SHM
MCID: SPR083
MIFTS: 28

Sporadic Hemiplegic Migraine (SHM)

Categories: Eye diseases, Genetic diseases, Neuronal diseases, Rare diseases

Aliases & Classifications for Sporadic Hemiplegic Migraine

MalaCards integrated aliases for Sporadic Hemiplegic Migraine:

Name: Sporadic Hemiplegic Migraine 43
Non-Familial Hemiplegic Migraine 43 70
Migraine, Sporadic Hemiplegic 6 70
Sporadic Hemiplegic Migraines 70
Shm 43

Classifications:



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UMLS 70 C0338485 C1832903 C2367445

Summaries for Sporadic Hemiplegic Migraine

MedlinePlus Genetics : 43 Sporadic hemiplegic migraine is a rare form of migraine headache. Migraines typically cause intense, throbbing pain in one area of the head. Some people with migraines also experience nausea, vomiting, and sensitivity to light and sound. These recurrent headaches typically begin in childhood or adolescence and can be triggered by certain foods, emotional stress, and minor head trauma. Each headache may last from a few hours to a few days.In sporadic hemiplegic migraine and some other types of migraine, a pattern of neurological symptoms called an aura occurs before onset of the headache. An aura commonly includes temporary visual changes such as blind spots (scotomas), flashing lights, zig-zagging lines, and double vision. In people with sporadic hemiplegic migraine, auras are also characterized by temporary numbness or weakness, often affecting one side of the body (hemiparesis). Additional features of an aura can include difficulty with speech, confusion, and drowsiness. An aura typically develops gradually over a few minutes and lasts about an hour.Some people with sporadic hemiplegic migraine experience unusually severe migraine episodes. These episodes can include fever, prolonged weakness, seizures, and coma. Although most people with sporadic hemiplegic migraine recover completely between episodes, neurological symptoms such as memory loss and problems with attention can last for weeks or months. Some affected individuals develop mild but permanent difficulty coordinating movements (ataxia), which may worsen with time, and rapid, involuntary eye movements called nystagmus. Mild to severe intellectual disability has been reported in some people with sporadic hemiplegic migraine.

MalaCards based summary : Sporadic Hemiplegic Migraine, also known as non-familial hemiplegic migraine, is related to migraine with or without aura 1 and familial or sporadic hemiplegic migraine. An important gene associated with Sporadic Hemiplegic Migraine is CACNA1A (Calcium Voltage-Gated Channel Subunit Alpha1 A), and among its related pathways/superpathways is Synaptic vesicle cycle. Affiliated tissues include eye, and related phenotypes are normal and respiratory system

Wikipedia : 73 Sporadic hemiplegic migraine (SHM) is a form of hemiplegic migraine headache isolated cases of which are... more...

Related Diseases for Sporadic Hemiplegic Migraine

Diseases in the Familial Hemiplegic Migraine family:

Migraine, Familial Hemiplegic, 1 Migraine, Familial Hemiplegic, 2
Migraine, Familial Hemiplegic, 3 Sporadic Hemiplegic Migraine
Familial or Sporadic Hemiplegic Migraine

Diseases related to Sporadic Hemiplegic Migraine via text searches within MalaCards or GeneCards Suite gene sharing:

(show top 50) (show all 81)
# Related Disease Score Top Affiliating Genes
1 migraine with or without aura 1 31.4 SCN1A CACNA1A ATP1A2
2 familial or sporadic hemiplegic migraine 31.3 SCN1A CACNA1A ATP1A2
3 familial hemiplegic migraine 30.3 SCN1A CACNA1A ATP1A2
4 migraine with aura 30.3 SCN1A CACNA1A ATP1A2
5 hemiplegia 30.2 SCN1A CACNA1A ATP1A2
6 encephalopathy 30.2 SCN1A CACNA1A
7 patent foramen ovale 30.0 SCN1A ATP1A2
8 headache 29.9 SCN1A CACNA1A ATP1A2
9 seizure disorder 29.9 SCN1A ATP1A2
10 migraine, familial hemiplegic, 2 29.9 SCN1A CACNA1A ATP1A2
11 migraine, familial hemiplegic, 1 29.9 SCN1A CACNA1A ATP1A2
12 status epilepticus 29.9 SCN1A CACNA1A
13 migraine without aura 29.6 SCN1A CACNA1A ATP1A2
14 epilepsy 29.6 SCN1A CACNA1A ATP1A2
15 migraine, familial hemiplegic, 3 29.4 SCN1A CACNA1A ATP1A2
16 spinocerebellar ataxia 6 29.4 SCN1A CACNA1A ATP1A2
17 episodic ataxia, type 2 29.4 SCN1A CACNA1A ATP1A2
18 familiar or sporadic hemiplegic migraine 11.7
19 seizures, benign familial infantile, 1 11.3
20 postgerminal center chronic lymphocytic leukemia/small lymphocytic lymphoma 11.0
21 ataxia and polyneuropathy, adult-onset 10.5
22 aphasia 10.4
23 ocular motor apraxia 10.3
24 ifap syndrome 2 10.2
25 pathologic nystagmus 10.2
26 head injury 10.2
27 leiomyosarcoma 10.1
28 cerebral arteriopathy, autosomal dominant, with subcortical infarcts and leukoencephalopathy, type 1 10.1
29 fibrosis of extraocular muscles, congenital, 1 10.1
30 systemic lupus erythematosus 10.1
31 sturge-weber syndrome 10.1
32 cystic fibrosis 10.1
33 pulmonary arteriovenous fistulas 10.1
34 yemenite deaf-blind hypopigmentation syndrome 10.1
35 developmental and epileptic encephalopathy 42 10.1
36 crest syndrome 10.1
37 amnestic disorder 10.1
38 arteriovenous malformation 10.1
39 telangiectasis 10.1
40 vascular disease 10.1
41 calcinosis 10.1
42 brain edema 10.1
43 lupus erythematosus 10.1
44 speech disorder 10.1
45 scotoma 10.1
46 mitochondrial disorders 10.1
47 pulmonary arteriovenous malformation 10.1
48 weber syndrome 10.1
49 raynaud phenomenon 10.1
50 cerebral hypoxia 10.1

Graphical network of the top 20 diseases related to Sporadic Hemiplegic Migraine:



Diseases related to Sporadic Hemiplegic Migraine

Symptoms & Phenotypes for Sporadic Hemiplegic Migraine

MGI Mouse Phenotypes related to Sporadic Hemiplegic Migraine:

46
# Description MGI Source Accession Score Top Affiliating Genes
1 normal MP:0002873 9.13 ATP1A2 CACNA1A SCN1A
2 respiratory system MP:0005388 8.8 ATP1A2 CACNA1A SCN1A

Drugs & Therapeutics for Sporadic Hemiplegic Migraine

Search Clinical Trials , NIH Clinical Center for Sporadic Hemiplegic Migraine

Genetic Tests for Sporadic Hemiplegic Migraine

Anatomical Context for Sporadic Hemiplegic Migraine

MalaCards organs/tissues related to Sporadic Hemiplegic Migraine:

40
Eye

Publications for Sporadic Hemiplegic Migraine

Articles related to Sporadic Hemiplegic Migraine:

(show top 50) (show all 108)
# Title Authors PMID Year
1
Mutation analysis of the CACNA1A calcium channel subunit gene in 27 patients with sporadic hemiplegic migraine. 61 6
12056940 2002
2
Phenotypes of spinocerebellar ataxia type 6 and familial hemiplegic migraine caused by a unique CACNA1A missense mutation in patients from a large family. 6
12707077 2003
3
Missense CACNA1A mutation causing episodic ataxia type 2. 6
11176968 2001
4
A new CACNA1A gene mutation in acetazolamide-responsive familial hemiplegic migraine and ataxia. 6
10408534 1999
5
R1352Q CACNA1A Variant in a Patient with Sporadic Hemiplegic Migraine, Ataxia, Seizures and Cerebral Oedema: A Case Report. 61
33790770 2021
6
Interictal hemodynamic abnormality during motor activation in sporadic hemiplegic migraine: An explorative study. 61
32977226 2020
7
CACNA1A Gene Variants in Eight Chinese Patients With a Wide Range of Phenotypes. 61
33425808 2020
8
White Matter Hyperintensities in Patients with Sporadic Hemiplegic Migraine. 61
31304994 2019
9
A case of late-onset sporadic hemiplegic migraine. 61
31510845 2019
10
Ictal-Interictal Comparison of FDG-PET Findings in Sporadic Hemiplegic Migraine. 61
32002978 2019
11
Sporadic Hemiplegic Migraine with Psychotic Episode and MRI Abnormalities - Therapeutic Dilemma: a Case Report. 61
30948700 2019
12
Neuronal injuries evidenced by transient cortical magnetic resonance enhancement in hemiplegic migraine: A case report. 61
30092648 2019
13
Migrainous Infarction in a Patient With Sporadic Hemiplegic Migraine and Cystic Fibrosis: A 99mTc-HMPAO Brain SPECT Study. 61
30620050 2019
14
New CACNA1A deletions are associated to migraine phenotypes. 61
30167989 2018
15
The Effect of OnabotulinumtoxinA on Aura Frequency and Severity in Patients With Hemiplegic Migraine: Case Series of 11 Patients. 61
29762872 2018
16
Flunarizine in migraine-related headache prevention: results from 200 patients treated in the UK. 61
29512871 2018
17
Pediatric sporadic hemiplegic migraine (ATP1A2 gene): a case report and brief literature review. 61
29904856 2018
18
Epilepsy in hemiplegic migraine: Genetic mutations and clinical implications. 61
28058944 2018
19
A Novel ATP1A2 Gene Variant Associated With Pure Sporadic Hemiplegic Migraine Improved After Patent Foramen Ovale Closure: A Case Report. 61
29867740 2018
20
De novo exonic duplication of ATP1A2 in Italian patient with hemiplegic migraine: a case report. 61
28593511 2017
21
Combined early treatment in hemiplegic attacks related to CACNA1A encephalopathy with brain oedema: Blocking the cascade? 61
27651281 2017
22
Stroke-like attack: first episode of sporadic hemiplegic migraine. 61
28527090 2017
23
Sporadic hemiplegic migraine and giant tumefactive perivascular space: Is there an association? 61
26696413 2016
24
Intermittent Theta Slowings in Contralateral Side of Weakness after Sleep Deprivation on Spot EEG in Sporadic Hemiplegic Migraine. 61
28101483 2016
25
Clinical and electroencephalographic abnormalities during the full duration of a sporadic hemiplegic migraine attack. 61
27155821 2016
26
A case report of sporadic hemiplegic migraine associated cerebral hypoperfusion: comparison of arterial spin labeling and dynamic susceptibility contrast perfusion MR imaging. 61
26250760 2016
27
Field-testing of the ICHD-3 beta/proposed ICD-11 diagnostic criteria for migraine with aura. 61
25424707 2015
28
[Sporadic hemiplegic migraine]. 61
25944138 2015
29
A Single Amino Acid Deletion (ΔF1502) in the S6 Segment of CaV2.1 Domain III Associated with Congenital Ataxia Increases Channel Activity and Promotes Ca2+ Influx. 61
26716990 2015
30
Biochemical characterization of sporadic/familial hemiplegic migraine mutations. 61
24704353 2014
31
Congenital ataxia and hemiplegic migraine with cerebral edema associated with a novel gain of function mutation in the calcium channel CACNA1A. 61
24836863 2014
32
Functional analysis of human Na(+)/K(+)-ATPase familial or sporadic hemiplegic migraine mutations expressed in Xenopus oocytes. 61
24921013 2014
33
Changes in (99m)Tc-ECD SPECT and magnetic resonance angiography with sporadic hemiplegic migraine in a child. 61
24662647 2014
34
Arterial surgery for sporadic hemiplegic migraine: preliminary results. 61
24703384 2014
35
Rare clinical findings in a patient with sporadic hemiplegic migraine: FDG-PET provides diminished brain metabolism at 10-year follow-up. 61
24270521 2014
36
Clinical spectrum in three families with familial hemiplegic migraine type 2 including a novel mutation in the ATP1A2 gene. 61
24096472 2014
37
Sporadic hemiplegic migraine with permanent neurological deficits. 61
24117121 2014
38
Familial hemiplegic migraine mutations affect Na,K-ATPase domain interactions. 61
23954377 2013
39
The diagnostic role for susceptibility-weighted MRI during sporadic hemiplegic migraine. 61
23766355 2013
40
May a suspicious psychiatric disorder hide sporadic hemiplegic migraine? Genetic test as prompting factor for diagnosis. 61
23397224 2013
41
Transient unilateral spatial neglect during aura in a woman with sporadic hemiplegic migraine. 61
23674829 2013
42
Sporadic hemiplegic migraine in a Nigerian woman. 61
24005593 2013
43
A pediatric sporadic hemiplegic migraine case with perfusion abnormality in perfusion MRI and Diamox 99mTc-HMPAO SPECT. 61
22547402 2013
44
Familial and sporadic hemiplegic migraine: diagnosis and treatment. 61
23203776 2013
45
Sporadic Hemiplegic Migraine with ATP1A2 and Prothrombin Gene Mutations. 61
24396618 2013
46
Migraine with aura in patients over 50 years of age: the Marseille’s registry. 61
22302276 2012
47
Sporadic hemiplegic migraine presenting as acute encephalopathy. 61
22136990 2012
48
Prolonged symptoms in sporadic hemiplegic migraine: aura or migrainous infarction? 61
23196733 2012
49
R583Q CACNA1A variant in SHM1 and ataxia: case report and literature update. 61
22527033 2012
50
Migraine-induced epistaxis and sporadic hemiplegic migraine: unusual features in the same patient. 61
22807908 2012

Variations for Sporadic Hemiplegic Migraine

ClinVar genetic disease variations for Sporadic Hemiplegic Migraine:

6
# Gene Name Type Significance ClinVarId dbSNP ID Position
1 CACNA1A NM_001127222.2(CACNA1A):c.1745G>A (p.Arg582Gln) SNV Pathogenic 8505 rs121908217 GRCh37: 19:13419266-13419266
GRCh38: 19:13308452-13308452

Expression for Sporadic Hemiplegic Migraine

Search GEO for disease gene expression data for Sporadic Hemiplegic Migraine.

Pathways for Sporadic Hemiplegic Migraine

Pathways related to Sporadic Hemiplegic Migraine according to GeneCards Suite gene sharing:

# Super pathways Score Top Affiliating Genes
1
Show member pathways
10.74 CACNA1A ATP1A2

GO Terms for Sporadic Hemiplegic Migraine

Cellular components related to Sporadic Hemiplegic Migraine according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 intercalated disc GO:0014704 9.16 SCN1A ATP1A2
2 T-tubule GO:0030315 8.96 SCN1A ATP1A2
3 neuronal cell body GO:0043025 8.8 SCN1A CACNA1A ATP1A2

Biological processes related to Sporadic Hemiplegic Migraine according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 regulation of ion transmembrane transport GO:0034765 9.37 SCN1A CACNA1A
2 ion transport GO:0006811 9.33 SCN1A CACNA1A ATP1A2
3 sodium ion transport GO:0006814 9.32 SCN1A ATP1A2
4 sodium ion transmembrane transport GO:0035725 9.26 SCN1A ATP1A2
5 ion transmembrane transport GO:0034220 9.13 SCN1A CACNA1A ATP1A2
6 cation transmembrane transport GO:0098655 8.8 SCN1A CACNA1A ATP1A2

Molecular functions related to Sporadic Hemiplegic Migraine according to GeneCards Suite gene sharing:

# Name GO ID Score Top Affiliating Genes
1 ion channel activity GO:0005216 9.16 SCN1A CACNA1A
2 voltage-gated ion channel activity GO:0005244 8.96 SCN1A CACNA1A
3 cation channel activity GO:0005261 8.62 SCN1A CACNA1A

Sources for Sporadic Hemiplegic Migraine

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
19 FMA
20 GARD
28 GO
29 GTR
30 HMDB
31 HPO
32 ICD10
33 ICD10 via Orphanet
34 ICD9CM
35 IUPHAR
36 KEGG
37 LifeMap
39 LOVD
41 MedGen
44 MeSH
45 MESH via Orphanet
46 MGI
49 NCI
50 NCIt
51 NDF-RT
53 NINDS
54 Novoseek
56 OMIM via Orphanet
57 OMIM® (Updated 05-Apr-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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