ABSTRACT
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant small-vessel disease caused by mutations of the NOTCH3 gene. It typically presents with migraine, recurrent brain ischaemia, and cognitive disorders. Seizures rarely present as the initial manifestation, with non-convulsive status epilepticus being even less frequent. We present a series of 3 related patients with this arteriopathy, 2 of whom presented status epilepticus as a manifestation of the disease.
Subject(s)
CADASIL , Leukoencephalopathies , Status Epilepticus , Humans , CADASIL/complications , CADASIL/diagnosis , CADASIL/genetics , Cerebral Infarction , Magnetic Resonance Imaging , Receptor, Notch3/genetics , Status Epilepticus/etiologyABSTRACT
INTRODUCTION: The coronavirus disease 2019 (COVID-19) pandemic is a major worldwide health disorder. There is an increasing number of neurological complications recognized with COVID-19 including patients with GBS and its variants. DEVELOPMENT: A review of the clinical cases of GBS associated to COVID-19 infection published in the last months has been developed. We included 48 patients (31 men, mean age 56.4 years). The most common COVID-19 symptoms were cough (60.4%) and fever (56.3%). Mean time from COVID-19 symptoms to neurologic manifestations was 12.1 days, but in nine patients (18.8%) developed GBS within seven days. Eleven patients (22.9%) presented cranial nerve involvement in the absence of muscle weakness; 36 presented the classic sensory motor variant (75%) and one had a pure motor variant (2.1%). The electrodiagnostic pattern was considered demyelinating in 82.4% of the generalized variants. The presence of hyposmia/dysgeusia was associated with a latency shorter than seven days to GBS onset of symptoms (30% vs 15.6%), and cranial nerve involvement in the absence of weakness (30.8% vs 17.1%). Most patients (87.5%) were treated with intravenous immunoglobulin. Neurological outcome was favorable in 64.6%; 29.2% had respiratory failure and 4.2% died shortly after being admitted. CONCLUSIONS: GBS in patients with SARS-CoV-2 infection resembles clinically and electrophysiology the classical forms. Further studies are necessary to understand whether GBS frequency is actually increased due to SARS-CoV-2 infection and explore pathogenic mechanisms.
TITLE: Síndrome de Guillain-Barré asociado a infección por COVID-19: revisión de casos publicados.Introducción. La pandemia por la enfermedad por coronavirus 2019 (COVID-19) es un importante problema para la salud mundial. Hay un incremento en las complicaciones neurológicas reconocidas por la COVID-19, incluyendo el síndrome de Guillain-Barré (SGB) y sus variantes. Desarrollo. Se realizó una revisión de los casos publicados en los últimos meses de SGB asociado a infección por COVID-19. Incluimos a 48 pacientes (31 hombres; edad media: 56,4 años). Los síntomas de COVID-19 más comunes fueron tos (60,4%) y fiebre (56,3%). El tiempo promedio entre los síntomas de COVID-19 y el SGB fue de 12,1 días, pero nueve pacientes (18,8%) desarrollaron SGB en menos de siete días. Once pacientes (22,9%) presentaron afectación de los nervios craneales en ausencia de debilidad muscular, 36 presentaron la variante clásica sensitivomotora (75%) y uno tuvo una variante motora pura (2,1%). El patrón electrofisiológico se consideró desmielinizante en el 82,4% de las variantes generalizadas. La presencia de hiposmia/disgeusia estuvo asociada con una latencia menor a los siete días hasta el inicio de los síntomas del SGB (30 frente a 15,6%) y a la afectación de los nervios craneales en ausencia de debilidad (30,8 frente a 17,1%). La mayoría de los pacientes (87,5%) fueron tratados con inmunoglobulina endovenosa. La evolución neurológica fue favorable en el 64,6%, el 29,2% tuvo insuficiencia respiratoria y hubo un 4,2% de muertes. Conclusiones. El SGB en pacientes con infección por SARS-CoV-2 es similar clínica y electrofisiológicamente a las formas clásicas. Se requieren más estudios para comprender si la frecuencia del SGB realmente aumentó debido a la pandemia por COVID-19 y explorar los mecanismos patógenos involucrados.
Subject(s)
COVID-19/complications , Guillain-Barre Syndrome/etiology , Pandemics , SARS-CoV-2 , Adolescent , Adult , Aged , Anosmia/etiology , Autoantibodies/blood , Autoantibodies/immunology , Autoantigens/immunology , Cranial Nerve Diseases/etiology , Dysgeusia/etiology , Female , Gangliosides/immunology , Guillain-Barre Syndrome/cerebrospinal fluid , Guillain-Barre Syndrome/immunology , Guillain-Barre Syndrome/therapy , Humans , Immunoglobulins, Intravenous/therapeutic use , Male , Middle Aged , Plasmapheresis , Respiratory Insufficiency/etiology , Retrospective Studies , Symptom Assessment , Treatment Outcome , Young AdultABSTRACT
Stroke is a severe and frequent complication of Fabry disease (FD), affecting both males and females. Cerebrovascular complications are the end result of multiple and complex pathophysiology mechanisms involving endothelial dysfunction and activation, development of chronic inflammatory cascades leading to a prothrombotic state in addition to cardioembolic stroke due to cardiomyopathy and arrhythmias. The recent coronavirus disease 2019 outbreak share many overlapping deleterious pathogenic mechanisms with those of FD and therefore we analyze the available information regarding the pathophysiology mechanisms of both disorders and hypothesize that there is a markedly increased risk of ischemic and hemorrhagic cerebrovascular complications in Fabry patients suffering from concomitant SARS-CoV-2 infections.
Subject(s)
COVID-19/complications , Fabry Disease/complications , Hemorrhagic Stroke/complications , Ischemic Stroke/complications , Aldosterone/metabolism , Angiotensin-Converting Enzyme 2/metabolism , Endothelium, Vascular/pathology , Female , Heart Diseases/complications , Heart Diseases/physiopathology , Hemorrhage/pathology , Humans , Inflammation , Male , Models, Theoretical , Nitric Oxide/metabolism , Reactive Oxygen Species/metabolism , Renin-Angiotensin System , Risk Factors , StrokeABSTRACT
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is an autosomal dominant small-vessel disease caused by mutations of the NOTCH3 gene. It typically presents with migraine, recurrent brain ischaemia, and cognitive disorders. Seizures rarely present as the initial manifestation, with non-convulsive status epilepticus being even less frequent. We present a series of 3 related patients with this arteriopathy, 2 of whom presented status epilepticus as a manifestation of the disease.
ABSTRACT
No disponible
Subject(s)
Humans , Male , Adult , Spinal Cord Diseases/diagnostic imaging , Sarcoidosis/diagnostic imaging , Magnetic Resonance ImagingABSTRACT
TITLE: Signo del tridente en la neurosarcoidosis medular.
Subject(s)
Central Nervous System Diseases/diagnostic imaging , Magnetic Resonance Imaging/methods , Sarcoidosis/diagnostic imaging , Spinal Cord/diagnostic imaging , Adrenal Cortex Hormones/therapeutic use , Adult , Anti-Inflammatory Agents/therapeutic use , Central Nervous System Diseases/drug therapy , Central Nervous System Diseases/pathology , Diagnosis, Differential , Giant Cells/ultrastructure , Granuloma/diagnostic imaging , Histiocytes/ultrastructure , Humans , Male , Neuromyelitis Optica/diagnosis , Sarcoidosis/drug therapy , Sarcoidosis/pathology , Spinal Cord/pathology , Spinal Cord Neoplasms/diagnosis , Tomography, X-Ray ComputedABSTRACT
An HIV-1-infected patient with progressive multifocal leukoencephalopathy presented clinical deterioration and contrast-enhancing lesions on brain nuclear MR after the initiation of highly active antiretroviral therapy (HAART). Brain biopsy identified an inflammatory reaction compatible with immune reconstitution inflammatory syndrome. Treatment with corticosteroids and transient suppression of HAART led to marked neurologic improvement.
Subject(s)
Acquired Immunodeficiency Syndrome/complications , Antiretroviral Therapy, Highly Active/adverse effects , Brain/drug effects , Encephalitis/chemically induced , Encephalitis/immunology , Leukoencephalopathy, Progressive Multifocal/drug therapy , Adult , Anti-Inflammatory Agents/therapeutic use , Aphasia/chemically induced , Aphasia/immunology , Aphasia/physiopathology , Brain/immunology , Brain/pathology , Chemotaxis, Leukocyte/drug effects , Chemotaxis, Leukocyte/immunology , Dexamethasone/therapeutic use , Encephalitis/physiopathology , Hemiplegia/chemically induced , Hemiplegia/immunology , Hemiplegia/physiopathology , Humans , Inclusion Bodies/immunology , Inclusion Bodies/pathology , Inclusion Bodies/virology , JC Virus/immunology , JC Virus/isolation & purification , Leukoencephalopathy, Progressive Multifocal/physiopathology , Leukoencephalopathy, Progressive Multifocal/virology , Macrophages/drug effects , Macrophages/immunology , Magnetic Resonance Imaging , Male , Oligodendroglia/immunology , Oligodendroglia/pathology , Oligodendroglia/virology , T-Lymphocytes/drug effects , T-Lymphocytes/immunology , Treatment Outcome , Withholding TreatmentSubject(s)
Headache/etiology , Headache/physiopathology , Neck Pain/etiology , Neck Pain/physiopathology , Vertebral Artery Dissection/physiopathology , Vertebral Artery/physiopathology , Adult , Age Factors , Aged , Cerebral Angiography , Diagnosis, Differential , Disease Progression , Early Diagnosis , Female , Headache/pathology , Humans , Ischemic Attack, Transient/etiology , Ischemic Attack, Transient/pathology , Ischemic Attack, Transient/physiopathology , Lateral Medullary Syndrome/etiology , Lateral Medullary Syndrome/pathology , Lateral Medullary Syndrome/physiopathology , Magnetic Resonance Angiography , Male , Middle Aged , Neck Pain/pathology , Predictive Value of Tests , Prognosis , Prospective Studies , Vertebral Artery/diagnostic imaging , Vertebral Artery/pathology , Vertebral Artery Dissection/diagnostic imaging , Vertebral Artery Dissection/pathologyABSTRACT
No disponible
Subject(s)
Middle Aged , Child , Adolescent , Aged , Aged, 80 and over , Adult , Male , Female , Humans , Angiography , Carotid Artery, Internal , Diagnosis, Differential , Aneurysm , Migraine DisordersABSTRACT
Ischemic optic neuropathy, is an exceptional complication of surgery. Moreover, bilateral and simultaneous visual deficit in ischemic optic neuropathy is very rare. We describe two patients who suffered bilateral and simultaneous ischemic optic neuropathy after elective total hip replacement. Anemia and hypotension are the most likely risk factors.
Subject(s)
Arthroplasty, Replacement, Hip , Brain Ischemia/complications , Optic Nerve Diseases/etiology , Postoperative Complications/pathology , Aged , Brain Ischemia/pathology , Humans , Male , Optic Nerve Diseases/pathology , Risk FactorsABSTRACT
La neuropatía óptica isquémica es una complicación perioperatoria infrecuente. Aún más inusual es la neuropatía óptica isquémica con compromiso ocular bilateral y simultáneo. Presentamos los casos de 2 pacientes que sufrieron neuropatía óptica isquémica bilateral y simultánea posterior a reemplazo total de cadera, en los cuales tanto la anemia como la hipotensión fueron identificados como los factores de riesgo más probables (AU)
