Paroxysmal headache with extracephalic irradiation: Proposal for a new variant of epicrania fugax in a series of five patients

Background Epicrania fugax consists of brief paroxysms of pain, which radiate in a line or in zigzag trajectory across the surface of the scalp or the face. Methods A prospective, descriptive study was performed in five patients presenting with an epicrania fugax-type pain with extracephalic irradiation. Results All patients were women, and the mean age at onset was 59.8 (standard deviation, 10.9). They had unilateral paroxysms of electrical pain starting at a particular point in the head (parietal, n = 3; vertex, n = 1; frontal, n = 1) and rapidly radiating downwards in a lineal trajectory to reach extracephalic regions (ipsilateral limbs, n = 2; shoulder, n = 2; low neck, n = 1) in 1–3 seconds. Pain intensity was moderate or severe. Three patients had nummular headache at the point where the paroxysms originated. One patient had spontaneous remission, and four patients achieved complete or almost complete response with therapy (onabotulinumtoxinA, n = 2; indomethacin, n = 1; amitriptyline, n = 1; lamotrigine, n = 1). Conclusion The spectrum of epicrania fugax may include paroxysms with extracephalic irradiation. The propagation of pain beyond the head and the face supports the involvement of central mechanisms in the pathophysiology of this entity.

Some rare headache disorders, including Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, Harlequin syndrome, lacrimal neuralgia, neck–tongue syndrome, and red ear syndrome

A fascinating array of rare headache disorders—Alice in Wonderland syndrome, blip syndrome, cardiac cephalalgia, epicrania fugax, exploding head syndrome, harlequin syndrome, lacrimal headache, ictal epileptic headache, neck–tongue syndrome, and red ear syndrome—are reviewed in this chapter. Appropriate diagnosis and treatment as appropriate can be most reassuring to the patient. There are additional rare headache disorders just waiting to be described by astute clinicians.

Epicrania fugax: Report of eight cases with some novel features

Introduction: Epicrania fugax (EF) is an epicranial headache syndrome characterized by short lasting headache paroxysms which spread in a typical linear/zigzag pattern. The definition of EF has evolved over time, and new phenotypes have been observed in various studies. Aim: We present eight cases diagnosed as EF according to the International Classification of Headaches Disorders, 3rd edition criteria and highlight certain novel characteristics which will help in the further categorization of this disorder. Methods: We prospectively studied eight cases of EF who presented to our headache clinic from January 2016 to September 2020. Demographic and detailed clinical data were collected and analyzed. Results: The mean age of onset of the cohort was 42 ± 10.47 years with a median duration of symptoms being 1 year. There were 5 male and 3 female patients. The mean visual analog scale score was 5.25 ± 1.83. Headache frequency had high intra and interindividual variability. Four patients had a typical spread of pain along the distribution of greater occipital, and supraorbital, nerves. Four patients had an atypical presentation with midline headache, transverse occipital, and bilateral simultaneous hemicranium involvement. Headache paroxysm was followed by a prolonged fixed pain in the same linear distribution in three patients. One patient had ipsilateral redness in the eye. All eight patients required medical intervention with amitriptyline or neuromodulators, five of whom reported a significant reduction in their headache symptoms. Conclusion: Our case series highlights a few interesting features which need further exploration with larger studies. Firstly, the atypical distribution of the pain trajectory forces us to dig deeper into the pathogenesis of the disorder. Secondly, our data also suggest a possibility of EF triggered “linear headaches.” EF is a relatively uncommon headache disorder that can be managed easily with neuromodulators but the lack of awareness of the condition often leads to delayed diagnosis.

Epicrania Fugax as the presenting symptom of a cerebellar abscess

Background Epicrania fugax is included in the appendix of the International Classification of Headache Disorders and is characterized as recurrent brief attacks of linear or zigzag pain moving across the cranial surface, commencing and terminating in the distribution of different nerves. We present a new case of epicrania fugax in which the headache was the presenting symptom of a cerebellar abscess. Case report We present a 58-year-old woman with prior history of Chiari I malformation who underwent suboccipital craniectomy. Two weeks after surgery, she experienced paroxysmal pain episodes of 1–3 seconds, with constant linear trajectory from the right occipital surface to the right orbital region, remaining pain free between episodes. Cranial tomography showed a hypodense intraaxial lesion in the right cerebellar hemisphere. Magnetic Resonance Imaging exhibited intralesional bleeding and peripheral enhancement after gadolinium administration. Post-surgical cerebellar abscess was diagnosed and antibiotic therapy was started; the patient underwent urgent surgical drainage. Pain disappeared after the surgery and the patient remains pain free with 12 months of follow-up. Conclusion Posterior fossa abnormalities have been described as a possible cause of secondary epicrania fugax. The presence of red flags should encourage conducting of paraclinical tests to rule out a symptomatic form.

Clinical Features and Psychiatric Comorbidity of Epicrania Fugax

ABSTRACT Background: Epicrania fugax (EF) is a rare newly described primary headache characterized by paroxysms of unilateral pain radiating across one hemicranium. Aim: We aimed to describe 10 new cases of EF and assess the psychiatric comorbidity. Materials and Methods: Cases of EF were identified from patients attending the neurology outpatient department of a tertiary level referral and teaching hospital by the first author during a period extending from January 1, 2015 to April 31, 2017. Case ascertainment was done as per ICHD 3 beta criteria from among patients presenting with complaints of headache after detailed history and clinical examination. Clinical and demographic features were noted and patients were subjected to Mini Neuropsychiatric Interview to screen for psychiatric comorbidity followed by Becks Anxiety/Depression Inventory. Results: A total of 10 subjects were obtained during the study period, 4 males, and 6 females. Mean age of subjects was 45.3 years (standard deviation-10). Seventy percent had anteroposterior, and 30% had posteroanterior radiation of pain. The most common character of pain was stabbing (50%) followed by electrical (40%) and pressing (10%). None of the subjects had autonomic symptoms or focal symptoms in the scalp while 30% subjects had hyperesthesia in the affected area of the scalp. Six subjects (60%) patients had episodic course while 40% had chronic course. Sixty percent had comorbid anxiety while one (10%) had comorbid depression. A significant relation was obtained between duration of disease and occurrence of anxiety as well as Becks Anxiety Inventory scores while there was no correlation with attack duration. There was also a nonsignificant correlation between visual analog score and occurrence of anxiety symptoms. Conclusions: Our study conclusively proves the existence of EF as a rare, distinct primary headache syndrome in our study population. It has a significant psychiatric comorbidity consisting of 60% of generalized anxiety disorder, 10% of panic attacks, and 10% of depression.

Case report: Wallenberg’s syndrome, a possible cause of symptomatic epicrania fugax

Background Epicrania fugax has been described as a primary headache. Nevertheless, a symptomatic form was recently found in a patient with a skull base meningioma abutting the trigeminal nerve. Here we report on a patient with facial pain with the features of epicrania fugax occurring after Wallenberg’s syndrome. Case report A 53-year-old man suffered a right-sided dorsolateral medullary ischaemic stroke. Nine months later, he presented with brief electric shock-like paroxysms of pain stemming from the right eye and radiating to the ipsilateral forehead, the temple or the cheek in a zigzag trajectory. Some episodes were accompanied by ipsilateral conjunctival injection and eyelid oedema. Treatment with eslicarbazepine abolished the pain. Conclusion A pain with the features of epicrania fugax may be associated with medullary lesions. This finding suggests that the central trigeminal pathways and/or the spinal trigeminal nucleus may play an important role in the pathophysiology of this type of pain.