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MCID: PRX009
MIFTS: 33
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Paroxysmal Hemicrania
Categories:
Neuronal diseases, Rare diseases
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MalaCards integrated aliases for Paroxysmal Hemicrania:Characteristics:Orphanet epidemiological data:58
paroxysmal hemicrania
Age of onset: Adult; Classifications:
ICD10:
33
Orphanet: 58
Rare neurological diseases |
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NIH Rare Diseases :
52
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. Visit the Orphanet disease page for more resources.
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, testes and brain, 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. |
Human phenotypes related to Paroxysmal Hemicrania:58 31 (show all 22)
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Drugs for Paroxysmal Hemicrania (from DrugBank, HMDB, Dgidb, PharmGKB, IUPHAR, NovoSeek, BitterDB):(show all 7)
Interventional clinical trials:
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MalaCards organs/tissues related to Paroxysmal Hemicrania:40
Eye,
Testes,
Brain,
Hypothalamus,
Pituitary,
Heart,
Pineal
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Articles related to Paroxysmal Hemicrania:(show top 50) (show all 399)
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Genes related to Paroxysmal Hemicrania (0 elite genes): - Elite gene
- Cancer Census gene in COSMIC
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Search
GEO
for disease gene expression data for Paroxysmal Hemicrania.
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