Cefalea en racimos en edad pediátrica: descripción de cuatro casos y revisión de la bibliografía / Cluster headache in pediatric population: four case reports and review of the literature

Introducción. La cefalea en racimos es una cefalea primaria de origen trigeminoautonómico cuyo inicio en la infancia es infrecuente. Se presentan cuatro casos en los que el inicio de la sintomatología se produjo entre los 2 y los 13 años. Casos clínicos. Se incluyen tres varones y una niña con inicio a los 2, 7, 13 y 12 años, respectivamente. Los cuatro pacientes cumplen los criterios propuestos por la tercera edición de la Clasificación Internacional de las Cefaleas ICHD-III (beta). Conclusiones. A pesar de ser poco frecuente durante la edad pediátrica, la cefalea en racimos debe formar parte del diagnóstico diferencial de un niño que consulta por cefalea. Subrayamos la importancia de conocer sus criterios diagnósticos para evitar el retraso diagnóstico que se ha descrito con frecuencia. En nuestros pacientes, el tratamiento con verapamilo resultó más eficaz que el tratamiento con flunaricina. Los tratamientos con mejor respuesta en fase aguda fueron la oxigenoterapia y los triptanes (AU)

Introduction. Cluster headache is a rare cause of primary headache in children. We report four cases with a mean age of onset of 8.6, ranged from 2 to 13 years. Case reports. Three males and one female with onset at 2, 7, 13, and 12 years-old, respectively, were included. The symptoms of all patients fulfill the criteria for the diagnosis of cluster headache according to the International Society of Headache. Conclusions. Despite being rare during childhood, cluster headache should be part of the differential diagnosis of headache in childhood. This report highlights the variable features of this disorder in children, often misdiagnosed. It can be useful in making a quick diagnosis and starting the appropriate treatment early. Verapamil was more effective than flunarizine in terminating the headache in our patients. Oxygen treatment and triptans resulted the treatments with the best response in acute cluster headache (AU)

Pathogenesis, Surgical Treatment, and Cure for SUNCT Syndrome.

BACKGROUND: Short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) are characterized by attacks of moderate to severe stabbing pain, strictly unilateral, with periorbital or temporal distribution, associated with cranial autonomic symptoms, such as lacrimation and redness of the ipsilateral eye. METHODS: To obtain mechanistic insights into the pathogenesis of SUNCT syndrome, more than 800 cases treated in our institution during the last 7 years were retrospectively reviewed. Two patients showed typical autonomic symptoms of SUNCT. RESULTS: Magnetic resonance imaging suggested potential compression of the trigeminal nerve by the intracranial artery in these cases and complete remission was achieved by microvascular decompression. CONCLUSIONS: Microvascular decompression provides an appropriate therapeutic choice if vascular compression of the trigeminal nerve is identified. From our 2 cases, we propose that, in some cases of SUNCT diagnosed previously, characteristic symptoms were induced by compression of the side surface of the first branch of the trigeminal nerve at the root exit zone by the intracranial artery.

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.

SUNCT responsive to percutaneous balloon compression of the gasserian ganglion--10-year follow-up.

We report the case of a woman with short-lasting unilateral, neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) whose severe headache attacks ceased after percutaneous balloon compression of the Gasserian ganglion. The patient remains pain free after 10-year follow-up. This may be the first literature report of SUNCT in Chile.

SUNCT syndrome or first division trigeminal neuralgia associated with cerebellar hypoplasia.

Short-lasting unilateral neuralgiform headache (SUNCT) and first division trigeminal neuralgia (TN) are rare and very similar periorbital unilateral pain syndromes. Few cases of SUNCT are associated with posterior skull lesions. We describe a 54-year-old man with symptoms compatible with both the previous painful syndromes, associated with a small posterior skull and a cerebellar hypoplasia. The short height and the reported bone fractures could be compatible with a mild form of osteogenesis imperfecta, previously described in one case associated with SUNCT. However, a hypoplastic posterior cranial fossa characterizes also Chiari I malformation. The difficult differential diagnosis between SUNCT and TN and their relation with posterior skull malformations is debated.

Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing syndrome treated successfully with transsphenoidal resection of a growth hormone-secreting pituitary adenoma.

Short-lasting, unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT) syndrome was first described in 1978 as one of the trigeminal autonomic cephalgias. In this paper the authors present a patient with a growth hormone-secreting pituitary adenoma who experienced resolution of SUNCT syndrome after transsphenoidal tumor resection.

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.

[Symptomatic SUNCT with cerebral abscess and subdural empyema]. / Syndrome SUNCT symptomatique d'abcès et d'empyème cérébraux.

SUNCT syndrome is a rare form of a primary headache disorder, although secondary causes, particularly posterior fossa abnormalities, are well known. We report a new case in a 67-year-old man suffering SUNCT syndrome secondary to pyogenic cerebral abscess and empyema localized in the convexity portion of the right frontal lobe.

Chronic high frequency stimulation of the posteromedial hypothalamus in facial pain syndromes and behaviour disorders.

Chronic high frequency stimulation (HFS) of the posteromedial hypothalamus (PMH) has been the first direct therapeutic application of functional neuroimaging data in a restorative reversible procedure for the treatment of an otherwise refractory neurological condition; in fact, the target coordinates for the stereotactic implantation of the electrodes have been provided by positron emission tomography (PET) studies, which were performed during cluster headache attacks. HFS of PMH produced a significant and marked reduction of pain attacks in patients with chronic cluster headache (CCH) and in one patient with short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing (SUNCT). The episodes of violent behaviour and psychomotor agitation during the attacks of CCH supported the idea that the posteromedial hypothalamus could be also involved in the control of aggressiveness; this has been previously suggested, in the seventies, by the results obtained in Sano's hypothalamotomies for the treatment of abnormal aggression and disruptive behaviour. On the basis of these considerations, we have performed HFS of the PMH and controlled successfully violent and disruptive behaviour in patients refractory to the conventional sedative drugs. Finally, we also tested the same procedure in three patients with refractory atypical facial pain, but unfortunately, they did not respond to this treatment.

SUNCT and high nocturnal prolactin levels: some new unusual characteristics.

SUNCT is a rare condition characterised by a short-lasting periorbital pain associated with autonomic symptoms and is usually unresponsive to pharmacological treatment. We report a case of SUNCT syndrome linked to a pituitary micro-adenoma, with only nocturnal attacks. The nocturnal levels of prolactin (PRL) were increased, while other hormonal, haematological, serological and biochemical investigations and levels of PRL did not reveal abnormal findings during the day-time. PRL serum secretion after thyrotropin-releasing hormone test was lower than nocturnal secretion, but not enough to induce severe attacks. We suggest that in our patient the rise of nocturnal levels of PRL could have a direct role in the worsening of this headache, perhaps secondarily to an altered regulation of the hypothalamic-hypophysial axis, however the actual influence of sleep and the interaction between all neurotransmitters and hormones needs to be clarified further.