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.

[Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (Kufs' disease)]. / Síndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs).

AIMS: The purpose of this paper is to report the case of a patient with Kluver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs' disease) and to review the literature dealing with the causes of this syndrome. CASE REPORT: A 38-year-old male examined because of behavioural changes and cognitive impairment. Brain biopsy findings were characteristic of adult-type ceroid lipofuscinosis. This patient fulfilled the criteria of Kufs' disease, since he had mixed clinical features belonging to both type A (neuropsychiatric disorders) and B (aphasia-apraxia-agnosia syndrome) of the disease. The initial symptoms included several clinical features of Klüver-Bucy syndrome (probable visual agnosia, apathy, increased sexual activity, lack of sexual inhibition, hypermetamorphopsia, increased oral behaviour and changes in dietary habits). CONCLUSIONS: Adult-type ceroid lipofuscinosis is an infrequent clinical entity that is difficult to diagnose owing to the absence of peripheral biological markers and the need to confirm such a diagnosis by means of a histopathological study.

Síndrome de Klüver-Bucy como manifestación inicial de ceroidolipofuscinosis del adulto (enfermedad de Kufs) / Klüver-Bucy syndrome as the initial symptom of adult-type ceroid lipofuscinosis (kufs’ disease)

Objetivos. Presentar un paciente con síndrome de Klüver-Bucy causado por ceroidolipo fuscinosis del adulto (enfermedad de Kufs) y revisar la literatura sobre a las causas de dicho síndrome. Caso clínico. Se trata de un varón de 38 años de edad valorado por cambios conductuales y deterioro cognitivo. La biopsia cerebral fue característica de ceroidolipo fuscinosis del adulto. Este paciente cumplía los criterios de la enfermedad de Kufs definida, ya que presentaba características clínicas mixtas de los tipos A (alteraciones neuropsiquiátricas) y B (síndrome afaso-apractoagnósico) de dicha enfermedad. Los síntomas iniciales incluyeron varias características clínicas del síndrome de Klüver-Bucy (probable agnosia visual, apatía, aumento de la actividad sexual, falta de inhibición sexual, hiper-metamorfopsia, aumento de la conducta oral y cambios en los hábitos dietéticos). Conclusiones. La ceroidolipo fuscinosis del adulto es una entidad infrecuente y de difícil diagnóstico dada la ausencia de marcadores biológicos periféricos y la necesidad de la confirmación de dicho diagnóstico mediante un estudio histopatológico

Aims. The purpose of this paper is to report the case of a patient with Klüver-Bucy syndrome caused by adult-type ceroid lipofuscinosis (Kufs’ disease) and to review the literature dealing with the causes of this syndrome. Case report. A38-year-old male examined because of behavioural changes and cognitive impairment. Brain biopsy findings were characteristic of adult-type ceroid lipofuscinosis. This patient fulfilled the criteria of Kufs’ disease, since he had mixedclinical features belonging to both type A (neuropsychiatric disorders) and B (aphasia-apraxia-agnosia syndrome) of the disease. The initial symptoms included several clinical features of Klüver-Bucy syndrome (probable visual agnosia, apathy, increased sexual activity, lack of sexual inhibition, hypermetamorphopsia, increased oral behaviour and changes in dietaryhabits). Conclusions. Adult-type ceroid lipofuscinosis is an infrequent clinical entity that is difficult to diagnose owing to the absence of peripheral biological markers and the need to confirm such a diagnosis by means of a histopathological study

[Guillain-Barre syndrome following resection of glioblastoma multiforme]. / Síndrome de Guillain-Barré tras resección de glioblastoma multiforme.

INTRODUCTION: Guillain-Barre syndrome (GBS) is an acute demyelinating polyneuropathy characterised by progressive muscular weakness and areflexia. Although the pathogenesis is uncertain, it is probably secondary to an aberrant immunological response to components of the peripheral nervous system. GBS has been linked to bacterial or viral infections, systemic diseases, neoplasias, pregnancy, traumatic injuries or organ transplant. An association with intracranial surgery has been reported, but this is exceptional. CASE REPORT: We report the case of a 67-year-old male submitted to surgical intervention due to a right-side occipital-parietal glioblastoma. The patient was well until ten days after the intervention, when he presented a rapidly progressive are flexive paraparesis with an ascending course. A spinal tap revealed cerebrospinal liquid with an elevated protein level and albuminocytologic dissociation. The electromyogram study and lumbar magnetic resonance with contrast confirmed the suspected diagnosis of GBS. After establishing treatment with immunoglobulins the patient progressed well and recovered quickly and completely from the paresis. CONCLUSIONS: GBS may appear after intracranial surgery and make it more difficult to reach a differential diagnosis with other entities. To optimise the prognosis, treatment must begin as early as possible. Intravenous immunomodulation with immunoglobulins or plasma exchange are effective in shortening the course of the disease.

Síndrome de Guillain-Barré tras resección de glioblastoma multiforme / Guillain-Barre síndrome following resection of gliobastoma multiforme

Introducción. El síndrome de Guillain-Barré (SGB) consiste en una polineuropatía desmielinizante aguda caracterizada por debilidad muscular y arreflexia progresivas. Aunque la patogénesis es incierta, probablemente es secundaria a una respuesta inmunológica aberrante frente a componentes del sistema nervioso periférico. El SGB se ha relacionado con infecciones bacterianas o virales, enfermedades sistémicas, neoplasias, embarazo, trauma o trasplante de órganos. Se ha descrito la asociación con cirugía intracraneal, pero es excepcional. Caso clínico. Se trata de un varón de 67 años intervenido de un glioblastoma parietooccipital derecho. Se encontraba previamente bien, pero diez días después de la cirugía presentó una paraparesia arrefléctica rápidamente progresiva y de curso ascendente. La punción lumbar mostró un líquido con aumento proteico y disociación albuminocitológica. El estudio de electromiografía y la resonancia magnética lumbar con contraste confirmaron la sospecha diagnóstica de SGB. Después de instaurar un tratamiento con inmunoglobulinas, el paciente experimentó una buena evolución, con rápida y completa recuperación de la paresia. Conclusiones. El SGB puede aparecer tras la cirugía intracraneal y dificultar el diagnóstico diferencial con otras entidades. Para optimizar el pronóstico, el tratamiento debe comenzar lo antes posible. La inmunomodulación intravenosa con inmunoglobulinas o recambio de plasma acorta el curso de la enfermedad de manera efectiva (AU)

Introduction. Guillain-Barre syndrome (GBS) is an acute demyelinating polyneuropathy characterised by progressive muscular weakness and areflexia. Although the pathogenesis is uncertain, it is probably secondary to an aberrant immunological response to components of the peripheral nervous system. GBS has been linked to bacterial or viral infections, systemic diseases, neoplasias, pregnancy, traumatic injuries or organ transplant. An association with intracranial surgery has been reported, but this is exceptional. Case report. We report the case of a 67-year-old male submitted to surgical intervention due to a right-side occipital-parietal glioblastoma. The patient was well until ten days after the intervention, when he presented a rapidly progressive areflexive paraparesis with an ascending course. A spinal tap revealed cerebrospinal liquid with an elevated protein level and albuminocytologic dissociation. The electromyogram study and lumbar magnetic resonance with contrast confirmed the suspected diagnosis of GBS. After establishing treatment with immunoglobulins the patient progressed well and recovered quickly and completely from the paresis. Conclusions. GBS may appear after intracranial surgery and make it more difficult to reach a differential diagnosis with other entities. To optimise the prognosis, treatment must begin as early as possible. Intravenous immunomodulation with immunoglobulins or plasma exchange are effective in shortening the course of the disease (AU)

[Orthostatic tremor induced by pharmaceuticals]. / Temblor ortostático inducido por fármacos.

INTRODUCTION: Dopamine blocking agents can induce gravel types of 'tardive syndromes' (buccolinguomasticatory syndrome, dystonia, akathisia, and less frequently tremor, tourettism, and myoclonus). To our knowledge, orthostatic tremor has not been previously described as a complication of exposure to these drugs. CASE REPORTS: We report four patients who developed orthostatic tremor after exposure to dopamine blocking drugs. Two of them had orthostatic tremor as the predominant but not exclusive type of tremor, and the other two had 'pure' high-frequency orthostatic tremor. Tremor disappeared completely in 3 patients and improved markedly in the other one after gradual withdrawal of the offending drugs (metoclopramide in case 1, sulpiride and thyethylperazine in case 2, and sulpiride in cases 3 and 4). CONCLUSIONS: We propose that this 'tardive orthostatic tremor' could be considered into the spectrum of drug-induced movement disorders.

Temblor ortostático inducido por fármacos / Orthostatic tremor induced by pharmaceuticals

Introducción. Los agentes bloqueadores de los receptores dopaminérgicos pueden inducir distintos tipos de síndromes tardíos (síndrome bucolinguomasticatorio, distonía, acatisia y menos frecuentemente temblor, síndrome de Tourette y mioclonías).En nuestro conocimiento, el temblor ortostático no se había descrito previamente como complicación de exposición a fármacos. Casos clínicos. Presentamos cuatro pacientes que desarrollaron temblor ortostático después de la exposición a agentes bloqueadores dopaminérgicos. Dos de ellos tenían temblor ortostático predominante, pero también otros tipos de temblor, y los otros dos tenían un temblor ortostático `puro' de alta frecuencia. El temblor mejoró claramente en tres de estos pacientes y desapareció por completo en otro al suspender progresivamente el tratamiento con los fármacos señalados (metoclopramida en el caso 1, sulpiride y tietilperacina en el caso 2 y sulpiride en los casos 3 y 4). Conclusiones. Proponemos que el `temblor ortostático tardío' debería considerarse como un trastorno de movimiento inducido por fármacos (AU)

Introduction. Dopamine blocking agents can induce gravel types of ‘tardive syndromes’ (buccolinguomasticatory syndrome, dystonia, akathisia, and less frequently tremor, tourettism, and myoclonus). To our knowledge, orthostatic tremor has not been previously described as a complication of exposure to these drugs. Case reports. We report four patients who developed orthostatic tremor after exposure to dopamine blocking drugs. Two of them had orthostatic tremor as the predominant but not exclusive type of tremor, and the other two had ‘pure’ high-frequency orthostatic tremor. Tremor disappeared completely in 3 patients and improved markedly in the other one after gradual withdrawal of the offending drugs (metoclopramide in case 1, sulpiride and thyethylperazine in case 2, and sulpiride in cases 3 and 4). Conclusions. We propose that this ‘tardive orthostatic tremor’ could be considered into the spectrum of drug-induced movement disorders (AU)