[Clinical and pathophysiological description of nummular headaches: a case series]. / Descripción clínica y fisiopatológica de la cefalea numular: serie de casos.

INTRODUCTION: Nummular headache was initially described as an infrequent primary headache characterised by chronic circumscribed mild-to-moderate pain in a small area of the head that may present with exacerbations. Since its initial description, dozens of cases of nummular headache have been reported which broaden the clinical and pathophysiological spectrum. AIMS: To review and establish the clinical characteristics of nummular headache, to analyse the epidemiology in our setting and to clarify some unanswered pathophysiological issues. PATIENTS AND METHODS: The study involved 83 cases (42 women and 41 men) who were diagnosed with nummular headache from July 2008 and followed up until 2018 at the Fundacion Alcorcon Hospital. The mean age of onset was 46 years. The time to diagnosis was seven months. RESULTS: The pain was moderate-intense and oppressive, with exacerbations in 62.5% of cases. The average size of the symptomatic area was 2.4 ± 0.7 cm, with a parietal (47%), temporal (24%), frontal (12%) or occipital (6%) location. It presented a chronic (82%) or episodic (18%) pattern. Symptoms of local sensory dysfunction were observed in 25 patients and three others showed skin alterations. Spontaneous remission was observed in 36% of cases, and the most commonly used drugs were non-steroidal anti-inflammatory drugs, paracetamol and gabapentin. Botulinum toxin was effective. CONCLUSIONS: Nummular headache is a disorder with well-defined characteristics and is considered an entity in its own right. Our data indicate that its origin lies in the epicranial structures. The conjunction of pain, sensory dysfunction and trophic disorders suggests a local form of a complex regional syndrome.

TITLE: Descripción clínica y fisiopatológica de la cefalea numular: serie de casos.Introducción. La cefalea numular se describió inicialmente como una cefalea primaria infrecuente caracterizada por dolor circunscrito crónico en una pequeña área craneal, de intensidad leve-moderada y que puede presentar exacerbaciones. Desde su descripción inicial se han publicado decenas de casos de cefalea numular que amplían el espectro clínico y fisiopatológico. Objetivos. Revisar y establecer las características clínicas de la cefalea numular, analizar la epidemiología en nuestro medio y aclarar incógnitas fisiopatológicas. Pacientes y métodos. Se estudiaron 83 casos (42 mujeres y 41 varones), que fueron diagnosticados de cefalea numular desde julio de 2003 a julio de 2008, y seguidos hasta 2018 en el Hospital Universitario Fundación Alcorcón. La edad media de inicio fue de 46 años. El tiempo de evolución hasta el diagnóstico fue de siete meses. Resultados. El dolor fue moderado-intenso y opresivo, con exacerbaciones en el 62,5% de los casos. El tamaño medio del área sintomática fue de 2,4 ± 0,7 cm, con una localización parietal (47%), temporal (24%), frontal (12%) u occipital (6%). Presentó un patrón crónico (82%) o episódico (18%). Mostraron síntomas de disfunción sensitiva local 25 pacientes, y otros tres, cambios en la piel. La remisión espontánea se observó en el 36% de los casos, y los fármacos más utilizados fueron los antiinflamatorios no esteroideos, el paracetamol y la gabapentina. La toxina botulínica fue eficaz. Conclusiones. La cefalea numular es un trastorno con características bien definidas y con entidad propia. Nuestros datos indican que su origen está en las estructuras epicraneales. La conjunción de dolor, disfunción sensitiva y alteraciones tróficas indica una forma local de síndrome regional complejo.

The Direct Referral to Endovascular Center criteria: a proposal for pre-hospital evaluation of acute stroke in the Madrid Stroke Network.

BACKGROUND AND PURPOSE: For patients with acute ischaemic stroke due to large-vessel occlusion, it has recently been shown that mechanical thrombectomy (MT) with stent retrievers is better than medical treatment alone. However, few hospitals can provide MT 24 h/day 365 days/year, and it remains unclear whether selected patients with acute stroke should be directly transferred to the nearest MT-providing hospital to prevent treatment delays. Clinical scales such as Rapid Arterial Occlusion Evaluation (RACE) have been developed to predict large-vessel occlusion at a pre-hospital level, but their predictive value for MT is low. We propose new criteria to identify patients eligible for MT, with higher accuracy. METHODS: The Direct Referral to Endovascular Center criteria were defined based on a retrospective cohort of 317 patients admitted to a stroke center. The association of age, sex, RACE scale score and blood pressure with the likelihood of receiving MT were analyzed. Cut-off points with the highest association were thereafter evaluated in a prospective cohort of 153 patients from nine stroke units comprising the Madrid Stroke Network. RESULTS: Patients with a RACE scale score ≥ 5, systolic blood pressure <190 mmHg and age <81 years showed a significantly higher probability of undergoing MT (odds ratio, 33.38; 95% confidence interval, 12-92.9). This outcome was confirmed in the prospective cohort, with 68% sensitivity, 84% specificity, 42% positive and 94% negative predictive values for MT, ruling out 83% of hemorrhagic strokes. CONCLUSIONS: The Direct Referral to Endovascular Center criteria could be useful for identifying patients suitable for MT.

Ciliary ganglioplegic migraine: migraine-related prolonged mydriasis.

BACKGROUND: Transient, unilateral mydriasis has been reported in migraine patients, and this has been regarded as a possible co-morbidity between migraine and Adie's tonic pupil. Mydriasis that outlasts the duration of migraine attacks is rare. METHODS: Through an eight-year period we have studied all patients referred to our neurological clinic because of migraine attack with mydriasis. All the patients underwent thorough neurologic and ophthalmologic examinations including MRI of the brain and testing of the pupil response to ocular instillation of dilute pilocarpine (0.125%). RESULTS: Seven women and two men, with a mean age of 33.8 ± 12.3 years (range: 19-52) were included. The patients presented during one hemicranial migraine attack with an ipsilateral mydriasis that persisted for a mean of three months, while migraine headaches remained with their typical episodic course. In all the patients a cholinergic supersensitivity in the symptomatic pupil was demonstrated, thus pointing to a dysfunction of the ipsilateral ganglionic parasympathetic fibers. CONCLUSIONS: Synchronous co-localization of the features suggests a pathogenic link between the pupil dysfunction and migraine, rather than a simultaneous coexistence of two independent disorders. Likely explanations include a latent Adie's pupil that could have been triggered during a particular migraine attack; a ciliar ganglionic lesion/dysfunction produced by the migrainous process; an ophthalmoplegic migraine with selective parasympathycoparesis; or an episodic ciliar ganglionitis with migrainous features. Ciliary ganglioplegic migraine is proposed as a nominal term pointing to the possible anatomic source of the migrainous-related pupil dysfunction; the pathogenesis remains unknown.

Ophthalmoplegic migraine. Two patients with an absolute response to indomethacin.

Two patients suffering from ophthalmoplegic migraine had a strictly unilateral headache absolutely responsive to indomethacin, but not to other non-steroidal anti-inflammatory drugs, analgesics or corticosteroids. Such observations raise a therapeutic alternative and suggest that ophthalmoplegic migraine may present with different headache phenotypes.

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.

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.

Nummular headache secondary to an intracranial mass lesion.

Nummular headache is a coin-shaped, chronic cephalalgia usually considered to stem from epicranial tissues. We describe a patient complaining of circumscribed pain in the head as the only symptom of a subtentorial meningioma. This observation underlines the need to revise the concept of circumscribed, referred pains in the head arising from pain-sensitive intracranial structures.

Pericranial tenderness is not related to nummular headache.

The aim of the present study was to investigate whether nummular headache (NH) patients show increased pericranial tenderness in relation to healthy subjects, and to compare pericranial tenderness between both NH and chronic tension-type headache (CTTH) patients. Three tenderness (total, cephalic and neck) scores were objectively and blinded assessed in 10 NH patients, 10 CTTH subjects and 10 healthy matched controls. No significant differences were found in any tenderness score between the symptomatic and non-symptomatic sides in NH, or between right and left sides in either CTTH or control groups. All tenderness scores were significantly greater in CTTH patients compared with both NH patients and controls (P < 0.001), but not significantly different between NH patients and controls. Therefore, NH patients had lower tenderness than patients with CTTH and did not show increased tenderness when compared with healthy subjects. In addition, tenderness in NH patients was quite symmetrical between both the symptomatic and the non-symptomatic sides. The absence of increased pericranial tenderness could be clinically useful in distinguishing NH from CTTH. Current findings expand the evidence supporting the notion that NH is a non-generalized and rather limited disorder, marking the presence of a well-delimited painful zone.

[Management and improvement of the process of outpatient treatment of transient ischemic attacks in Neurology departments]. / Gestión y mejora del proceso de tratamiento ambulatorio del accidente isquémico transitorio en consultas de Neurología.

AIMS: To describe our experience in the ambulatory treatment of transient ischemic attacks (TIA) in a second-level hospital with the implementation of an example of process management within the different services involved in attending such conditions. PATIENTS AND METHODS: The following aspects of the process--mission, limits, flowchart, eligibility criteria and performance indicators (process, sentinel and effectiveness)--were defined by multidisciplinary consensus (Emergency, Vascular Surgery, Cardiology, Neurology and Radiology departments). This process was implemented from April 2002 to May 2004, and monthly assessments were carried out to check for problems and to introduce corrective measures. After ending the process, patients with TIA were admitted as inpatients for a hospital study. RESULTS: Definition of the process: medical care (diagnosis and treatment) of TIA patients who are eligible for a complete outpatient study within seven days. Patients evaluated: 254. Indicator 1 (percentage of suitable referrals): 2002: 53%, 2003: 75%, 2004: 73%. Indicator 2 (percentage of complete studies in less than one week): 2002: 35%, 2003: 57%, 2004: 50%; hospitalised: 90%. Indicator 3 (infarcts during the study): 2002: 4.3%, 2003: 0%, 2004: 0%; hospitalised: 0%. Indicator 4 (percentage of adjustments made to treatment): 2002: 39%, 2003: 31%, 2004: 62%; hospitalised: 72%. Mean delay before visit: in February 2002: 90 days, in April 2002: 7.67 days and in April 2003: 5.37. Problems detected: delays in referrals, failure to fit hospitalisation criteria, delays in carrying out examinations not included in the protocol, unsuitable indicator design. Steps taken: redefinition of indicators, modification of the referral system, adjustments made to the circuits involved in carrying out tests, review of hospitalisation criteria. CONCLUSIONS: Process management is an ideal tool for achieving ongoing improvements in clinical praxis. Early monitoring makes it possible to detect problems and to implement corrective measures. In our area, the study of TIA must be performed in a hospital inpatient regimen in order to comply with the guidelines for clinical practice.

[Hematoma of the olfactory bulbs following craneoencephalic trauma]. / Hematoma de bulbos olfatorios tras traumatismo craneoencefalico.

The ability to smell is commonly altered by head injuries. However, the nature, prevalence, prognosis and etiology of such alterations are poorly understood. Patients complaining of head-trauma and a related olfactory dysfunction typically have anosmia and rarely regain normal olfactory ability. Parosmia prevalences decreases over time in such patients, and damage to olfaction-related brain structures can be observed in most such patients using an appropriate MRI protocol. In this article, we review the recent knowledge about postraumatic anosmia.

Duration of attacks of first division trigeminal neuralgia.

Objective measurements of duration of attacks have been performed in 8 (5 female and 3 male) patients suffering from primary first division (V-1) trigeminal neuralgia. The mean age of the patients was 67.5 +/- 11.4 years, and the mean age at onset 64.0 +/- 9.7 years. During the study the patients were off treatment. A total of 192 attacks were witnessed by the authors and exactly timed by a stop-watch. The duration of attacks ranged from 2 to 32 s, with a mean of 6.5 +/- 6.1 s. The unweighted mean was 8.8 +/- 5.7 s, with a range of 2.4-17.5 s. With the present data the duration of attacks of V-1 neuralgia has been exactly determined, and the clinical distinction of V-1 neuralgia from other shortlasting headaches, particularly from SUNCT, has been substantially clarified.

[Is progressive anarthria a clinical form of Pick complex?]. / Anartria progresiva: forma clínica del complejo Pick?

INTRODUCTION: Progressive anarthria is defined as a clinical entity with a degenerative origin consisting in progressive difficulty in articulating while grammatical, semantic and graphic aspects of language are preserved. It is included within the group of processes affecting restricted areas of the brain although its exact nosological location is not clear. We report two cases that progressed clinically towards frontotemporal dementia and corticobasal degeneration, respectively. CASE REPORTS: Case 1: a male who, at the age of 72, began with speech difficulties while comprehension and reading/writing skills were preserved. Three years later he developed apathy, bulimia, sexual indiscretions and aggressiveness, with preservation of visual memory, visual-constructional capacity and elementary writing skills. Case 2: a male who, at the age of 70, began with speech disorders, which were associated two years later to generalised slowness with Hoehn and Yahr stage II akinetic-rigid symptoms; another two years later, he developed a dystonic attitude and melokinetic apraxia in the left upper limb. CONCLUSIONS: The two cases, which were initially compatible with progressive anarthria, progressed clinically towards frontotemporal dementia and corticobasal degeneration, which are entities that are included in 'Pick complex'. This is a concept that we believe to be useful from a clinical point of view, given the variability that exists in the histology of the entities that have been proposed as members of this syndrome group, together with the progression of the cases described in the literature and the ones we have reported in this work.

Cluster headache: interictal asymmetric increment in intraocular pressure elicited by Valsalva manoeuvre.

Changes in intraocular pressure (IOP) elicited by a Valsalva manoeuvre were studied in 11 male patients (mean age 39.8 years) suffering from episodic cluster headache (CH), and 12 healthy male controls (mean age 39.9 years). The tests were performed at rest and while exhaling hard through a mouthpiece connected to a mercury manometer. In the CH group, during symptomatic periods, between attacks, Valsalva manoeuvre elicited an asymmetric increase in IOP with significantly higher values on the symptomatic side (P = 0011), whereas no asymmetric increments in IOP were found during asymptomatic periods. Outside the cluster period the IOP values both baseline and with Valsalva manoeuvre did not differ from controls. The increment in IOP took place within a few seconds, as in spontaneous CH attacks, thus pointing to a rapid increase in intraocular blood volume or vasodilatation. These findings may reflect a latent increased vascular reactivity of the symptomatic orbit during CH period.

Objective assessment of autonomic signs during triggered first division trigeminal neuralgia.

A total of 26 episodes of V-1 trigeminal neuralgia attacks have been recorded in two female patients. Autonomic phenomena were assessed according to a semiquantitative scale. Attacks lasted 17 +/- 5 s. Mild lacrimation without conjunctival hyperaemia, rhinorrhea or ptosis was observed, even in relatively long lasting episodes. This is in clear contradiction with SUNCT (shortlasting, unilateral, neuralgiform headache with conjunctival injection, tearing and rhinorrhea) attacks that are always dramatically accompanied by both lacrimation and conjunctival injection of the symptomatic side from the very onset of symptoms. Carbamazepine provided complete and sustained relief of symptoms in both patients. Herein we will show differential autonomic features of V-1 trigeminal neuralgia vs. SUNCT that will both aid the clinician to distinguish both syndromes and stress that both entities are nosologicaly different.

Numular headache: a coin-shaped cephalgia.

Numular headache is a chronic, mild to moderate, pressurelike pain in a circumscribed cranial area of approximately 2 to 6 cm in diameter. Pain usually is limited to the parietal region, although it may appear in any cranial site. It is a benign process of usually unknown origin.

Nonconvulsive status epilepticus associated with cephalosporins in patients with renal failure.

PURPOSE: Nonconvulsive status epilepticus is an unusual complication of cephalosporin therapy, with only a few isolated cases reported. SUBJECTS AND METHODS: We reviewed the clinical and electroencephalographic (EEG) characteristics of 10 patients with renal failure in whom developed alteration of consciousness without convulsions associated with continuous epileptiform EEG activity while being treated with cephalosporins. RESULTS: Nonconvulsive status epilepticus developed in 5 men and 5 women, with a mean (+/- SD) age of 69 +/- 14 years, while receiving intravenous cephalosporins (ceftriaxone, 2 patients; ceftazidime, 2; and cefepime, 6). All patients had renal failure; 1 also had hepatic failure. Patients presented with progressive disorientation or agitation, sometimes associated with mild facial or limb myoclonus, that had begun 1 to 10 days (mean, 5 +/- 2 days) after starting cephalosporin treatment. The EEG showed continuous or intermittent bursts of generalized, high-voltage, 1 to 2 Hz sharp wave activity or sharp and slow wave activity that resembled, but could be differentiated from, the triphasic waves seen in metabolic encephalopathies. Intravenous clonazepam suppressed the epileptiform activity completely in 5 patients and partially in the other 5. Cephalosporins were withdrawn, and antiepileptic therapy was started for all patients. All patients improved, 2 in less than 24 hours and the remainder within 2 to 7 days. CONCLUSIONS: Cephalosporins can cause nonconvulsive status epilepticus in patients with renal failure. The clinical picture is difficult to differentiate from a that of metabolic encephalopathy unless an EEG is obtained. Physicians should be aware of this potentially dangerous complication.

[Crossed apraxia secondary to a right parietal infarct]. / Apraxia cruzada secundaria a infarto parietal derecho.

INTRODUCTION: Crossed apraxia is an unusual alteration in praxic function due to a cerebral lesion which is contralateral to that which would be expected. We report a case of crossed apraxia due to a right parietal lesion in a left handed patient who had been obliged to be right handed. We discuss the peculiar neuropsychological profile and possible relation of this to left handedness. CLINICAL CASE: A 75 year old man, left handed but obliged to use the right hand, suffered an acute episode of alteration in the management of utensils (lids, taps, medicine containers), although he maintained his usual social behaviour and orientation. On neurosomatic examination no focal deficits were observed. On MMST he scored 29/30, on the Edinburgh questionnaire he scored 35 (left handed dominance). On a Barcelona PIENC battery his scores were in the pathological range for subtests on understanding orders, carrying out symbolic gestures and imitating gestures, in perceiving super imposed images and visual memory. MRI showed a right parietal laminar infarct. CONCLUSIONS: The patient showed clear bilateral ideo motor apraxia, which could not be justified/explained by visuo spatial agnosia secondary to a right parietal ischaemic lesion. It is usually considered that ideatory and ideomotor apraxias are due to left hemisphere lesions, whilst contsructive apraxia and apraxia related to dress are due to right hemisphere lesions. In this case, there was ideomotor apraxia secondary to a right parietal lesion, that is to say a crossed ideomotor apraxia, without crossing of other neuropsychological functions. This supports the theory of independence of hemisphere dominance for different cognitive functions. The frustrated left handedness of the patient might be related to the greater probability of unusual hemisphere dominance for ideomotor praxis function in this particular case.

[What clinical significance do vascular anomalies of the outer ear canal have?]. / Cuál es el significado clínico de las anomalías vasculares del conducto auditivo interno?

Vascular anomalies of the internal auditory canal (IAC) are relatively common, being found in 20-25% of magnetic resonance imaging (MRI) studies performed in patients with unilateral sensorineural hearing loss. They are usually asymptomatic and their diagnosis is almost always based on MRI, as they are generally undetected by routine clinical examination. Recently, the clinical relevance of these IAC anomalies and the effectiveness of vascular decompression have been debated. We present a series of 7 vascular anomalies of the IAC found in a series of 83 consecutive MRI scans performed in a screening program for IAC and cerebellopontine angle pathology. We reviewed their clinical and radiological presentation to evaluate their clinical relevance. In this series, we found no correlation between the presence of such vascular anomalies and the clinical symptoms that motivated MRI, so we conclude that most of these anomalies were accidental findings.