scholarly journals A case of remitting hemicrania continua with seasonal variation and clustering: a diagnostic confusion with cluster headache

2019 ◽  
Vol 12 (5) ◽  
pp. e229650
Author(s):  
Sanjay Prakash ◽  
Kalu Singh Rawat
Keyword(s):  
Discrete Series ◽  
Primary Headache ◽  
Complete Response ◽  
Hemicrania Continua ◽  
Therapeutic Trial ◽  
Cranial Autonomic Symptoms ◽  
Unilateral Headache ◽  
Continuous Background ◽  
Diagnostic Confusion ◽  
History Of

Hemicrania continua (HC) is an indomethacin responsive primary headache that is characterised by a continuous strictly unilateral headache with periodic exacerbations. About 15% may have a remitting subtype of HC. Herein, we are reporting a 36-year-old man who had a 5-year history of episodic right-sided headaches. The headaches used to occur in a discrete series lasting 4–6 weeks, separated by pain-free remissions of 10–11 months. In each relapse, he had continuous background pain with superimposed exacerbations. The superimposed exacerbations were 1–2 attacks per day, lasting for 2–5 hours, and were associated with ipsilateral cranial autonomic symptoms. However, the patient did not respond to usual therapies of custer headache (CH). He had a complete response to indomethacin. We suggest that remitting subtype of HC may mimic CH. A therapeutic trial of indomethacin should be done in all strictly unilateral headaches who are not responding to other drugs.

Activation of the Brainstem but not of the Hypothalamus in Hemicrania Continua without Autonomic Symptoms

Cephalalgia ◽  
2009 ◽  
Vol 29 (9) ◽  
pp. 974-979 ◽  
Author(s):  
P Irimia ◽  
J Arbi ◽  
E Prieto ◽  
R Fernández-Torrón ◽  
E Martínez-Vila
Keyword(s):  
Primary Headache ◽  
Brain Structures ◽  
Complete Response ◽  
Hemicrania Continua ◽  
Single Observation ◽  
Autonomic Symptoms ◽  
Positron Emission ◽  
History Of ◽  
The Brain ◽  
No Activation

A 64-year-old woman presented with a 6-month history of right-sided continuous headache, without autonomic symptoms and complete response to indomethacin. Clinical examination and structural brain imaging were normal. A diagnosis of hemicrania continua (HC) was made. We sought to determine the brain structures active during the pain in a patient who met all of the diagnostic criteria for HC with the exception of autonomic symptoms. A brain positron emission tomography study was performed during pain, and completely pain-free after indomethacin administration. Comparing the pain with pain-free states, the region of the dorsal pons was significantly activated. There was no activation in the hypothalamus, as previously reported in HC with autonomic symptoms. Although definitive conclusions can not be drawn from a single observation, the lack of autonomic symptoms along with the absence of hypothalamic activation suggests that the clinical presentation may predict the pattern of brain activation in primary headache syndromes.

Hemicrania Continua Evolving from Episodic Paroxysmal Hemicrania

Cephalalgia ◽  
2006 ◽  
Vol 26 (9) ◽  
pp. 1143-1145 ◽  
Author(s):  
F Castellanos-Pinedo ◽  
M Zurdo ◽  
E Martínez-Acebes
Keyword(s):  
Family History ◽  
Migraine Without Aura ◽  
Clinical Course ◽  
Previous History ◽  
Primary Headache ◽  
Complete Response ◽  
Hemicrania Continua ◽  
Paroxysmal Hemicrania ◽  
History Of ◽  
Probable Migraine

A 45-year-old woman, who had been diagnosed in our unit with episodic paroxysmal hemicrania, was seen 2 years later for ipsilateral hemicrania continua in remitting form. Both types of headache had a complete response to indomethacin and did not occur simultaneously. The patient had a previous history of episodic moderate headaches that met criteria for probable migraine without aura and also had a family history of headache. The clinical course in this case suggests a pathogenic relationship between both types of primary headache.

scholarly journals Hemicrania Continua Associated with Classic Scintillating Scotoma

2018 ◽  
Vol 10 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Eva Auffenberg ◽  
Friedemann Bender ◽  
Tobias Freilinger
Keyword(s):  
Primary Headache ◽  
Headache Disorder ◽  
Complete Response ◽  
Hemicrania Continua ◽  
Response To Treatment ◽  
Visual Aura ◽  
Primary Headache Disorder ◽  
Cranial Autonomic Symptoms ◽  
Potential Link ◽  
Scintillating Scotoma

Hemicrania continua (HC) is a rare primary headache disorder, characterized by persistent unilateral pain associated with cranial autonomic symptoms and prompt response to indomethacin. While migrainous features (including aura) have been recognized in cluster headache, there have been only single reports of HC with aura. Here, we report the case of a 53-year-old man with constant right-sided headache and superimposed exacerbations to severe pain lasting for several hours. Secondary etiologies were excluded, and a diagnosis of HC was established after prompt and complete response to treatment with indomethacin. During an episode of pain exacerbation, for the first time the patient experienced an episode of transient visual disturbances compatible with scintillating scotoma. We propose a potential link between HC and visual aura, which parallels similar observations in other trigeminal autonomic cephalalgias and more specifically confirms previous observational data on aura in HC, thus highlighting potentially shared pathophysiological mechanisms.

Classification, diagnostic criteria, and epidemiology

2020 ◽  
pp. 177-181
Author(s):  
Thijs H. Dirkx ◽  
Peter J. Koehler
Keyword(s):  
Cluster Headache ◽  
Epidemiological Data ◽  
Hemicrania Continua ◽  
Paroxysmal Hemicrania ◽  
Primary Headaches ◽  
Conjunctival Injection ◽  
Headache Disorders ◽  
Cranial Autonomic Symptoms ◽  
Unilateral Headache

The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania, SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing), SUNA (with cranial autonomic symptoms), and hemicrania continua, belong to the primary headaches. They are characterized by severe unilateral headache attacks in association with ipsilateral cranial autonomic features. Cluster headache is the most frequent of the TACs. The other TACs are rare, but epidemiological data are scarce and variable. The various types of TAC are distinguished not only by differences in attack frequency and duration, but also by differences with respect to treatment response. The typical headache syndromes, fulfilling the International Classification of Headache Disorders-3 criteria, have also been described in association with other disorders and imaging is required to exclude intracranial pathology in all newly diagnosed patients.

scholarly journals Phenotypic and treatment outcome data on SUNCT and SUNA, including a randomised placebo-controlled trial

Cephalalgia ◽  
2017 ◽  
Vol 38 (9) ◽  
pp. 1554-1563 ◽  
Author(s):  
Hsing-Yu Weng ◽  
Anna S Cohen ◽  
Christoph Schankin ◽  
Peter J Goadsby
Keyword(s):  
Controlled Trial ◽  
Clinical Manifestations ◽  
Primary Headache ◽  
Outcome Data ◽  
Short Term ◽  
Autonomic Symptoms ◽  
Primary Headache Disorders ◽  
Cranial Autonomic Symptoms ◽  
History Of

Background 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 two rare headache syndromes classified broadly as Trigeminal Autonomic Cephalalgias (TACs). Methods Here, 65 SUNCT (37 males) and 37 SUNA (18 males) patients were studied to describe their clinical manifestations and responses to treatment. Results Pain was almost always unilateral and side-locked. There were three types of attack: Single stabs, stab groups, and a saw-tooth pattern, with some patients experiencing a mixture of two types. As to cranial autonomic symptoms, SUNA patients mainly had lacrimation (41%) and ptosis (40%). Most cases of the two syndromes had attack triggers, and the most common triggers were touching, chewing, or eating for SUNCT, and chewing/eating and touching for SUNA. More than half of each group had a personal or family history of migraine that resulted in more likely photophobia, phonophobia and persistent pain between attacks. For short-term prevention, both syndromes were highly responsive to intravenous lidocaine by infusion; for long-term prevention, lamotrigine and topiramate were effective for SUNCT, and lamotrigine and gabapentin were efficacious in preventing SUNA attacks. A randomized placebo-controlled cross-over trial of topiramate in SUNCT using an N-of-1 design demonstrated it to be an effective treatment in line with clinical experience. Conclusions SUNCT and SUNA are rare primary headache disorders that are distinct and very often tractable to medical therapy.

Facial presentations of migraine, TACs, and other paroxysmal facial pain syndromes

Neurology ◽  
2019 ◽  
Vol 93 (12) ◽  
pp. e1138-e1147 ◽  
Author(s):  
Christian Ziegeler ◽  
Arne May
Keyword(s):  
Facial Pain ◽  
Care Center ◽  
Tertiary Care ◽  
Primary Headache ◽  
Hemicrania Continua ◽  
Paroxysmal Hemicrania ◽  
Primary Headaches ◽  
Cranial Autonomic Symptoms ◽  
Pain Syndromes ◽  
The Face

ObjectiveTo assess the prevalence of facial pain (V2 and/or V3) presentations among nearly 3,000 patients with headache treated in a university tertiary care center.MethodsBetween 2010 and 2018, we routinely assessed the prevalence of facial pain presentations of all patients with primary headaches.ResultsOf 2,912 patient datasets, 291 patients reported facial pain either as an independent or as an additional symptom. Among patients with migraine, 2.3% (44 of 1,935) reported a facial involvement, most commonly in V2. Of these, 18 patients (40.9%) experienced the pain predominantly in the face. In patients with cluster headache, 14.8% (42 of 283) reported a facial involvement, of which 31.0% perceived the pain predominantly in the face. A facial involvement was seen in 45.0% of patients with paroxysmal hemicrania (9 of 20), 21.4% of patients with hemicrania continua (9 of 42), and 20.0% of patients with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing/short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (3 of 15). In addition, we present 6 patients who reported a constant side-locked facial pain with superseded well-defined facial pain attacks of 10- to 30-minute duration that appeared several times per day.ConclusionOur data suggest that a facial involvement in primary headaches is infrequent but not uncommon. A sole facial presentation of primary headache symptomatology seems to be exceptionally rare. We describe 3 different types of facial pain involvement and, in this context, distinguish patients with paroxysmal orofacial pain syndromes that have not been previously described. These patients may represent a new entity that could tentatively be called constant unilateral facial pain with added attacks.

scholarly journals Hemicrania continua: a report of ten new cases

2002 ◽  
Vol 60 (3B) ◽  
pp. 695-698 ◽  
Author(s):  
Marcelo E. Bigal ◽  
Stewart J Tepper ◽  
Fred D. Sheftell ◽  
Alan M. Rapoport
Keyword(s):  
Clinical Characteristics ◽  
Primary Headache ◽  
Hemicrania Continua ◽  
Partial Relief ◽  
Unilateral Headache ◽  
Tertiary Center

Hemicrania continua (HC) is an uncommon primary headache first described as a syndrome in 1984. Being quite unusual, its clinical characterization still demands better description. The aim of this study is to present the main clinical characteristics of 10 patients with the diagnosis of HC seen in a tertiary center, critically discussing their main features. All subjects had strictly unilateral headache without side shift and absolute response to indomethacin. Seven patients (70%) presented autonomic features during pain exacerbations. Four (40%) had migrainous symptoms during the exacerbations and one presented partial relief with dihydroergotamine. One patient had pain excruciatingly severe during the exacerbations. Although the cardinal features of HC - continuous, unilateral, indomethacin responsive, remain strongly reliable, a refinement on the clinical characterization is needful and desired.

Hemicrania Continua: Who Responds to Indomethacin?

Cephalalgia ◽  
2009 ◽  
Vol 29 (3) ◽  
pp. 300-307 ◽  
Author(s):  
MJ Marmura ◽  
SD Silberstein ◽  
M Gupta
Keyword(s):  
Medication Overuse ◽  
Primary Headache ◽  
Headache Disorder ◽  
Rapid Onset ◽  
Hemicrania Continua ◽  
Paroxysmal Hemicrania ◽  
Autonomic Symptoms ◽  
Primary Headache Disorder ◽  
Unilateral Headache ◽  
Headache Onset

Hemicrania continua (HC) is a primary headache disorder characterized by a continuous, moderate to severe, unilateral headache and defined by its absolute responsiveness to indomethacin. However, some patients with the clinical phenotype of HC do not respond to indomethacin. We reviewed the records of 192 patients with the putative diagnosis of HC and divided them into groups based on their headaches' response to indomethacin. They were compared for age, gender, presence or absence of specific autonomic symptoms, medication overuse, rapidity of headache onset, and whether or not the headaches met criteria for migraine when severe. Forty-three patients had an absolute response and 122 patients did not respond to adequate doses of indomethacin. The two groups did not differ significantly in terms of age, sex, presence of rapid-onset headache, or medication overuse. Autonomic symptoms, based on a questionnaire, did not predict response. Eighteen patients could not complete a trial of indomethacin due to adverse events. Nine patients could not be included in the HC group despite improvement with indomethacin: one patient probably had primary cough headache, another paroxysmal hemicrania; three patients improved but it was uncertain if they were absolutely pain free, and four patients dramatically improved but still had a baseline headache. We found no statistically significant differences between patients who did and did not respond to indomethacin. All patients with continuous, unilateral headache should receive an adequate trial of indomethacin. Most patients with unilateral headache suggestive of HC did not respond to indomethacin.

Rigorously defined hemicrania continua presenting bilaterally

Cephalalgia ◽  
2011 ◽  
Vol 31 (14) ◽  
pp. 1490-1492 ◽  
Author(s):  
Andrew M Southerland ◽  
Ivan S Login
Keyword(s):  
Phenotypic Variability ◽  
Case Series ◽  
Primary Headache ◽  
Tension Type Headache ◽  
Hemicrania Continua ◽  
Primary Headaches ◽  
Therapeutic Trial ◽  
Primary Headache Disorders ◽  
Low Incidence ◽  
Type Headache

Background: Hemicrania continua (HC) is a headache syndrome characterized by continuous, unilateral head pain, autonomic features, and a complete therapeutic response to indomethacin. Although HC is classified as a unique entity among primary headache disorders, it clearly shares features with other primary headaches, including trigeminal autonomic cephalalgias, and chronic daily headaches, such as chronic migraine and chronic tension-type headache. In addition, the diagnosis is often delayed secondary to a relatively low incidence and the occurrence of some phenotypic variability as found in previous case series. Case: A 62-year-old woman presented with 5 months of unremitting, bilateral headache with significant autonomic symptoms during exacerbations of pain. Neurological examination and imaging studies were normal. After failure to respond to numerous previous therapeutic medicines and interventions, she experienced complete resolution following administration of indomethacin and eventual remission on sustained treatment. Conclusion: This case demonstrates that hemicrania continua with requisite autonomic features can occur in a purely bilateral form. Although the definitive aspects of HC continue to evolve, a bilateral headache meeting the current criteria warrants a therapeutic trial of indomethacin.

Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case

Cephalalgia ◽  
2017 ◽  
Vol 38 (4) ◽  
pp. 804-807 ◽  
Author(s):  
Antonio Russo ◽  
Marcello Silvestro ◽  
Mario Cirillo ◽  
Alessandro Tessitore ◽  
Gioacchino Tedeschi
Keyword(s):  
Clinical Laboratory ◽  
Sinus Thrombosis ◽  
Cranial Nerves ◽  
Clinical Manifestations ◽  
Primary Headache ◽  
Hemicrania Continua ◽  
Inflammatory Disorder ◽  
Hypertrophic Pachymeningitis ◽  
Cranial Autonomic Symptoms ◽  
First Time

Background Hemicrania continua (HC) is a primary headache syndrome characterized by a unilateral, moderate, continuous headache with exacerbations marked by migrainous and cranial autonomic symptoms. However, clinical phenotypes similar to primary HC may be subtended by several disorders. Case report We report the case of a 62-year-old man experiencing, over the previous year, a headache completely consistent with HC and its absolute responsiveness to indomethacin therapy. Later, the patient developed diplopia caused by sixth cranial nerve palsy ipsilateral to headache. In this frame, clinical, laboratory and neuroimaging characteristics supported the diagnosis of idiopathic hypertrophic pachymeningitis (IHP). Conclusions IHP is a rare fibrosing inflammatory disorder leading to a localized or diffuse dura mater thickening. IHP clinical manifestations are a progressively worsening headache and signs related to cranial nerves involvement and venous sinus thrombosis. Here, we report, for the first time, a HC phenotype subtended by IHP.

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