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1.  Chronic paroxysmal hemicrania in paediatric age: report of two cases 
The Journal of Headache and Pain  2011;12(2):263-267.
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.
doi:10.1007/s10194-011-0315-7
PMCID: PMC3072501  PMID: 21340658
Chronic paroxysmal hemicrania; Trigeminal autonomic cephalgias; Children; Indomethacin
2.  Chronic paroxysmal hemicrania in paediatric age: report of two cases 
The Journal of Headache and Pain  2011;12(2):263-267.
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.
doi:10.1007/s10194-011-0315-7
PMCID: PMC3072501  PMID: 21340658
Chronic paroxysmal hemicrania; Trigeminal autonomic cephalgias; Children; Indomethacin
3.  Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) secondary to epidermoid cyst in the right cerebellopontine angle successfully treated with surgery 
The Journal of Headache and Pain  2011;12-12(3):385-387.
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare headache syndrome classified among the trigeminal autonomic cephalalgias. It is usually idiopathic, although infrequent secondary forms have been described. Recently, the term short-lasting unilateral headache with cranial autonomic symptoms (SUNA) has been defined by the International Headache Society (ICHD-2) as similar to SUNCT with less prominent absent conjunctival injection and lacrimation. We report a patient with paroxysmal orbito-temporal pains, phenotypically suggesting SUNA, secondary to epidermoid cyst in the cerebellopontine angle which disappeared after tumor resection. Neuroimaging should be considered in all patients with SUNA, notably in those with atypical presentation as our patient who presented on examination trigeminal hypoesthesia and tinnitus. Realization of a brain MRI would rule out injuries that causes this type of syndrome.
doi:10.1007/s10194-011-0326-4
PMCID: PMC3094668  PMID: 21409598
SUNA; Epidermoid cyst; Cerebellopontine angle; Surgery; Treatment
4.  Short-lasting unilateral neuralgiform headache attacks with cranial autonomic symptoms (SUNA) secondary to epidermoid cyst in the right cerebellopontine angle successfully treated with surgery 
The Journal of Headache and Pain  2011;12(3):385-387.
Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome is a rare headache syndrome classified among the trigeminal autonomic cephalalgias. It is usually idiopathic, although infrequent secondary forms have been described. Recently, the term short-lasting unilateral headache with cranial autonomic symptoms (SUNA) has been defined by the International Headache Society (ICHD-2) as similar to SUNCT with less prominent absent conjunctival injection and lacrimation. We report a patient with paroxysmal orbito-temporal pains, phenotypically suggesting SUNA, secondary to epidermoid cyst in the cerebellopontine angle which disappeared after tumor resection. Neuroimaging should be considered in all patients with SUNA, notably in those with atypical presentation as our patient who presented on examination trigeminal hypoesthesia and tinnitus. Realization of a brain MRI would rule out injuries that causes this type of syndrome.
doi:10.1007/s10194-011-0326-4
PMCID: PMC3094668  PMID: 21409598
SUNA; Epidermoid cyst; Cerebellopontine angle; Surgery; Treatment
5.  Different forms of trigeminal autonomic cephalalgias in the same patient: description of a case 
The Journal of Headache and Pain  2010;11(3):281-284.
The trigeminal autonomic cephalalgias (TACs), including cluster headache, paroxysmal hemicrania and SUNCT, are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head. Hemicrania continua (HC) shares many clinical characteristics with TACs, including unilateral pain and ipsilateral autonomic features. Nevertheless, HC is separately classified in the revised International Classification of Headache Disorders (ICHD-II). Here, we describe the case of a 45-year-old man presenting an unusual concurrence of different forms of primary headaches associated with autonomic signs, including subsequently ipsilateral cluster headache, SUNCT and HC. This report supports the theory that common mechanisms could be involved in pathophysiology of different primary headache syndromes.
doi:10.1007/s10194-010-0210-7
PMCID: PMC3451915  PMID: 20376519
Hemicrania continua; Cluster headache; SUNCT; TACs
6.  Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases 
The Journal of Headache and Pain  2012;13(2):167-169.
Background
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT.
Case reports
We report three patients of SUNCT syndrome with preserved intervening refractory period of variable duration observed at different times.
Discussion
Trigeminal neuralgias with autonomic features, SUNA and SUNCT share a common pathophysiological mechanism and actually represent a continuum. It is well known that patient with trigeminal neuralgia may transform into SUNCT/SUNA. Similarly, being a continuum, the presence or the absence of refractory period and its duration may change in a patient with SUNCT/SUNA at different time points.
Conclusion
The presence of refractory period should not exclude the diagnosis of SUNCT in a patient with other clinical features suggestive of SUNCT.
Electronic supplementary material
The online version of this article (doi:10.1007/s10194-011-0412-7) contains supplementary material, which is available to authorized users.
doi:10.1007/s10194-011-0412-7
PMCID: PMC3274582  PMID: 22227716
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT); Refractory period; Trigeminal neuralgia with autonomic features; Short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA)
7.  Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) with preserved refractory period: report of three cases 
The Journal of Headache and Pain  2012;13(2):167-169.
Background
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA) are rare primary headache syndromes characterized by spontaneous or triggered attacks of unilateral, brief, multiple, orbitofrontal pain associated with ipsilateral autonomic features. SUNCT is considered as a subset of SUNA. In SUNA, there may be cranial autonomic symptoms other than conjunctival injection and lacrimation, or either of two is present. SUNCT/SUNA can be triggered immediately after or at the decrescendo phase of the ongoing attack without any intervening refractory period. Refractory period is usually present in trigeminal neuralgia. Absent refractory period is thought to reliably differentiate SUNCT/SUNA from trigeminal neuralgia and has been proposed for inclusion into the International Classification of Headache Disorders (ICHD) diagnostic criteria for SUNCT.
Case reports
We report three patients of SUNCT syndrome with preserved intervening refractory period of variable duration observed at different times.
Discussion
Trigeminal neuralgias with autonomic features, SUNA and SUNCT share a common pathophysiological mechanism and actually represent a continuum. It is well known that patient with trigeminal neuralgia may transform into SUNCT/SUNA. Similarly, being a continuum, the presence or the absence of refractory period and its duration may change in a patient with SUNCT/SUNA at different time points.
Conclusion
The presence of refractory period should not exclude the diagnosis of SUNCT in a patient with other clinical features suggestive of SUNCT.
Electronic supplementary material
The online version of this article (doi:10.1007/s10194-011-0412-7) contains supplementary material, which is available to authorized users.
doi:10.1007/s10194-011-0412-7
PMCID: PMC3274582  PMID: 22227716
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT); Refractory period; Trigeminal neuralgia with autonomic features; Short-lasting unilateral neuralgiform attacks with cranial autonomic features (SUNA)
8.  A case of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT). Discussion of clinical features and differential diagnosis 
The Journal of Headache and Pain  2005;6(6):469-470.
Chronic short–lasting headaches, in which trigeminal autonomic cephalalgias (TACs) are included, are relatively rare syndromes and not always well recognised. We present a case highly suggestive of short–lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) and we try to affirm essential points to distinguish the diagnosis from other TACs and chronic short–lasting headaches. We conclude that the qualifying points for differential diagnosis are number and duration of attacks in a day, presence of autonomic features and lack of indomethacin effect.
doi:10.1007/s10194-005-0261-3
PMCID: PMC3452302  PMID: 16388343
SUNCT; TACs
9.  Paroxysmal hemicrania responding to topiramate 
Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with cluster headache and short‐lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) as a trigeminal autonomic cephalalgia. CPH is exquisitely responsive to indomethacin so much so that the response is one of the current diagnostic criteria. The case of a patient with CPH, who had marked epigastric symptoms with indomethacin treatment and responded well to topiramate 150 mg daily, is reported. Cessation of topiramate caused return of episodes, and the response has persisted for 2 years. Topiramate may be a treatment option in CPH.
doi:10.1136/jnnp.2006.096651
PMCID: PMC2117807  PMID: 17172571
10.  Paroxysmal hemicrania responding to topiramate 
BMJ Case Reports  2009;2009:bcr06.2009.2007.
Chronic paroxysmal hemicrania (CPH) is a rare primary headache syndrome, which is classified along with cluster headache and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) as a trigeminal autonomic cephalalgia. CPH is exquisitely responsive to indomethacin, so much so that the response is one of the current diagnostic criteria. The case of a patient with CPH, who had marked epigastric symptoms with indomethacin treatment and responded well to topiramate 150 mg daily, is reported. Cessation of topiramate caused return of episodes, and the response has persisted for 2 years. Topiramate may be a treatment option in CPH.
doi:10.1136/bcr.06.2009.2007
PMCID: PMC3027924  PMID: 21734918
11.  Neuroimaging in cluster headache and other trigeminal autonomic cephalalgias 
The central nervous system mechanisms involved in trigeminal autonomic cephalalgias, a group of primary headaches characterized by strictly unilateral head pain that occurs in association with ipsilateral craniofacial autonomic features, are still not comprehensively understood. However, functional imaging methods have revolutionized our understanding of mechanisms involved in these primary headache syndromes. The present review provides a brief overview of the major modern functional neuroimaging techniques used to examine brain structure, biochemistry, metabolic state, and functional capacity. The available functional neuroimaging data in cluster headache and other TACs will thus be summarized. Although the precise brain structures responsible for these primary headache syndromes still remain to be determined, neuroimaging data suggest a major role for posterior hypothalamus activation in initiating and maintaining attacks. Furthermore, pathophysiological involvement of the pain neuromatrix and of the central descending opiatergic pain control system was observed. Given the rapid advances in functional and structural neuroimaging methodologies, it can be expected that these non-invasive techniques will continue to improve our understanding into the nature of the brain dysfunction in cluster headache and other trigeminal autonomic cephalalgias.
doi:10.1007/s10194-011-0403-8
PMCID: PMC3253152  PMID: 22116532
Trigeminal autonomic cephalalgias; Hypothalamus; Pain neuromatrix; Single photon emission computed tomography; Positron emission tomography; Magnetic resonance spectroscopy; Functional MRI; Voxel-based morphometry
12.  Neuroimaging in cluster headache and other trigeminal autonomic cephalalgias 
The central nervous system mechanisms involved in trigeminal autonomic cephalalgias, a group of primary headaches characterized by strictly unilateral head pain that occurs in association with ipsilateral craniofacial autonomic features, are still not comprehensively understood. However, functional imaging methods have revolutionized our understanding of mechanisms involved in these primary headache syndromes. The present review provides a brief overview of the major modern functional neuroimaging techniques used to examine brain structure, biochemistry, metabolic state, and functional capacity. The available functional neuroimaging data in cluster headache and other TACs will thus be summarized. Although the precise brain structures responsible for these primary headache syndromes still remain to be determined, neuroimaging data suggest a major role for posterior hypothalamus activation in initiating and maintaining attacks. Furthermore, pathophysiological involvement of the pain neuromatrix and of the central descending opiatergic pain control system was observed. Given the rapid advances in functional and structural neuroimaging methodologies, it can be expected that these non-invasive techniques will continue to improve our understanding into the nature of the brain dysfunction in cluster headache and other trigeminal autonomic cephalalgias.
doi:10.1007/s10194-011-0403-8
PMCID: PMC3253152  PMID: 22116532
Trigeminal autonomic cephalalgias; Hypothalamus; Pain neuromatrix; Single photon emission computed tomography; Positron emission tomography; Magnetic resonance spectroscopy; Functional MRI; Voxel-based morphometry
13.  An unusual form of TAC–TAC sine autonomic phenomena 
The Journal of Headache and Pain  2008;9(5):331-332.
The trigeminal autonomic cephalalgias (TACs) are characterized by the cardinal combination of short-lasting unilateral pain and autonomic phenomena affecting the head, including cluster headache, paroxysmal hemicrania and SUNCT. Infrequently reported have been cases with dissociation of these two cardinal features- usually with autonomic phenomena without pain, rather than the reverse. Herein is described the case of a 56-year-old man presenting with an unusual form of TAC resembling chronic paroxysmal hemicrania but with a lower frequency of occurrence, with temporal features not typical of previously described TACs, and consisting of severe unilateral head pain without any autonomic symptoms or signs, absolutely responsive to indomethacin
doi:10.1007/s10194-008-0060-8
PMCID: PMC3452201  PMID: 18685807
Trigeminal autonomic cephalalgia; Paroxysmal hemicrania; Dissociation; Trigeminal-autonomic reflex
14.  The differential diagnosis of chronic daily headaches: an algorithm-based approach 
The Journal of Headache and Pain  2007;8(5):263-272.
Chronic daily headaches (CDHs) refers to primary headaches that happen on at least 15 days per month, for 4 or more hours per day, for at least three consecutive months. The differential diagnosis of CDHs is challenging and should proceed in an orderly fashion. The approach begins with a search for “red flags” that suggest the possibility of a secondary headache. If secondary headaches that mimic CDHs are excluded, either on clinical grounds or through investigation, the next step is to classify the headaches based on the duration of attacks. If the attacks last less than 4 hours per day, a trigeminal autonomic cephalalgia (TAC) is likely. TACs include episodic and chronic cluster headache, episodic and chronic paroxysmal hemicrania, SUNCT, and hypnic headache. If the duration is ≥4 h, a CDH is likely and the differential diagnosis encompasses chronic migraine, chronic tension-type headache, new daily persistent headache and hemicrania continua. The clinical approach to diagnosing CDH is the scope of this review.
doi:10.1007/s10194-007-0418-3
PMCID: PMC2793374  PMID: 17955166
Chronic daily headache; Differential diagnosis; Strategy
15.  The differential diagnosis of chronic daily headaches: an algorithm-based approach 
The Journal of Headache and Pain  2007;8(5):263-272.
Chronic daily headaches (CDHs) refers to primary headaches that happen on at least 15 days per month, for 4 or more hours per day, for at least three consecutive months. The differential diagnosis of CDHs is challenging and should proceed in an orderly fashion. The approach begins with a search for “red flags” that suggest the possibility of a secondary headache. If secondary headaches that mimic CDHs are excluded, either on clinical grounds or through investigation, the next step is to classify the headaches based on the duration of attacks. If the attacks last less than 4 hours per day, a trigeminal autonomic cephalalgia (TAC) is likely. TACs include episodic and chronic cluster headache, episodic and chronic paroxysmal hemicrania, SUNCT, and hypnic headache. If the duration is ≥4 h, a CDH is likely and the differential diagnosis encompasses chronic migraine, chronic tension-type headache, new daily persistent headache and hemicrania continua. The clinical approach to diagnosing CDH is the scope of this review.
doi:10.1007/s10194-007-0418-3
PMCID: PMC2793374  PMID: 17955166
Chronic daily headache; Differential diagnosis; Strategy
16.  Paroxysmal hemicrania as the clinical presentation of giant cell arteritis 
Clinics and Practice  2011;1(4):e111.
Head pain is the most common complaint in patients with giant cell arteritis but the headache has no distinct diagnostic features. There have been no published reports of giant cell arteritis presenting as a trigeminal autonomic cephalalgia. We describe a patient who developed a new onset headache in her fifties, which fit the diagnostic criteria for paroxysmal hemicrania and was completely responsive to corticosteroids. Removal of the steroid therapy brought a reemergence of her headaches. Giant cell arteritis should be considered in the evaluation of secondary causes of paroxysmal hemicrania; in addition giant cell arteritis needs to be ruled out in patients who are over the age of 50 years with a new onset trigeminal autonomic cephalalgia.
doi:10.4081/cp.2011.e111
PMCID: PMC3981414  PMID: 24765352
giant cell arteritis; paroxysmal hemicrania; headache; indomethacin.
17.  Bilateral SUNCT-like headache in a patient with prolactinoma responsive to lamotrigine 
The Journal of Headache and Pain  2009;10(6):469-472.
Short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT) syndrome is a rare trigeminal autonomic cephalalgia. The cases of SUNCT with attacks that affected both sides simultaneously have only rarely been reported and some of them had underlying pathology. We have reported a case of bilateral SUNCT-like headache secondary to a prolactinoma and responsive to lamotrigine treatment.
doi:10.1007/s10194-009-0146-y
PMCID: PMC3476218  PMID: 19763771
SUNCT syndrome; Prolactinoma; Lamotrigine
18.  Diagnostic and therapeutic errors in trigeminal autonomic cephalalgias and hemicrania continua: a systematic review 
Trigeminal autonomic cephalalgias (TACs) and hemicrania continua (HC) are relatively rare but clinically rather well-defined primary headaches. Despite the existence of clear-cut diagnostic criteria (The International Classification of Headache Disorders, 2nd edition - ICHD-II) and several therapeutic guidelines, errors in workup and treatment of these conditions are frequent in clinical practice. We set out to review all available published data on mismanagement of TACs and HC patients in order to understand and avoid its causes. The search strategy identified 22 published studies. The most frequent errors described in the management of patients with TACs and HC are: referral to wrong type of specialist, diagnostic delay, misdiagnosis, and the use of treatments without overt indication. Migraine with and without aura, trigeminal neuralgia, sinus infection, dental pain and temporomandibular dysfunction are the disorders most frequently overdiagnosed. Even when the clinical picture is clear-cut, TACs and HC are frequently not recognized and/or mistaken for other disorders, not only by general physicians, dentists and ENT surgeons, but also by neurologists and headache specialists. This seems to be due to limited knowledge of the specific characteristics and variants of these disorders, and it results in the unnecessary prescription of ineffective and sometimes invasive treatments which may have negative consequences for patients. Greater knowledge of and education about these disorders, among both primary care physicians and headache specialists, might contribute to improving the quality of life of TACs and HC patients.
doi:10.1186/1129-2377-14-14
PMCID: PMC3620440  PMID: 23565739
Cluster headache; Paroxysmal hemicrania; SUNCT; Trigeminal autonomic cephalalgias; Hemicrania continua; Error; Pitfall; Misdiagnosis; Mismanagement
19.  The red ear syndrome 
Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed.
doi:10.1186/1129-2377-14-83
PMCID: PMC3850925  PMID: 24093332
Red ear syndrome; Migraine; Trigemino-autonomic reflex; Trigeminal autonomic cephalalgias; Parasympathetic system; Erythromelalgia
20.  Linear headache: a recurrent unilateral head pain circumscribed in a line-shaped area 
Background
A headache circumscribed in a line-shaped area but not confined to the territory of one particular nerve had ever been described in Epicrania Fugax (EF) of which the head pain is moving and ultrashort. In a 25-month period from Feb 2012 to Mar 2014, we encountered 12 patients with a paroxysmal motionless head pain restricted in a linear trajectory. The head pain trajectory was similar to that of EF, but its all other features obviously different from those of EF. We named this distinctive but undescribed type of headache linear headache (LH).
Methods
A detailed clinical feature of the headache was obtained in all cases to differentiate with EF, trigeminal autonomic cephalalgias (TACs) and cranial neuralgia. Similarities and differences in clinical features were compared between LH and migraine.
Results
The twelve LH patients (mean age 43.9 ± 12.2) complained of a recurrent, moderate to severe, distending (n = 9), pressure-like (n = 3) or pulsating (n = 3) pain within a strictly unilateral line-shaped area. The painful line is distributed from occipital or occipitocervical region to the ipsilateral eye (n = 5), forehead (n = 6) or parietal region (n = 1). The pain line has a trajecory similar to that of EF but no characteristics of moving. The headache duration would be ranged from five minutes to three days, but usually from half day to one day in most cases (n = 8). Six patients had the accompaniment of nausea with or without vomiting, and two patients had the accompaniment of ipsilateral dizziness. The attacks could be either spontaneous (n = 10) or triggered by noise, depression and resting after physical activity (n = 1), or by stress and staying up late (n = 1). The frequency of attacks was variable. The patients had well response to flunarizine, sodium valproate and amitriptyline but not to carbamazepine or oxcarbazepine. LH is different from EF, trigeminal autonomic cephalalgias (TACs) and cranial neuralgia, but it had couple of features similar to that of migraine.
Conclusions
The clinical picture of LH might be a subtype of migraine, or represent a novel syndrome.
doi:10.1186/1129-2377-15-45
PMCID: PMC4078094  PMID: 24966056
Linear headache; Epicrania fugax; Migraine; Cranial neuralgia; Trigeminal autonomic cephalalgias
21.  A case of early-onset and monophasic trigeminal autonomic cephalalgia: could it be a SUNCT? 
The Journal of Headache and Pain  2010;11(4):363-365.
A 2-year-old female came to the Neurological Emergency Room of “Giovanni XXIII” Hospital in Bari, 6 h after the onset of severe facial pain, which occurred soon after awakening. Stabbing pain affected the right frontal and periorbital area, with ipsilateral conjunctival injection, swelling of the eyelids and tearing. Except the duration, from 5 to 30 s., the attacks were stereotyped including the occurrence and features of autonomic signs. Based on the typical clinical findings and the normal magnetic resonance imaging (MRI), we diagnosed short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome (SUNCT). The spontaneous remission within a few hours made prophylactic therapy unnecessary. At the last follow-up, after 3 months, the patient was still symptom free. In our case, after an active period lasting 2 days the disease disappeared completely. However the typical features of the disease (unilateral pain, short duration and high frequency of the attacks, autonomic signs ipsilateral to pain, numbers of attacks) were all present. While the diagnostic criteria of the International Headache Society classification for SUNCT did not include the duration of disease, it is likely that the active period lasting 2 days could be an expression of the clinical variability of the disease.
doi:10.1007/s10194-010-0219-y
PMCID: PMC3476350  PMID: 20473543
22.  Paroxysmal hemicrania: a retrospective study of a consecutive series of 22 patients and a critical analysis of the diagnostic criteria 
Background
Paroxysmal hemicrania (PH) is a probably underreported primary headache disorder. It is characterized by repeated attacks of severe, strictly unilateral pain lasting 2 to 30 minutes localized to orbital, supraorbital, and temporal areas accompanied by ipsilateral autonomic features. The hallmark of PH is the absolute cessation of the headache with indomethacin. However, these all features may not be present in all cases and a few cases may remain unclassified according to the 2nd Edition of The International classification of Headache Disorders (ICHD-II) criteria for PH.
Methods
Twenty-two patients were included in this retrospective observation.
Results
We describe 17 patients, observed over six years, who fulfilled the ICHD-II criteria for PH. In parallel, we identified five more patients in whom one of the features of the diagnostic criteria for PH was missing. Two patients did not show any evidence of cranial autonomic feature during the attacks of headache. Another two patients did not fulfill the criteria for PH as the maximum attack frequency was less than five. One patient had an incomplete response to indomethacin.
Conclusion
A subset of patients may not have all the defined features of PH and there is a need for refinement of the existing diagnostic criteria.
doi:10.1186/1129-2377-14-26
PMCID: PMC3620407  PMID: 23566235
Chronic daily headache; Paroxysmal hemicrania; Hemicrania continua; Cluster headache
23.  SUNA and red ear syndrome: a new association and pathophysiological considerations 
Red ear syndrome (RES) is characterised by attacks of unilateral or bilateral burning ear pain associated with erythema. Primary and secondary forms have been described. Primary RES appears to have a frequent association with primary headaches especially migraine. Here, we describe the case of a woman with short-lasting unilateral neuralgiform attacks with cranial autonomic symptoms (SUNA) and recurrent episodes of ipsilateral red ear triggerable by cutaneous stimulation. Lamotrigine was beneficial for her SUNA but not for the RES. Both these disorders are extremely rare therefore their coexistence in the same individual may suggest similar pathophysiological mechanisms rather than a chance association.
doi:10.1186/1129-2377-14-32
PMCID: PMC3631130  PMID: 23565730
SUNA; SUNCT; Red ear syndrome; Trigeminal autonomic cephalalgias; Cranial autonomic symptoms
24.  Sphenopalatine ganglion stimulation for the treatment of cluster headache 
Cluster headache is a severe, debilitating disorder with pain that ranks among the most severe known to humans. Patients with cluster headaches have few therapeutic options and further, 10–20% develop drug-resistant attacks. The often brief duration of cluster attacks makes abortive therapy a challenge, and preventive medications are almost always provided to patients, but the side effects of these preventive medications can be significant. The sphenopalatine ganglion (SPG) is believed to play a role in headache pain and cranial autonomic symptoms associated with cluster headache, which is a result of activation of the trigeminal-autonomic reflex. For over 100 years, the SPG has been a clinical target to treat primary headache disorders using pharmacologic and nonpharmacologic methods. Radiofrequency lesioning and nerve-resection therapies, while initially beneficial, are irreversible procedures, and the use of neurostimulation provides one method of interfacing with the neural pathways without causing permanent damage to neural tissue. SPG neurostimulation is both reversible and adjustable, and has recently been tested in both proof-of-concept work and in a randomized, sham-controlled trial for the treatment of cluster headache.
A randomized, sham-controlled study of 32 patients was performed to evaluate further the use of SPG stimulation for the acute treatment of chronic cluster headache. Of the 32 patients, 28 completed the randomized experimental period. Overall, 68% of patients experienced an acute response, a frequency response, or both. In this study the majority of adverse events were related to the implantation procedure, which typically resolved or remained mild in nature at 3 months following the implant procedure. This and other studies highlight the promise of using SPG stimulation to treat the pain-associated cluster headache. SPG stimulation could be a safe and effective option for chronic cluster headache.
doi:10.1177/1756285613510961
PMCID: PMC3994920  PMID: 24790646
cluster headache; neurostimulation; sphenopalatine ganglion
25.  Trigemino-autonomic headache related to Gasperini syndrome 
The Journal of Headache and Pain  2010;11(6):535-538.
We report the association of ipsilateral trigemino-autonomic headache to a case of right-sided nuclear facial and abducens palsy (Gasperini syndrome), ipsilateral hypacusis and right hemiataxia, caused by the occlusion of the right anterior inferior cerebellar artery. Short-lasting attacks of mild to moderate ipsilateral fronto-periorbital head pain, accompanied by lacrimation and mild conjunctival injection during more severe attacks, were present from the onset of symptoms, with a gradual worsening over the next few months and remitting during naproxen therapy. Magnetic resonance imaging showed an infarct in the right cerebellar peduncle, extending toward the pontine tegmentum, also involving the ipsilateral spinal trigeminal nucleus and tract and the trigeminal entry zone. Gasperini syndrome may be accompanied by ipsilateral trigemino-autonomic head pain.
doi:10.1007/s10194-010-0251-y
PMCID: PMC3476227  PMID: 20803228
Gasperini syndrome; Trigemino-autonomic headache; Anterior inferior cerebellar artery; Infarction

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