Trigeminal Autonomic Cephalalgias in Children and Adolescents: Cluster Headache and Related Conditions.

Cluster headache is a primary headache disorder that can occur in children and adolescents, and is a member of the broader diagnostic group of trigeminal autonomic cephalalgias. It is characterized by repeated attacks typically lasting between 15 and 180 minutes of severe unilateral side-locked headache with cranial autonomic features. Acute treatment of the cluster attack can include the use of triptans or high-flow oxygen. Preventive measures typically start with the use of verapamil. The other trigeminal autonomic cephalalgias, paroxysmal hemicrania, short-lasting unilateral neuralgiform headache with conjunctival injection and tearing (SUNCT), and short-lasting unilateral neuralgiform headache with cranial autonomic features (SUNAA), have also been reported in children, and should be considered when the clinical presentation is at all unusual.

The effect of posterior hypothalamus region deep brain stimulation on sleep.

BACKGROUND: Early observations by von Economo showed that the posterior part of the hypothalamus (PH) plays a prominent role in sleep-wake regulation. The PH is a candidate area involved in cluster headaches and other trigeminal autonomic cephalalgias (TACs) and is targeted for deep brain stimulation (DBS). CASE REPORTS: Sleep studies in two men, 69- and 39-years-old, with pre-existing sleep disorders, before and after PH-DBS for pharamacoresistant cluster headache and SUNCT syndrome showed that PH-DBS led to a dramatic alteration of the patients' sleep patterns. This coincided with an improvement of the predominantly diurnal TACs, suggesting a PH-DBS-induced change in sleep patterns. Hypnograms after DBS demonstrated disrupted sleep and a prolonged period of wakefulness after midnight in both patients, which was reproduced the second night. CONCLUSIONS: PH-DBS, a promising treatment for severe refractory TACs, affects sleep quality and pre-existing sleep disorders. This needs to be considered when treating patients with PH-DBS.

[The hypothalamus in SUNCT syndrome: similarities and differences with the other trigeminal-autonomic cephalalgias]. / El hipotálamo en el SUNCT: similitudes y diferencias con las otras cefaleas trigeminoautonómicas.

INTRODUCTION: SUNCT belongs to the group of trigeminal-autonomic cephalalgias (TAC) --cluster headache and paroxysmal hemicranias--, since its shares a series of features with them. SUNCT was finally included in this group when the hypothalamus was proved to play a key role in its pathophysiology, an aspect that it has in common with other TAC. However, its clinical resemblance to trigeminal neuralgia of the first branch is notable, although it is accepted that the genesis of the trigeminal neuralgia is peripheral. DEVELOPMENT: The article presents the evidence available to date that has made it possible to associate the hypothalamus with SUNCT, as well as outlining its similarities and differences with respect to other TAC. This evidence is clinical, hormonal, from functional neuroimaging (activation of the posteroinferior hypothalamus) and from therapeutic outcomes (with deep hypothalamic stimulation). Likewise, a detailed description is provided of both the neuroanatomical bases (the hypothalamus as part of the neural networks involved in processes concerned with behaviour, memory, antinociceptive control, waking-sleep control and other circadian rhythms, etc.) and the neurochemical bases (orexins, somatostatin and endogenous opiates) that would support the hypotheses which researchers are attempting to establish to fit the evidence discussed earlier, which would have many points that overlap from one TAC to another. CONCLUSIONS: The question as to whether the hypothalamus is the/a generator of TAC or whether it is an element that allows its development remains open to debate, as does the issue of which would be the most plausible explanation for the phenotypic differences between them. Future studies will allow the enigma of SUNCT and the other TAC to be explained.

El hipotálamo en el SUNCT: similitudes y diferencias con las otras cefaleas trigeminoautonómicas / The hypothalamus in sunct syndrome: similarities and differences with the other trigeminal-autonomic cephalalgias

Introducción. El SUNCT forma parte del grupo de cefaleas trigeminoautonómicas (CTA) –cefalea en racimos y hemicraniasparoxísticas–, al compartir con ellas una serie de características. El impulso final para incluir el SUNCT en estegrupo fue la demostración de que el hipotálamo es pieza clave en su fisiopatología, dato común con las otras CTA. Sin embargo,su parecido clínico con la neuralgia trigeminal de la primera rama también es llamativo, aunque se admite que la génesisde la neuralgia trigeminal es periférica. Desarrollo. Se exponen las evidencias disponibles hasta el momento actual que hanpermitido involucrar al hipotálamo en el SUNCT, así como sus similitudes y diferencias con las otras CTA. Se trata de evidenciasclínicas, hormonales, de neuroimagen funcional (activación del hipotálamo posteroinferior) y de resultado terapéutico(con estimulación hipotalámica profunda). Asimismo, se detallan las bases neuroanatómicas (el hipotálamo como parte de lasredes neurales involucradas en procesos comportamentales, de memoria, de control antinociceptivo, de control del ritmo vigilia-sueño y otros ritmos circadianos, etc.) y neuroquímicas (orexinas, somatostatina y opiáceos endógenos), que sustentaríanlas hipótesis que tratan de establecerse en virtud de las evidencias previamente comentadas, las cuales tendrían muchospuntos de solapamiento entre las distintas CTA. Conclusión. Queda aún abierto el debate sobre si el hipotálamo es el generadorde las CTA o si es un elemento permisivo en su desarrollo, y cuál sería la explicación más plausible para las diferenciasfenotípicas entre ellas. Posteriores estudios podrán ir aclarando el enigma del SUNCT y de las otras CTA(AU)

Introduction. SUNCT belongs to the group of trigeminal-autonomic cephalalgias (TAC) –cluster headache andparoxysmal hemicranias–, since its shares a series of features with them. SUNCT was finally included in this group when thehypothalamus was proved to play a key role in its pathophysiology, an aspect that it has in common with other TAC. However,its clinical resemblance to trigeminal neuralgia of the first branch is notable, although it is accepted that the genesis of thetrigeminal neuralgia is peripheral. Development. The article presents the evidence available to date that has made it possibleto associate the hypothalamus with SUNCT, as well as outlining its similarities and differences with respect to other TAC. Thisevidence is clinical, hormonal, from functional neuroimaging (activation of the posteroinferior hypothalamus) and fromtherapeutic outcomes (with deep hypothalamic stimulation). Likewise, a detailed description is provided of both theneuroanatomical bases (the hypothalamus as part of the neural networks involved in processes concerned with behaviour,memory, antinociceptive control, waking-sleep control and other circadian rhythms, etc.) and the neurochemical bases(orexins, somatostatin and endogenous opiates) that would support the hypotheses which researchers are attempting toestablish to fit the evidence discussed earlier, which would have many points that overlap from one TAC to another.Conclusions. The question as to whether the hypothalamus is the/a generator of TAC or whether it is an element that allows itsdevelopment remains open to debate, as does the issue of which would be the most plausible explanation for the phenotypicdifferences between them. Future studies will allow the enigma of SUNCT and the other TAC to be explained(AU)

SUNA syndrome with seasonal pattern.

SUNA is a trigeminal autonomic cephalalgia (TAC) characterized by short unilateral attacks centered on the ophthalmic trigeminal distribution, and accompanied by at least one of a number of cranial autonomic symptoms that can include lacrimation, redness of the ipsilateral eye, nasal congestion, rhinorrhea, and eyelid edema. It exists in episodic and chronic form. We have described an atypical case of episodic SUNA with an exclusive seasonal pattern as previously reported in other trigeminal autonomic cephalalgia, commonly known as TACs.

Attack-related brainstem activation in a patient with SUNCT syndrome: an ictal fMRI study.

The authors report functional magnetic resonance imaging (fMRI) study data of a 60-year-old patient having short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome. Three consecutive pain attacks were detected during the imaging session and strong brainstem activation was found. It was concluded that the brainstem can be involved in the pain signal transmission in SUNCT syndrome.