015
23
MRI Findings in Nonlesional Hypertrophic Olivary Degeneration. ( 26073621 )
2015
24
Hypertrophic olivary degeneration secondary to traumatic brain injury: a unique form of trans-synaptic degeneration. ( 26139651 )
2015
25
Hypertrophic olivary degeneration resulting from posterior fossa masses and their treatments. ( 26104225 )
2015
26
Hypertrophic olivary degeneration due to the presence of pontine cavernomas. ( 26588643 )
2015
27
Hypertrophic olivary degeneration secondary to central tegmental tract injury. ( 26185351 )
2015
28
Neuro-BehAset: Pons Involvement with Longitudinal Extension to Midbrain and Hypertrophic Olivary Degeneration. ( 26265903 )
2015
29
Hypertrophic olivary degeneration and holmes' tremor secondary to bleeding of cavernous malformation in the midbrain. ( 25332842 )
2014
30
Hypertrophic olivary degeneration after pontine hemorrhage: a cause of delayed neurological deterioration. ( 24029616 )
2013
31
Hypertrophic olivary degeneration on magnetic resonance imaging in mitochondrial syndromes associated with POLG and SURF1 mutations. ( 22729384 )
2013
32
Bilateral hypertrophic olivary degeneration in Wilson disease. ( 23482821 )
2013
33
A rare presentation of hypertrophic olivary degeneration secondary to primary central nervous system lymphoma. ( 23857254 )
2013
34
Hypertrophic olivary degeneration with gadolinium enhancement after posterior fossa surgery in a child with medulloblastoma. ( 24122017 )
2013
35
Hypertrophic Olivary Degeneration Due to Pontine Hemorrhage. ( 23921433 )
2013
36
Hypertrophic olivary degeneration after pontine hemorrhage. ( 21643689 )
2012
37
Collision in the inferior olive: hypertrophic olivary degeneration complicated by radiation necrosis in brainstem primitive neuroendocrine tumor. ( 22726977 )
2012
38
Hypertrophic olivary degeneration after resection of a pontine cavernous malformation: a case report. ( 22470783 )
2011
39
Palatal myoclonus secondary to hypertrophic olivary degeneration. ( 19623523 )
2009
40
Hypertrophic olivary degeneration following surgical excision of brainstem cavernous hemangioma: a case report. ( 10631896 )
1999
41
Diagnosis please. Case 17: hypertrophic olivary degeneration secondary to pontine hemorrhage. ( 10580959 )
1999
42
Midbrain tremor and hypertrophic olivary degeneration after pontine hemorrhage. ( 9159743 )
1997

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 ExPASy
19 FMA
28 GO
29 GTR
30 HGMD
31 HMDB
32 HPO
33 ICD10
34 ICD10 via Orphanet
35 ICD9CM
36 IUPHAR
37 KEGG
38 LifeMap
40 LOVD
42 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
54 NINDS
55 Novoseek
57 OMIM
58 OMIM via Orphanet
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 SNOMED-CT via Orphanet
71 TGDB
72 Tocris
73 UMLS
74 UMLS via Orphanet
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