Non-late-onset neutropaenia following treatment of multiple sclerosis with ocrelizumab.

Late-onset neutropaenia is defined as an absolute neutrophil count of <1.5×103cells/µL starting>4 weeks after the last dose of rituximab, in the absence of other identifiable causes. Late-onset neutropaenia is a rare adverse reaction to rituximab (observed in approximately 5% of patients). Rheumatic diseases constitute the main indication for rituximab; in these patients, neutropaenia appears after a mean of>28 days. Ocrelizumab is another monoclonal antibody that binds to CD20 (a glycosylated phosphoprotein mainly expressed on the membranes of B-lymphocytes); in January 2018, it was approved for the treatment of relapsing-remitting and primary progressive multiple sclerosis. We present a case of neutropaenia following intravenous infusion of ocrelizumab in a patient with primary progressive multiple sclerosis who presented with neutropaenic fever, herpetic stomatitis, and ecthyma gangrenosum only 20 days after infusion.

Non-late-onset neutropaenia following treatment of multiple sclerosis with ocrelizumab / Neutropenia de aparición no tardía tras el tratamiento con ocrelizumab para la esclerosis múltiple

Late-onset neutropaenia is defined as an absolute neutrophil count of <1.5 × 103 cells/μL starting > 4 weeks after the last dose of rituximab, in the absence of other identifiable causes.Late-onset neutropaenia is a rare adverse reaction to rituximab (observed in approximately 5% of patients). Rheumatic diseases constitute the main indication for rituximab; in these patients, neutropaenia appears after a mean of > 28 days.Ocrelizumab is another monoclonal antibody that binds to CD20 (a glycosylated phosphoprotein mainly expressed on the membranes of B-lymphocytes); in January 2018, it was approved for the treatment of relapsing-remitting and primary progressive multiple sclerosis.We present a case of neutropaenia following intravenous infusion of ocrelizumab in a patient with primary progressive multiple sclerosis who presented with neutropaenic fever, herpetic stomatitis, and ecthyma gangrenosum only 20 days after infusion.(AU)

La neutropenia de aparición tardía se define como un recuento absoluto de neutrófilos < 1,5 × 103/μl que se produce > 4 semanas después de la última dosis de rituximab, precedido por un recuento de neutrófilos normal y sin otra causa identificable. Es una complicación rara del tratamiento con rituximab, habiéndose observado en aproximadamente el 5% de los pacientes tratados, siendo las enfermedades reumáticas su principal indicación, con un tiempo medio hasta el desarrollo de la neutropenia de al menos 28 días. El ocrelizumab, al igual que el rituximab, es un anticuerpo monoclonal dirigido a CD20, una fosfoproteína glicosilada de membrana que se encuentra predominantemente en los linfocitos B y que se aprobó en enero de 2018 para el tratamiento de la esclerosis múltiple remitente recurrente y la esclerosis múltiple progresiva primaria. Se describe un caso de neutropenia después de la infusión de ocrelizumab en un paciente con esclerosis múltiple progresiva primaria que presentó neutropenia febril, estomatitis herpética y ectima gangrenoso solo 20 días después de la infusión.(AU)

Non-late-onset neutropaenia following treatment of multiple sclerosis with ocrelizumab.

Late-onset neutropaenia is defined as an absolute neutrophil count of <1.5×103cells/µL starting>4 weeks after the last dose of rituximab, in the absence of other identifiable causes. Late-onset neutropaenia is a rare adverse reaction to rituximab (observed in approximately 5% of patients). Rheumatic diseases constitute the main indication for rituximab; in these patients, neutropaenia appears after a mean of>28 days. Ocrelizumab is another monoclonal antibody that binds to CD20 (a glycosylated phosphoprotein mainly expressed on the membranes of B-lymphocytes); in January 2018, it was approved for the treatment of relapsing-remitting and primary progressive multiple sclerosis. We present a case of neutropaenia following intravenous infusion of ocrelizumab in a patient with primary progressive multiple sclerosis who presented with neutropaenic fever, herpetic stomatitis, and ecthyma gangrenosum only 20 days after infusion.

Focalidad neurológica como primera manifestación de un mixoma cardiaco / Neurological focal signs as a first manifestation of cardiac myxoma

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Meningoencefalitis focal de causa autoinmune / Autoimmune-induced focal meningoencephalitis

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Fístula de LCR en una mujer con antecedente de hipertensión intracraneal idiopática / CSF fistula in a woman with a history of idiopathic intracranial hypertension

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[Unilateral papillitis as the presenting symptom of neurosarcoidosis]. / Papilitis unilateral como presentación de neurosarcoidosis.

INTRODUCTION: Sarcoidosis is a granulomatous, multisystemic disease, of unknown etiology. Its prevalence is low, 40 cases by 100,000 inhabitants in Northern European countries. Respiratory symptoms are the most common, however this disease can affect other vital systems. Neurooftalmological manifestations have been reported one out of five cases, such as uveitis, cranial nerves palsy, and choroiditis; papillitis is seldom described. When initial symptoms of sarcoidosis are neurological features (less than 5%), diagnosis is challenging. CASE REPORTS: We present two cases of neurosarcoidosis in our hospital, during the last two years, in patients without previous diagnosis, which came for loss of visual acuity. The diagnostic was achieved by magnetic resonanace white matter lesions hyperintenses in T2, computed tomography thoracic mediastinic adenopathy and elevated determinations of angiotensin-converting enzyme, according to diagnostic criteria of neurosarcoidosis published in 2004. CONCLUSIONS: We propose to include neurosarcoidosis among known etiologies of unilateral papilitis. Since that, determination of angiotensin-converting enzyme should be performed in those cases.

Papilitis unilateral como presentación de neurosarcoidosis / Unilateral papillitis as the presenting symptom of neurosarcoidosis

La sarcoidosis es una enfermedad multisistémica granulomatosa, más frecuente en mujeres, con unaprevalencia de 40 casos por 100.000 habitantes y una clínica preferentemente pulmonar. La afectación neurooftalmológica se da en el 15-25% de los pacientes y la clínica habitual es la parálisis de los pares craneales, la uveítis y la coroiditis. El iniciode esta enfermedad con clínica neurológica apenas se da en el 5% de los pacientes con sarcoidosis. Casos clínicos. Presentamos dos casos de neurosarcoidosis en nuestro centro en los últimos dos años, en pacientes sin diagnóstico sistémico previo, que consultaron por una disminución de la agudeza visual. En la resonancia magnética craneal se encontraron lesiones en lasustancia blanca, hiperintensas en la secuencia T2; la tomografía computarizada de tórax mostró adenopatías mediastínicas y las determinaciones de la enzima convertidora de angiotensina estuvieron por encima de la normalidad. Se diagnosticó a los pacientes mediante criterios clínicos publicados en 2004. Conclusiones. Destacamos la importancia de incluir la neurosarcoidosisen el diagnóstico diferencial de la papilitis unilateral, así como la determinación de la enzima convertidora de angiotensina como parte de su estudio

Sarcoidosis is a granulomatous, multisystemic disease, of unknown etiology. Its prevalence is low, 40cases by 100.000 inhabitants in Northern European countries. Respiratory symptoms are the most common, however this disease can affect other vital systems. Neurooftalmological manifestations have been reported one out of five cases, such as uveitis, cranial nerves palsy, and choroiditis; papillitis is seldom described. When initial symptoms of sarcoidosis areneurological features (less than 5%), diagnosis is challenging. Case reports. We present two cases of neurosarcoidosis in our hospital, during the last two years, in patients without previous diagnosis, which came for loss of visual acuity. The diagnosticwas achieved by magnetic resonanace white matter lesions hyperintenses in T2, computed tomography thoracic mediastinic adenopathy and elevated determinations of angiotensin-converting enzyme, according to diagnostic criteria of neurosarcoidosis published in 2004. Conclusions. We propose to include neurosarcoidosis among known etiologies of unilateral papilitis. Sincethat, determination of angiotensin-converting enzyme should be performed in those cases

[Alzheimer's disease, category-specific impairment and relevant variables in object naming]. / Enfermedad de Alzheimer, deterioro categorial y variables relevantes en la denominación de objetos.

INTRODUCTION: The study of the dissociations or category-specific effects between the domains of living beings and non-living beings in Alzheimer's disease (AD) is a controversial issue in the cognitive neurosciences. The lack of agreement among the different studies may be due to deficient control of certain cognitive and psycholinguistic variables that affect processing of the items. AIM: To determine whether the presence of category-specific effects in AD can be caused by inadequate control of variables, such as the typicality or familiarity of the items. Furthermore, since the groups may contain different types of patients with opposing impairments (which would mask this kind of effect in the group analysis), both group and individual analyses were conducted. SUBJECTS AND METHODS: A retrospective study was carried out to evaluate 66 participants (32 patients with AD) using a colour photo naming task with items controlled for seven disruptive variables. RESULTS: No evidence of living/non-living dissociation was found in the analyses by groups, although the individual-based analysis did show some cases of category-specific effects. CONCLUSIONS: Our data show that category-specific effects are not as widespread as they were believed to be and that the lack of control over the so-called disturbing variables may play an important role in studies on category-specific impairment. Our study also highlights the importance of conducting individual analyses in order to avoid overlooking certain effects that are masked in the group studies.

Enfermedad de Alzheimer, deterioro categorial y variables relevantes en la denominación de objetos / Alzheiher´s disease, category-specific impairment and relevant variables in object naming

Introducción. El estudio de las disociaciones o efectos categoriales entre los dominios de seres vivos y seres novivos en la enfermedad de Alzheimer (EA) es un tema polémico para las neurociencias cognitivas. La falta de acuerdo entrelos estudios puede deberse a un deficiente control sobre determinadas variables cognitivas y psicolingüísticas que influyen enel procesamiento de los ítems. Objetivo. Verificar si en la EA la presencia de efectos categoriales puede deberse a un controlinadecuado de variables como la tipicidad o la familiaridad de los ítems. Además, dado que en los grupos puede haber tiposde pacientes con deterioros opuestos –que enmascararían esta clase de efectos en los análisis de grupo– se realizaron análisisde grupo e individuales. Sujetos y métodos. Se evaluó a 66 participantes (32 pacientes con EA) de forma retrospectiva conuna tarea de denominación de fotografías en color con ítems controlados en siete variables perturbadoras. Resultados. No sehalló evidencia de disociación vivo/no vivo al realizar análisis de grupo, aunque el análisis individualizado mostró algunoscasos de efectos categoriales. Conclusiones. Nuestros datos muestran que los efectos categoriales no son tan generales comose pensaba y que la falta de control de las llamadas variables perturbadoras puede desempeñar una función importante en losestudios sobre deterioro categorial. Además, se verifica la importancia de realizar análisis individuales que impidan pasarpor alto algunos efectos enmascarados por los estudios de grupo

Introduction. The study of the dissociations or category-specific effects between the domains of living beings andnon-living beings in Alzheimer’s disease (AD) is a controversial issue in the cognitive neurosciences. The lack of agreementamong the different studies may be due to deficient control of certain cognitive and psycholinguistic variables that affectprocessing of the items. Aim. To determine whether the presence of category-specific effects in AD can be caused by inadequatecontrol of variables, such as the typicality or familiarity of the items. Furthermore, since the groups may containdifferent types of patients with opposing impairments (which would mask this kind of effect in the group analysis), both groupand individual analyses were conducted. Subjects and methods. A retrospective study was carried out to evaluate 66 participants(32 patients with AD) using a colour photo naming task with items controlled for seven disruptive variables. Results. Noevidence of living/non-living dissociation was found in the analyses by groups, although the individual-based analysis didshow some cases of category-specific effects. Conclusions. Our data show that category-specific effects are not as widespreadas they were believed to be and that the lack of control over the so-called disturbing variables may play an important role instudies on category-specific impairment. Our study also highlights the importance of conducting individual analyses in orderto avoid overlooking certain effects that are masked in the group studies

Hypnic headache associated with stage 3 slow wave sleep.

We describe the polysomnographic data of a 79-year-old woman with an 11-year history of nocturnal headaches that were clinically consistent with hypnic headache. A polysomnographic study showed arousal at stage 3 slow wave sleep because of a headache episode. Although this finding could be nonspecific, it suggests the possible relationship between stage 3 slow wave sleep and hypnic headache.

[Respiratory form of onset of motor neuron disease]. / Forma de comienzo respiratorio de la enfermedad de motoneurona.

OBJECTIVE: To present a case of respiratory failure as the form of onset of amyotrophic lateral sclerosis, to review the main clinical findings, data of the investigations done which suggest the presence of this disorder and describe its therapeutic management. CLINICAL CASE: A 68 year old man presented with a subacute illness characterized by a sleep disorder with sleep fragmentation, snoring of increasing intensity, without clear pauses of apnea, progressive diurnal hypersomnia accompanied by progressive dyspnea followed by respiratory failure with respiratory acidosis and difficulty in manipulating things with his hands. Diagnostic investigations showed a restrictive pattern without pulmonary fibrosis, due to paralysis of the diaphragm, and the presence of electromyographic signs compatible with motorneuron disease. The patient was treated with riluzole 100 mg/day and non-invasive mechanical ventilation and maintained an acceptable quality of life. CONCLUSIONS: Motorneuron disease may start with acute or progressive respiratory failure without a clear etiological cause and may appear to be similar to obstructive sleep apnea syndrome. The treatment of choice for this respiratory problem is non-invasive mechanical ventilation. Absence of symptoms of bulbar involvement is essential for a favourable prognosis.

Serum levels of coenzyme Q10 in patients with multiple sclerosis.

To elucidate whether serum coenzyme Q10 levels are related with the risk for multiple sclerosis (MS) or are a marker for the activity of the disease, we compared serum levels of coenzyme Q10 and the coenzyme Q10/cholesterol ratio, in 31 patients with MS (during exacerbations) and 19 matched controls using a high performance liquid chromatography technique. The mean serum coenzyme Q10 levels and the coenzyme Q10/cholesterol ratio did not differ significantly between the 2 study groups. The values did not correlate with age, age at onset, and duration of the disease. These results suggest that serum coenzyme Q10 concentrations are unrelated with the risk for MS and are not a useful marker of activity of the disease.

Forma de comienzo respiratorio de la enfermedad de motoneurona / Respiratory form of onset of motorneuron disease

Objetivo. Presentar un caso de insuficiencia como forma de comienzo de la esclerosis lateral amiotrófica, así como revisar sus manifestaciones clínicas principales, los datos de pruebas diagnósticas sugestivos de esta enfermedad y su manejo terapéutico. Caso clínico. Varón de 68 años que presentó cuadro subagudo caracterizado por la aparición de trastorno del sueño con fragmentaciones del mismo y ronquidos crecientes en intensidad, sin claras pausa de apnea, junto con hipersomnia diurna progresiva, acompañado de disnea progresiva y posterior fallo respiratorio con acidosis respiratoria y de dificultad manipulatoria en manos. Los tests diagnósticos objetivaron un patrón restrictivo sin la presencia de fibrosis pulmonar debido a parálisis diafragmática y a la presencia de signos electromiográficos compatibles con enfermedad de motoneurona. El paciente recibió tratamiento con riluzole 100 mg/día y ventilación mecánica no invasiva manteniendo una calidad de vida aceptable. Conclusiones. La enfermedad de motoneurona puede comenzar como un cuadro de insuficiencia respiratoria aguda o progresiva sin una causa etiológica clara, pudiendo simular un síndrome de apnea obstructiva del sueño. El tratamiento de elección para su problema respiratorio sería la ventilación mecánica no invasiva, siendo fundamental para un pronóstico más favorable la ausencia de síntomas de afectación bulbar (AU)

Respiratory chain enzyme activities in spermatozoa from untreated Parkinson's disease patients.

We studied respiratory chain enzyme activities in spermatozoa homogenates from 12 untreated Parkinson's disease (PD) male patients and from 23 age matched healthy male controls. When compared with controls, PD patients showed significantly lower specific activities for complexes I+ III, II+III, and IV. However, citrate synthase corrected activities were similar in patients and controls. Values for enzyme activities in the PD group did not correlate with age at onset, duration, scores of the Unified Parkinson's Disease Rating Scales and Hoehn and Yahr staging. These results suggest that this tissue cannot be used to develop a diagnostic test for PD.

Serum levels of beta-carotene, alpha-carotene and vitamin A in patients with Alzheimer's disease.

To elucidate the possible role of carotenoids and vitamin A as risk factors for Alzheimer's disease (AD), we compared serum levels of beta-carotene and alpha-carotene, and vitamin A, measured by isocratic high performance liquid chromatography, of 38 AD patients and 42 controls. The serum levels of alpha-carotene did not differ significantly between AD patients and control groups. However, the serum levels of beta-carotene and vitamin A were significantly lower in the AD-patient group. These values did not correlate to age, age at onset or score on the MiniMental State Examination. Weight and body mass index were significantly lower in AD patients than in controls. These results suggest that low serum beta-carotene concentrations in AD patients could be related to a deficiency in dietary intake of this provitamin, although its possible relationship with risk for AD could not be excluded.