[Pathological hip fracture of pediatric age. Revision of the cystic angiomatosis of the bone]. / Fractura patológica de cadera en edad pediátrica. Revisión de la angiomatosis quística ósea.

INTRODUCTION: Bone cystic angiomatosis is a disease with only 200 cases described, based on angiomatous nonmalignant proliferation, in bone tissue and viscera. It focuses on pelvis, long bones and scapular waist. Clinical evolution ranges from self-limiting forms to massive aggressive osteolysis (Gorham-Stout).Its diagnosis is of exclusion, with nonspecific clinical, radiological (lytic images) and histopathological findings. CLINICAL CASE: Our study is based on the clinical case of a 14-year-old man diagnosed with bone cystic angiomatosis, with disseminated bone involvement, who came to our center for pain and functional impotence in the right hip, diagnosing a pertrochanteric fracture. It was operated by open reduction and osteosynthesis with four-hole VERSA sliding plate screw. An allogeneic graft of cancellous bone was used from femoral head. The evolution was satisfactory, with ambulation at full load per month without requiring analgesic medication and control X-ray at eight months that showed signs of complete integration of the graft. The analgesic pain scale (visual analogue scale) showed a score of 2/10 per month. DISCUSSION: There is controversy regarding the treatment of these fractures by osteosynthesis with bone graft. We are facing a case without serious clinical criteria, but with multiple foci of osteolysis. It was decided not to intervene prophylactically the contraleteral hip, since it is asymptomatic and there are different patterns of evolution described in the literature and risks in surgery.

INTRODUCCIÓN: La angiomatosis quística ósea es una enfermedad con solamente 200 casos descritos basados en la proliferación no maligna angiomatosa en tejido óseo y vísceras. Se observa más en la pelvis, los huesos largos y la cintura escapular. La evolución clínica varía de formas autolimitantes a osteólisis agresiva masiva (Gorham-Stout). Su diagnóstico es por exclusión, con datos clínico-radiológicos (imágenes líticas) y resultados histopatológicos no específicos. CASO CLÍNICO: Nuestro estudio se basa en el caso clínico de un paciente de 14 años diagnosticado con angiomatosis quística ósea, con afección ósea diseminada, que vino a nuestro centro por el dolor y la impotencia funcional en la cadera derecha, diagnosticando una fractura pertrocantérea. Se le hizo una osteosíntesis con tornillo deslizante de la placa VERSA de cuatro agujeros. Se utilizó un injerto alogénico de hueso esponjoso de la cabeza femoral. La evolución fue satisfactoria con ambulación a plena carga por mes sin requerir medicación analgésica y control de rayos X a los ocho meses, los cuales mostraron signos de completa integración del injerto. La escala analgésica del dolor (EVA) mostró una puntuación de 2/10 por mes. DISCUSIÓN: Hay controversia con respecto al tratamiento de estas fracturas por la osteosíntesis con el injerto del hueso. Nos enfrentamos a un caso sin criterios clínicos serios, pero con múltiples focos de osteólisis. Se decidió no intervenir profilácticamente la cadera contralateral, ya que es asintomática y hay diferentes patrones de evolución descritos en la literatura y los riesgos en la cirugía.

[«Apuntes en Neurologia¼ (Notes in Neurology): a synthesis of the evidence on common paroxysmal neurological disorders and on neurodegenerative disorders]. / «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.

«Apuntes en Neurologia¼ is an initiative in which prominent national and international leaders, with broad academic recognition, came together to synthesise the most outstanding clinical aspects within their area of interest and to discuss the latest developments in a more accessible language. Understanding the factors that affect the onset and progression of any neurological disease through a review is important to be able to develop strategies to reduce the burden of these diseases. Moreover, knowledge of the clinical aspects is essential to solve the problems of daily clinical practice. The data collected here reflect the weight of evidence and some of them anticipate a promising future in the treatment of these diseases. This first edition focuses on common paroxysmal neurological disorders such as migraine, epilepsy and sleep disorders, as well as neurodegenerative disorders such as Parkinson's disease and cognitive impairment. These are clearly different pathologies, although some of them such as migraine and epilepsy, may share clinical symptoms. Sleep disorders, however, are important manifestations of neurodegenerative diseases that are sometimes clinically apparent long before the onset of other neurological symptoms. After recalling pathophysiology and diagnosis, the current review focuses on bringing together the main advances in five of the major neurological diseases.

TITLE: «Apuntes en Neurologia¼: una sintesis de la evidencia en trastornos neurologicos comunes paroxisticos y en trastornos neurodegenerativos.«Apuntes en Neurologia¼ es una iniciativa en la cual lideres de primera linea nacional e internacional, con amplio reconocimiento academico, se reunieron para sintetizar los aspectos clinicos mas destacables dentro de su area de interes y acercar las novedades en una lengua mas proxima. Entender los factores que afectan al inicio y progresion de cualquier enfermedad neurologica a traves de una revision es importante para el desarrollo de estrategias en pro de reducir la carga de estas enfermedades, y conocer los aspectos clinicos es esencial para poder resolver los problemas de la practica clinica diaria. Los datos aqui recogidos reflejan el peso de la evidencia y algunos de ellos anticipan un futuro prometedor en el tratamiento de estas enfermedades. Esta primera edicion se centra en trastornos neurologicos comunes paroxisticos como la migraña, la epilepsia y las alteraciones del sueño, y en trastornos neurodegenerativos como la enfermedad de Parkinson y el deterioro cognitivo. Se trata de patologias claramente diferentes, si bien algunas de ellas, como la migraña y la epilepsia, pueden compartir sintomatologia clinica. Los trastornos del sueño, por su parte, son manifestaciones importantes de enfermedades neurodegenerativas que, en ocasiones, son clinicamente evidentes mucho antes del inicio de otros sintomas neurologicos. Tras recordar la fisiopatologia y el diagnostico, la revision actual se centra en acercar los principales avances en cinco de las principales enfermedades neurologicas.

Effectiveness of manual therapy versus surgery in pain processing due to carpal tunnel syndrome: A randomized clinical trial.

BACKGROUND: People with carpal tunnel syndrome (CTS) exhibit widespread pressure pain and thermal pain hypersensitivity as a manifestation of central sensitization. The aim of our study was to compare the effectiveness of manual therapy versus surgery for improving pain and nociceptive gain processing in people with CTS. METHODS: The trial was conducted at a local regional Hospital in Madrid, Spain from August 2014 to February 2015. In this randomized parallel-group, blinded, clinical trial, 100 women with CTS were randomly allocated to either manual therapy (n = 50), who received three sessions (once/week) of manual therapies including desensitization manoeuvres of the central nervous system, or surgical intervention (n = 50) group. Outcomes including pressure pain thresholds (PPT), thermal pain thresholds (HPT or CPT), and pain intensity which were assessed at baseline, and 3, 6, 9 and 12 months after the intervention by an assessor unaware of group assignment. Analysis was by intention to treat with mixed ANCOVAs adjusted for baseline scores. RESULTS: At 12 months, 95 women completed the follow-up. Patients receiving manual therapy exhibited higher increases in PPT over the carpal tunnel at 3, 6 and 9 months (all, p < 0.01) and higher decrease of pain intensity at 3 month follow-up (p < 0.001) than those receiving surgery. No significant differences were observed between groups for the remaining outcomes. CONCLUSIONS: Manual therapy and surgery have similar effects on decreasing widespread pressure pain sensitivity and pain intensity in women with CTS. Neither manual therapy nor surgery resulted in changes in thermal pain sensitivity. SIGNIFICANCE: The current study found that manual therapy and surgery exhibited similar effects on decreasing widespread pressure pain sensitivity and pain intensity in women with carpal tunnel syndrome at medium- and long-term follow-ups investigating changes in nociceptive gain processing after treatment in carpal tunnel syndrome.

Consensus recommendations for anaesthetic peripheral nerve block. / Guía consenso sobre técnicas de infiltración anestésica de nervios pericraneales.

INTRODUCTION: Anaesthetic block, alone or in combination with other treatments, represents a therapeutic resource for treating different types of headaches. However, there is significant heterogeneity in patterns of use among different professionals. DEVELOPMENT: This consensus document has been drafted after a thorough review and analysis of the existing literature and our own clinical experience. The aim of this document is to serve as guidelines for professionals applying anaesthetic blocks. Recommendations are based on the levels of evidence of published studies on migraine, trigeminal autonomic cephalalgias, cervicogenic headache, and pericranial neuralgias. We describe the main technical and formal considerations of the different procedures, the potential adverse reactions, and the recommended approach. CONCLUSION: Anaesthetic block in patients with headache should always be individualised and based on a thorough medical history, a complete neurological examination, and expert technical execution.

[Neurophysiological advances in carpal tunnel syndrome: process of central sensitisation or local neuropathy]. / Avances neurofisiologicos en el sindrome del tunel del carpo: proceso de sensibilizacion central o neuropatia local.

INTRODUCTION: Carpal tunnel syndrome (CTS) is considered a simple entrapment of the median nerve at the carpal tunnel. In the last years, several studies have demonstrated the presence of peripheral and central sensitization mechanisms. AIM: To review the basis neurophysiology of peripheral and central sensitization by applying them to CTS and to determine their clinical repercussions. DEVELOPMENT: Several studies have revealed that patients with CTS exhibit somato-sensory changes in areas innervated by the median nerve and also in areas non-related with the median nerve. Individuals with CTS exhibited widespread mechanical and thermal pain hyperalgesia, although they suffered from unilateral symptoms. Further, patients also showed wide-spread impairments in vibration conduction, deficits in fine motor control and changes in the somato-sensory cortex. These evidences support the presence of a complex process of peripheral and central sensitization in patients with CTS which may constitute a negative prognosis factor for the management of these patients. CONCLUSIONS: The advances in neurosciences in the last years support the presence of peripheral and central sensitization mechanisms in CTS. These mechanisms justify the necessity of conceptual changes and in the management, both conservative and surgical, of this syndrome. Additionally, central sensitization can also play a relevant role in the prognosis of CTS since it can constitute a negative prognosis factor for its treatment.

[Characterisation of pain in patients with carpal tunnel syndrome according to electromyographic severity criteria]. / Caracterizacion del dolor en pacientes con sindrome del tunel del carpo segun criterios electromiograficos de gravedad.

INTRODUCTION: The symptoms of carpal tunnel syndrome (CTS) vary greatly and can depend on the severity of the clinical symptoms. AIMS: To characterise the description and self-perception of pain in a sample of patients with CTS and to determine whether that perception of pain differs among the patients on the basis of electrodiagnostic severity criteria. PATIENTS AND METHODS: The sample consisted of 92 females with CTS, who were classified as having mild, moderate or severe CTS, bearing in mind electrodiagnostic criteria. Different instruments were used in the study: a numerical scale (0-10) to evaluate the intensity of the pain, the McGill questionnaire to classify the descriptors that represent the quality of the pain, and the Boston questionnaire for assessing symptom severity and disability. RESULTS: The most representative descriptors were the presence of tingling (92%), heaviness (67%) and a feeling of swelling (64%). The women with mild CTS showed a longer history of pain than those with moderate and severe CTS (p < 0.05). There were no significant differences among the intensity of the pain, the disability questionnaire and most of the descriptors on the McGill questionnaire between the different groups of mild, moderate or severe CTS. The women with severe CTS described the pain as being periodic, repetitive and terribly unpleasant more often than those with mild or moderate CTS (p < 0.05). CONCLUSIONS: The presence of tingling and the feeling of heaviness are the most prevalent symptoms of CTS. There are no great differences in the quality of the pain among cases of mild, moderate or severe CTS.

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.

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 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.

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.

[Patent foramen ovale and migraine: where is the connection]. / Foramen oval permeable y migraña: ¿dónde está la conexión?

Patent foramen ovale (PFO) is an anatomic remnant of the fetal circulation that is highly prevalent in the general population. Several observational studies show that migraine with aura and PFO have higher coincidences than expected by chance. Both conditions might be linked by certain pathophysiological mechanisms, such as right-to-left shunting of venous agents or paradoxical embolism. Alternatively, they might share a common genetic background. No relevant differences have been found between the clinical features of migraine with FOP and those of migraine without FOP. The results of several observational studies suggest that PFO closure may have a beneficial effect on migraine. However, the first randomized, double-blind, and sham-controlled trial has failed to support such conclusion. Until there is more evidence from ongoing trials, PFO closure should not be performed in clinical practice for the treatment of migraine.

[Anatomical and neurochemical bases accounting for the frequent association between headaches and sleep: the hypnic headache paradigm]. / Bases anatomicas y neuroquimicas que explican la frecuente asociacion de las cefaleas con el sueno: el paradigma de la cefalea hipnica.

INTRODUCTION: The relationship between headache and sleep is complex and runs in two directions. Headache may be the consequence of a (primary or secondary) sleep disorder or its cause (in chronic tension-type headache and/or chronic migraine with or without painkiller abuse). It can also be related to sleep in an intrinsic way, as in the case of hypnic headache (which only appears during sleep) or other primary headaches, such as migraine and certain trigeminal-autonomic cephalgias (which can appear during the waking state or during sleep); this type of headache occurs mostly during REM sleep. DEVELOPMENT: The neural pathways that control sleep and pain are anatomically, physiologically and neurochemically cross-linked. These neural systems are located in the brain stem, the hypothalamus and the basal brain. Such cross-links are produced on two different levels. On the one hand, they occur in the serotoninergic nuclei of the brain stem, whose activity in physiologically diminished during REM sleep and which are involved in antinociceptive control. On the other hand, they are also to be found in the hypothalamus, where serotoninergic terminals have been detected in the suprachiasmatic nucleus (SCN). As cells in the SCN are lost with age, their circadian and homeostatic functioning fails, the activity of the hypothalamus-pineal axis is reduced and the endogenous melatonin rhythm is altered. This results in a disorder affecting the control of the sleep-waking cycle and antinociceptive control. CONCLUSIONS: Dysfunctions in these neuronal networks in the brain stem (especially in the serotoninergic nuclei) or the hypothalamus (SCN) can account for headaches that begin in the REM phase of sleep and affect biologically predisposed subjects.

Predictor variables for identifying patients with chronic tension-type headache who are likely to achieve short-term success with muscle trigger point therapy.

To date, no studies have investigated the predictive validity of variables from the initial examination to identify patients with tension-type headache pain who are likely to benefit from muscle trigger point (TrP) therapy. The purpose of this study was to develop a preliminary clinical prediction rule (CPR) to identify chronic tension-type headache (CTTH) patients who are likely to experience a successful response from TrP therapy. Consecutive patients with CTTH underwent a standardized examination and then received six sessions of TrP therapy over 3 weeks (two sessions per week). They were classified as having experienced a successful outcome at short-term (1 week after discharge) and 1-month follow-up based on a 50% reduction on at least one headache parameter (intensity, frequency or duration) and self-report perceived recovery. Potential predictor variables were entered into a stepwise logistic regression model to determine the most accurate set of variables for identifying treatment success. Data from 35 patients were included, of which 19 (55%) experienced a successful outcome. A CPR with four variables for short-term (headache duration < 8.5 h/day, headache frequency < 5.5 days/week, bodily pain < 47 and vitality < 47.5) and a CPR with two variables for 1-month (headache frequency < 5.5 days/week and bodily pain < 47) follow-up were identified. At short-term follow-up, if three of four variables [positive likelihood ratio (LR) 3.4] were present, the chance of experiencing a successful outcome improved from 54% to 80%, and if all the variables (positive LR 5.9) were present, the probability of success was 87.4%. At 1-month follow-up, if one of two variables (positive LR 2.2) was present, the probability of success increased from 54% to 72%, and if both variables (positive LR 4.6) were present, the probability of success was 84.4%. The present CPR provides the potential to identify CTTH patients who are likely to experience short-term and 1-month follow-up success with a muscle TrP therapy approach. Future studies are necessary to validate the CPR.

Epicrania fugax: an ultrabrief paroxysmal epicranial pain.

Ten patients (one man and nine women, mean age 48.8 +/- 20.1) presented with a stereotypical and undescribed type of head pain. They complained of strictly unilateral, shooting pain paroxysms starting in a focal area of the posterior parietal or temporal region and rapidly spreading forward to the ipsilateral eye (n = 7) or nose (n = 3) along a lineal or zigzag trajectory, the complete sequence lasting 1-10 s. Two patients had ipsilateral lacrimation, and one had rhinorrhoea at the end of the attacks. The attacks could be either spontaneous or triggered by touch on the stemming area (n = 2), which could otherwise remain tender or slightly painful between the paroxysms (n = 5). The frequency ranged from two attacks per month to countless attacks per day, and the temporal pattern was either remitting (n = 5) or chronic (n = 5). This clinical picture might be a variant of an established headache or represent a novel syndrome.

Nummular headache with trophic changes inside the painful area.

Nummular headache (NH) is a primary disorder characterized by chronic pain that is exclusively felt in a small area of the head surface. We describe five patients with circumscribed head pain and sensory dysfunction consistent with NH, who in addition developed colocalized trophic changes. All of them had a round or oval patch of skin depression (1-2 cm in diameter) inside of the painful area (2-4.5 cm in diameter). Three of them also showed hair loss, reddish colour, and local increased temperature. Skin biopsies were performed in three patients, and were not specific for any particular dermatological disease. Local trophic changes may be a clinical feature of NH. Together with pain and sensory disturbances, they could represent a restricted form of complex regional pain syndrome. This should be taken as a possible evolution of the underlying morbid process of NH.