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.

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.

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.

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.

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).

"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.

The Rare Painful Phenomena - Chronic Paroxysmal Hemicrania-tic Syndrome as a Clinically Isolated Syndrome of the Central Nervous System.

The association of paroxysmal hemicrania with trigeminal neuralgia (TN) has been described and called paroxysmal hemicrania-tic syndrome (PH-tic). We report the case of a patient diagnosed as having chronic PH-tic (CPH-tic) syndrome as a clinically isolated syndrome of the central nervous system (CNS) (CIS).A forty year old woman was admitted to our hospital suffering from right facial pain for the last 2 years. The attacks were paroxysmal, neuralgiform, consisting of throb-like sensations, which developed spontaneously or were triggered by different stimuli in right facial (maxilar and mandibular) areas. Parallel with those, she felt a throbbing orbital and frontal pain with homolateral autonomic symptoms such as conjunctival injection, lacrimation, and the feeling that the ear on the same side was full. This pain lasted most often between 15 and 20 minutes. Beyond hemifacial hypoesthesia in the region of right maxilar and mandibular nerve, the other neurological finding was normal. Magnetic resonance imaging (MRI) study showed a T2-weighted multiple hyperintense paraventricular lesion and hyperintense lesion in the right trigeminal main sensory nucleus and root inlet, all of them being hypointense on T1-weighted image. All of these lesions were hypointense in gadolinium-enhanced T1-weighted images. Neurophysiological studies of trigeminal nerve (somatosensory evoked potentials and blink reflex) correlated with MRI described lesions. The patient's pain bouts were improved immediately after treatment with indomethacin, and were completely relieved with lamotrigine for a longer period. According to the actual McDonald's criteria, clinical state was defined as CIS which was clinically presented by CPH-tic syndrome.Even though it is a clinical rarity and its etiology is usually idiopathic, CPH-tic syndrome can also be symptomatic. When dealing with symptomatic cases, like the one described here, when causal therapy is not possible due to the nature of the primary pathological process, a therapeutic approach, although symptomatic, can be fully effective in controlling this painful syndrome. The case report could be a contribution to the pathophysiological and clinical understanding of the association of CPH and TN.Key words: Paroxysmal hemicrania, trigeminal neuralgia, clinically isolated syndrome.

Putative pathophysiological mechanisms in recurrent hemicrania from aortic dissection: a case report.

BACKGROUND: Transient or permanent neurological symptoms occur in 17-40% of patients with aortic dissection. They can distract from or even mask the underlying life-threatening condition. CASE PRESENTATION: We present the case of a young Caucasian man who consulted for recurrent episodes of stereotyped right-sided sudden-onset severe headache. Upon questioning, he also reported a dull chest pain. Clinical examination and brain magnetic resonance imaging were unremarkable. The concomitant presence of chest pain made us consider aortic dissection. Contrast-enhanced cervico-thoraco-abdominal computerized tomography revealed type A aortic dissection. The patient underwent surgical replacement of the ascending aorta and reported no further episode of headache thereafter. Differential diagnosis of headache in this case includes paroxysmal hemicrania, cluster headache, migraine, trigeminal neuralgia and short lasting unilateral neuralgiform headache with conjunctival injection and tearing. Failure to match diagnostic criteria for any of these primary headache disorders and the resolution of pain episodes following surgery led us to postulate that these new-onset hemicrania episodes were symptomatic of aortic dissection. We hypothesize that aortic wall ischemia could have activated the trigeminovascular system and thereby caused hemicranial pain. Such an effect might be mediated by two different pathways that can be referred to as anatomical and humoral. The humoral hypothesis would posit that ischemia results in synthesis of pro-inflammatory mediators released from the aortic wall into the blood stream, such that they reach the central nervous system and directly stimulate specific receptors. The anatomical hypothesis would imply that pain signals generated by nociceptors in the aortic wall are transferred to the trigeminal ganglion via the cardiac plexus, the first cervical ganglion and the internal carotid nerve such that pain perception is referred to related cranio-cervical dermatomes. CONCLUSION: In cases of isolated headache that does not match key diagnostic criteria for a primary headache entity; a thorough review of systems should be performed to look for symptoms that may indicate symptomatic headache from potentially life-threatening conditions. Neurologists should consider aortic dissection in patients presenting with acute headache and chest pain. Further clinical or experimental studies are required to refute or validate the pathophysiological hypothesis discussed here.

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.

Trigeminal autonomic cephalalgias other than cluster headache.

Trigeminal autonomic cephalalgias are short-lasting primary headache disorders associated with autonomic symptoms. Paroxysmal hemicrania is a rare headache disorder similar to cluster headache. Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) are unusual headache syndromes typified by a high frequency of severe, brief, unilateral attacks that usually occur in the distribution of the trigeminal nerve. SUNCT is a subtype of SUNA in which both conjunctival injection and tearing are present. SUNA differs from SUNCT in that autonomic symptoms are less prominent.

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.

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.

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.

[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.

Resolution of paroxysmal hemicrania after resection of intracranial meningioma.

Paroxysmal hemicrania is a trigeminal autonomic cephalalgia first described in 1976, characterized by episodic attacks of excruciating unilateral periorbital and temporal stabbing, pulsatile, craw-like, or boring headaches lasting 2 - 30 minutes, accompanied by autonomic features, and alleviated by indomethacin. Paroxysmal hemicrania is divided into an episodic or chronic form, depending on the duration and frequency of the attacks. We describe a case of paroxysmal hemicrania in a patient with a contralateral anterior clinoid meningioma, which resolved after tumor resection. Most cases of autonomic cephalgias are primary headaches and not caused by underlying intracranial structural lesions. Based on our patient and a literature review of secondary causes of trigeminal autonomic cephalalgias, we recommend that all patients with trigeminal autonomic cephalalgias including paroxysmal hemicrania undergo neuroimaging studies. The preferred neuro-radiologic procedure should be a cranial MRI to exclude underlying structural intracranial lesions, particularly in the sellar and parasellar regions.

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.