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Annals of Indian Academy of Neurology Apr 2018Paroxysmal hemicrania (PH) is a primary headache disorder belonging to the group of trigeminal autonomic cephalalgias(TACs). Patients typically experience intense... (Review)
Review
Paroxysmal hemicrania (PH) is a primary headache disorder belonging to the group of trigeminal autonomic cephalalgias(TACs). Patients typically experience intense lateralzsed headaches with pain primarily in the ophthalmic trigeminal distribution (V1) associated with superimposed ipsilateral cranial autonomic features. PH is distinguished from other TACs by an exquisite responsiveness to therapeutic doses of indomethacin. Patients may need to be maintained on indomethacin for several months before trials of reduction can be attempted. The disorder does have a tendency toward chronicity. PH is uncommon, but early recognition will prompt initiation of effective treatment to avoid unsuccessful trials of drugs effective in other primary headaches. As with other TACs, hypothalamic and trigeminovascular mechanisms are implicated in the pathophysiologic mechanism of PH. Neuroimaging findings in PH demonstrate a posterior hypothalamic activation similar to that observed in the other TACs. This review will address the epidemiology, clinical presentation, pathophysiology, evaluation, and treatment of PH.
PubMed: 29720814
DOI: 10.4103/aian.AIAN_317_17 -
Neurology India 2021Paroxysmal Hemicrania (PH) is classified under trigeminal autonomic cephalalgia (TAC) as per the International Classification of Headache Disorders (ICHD). Since the... (Review)
Review
Paroxysmal Hemicrania (PH) is classified under trigeminal autonomic cephalalgia (TAC) as per the International Classification of Headache Disorders (ICHD). Since the first description by 0ttar Sjaastad and Inge Dale in 1974, PH has been reported by many authors. A greater understanding of PH phenotype and pathophysiology has resulted in the evolution of its diagnostic criteria, and management. We tabulated major case series of PH to describe the epidemiology, clinical features and recent updates of PH. PH is a rare headache characterized by daily, multiple paroxysms of unilateral, short-lasting (mean duration <20 minutes), side-locked headache in the distribution of ophthalmic division of trigeminal nerve with associated profound cranial autonomic symptoms. Recent ICHD classification added "restlessness" to the criteria for PH. Pain should completely respond to indomethacin to fulfil the diagnostic criteria of PH. PH should be differentiated from cluster headache, SUNCT/SUNA, and other short-lasting side-locked headaches. Trigeminal afferents possibly produce pain in PH and trigeminal-autonomic reflex explains the occurrence of autonomic features. Recently, a "permissive" central role of the hypothalamus is unveiled based on functional imaging studies. Other Cox-2 inhibitors, topiramate, calcium-channel blockers, epicranial nerve blocks have been shown to improve headache in some patients of PH who cannot tolerate indomethacin. Hypothalamic deep brain stimulation has been used in treatment-refractory cases.
Topics: Cluster Headache; Headache; Headache Disorders; Humans; Paroxysmal Hemicrania; Trigeminal Autonomic Cephalalgias
PubMed: 34003159
DOI: 10.4103/0028-3886.315982 -
Cephalalgia : An International Journal... Nov 2023Paroxysmal hemicrania and hemicrania continua are indometacin-sensitive trigeminal autonomic cephalalgias, a terminology which reflects the predominant distribution of... (Review)
Review
Paroxysmal hemicrania and hemicrania continua are indometacin-sensitive trigeminal autonomic cephalalgias, a terminology which reflects the predominant distribution of the pain, observable cranial autonomic features and shared pathophysiology. Understanding the latter is limited, both by low prevalence and the intricacies of studying brain function, requiring multimodal techniques to glean insights into such disorders. Similarly obscure is the curious response to indometacin. This review will address what is currently known about pathophysiology, the rationale for the current classification and, features which may confound the diagnosis, such as lack of cranial autonomic symptoms and those which are typically associated with migraine such as nausea, photophobia, phonophobia and aura. Despite these characteristics, a dramatic response to indometacin, which is not seen in migraine nor the other trigeminal autonomic cephalalgias , provides the hallmark of the diagnosis. The main clinical differential for paroxysmal hemicrania is based on temporal pattern and lies between cluster headache and short-lasting-neuralgiform headache attacks with tearing or additional cranial autonomic symptoms. For hemicrania continua it is more challenging as the main differential for which the disorder is often treated is migraine. A prior episodic pattern, often days at a time, and the tendency to exacerbation with analgesics will further deflect from the diagnosis. The relevance of this is that there is little overlap in therapeutics between paroxysmal hemicrania and hemicrania continua and other headache disorders and there are limited effective alternatives to indometacin. The most effective are other non-steroidal anti-inflammatory drugs including the newer COX-II inhibitors. Even though early reports suggest that a higher indometacin dose-requirement may herald a secondary precipitating pathology, this does not seem to be the case, with syndrome and response to treatment being similar with the primary disorder. In this context imaging of new onset paroxysmal hemicrania or hemicrania continua and implication of the results will be discussed as will alternative treatment options.
Topics: Humans; Paroxysmal Hemicrania; Trigeminal Autonomic Cephalalgias; Headache; Migraine Disorders; Vascular Headaches; Indomethacin
PubMed: 37950675
DOI: 10.1177/03331024231214239 -
Headache Sep 2022We aimed to report the accessible demographic, clinical, and radiological characteristics of reported pediatric paroxysmal hemicrania (PH). (Review)
Review
OBJECTIVE
We aimed to report the accessible demographic, clinical, and radiological characteristics of reported pediatric paroxysmal hemicrania (PH).
INTRODUCTION
It has been a while since PH in a child was first described. However, it is still unknown whether children's PH follows the same patterns as adults.
METHODS
This study followed the latest version of PRISMA (Preferred Reporting Items for Systematic Reviews and Meta-Analyses). PubMed, Web of Science, and Scopus were searched systematically without time limitation. We included all English-language, peer-reviewed articles, including observational or interventional studies reporting PH cases in children or adolescents based on the International Classification of Headache Disorders (ICHD) criteria. Data extracted included PH class; sex; age; age of onset; frequency, duration, site, severity, and quality of pains; triggers; and autonomic and migrainous symptoms, as well as a sense of restlessness/agitation, response to treatment, laboratory investigations, imaging, comorbidity, and family history. For quality assessment, two independent reviewers (MB and VM) assessed the methodological quality of the included studies through the Joanna Briggs Institute's critical appraisal checklist.
RESULTS
A total of 182 records were identified and reduced to 116 after removing duplicates. After screening, 22 articles met the inclusion criteria. Overall, the studies represented 35 children or adolescents with PH. We found a boy-to-girl ratio of 1.125:1. Onset occurred at a broad range of 1 to 14 years old. The mean age of onset among reported cases in children and adolescents was 6.5 years, while the mean age of diagnosis was 8.2 years. [Correction added on 22 August 2022, after first online publication: In the preceding sentence, 6.3 and 7.9 years were changed to 6.5 and 8.2 years, respectively.] The attacks' frequency and duration were greatly varied. Left-sided pain occurred twice as often as right-sided pain. The characteristics of the pain were usually severe in intensity. In nearly all of the cases, it was accompanied by ipsilateral cranial autonomic features. While most attacks were spontaneous, there were some common triggers. The physical examination, electroencephalogram, and brain magnetic resonance imaging had normal findings. Almost all patients benefited from indomethacin and showed complete responses to treatment, while some needed combination treatment of indomethacin with other medications.
CONCLUSION
Although pediatric-onset PH has similar features to adult-onset PH, there are some challenges with ICHD criteria for younger children that limit the ability to confidently assign a diagnosis. Moreover, owing to concomitant migrainous features, PH may be confused with migraine in children and adolescents.
Topics: Adolescent; Adult; Child; Child, Preschool; Female; Headache Disorders; Humans; Indomethacin; Infant; Male; Migraine Disorders; Pain; Paroxysmal Hemicrania
PubMed: 35833494
DOI: 10.1111/head.14354 -
Current Pain and Headache Reports Apr 2014Paroxysmal hemicrania (PH) is an underreported and underdiagnosed primary headache disorder. It usually begins in the third or fourth decade of life. The recent... (Review)
Review
Paroxysmal hemicrania (PH) is an underreported and underdiagnosed primary headache disorder. It usually begins in the third or fourth decade of life. The recent observations indicate that it is equally prevalent in both males and females. PH is characterized by severe, strictly unilateral head pain attacks that occur in association with ipsilateral autonomic features. The attacks in PH are shorter and more frequent compared with cluster headache (CH) but otherwise PH and CH have similar clinical features. The hallmark of PH is the absolute cessation of the headache with indomethacin. However, a range of drugs may show partial to complete relief in certain groups of patients. Neuromodulatory procedures, such as greater occipital nerve blockade, blockade of sphenopalatine ganglion and neurostimulation of the posterior hypothalamus, are reserved for refractory PH.
Topics: Adult; Age of Onset; Anti-Inflammatory Agents, Non-Steroidal; Diagnosis, Differential; Electric Stimulation Therapy; Female; Humans; Hypothalamus; Indomethacin; Male; Middle Aged; Nerve Block; Occipital Lobe; Paroxysmal Hemicrania; Sphenopalatine Ganglion Block
PubMed: 24523000
DOI: 10.1007/s11916-014-0407-6 -
Schmerz (Berlin, Germany) Dec 2011Paroxysmal hemicrania is experienced as headache attacks with pain and accompanying symptoms similar to those of cluster headaches. Attacks are, however of shorter... (Review)
Review
Paroxysmal hemicrania is experienced as headache attacks with pain and accompanying symptoms similar to those of cluster headaches. Attacks are, however of shorter duration, occur more frequently, affect predominantly women and respond reliably to indomethacin. Paroxysmal hemicrania can also occur secondary to an identifiable cause. To exclude symptomatic, paroxysmal hemicrania, especially with an atypical clinical picture and poor response to indomethacin, a careful diagnostic approach is necessary. The SUNCT syndrome (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is characterized by one-sided pain attacks of short duration, much shorter than other trigeminal autonomic cephalgias. Classically, the pain is accompanied by ipsilateral lacrimation and conjunctival injection. Some patients have been described with both cluster headache and trigeminal neuralgia. These patients should receive both diagnoses. It is important to differentiate these headache entities as specific therapy is needed for each to achieve optimal pain relief.
Topics: Adult; Aged; Anesthetics, Local; Anti-Inflammatory Agents, Non-Steroidal; Anticonvulsants; Child; Cluster Headache; Diagnosis, Differential; Humans; Indomethacin; Lidocaine; Neurologic Examination; Pain Measurement; Paroxysmal Hemicrania; SUNCT Syndrome; Secondary Prevention; Trigeminal Neuralgia
PubMed: 22120922
DOI: 10.1007/s00482-011-1108-2 -
Neurology Jul 1993
Topics: Humans; Indomethacin; Migraine Disorders
PubMed: 8327159
DOI: 10.1212/wnl.43.7.1445-a -
Neurological Sciences : Official... Nov 2019Hemicrania continua and paroxysmal hemicrania are considered different headaches belonging to a group of trigeminal autonomic cephalalgias. However, they share many... (Comparative Study)
Comparative Study
OBJECTIVE
Hemicrania continua and paroxysmal hemicrania are considered different headaches belonging to a group of trigeminal autonomic cephalalgias. However, they share many clinical features. Both headaches also show complete response to indomethacin, which is a mandatory criterion for their diagnosis. Are they really different headaches? To answer this question, we compared the pain characteristics and autonomic features between two headaches. We also determined whether paroxysmal hemicrania transforms into hemicrania continua or vice versa in their natural history.
METHODS
The patients with hemicrania continua and paroxysmal hemicrania were compared for severity, location, character, and mean effective indomethacin dose. The number of autonomic features and their severity was also compared. The natural history of headache was looked into to see the evolution of hemicrania continua and paroxysmal hemicrania from episodic and chronic pains, respectively.
RESULTS
We included 35 patients with hemicrania continua and 27 patients with paroxysmal hemicrania from July 2015 to March 2017. The mean age of patients with paroxysmal hemicrania was 34.42 years, and hemicrania continua was 37 years. Both groups were similar for majority of pain characteristics and number/severity of autonomic features. However, paroxysmal hemicrania had higher pain severity. Five patients transformed from paroxysmal hemicrania to hemicrania continua, and 3 patients transformed from hemicrania continua to paroxysmal hemicrania.
CONCLUSION
Paroxysmal hemicrania and hemicrania continua were similar on majority of pain characteristics and autonomic features. The paroxysmal hemicrania and hemicrania continua are not exclusive headaches and can transform into each other.
Topics: Adult; Disease Progression; Female; Humans; Male; Paroxysmal Hemicrania; Retrospective Studies; Severity of Illness Index; Trigeminal Autonomic Cephalalgias
PubMed: 31256266
DOI: 10.1007/s10072-019-03980-7