MCID: PRX009
MIFTS: 32

Paroxysmal Hemicrania

Categories: Rare diseases, Neuronal diseases

Aliases & Classifications for Paroxysmal Hemicrania

MalaCards integrated aliases for Paroxysmal Hemicrania:

Name: Paroxysmal Hemicrania 53 54 59 73

Characteristics:

Orphanet epidemiological data:

59
paroxysmal hemicrania
Age of onset: Adult;

Classifications:

Orphanet: 59  
Rare neurological diseases


External Ids:

Orphanet 59 ORPHA157835
ICD10 via Orphanet 34 G44.0
MESH via Orphanet 45 D051302
UMLS via Orphanet 74 C1399352
UMLS 73 C1399352

Summaries for Paroxysmal Hemicrania

NIH Rare Diseases : 53 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 Triamcinolone and triamcinolone acetonide have been mentioned in the context of this disorder. Affiliated tissues include pituitary, eye and testes, and related phenotypes are migraine and recurrent paroxysmal headache

NINDS : 54 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 11)
# Related Disease Score Top Affiliating Genes
1 headache 10.6
2 hemicrania continua 10.5
3 cluster headache 10.5
4 sunct headache 10.0
5 trigeminal neuralgia 10.0
6 aneurysm 9.9
7 aging 9.9
8 pituitary apoplexy 9.9
9 poems syndrome 9.9
10 intracranial hypertension 9.9
11 cough headache 9.9

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:

59 32 (show all 22)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 migraine 59 32 hallmark (90%) Very frequent (99-80%) HP:0002076
2 recurrent paroxysmal headache 59 32 hallmark (90%) Very frequent (99-80%) HP:0002331
3 ptosis 59 32 frequent (33%) Frequent (79-30%) HP:0000508
4 photophobia 59 32 frequent (33%) Frequent (79-30%) HP:0000613
5 phonophobia 59 32 frequent (33%) Frequent (79-30%) HP:0002183
6 rhinitis 59 32 frequent (33%) Frequent (79-30%) HP:0012384
7 conjunctival hyperemia 59 32 frequent (33%) Frequent (79-30%) HP:0030953
8 flushing 59 32 frequent (33%) Frequent (79-30%) HP:0031284
9 rhinorrhea 59 32 frequent (33%) Frequent (79-30%) HP:0031417
10 miosis 59 32 occasional (7.5%) Occasional (29-5%) HP:0000616
11 diabetes mellitus 59 32 occasional (7.5%) Occasional (29-5%) HP:0000819
12 hypertension 59 32 occasional (7.5%) Occasional (29-5%) HP:0000822
13 nausea and vomiting 59 32 occasional (7.5%) Occasional (29-5%) HP:0002017
14 olfactory auras 59 32 occasional (7.5%) Occasional (29-5%) HP:0011161
15 restless legs 59 32 occasional (7.5%) Occasional (29-5%) HP:0012452
16 stiff neck 59 32 occasional (7.5%) Occasional (29-5%) HP:0025258
17 neck pain 59 32 occasional (7.5%) Occasional (29-5%) HP:0030833
18 palpebral edema 59 32 occasional (7.5%) Occasional (29-5%) HP:0100540
19 increased lacrimation 59 Frequent (79-30%)
20 cluster headache 59 Excluded (0%)
21 trigeminal neuralgia 59 Excluded (0%)
22 epiphora 32 frequent (33%) HP:0009926

Drugs & Therapeutics for Paroxysmal Hemicrania

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


# Name Status Phase Clinical Trials Cas Number PubChem Id
1
Triamcinolone Approved, Vet_approved 124-94-7 31307
2 triamcinolone acetonide
3 Triamcinolone diacetate
4 Triamcinolone hexacetonide

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:

41
Pituitary, Eye, Testes, Heart, Brain

Publications for Paroxysmal Hemicrania

Articles related to Paroxysmal Hemicrania:

(show top 50) (show all 167)
# Title Authors Year
1
Paroxysmal Hemicrania. ( 29720814 )
2018
2
When Treatment Establishes Diagnosis: A Case Report of Posttraumatic Chronic Paroxysmal Hemicrania. ( 29708585 )
2018
3
Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal. ( 28730562 )
2017
4
Indomethacin-Responsive Paroxysmal Hemicrania in an Elderly Man: An Unusual Presentation of Pituitary Apoplexy. ( 28980712 )
2017
5
Occipital Nerve Stimulation for Medically Refractory Chronic Paroxysmal Hemicrania. ( 28980700 )
2017
6
Paroxysmal Hemicrania-Like Headache Secondary to Phosphodiesterase Inhibitors Administration: A Case Report. ( 28188618 )
2017
7
The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System. ( 28158167 )
2017
8
Coexistence of contralateral cluster headache and probable paroxysmal hemicrania: a case report. ( 27047722 )
2016
9
Can paroxysmal hemicrania be bilateral? A case report. ( 27023075 )
2016
10
Pediatric paroxysmal hemicrania: a case report and some clinical considerations. ( 26265294 )
2015
11
O043. Frequency-dependent habituation deficit of the nociceptive blink reflex in cluster headache and paroxysmal hemicrania. ( 28132297 )
2015
12
Paroxysmal hemicrania: an update. ( 24523000 )
2014
13
The coexistence of paroxysmal hemicrania and temporomandibular disorder: importance of multidisciplinary approach. ( 24748314 )
2014
14
Paroxysmal hemicrania: a retrospective study of a consecutive series of 22 patients and a critical analysis of the diagnostic criteria. ( 23566235 )
2013
15
Indomethacin-responsive TACs (paroxysmal hemicrania, hemicrania continua, and LASH): further proof of a distinct spectrum of headache disorders. ( 23278259 )
2013
16
Paroxysmal hemicrania as the clinical presentation of giant cell arteritis. ( 24765352 )
2011
17
Chronic paroxysmal hemicrania in paediatric age: report of two cases. ( 21340658 )
2011
18
Further on menstrual paroxysmal hemicrania. ( 21278240 )
2011
19
Hemicrania continua changed to chronic paroxysmal hemicrania after treatment with cyclooxygenase-2 inhibitor. ( 21284612 )
2011
20
Episodic paroxysmal hemicrania without autonomic symptoms: are there possible subgroups in PH? ( 19515120 )
2010
21
Indomethacin responsive headache syndromes: chronic paroxysmal hemicrania and Hemicrania continua. How they were discovered and what we have learned since. ( 20626997 )
2010
22
Resolution of paroxysmal hemicrania after resection of intracranial meningioma. ( 20148710 )
2010
23
Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua. ( 20352588 )
2010
24
Does sphenopalatine endoscopic ganglion block have an effect in paroxysmal hemicrania? A case report. ( 19438918 )
2010
25
Successful treatment of chronic paroxysmal hemicrania with posterior hypothalamic stimulation: technical case report. ( 19834392 )
2009
26
Coexistence of cluster headache and paroxysmal hemicrania: does it exist? A case report and literature review. ( 19277837 )
2009
27
Paroxysmal hemicrania responding to topiramate. ( 21734918 )
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
Three-year follow-up of a girl with chronic paroxysmal hemicrania. ( 19068259 )
2009
31
Paroxysmal hemicrania and POEMS syndrome: further evidence that neuropeptides are implicated in primary headache? ( 18644027 )
2008
32
Paroxysmal hemicrania: a prospective clinical study of 31 cases. ( 18252775 )
2008
33
Headache secondary to haemorrhagic stroke resembling paroxysmal hemicrania. ( 18949281 )
2008
34
Chronic paroxysmal hemicrania and hemicrania continua responding to topiramate: two case reports. ( 17954009 )
2008
35
A patient with chronic cluster headache responsive to high-dose indomethacin: is there an overlap with chronic paroxysmal hemicrania? ( 18460005 )
2008
36
Menstrual paroxysmal hemicrania, a possible new entity? ( 17655719 )
2007
37
Paroxysmal hemicrania responding to topiramate. ( 17172571 )
2007
38
Paroxysmal hemicrania-tic and Chiari I malformation: an unusual association. ( 17944959 )
2007
39
Paroxysmal hemicrania triggered by GTN. ( 17645760 )
2007
40
Trigeminal autonomic cephalalgias. Part 2: Paroxysmal hemicrania. ( 17656136 )
2007
41
Hemicrania continua evolving from episodic paroxysmal hemicrania. ( 16919066 )
2006
42
Chronic paroxysmal hemicrania presenting as recurrent orbital inflammation. ( 16643568 )
2006
43
Association between chronic paroxysmal hemicrania and primary trochlear headache: pathophysiology and treatment. ( 16961797 )
2006
44
Paroxysmal hemicrania, SUNCT, and hemicrania continua. ( 16628536 )
2006
45
Posterior hypothalamic activation in paroxysmal hemicrania. ( 16489610 )
2006
46
Chronic paroxysmal hemicrania: from the index patient to the disease. ( 16834945 )
2006
47
Chronic paroxysmal hemicrania in a patient with a macroprolactinoma. ( 16686914 )
2006
48
Impact of extreme light exposure during summer season in an arctic area on patients with migraine and chronic paroxysmal hemicrania (CPH). ( 16919069 )
2006
49
Black, white and shades of grey: SUNCT or short-lasting chronic paroxysmal hemicrania? ( 17119794 )
2006
50
Chronic paroxysmal hemicrania in a 3-year, 10-month-old female. ( 16504793 )
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 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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