Abstract
Background
Epicrania fugax is a brief paroxysmal severe stabbing pain that travels on scalp in a straight/zig-zag line. There are no known red-flags against primary epicrania fugax.
Methods
We identified four patients with secondary epicrania fugax characteristically triggered by head/neck movement.
Results
Four patients (age range 25–65 years, 2 females) with epicrania fugax were secondary to (a) acute subdural hematoma, (b) C1, C2 vertebral expansile metastasis (adenocarcinoma of prostrate), (c) type 1 Chiari malformation with cervical syrinx, and (d) pontine/cerebellar tuberculoma. All patients had severe pain (visual analog score 8–9/10) triggered by head/neck movement or on assuming upright posture. Patients with subdural hematoma and tuberculoma improved with dexamethasone. One with Chiari malformation showed partial response to gabapentin and patient with metastatic disease did not improve despite local radiotherapy, hormone treatment, gabapentin, dexamethasone and tapentadol.
Conclusions
Epicrania fugax triggered by head/neck movement should be evaluated for an underlying structural etiology.
Keywords
Introduction
Epicranial headaches are headaches perceived on the surface of the scalp. They include primary stabbing headache (PSH), nummular headache (NH) and epicrania fugax (EF). EF is a primary headache disorder characterized by brief paroxysms of pain that runs across the scalp. 1 EF is included in the appendix of the third edition of International Classification of Headache Disorders (ICHD-3) and is diagnosed by (a) recurrent stabbing head pain attacks lasting 1–10 s (b) pain moving with a linear or zig-zag trajectory across the surface of one hemicranium, commencing and terminating in the distributions of different nerves (c) not better accounted for by another ICHD-3 diagnosis. 2 In recent years, a few case reports of EF secondary to a structural lesion have been published. There are no known clinical features that could differentiate primary from secondary EF. Herein we report four such cases of EF having either a striking trigger of headache by head/neck movement or change in body position.
Cases
The clinical, radiological features, diagnosis, treatment offered and outcome of the four cases are given in Table 1. A written informed consent for patient information and images to be published was provided by the patient(s) or a legally authorized representative.
Clinical, radiological, treatment and outcome details of the patients with secondary EF.
Case 1
A 63-year-old man presented with intermittent bouts of severe [visual analog scale (VAS) 9/10] sharp electric current like pain that started from right occipital area and travelled in a straight line towards the right frontal region for 2 days. The headache was associated with redness and watering of right eye and vomiting. The headache occurred each time with movement of neck in any direction, and it was worse on sitting from supine position. Patient had a minor head trauma 2 days prior to the onset of headache. He had cluster headache (in remission for 2 years) for last 7 years and hypertension. Except for neck rigidity, his neurological examination was normal. Unlike his cluster headache episode that improved with oxygen, these episodes improved spontaneously within a few (8–10) s. His cranial MRI showed right parietal subdural hematoma with mild mass effect (Figure 1A). Neurosurgeon advised conservative treatment and close observation for any deterioration. His headache responded within 3 days of oral dexamethasone that was started with a possibility of chemical meningitis. At 1 month, he remained free of headache.
(A) T2-weighted MRI axial image showing acute right subdural hematoma (arrow) with mild mass effect, (inset) cartoon showing posteroanterior direction of linear pain, (B) T2-weighted MRI sagittal image of cervical spine showing an expansile mass in C1, C2 vertebrae, displaced cervical spinal cord and cervical canal narrowing, (inset) cartoon showing bilateral (right > left) posteroanterior direction of linear pain, (C) T2-weighted sagittal MRI of cervical spine showing cerebellar tonsillar herniation, posterior fossa crowding with cervical syrinx formation, (inset) cartoon showing bilateral (right > left) posteroanterior direction of linear pain, (D) T1 post-contrast sagittal MRI showing multiple conglomerate ring-enhancing lesions involving pons and cerebellum, (inset) cartoon showing transverse right to left direction of linear pain.
Case 2
A 65-year-old man, presented with neck/shoulder pain and dull aching right hemicranial headache for 6 months. For the past 3 months, he also had intermittent transient stabbing type of pain bilaterally (right > left) travelling in a straight line from cervical to frontal region with a VAS of 9/10 and without any trigeminal autonomic symptoms. These episodes were triggered with neck movement/rotation and lasted for 3–5 s. He had anorexia and weight loss of 10 kg over past 6 months. There was tenderness on palpation of the neck and scalp. His MRI of cervical spine showed expansile lesion at C1–C2 vertebrae with paravertebral soft tissue (Figure 1B). Biopsy from the vertebral lesion was suggestive of metastatic adenocarcinoma and trans-rectal ultrasound guided biopsy from the prostate showed acinar adenocarcinoma. Despite hormonal treatment and radiotherapy, he continued to have brief episodes of EF with neck movements.
Case 3
A 31-year-old lady, presented with transient short-lasting headaches for 1 year, vomiting for 3 months, and imbalance while walking for 1.5 months. The headache episodes were severe (VAS 8/10) stabbing in nature, originated at the neck, and spread to forehead in a straight line (right > left). These headaches occurred on sitting from lying down, lasted for 5–10 s. She remained headache free between the episodes. She also had difficulty in navigating through narrow passages. Examination revealed gaze-evoked nystagmus, spasticity in all 4 limbs, brisk deep tendon reflexes, right plantar extensor, dysdiadochokinesia and impaired finger nose test on right side. MRI brain and cervical spine showed cerebellar tonsillar herniation with syrinx in the cervical spinal cord and dilated 4th ventricle; features suggestive of Chiari malformation 1 (Figure 1C). Patient chose conservative treatment over surgical repair. With the use of cervical collar and gabapentin her headaches and vomiting showed partial improvement.
Case 4
A 25-year-old female, presented with continuous right frontotemporal headache with intermittent sharp severe (VAS 8/10) linear headache travelling from right temporal to left temporal region, lasting 10–12 s and triggered by forward neck bending. She was adequately treated for 2 years with anti-tubercular treatment for tuberculous meningitis 5 years ago. Except for meningeal signs, her examination was normal. Her cranial MRI showed multiple conglomerated ring-enhancing lesions with perilesional edema in right side of midbrain, pons extending into the right cerebellar hemisphere with mild hydrocephalus (Figure 1D). She was started on 4 drug antitubercular treatment and dexamethasone. Her headache resolved completely within three days of initiating treatment. At 12 months follow-up, she was asymptomatic.
Discussion
EF is a dynamic headache that travels between two points on the scalp in a straight/zig-zag line in anteroposterior or posteroanterior direction. 3 Some patients have interictal pain like that of NH at the site of origin of linear pain. 4 Atypical variants of EF are also known that show a transverse trajectory, facial variant (either upward or downward with/without cranial extension) and EF with extracephalic radiation.5–7 One-fourth patients with EF are either side changing or bilateral and 30% may have ipsilateral autonomic features.4,8
The most identified triggers for EF are physical activity, neck movements, emotional stress and touch at the origin of the pain. 8 All our patients had their EF attacks triggered by head or neck movement or assuming the upright posture; all of them had an underlying structural lesion.
The first symptomatic EF was reported by Fernández-Matarrubia et al., in an elderly woman with ipsilateral anteroposterior EF. She had an underlying sphenoid wing meningioma, and she did not have any trigger. She had partial response to neuromodulators. 9 Jaimes et al. reported a case of severe ipsilateral anteroposterior EF that developed as a late complication of right lateral medullary infarct secondary to right vertebral artery dissection. 10 García-Azorín et al. presented a case of cerebellar abscess with EF in a 58-year-old woman, who was operated for symptomatic type I Chiari malformation and developed cerebellar abscess 6 weeks after the surgery when she presented with severe posteroanterior EF. Her EF disappeared after surgical evacuation of cerebellar abscess. 11 Three out of 8 patients with facial variant of EF reported by Gutiérrez-Viedma et al. had ipsilateral neurovascular compression of the trigeminal nerve by superior cerebellar artery. 6 A common point to all these cases is an intracranial lesion particularly in the posterior fossa. Two of our patients had posterior fossa lesions, one had cervical spine involvement and one had parietal subdural hematoma.
Recently, a symptomatic posteroanterior EF was reported by Cuadrado et al., in which the epicranial pain paroxysms originated from the surgical scar, which was formed after the excision of the subcutaneous hemangioma. 12
All our patients had their headache attacks triggered by the movement of the head, neck or on assuming an upright position. To our knowledge, this series is the first to describe the association of head/neck movement trigger with a secondary EF.
The pathogenesis of EF is not well known. The unilateral origin of pain, linear travel of pain across the scalp, termination in the territory of other epicranial nerve, response to anesthetic block of nerves and recent case of EF secondary to surgical scalp scar supports peripheral generation of pain in epicranial nerves and its travel across the scalp due to ephaptic transmission.1,12 However, presence of autonomic symptoms, cases of EF secondary to central causes like posterior fossa structural lesions and atypical facial and extracephalic variants supports a hypothesis that there is either a central generation (trigemino-cervical complex) or a central permissive role (hypothalamus) or both for generation of pain in EF.3,9–11 Neck movement might trigger EF due to transient exacerbation of intracranial pressure and stretching of C1–C3 nerve roots with change in head position in patients with intracranial/high cervical space occupying lesions. This transient stretching of C1–C3 nerve roots can result in either peripheral origin of EF or may produce pain due to convergence of C1–C3 fibers with trigeminal nerve sensory afferents to the trigemino-cervical complex. Stretching of C1–C3 nerve fibers or their involvement due to varied causes have been postulated for origin of pain in occipital neuralgia and cervicogenic headaches. 13 However, the association of neck movements with secondary EF and its underlying mechanism needs systematic evaluation in large prospective studies.
There are no randomized controlled trials for the management of EF. 3 Our patients with subdural hematoma and pontine tuberculoma responded to dexamethasone. Patient with Chiari malformation responded to gabapentin and the one with cervical spine metastasis did not respond despite local radiotherapy, hormonal treatment and combination of gabapentin, dexamethasone, and tapentadol. There is anecdotal evidence of effect with lamotrigine, pregabalin, carbamazepine, oxcarbazepine, eslicarbazepine, and lacosamide in patients with EF.3,6,8 Anesthetic blocks of the greater occipital, auriculotemporal or supraorbital nerves and botulinum toxin have been used in drug refractory cases.1,6–8,12
Conclusion
We propose that the EF triggered by neck/head movements or by change in posture should be evaluated for an underlying intracranial space occupying lesion or a high cervical lesion.
Highlights
There are no known red flags for the primary epicrania fugax.
We describe four patients with head/neck movement triggered epicrania fugax.
These patients had subdural hematoma, pontine/cerebellar tuberculoma, cervical spine C1, C2 metastasis from prostrate and Chiari type 1 malformation with cervical syrinx.
We propose that epicrania fugax triggered by head/neck movements may be evaluated for underlying intracranial/high cervical space occupying lesion.
Footnotes
Declaration of conflicting interests
The authors declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
Ethical approval
A written informed consent for patient information and images to be published was provided by the patient(s) or a legally authorized representative.
Funding
The authors received no financial support for the research, authorship, and/or publication of this article.
