A Rare Association of Trigeminal Autonomic Cephalgia: Pontine Capillary Telangiectasia.

This report describes a case of pontine capillary telangiectasia in a 43-year-old woman with a clinical diagnosis of trigeminal autonomic cephalgia. The possible association with pontine capillary telangiectasia and trigeminal autonomic cephalgia is discussed.

SUNCT, SUNA and trigeminal neuralgia: different disorders or variants of the same disorder?

PURPOSE OF REVIEW: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT), short-lasting unilateral neuralgiform headache attacks with autonomic symptoms (SUNA) and trigeminal neuralgia are considered different disorders, thus grouped in separate sections of the International Classification of Headache Disorders 3 beta. However, the clinical, radiological and therapeutic overlap between SUNCT, SUNA, and trigeminal neuralgia has challenged this traditional view. This review summarizes the available clinical and pathophysiological evidence on whether SUNCT, SUNA and trigeminal neuralgia should be considered separate entities or variants of the same disorder. RECENT FINDINGS: Data on the clinical phenotype and effective management strategies in SUNCT and SUNA syndromes have shown striking similarities with trigeminal neuralgia. Moreover, studies exploring radiological findings supported the hypothesis of common aetiological and pathophysiological basis between SUNCT/SUNA and trigeminal neuralgia. However, a limitation of most studies is that they have included small samples of patients and therefore any conclusions need to be drawn cautiously. SUMMARY: Despite being considered distinct conditions, emerging clinical and radiological evidence supports a broader nosological concept of SUNCT, SUNA, and trigeminal neuralgia. These conditions may constitute a continuum of the same disorder, rather than separate clinical entities. Further evidence is required to shed light on this nosological issue, given its potential impact on clinical practice and further research studies in this area.

SUNCT/SUNA and neurovascular compression: new cases and critical literature review.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) and short-lasting unilateral neuralgiform headache with cranial autonomic symptoms (SUNA) are primary headache syndromes. A growing body of literature has focused on brain magnetic resonance imaging (MRI) evidence of neurovascular compression in these syndromes. OBJECTIVE: The objective of this article is to assess whether SUNCT is a subset of SUNA or whether the two are separate syndromes and clarify the role of neurovascular compression. METHOD: We describe three new SUNCT cases with MRI findings of neurovascular compression and critically review published SUNCT/SUNA cases. RESULTS: We identified 222 published SUNCT/SUNA cases. Our three patients with neurovascular compression added to the 34 cases previously described (16.9%). SUNCT and SUNA share the same clinical features and therapeutic options. At present, there is no available abortive treatment for attacks. Lamotrigine was effective in 64% of patients; topiramate and gabapentin in about one-third of cases. Of the 34 cases with neurovascular compression, seven responded to drug therapies, 16 patients underwent microvascular decompression of the trigeminal nerve (MVD) with effectiveness in 75%. CONCLUSIONS: We suggest that SUNCT and SUNA should be considered clinical phenotypes of the same syndrome. Brain MRI should always be performed with a dedicated view to exclude neurovascular compression. The high percentage of remission after MVD supports the pathogenetic role of neurovascular compression.

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.

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.

Pathophysiology of trigeminal autonomic cephalalgias.

Cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) are primary headaches recently classified together as trigeminal autonomic cephalalgias (TACs). The causes of these headaches have long been debated, with "peripheral" hypotheses in opposition to "central" hypotheses. The available information indicates that the pain originates from within the brain in cluster headache. The hypothalamic activation observed during TAC attacks by use of functional neuroimaging, and the success of hypothalamic stimulation as a treatment, confirm that the posterior hypothalamus is crucial in the pathophysiology of these headaches. The posterior hypothalamus is now known to modulate craniofacial pain, and hypothalamic activation occurs in other pain disorders, suggesting that this brain area is likely to have a more complex role in the pathophysiology of TACs than that of a mere trigger. Hypothalamic activation might play a part in terminating rather than triggering attacks, and might also give rise to a central permissive state, allowing attacks to take place.

Neuroimaging in trigeminal autonomic cephalgias: when, how, and of what?

PURPOSE OF REVIEW: Trigeminal autonomic cephalgias (TACs) are characterized by frequent, short-lasting headache attacks with ipsilateral facial autonomic features. They include cluster headache, paroxysmal hemicrania, and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing. The pathogenesis of TACs is largely unknown, but many case reports in the literature suggest that TACs are secondary to structural lesions. Thus, the question arises whether TAC patients should undergo neuroimaging. Here, we review the recent literature on secondary TACs and attempt to formulate guidelines for neuroimaging. RECENT FINDINGS: Recently, we published two reviews of, in total, 33 case reports of patients with a secondary TAC or TAC-like syndrome. Since then, 23 additional cases have been published. Here, we provide a summary of these 56 case reports. TACs were found to be associated with a wide range of both intracranial and extracranial neurovascular and structural lesions. We could not identify a 'typical' clinical warning profile for secondary TACs as these patients could present with clinical features that are entirely characteristic of a TAC, including alternating attack and attack-free periods, and excellent response to TAC-specific treatments. SUMMARY: Even clinically typical TACs can be caused by structural lesions. There are no 'typical' warning signs or symptoms. Neuroimaging should be considered in all patients with TAC or TAC-like syndromes, notably in those with atypical presentation. Depending on the degree of suspicion, additional imaging should be considered assessing intracranial and cervical vasculature, and the sellar and paranasal region.

Functional and structural neuroimaging in trigeminal autonomic cephalalgias.

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

[Trigeminal autonomic cephalalgias: three forms of unilateral, short-lasting headache with facial autonomic symptoms]. / Trigeminale autonome cefalalgie: drie vormen van eenzijdige, kortdurende hoofdpijn met faciale autonome verschijnselen.

--Trigeminal autonomic cephalalgias (TACs) include cluster headache, paroxysmal hemicranias and short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCTs). --Because they are rare, it is often difficult to recognise TACs in practice. Hallmarks of TACs include the strictly unilateral pain near the eye or temple, the accompanying autonomic symptoms, and the specific pattern in the timing of the attacks. --The TAC subtypes differ in the duration and frequency of attacks. Differentiating TAC subtypes is important because it affects the treatment approach. --In rare cases, TAC results from an underlying structural disorder. Neuroimaging (cerebral MRI) is advised in all patients with TAC.

Neuromodulatory approaches to the treatment of trigeminal autonomic cephalalgias.

The trigeminal autonomic cephalalgias (TACs) are a group of primary headache syndromes characterised by intense pain and associated activation of cranial parasympathetic autonomic outflow pathways out of proportion to the pain. The TACs include cluster headache, paroxysmal hemicrania and SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing). The pathophysiology of these syndromes involves activation of the trigeminal-autonomic reflex, whose afferent limb projects into the trigeminocervical complex in the caudal brainstem and upper cervical spinal cord. Functional brain imaging has shown activations in the posterior hypothalamic grey matter in TACs. This paper reviews the anatomy and physiology of these conditions and the brain imaging findings. Current treatments are summarised and the role of neuromodulation procedures, such as occipital nerve stimulation and deep brain stimulation in the posterior hypothalamus are reviewed. Neuromodulatory procedures are a promising avenue for these highly disabled patients with treatment refractory TACs.

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.

Cefaleas primarias revisión de criterios diagnósticos y abordajes terapéuticos / Primary cephaleas: a revision of its diagnosis and therapeutic approach

Esta revisión aborda las cefaleas primarias, su diagnóstico y tratamiento. Para la IHS son: migraña, cefalea tensional, cluster y otros. Migraña: La segunda en prevalencia y la de mayor morbilidad, el aura diferencia la migraña con aura (Mca) de la sin aura (Msa). Epidemiología: prevalencia 10% con una relación 3: 1 mujer-hombre. Fisiopatología: es un trastorno nociceptivo central primario, con compromiso secundario vascular. Tratamiento: específico y no específico, tanto de las crisis como preventivo. Trastornos psiquiátricos y migraña: existe evidencia de correlación clínico patológica y farmacológica mayormente entre Mca y diversas patologías psiquiátricas. Migraña en el niño: se presenta con diferencias clínicas responde a otra farmacoterapia y es de gran importancia el enfoque familiar. Migraña y hormonas: el principal factor hormonal relacionado a la migraña es la caída hormonal. Migraña y epilepsia: comparte la hiperexcitabilidad cerebral y medicación. Migraña y enfermedad cerebrovascular: factores vasculares e isquémicos comunes. Medicina basada en la evidencia: supone un importante avance en la medición de la efectividad de los tratamientos. Cefalea tensional: la más frecuente de todas las cefaleas, subdiagnosticada. Diferencias entre episódica y crónica. Epidemiología: leve preponderancia femenina. Fisiopatología y factores influyentes: destaca el rol del sistema nociceptivo, el sedentarismo, el estrés y la tensión muscular. Tratamiento: farmacológico y no farmacológico. Cefalea en racimo: de menor prevalencia pero con clínica frondosa, muy invalidante pero con tratamiento efectivo. Otras cefaleas primarias: es importante desensibilizar de los factores desencadenantes. Conclusión: rescatamos conceptos sobre la tarea del médico de aliviar el sufrimiento del paciente, a través de un diagnóstico y un tratamiento adecuados.

This review focuses on primary headaches, its diagnosis and treatment. For the IHS specialists they are: migraine, tension-type headache (TTH), cluster and others. Migraine: the second in prevalence and the first in morbility, clinical features and differences between migraine with (Ma) and without aura (Mo). Epidemiology: 10% prevalence and 3: 1 women to men proportion. Patophysiology: primary central nociception disorder with secondary vascular involvement. Treatment: specific and non-specific, acute and preventive. Psychiatric disorders and migraine: there is evidence of clinical and pharmacologicallinks, mainly between Ma and several psychiatric disorders. Migraine in children’s: important clinical and therapeutic differences from adult, importance of family approach. Migraine and hormones: the importance of estrogens drops, as trigger factor, treatment. Migraine and epilepsy: both shares neuronal hyperexcitability pattern. Migraine and stroke: vascular and ischemic factors involved. Evidence-based medicine: improves treatment's results and studies outcome evaluation. TTH: first in prevalence, still highly sub diagnosed. Main clinical presentations: episodic and chronic. Epidemiology: slight female preponderance. Patophysiology and trigger factors: the role of limbic nociceptive system, sedentarism stress and muscular tension. Treatment: pharmacological and non-pharmacological. Cluster headache: low prevalence but high daily-living activities impact, effective treatment. Other primary headaches: variability of trigger factors and role of desensitization process. Conclusion: we remark the complexity of headache and the of physicians' role: to relief patients suffering, throughout a precise diagnosis and treatment.

Cefaleas primarias revisión de criterios diagnósticos y abordajes terapéuticos / Primary cephaleas: a revision of its diagnosis and therapeutic approach

Esta revisión aborda las cefaleas primarias, su diagnóstico y tratamiento. Para la IHS son: migraña, cefalea tensional, cluster y otros. Migraña: La segunda en prevalencia y la de mayor morbilidad, el aura diferencia la migraña con aura (Mca) de la sin aura (Msa). Epidemiología: prevalencia 10% con una relación 3: 1 mujer-hombre. Fisiopatología: es un trastorno nociceptivo central primario, con compromiso secundario vascular. Tratamiento: específico y no específico, tanto de las crisis como preventivo. Trastornos psiquiátricos y migraña: existe evidencia de correlación clínico patológica y farmacológica mayormente entre Mca y diversas patologías psiquiátricas. Migraña en el niño: se presenta con diferencias clínicas responde a otra farmacoterapia y es de gran importancia el enfoque familiar. Migraña y hormonas: el principal factor hormonal relacionado a la migraña es la caída hormonal. Migraña y epilepsia: comparte la hiperexcitabilidad cerebral y medicación. Migraña y enfermedad cerebrovascular: factores vasculares e isquémicos comunes. Medicina basada en la evidencia: supone un importante avance en la medición de la efectividad de los tratamientos. Cefalea tensional: la más frecuente de todas las cefaleas, subdiagnosticada. Diferencias entre episódica y crónica. Epidemiología: leve preponderancia femenina. Fisiopatología y factores influyentes: destaca el rol del sistema nociceptivo, el sedentarismo, el estrés y la tensión muscular. Tratamiento: farmacológico y no farmacológico. Cefalea en racimo: de menor prevalencia pero con clínica frondosa, muy invalidante pero con tratamiento efectivo. Otras cefaleas primarias: es importante desensibilizar de los factores desencadenantes. Conclusión: rescatamos conceptos sobre la tarea del médico de aliviar el sufrimiento del paciente, a través de un diagnóstico y un tratamiento adecuados.(AU)

This review focuses on primary headaches, its diagnosis and treatment. For the IHS specialists they are: migraine, tension-type headache (TTH), cluster and others. Migraine: the second in prevalence and the first in morbility, clinical features and differences between migraine with (Ma) and without aura (Mo). Epidemiology: 10% prevalence and 3: 1 women to men proportion. Patophysiology: primary central nociception disorder with secondary vascular involvement. Treatment: specific and non-specific, acute and preventive. Psychiatric disorders and migraine: there is evidence of clinical and pharmacologicallinks, mainly between Ma and several psychiatric disorders. Migraine in childrenãs: important clinical and therapeutic differences from adult, importance of family approach. Migraine and hormones: the importance of estrogens drops, as trigger factor, treatment. Migraine and epilepsy: both shares neuronal hyperexcitability pattern. Migraine and stroke: vascular and ischemic factors involved. Evidence-based medicine: improves treatments results and studies outcome evaluation. TTH: first in prevalence, still highly sub diagnosed. Main clinical presentations: episodic and chronic. Epidemiology: slight female preponderance. Patophysiology and trigger factors: the role of limbic nociceptive system, sedentarism stress and muscular tension. Treatment: pharmacological and non-pharmacological. Cluster headache: low prevalence but high daily-living activities impact, effective treatment. Other primary headaches: variability of trigger factors and role of desensitization process. Conclusion: we remark the complexity of headache and the of physicians role: to relief patients suffering, throughout a precise diagnosis and treatment.(AU)