Headache from clinically confirmed hemicrania continua arising from the sternocleidomastoid muscle: a case report.

BACKGROUND: A patient with a history of cluster headaches, now in remission, presented with confirmed hemicrania continua that resolved with a local anaesthetic injection into the Sternocleidomastoid (SCM) muscle. To the best of our knowledge, this is the first reported case of a trigeminal autonomic cephalalgia arising from a soft tissue source in the neck. CASE PRESENTATION: A 66-year-old man with a history of cluster headaches presented with a six-month history of a new constant right-sided headache. The new headaches were associated with tearing and redness of the right eye and responded to indomethacin, thus meeting the International Classification of Headache Disorders (ICHD-3) diagnostic criteria for hemicrania continua. The history and physical examination suggested a cervical source of the headache arising from the ipsilateral SCM muscle. Injection of the muscle with 1% lidocaine resulted in the elimination of the pain for 1 month without indomethacin. CONCLUSIONS: Due to the convergence of trigeminal, cervical and autonomic nerve fibres, various combinations of headache syndromes can result. This case report demonstrates how a meticulous examination is a crucial component of headache evaluation. Treatment directed to this muscle spared this patient further daily indomethacin and associated side effects.

LASH syndrome: New cases with a broadening clinical phenotype.

OBJECTIVE: To describe three new cases of the headache syndrome of long-lasting autonomic symptoms with hemicrania (LASH), and to establish a clinical phenotype utilizing all LASH cases noted in the medical literature. METHODS: A case series of patients was evaluated in an academic headache clinic over a two-year time period. LASH syndrome was defined by episodic headache attacks with associated cranial autonomic symptoms that start before headache onset, last the entire duration of the headache and continue on for a period of time after the headache ceases. RESULTS: Three patients were noted to have LASH syndrome in a two-year time period (2017-2018). One patient was diagnosed with primary LASH, while two others had probable secondary LASH from a secretory pituitary tumor. The primary LASH patient was female. She had on average one headache per week lasting 1-3 days in duration. She experienced migrainous associated symptoms along with their cranial autonomic symptoms. She also developed a fixed Horner's syndrome along with a typical headache attack, which was present for 6 months at the time of consultation. She had complete headache relief with indomethacin and her miosis and ptosis also resolved with treatment. Secondary LASH was diagnosed in two patients (one male, one female) both with prolactin secreting pituitary microadenomas. One of the patients had his headaches abolish with dopamine agonist therapy while the other patient did not respond to hormonal modulation but became pain free on indomethacin. Secondary LASH patients had less frequent headache episodes and lacked any migrainous associated features, but exhibited agitation with headache. CONCLUSION: LASH syndrome may be rare, but more reported cases are entering the headache literature. The temporal profile of onset and offset of cranial autonomic symptoms is key to making the diagnosis. Primary and secondary LASH may present differently based on gender predominance, the presence of migrainous associated features, and attack frequency. Secondary LASH appears to be indomethacin responsive, suggesting that medication effectiveness should not obviate the need to do testing for secondary etiologies.

Idiopathic hypertrophic pachymeningitis mimicking hemicrania continua: An unusual clinical case.

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.

The pathophysiology of the trigeminal autonomic cephalalgias, with clinical implications.

The hallmark of primary headaches belonging to the group known as the trigeminal autonomic cephalalgias is unilateral headache accompanied by cranial autonomic symptoms. Being relatively rare and poorly understood, they represent a clinical challenge, leading to underdiagnosis and undertreatment. While the headache is the most obvious and disabling symptom, it is only part of a complex symptomatology which hints at the involved pathophysiological mechanisms. Activation of the trigeminal-autonomic reflex results in the aforementioned cranial autonomic symptoms, which are well understood; however, it is obvious that this brainstem reflex is regulated by higher centers that seemingly play a pivotal role in the attacks and the wide range of other symptoms indicating a homeostatic disturbance. These symptoms, as well as a number of well-validated findings, implicate the hypothalamus in the pathophysiology. over the course of the past 2-3 decades, novel therapies and technological advances have helped increase our knowledge of these clinical syndromes, and will likely continue to do so in the coming years as we witness the arrival of new drugs and neurostimulation options. In this review, the clinical presentation for cluster headache, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing, and hemicrania continua is covered, along with our current understanding of the common pathophysiology and clinical manifestations.

Two Cases of Hemicrania Continua-Trigeminal Neuralgia Syndrome: Expanding the Spectrum of Trigeminal Autonomic Cephalalgia-Tic (TAC-TIC) Syndrome.

BACKGROUND: Trigeminal neuralgia (TN) has been described in association with various primary headache disorders. So far, no case of TN has been reported in association with hemicrania continua (HC). CASE REPORT: Here, we report two patients of hemicrania continua associated with TN (HC-tic syndrome). These patients had both headaches concurrently. Both patients responded to a combination of carbamazepine and indomethacin. The skipping or tapering of carbamazepine led to the recurrence of the neuralgic pain of TN. In the same way, the skipping of indomethacin resulted in the relapse of the pain, typical of HC. CONCLUSION: With these two cases of HC-tic syndrome, we suggest that TN has a special predilection for all types of TACs. Various speculations suggest that such associations are more than a simple coincidence, and both diseases may be causally interrelated. The identification of this association is important as both disorders may need separate drugs.

Which Matters More? A Retrospective Cohort Study of Headache Characteristics and Diagnosis Type in Soldiers with mTBI/Concussion.

OBJECTIVE: To describe the diagnostic types and characteristics of headaches in soldiers with mild traumatic brain injury during the wars in Afghanistan and Iraq. BACKGROUND: Persistent post-traumatic headache interferes with returns to activity or duty. The most commonly cited headache diagnosis after concussion is migraine. We hypothesize that headache diagnosis type, eg, migraine, is not sufficient to predict relationships with occupational outcomes after concussion. METHODS: The study sample consisted of all new patients referred for headache evaluation at the Brain Injury Center at Womack Army Medical Center over a 1-year time period. The design was retrospective and observational. Clinical data reported included demographics, causes of injury, headache characteristics, and headache diagnosis type. After reviewing records for retention or severance from military service, the primary occupational outcome measure was departure from service due to medical cause as determined by a Medical Evaluation Board (MEB). The primary outcome measure was to test the strength of association between leaving service for MEB and headache characteristics or diagnosis. RESULTS: A total of 95 patients (94% male) with concussion described 166 distinct headache types, the most common being migraine (60%) and trigeminal autonomic cephalalgia (24%). A total of 25% of all patients remained on active duty. A continuous headache of any type was present in 75% of patients and of these, 23% remained on active duty. Of the 51% of patients who had both a continuous and non-continuous headache, 17% remained on active duty (P < .001). Therefore, we report that a continuous headache, regardless of diagnosis type was associated with negative occupational outcomes. Regardless of headache duration, headache diagnosis type alone was not associated with soldiers' separations from service. CONCLUSIONS: Persistent post-traumatic headache is most likely to present with continuous pain. Migraine is the most common primary diagnosis type. The presence of a continuous headache was strongly associated with negative occupational outcomes. Primary headache diagnosis type was not. Headache characteristics, therefore, may be more important than diagnosis type when determining active duty status. Further prospective research is indicated.

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).

Trigeminal autonomic cephalgia caused by recurrent posterior scleritis.

A 40-year-old woman presented with a side-locked headache with autonomic features, which then switched sides before reverting to the original side. The atypical features of side swapping, partial response to indometacin and abnormal optic disc appearances ultimately led to a diagnosis of recurrent posterior scleritis. We discuss the differential diagnosis of trigeminal autonomic cephalgias and its secondary causes, and provide practical pointers for its investigation and management.

Trigeminal autonomic headaches caused by a multinodular goiter--a case report.

BACKGROUND: Though thyroid growths are considered to be a frequent cause of Horner's syndrome, concurrent headache attacks are not commonly seen. CASE: A 63-year-old woman presented with severe, daily occurring, unilateral headache attacks with ipsilateral Horner's syndrome. Magnetic resonance imaging arteriography showed a multinodular goiter displacing the left common carotid artery. CONCLUSION: This case exemplifies the combination of headache attacks and Horner's syndrome due to mechanical pressure of an enlarged thyroid, mimicking the symptoms both of carotid dissection as well as trigeminal autonomic cephalgias like paroxysmal hemicrania.

A translational in vivo model of trigeminal autonomic cephalalgias: therapeutic characterization.

Trigeminal autonomic cephalalgias are highly disabling primary headache disorders, characterized by severe unilateral head pain and associated ipsilateral cranial autonomic features. There is limited understanding of their pathophysiology and how and where treatments act to reduce symptoms; this is significantly hindered by a lack of animal models. We have developed the first animal model to explore trigeminal autonomic cephalalgias, using stimulation within the brainstem, at the level of the superior salivatory nucleus, to activate the trigeminal autonomic reflex arc. Using electrophysiological recording of neurons of the trigeminocervical complex and laser Doppler blood flow changes around the ipsilateral lacrimal duct, superior salivatory nucleus stimulation exhibited both neuronal trigeminovascular and cranial autonomic manifestations. These responses were specifically inhibited by the autonomic ganglion blocker hexamethonium bromide. These data demonstrate that brainstem activation may be the driver of both sensory and autonomic symptoms in these disorders, and part of this activation may be via the parasympathetic outflow to the cranial vasculature. Additionally, both sensory and autonomic manifestations were significantly inhibited by highly effective treatments for trigeminal autonomic cephalalgias, such as oxygen, indomethacin and triptans, and some part of their therapeutic action appears to be specifically on the parasympathetic outflow to the cranial vasculature. Treatments more used to migraine, such as naproxen and a calcitonin gene-related peptide receptor inhibitor, olcegepant, were less effective in this model. This is the first model to represent the phenotype of trigeminal autonomic cephalalgias and their response to therapies, and indicates the parasympathetic pathway may be uniquely involved in their pathophysiology and targeted to relieve symptoms.

A history of cigarette smoking is associated with the development of cranial autonomic symptoms with migraine headaches.

OBJECTIVE: To look at the smoking history of migraine patients and to determine if a history of cigarette smoking is associated with the development of cranial autonomic symptoms with migraine headaches. BACKGROUND: It has recently been noted that a significant number of migraine patients may develop autonomic symptoms during their attacks of headache. Why some headache patients activate the trigeminal autonomic reflex and develop cranial autonomic symptoms while others do not is unknown. Cluster headache occurs more often in patients with a history of cigarette smoking, suggesting a link between tobacco exposure and cluster headache pathogenesis. Could cigarette smoking in some manner lead to activation of the trigeminal-autonomic reflex in headache patients? If cigarette smoking does lower the threshold for activation of the trigeminal autonomic reflex then do migraine patients who have a history of cigarette smoking more often develop cranial autonomic symptoms than migraineurs who have never smoked? METHODS: Consecutive patients diagnosed with migraine (episodic or chronic) who were seen over a 7-month time period at a newly established headache center were asked about the presence of cranial autonomic symptoms during an attack of head pain. Patients were deemed to have positive autonomic symptoms along with headache if they experienced at least one of the following symptoms: eyelid ptosis or droop, eyelid or orbital swelling, conjunctival injection, lacrimation, or nasal congestion/rhinorrhea. A smoking history was determined for each patient including was the patient a current smoker, past smoker, or had never smoked. Patients were deemed to have a positive history of cigarette smoking if they had smoked continuously during their lifetime for at least at 1 year. RESULTS: A total of 117 migraine patients were included in the analysis (96 female, 21 male). Forty-six patients had a positive smoking history, while 71 patients had no smoking history. Some 70% (32/46) of migraineurs with a positive history of cigarette smoking had cranial autonomic symptoms along with their headaches, while only 42% (30/71) of the nonsmoking patients experienced at least 1 autonomic symptom along with headaches and this was a statistically significant difference (P < .005). In total, 74% of current smokers had autonomic symptoms with their headaches compared with 61% of past smokers and this was not a statistically significant difference. There was a statistically significant difference between the number of current smokers who had autonomic symptoms with their headaches compared with the number of patients who never smoked and had autonomic symptoms (P < .05). Overall, 52% of the studied migraineurs had autonomic symptoms. There was a statistically significant difference between autonomic symptom occurrence in male and female smokers vs male and female nonsmokers. Each subtype of cranial autonomic symptoms was all more frequent in smokers. CONCLUSION: A history of cigarette smoking appears to be associated with the development of cranial autonomic symptoms with migraine headaches.

Trigemino-autonomic headache related to Gasperini syndrome.

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.

Cranial autonomic symptoms in patients with pituitary adenoma presenting with headaches.

Different types of symptomatic trigeminal autonomic cephalalgias (TACs) have been reported in patients with pituitary adenoma. We investigated the significance of the presence of cranial autonomic symptoms (CAS) in patients with pituitary adenoma presenting with headaches. The records of patients with pituitary adenoma from 1998 to 2004 in our headache clinic were reviewed including headache profile, presence or absence of CAS, and the characteristics of the pituitary adenoma. CAS were ascertained if one or more autonomic symptoms defined for the diagnosis of TACs in the International Classification of Headache Disorders, 2nd edition (ICHD-2) was identified. Thirty-three patients (24F/9M) with pituitary adenoma presenting with headache were recruited for this study: 18 with CAS (55%) and 15 without. Chronic migraine was the most common headache phenotype (n=16, 48%). Three patients were diagnosed as hemicrania continua-like and three, cluster headache-like. In the group with CAS (CAS+), the sides of the tumor were significantly concordant with the sides of headaches (kappa=0.58, p<0.001) and those of CAS (kappa=0.67, p<0.001). However, this relationship was not demonstrated in those without CAS (CAS-) (kappa=0.07, p=0.61). Compared with the patients in the CAS- group, the patients in the CAS+ group had higher frequencies of macroadenoma (78% vs. 40%, p=0.027) and acromegaly (50% vs. 7%, p=0.009). The presence and absence of CAS in pituitary adenoma-associated headache were associated with different characteristics of the underlying pituitary adenomas including side concordance and incidence of acromegaly and macroadenoma. The pathogeneses for headache might differ between these two groups.

[Trigeminal autonomic cephalgias. After initial diagnosis, consider secondary causes]. / Trigeminoautonome Zephalgien. Bei Erstdiagnose auf sekundäre Formen achten.

Trigeminal autonomic cephalgias (TAC) are classified as primary headache syndromes. The use of instrumental procedures including neuroimaging in the diagnostic workup of the TACs is controversially discussed in the literature. Several case reports have been previously published, reporting trigeminal autonomic cephalgias related to structural lesions. We contribute two of our own cases of symptomatic TACs and demonstrate that a "classic" clinical presentation does not preclude a symptomatic etiology. Thus, we advocate a systematic diagnostic evaluation including neuroimaging in every patient presenting with symptoms indicative of TAC for the first time.

Unilateral or "side-locked" migrainous headache with autonomic symptoms linked to night guard use.

Night guards are commonly prescribed as a palliative measure for bruxism, temporomandibular joint symptoms, and associated disorders. We describe a patient with a 10- to 12-year history of night guard use with concurrent unilateral side-locked migrainous headaches with autonomic symptoms characteristic of trigeminal autonomic cephalgia. These headaches were refractory to numerous pharmacological interventions. Upon self-initiated cessation of night guard use, there was complete remission of headaches. We believe the headaches were initiated by night guard-initiated irritation of the trigeminal nerve and a trigeminal autonomic reflex resulting in unilateral migrainous headache with autonomic signs.

Symptomatic trigeminal autonomic cephalalgia associated with allodynia in a patient with multiple sclerosis.

A patient with symptomatic trigeminal autonomic cephalalgia (TAC) provides a chance to understand the pathophysiology and anatomic correlates of TAC. A 28-year-old woman experienced intermittent sharp and excruciating pain over her right temporal, ear and neck regions for 3 days. The headaches lasted 10-20 minutes each, occurred 1-2 times a day, and were accompanied by prominent ipsilateral lacrimation and conjunctival injection. The patient had hiccups, 4-limb numbness and impaired visual acuity in both eyes. She had also had 3 episodes of left-side optic neuritis in the past half year. Neurologic examination showed brushing allodynia over the right face and scalp during the headache attacks. The visual acuity of her right eye was 6/60 and that of the left eye was 1/60. Brain magnetic resonance imaging showed non-enhancing lesions on the right lateral tegmentum of the lower pons where the spinal trigeminal nucleus is located and the floor of the 4th ventricle. The patient was diagnosed as having multiple sclerosis with symptomatic TAC. Her headaches, autonomic signs and allodynia subsided 3 days after pulse therapy and gabapentin treatment were given. We suggest that the spinal trigeminal nucleus lesion was responsible for the symptomatology of TAC and cutaneous allodynia in our patient.

Sex, Gender, and Orofacial Pain.

This review examines gender prevalence in orofacial pain to elucidate underlying factors that can explain such differences. This review highlights how gender affects (1) the association of hormonal factors and pain modulation; (2) the genetic aspects influencing pain sensitivity and pain perception; (3) the role of resting blood pressure and pain threshold; and (4) the impact of sociocultural, environmental, and psychological factors on pain.

Primary Headaches.

The primary headaches are composed of multiple entities that cause episodic and chronic head pain in the absence of an underlying pathologic process, disease, or traumatic injury. The most common of these are migraine, tension-type headache, and the trigeminal autonomic cephalalgias. This article reviews the clinical presentation, pathophysiology, and treatment of each to help in differential diagnosis. These headache types share many common signs and symptoms, thus a clear understanding of each helps prevent a delay in diagnosis and inappropriate or ineffective treatment. Many of these patients seek dental care because orofacial pain is a common presenting symptom.

Trigeminal autonomic cephalgias due to structural lesions: a review of 31 cases.

Trigeminal autonomic cephalgias (TACs) include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache with conjunctival injection and tearing. Associated structural lesions may be found, but a causal relationship is often difficult to establish. We sought to identify clinical predictors of underlying structural abnormalities by reviewing previously described and new TAC and TAC-like cases associated with a structural lesion. We found that even typical TACs can be caused by an underlying lesion. Clinical warning signs and symptoms are relatively rare. We recommend neuroimaging in all patients with a TAC or TAC-like syndrome.

[Cluster headache and other trigeminal-autonomic headaches]. / Algie vasculaire de la face et autres céphalées trigeminoautonomiques.

Cluster headache is a primary headache with a male predominance that presents in two forms: episodic and chronic, occurring at 45-to 60-day intervals with one to three headaches a day lasting 45 min to 2 h. An attack starts by a violent unilateral retro-ocular pain with sympathetic signs such as tearing and rhinorrhea. Diagnosis is made by questioning and therefore requires no complementary tests. Treatment for the attack consists of injectable sumatriptan or oxygen therapy, with long-term treatment with verapamil, lithium salts, or Topiramate; in certain cases in which the number of attacks is greater than two, injections of corticosteroids at the emergence of the Arnold nerve can be used, or in cases of attacks resistant to all treatments, hypothalamus stimulation surgery can be useful.