MCID: PRX009
MIFTS: 33

Paroxysmal Hemicrania

Categories: Neuronal diseases, Rare diseases

Aliases & Classifications for Paroxysmal Hemicrania

MalaCards integrated aliases for Paroxysmal Hemicrania:

Name: Paroxysmal Hemicrania 20 53 58 17 70

Characteristics:

Orphanet epidemiological data:

58
paroxysmal hemicrania
Age of onset: Adult;

Classifications:

Orphanet: 58  
Rare neurological diseases


External Ids:

MESH via Orphanet 45 D051302
ICD10 via Orphanet 33 G44.0
UMLS via Orphanet 71 C1399352
Orphanet 58 ORPHA157835
UMLS 70 C1399352

Summaries for Paroxysmal Hemicrania

GARD : 20 The following summary is from Orphanet, a European reference portal for information on rare diseases and orphan drugs. Orpha Number: 157835 Definition A rare primary headache disorder characterized by multiple attacks of unilateral pain that occur in association with ipsilateral cranial autonomic symptoms. The hallmarks of this syndrome are the relative shortness of the attacks and the complete response to indomethacin therapy. Epidemiology Paroxysmal hemicrania (PH) prevalence is unknown. There is a male-to-female ratio of 1:1. Clinical description The mean age of onset is 40 years (range 3-81 years). PH patients experience purely unilateral, severe to very severe attacks of head pain. The pain is often in the temporal, orbital or supraorbital region. The pain has an abrupt start and cessation and is accompanied by ipsilateral autonomic features, including lacrimation, conjunctival injection, rhinorrhoea, nasal congestion, periorbital oedema, facial flushing, miosis and/or ptosis. PH attacks last 2-30 minutes and occur more than 5 times a day up to 40 times a day with a mean of 11 a day. Patients can have unilateral photophobia and phonophobia ipsilateral to the side of the attack. Osmophobia, nausea or vomiting during the attacks has been reported. Although attacks are most usually spontaneous, attacks can be triggered by neck movements, or pressure over the neck or greater occipital nerves. PH can be classified as episodic or chronic, depending on the duration of pain-free period when untreated. Episodic paroxysmal hemicrania patients have bouts lasting from 7 days to 1 year and separated by pain-free periods more than 3 months, whereas in chronic patients the pain-free period is less than 3 months. As this is a primary headache disorder, patients have normal neurological tests. Etiology Etiology remains unclear. Diagnostic methods Diagnosis is based on clinical history and response to indomethacin. Patients with suspected paroxysmal hemicrania should undergo an oral indomethacin trial or placebo-controlled intramuscular indomethacin test. In adults, one could start with 25mg three times a day, titrating up to 75mg three times a day over the course of 3 weeks. With intramuscular indomethacin 100-200 mg could be used. Differential diagnosis Cluster headache is the main differential diagnosis, given the overlap in duration of the attacks. It is very important to differentiate PH from cluster headache, as treatment differs greatly. Genetic counseling Rare cases of familial PH have been reported. Management and treatment A complete response to indomethacin confirms the diagnosis of PH. Treatment with indomethacin with a median dose of 150 mg/day (ranging from 30-300mg/day) results in dramatic relief of the disabling symptoms caused by PH. Prognosis This is a debilitating headache condition if not accurately diagnosed and managed.

MalaCards based summary : Paroxysmal Hemicrania is related to headache and cluster headache. 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 eye, pituitary and hypothalamus, and related phenotypes are migraine and recurrent paroxysmal headache

NINDS : 53 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

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:

58 31 (show all 22)
# Description HPO Frequency Orphanet Frequency HPO Source Accession
1 migraine 58 31 hallmark (90%) Very frequent (99-80%) HP:0002076
2 recurrent paroxysmal headache 58 31 hallmark (90%) Very frequent (99-80%) HP:0002331
3 ptosis 58 31 frequent (33%) Frequent (79-30%) HP:0000508
4 photophobia 58 31 frequent (33%) Frequent (79-30%) HP:0000613
5 phonophobia 58 31 frequent (33%) Frequent (79-30%) HP:0002183
6 rhinitis 58 31 frequent (33%) Frequent (79-30%) HP:0012384
7 epiphora 58 31 frequent (33%) Frequent (79-30%) HP:0009926
8 rhinorrhea 58 31 frequent (33%) Frequent (79-30%) HP:0031417
9 conjunctival hyperemia 58 31 frequent (33%) Frequent (79-30%) HP:0030953
10 flushing 58 31 frequent (33%) Frequent (79-30%) HP:0031284
11 nausea and vomiting 58 31 occasional (7.5%) Occasional (29-5%) HP:0002017
12 diabetes mellitus 58 31 occasional (7.5%) Occasional (29-5%) HP:0000819
13 hypertension 58 31 occasional (7.5%) Occasional (29-5%) HP:0000822
14 palpebral edema 58 31 occasional (7.5%) Occasional (29-5%) HP:0100540
15 miosis 58 31 occasional (7.5%) Occasional (29-5%) HP:0000616
16 neck pain 58 31 occasional (7.5%) Occasional (29-5%) HP:0030833
17 stiff neck 58 31 occasional (7.5%) Occasional (29-5%) HP:0025258
18 restless legs 58 31 occasional (7.5%) Occasional (29-5%) HP:0012452
19 focal sensory seizure with olfactory features 31 occasional (7.5%) HP:0011161
20 trigeminal neuralgia 58 Excluded (0%)
21 olfactory auras 58 Occasional (29-5%)
22 cluster headache 58 Excluded (0%)

Drugs & Therapeutics for Paroxysmal Hemicrania

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

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

Interventional clinical trials:


# Name Status NCT ID Phase Drugs
1 The Pain Biomarker Study: Changes in Circulating Pain Signalling Molecules With Activation of Pain Receptors Active, not recruiting NCT03511846 Phase 1 Oral capsaicin;Topical capsaicin;Intranasal capsaicin;Low Flow Oxygen;High Flow Oxygen
2 The Will Erwin Headache Research Center Study of Cluster Headache and Trigeminal Neuralgia 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:

40
Eye, Pituitary, Hypothalamus, Pineal, Brain, Heart

Publications for Paroxysmal Hemicrania

Articles related to Paroxysmal Hemicrania:

(show top 50) (show all 407)
# Title Authors PMID Year
1
Differential actions of indomethacin: clinical relevance in headache. 61
32796319 2021
2
Long-Term Outcome of Indomethacin Treatment in Pediatric Patients with Paroxysmal Hemicrania-A Case Series. 61
33546261 2021
3
SUNCT/SUNA in children and adolescents: Application of ICHD-3 criteria and treatment response: Case series of 13 SUNCT/SUNA pediatric cases. 61
32867532 2021
4
Carotid body tumor as a potential cause of paroxysmal hemicrania. 61
33134628 2020
5
Cluster headache and TACs: state of the art. 61
32845489 2020
6
Paroxysmal hemicrania masquerading as a stroke in an elderly gentleman: case report. 61
33028219 2020
7
Very young age of onset in trigemino-autonomic cephalalgias - case report and review of the literature. 61
32664738 2020
8
[Cluster headache and other trigeminal autonomic cephalalgias]. 61
32779921 2020
9
Cerebral Sinus Venous Thrombosis Mimicking Probable Paroxysmal Hemicrania: A Case Report. 61
32118290 2020
10
Headache Attack Similar to Paroxismal Hemicrania Seen During Flight. 61
32493562 2020
11
Do paroxysmal hemicrania and hemicrania continua represent different headaches? A retrospective study. 61
31256266 2019
12
Trigeminal Autonomic Cephalalgias. 61
31563236 2019
13
Multiple cranial nerve blocks for the transitional treatment of chronic headaches. 61
31084198 2019
14
Facial presentations of migraine, TACs, and other paroxysmal facial pain syndromes. 61
31434691 2019
15
Non-invasive vagus nerve stimulation is beneficial in chronic paroxysmal hemicrania. 61
30709897 2019
16
Are repetitive pericranial nerve blocks effective in the management of chronic paroxysmal hemicrania?: A case report. 61
31374009 2019
17
Presentation and Management of Headache in Pituitary Apoplexy. 61
31359174 2019
18
Case Report: Shortest Course of Pediatric Paroxysmal Hemicrania. 61
31264201 2019
19
The Treatment of Trigeminal Autonomic Cephalalgias: An Overview. 61
30703174 2019
20
Trait- and Frequency-Dependent Dysfunctional Habituation to Trigeminal Nociceptive Stimulation in Trigeminal Autonomic Cephalalgias. 61
29655843 2018
21
Cluster Headache and Other Trigeminal Autonomic Cephalalgias. 61
30074553 2018
22
The pathophysiology of the trigeminal autonomic cephalalgias, with clinical implications. 61
28942483 2018
23
When Treatment Establishes Diagnosis: A Case Report of Posttraumatic Chronic Paroxysmal Hemicrania. 61
29708585 2018
24
Paroxysmal Hemicrania. 61
29720814 2018
25
Therapeutic Approaches for the Management of Trigeminal Autonomic Cephalalgias. 61
29516437 2018
26
Overview of Trigeminal Autonomic Cephalalgias: Nosologic Evolution, Diagnosis, and Management. 61
29720817 2018
27
Functional Neuroimaging in Trigeminal Autonomic Cephalalgias. 61
29720819 2018
28
Classification of Trigeminal Autonomic Cephalalgia: What has Changed in International Classification of Headache Disorders-3 Beta? 61
29720818 2018
29
Hints on Diagnosing and Treating Headache. 61
29789115 2018
30
Chronic paroxysmal headache secondary to an orbital metastatic leiomyosarcoma: A case report. 61
28058942 2018
31
Therapeutical approaches to paroxysmal hemicrania, hemicrania continua and short lasting unilateral neuralgiform headache attacks: a critical appraisal. 61
28730562 2017
32
Indomethacin-Responsive Paroxysmal Hemicrania in an Elderly Man: An Unusual Presentation of Pituitary Apoplexy. 61
28980712 2017
33
Tics in TACs: A Step into an Avalanche? Systematic Literature Review and Conclusions. 61
28542727 2017
34
Occipital Nerve Stimulation for Medically Refractory Chronic Paroxysmal Hemicrania. 61
28980700 2017
35
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing-like attacks in a pediatric patient found to have a pontine capillary telangiectasia and developmental venous anomaly: A case report exploring the root of the problem. 61
27342226 2017
36
The anterior hypothalamus in cluster headache. 61
27530226 2017
37
LASH: A Review of the Current Literature. 61
28681219 2017
38
"Cough Hemicrania" - An Overlapping Form of Headache: Case Reports. 61
28411371 2017
39
Management of children and young people with headache. 61
27998893 2017
40
Paroxysmal Hemicrania-Like Headache Secondary to Phosphodiesterase Inhibitors Administration: A Case Report. 61
28188618 2017
41
The cavernous sinus in cluster headache - a quantitative structural magnetic resonance imaging study. 61
27009562 2017
42
The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System. 61
28158167 2017
43
Cracked tooth syndrome mimicking trigeminal autonomic cephalalgia: A report of four cases. 61
28168240 2017
44
Can paroxysmal hemicrania be bilateral? A case report. 61
27023075 2016
45
Alcohol-induced headaches: Evidence for a central mechanism? 61
27114660 2016
46
Trigeminal Autonomic Cephalalgias in Children and Adolescents: Cluster Headache and Related Conditions. 61
27017018 2016
47
Coexistence of contralateral cluster headache and probable paroxysmal hemicrania: a case report. 61
27047722 2016
48
Trigeminal autonomic cephalalgias. 61
24888770 2016
49
Linear headache: clinical characteristics of eight new cases. 61
27057481 2016
50
O043. Frequency-dependent habituation deficit of the nociceptive blink reflex in cluster headache and paroxysmal hemicrania. 61
28132297 2015

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
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 20-May-2021)
61 PubMed
63 QIAGEN
68 SNOMED-CT via HPO
69 Tocris
70 UMLS
71 UMLS via Orphanet
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