Epidaily, a scale for comprehensive functional assessment of patients with epilepsy.

OBJECTIVE: To develop and validate an epilepsy-specific scale for comprehensive functional assessment of patients with epilepsy, named Epidaily. METHODS: The multidisciplinary research group created through brainstorming a list of 47 items to explore the cognitive, social, basic and instrumental functionality of the patient. A group of epilepsy experts independent of the research group evaluated the suitability of all the items, which then were selected and reviewed by the research group to conform the Epidaily scale. On a sample of 102 patients, a reliability analysis was performed, as well as a validation one using as reference scale the score on the Activities of Daily Living Questionnaire (ADLQ), which evaluates basic and instrumental functionality. RESULTS: Epidaily consisted of 10 items distributed in four dimensions, with a possible score from 0 to 100 (perfect functionality). Inter-observer reliability was excellent, with an intraclass correlation coefficient of 0.98 (95% confidence interval 0.97-0.99). Criterion validity was demonstrated by the high positive correlation of the Epidaily score with the ADLQ score (Spearman's rho coefficient 0.85, p < 0.001). Significant relation was found between ADLQ and Epidaily in the linear regression analysis (p < 001), which reported that Epidaily explains 85.5% of the variability of ADLQ (R-squared 0.85). Discriminant validity was also proved, as Epidaily allowed to classify epilepsy severity based on Cramer et al epilepsy severity classification. The median time to obtain the Epidaily score was 5 min (interquartile range 4-6). SIGNIFICANCE: Epidaily is a brief and versatile scale, with excellent inter-observer reliability, which has been validated for comprehensive functional assessment of patients with epilepsy.

Therapy delay in status epilepticus extends its duration and worsens its prognosis.

BACKGROUND: Timing in status epilepticus (SE) attention is probably the most relevant modifiable prognostic factor and may influence SE duration and prognosis. We aimed to describe the precise relationship between management timing, duration, and prognosis of SE. METHODS: Observational longitudinal prospective study on a cohort of all patients diagnosed with SE admitted to our tertiary hospital from September 2017 to August 2019, with a 3-month follow-up. Univariate and multivariable analyses were performed to identify clinical and timing variables associated with SE duration and prognosis. RESULTS: Eighty-three SE affecting 76 patients were included. Median age was 73 years, 61.4% were women, median baseline modified Rankin Scale (mRS) was 2, and 55.4% had prior epilepsy. In the out-of-hospital group (n = 50), median time to emergencies was 1.3 h and to hospital admission 2.8 h. In the global series, median time to neurologist was 4.3 h, and median time to therapy initiation was 4.5 h. These four times positively correlated with SE duration (all Spearman's rho coefficient >0.5, all p < .001). SE median duration was 24 h and was extended 1.2 h for each hour of treatment delay. A longer SE duration was associated with increased mortality and morbidity, both at hospital discharge and at 3-month follow-up (both p < .05). After 3 months, mortality was 30.1%, while recovery to baseline mRS occurred in 39.5%, with an overall median mRS of 4. CONCLUSIONS: There were pervasive delays in all phases of SE attention, which conditioned a longer SE duration, and this led to increased long-term morbimortality.

Non-Convulsive Status Epilepticus in Behavioral Variant Frontotemporal Dementia.

Epilepsy in frontotemporal dementia is considered to be less frequent than in Alzheimer's disease. We report two cases of patients with non-convulsive status epilepticus associated with behavioral variant frontotemporal dementia. In the first case, status epilepticus was the first symptom of the disease, and consisted of loss of consciousness and mutism. In the second case, status epilepticus led to a clinical worsening one year after the diagnosis. Our study highlights the importance of suspecting non-convulsive status epilepticus in patients with frontotemporal dementia, and including frontotemporal dementia within the differential diagnosis of new-onset seizures.

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.

Patients' knowledge on epilepsy and SUDEP improves after a semi-structured health interview.

BACKGROUND: Patients' education is the most relevant contributor to patient self-management of epilepsy. We aimed to assess the acquisition of knowledge after a semi-structured interview. METHODS: We performed a quasi-experimental prospective study with a cohort of patients with epilepsy admitted for prolonged video electroencephalogram (VEEG). We measured patients' baseline knowledge with a 10-item true-false test (test A). Then, a qualified nurse carried out a semi-structured interview. We measured acquired knowledge with another 10-item true-false exam (test B), prior to VEEG discharge and after a 3- to 5-month follow-up, and we compared the difference between the scores in test A and test B. Finally, we conducted a satisfaction and suitability survey on the interview at follow-up. RESULTS: Thirty-two patients participated, half were women. Their median age was 39.5, and the median length of schooling was 14 years. The median time since epilepsy onset was 13 years, 75% had suffered tonic-clonic seizures. The median score on test A was 7, while the median score on test B was 8.5 (p < 0.001) both at VEEG discharge and after follow-up. After the interview, 84.4% of participants reported that they were very satisfied with the information received; 87.5% stated that they had not previously heard about SUDEP (sudden unexpected dead in epilepsy); and 93.8% considered it important to receive detailed information about SUDEP. CONCLUSIONS: Patient education through a semi-structured comprehensive interview improves knowledge of patients with epilepsy about their disease. The calm atmosphere and the qualified nursing working at VEEG units make them an appropriate setting for talking about epilepsy and its risks, including SUDEP.

Use of intravenous brivaracetam in status epilepticus: A multicenter registry.

OBJECTIVE: The pharmacokinetics of brivaracetam (BRV), added to its effectiveness observed in animal models of status epilepticus (SE), makes this drug attractive for use in emergency situations. Our objective was to evaluate the use of intravenous BRV in a multicenter study. METHODS: A retrospective multicenter registry of SE cases treated with BRV was created. These patients were evaluated between January and December 2018 at seven hospitals in Spain. Demographic variables, SE characteristics, concomitant drugs, loading doses, and response to treatment were collected. RESULTS: Forty-three patients were registered. The mean age was 56 ± 23.1 years, 51.2% were male, 29 had previous epilepsy, 24 (55.8%) had prominent motor symptoms, and 19 had nonconvulsive symptoms. Regarding the etiology, 19 (44.2%) were considered acute symptomatic, 16 (17.2%) remote symptomatic, four (9.3%) progressive symptomatic, and four (9.3%) cryptogenic. Regarding concomitant antiepileptic drugs (AEDs), 17 had previously received levetiracetam (LEV). In 14 patients, BRV was used early (first or second AED). The median loading dose was 100 mg (range = 50-400), and the weight-adjusted dose was 1.8 mg/kg (range = 0.4-7.3). BRV was effective in 54% (n = 23), and a response was observed in <6 hours in 13 patients. We observed a tendency for it to be more effective when administered earlier (P = 0.09), but there were no differences regarding SE type and the concomitant use of LEV. In those with the fastest responses, we observed that both the total administered dose (300 mg vs 100 mg, P = 0.008) and the weight-adjusted dose (3.85 mg vs 1.43 mg, P = 0.006) were significantly higher. The receiver operating characteristic curve showed that the best cutoff point for a faster response was 1.82 mg/kg. SIGNIFICANCE: BRV is useful for the treatment of SE, even when patients are already being treated with LEV. The response rate seems higher when it is administered earlier and at higher doses (>1.82 mg/kg).

Cost of status epilepticus (SE): Effects of delayed treatment and SE duration.

BACKGROUND: The health expenditure related to status epilepticus (SE) is high because of lengthy hospitalization requirements and possible sequelae. We aimed to study the factors associated with this cost including the different timings of the treatment and SE duration. METHODS: We evaluated retrospectively all SE recorded in 2 hospitals. The factors studied included the mean cost of hospitalization, demographics, clinical data, duration of hospitalization, in-hospital/out-of-hospital debut, time from onset to treatment, duration of SE, and destination at discharge. RESULTS: Three hundred five patients were evaluated (December/2012-July/2017), 195 with out-of hospital and 110 with in-hospital debut. The cost of SE with out-of-hospital onset was significantly lower (6559€ vs 15,174€; p = 0.0001). In out-of-hospital cases, the factors independently related to expenditure were the level of consciousness (p < 0.001), presence of complications (p = 0.005), a potentially fatal etiology (p = 0.008), and duration of the episode (p = 0.003). Duration was significantly higher in patients discharged to a convalescence center (p = 0.006); this variable was significantly related to the time SE onset-arrival to hospital, and SE onset-administration of the treatment. In the in-hospital cases, cost was related to male sex (p = 0.002), the development of complications (p = 0.003), and the etiology (p = 0.016) but was not directly related to the SE duration or to the time onset-treatment. CONCLUSIONS: The duration of SE and the speed with which proper management is applied have a direct impact on the healthcare expenditure resulting from out-of-hospital SE. In contrast, the etiology and development of complications are the main factors responsible for expenditure related to in-hospital SE.

OnabotulinumtoxinA injections for atypical odontalgia: an open-label study on nine patients.

BACKGROUND: Atypical odontalgia (AO) manifests as continuous pain in the region of one or several teeth, in the absence of signs of dental pathology. Currently, there is insufficient evidence to establish treatment guidelines for AO. The aim of this study was to describe the effectiveness and safety of treatment with OnabotulinumtoxinA (OnabotA) on a series of patients with AO. METHODS: Nine patients with AO (four males and five females, aged between 31 and 77 years) received injections of OnabotA in the region of pain. The dosage used in each procedure ranged between 10 and 30 U, spread between 4 and 12 injection sites along the gums (n=9), the lips (n=3), and the hard palate (n=1). The median follow-up time was 27 months (interquartile range, IQR 20-40) and the median number of injection sessions per patient was seven (IQR 4.5-9). The assessment variables included the change in the maximal intensity of pain on a 0-10 numerical rating scale (NRS), the response latency, and the duration of the effect. RESULTS: All patients experienced a significant improvement, with ≥50% of reduction in the intensity of the maximal pain. The median of reduction of maximal pain after treatment was six points on the NRS (IQR 5-8.5). The response latency was 2-15 days and the duration of the effect was 2-6 months. No significant adverse reactions were registered. CONCLUSION: OnabotA may be a safe and effective option for the treatment of AO.

The relevance of timing in nonconvulsive status epilepticus: A series of 38 cases.

BACKGROUND: Timing in the management of nonconvulsive status epilepticus (NCSE) seems to be one of the most important modifiable prognostic factors. We aimed to determine the precise relationship between timing in NCSE management and its outcome. METHODS: We performed a cross-sectional study in which clinical data were prospectively obtained from all consecutive adults with NCSE admitted to our hospital from 2014 to 2016. Univariate and multivariable regression analyses were performed to identify clinical and timing variables associated with NCSE prognosis. RESULTS: Among 38 NCSE cases, 59.9% were women, and 39.5% had prior epilepsy history. The median time to treatment (TTT) initiation and the median time to assessment by a neurologist (TTN) were 5h, and the median time to first electroencephalography assessment was 18.5h; in the cases with out-of-hospital onset (n=24), the median time to hospital (TTH) arrival was 2.8h. The median time to NCSE control (TTC) was 16.5h, and it positively correlated with both the TTH (Spearman's rho: 0.439) and the TTT (Spearman's rho: 0.683). In the multivariable regression analyses, the TTC was extended 1.7h for each hour of hospital arrival delay (p=0.01) and 2.7h for each hour of treatment delay (p<0.001). Recognition delay was more common in the episodes with in-hospital onset, which also had longer TTN and TTC, and increased morbidity. CONCLUSIONS: There were pervasive delays in all phases of NCSE management. Delays in hospital arrival or treatment initiation may result in prolonged TTC. Recognition of in-hospital episodes may be more delayed, which may lead to poorer prognosis in these cases.

Infratrochlear neuralgia: A prospective series of seven patients treated with infratrochlear nerve blocks.

Background Infratrochlear neuralgia is a recently described painful cranial neuropathy that causes pain in the internal angle of the orbit and the medial upper eyelid, the upper bridge of the nose and/or the lacrimal caruncle. We aim to present seven new cases of infratrochlear neuralgia treated with anaesthetic nerve blocks. Methods Over an 18-month period, we prospectively identified seven cases of infratrochlear neuralgia among the patients attending the Headache Unit in a tertiary hospital. Anaesthetic blocks were performed by injecting 0.5 cc of bupivacaine 0.5% at the emergence of the nerve above the internal canthus. Results All patients were women, and the mean age was 49.1 years (standard deviation, 17.9). The pain appeared at the internal angle of the orbit and/or the medial upper eyelid in six cases, and the whole territory of the infratrochlear nerve in one case. Six patients had continuous pain and one had episodes lasting 8-24 hours. All patients showed sensory disturbances within the painful area and tenderness upon palpation of the infratrochlear nerve. Nerve blocks resulted in complete and long-lasting relief in four patients and short-lasting relief in the other three patients. Conclusions Infratrochlear neuralgia should be considered among the neuralgic causes of orbital and periorbital pain. Anaesthetic blocks may assist clinicians in the diagnosis and may also be an effective therapy.

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.

Supratrochlear Neuralgia: A Prospective Case Series of 15 Patients.

OBJECTIVE: The aim of this study was to describe clinical features unique to supratrochlear neuralgia. BACKGROUND: The supratrochlear nerve supplies the medial aspect of the forehead. Due to the intricate relationship between supraorbital and supratrochlear nerves, neuralgic pain in this region has been traditionally attributed to supraorbital neuralgia. No cases of supratrochlear neuralgia have been reported so far. METHODS: From 2009 through 2016, we prospectively recruited patients with pain confined to the territory of the supratrochlear nerve. RESULTS: Fifteen patients (13 women, 2 men; mean age 51.4 years, standard deviation 14.9) presented with pain in the lower paramedian forehead, extending to the eyebrow in two patients and to the internal angle of the orbit in another. Pain was unilateral in 11 patients (six on the right, five on the left), and bilateral in four. Six patients had continuous pain and nine described intermittent pain. Palpation of the supratrochlear nerve at the medial third of the supraorbital rim resulted in hypersensitivity in all cases. All but one patient exhibited sensory disturbances within the painful area. Fourteen patients underwent anesthetic blockades of the supratrochlear nerve, with immediate relief in all cases and long-term remission in three. Six of them had received unsuccessful anesthetic blocks of the supraorbital nerve. Five patients were treated successfully with oral drugs and one patient was treated with radiofrequency. CONCLUSIONS: Supratrochlear neuralgia is an uncommon disorder causing pain in the medial region of the forehead. It may be differentiated from supraorbital neuralgia and other similar headaches and neuralgias based on the topography of the pain and the response to anesthetic blockade.

Epicrania fugax combining forward and backward paroxysms in the same patient: the first four cases.

BACKGROUND: The first description of epicrania fugax (EF) reported brief painful paroxysms that start in posterior regions of the scalp and move forward to reach the ipsilateral forehead, eye, or nose. A backward variation, wherein pain stems from frontal areas and radiates to the posterior scalp, has also been acknowledged. We report four patients with features reminiscent of EF and the coexistence of forward and backward pain paroxysms. METHODS: We considered all patients attending the headache outpatient office at two tertiary hospitals from March 2008 to March 2016. We enrolled four patients with paroxysms fulfilling criteria for EF and a combination of forward and backward radiations. RESULTS: In all cases, pain paroxysms moved both in forward and backward directions with either a zigzag (n=2) or linear (n=2) trajectory. Three patients presented two stemming points, in the occipital scalp and forehead (n=2) or in the parietal area and eye (n=1), whereas the fourth patient only had a stemming point located in the parietal region. Pain quality was mainly stabbing, and its intensity was moderate (n=1) or severe (n=3). The duration of the paroxysms was highly variable (3-30 seconds), and two patients reported autonomic symptoms. CONCLUSION: The clinical picture presented by our patients does not fit with other types of known headache or neuralgia syndromes; we propose it corresponds to a bidirectional variant of EF.

Greater occipital nerve block for the acute treatment of prolonged or persistent migraine aura.

Background Presently, there is no evidence to guide the acute treatment of migraine aura. We aimed to describe the effect of greater occipital nerve (GON) anaesthetic block as a symptomatic treatment for long-lasting (prolonged or persistent) migraine aura. Methods Patients who presented with migraine aura lasting > 2 hours were consecutively recruited during one year at the Headache Unit and the Emergency Department of a tertiary hospital. All patients underwent a bilateral GON block with bupivacaine 0.5%. Patients were followed up for 24 hours. Results A total of 22 auras were treated in 18 patients. Auras consisted of visual ( n = 13), visual and sensory ( n = 4) or sensory symptoms alone ( n = 5). Eleven episodes met diagnostic criteria for persistent aura (>1 week) without infarction. The response was complete without early recurrence in 11 cases (50%), complete with recurrence in < 24 hours in two cases (9.1%), and partial with ≥ 50% improvement in six cases (27.3%). Complete responses without recurrence were more common in cases with prolonged auras lasting < 1 week than in those with persistent auras (72.7% vs. 27.3%; p = 0.033). Conclusions GON block could be an effective symptomatic treatment for prolonged or persistent migraine aura. Randomised controlled trials are still required to confirm these results.

Facial epicrania fugax: A prospective series of eight new cases.

Introduction Epicrania fugax (EF) is a primary headache whose main feature is the dynamic character of the pain. EF pain typically moves across different dermatomes in a linear or zigzag trajectory. Recently, a facial variant of EF has been described, with the pain starting in the lower face and radiating upwards. Aim We report eight patients with an EF-type of pain of facial location and either upward or downward radiation. Methods For each patient, we recorded relevant demographic and clinical data. Magnetic resonance imaging (MRI) with fast imaging employing steady state acquisition (FIESTA) was obtained in all cases for the assessment of neurovascular compression of the trigeminal nerve. Results There were seven women and one man, and the mean age was 76.1 years (standard deviation, 11.3). Six patients had a paroxysmal pain starting at the lower face and moving upwards, while two patients had downward radiation. The pain always followed a fixed linear trajectory across different dermatomes. All cases had triggers, and pain intensity was consistently severe. Half of the patients had accompanying autonomic features. Neurovascular compression with imprinting over the trigeminal root on the symptomatic side was identified in three patients. All cases responded to antiepileptic drugs, and three had spontaneous remissions. Conclusions This series reinforces the facial variant of EF and extends the phenotype with cases of downward radiation. It also contributes to enriching the differential diagnosis of facial pain. Neurovascular compression of the trigeminal nerve may be found in some cases, although a possible pathogenic link needs further research.

Lacrimal Nerve Blocks for Three New Cases of Lacrimal Neuralgia.

OBJECTIVE: Our aim was to report three new cases of lacrimal neuralgia and their response to superficial nerve blocks. BACKGROUND: Lacrimal neuralgia has been recently described as a pain in the territory supplied by the lacrimal nerve, at the lateral upper eyelid and/or the adjacent area of the temple. The pain is typically accompanied by tenderness on palpation of the lacrimal nerve at the superoexternal angle of the orbit. METHODS: Between January 2015 and June 2016, we prospective identified three cases of lacrimal neuralgia among the patients attending the Headache Unit in a tertiary hospital. Anesthetic blocks were performed in all cases by inserting a 30-G needle on the emergence of the nerve and injecting 0.5 cc of bupivacaine 0.5% subcutaneously. RESULTS: Three women aged 44, 49, and 51 presented with pain in the territory supplied by the lacrimal nerve. Two of them localized their pain in a small area of the right temple, while the remaining patient had pain in the right upper lateral eyelid and a small area of the lower lateral eyelid. The pain was continuous in two patients and episodic with attacks lasting 48 hours in one patient. All patients had tenderness on palpation of the lacrimal nerve. Anesthetic blocks confirmed the diagnosis of lacrimal neuralgia and provided the patients with long-lasting pain relief. CONCLUSIONS: Lacrimal neuralgia should be considered among the neuralgic causes of orbital and periorbital pain. Superficial nerve blocks may assist clinicians in the diagnosis and may also be a therapeutic option.

Living With Cluster Headache: A Qualitative Study of Patients' Perspectives.

OBJECTIVE: Our aim was to explore the views and experiences of a group of Spanish men suffering from cluster headache (CH). BACKGROUND: CH has considerable effects on patients' quality of life, impairs everyday activities, and can modify lifestyle. This is the first time the experience of patients with CH is examined in a clinical study using a qualitative, phenomenological approach. METHODS: We conducted a qualitative phenomenological study exploring how 20 male patients with CH, followed at the Headache Unit of a Spanish hospital, perceived their disease. Data were collected through in-depth interviews, researchers' field notes and patients' personal letters. A systematic text condensation analysis was performed following appropriate guidelines for qualitative research. RESULTS: Mean age was 41.15 years (standard deviation, 11.25). Seventeen patients had episodic CH and three patients had chronic CH. Five main themes describing the significance of suffering CH emerged: (a) meaning of disease, (b) experience of attacks, (c) meaning of treatment, (d) healthcare, and (e) social and family interaction. Patients with CH often live in fear and uncertainty because of their condition. Intensity and frequency of attacks, the use of ineffective treatments, skepticism perceived from social and workplace environments and physician unawareness play a significant role. CONCLUSIONS: Qualitative research offers insight into the way CH patients experience their disease, and may be helpful in establishing a fruitful relationship with these patients.

Pain Paroxysms With Coronal Radiation: Case Series and Proposal of a New Variant of Epicrania Fugax.

OBJECTIVE: We aim to report 4 patients with brief pain paroxysms whose clinical features remind of typical epicrania fugax (EF), except for the direction along a transverse trajectory. BACKGROUND: EF has been defined as a brief paroxysmal head pain, with stabbing quality, describing a linear or zig-zag trajectory across the surface of one hemicranium. METHODS: We considered all patients attending the headache outpatient office at 3 tertiary hospitals from March 2008 to December 2015. We identified a total of 4 cases with dynamic pain paroxysms moving in coronal direction. RESULTS: The mean age at onset was 49 years (range 34-75). All the patients suffered a dynamic pain from parietal or temporal areas to the contralateral ones, crossing the midline in a linear or zig zag trajectory, the entire sequence lasting 2-3 seconds. No triggers were identified. One patient noticed mild interictal tenderness at the stemming point. The frequency of the attacks varied from 2 per week to 1 every 2 months. No underlying disorders were identified by physical and neurological exams and neuroimaging and laboratory tests. CONCLUSIONS: Our patients presented with a paroxysmal head pain that might correspond to a transverse variant of EF. These observations may not only expand the EF phenotype but also reinforce the distinction between EF and pericranial neuralgias.