Clinical predictors of therapeutic failure of occipital nerve stimulation in refractory chronic cluster headache.

BACKGROUND: Occipital nerve stimulation (ONS) is a treatment with evidence in refractory chronic cluster headache (CCH). However, the variable response rate and cost make it necessary to investigate predictors of response. METHODS: This is a cross-sectional study conducted through the review of medical records of CCH patients from six hospitals in Madrid. Epidemiological and clinical variables were compared between patients with ONS failure and the rest. ONS failure was defined as the need for device withdrawal or switch off because of lack of response or adverse events. RESULTS: From a series of 88 CCH, 26 (29.6%) underwent ONS surgery, of whom 13/26 (50.0%) failed because lack of response. ONS failure group had an earlier headache onset (mean ± SD) of 27.7 ± 6.9 vs. 36.7 ± 11.8 years, p = 0.026) and a higher smoking rate (100% vs. 42.9%, p = 0.006). Stational fluctuations (58.3% vs. 7.7%, p = 0.007) and nocturnal exacerbations (91.7% vs. 53.9%, p = 0.035) were more frequent in the ONS failure group as well. There was no difference between groups in diagnostic delay, years of evolution prior to surgery, mental illness, comorbidity with other headache disorders or chronic pain conditions or prior response to occipital nerves anesthetic blocks. CONCLUSIONS: Some clinical features such as an early debut, smoking and seasonal or circadian fluctuations could be related to failure of ONS in refractory CCH.

Neurotological emergencies: a narrative review.

INTRODUCTION: Vestibular symptoms, including vertigo, dizziness, and gait unsteadiness, are a frequent reason of urgent medical assistance. Their causes are numerous and diverse, including neurological, otorhinolaryngological, and systemic diseases. Therefore, following a systematic approach is essential to differentiate striking but benign conditions from others that can compromise the patient's life. This study is intended to review vestibular disorders from a practical perspective and provide guidance to physicians involved in the emergency care of patients with vestibular symptoms. MATERIALS AND METHODS: A narrative review was performed, revisiting the main causes of vestibular disorders. RESULTS: Based on the speed of onset, duration, and history of similar episodes in the past, vestibular disorders can be categorized into three syndromic entities (acute, recurrent, and chronic vestibular syndromes). The most representative conditions pertaining to each group were reviewed (including their diagnosis and treatment) and a practical algorithm was proposed for their correct management in the acute care setting. CONCLUSIONS: Carrying out a correct categorization of the vestibular disorders is essential to avoid diagnostic pitfalls. This review provides useful tools for clinicians to approach their patients with vestibular symptoms at the emergency room.

Sensitivity of the SNNOOP10 list in the high-risk secondary headache detection.

AIM: To evaluate the diagnostic accuracy of the SNNOOP10 list in the detection of high-risk headaches. METHODS: Patients that visited the Hospital Clínico San Carlos (Madrid) emergency department due to headache that were allocated to a Manchester Triage System level between critical and urgent were prospectively included but retrospectively analysed. A researcher blind to the patients' diagnosis administered a standardised questionnaire and afterwards a neurologist blind to the questionnaire results diagnosed the patient according to the International Classification of Headache Disorders. The primary endpoint was to assess the sensitivity of the SNNOOP10 list in the detection of high-risk headaches. Secondary endpoints included the evaluation of the sensitivity, specificity, positive predictive value, negative predictive value and area under the curve of each SNNOOP10 item. RESULTS: Between April 2015 and October 2021, 100 patients were included. Patients were 44 years old (inter-quartile range: 33.6-64.7) and 57% were female. We identified 37 different diagnoses. Final diagnosis was a primary headache in 33%, secondary headache in 65% and cranial neuralgia in 2%. There were 46 patients that were considered as having high-risk headache. Patients from the primary headache group were younger and more frequently female. Sensitivity of SNNOOP10 list was 100% (95% confidence interval: 90.2%-100%). The items with higher sensitivity were neurologic deficit or disfunction (75.5%), pattern change or recent onset of the headache (64.4%), onset after 50 years (64.4%). The most specific items were posttraumatic onset of headache (94.5%), neoplasm in history (89.1%) and systemic symptoms (89%). The area under the curve of the SNNOOP10 list was 0.66 (95% CI: 0.55-0.76). CONCLUSION: The red flags from the SNNOOP10 list showed a 100% sensitivity in the detection of high-risk headache disorders.

COVID-19 anosmia and gustatory symptoms as a prognosis factor: a subanalysis of the HOPE COVID-19 (Health Outcome Predictive Evaluation for COVID-19) registry.

Olfactory and gustatory dysfunctions (OGD) are a frequent symptom of coronavirus disease 2019 (COVID-19). It has been proposed that the neuroinvasive potential of the novel SARS-CoV-2 could be due to olfactory bulb invasion, conversely studies suggest it could be a good prognostic factor. The aim of the current study was to investigate the prognosis value of OGD in COVID-19. These symptoms were recorded on admission from a cohort study of 5868 patients with confirmed or highly suspected COVID-19 infection included in the multicenter international HOPE Registry (NCT04334291). There was statistical relation in multivariate analysis for OGD in gender, more frequent in female 12.41% vs 8.67% in male, related to age, more frequent under 65 years, presence of hypertension, dyslipidemia, diabetes, smoke, renal insufficiency, lung, heart, cancer and neurological disease. We did not find statistical differences in pregnant (p = 0.505), patient suffering cognitive (p = 0.484), liver (p = 0.1) or immune disease (p = 0.32). There was inverse relation (protective) between OGD and prone positioning (0.005) and death (< 0.0001), but no with ICU (0.165) or mechanical ventilation (0.292). On univariable logistic regression, OGD was found to be inversely related to death in COVID-19 patients. The odds ratio was 0.26 (0.15-0.44) (p < 0.001) and Z was - 5.05. The presence of anosmia is fundamental in the diagnosis of SARS.CoV-2 infection, but also could be important in classifying patients and in therapeutic decisions. Even more knowing that it is an early symptom of the disease. Knowing that other situations as being Afro-American or Latino-American, hypertension, renal insufficiency, or increase of C-reactive protein (CRP) imply a worse prognosis we can make a clinical score to estimate the vital prognosis of the patient. The exact pathogenesis of SARS-CoV-2 that causes olfactory and gustative disorders remains unknown but seems related to the prognosis. This point is fundamental, insomuch as could be a plausible way to find a treatment.

[Lymphocytic hypophysitis and hypertrophic pachymeningitis: description of a possible case associated to IgG4 pathologies]. / Hipofisitis linfocitaria y paquimeningitis hipertrófica: descripción de un posible caso asociado a las patologías IgG4.

INTRODUCTION: Pathologies related to lymphocyte infiltration and inflammatory cells are classified, according to their topography, into idiopathic orbital pathology, idiopathic hypertrophic pachymeningitis, Tolosa-Hunt syndrome and lymphocytic hypophysitis. Here we review the case of a female patient who began with lymphocytic hypophysitis eight years ago and has since presented several episodes of hypertrophic pachymeningitis in a variety of locations. CASE REPORT: Our study involves a 55-year-old female, being monitored owing to a two-month history of headaches, who was admitted to hospital as the result of a deterioration of her clinical signs and symptoms with paralysis of the whole right-side third cranial nerve, watery eyes and rhinorrhea. A magnetic resonance brain scan revealed the presence of a sellar lesion consistent with lymphocytic hypophysitis. Treatment was established with corticoids with an improvement at first, but two years later the patient's clinical features worsened, with headaches and periocular paraesthesias on the right-hand side. Another MRI scan was performed and several extra-axial lesions were observed, the results of the other studies being normal. Idiopathic hypertrophic pachymeningitis was therefore diagnosed and treatment with corticoids was started again. In the ensuing follow-up the patient presented new lesions in different locations and poor pain control, and so immunomodulator treatment was thus added. Given the association between lymphocytic hypophysitis and hypertrophic pachymeningitis, the study was completed with determination of the IgG4 in serum, which was negative. CONCLUSIONS: The association of lymphocytic hypophysitis and hypertrophic pachymeningitis with IgG4-related pathologies have recently been reported. Although the diagnosis has not been confirmed in our patient, this relationship must be taken into account in these idiopathic cases and, above all, if they are associated with other systemic manifestations.

TITLE: Hipofisitis linfocitaria y paquimeningitis hipertrofica: descripcion de un posible caso asociado a las patologias IgG4.Introduccion. Las patologias relacionadas con la infiltracion de linfocitos y celulas inflamatorias se clasifican, segun su topografia, en patologia orbitaria idiopatica, paquimeningitis hipertrofica idiopatica, sindrome de Tolosa-Hunt e hipofisitis linfocitaria. Presentamos a una paciente que comenzo con una hipofisitis linfocitaria hace ocho años y ha presentado varios episodios de paquimeningitis hipertrofica variables en su localizacion. Caso clinico. Mujer de 55 años, en seguimiento por cefalea de dos meses de evolucion, que ingreso por empeoramiento clinico con paralisis del III par derecho completo, lagrimeo y rinorrea. Se realizo una resonancia magnetica cerebral que demostro la presencia de una lesion sellar compatible con hipofisitis linfocitaria. Se inicio tratamiento con corticoides con mejoria inicial, pero, tras dos años, la paciente presento empeoramiento clinico con cefalea y parestesias perioculares derechas. Se repitio la resonancia, donde se observaron varias lesiones extraaxiales, siendo el resto de estudios normales, por lo que se diagnostico paquimeningitis hipertrofica idiopatica y se inicio tratamiento de nuevo con corticoides. En el seguimiento posterior, la paciente presento nuevas lesiones en diferentes localizaciones y mal control algico, por lo que se añadio tratamiento inmunomodulador. Ante la asociacion de hipofisitis linfocitaria y paquimeningitis hipertrofica, se completo el estudio con determinacion de la IgG4 en el suero, que resulto negativa. Conclusiones. La asociacion de hipofisitis linfocitaria y paquimeningitis hipertrofica con las patologias relacionadas con la IgG4 se ha descrito recientemente. A pesar de que en nuestra paciente no se ha confirmado el diagnostico, se debe considerar esta relacion en aquellos casos idiopaticos y, sobre todo, si se asocian otras manifestaciones sistemicas.

[Neurological examination in patients undergoing sedation with propofol: a descriptive study]. / Exploracion neurologica en pacientes sometidos a sedacion con propofol: estudio descriptivo.

INTRODUCTION: The neurological examination is a resource used in evaluating patients who are in coma. Anaesthesia can be a factor that exerts an influence on the findings. We evaluated the examination of the comatose patient in the case of patients anaesthetised with propofol in order to define its clinical value. PATIENTS AND METHODS: A prospective study was conducted among those who had undergone heart surgery in the intensive care unit of a tertiary hospital during the months of April and May 2011. Aspects that were analysed were the examination of the anaesthetic coma, pharmacological or medical factors that can have an influence and a full neurological examination following recovery. Patients with previous neurological symptoms were excluded. RESULTS: Thirty patients were selected (16 males and 14 females); mean age: 72 ± 10 years. All the patients were sedated with propofol. During sedation, 17 (46.7%) presented unreactive pupils. No spontaneous eye movements were observed in 100% of the sample. Ocular alterations in primary position were observed in 23.3% of them. Oculocephalic reflexes were absent in 93.3% and oculovestibular reflexes, in 100%; the corneal reflex, was absent in 70% (with asymmetry), as was the ciliospinal reflex, in 83.3%. The algesic motor response was absent in 93.3%, as were the cutaneous plantar extensor reflexes, in 20%; and 66.7% were indifferent. Following anaesthesia, the neurological examination was normal in 80%, there were slight orientation impairments in 16.7% and a hemispheric syndrome was found in one patient. CONCLUSIONS: Anaesthesia with propofol alters the reversible cutaneous-plantar, pupillary, trunk and motor response reflexes on withdrawing sedation. The alterations may be asymmetrical. Neurological examinations should not be used in the case of patients sedated with propofol in order to make clinical decisions.

TITLE: Exploracion neurologica en pacientes sometidos a sedacion con propofol: estudio descriptivo.Introduccion. La exploracion neurologica es un recurso en la evaluacion de los pacientes en coma. La anestesia puede ser un factor influyente en los hallazgos. Evaluamos la exploracion del paciente en coma en pacientes anestesiados con propofol para definir su valor clinico. Pacientes y metodos. Estudio prospectivo en sometidos a cirugia cardiaca en los cuidados intensivos de un hospital terciario durante los meses de abril y mayo de 2011. Se analiza la exploracion del coma anestesico, factores farmacologicos o medicos que pueden influir y exploracion neurologica completa tras la recuperacion. Se excluyeron pacientes con sintomas neurologicos previos. Resultados. Se seleccionan 30 pacientes (16 hombres y 14 mujeres); edad media: 72 ± 10 años. Todos los pacientes estaban sedados con propofol. Durante la sedacion, 17 (46,7%) presentaban pupilas arreactivas. En el 100% no se observaron movimientos espontaneos de los ojos. El 23,3% presentaba alteracion ocular en posicion primaria. Los reflejos oculocefalicos estaban ausentes en el 93,3%, y los oculovestibulares, en el 100%; el reflejo corneal, en el 70% (con asimetria); el espinociliar, en el 83,3%. La respuesta motora algesica estaba ausente en el 93,3%; en un 20%, los reflejos cutaneoplantares extensores; e indiferentes, en el 66,7%. Tras la anestesia, la exploracion neurologica fue normal en el 80%, hubo leves alteraciones de orientacion en un 16,7% y se encontro un sindrome hemisferico en un paciente. Conclusiones. La anestesia con propofol altera los reflejos pupilares, del tronco, respuestas motoras y reflejo cutaneoplantar reversibles al retirar la sedacion. Las alteraciones pueden ser asimetricas. No se deberia utilizar la exploracion neurologica en el paciente sedado con propofol para la toma de decisiones clinicas.

Extreme delta brush in a patient with anti-NMDAR encephalitis.

Anti-N-methyl-D-aspartate receptor encephalitis is a severe, potentially treatable, disorder and prognosis depends on early recognition and prompt immunotherapy. We report a case of anti-N-methyl-D-aspartate receptor encephalitis with atypical age and gender, and a characteristic electroencephalographic pattern that supported the diagnosis. A 66-year-old male presented with psychiatric disturbances and focal seizures with alteration of consciousness, and progressed to a state of akinetic mutism. Auxiliary tests were negative or non-specific for anti-NMDAR encephalitis. Electroencephalographic monitoring revealed a unique pattern; the extreme delta brush. The patient improved with immunotherapy and was asymptomatic at six months of follow-up. Ancillary testing was positive for anti-N-methyl-D-aspartate receptor antibodies. Extreme delta brush is a recently described electroencephalographic pattern presenting in only one third of patients with anti-N-methyl-D-aspartate receptor encephalitis. The identification of this pattern, as in our case, may guide early diagnosis and treatment of anti-N-methyl-D-aspartate receptor encephalitis.

Dural cavernous haemangioma of the anterior cranial fossa.

Extra-axial cavernous haem angiomas are uncommon lesions histologically identical to cavernomas in other locations. However, their radiological features and clinical behaviour may differ. They are frequently misdiagnosed preoperatively, as they often mimic other tumours. We describe a patient suffering from loss of the sense of smell, due to a cavernous haemangioma implanted in the dura mater of the anterior cranial fossa close to the olfactory bulb. To our knowledge, this is the first patient reported with such a lesion.

Paroxysmal head pain with backward radiation: will epicrania fugax go in the opposite direction?

Epicrania fugax (EF) has been recently described as a paroxysmal head pain starting in a focal cranial area of the posterior scalp and rapidly spreading forward to the ipsilateral eye or nose along a linear or zigzag trajectory. Here we report two patients presenting with the same clinical features, except for the starting site and the direction of the pain. Unilateral pain paroxysms occurred on either side of the head, with a quick backward radiation along a linear trajectory. The pain always stemmed from a particular point located at the fronto-parietal region, and reached the parieto-occipital region in several seconds. The symptoms did not fit any of the acknowledged headaches and neuralgias, and might correspond to a reverse variant of EF.

Venous angioma associated with atypical ophthalmoplegic migraine.

BACKGROUND: Ophthalmoplegic migraine is a rare syndrome in which episodic headaches are associated with ophthalmoplegia. Several recent reports emphasize the possibility, especially in atypical cases, of a heterogeneous type of ophthalmoplegic migraine. METHODS: We describe the case of an 18-year-old woman with recurrent episodic headache accompanied by binocular diplopia due to left third cranial nerve palsy. The symptoms resolve in hours. A diagnosis of atypical ophthalmoplegic migraine was established. RESULTS: Digital angiography revealed a venous angioma draining into the left cavernous sinus. CONCLUSIONS: This case reveals the importance of differential diagnosis in atypical migraine in which the symptomatology could be secondary to intracranial lesions. We propose venous stasis as a cause of symptoms.