ABSTRACT
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)
Subject(s)
Humans , Male , Female , Child , Adolescent , Cluster Headache/diagnosis , Cluster Headache/psychology , Pediatrics/education , Therapeutics/methods , Pharmaceutical Preparations/administration & dosage , Oxygen Inhalation Therapy/methods , SUNCT Syndrome/pathology , Cluster Headache/complications , Cluster Headache/metabolism , Pediatrics , Therapeutics/standards , Pharmaceutical Preparations/metabolism , Oxygen Inhalation Therapy/instrumentation , SUNCT Syndrome/diagnosisABSTRACT
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.
Subject(s)
SUNCT Syndrome/pathology , SUNCT Syndrome/surgery , Adolescent , Adult , Aged , Aged, 80 and over , Cerebral Arteries/pathology , Cerebral Arteries/surgery , Female , Headache/etiology , Humans , Magnetic Resonance Imaging , Male , Microvascular Decompression Surgery , Middle Aged , Nerve Compression Syndromes/pathology , Nerve Compression Syndromes/surgery , Pain/etiology , Retrospective Studies , Treatment Outcome , Trigeminal Nerve Diseases/pathology , Trigeminal Nerve Diseases/surgery , Young AdultABSTRACT
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.
Subject(s)
Hypothalamus, Posterior/physiopathology , SUNCT Syndrome/physiopathology , Trigeminal Nerve/physiopathology , Trigeminal Neuralgia/physiopathology , Anticonvulsants/therapeutic use , Carbamazepine/analogs & derivatives , Carbamazepine/therapeutic use , Deep Brain Stimulation , Functional Neuroimaging , Humans , Hypothalamus, Posterior/pathology , Lamotrigine , Magnetic Resonance Imaging , Oxcarbazepine , SUNCT Syndrome/pathology , SUNCT Syndrome/therapy , Triazines/therapeutic use , Trigeminal Autonomic Cephalalgias/pathology , Trigeminal Autonomic Cephalalgias/physiopathology , Trigeminal Autonomic Cephalalgias/therapy , Trigeminal Nerve/pathology , Trigeminal Nerve/surgery , Trigeminal Neuralgia/pathology , Trigeminal Neuralgia/therapyABSTRACT
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.
Subject(s)
Nerve Compression Syndromes/pathology , SUNCT Syndrome/pathology , Arteries/pathology , Cerebellum/blood supply , Female , Humans , Magnetic Resonance Imaging , Male , Microvascular Decompression Surgery , Middle Aged , Nerve Compression Syndromes/surgery , SUNCT Syndrome/surgery , Trigeminal Autonomic Cephalalgias/pathology , Trigeminal Autonomic Cephalalgias/surgery , Trigeminal Nerve/pathology , Trigeminal Nerve/surgerySubject(s)
Cavernous Sinus/pathology , Intracranial Aneurysm/complications , SUNCT Syndrome/complications , Angiography, Digital Subtraction , Brain/pathology , Empty Sella Syndrome/pathology , Endovascular Procedures , Eye Diseases/etiology , Female , Humans , Intracranial Aneurysm/pathology , Intracranial Aneurysm/surgery , Magnetic Resonance Imaging , Middle Aged , Pain/etiology , SUNCT Syndrome/pathology , SUNCT Syndrome/surgery , StentsSubject(s)
Amines/therapeutic use , Analgesics/therapeutic use , Cyclohexanecarboxylic Acids/therapeutic use , Magnetic Resonance Imaging/methods , SUNCT Syndrome/drug therapy , SUNCT Syndrome/pathology , gamma-Aminobutyric Acid/therapeutic use , Education, Medical, Continuing , Female , Gabapentin , Humans , Middle Aged , SUNCT Syndrome/diagnosisSubject(s)
Brain/pathology , Multiple Sclerosis/complications , Multiple Sclerosis/pathology , SUNCT Syndrome/etiology , SUNCT Syndrome/pathology , Anticonvulsants/therapeutic use , Brain/physiopathology , Carbamazepine/therapeutic use , Female , Humans , Immunoglobulin G/cerebrospinal fluid , Magnetic Resonance Imaging , Middle Aged , Multiple Sclerosis/physiopathology , Nerve Fibers, Myelinated/pathology , Neural Pathways/pathology , Neural Pathways/physiopathology , Oligoclonal Bands/cerebrospinal fluid , Pons/pathology , Pons/physiopathology , SUNCT Syndrome/physiopathology , Spinal Cord/pathology , Spinal Cord/physiopathology , Trigeminal Neuralgia/drug therapy , Trigeminal Neuralgia/etiology , Trigeminal Nuclei/pathology , Trigeminal Nuclei/physiopathologyABSTRACT
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.
Subject(s)
Catheterization/methods , Neurosurgical Procedures/methods , SUNCT Syndrome/pathology , SUNCT Syndrome/surgery , Trigeminal Ganglion/surgery , Aged , Analgesics/therapeutic use , Catheterization/instrumentation , Female , Follow-Up Studies , Humans , Neurosurgical Procedures/instrumentation , Patient Satisfaction , SUNCT Syndrome/physiopathology , Time , Treatment Outcome , Trigeminal Ganglion/pathology , Trigeminal Ganglion/physiopathologyABSTRACT
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.
Subject(s)
Cerebellum/abnormalities , Cranial Fossa, Posterior/abnormalities , Nervous System Malformations/complications , SUNCT Syndrome/etiology , Trigeminal Neuralgia/etiology , Amines/therapeutic use , Arnold-Chiari Malformation/complications , Arnold-Chiari Malformation/pathology , Arnold-Chiari Malformation/physiopathology , Carbamazepine/therapeutic use , Cyclohexanecarboxylic Acids/therapeutic use , Drug Therapy, Combination , Fractures, Bone/complications , Fractures, Bone/congenital , Gabapentin , Growth Disorders/complications , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Nervous System Malformations/pathology , Nervous System Malformations/physiopathology , Ophthalmic Nerve/physiopathology , Osteogenesis Imperfecta/complications , SUNCT Syndrome/pathology , SUNCT Syndrome/physiopathology , Treatment Outcome , Trigeminal Neuralgia/pathology , Trigeminal Neuralgia/physiopathology , gamma-Aminobutyric Acid/therapeutic useABSTRACT
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.
Subject(s)
Adenoma/surgery , Growth Hormone-Secreting Pituitary Adenoma/surgery , SUNCT Syndrome/surgery , Adenoma/complications , Adenoma/pathology , Adult , Growth Hormone-Secreting Pituitary Adenoma/complications , Growth Hormone-Secreting Pituitary Adenoma/pathology , Humans , Male , SUNCT Syndrome/etiology , SUNCT Syndrome/pathologyABSTRACT
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.
Subject(s)
Diagnostic Imaging/methods , Trigeminal Autonomic Cephalalgias/pathology , Trigeminal Autonomic Cephalalgias/physiopathology , Brain/pathology , Brain/physiology , Humans , Paroxysmal Hemicrania/diagnosis , Paroxysmal Hemicrania/pathology , Paroxysmal Hemicrania/physiopathology , SUNCT Syndrome/diagnosis , SUNCT Syndrome/pathology , SUNCT Syndrome/physiopathology , Trigeminal Autonomic Cephalalgias/diagnosis , Trigeminal Nerve/pathology , Trigeminal Nerve/physiologyABSTRACT
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.
Subject(s)
Brain Abscess/complications , Empyema, Subdural/complications , SUNCT Syndrome/complications , Aged , Brain Abscess/pathology , Empyema, Subdural/pathology , Frontal Lobe/pathology , Humans , Magnetic Resonance Imaging , Male , SUNCT Syndrome/pathologyABSTRACT
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.
Subject(s)
Behavioral Symptoms/surgery , Deep Brain Stimulation/methods , Facial Neuralgia/therapy , Hypothalamus, Posterior/surgery , Adult , Aged , Behavioral Symptoms/pathology , Behavioral Symptoms/physiopathology , Dose-Response Relationship, Radiation , Facial Neuralgia/pathology , Facial Neuralgia/physiopathology , Female , Functional Laterality , Humans , Hypothalamus, Posterior/physiopathology , Male , Middle Aged , SUNCT Syndrome/pathology , SUNCT Syndrome/physiopathology , SUNCT Syndrome/surgery , Time Factors , Treatment OutcomeABSTRACT
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.