Neurophysiological investigations in a case of primary paroxysmal hemicrania-tic syndrome.

BACKGROUND: The association between paroxysmal hemicrania (PH) and trigeminal neuralgia-the so-called PH-tic syndrome-has rarely been described. However, a correct diagnosis is crucial since both disorders require specific treatments. Little is known about pathophysiological mechanisms, and, to date, there are no electrophysiological studies in patients with PH-tic syndrome. CASE: We describe the case of a 52-year-old man with a PH-tic syndrome successfully treated with an association of carbamazepine (1200 mg/day) and indomethacin (150 mg/die). Patient underwent trigeminal reflex testing, including blink and masseter inhibitory reflex, and laser-evoked potential (LEP) recording after supraorbital region stimulation in the affected and unaffected side. Both neurophysiological investigations resulted normal; LEPs failed to detect any latency asymmetry between both sides. CONCLUSIONS: Neurophysiological findings demonstrate for the first time the integrity of somatosensory system in a primary PH-tic syndrome case. Central pathophysiological mechanisms and hypothalamic dysregulation may contribute to the development of this rare syndrome.

Indomethacin-responsive trigeminal autonomic cephalgias: a review of key characteristics and pathophysiology.

Trigeminal autonomic cephalgias (TACs) are a well-defined subset of uncommon primary headaches that share comparable onset, pathophysiology and symptom patterns. TACs are characterised by the presentation of one-sided and high-intensity trigeminal pain together with unilateral cranial autonomic signs, which can include lacrimation, rhinorrhea, and miosis. The International Classification of Headache Disorders 3rd Edition recognises four different headache entities in this group, with cluster headache as the most recognised among them. Hemicrania continua (HC) and paroxysmal hemicrania (PH) are both distinctive cephalgias of which the diagnostic criteria include an absolute response to indomethacin. Consequently, for this reason they are often referred to as 'indomethacin-responsive' TACs. The main focus of this review was to discuss the state of knowledge regarding the pathophysiology and key characteristics of PH and HC. Given the limited understanding of these conditions, and their exceptionally uncommon prevalence, a correct diagnosis can pose a clinical challenge and the search for an effective treatment may be prolonged, which frequently has a serious impact upon patients' quality of life. The information provided in this review is meant to help physicians to differentiate indomethacin-sensitive cephalgias from other distinct headache disorders with a relatively similar clinical presentation, such as cluster headache, trigeminal neuralgia, and various migraine conditions.

Case Report: Shortest Course of Pediatric Paroxysmal Hemicrania.

Paroxysmal hemicrania (PH) is a rare primary headache disorder, especially among children. We describe herein a case with the shortest course of pediatric PH among previously reported cases, and the first case report of Japanese pediatric PH. An 11-year-old boy was referred to our clinic by his primary care physician for a headache evaluation. He had been complaining of severe, sharp, pulsating headache for 5 days. Attacks were restricted to the left side with a duration ranging from 2 to 20 minutes, 20-30 times a day. Attacks were associated with left autonomic symptoms (conjunctival injection, lacrimation, nasal congestion, eyelid edema, and ptosis). Two days after we prescribed indomethacin at 0.9 mg/kg/day, the patient was headache free. He stopped taking indomethacin 14 days after consultation because of drug eruptions. As of the time of writing, more than 1 year later, he has experienced no recurrence of headache. This case indicates the importance of improving awareness among general doctors regarding PH in children, and of conducting further investigations about low-dose, short-term indomethacin treatment.

Do paroxysmal hemicrania and hemicrania continua represent different headaches? A retrospective 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.

"Cough Hemicrania" - An Overlapping Form of Headache: Case Reports.

OBJECTIVE: To describe two patients with recurring unilateral brief headaches that fulfilled criteria for both primary cough headache (CH) and chronic paroxysmal hemicrania (CPH). BACKGROUND: CH is typically a bilateral headache, specifically triggered by cough, straining, or other Valsalva maneuvers. The report of cases sharing features with other primary headache disorders, such as CPH, suggest common pathogenic mechanisms. METHODS: Case reports. RESULTS: Two patients (one man), aged 55 and 64, had a two-year history of daily, unilateral, side-locked headache attacks, lasting about 15 minutes, and associated with ipsilateral tearing. Headaches were triggered by cough, sneezing, laughing, or bending forward. Both patients experienced a marked and sustained improvement with indomethacin, and both relapsed when it was interrupted. CONCLUSIONS: These cases suggest the existence of transitional phenotypes, or shared pathogenic mechanisms, between CH and CPH, two indomethacin-responsive headaches. A more comprehensive analysis of different CH subtypes is necessary to understand their relation with other primary headaches.

LASH: A Review of the Current Literature.

PURPOSE OF REVIEW: The purpose of this review is to evaluate and explain our current understanding of a very rare disorder, long-lasting autonomic symptoms with associated hemicranias (LASH). RECENT FINDINGS: At present, there are four known cases in the literature of LASH. Its characteristics and reported response to indomethacin link it most closely to the trigeminal autonomic cephalalgias (TACs). Its pathophysiology and epidemiology remain unclear. Variance in the pain and autonomic symptom relationship in the existing TAC literature along with the reports of TAC sine headache suggests that LASH may represent a far end of the spectrum of TACs, with most similarities to paroxysmal hemicrania (PH) and hemicrania continua (HC).

Do trigeminal autonomic cephalalgias represent primary diagnoses or points on a continuum?

The question of whether the trigeminal autonomic cephalalgias (TACs) represent primary diagnoses or points on a continuum has been debatable for a number of years. Patients with TACs may present with similar clinical characteristics, and occasionally, TACS respond to similar treatments. Prima facie, these disorders may seem to be intimately related. However, due to the current evidence, it would be challenging to accurately conclude whether they represent different primary headache diagnoses or the same primary headache disorder represented by different points on the same continuum. Ultimately, the TACs may utilize similar pathways and activate nociceptive responses that result in similar clinical phenotypes but "original and initiating" etiology may differ, and these disorders may not be points on the same continuum. This paper seeks to provide a brief comparison of TACs via diagnostic criteria, secondary causes, brief overview of pathophysiology, and the use of some key treatments and their mechanism of actions to illustrate the TAC similarities and differences.

When indomethacin fails: additional treatment options for "indomethacin responsive headaches".

Indomethacin has been used for the treatment of headache disorders since the 1960's, shortly after it was introduced as a treatment for pain and joint swelling in rheumatologic conditions. A subgroup of primary headache disorders, often refractory to other pharmacologic treatment such as triptans and the usual non-steroidal anti-inflammatories, was noted to be exquisitely and absolutely responsive to the analgesic effects of indomethacin. These disorders have been better characterized over the past decade and classified into primary headache disorders of paroxysmal hemicrania (PH) and hemicrania continua (HC). Since the current ICHD-3 beta requires response to indomethacin as a diagnostic criterion, studies on alternative treatments in HC and PH generally occur in patients with intolerance to its gastro-intestinal side effects rather than loss of analgesia effectiveness. More rarely, the development of new headaches have been reported in chronic indomethacin use. In these settings, other classes of medications such as selective cyclooxygenase-2 inhibitors (celecoxib), anti-epileptic agents (topiramate), calcium channel blockers (verapamil, flunarizine), melatonin, and local nerve blocks with anesthetic and steroids have been shown to be effective in case reports and series. We review the literature and provide our clinical recommendations on alternative therapies for the "indomethacin-responsive headaches".

Paroxysmal hemicrania: an update.

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.

[Update on trigeminal autonomic cephalalgia]. / Neues zu trigeminoautonomen Kopfschmerzen.

Cluster headache, paroxysmal hemicrania and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT syndrome) are classified under trigeminal autonomic cephalalgia (TAC). The proposed revision of the international classification of headache disorders (ICDH-3 beta) adds hemicrania continua to this diagnostic group. Moreover, diagnostic criteria of the other TACs were modified and are characterized by persistent headache or headache attacks accompanied by cranial autonomic symptoms. The main difference between the various TACs is the duration of attacks. Differentiation is important because different pharmacological strategies are necessary.

Chronic paroxysmal hemicrania in paediatric age: report of two cases.

Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with hemicrania continua and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) as trigeminal autonomic cephalalgia (TACs). CPH is characterised by short-lasting (2-30 min), severe and multiple (more than 5/day) pain attacks. Headache is unilateral, and fronto-orbital-temporal pain is combined with cranial autonomic symptoms. According to the International Classification of Headache Disorders, 2nd edition, the attacks are absolutely responsive to indomethacin. CPH has been only rarely and incompletely described in the developmental age. Here, we describe two cases concerning a 7-year-old boy and a 11-year-old boy with short-lasting, recurrent headache combined with cranial autonomic features. Pain was described as excruciating, and was non-responsive to most traditional analgesic drugs. The clinical features of our children's headache and the positive response to indomethacin led us to propose the diagnosis of CPH. Therefore, our children can be included amongst the very few cases of this trigeminal autonomic cephalgia described in the paediatric age.

Trigeminal autonomic cephalalgias: paroxysmal hemicrania, SUNCT/SUNA, and hemicrania continua.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders that include cluster headache (CH), paroxysmal hemicrania (PH), and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/cranial autonomic features (SUNCT/SUNA). Hemicrania continua (HC) is often included with this group, although the second edition of The International Classification of Headache Disorders did not link the entities. Trigeminal autonomic cephalalgias are generally characterized by relatively short-lasting attacks of severe pain and lateralized associated features including the pain, cranial autonomic symptoms, and where present, migrainous symptoms, such as photophobia. Paroxysmal hemicrania has intermediate duration and intermediate attack frequency. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing has the shortest attack duration and the highest attack frequency. Hemicrania continua has a continuous pain with exacerbations that can include cranial autonomic symptoms as part of the phenotype. The syndromes share much in their pathophysiology and investigation paths; however, their treatment is distinct, so that the accurate differentiation is important for optimal management.

Hemicrania continua: a second case in which the remitting form evolved from the chronic form.

We report the case of a woman with a 15-year history of chronic hemicrania continua, which over time evolved to remitting hemicrania continua. This is the second reported case in the literature documenting this transition.

Paroxysmal head pain with backward radiation: will epicrania fugax go in the opposite direction?

Epicrania fugax (EF) has been recently described as a paroxysmal head pain starting in a focal cranial area of the posterior scalp and rapidly spreading forward to the ipsilateral eye or nose along a linear or zigzag trajectory. Here we report two patients presenting with the same clinical features, except for the starting site and the direction of the pain. Unilateral pain paroxysms occurred on either side of the head, with a quick backward radiation along a linear trajectory. The pain always stemmed from a particular point located at the fronto-parietal region, and reached the parieto-occipital region in several seconds. The symptoms did not fit any of the acknowledged headaches and neuralgias, and might correspond to a reverse variant of EF.

[Episodic paroxysmal hemicrania with seasonal waxing and waning pattern]. / Mevsimsel artma ve azalma gösteren episodik paroksismal hemikrania.

The chronic form of paroxysmal hemicrania was defined first. Although the episodic form was thought to be inevitably chronic, in time, episodic forms that never reach the chronic phase were identified. The supposed incidence of paroxysmal hemicrania is 1/50.000, and the ratio of the episodic to chronic form is 1:4. A seasonal type of episodic form, which is limited to three cases in the literature, has also been reported. In this article, a case who remained episodic for 40 years with seasonal waxing and waning attack variations is reported.

Headache Attack Similar to Paroxismal Hemicrania Seen During Flight.

INTRODUCTION: Airplane headache (AH) is unique to plane travel and looks like a short duration migraine attack or paroxysmal hemicrania (PH) attack without any autonomic symptoms. Until now, there has been no documented association between AH and PH.CASE REPORT: We report a 50-yr-old healthy woman with a very severe and sudden pulsating headache located in the left frontal region with radiation into the left eye during takeoff which diminished within 10-15 min during her airplane journeys.DISCUSSION: The patient was diagnosed with AH and she had good response to indomethacin. The pain was unique to plane travel but looked like PH. We discuss the association between AH and PH attack in the light of diagnostic criteria and therapeutic approach in the report.Koçer A. Headache attack similar to paroxysmal hemicrania seen during flight. Aerosp Med Hum Perform. 2020; 91(4):373-375.

Three-year follow-up of a girl with chronic paroxysmal hemicrania.

This is a follow-up report of a girl, 5 years 4 months old, with classic symptoms of chronic paroxysmal hemicrania from the age of 2 years 3 months who had a complete response to indomethacin therapy. The patient suffered from frequent episodes of severe unilateral headaches for 1 year and 10 months before the diagnosis of chronic paroxysmal hemicrania was established. Indomethacin treatment lasted for 2 years and 6 months. During the first year of treatment, several doses of indomethacin were missed, which was followed by immediate return of hemicrania episodes and then quick resolution of symptoms after administration of indomethacin. After 2 years and 6 months of treatment, the parents missed the treatment for 1 week and the episodes did not recur. The treatment was discontinued. The patient was free from pain and off the medication 1 year later.

Treatment of hemicrania continua by occipital nerve stimulation with a bion device: long-term follow-up of a crossover study.

BACKGROUND: Hemicrania continua (HC) is a primary headache that comprises persistent unilateral pain, is associated with cranial autonomic features, and is responsive to indometacin. Some patients are unable to tolerate this treatment or it is contraindicated; for these patients, the medical options for therapy are restricted. Occipital nerve stimulation (ONS) is an effective treatment for medically intractable primary headache, but only three cases of HC treated with ONS have been reported. Here, we report long-term safety and efficacy data for ONS in six patients with HC. ONS was provided by a unilateral neurostimulation device, known as a bion, which might be described as a second-generation ONS device. METHODS: Six patients aged 18 years or older who were diagnosed with HC had a suboccipital bion device implanted ipsilateral to their headache and received continuous unilateral ONS. A crossover study design was used: the bion was on for the first 3 months, off for the fourth month, and on again during long-term follow-up. Detailed prospective headache diaries were kept for 1 month before implantation and for 5 months afterwards. Long-term data were obtained from patients' estimates of their outcome. The outcome of this study was assessed by a comparison of headache pain severity before and after ONS. FINDINGS: At a median follow-up of 13.5 months (range 6-21 months), five of six patients reported sufficient benefit to recommend the device to other patients with HC. At long-term follow-up, four of six patients reported a substantial improvement (80-95%), one patient reported a 30% improvement, and one patient reported that his pain was worse by 20%. The onset of the benefit of ONS was delayed by days to weeks, and headaches did not recur for a similar period when the device was switched off. Adverse events were mild and associated with transient overstimulation. INTERPRETATION: ONS appears to be a safe and effective treatment for HC, particularly when indometacin is not tolerated or is contraindicated. The bion device was well tolerated, easily inserted without significant morbidity, and is one-twentieth of the volume of current devices. Such miniaturised devices are a potential new option for treatment of HC.

Functional and structural neuroimaging in trigeminal autonomic cephalalgias.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache disorders characterized by unilateral trigeminal distribution pain that occurs in association with ipsilateral cranial autonomic features. They include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Until recently, primary headache disorders, including the TACs, were widely considered to be caused by peripheral mechanisms such as vascular changes or neurogenic inflammation. Developments in neuroimaging are revolutionizing our understanding of the pathophysiology of primary headache syndromes. Functional imaging studies have demonstrated hypothalamic activation in all the TACs. Furthermore, neuroimaging studies using voxel-based morphometry and magnetic resonance spectroscopy techniques have demonstrated structural and biochemical alterations, respectively, in the hypothalamus of patients with cluster headache. These studies suggest that the hypothalamus plays a crucial role in the pathophysiology of TACs, thereby supporting the notion that these disorders are primarily due to central rather than peripheral mechanisms.