CPD5
MCID: DYS211
MIFTS: 30

Dyssynergia Cerebellaris Myoclonica of Hunt (CPD5)

Categories: Ear diseases, Infectious diseases, Neuronal diseases, Rare diseases
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Aliases & Classifications for Dyssynergia Cerebellaris Myoclonica of Hunt

MalaCards integrated aliases for Dyssynergia Cerebellaris Myoclonica of Hunt:

Name: Dyssynergia Cerebellaris Myoclonica of Hunt 57
Ramsay Hunt Syndrome 57 58 75
Cerebelloparenchymal Disorder V 57 71
Facial Nerve Palsy Due to Herpes Zoster Infection 58
Facial Nerve Paralysis Due to Vzv 58
Facial Nerve Palsy Due to Vzv 58
Spinodentate Atrophy 57
Myoclonus and Ataxia 57
Cpd5 57

Characteristics:


Inheritance:

Autosomal recessive 57

Prevelance:

Ramsay Hunt Syndrome: 1-9/100000 (United States) 58

Age Of Onset:

Ramsay Hunt Syndrome: All ages 58

Classifications:

Orphanet: 58  
Rare neurological diseases
Rare infectious diseases


External Ids:

OMIM® 57 213400
ICD10 via Orphanet 32 B02.2+ G53.0*
UMLS via Orphanet 72 C0017409
Orphanet 58 ORPHA3020
SNOMED-CT via HPO 69 127324008 17450006 85102008
UMLS 71 C3489626

Summaries for Dyssynergia Cerebellaris Myoclonica of Hunt

Orphanet: 58 A rare infectious disease characterized by herpes zoster oticus associated with peripheral facial nerve palsy, often also with other cranial nerve lesions. Patients present with a painful erythematous vesicular rash in and around one ear and facial paralysis on the same side. Other frequent manifestations include hearing loss, tinnitus, vertigo, nausea, vomiting, and nystagmus.

MalaCards based summary: Dyssynergia Cerebellaris Myoclonica of Hunt, also known as ramsay hunt syndrome, is related to geniculate ganglionitis and myoclonic cerebellar dyssynergia. Affiliated tissues include globus pallidus, brain and kidney, and related phenotypes are ataxia and myoclonus

Wikipedia: 75 Three different neurological syndromes carry the name of Ramsay Hunt syndrome. Their only connection is... more...

More information from OMIM: 213400

Related Diseases for Dyssynergia Cerebellaris Myoclonica of Hunt

Diseases related to Dyssynergia Cerebellaris Myoclonica of Hunt via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 149)
# Related Disease Score Top Affiliating Genes
1 geniculate ganglionitis 11.3
2 myoclonic cerebellar dyssynergia 11.2
3 myoclonic epilepsy of unverricht and lundborg 11.2
4 herpes zoster 11.2
5 bell's palsy 11.2
6 herpes zoster oticus 11.2
7 facial paralysis 11.1
8 chickenpox 11.1
9 creutzfeldt-jakob disease 11.0
10 papillon-lefevre syndrome 10.8
11 exanthem 10.8
12 polyneuropathy 10.7
13 neuritis 10.7
14 sensorineural hearing loss 10.7
15 encephalitis 10.6
16 neuropathy 10.5
17 pathologic nystagmus 10.5
18 viral infectious disease 10.5
19 meningitis 10.5
20 myoclonus 10.4
21 unverricht-lundborg syndrome 10.4
22 mitochondrial encephalomyopathy 10.4
23 immune deficiency disease 10.4
24 vestibular neuronitis 10.4
25 acoustic neuroma 10.4
26 neuroma 10.4
27 myoclonus epilepsy 10.4
28 meningoencephalitis 10.3
29 epilepsy 10.3
30 severe acute respiratory syndrome 10.3
31 inappropriate adh syndrome 10.3
32 laryngitis 10.3
33 herpes simplex 10.3
34 photoparoxysmal response 1 10.2
35 guillain-barre syndrome, familial 10.2
36 keratitis, hereditary 10.2
37 thrombophilia due to thrombin defect 10.2
38 celiac disease 1 10.2
39 kearns-sayre syndrome 10.2
40 fanconi anemia, complementation group e 10.2
41 stroke, ischemic 10.2
42 osteonecrosis 10.2
43 aseptic meningitis 10.2
44 guillain-barre syndrome 10.2
45 labyrinthitis 10.2
46 pharyngitis 10.2
47 sensory peripheral neuropathy 10.2
48 cerebellar disease 10.2
49 brain stem infarction 10.2
50 movement disease 10.2

Graphical network of the top 20 diseases related to Dyssynergia Cerebellaris Myoclonica of Hunt:



Diseases related to Dyssynergia Cerebellaris Myoclonica of Hunt

Symptoms & Phenotypes for Dyssynergia Cerebellaris Myoclonica of Hunt

Human phenotypes related to Dyssynergia Cerebellaris Myoclonica of Hunt:

30
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 ataxia 30 HP:0001251
2 myoclonus 30 HP:0001336
3 atrophy of the dentate nucleus 30 HP:0007047

Symptoms via clinical synopsis from OMIM®:

57 (Updated 08-Dec-2022)
Neuro:
ataxia
myoclonic jerks

Lab:
dentate neuron loss
superior cerebellar peduncle fiber loss

Clinical features from OMIM®:

213400 (Updated 08-Dec-2022)

Drugs & Therapeutics for Dyssynergia Cerebellaris Myoclonica of Hunt

Search Clinical Trials, NIH Clinical Center for Dyssynergia Cerebellaris Myoclonica of Hunt

Genetic Tests for Dyssynergia Cerebellaris Myoclonica of Hunt

Anatomical Context for Dyssynergia Cerebellaris Myoclonica of Hunt

Organs/tissues related to Dyssynergia Cerebellaris Myoclonica of Hunt:

MalaCards : Globus Pallidus, Brain, Kidney, Bone, Lung, Cerebellum, Tongue

Publications for Dyssynergia Cerebellaris Myoclonica of Hunt

Articles related to Dyssynergia Cerebellaris Myoclonica of Hunt:

(show top 50) (show all 713)
# Title Authors PMID Year
1
The Ramsay Hunt syndrome is a useful clinical entity. 62 57
2494439 1989
2
Ramsay Hunt syndrome, Unverricht-Lundborg disease, or what? 62 57
2494437 1989
3
The Ramsay Hunt syndrome is no longer a useful diagnostic category. 62 57
2494436 1989
4
Familial myoclonus and ataxia. Pathophysiologic implications. 62 57
13947815 1963
5
Progressive myoclonus epilepsies: specific causes and diagnosis. 57
3088452 1986
6
Familial chorea and myoclonus epilepsy. 57
99688 1978
7
Combined degeneration of globus pallidus and dentate nucleus and their projections. 57
13657305 1959
8
Direction-changing spontaneous nystagmus in patients with dizziness. 62
36454383 2022
9
Clinical analysis of acute peripheral facial palsy in older adults. 62
35920320 2022
10
SORL1 gene mutation and octapeptide repeat insertion in PRNP gene in a case presenting with rapidly progressive dementia and cerebral amyloid angiopathy. 62
35789031 2022
11
Varicella zoster virus associated-polyradiculoneuritis in an elderly woman: A new subtype of varicella zoster virus neuropathy. 62
36450490 2022
12
Features of Audio-Vestibular Deficit and 3D-FLAIR Temporal Bone MRI in Patients with Herpes Zoster Oticus. 62
36423176 2022
13
Bilateral sequential Ramsay Hunt syndrome in an immunocompromised adult: a rare entity. 62
36448928 2022
14
Fear of falling as a potential complication of Ramsay Hunt syndrome in older adults: a case report. 62
36434527 2022
15
Clinical Features and Prognosis of Facial Palsy and Hearing Loss in Patients With Ramsay Hunt Syndrome. 62
36465761 2022
16
Ramsay Hunt syndrome following COVID-19 vaccination. 62
34987077 2022
17
Presence of Varicella Zoster Virus DNA in Saliva May Be Associated with the Severity of Ramsay Hunt Syndrome. 62
36140276 2022
18
A Case Report of Infantile Ramsay Hunt Syndrome. 62
36032869 2022
19
Ramsay Hunt Syndrome: A diagnostic dilemma. 62
36393956 2022
20
Benefits of High-Dose Corticosteroid and Antiviral Agent Combination Therapy in the Treatment of House-Brackman Grade VI Ramsay Hunt Syndrome. 62
35878642 2022
21
Two cases in 2 months of herpes zoster oticus (Ramsay Hunt syndrome) presenting to a rural emergency department having been mis-diagnosed as otitis externa. 62
35475318 2022
22
Intratympanic corticosteroid for Bell's palsy and Ramsay Hunt syndrome: Systematic review and meta-analysis. 62
34980517 2022
23
Ramsay Hunt Syndrome With Cranial Polyneuropathy and Delayed Facial Nerve Palsy: A Case Report. 62
36051720 2022
24
Ramsay Hunt Syndrome. 62
35219691 2022
25
Ramsay Hunt syndrome with multiple cranial neuropathy: a literature review. 62
34687339 2022
26
Cerebral venous thrombosis: A rare complication of Ramsay Hunt syndrome (case report and literature review). 62
35364475 2022
27
Brainstem Infarction and Vertebral Artery Vasculopathy After Ramsay Hunt Syndrome. 62
35351788 2022
28
Authors reply to letter to the editor considering "Ramsay Hunt Syndrome with multiple cranial neuropathy: a literature review". 62
35279738 2022
29
Letter to the Editor considering Ramsay Hunt syndrome with multiple cranial neuropathy: a literature review. 62
34767072 2022
30
Comparative Blink Analysis in Patients With Established Facial Paralysis Using High-Speed Video Analysis. 62
34743154 2022
31
Ramsay Hunt syndrome: New impressions in the era of molecular genetics. 62
35430109 2022
32
Distribution of Medical Service Use for Facial Palsy Between Medicine and Traditional Korean Medicine Based on Population-Based Data of Korea. 62
35437967 2022
33
Temporal Changes in Brain Perfusion in a Patient with Myoclonus and Ataxia Syndrome Associated with COVID-19. 62
35110499 2022
34
Where are the vesicles? A case report of Ramsay Hunt syndrome. 62
35440954 2022
35
Long-term neurological outcomes of children with neuroblastoma with opsoclonus-myoclonus syndrome. 62
35378965 2022
36
Streamlining the Diagnosis of Atypical Facial Palsies: A 5-year Review of 805 Patients. 62
35169520 2022
37
Acute myelitis with multicranial neuritis caused by Varicella zoster virus: a case report. 62
35123429 2022
38
Meningitis/meningoencephalitis caused by varicella zoster virus reactivation: a retrospective single-center case series study. 62
35173869 2022
39
Facial paralysis and vaccinations: a vaccine adverse event reporting system review. 62
34184737 2022
40
[Note for "Ramsay Hunt syndrome and mRNA SARS-COV-2 vaccination"]. 62
34642525 2022
41
Note for "Ramsay Hunt syndrome and mRNA SARS-COV-2 vaccination". 62
34920987 2022
42
Ramsay Hunt Syndrome Presenting as Acute Unilateral Combined Peripheral and Central Vestibulopathies. 62
35021287 2022
43
Ramsay Hunt syndrome and mRNA SARS-COV-2 vaccination. 62
34991852 2022
44
Ramsay Hunt Syndrome in an Epileptic Patient. 62
35435831 2022
45
Ramsay Hunt syndrome and mRNA SARS-COV-2 vaccination. 62
34462613 2022
46
Ramsay Hunt syndrome following mRNA SARS-COV-2 vaccine. 62
34548267 2022
47
Case Report: Opsoclonus-Myoclonus Syndrome Associated With Contactin-Associated Protein-Like 2 and Acetylcholine Receptor Autoantibodies in the Setting of Non-Small Cell Lung Carcinoma. 62
34950395 2022
48
The Spectrum of Vestibular Disorders Presenting With Acute Continuous Vertigo. 62
35911992 2022
49
Ramsay Hunt Syndrome Leading to the Brainstem and Cerebellar Involvement: A Case Report of a Rare Co-occurrence. 62
35342241 2022
50
[Ramsay Hunt syndrome following mRNA SARS-COV-2 vaccine]. 62
34305230 2022

Variations for Dyssynergia Cerebellaris Myoclonica of Hunt

Expression for Dyssynergia Cerebellaris Myoclonica of Hunt

Search GEO for disease gene expression data for Dyssynergia Cerebellaris Myoclonica of Hunt.

Pathways for Dyssynergia Cerebellaris Myoclonica of Hunt

GO Terms for Dyssynergia Cerebellaris Myoclonica of Hunt

Sources for Dyssynergia Cerebellaris Myoclonica of Hunt

2 CDC
6 CNVD
8 Cosmic
9 dbSNP
10 DGIdb
16 EFO
17 ExPASy
18 FMA
19 GARD
27 GO
28 GTR
29 HMDB
30 HPO
31 ICD10
32 ICD10 via Orphanet
33 ICD11
34 ICD9CM
35 IUPHAR
36 LifeMap
38 LOVD
40 MedGen
43 MeSH
44 MESH via Orphanet
45 MGI
48 NCI
49 NCIt
50 NDF-RT
52 NINDS
53 Novoseek
55 ODiseA
56 OMIM via Orphanet
57 OMIM® (Updated 08-Dec-2022)
61 PubChem
62 PubMed
64 QIAGEN
69 SNOMED-CT via HPO
70 Tocris
71 UMLS
72 UMLS via Orphanet
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