Auriculotemporal Neuralgia: Eight New Cases Report.

BACKGROUND: Auriculotemporal neuralgia (ATN) is an infrequent syndrome consisting in strictly unilateral pain in the temporal region associated with nerve tenderness, which can be successfully treated with anesthetic blockade. We analysed clinical characteristics and treatment response in a series of eight patients. METHODS: Series of consecutive patients diagnosed with ATN at Headache Clinics of two university hospitals in Spain. Data on demographic and pain characteristics, as well as response to treatment are presented. RESULTS: Eight patients (seven women). Mean age at onset was 52.8 ± 14.3 years. Pain was strictly unilateral (left-sided in five cases, right-sided in three), and triggered by pressing the preauricular area. Four patients presented background pain, mostly dull in quality, with an intensity of 5.75 ± 1.2 on the verbal analogical scale (VAS). In six, burning exacerbations occurred, ranging from 2 seconds to 30 minutes, with intensity 7.3 ± 1.5 on VAS. Complete relief was achieved with gabapentin in three cases, anaesthetic blockade in three and spontaneously in two. CONCLUSION: ATN is uncommon in headache units. Gabapentin is a good alternative therapeutic option to anesthetic blockade.

Personality traits in patients with cluster headache: a comparison with migraine patients.

BACKGROUND: Cluster headache (CH) has been associated with certain personality traits and lifestyle features, but there are few studies assessing personality profiles in CH. We aimed to analyze personality traits in patients with CH, and to compare them with those found in migraine. METHODS: We included all consecutive patients with CH attending 5 outpatient offices between January and December 2013. Personality traits were evaluated using the Salamanca screening test, a validated inventory assessing 11 personality traits grouped in 3 clusters. We analyzed the test results in this population, and compared them with those of a migraine population previously assessed with the same test. RESULTS: Eighty patients with CH (75 men, 5 women; mean age, 43.2 ± 9.9 years) were recruited. The reference population consisted of 164 migraine patients (30 men, 134 women; mean age 36.4 ± 12.7 years). In CH patients, the most frequent personality traits were anancastic (52.5 %), anxious (47.5 %), histrionic (45 %), schizoid (42.5 %), impulsive (32.5 %) and paranoid (30 %). When compared to migraine patients, paranoid (p < 0.001; χ2 test), and schizoid traits (p = 0.007; χ2 test) were significantly more prevalent in CH patients. In logistic regression analysis the paranoid trait was significantly associated with CH (p = 0.001; OR: 3.27, 95 % CI [1.66-6.43]). CONCLUSION: According to the Salamanca screening test, personality traits included in cluster A (odd or eccentric disorders) are more prevalent in CH patients than in a population of migraineurs. Larger studies are needed to determine whether certain personality traits are related to CH.

Hallucinations and aberrant perceptions are prevalent among the young healthy adult population.

INTRODUCTION: Hallucinations are frequent in clinical practice, with an incidence of up to 38.7% in the general population. We aim to determine the prevalence of hallucinations among healthy young adults in our environment. SUBJECTS AND METHODS: We designed an observational study, using as subjects 3rd to 6th year medical students at the Universidad Complutense de Madrid who complete clinical rotations in the Hospital Clínico San Carlos. After a screening questionnaire, an individual interview was conducted via telephone or e-mail to those students who reported hallucinations. We obtained clinical and epidemiological data through a semi-structured clinical interview performed by a third year neurology resident. RESULTS: N=134 (average age was 22.1 years; 77.6% were women). 74 respondents answered affirmatively to one or more screening questions, and 54 completed the follow-up interview. 22.2% described visual phenomena and 64.8%, auditory. The majority reported sleep-related experiences and auditory perceptions related to hyper vigilance, such as hearing the telephone or the doorbell ring when in fact it had not (38.8%). All subjects had good insight into their experiences and none had psychotic symptoms. Two cases were associated with substance abuse. CONCLUSIONS: Hallucinations are frequent among the general population. Traditionally, auditory phenomena have been associated with psychotic pathology, and other studies show a low population incidence (0.6%). However, in our sample, short auditory perceptions with immediate analysis were frequent and not pathological.

Migraine-triggered hemifacial spasm: three new cases.

BACKGROUND: The occurrence of hemifacial spasm (HFS) during an episode of migraine has been seldom reported. Here we describe three new cases presenting with HFS in association with migraine attacks. CASE RESULTS: Three patients (one woman and two men, aged 31-36 years) developed HFS in close temporal relationship with migraine headaches. All of them started having the muscle spasms after pain onset. Two of them had electromyographic evidence of facial nerve damage, and continued having HFS once the pain abated. CONCLUSIONS: Migraine attacks may be associated with HFS. The appearance of HFS could be related to migraine activity. A mechanism of central hyperexcitability in connection with nociceptive inputs on the trigeminal nucleus caudalis and/or a dilation of vessels compressing the facial nerve at the root exit zone could lead to the development of HFS in predisposed patients. 'Migraine-triggered hemifacial spasm' could possibly be regarded as a complication of migraine.

[SOD1-N196 mutation in a family with amyotrophic lateral sclerosis]. / Mutación SOD1-N19S en una familia de esclerosis lateral amiotrófica.

INTRODUCTION: N19S mutation is produced by substitution in the 139 position of SOD1 and was described by Mayeux in a patient with amyotrophic lateral sclerosis (ALS). He suggested that it did not have a causal effect as it was found in asymptomatic and sporadic cases. Other authors in later articles did not agree. MATERIAL AND METHODS: We describe a family with 4 members with ALS patients and attempt to find the carrier of the N19S mutation of the propositus. Molecular studies were performed on 15 members of the family of a different order. RESULTS: The ALS cases were found in the maternal line of the propositus. The presence of the mutation was detected in 3 people, the other two were asymptomatic. One of patients with ALS in the family, who died previously, did not have the mutation. Two of the sons of this case and another of the other case did not show it. On the other hand, N19S mutation was only present in paternal branch of the propositus, where there were no cases. CONCLUSION: The described family supports the hypothesis by Mayeux and against that mutation N19S has pathological consequences, since mutation is only in the family line where there are no cases with ALS. In consequence, although the described case is included as a familiar form, it cannot be attributed to the mutation, and its relationship with N19S should be considered as casual.

Validation of the Spanish-language version of the Montreal Cognitive Assessment as a screening test for cognitive impairment in multiple sclerosis.

BACKGROUND: The neuropsychological batteries traditionally used for the assessment of cognitive impairment (CI) in patients with multiple sclerosis are complex tests requiring a long time to administer. Simpler tests are needed to detect cognitive impairment in daily clinical practice. OBJECTIVE: We aimed to evaluate the diagnostic validity and reliability of the Montreal Cognitive Assessment (MoCA) test as a screening tool for CI in patients with multiple sclerosis, as compared against the Brief Neuropsychological Battery. MATERIAL AND METHODS: We recruited 52 patients with multiple sclerosis (61.5% women; mean age [standard deviation]: 41.7 [11.5] years). We analysed the reliability (internal consistency, interobserver reliability, and test-retest reliability), construct validity (factor analysis, Pearson correlation coefficient, and coefficient of determination), and criterion validity (ROC curve, sensitivity, specificity, total agreement, positive and negative predictive values, positive and negative likelihood ratios, and Fagan nomogram) of the MoCA test in this population. RESULTS: The prevalence of CI was 21.2% according to findings from the Brief Neuropsychological Battery, and 25% according to the MoCA test. The MoCA test showed good internal consistency (Cronbach alpha, 0.822) and interobserver and test-retest reliability (intraclass correlation coefficient 0.80 and 0.96, respectively). The correlation coefficient between total Brief Neuropsychological Battery and MoCA test scores was 0.82. The optimal cut-off point on the ROC curve was 25-26, yielding 91% sensitivity and 93% specificity. CONCLUSION: The MoCA test is a valid and reliable tool for screening for CI in patients with multiple sclerosis.

Calcitonin gene-related peptide in migraine: from pathophysiology to treatment.

INTRODUCTION: It has been observed in recent years that levels of such molecules as calcitonin gene-related peptide (CGRP) and, to a lesser extent, the pituitary adenylate cyclase-activating peptide are elevated during migraine attacks and in chronic migraine, both in the cerebrospinal fluid and in the serum. Pharmacological reduction of these proteins is clinically significant, with an improvement in patients' migraines. It therefore seems logical that one of the main lines of migraine research should be based on the role of CGRP in the pathophysiology of this entity. DEVELOPMENT: The Spanish Society of Neurology's Headache Study Group decided to draft this document in order to address the evidence on such important issues as the role of CGRP in the pathophysiology of migraine and the mechanism of action of monoclonal antibodies and gepants; and to critically analyse the results of different studies and the profile of patients eligible for treatment with monoclonal antibodies, and the impact in terms of pharmacoeconomics. CONCLUSIONS: The clinical development of gepants, which are CGRP antagonists, for the acute treatment of migraine attacks, and CGRP ligand and receptor monoclonal antibodies offer promising results for these patients.

Neuro-otological symptoms in patients with migraine.

INTRODUCTION: Neurootological symptoms are common in patients with migraine, and have been reported to be associated with diverse conditions. PATIENTS AND METHODS: A total of 70 patients with a diagnosis of episodic migraine, with or without aura, attending our Migraine Unit were selected. The specific variables studied were the diagnosis of instability, psycho-physiological dizziness, presyncopal symptoms, benign paroxysmal positional vertigo (BPPV), migraine associated recurrent vertigo (MARV), and Meniere's disease. RESULTS: A total of 44.3% of cases had orthostatism or syncope, 15.7% with instability (possibly due to bilateral vestibular hypofunction), 14.2% with MARV and 8.6% with BPPV. The presence of BPPV was observed in older patients (40 years), whilst MARV was a condition seen in younger ones (35 years). These findings are of interest and remind us that benign paroxysmal vertigo is a childhood condition and age is a risk for BPPV. CONCLUSIONS: Migraine patients often present with neuro-otological symptoms that can be classified as inter-episodic and episodic symptoms, and specific and non-specific migraine symptoms. This approach is of obvious pathophysiological interest, given that MARV and the possible vestibular hypofunction of migraine patients are symptoms that share physiological aspects with migraine, while the orthostatism symptoms and BPPV are non-specific and are seen to be associated with other conditions.

Document of revision and updating of medication overuse headache (MOH). / Documento de revisión y actualización de la cefalea por uso excesivo de medicación (CUEM).

INTRODUCTION: Medication overuse headache is a secondary headache in which the regular or frequent use of analgesics can increase the frequency of the episodes, causing the transition from episodic to chronic headache. The prevalence of medication overuse headache is approximately 1-2%, with higher rates among women aged 30-50 years and with comorbid psychiatric disorders such as depression or anxiety, or other chronic pain disorders. It is important to be familiar with the management of this disease. To this end, the Spanish Society of Neurology's Headache Study Group has prepared a consensus document addressing this disorder. DEVELOPMENT: These guidelines were prepared by a group of neurologists specialising in headache after a systematic literature review and provides consensus recommendations on the proper management and treatment of medication overuse headache. The treatment of medication overuse headache is often complex, and is based on 4 fundamental pillars: education and information about the condition, preventive treatment, discontinuation of the drug being overused, and treatment for withdrawal symptoms. Follow-up of patients at risk of recurrence is important. CONCLUSIONS: We hope that this document will be useful in daily clinical practice and that it will update and improve understanding of medication overuse headache management.

Generalized neck-shoulder hyperalgesia in chronic tension-type headache and unilateral migraine assessed by pressure pain sensitivity topographical maps of the trapezius muscle.

Spatial changes in pressure pain hypersensitivity are present throughout the cephalic region (temporalis muscle) in both chronic tension-type headache (CTTH) and unilateral migraine. The aim of this study was to assess pressure pain sensitivity topographical maps on the trapezius muscle in 20 patients with CTTH and 20 with unilateral migraine in comparison with 20 healthy controls in a blind design. For this purpose, a pressure algometer was used to assess pressure pain thresholds (PPT) over 11 points of the trapezius muscle: four points in the upper part of the muscle, two over the levator scapulae muscle, two in the middle part, and the remaining three points in the lower part of the muscle. Pressure pain sensitivity maps of both sides (dominant/non-dominant; symptomatic/non-symptomatic) were depicted for patients and controls. CTTH patients showed generalized lower PPT levels compared with both migraine patients (P = 0.03) and controls (P < 0.001). The migraine group had also lower PPT than healthy controls (P < 0.001). The most sensitive location for the assessment of PPT was the neck portion of the upper trapezius muscle in both patient groups and healthy controls (P < 0.001). PPT was negatively related to some clinical pain features in both CTTH and unilateral migraine patients (all P < 0.05). Side-to-side differences were found in strictly unilateral migraine, but not in those subjects with bilateral pain, i.e. CTTH. These data support the influence of muscle hyperalgesia in both CTTH and unilateral migraine patients and point towards a general pressure pain hyperalgesia of neck-shoulder muscles in headache patients, particularly in CTTH.

Pressure pain sensitivity of the scalp in patients with nummular headache: a cartographic study.

Nummular headache (NH) is characterized by focal pain fixed within a small round or elliptical area of the head surface. Sensory dysfunction is apparently restricted to the symptomatic area, but a thorough analysis of cranial pain sensitivity has not been performed. Pressure pain sensitivity maps were constructed for 21 patients with NH and 21 matched healthy controls. In each subject pressure pain thresholds (PPT) were measured on 21 points distributed over the scalp. In each patient PPT were also measured in the symptomatic area and at a non-symptomatic symmetrical point. In both groups an anterior to posterior gradient was found on each side, with no significant differences of PPT measurements between sides or groups. In patients with NH, only the symptomatic area showed a local decrease of PPT (significant in comparison with the non-symptomatic symmetrical point, P < 0.001). These findings further support that NH is a non-generalized disorder with a peripheral source.

Validation of the Spanish-language version of the Montreal Cognitive Assessment as a screening test for cognitive impairment in multiple sclerosis. / Validación de la versión española de la Escala Cognitiva de Montreal (MoCA) como herramienta de cribado de deterioro cognitivo asociado a la esclerosis múltiple.

INTRODUCTION: The neuropsychological batteries traditionally used for the assessment of cognitive impairment (CI) in patients with multiple sclerosis are complex tests requiring a long time to administer. Simpler tests are needed to detect cognitive impairment in daily clinical practice. OBJECTIVE: We aimed to evaluate the diagnostic validity and reliability of the Montreal Cognitive Assessment (MoCA) test as a screening tool for CI in patients with multiple sclerosis, as compared against the Brief Neuropsychological Battery. MATERIAL AND METHODS: We recruited 52 patients with multiple sclerosis (61.5% women; mean age [standard deviation]: 41.7 [11.5] years). We analysed the reliability (internal consistency, interobserver reliability, and test-retest reliability), construct validity (factor analysis, Pearson correlation coefficient, and coefficient of determination), and criterion validity (ROC curve, sensitivity, specificity, total agreement, positive and negative predictive values, positive and negative likelihood ratios, and Fagan nomogram) of the MoCA test in this population. RESULTS: The prevalence of CI was 21.2% according to findings from the Brief Neuropsychological Battery, and 25% according to the MoCA test. The MoCA test showed good internal consistency (Cronbach alpha, 0.822) and interobserver and test-retest reliability (intraclass correlation coefficient 0.80 and 0.96, respectively). The correlation coefficient between total Brief Neuropsychological Battery and MoCA test scores was 0.82. The optimal cut-off point on the ROC curve was 25-26, yielding 91% sensitivity and 93% specificity. CONCLUSION: The MoCA test is a valid and reliable tool for screening for CI in patients with multiple sclerosis.

Evidence of and experience with the use of onabotulinumtoxinA in trigeminal neuralgia and primary headaches other than chronic migraine. / Evidencia y experiencia del uso de onabotulinumtoxinA en neuralgia del trigémino y cefaleas primarias distintas de la migraña crónica.

INTRODUCTION: In the field of headaches, onabotulinumtoxinA (onabotA) is well established as a treatment for chronic migraine (CM). In recent years, it has been used increasingly to treat other primary headaches (high-frequency episodic migraine, trigeminal-autonomic cephalalgias, nummular headache) and trigeminal neuralgia. As this treatment will progressively be incorporated in the management of these patients, we consider it necessary to reflect, with a fundamentally practical approach, on the possible indications of onabotA, beyond CM, as well as its administration protocol, which will differ according to the type of headache and/or neuralgia. DEVELOPMENT: This consensus document was drafted based on a thorough review and analysis of the existing literature and our own clinical experience. The aim of the document is to serve as guidelines for professionals administering onabotA treatment. The first part will address onabotA's mechanism of action, and reasons for its use in other types of headache, from a physiopathological and clinical perspective. In the second part, we will review the available evidence and studies published in recent years. We will add an "expert recommendation" based on our own clinical experience, showing the best patient profile for this treatment and the most adequate dose and administration protocol. CONCLUSION: Treatment with onabotA should always be individualised and considered in selected patients who have not responded to conventional therapy.

Bifocal nummular headache: the first three cases.

Nummular headache (NH) has been defined as a focal head pain that is exclusively felt in a small area of the head surface. Here we describe three patients who presented with focal head pain in two separate areas. This finding seems to be consistent with bifocal NH and further enlarges the clinical diversity of this headache disorder. The pathogenic mechanisms of NH may be active in multiple cranial areas in some particular patients.

Pressure pain sensitivity mapping of the temporalis muscle revealed bilateral pressure hyperalgesia in patients with strictly unilateral migraine.

Previous studies on pressure pain sensitivity in patients with migraine have shown conflicting results. There is emerging evidence suggesting that pain sensitivity is not uniformly distributed over the muscles, indicating the existence of topographical changes in pressure pain sensitivity. The aim of this study was to calculate topographical pressure pain sensitivity maps of the temporalis muscle in a blind design in patients with strictly unilateral migraine compared with controls. For this purpose, an electronic pressure algometer was used to measure pressure pain thresholds (PPT) over nine points of the temporalis muscle: three points in the anterior, medial and posterior parts, respectively. Pressure pain sensitivity maps of both sides (dominant or non-dominant; symptomatic or non-symptomatic) were calculated. The analysis of variance showed significant differences in PPT values between both groups (F = 279.2; P < 0.001) and points (F = 4.033; P < 0.001). Patients showed lower PPT at all nine points than healthy controls (P < 0.001). We also found lower PPT in the centre of the muscle compared with the posterior part of the muscle within both groups (P < 0.01). Interaction between group and points (F = 1.9; P < 0.05) was also found. Within the migraine group, PPT levels were decreased bilaterally from the posterior to the anterior column of the temporalis muscle (Student-Newman- Keuls analysis; P < 0.05), with the most sensitive in the anterior part of the muscle. For controls, PPT did not follow such anatomical distribution, the most sensitive point being the centre of the mid-muscle belly. This study showed bilateral sensitization to pressure in unilateral migraine, suggesting the involvement of central components.

Evidence and experience with onabotulinumtoxinA in chronic migraine: Recommendations for daily clinical practice. / Evidencia y experiencia de bótox en migraña crónica: Recomendaciones para la práctica clínica diaria.

OnabotulinumtoxinA has been demonstrated to be effective as a preventive treatment in patients with chronic migraine (CM). Five years after the approval of onabotulinumtoxinA in Spain, the Headache Study Group of the Spanish Society of Neurology considered it worthwhile to gather a group of experts in treating patients with CM in order to draw up, based on current evidence and our own experience, a series of guidelines aimed at facilitating the use of the drug in daily clinical practice. For this purpose, we posed 12 questions that we ask ourselves as doctors, and which we are also asked by our patients. Each author responded to one question, and the document was then reviewed by everyone. We hope that this review will constitute a practical tool to help neurologists treating patients with CM.

Calcitonin gene-related peptide in migraine: from pathophysiology to treatment. / CGRP en migraña: de la fisiopatología a la terapéutica.

INTRODUCTION: It has been observed in recent years that levels of such molecules as calcitonin gene-related peptide (CGRP) and, to a lesser extent, the pituitary adenylate cyclase-activating peptide are elevated during migraine attacks and in chronic migraine, both in the cerebrospinal fluid and in the serum. Pharmacological reduction of these proteins is clinically significant, with an improvement in patients' migraines. It therefore seems logical that one of the main lines of migraine research should be based on the role of CGRP in the pathophysiology of this entity. DEVELOPMENT: The Spanish Society of Neurology's Headache Study Group decided to draft this document in order to address the evidence on such important issues as the role of CGRP in the pathophysiology of migraine and the mechanism of action of monoclonal antibodies and gepants; and to critically analyse the results of different studies and the profile of patients eligible for treatment with monoclonal antibodies, and the impact in terms of pharmacoeconomics. CONCLUSIONS: The clinical development of gepants, which are CGRP antagonists, for the acute treatment of migraine attacks, and CGRP ligand and receptor monoclonal antibodies offer promising results for these patients.