Temporary resolution of hemicrania continua following ipsilateral ear piercing

BackgroundHemicrania continua is an uncommon subtype of trigeminal autonomic cephalgia that exhibits dramatic therapeutic response to indomethacin. Unfortunately, indomethacin is associated with a range of adverse effects, including neuropsychiatric complications, which limits its use in many patients. Although no other effective pharmacologic agents exist, there is emerging evidence for interventional treatments such as occipital nerve and vagus nerve stimulation, which may act by modulating neural activity within the trigeminovascular system.CaseWe present a 30-year-old woman with long-standing refractory hemicrania continua who suffered adverse effects to indomethacin. She experienced temporary, but near-complete, symptom resolution following piercing of the crus of the ear helix ipsilateral to her headache, whereas contralateral piercing produced no benefit.ConclusionsTo our knowledge, this case is the first to describe a therapeutic benefit following ear piercing in a patient with trigeminal autonomic cephalgia. We argue that symptom relief was obtained through a similar mechanism to occipital or vagus nerve stimulation.

Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT) Status Like Condition: A Rare Case Report and Review of the Literature.

Background: SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is rare trigeminal autonomic cephalgia characterized by recurrent, brief, excruciating unilateral, intermittent headache paroxysms over orbital, frontal or temporal region occurring multiple times per day and it can rarely present as “SUNCTstatus like condition” (SSLC). Case Report: A 28-year old male with a history of SUNCT headache for 6 months presented with left forehead stabs lasting for 30 seconds with a frequency of 40-45 episodes per hour for three days followed by infective gastroenteritis. His neurological examination was normal, except left-sided ptosis, tearing, and conjunctival injection. His MRI brain with contrast, MR angiography, and laboratory investigations were unremarkable except mild hypokalemia. He was treated with intravenous fluids, potassium replacement, and high dose methylprednisolone along with an escalated dose of carbamazepine. Review and Conclusion: We have reviewed the previously reported seven cases and our case of SSLC. Female: Male ratio was 3:1and the mean age was 40.87 years. Three patients responded to high dose steroids and three to lignocaine along with rapid escalation or change of anticonvulsant drugs. One case responded to the high dose of lamotrigine, and in a pregnant lady, the pain subsided only after the termination of the pregnancy. One case was secondary to multiple sclerosis, while the rest of seven were primary episodic SSLC. The condition is highly disabling, and the treatment with steroids or lignocaine, along with the rapid escalation of preventive drugs, can provide long-lasting relief

Short-Lasting Unilateral Neuralgiform Headache Attacks with Conjunctival Injection and Tearing (SUNCT) Status Like Condition: A Rare Case Report and Review of the Literature.

Background: SUNCT (short-lasting unilateral neuralgiform headache attacks with conjunctival injection and tearing) is rare trigeminal autonomic cephalgia characterized by recurrent, brief, excruciating unilateral, intermittent headache paroxysms over orbital, frontal or temporal region occurring multiple times per day and it can rarely present as “SUNCTstatus like condition” (SSLC). Case Report: A 28-year old male with a history of SUNCT headache for 6 months presented with left forehead stabs lasting for 30 seconds with a frequency of 40-45 episodes per hour for three days followed by infective gastroenteritis. His neurological examination was normal, except left-sided ptosis, tearing, and conjunctival injection. His MRI brain with contrast, MR angiography, and laboratory investigations were unremarkable except mild hypokalemia. He was treated with intravenous fluids, potassium replacement, and high dose methylprednisolone along with an escalated dose of carbamazepine. Review and Conclusion: We have reviewed the previously reported seven cases and our case of SSLC. Female: Male ratio was 3:1and the mean age was 40.87 years. Three patients responded to high dose steroids and three to lignocaine along with rapid escalation or change of anticonvulsant drugs. One case responded to the high dose of lamotrigine, and in a pregnant lady, the pain subsided only after the termination of the pregnancy. One case was secondary to multiple sclerosis, while the rest of seven were primary episodic SSLC. The condition is highly disabling, and the treatment with steroids or lignocaine, along with the rapid escalation of preventive drugs, can provide long-lasting relief

063 Headache with periorbital ecchymosis; a rare clinical entity

ObjectivesPrimary headache disorders are common with migraine and tension headache accounting for the vast majority of cases. A smaller proportion suffer from trigeminal autonomic cephalgia (TAC). We present a 23-year-old Caucasian female who described characteristic, episodic headaches starting with a dull retro-orbital/bi-frontal pressure evolving, over the course of 1 minute, to experience florid periorbital ecchymosis. While this phenomenon has been described in the literature, the characteristics of our case are unique and noteworthy of reporting.MethodsWe reviewed the literature surrounding this rare entity by using PubMed/OVID databases and the search terms ‘Headache AND ecchymosis’.ResultsCase reports exist in older patients1–3, where the headache is side locked and associated with other autonomic characteristics such as periorbital oedema, conjunctival injection and tearing. Our case is a young female with only ecchymosis in a unilateral and/or bilateral manner and no other autonomic or indeed migraine features. The patient underwent vascular/cranial imaging and blood tests to exclude haematological, autoimmune, vasculitic causes for this presentation which were unrewarding.ConclusionVariations on this clinical entity are described;1–4 we hope this report may bring attention to this fascinating phenomenon. The pathophysiological process is likely to be similar to those implicated in TACs, namely activation of the trigemino-neurovascular system and facial autonomic pathways. The release of neuromediators such as CGRP, VIP and Substance P cause blood vessel fragility resulting in diapedesis. Optimal treatment regimens are unknown but various agents have been trialled. Our patient declined treatment and continues to be followed.ReferencesDeBroff B, et al. Migraine Associated with Periorbital Ecchymosis. Headache 1990;30:260–263.Dafer R, et al. Atypical Chronic Headache and Recurrent Facial Ecchymosis: A Case Report. Neuro-Ophthalmology 2011;35:76–77.Nozzolillo D, et al. Migraine associated with facial ecchymoses ipsilateral to the symptomatic side. J Headache Pain ( 2004) 5:256–259.Sethi PK, et al. Teaching neuroimages: Red forehead dot syndrome and migraine revisited. Neurology 2015;85;e28.

Cluster Headache

Cluster headache, a trigeminal autonomic cephalgia, is a syndrome involving unilateral head pain associated with autonomic symptoms. The diagnosis is clinical. The pathophysiology of cluster headache is unknown. It is believed to involve the trigeminal nerve and ganglion, with autonomic dysfunction and vascular irritability. Initial treatment is with parenteral triptans and inhaled oxygen. Preventive agents include topiramate, verapamil, and lithium. Occipital nerve blocks and stimulation have been effective in small studies. Surgery is limited to those patients that have persistent, chronic cluster headache with a minimum of three attacks per week, despite treatment with at least three preventative agents. Deep brain stimulation of the posterior hypothalamus has been shown to be effective in the treatment of chronic cluster headache.

Acupuncture for Episodic Cluster Headache: A Trigeminal Approach

Following evidence that acupuncture is clinically feasible and cost-effective in the treatment of headache, the UK National Institute for Health and Care Excellence recommends acupuncture as prophylactic treatment for migraine and tension headache. There has thus been expectation that other forms of headache should benefit also. Unfortunately, acupuncture has not generally been successful for cluster headache. This may be due to acupuncturists approaching the problem as one of severe migraine. In fact, cluster headache is classed as a trigeminal autonomic cephalgia. In this case report, episodic cluster headache is treated in the same way as has been shown effective for trigeminal neuralgia. Acupuncture is applied to the contralateral side at points appropriate for stimulating branches of the trigeminal nerve. Thus, ST2 is used for the infraorbital nerve, BL2 and Yuyao for the supratrochlear and supraorbital nerves, and Taiyang for the temporal branch of the zygomatic nerve.