MCID: PRX009
MIFTS: 32

Paroxysmal Hemicrania

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Paroxysmal Hemicrania

MalaCards integrated aliases for Paroxysmal Hemicrania:

Name: Paroxysmal Hemicrania 54 55 60 17 74

Characteristics:

Orphanet epidemiological data:

60
paroxysmal hemicrania
Age of onset: Adult;

Classifications:

Orphanet: 60  
Rare neurological diseases


External Ids:

MESH via Orphanet 46 D051302
ICD10 via Orphanet 35 G44.0
UMLS via Orphanet 75 C1399352
Orphanet 60 ORPHA157835
UMLS 74 C1399352

Summaries for Paroxysmal Hemicrania

NIH Rare Diseases : 54 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs.Orpha Number: 157835Disease definitionParoxysmal hemicrania (PH) is a primary headache disorder characterized by multiple attacks of unilateral pain that occur in association with cranial autonomic symptoms. The hallmarks of this syndrome are the relative shortness of the attacks and the complete response to indomethacin therapy.EpidemiologyThe prevalence is estimated to be 1:50,000, with a mean age of onset of 40 years (range 5-68 years) and a slight female predominance (in initial reports) or male-to-female ratio of 1:1.Clinical descriptionMost patients (>60%) describe the pain as severe to very severe, rating it at 10 on a verbal rating scale of 0-10. The pain has a temporal, orbital or supraorbital localization. In rare cases, the pain may involve other areas of the face, head or neck. Accompanying ipsilateral autonomic features include lacrimation, conjunctival injection, rhinorrhea, nasal congestion, ptosis and facial flushing. Unilateral phono- and photophobia are frequent. Osmophobia, nausea or vomiting during the attacks has been reported. The attacks are triggered by stress or relaxation after stress, agitation, exercise, alcohol, neck movement, environmental temperature changes, and dietary products (cheese, chocolate, and coffee). The duration of attacks ranges from 2-30 minutes. Attacks usually have a frequency of above five per day (with a mean of 20 attacks per day). In 80% of patients, the disease is described as chronic (when patients have daily attacks without remission for at least one month). As a rule, patients with PH have normal neurological tests. PH has been reported in association with migraine, cluster headache, trigeminal neuralgia and cough headaches.EtiologyEtiopathology remains unclear. Trauma, arteriovenous malformations and pituitary adenomas may play a causative role.Diagnostic methodsDiagnosis is clinical.Differential diagnosisCluster headache and hemicrania continua (see these terms) are the main differential diagnoses. It is very important to differentiate PH from other trigeminal autonomic cephalgias, as each of these syndromes has a highly selective response to treatment.Genetic counselingFamilial cases of PH have been reported.Management and treatmentA complete response to indomethacin confirms the diagnosis of PH. Treatment with indomethacin with a median dose of 150 mg/day (ranging from 30-300/day) results in dramatic relief of the disabling symptoms caused by PH.Visit the Orphanet disease page for more resources.

MalaCards based summary : Paroxysmal Hemicrania is related to headache and hemicrania continua. An important gene associated with Paroxysmal Hemicrania is CALCA (Calcitonin Related Polypeptide Alpha). The drugs Capsaicin and Pharmaceutical Solutions have been mentioned in the context of this disorder. Affiliated tissues include pituitary, testes and eye, and related phenotypes are migraine and recurrent paroxysmal headache

NINDS : 55 Paroxysmal hemicrania is a rare form of headache that usually begins in adulthood. Patients experience severe throbbing, claw-like, or boring pain usually on one side of the face; in, around, or behind the eye; and occasionally reaching to the back of the neck. This pain may be accompanied by red and tearing eyes, a drooping or swollen eyelid on the affected side of the face, and nasal congestion. Patients may also feel dull pain, soreness, or tenderness between attacks. Attacks of paroxysmal hemicrania typically occur from 5 to 40 times per day and last 2 to 30 minutes. The disorder has two forms: chronic, in which patients experience attacks on a daily basis for a year or more, and episodic, in which the headaches may remit for months or years. Certain movements of the head or neck or external pressure to the neck may trigger these headaches in some patients. The disorder is more common in women than in men.

Related Diseases for Paroxysmal Hemicrania

Diseases related to Paroxysmal Hemicrania via text searches within MalaCards or GeneCards Suite gene sharing:

(show all 13)
# Related Disease Score Top Affiliating Genes
1 headache 10.8
2 hemicrania continua 10.7
3 cluster headache 10.6
4 sunct headache 10.3
5 trigeminal neuralgia 10.2
6 meningioma, familial 10.0
7 brain meningioma 10.0
8 pituitary apoplexy 10.0
9 spinal meningioma 10.0
10 poems syndrome 10.0
11 secretory meningioma 10.0
12 intracranial hypertension 10.0
13 cough headache 10.0

Graphical network of the top 20 diseases related to Paroxysmal Hemicrania:



Diseases related to Paroxysmal Hemicrania

Symptoms & Phenotypes for Paroxysmal Hemicrania

Human phenotypes related to Paroxysmal Hemicrania:

60 33 (show all 22)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 migraine 60 33 hallmark (90%) Very frequent (99-80%) HP:0002076
2 recurrent paroxysmal headache 60 33 hallmark (90%) Very frequent (99-80%) HP:0002331
3 ptosis 60 33 frequent (33%) Frequent (79-30%) HP:0000508
4 photophobia 60 33 frequent (33%) Frequent (79-30%) HP:0000613
5 phonophobia 60 33 frequent (33%) Frequent (79-30%) HP:0002183
6 rhinitis 60 33 frequent (33%) Frequent (79-30%) HP:0012384
7 conjunctival hyperemia 60 33 frequent (33%) Frequent (79-30%) HP:0030953
8 flushing 60 33 frequent (33%) Frequent (79-30%) HP:0031284
9 rhinorrhea 60 33 frequent (33%) Frequent (79-30%) HP:0031417
10 epiphora 33 frequent (33%) HP:0009926
11 diabetes mellitus 60 33 occasional (7.5%) Occasional (29-5%) HP:0000819
12 hypertension 60 33 occasional (7.5%) Occasional (29-5%) HP:0000822
13 nausea and vomiting 60 33 occasional (7.5%) Occasional (29-5%) HP:0002017
14 palpebral edema 60 33 occasional (7.5%) Occasional (29-5%) HP:0100540
15 miosis 60 33 occasional (7.5%) Occasional (29-5%) HP:0000616
16 olfactory auras 60 33 occasional (7.5%) Occasional (29-5%) HP:0011161
17 restless legs 60 33 occasional (7.5%) Occasional (29-5%) HP:0012452
18 stiff neck 60 33 occasional (7.5%) Occasional (29-5%) HP:0025258
19 neck pain 60 33 occasional (7.5%) Occasional (29-5%) HP:0030833
20 trigeminal neuralgia 60 Excluded (0%)
21 increased lacrimation 60 Frequent (79-30%)
22 cluster headache 60 Excluded (0%)

Drugs & Therapeutics for Paroxysmal Hemicrania

Drugs for Paroxysmal Hemicrania (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):

(show all 9)
# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Capsaicin Approved Phase 1 404-86-4 1548943
2 Pharmaceutical Solutions Phase 1
3 Antipruritics Phase 1
4 Dermatologic Agents Phase 1
5 Peripheral Nervous System Agents Phase 1
6
Triamcinolone Approved, Vet_approved 124-94-7 31307
7 Triamcinolone hexacetonide
8 triamcinolone acetonide
9 Triamcinolone diacetate

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 Pain Biomarker Study Active, not recruiting NCT03511846 Phase 1 Oral capsaicin;Topical capsaicin;Intranasal capsaicin;Low Flow Oxygen;High Flow Oxygen
2 French Database of Occipital Nerves Stimulation in the Treatment of Refractory Chronic Headache Disorders Unknown status NCT01842763
3 The Will Erwin Headache Research Center - Cluster Headache Study Recruiting NCT02910323

Search NIH Clinical Center for Paroxysmal Hemicrania

Genetic Tests for Paroxysmal Hemicrania

Anatomical Context for Paroxysmal Hemicrania

MalaCards organs/tissues related to Paroxysmal Hemicrania:

42
Pituitary, Testes, Eye, Brain, Heart

Publications for Paroxysmal Hemicrania

Articles related to Paroxysmal Hemicrania:

(show top 50) (show all 174)
# Title Authors Year
1
Non-invasive vagus nerve stimulation is beneficial in chronic paroxysmal hemicrania. ( 30709897 )
2019
2
When Treatment Establishes Diagnosis: A Case Report of Posttraumatic Chronic Paroxysmal Hemicrania. ( 29708585 )
2018
3
Paroxysmal Hemicrania. ( 29720814 )
2018
4
Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal. ( 28730562 )
2017
5
Paroxysmal Hemicrania-Like Headache Secondary to Phosphodiesterase Inhibitors Administration: A Case Report. ( 28188618 )
2017
6
The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System. ( 28158167 )
2017
7
Occipital Nerve Stimulation for Medically Refractory Chronic Paroxysmal Hemicrania. ( 28980700 )
2017
8
Indomethacin-Responsive Paroxysmal Hemicrania in an Elderly Man: An Unusual Presentation of Pituitary Apoplexy. ( 28980712 )
2017
9
Coexistence of contralateral cluster headache and probable paroxysmal hemicrania: a case report. ( 27047722 )
2016
10
Can paroxysmal hemicrania be bilateral? A case report. ( 27023075 )
2016
11
O043. Frequency-dependent habituation deficit of the nociceptive blink reflex in cluster headache and paroxysmal hemicrania. ( 28132297 )
2015
12
Pediatric paroxysmal hemicrania: a case report and some clinical considerations. ( 26265294 )
2015
13
Paroxysmal hemicrania: an update. ( 24523000 )
2014
14
The coexistence of paroxysmal hemicrania and temporomandibular disorder: importance of multidisciplinary approach. ( 24748314 )
2014
15
Indomethacin-responsive TACs (paroxysmal hemicrania, hemicrania continua, and LASH): further proof of a distinct spectrum of headache disorders. ( 23278259 )
2013
16
Paroxysmal hemicrania: a retrospective study of a consecutive series of 22 patients and a critical analysis of the diagnostic criteria. ( 23566235 )
2013
17
Hemicrania continua changed to chronic paroxysmal hemicrania after treatment with cyclooxygenase-2 inhibitor. ( 21284612 )
2011
18
Further on menstrual paroxysmal hemicrania. ( 21278240 )
2011
19
Chronic paroxysmal hemicrania in paediatric age: report of two cases. ( 21340658 )
2011
20
Paroxysmal hemicrania as the clinical presentation of giant cell arteritis. ( 24765352 )
2011
21
Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua. ( 20352588 )
2010
22
Indomethacin responsive headache syndromes: chronic paroxysmal hemicrania and Hemicrania continua. How they were discovered and what we have learned since. ( 20626997 )
2010
23
Does sphenopalatine endoscopic ganglion block have an effect in paroxysmal hemicrania? A case report. ( 19438918 )
2010
24
Episodic paroxysmal hemicrania without autonomic symptoms: are there possible subgroups in PH? ( 19515120 )
2010
25
Resolution of paroxysmal hemicrania after resection of intracranial meningioma. ( 20148710 )
2010
26
Coexistence of cluster headache and paroxysmal hemicrania: does it exist? A case report and literature review. ( 19277837 )
2009
27
Three-year follow-up of a girl with chronic paroxysmal hemicrania. ( 19068259 )
2009
28
Paroxysmal hemicrania in children--symptoms, diagnostic criteria, therapy and outcome. ( 19236385 )
2009
29
Paroxysmal hemicrania with visual aura in a 17-year-old boy. ( 19348039 )
2009
30
Successful treatment of chronic paroxysmal hemicrania with posterior hypothalamic stimulation: technical case report. ( 19834392 )
2009
31
Paroxysmal hemicrania responding to topiramate. ( 21734918 )
2009
32
A patient with chronic cluster headache responsive to high-dose indomethacin: is there an overlap with chronic paroxysmal hemicrania? ( 18460005 )
2008
33
Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports. ( 17954009 )
2008
34
Bilateral paroxysmal hemicrania with autonomic symptoms: the first case report. ( 17941880 )
2008
35
Paroxysmal hemicrania: a prospective clinical study of 31 cases. ( 18252775 )
2008
36
Paroxysmal hemicrania and POEMS syndrome: further evidence that neuropeptides are implicated in primary headache? ( 18644027 )
2008
37
Headache secondary to haemorrhagic stroke resembling paroxysmal hemicrania. ( 18949281 )
2008
38
Paroxysmal hemicrania responding to topiramate. ( 17172571 )
2007
39
Paroxysmal hemicrania triggered by GTN. ( 17645760 )
2007
40
Menstrual paroxysmal hemicrania, a possible new entity? ( 17655719 )
2007
41
Trigeminal autonomic cephalalgias. Part 2: Paroxysmal hemicrania. ( 17656136 )
2007
42
Paroxysmal hemicrania-tic and Chiari I malformation: an unusual association. ( 17944959 )
2007
43
Paroxysmal hemicrania, SUNCT, and hemicrania continua. ( 16628536 )
2006
44
Chronic paroxysmal hemicrania, hemicrania continua and SUNCT: the fate of the three first described cases. ( 16817054 )
2006
45
Hemicrania continua evolving from episodic paroxysmal hemicrania. ( 16919066 )
2006
46
Posterior hypothalamic activation in paroxysmal hemicrania. ( 16489610 )
2006
47
Chronic paroxysmal hemicrania in a 3-year, 10-month-old female. ( 16504793 )
2006
48
Paroxysmal hemicrania in a family. ( 16556252 )
2006
49
Chronic paroxysmal hemicrania presenting as recurrent orbital inflammation. ( 16643568 )
2006
50
Chronic paroxysmal hemicrania in a patient with a macroprolactinoma. ( 16686914 )
2006

Variations for Paroxysmal Hemicrania

Expression for Paroxysmal Hemicrania

Search GEO for disease gene expression data for Paroxysmal Hemicrania.

Pathways for Paroxysmal Hemicrania

GO Terms for Paroxysmal Hemicrania

Sources for Paroxysmal Hemicrania

3 CDC
7 CNVD
9 Cosmic
10 dbSNP
11 DGIdb
17 EFO
18 ExPASy
20 FMA
29 GO
30 GTR
31 HGMD
32 HMDB
33 HPO
34 ICD10
35 ICD10 via Orphanet
36 ICD9CM
37 IUPHAR
38 KEGG
39 LifeMap
41 LOVD
43 MedGen
45 MeSH
46 MESH via Orphanet
47 MGI
50 NCI
51 NCIt
52 NDF-RT
55 NINDS
56 Novoseek
58 OMIM
59 OMIM via Orphanet
63 PubMed
65 QIAGEN
70 SNOMED-CT via HPO
71 SNOMED-CT via Orphanet
72 TGDB
73 Tocris
74 UMLS
75 UMLS via Orphanet
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