ABSTRACT
INTRODUCTION: Vigabatrin (VGB) is a first-line drug for the treatment of infantile spasms. Recently, several reports claim the existence of abnormalities in magnetic resonance imaging (MRI) (particularly affecting basal ganglia, and visible in T2 and diffusion sequences) in infants with spasms that were receiving high doses of VGB (> 100 mg/kg/day), which appear to be reversible after withdrawal of treatment. CASE REPORTS: We present two cases with an epileptic encephalopathy in the first year of life and seizures consisting of infantile spasms. Both were treated with several antiepileptic drugs, including VGB up to a maximum dosage of 200 mg/kg/day. At the age of 11 and 28 months, respectively, MRI images showed marked signal hyperintensities on T2-sequences on bilateral globus pallidus, thalamus, posterior portion of the brainstem and dentate nuclei, also visible on diffusion sequences. Both had previous unaltered MRI studies. After excluding an underlying metabolic disease, VGB withdrawal is decided, appreciating the reversibility of those lesions in a prospective MRI study, three months later. CONCLUSIONS: We must consider and carefully evaluate findings on brain MRI in infants receiving VGB at high doses for treatment of spasms. The apparent cytotoxic effect on basal ganglia could simulate metabolic/mitochondrial diseases. By knowing this effect of VGB and its typical MRI features, unnecessary testing can be avoided in young infants with epileptic encephalopathies, including complex procedures like muscle biopsy or a new metabolic screening.
TITLE: Alteraciones reversibles en la neuroimagen asociadas al tratamiento con vigabatrina en lactantes con espasmos epilepticos.Introduccion. La vigabatrina (VGB) es un farmaco de primera linea para el tratamiento de espasmos infantiles. Diversos estudios han hallado anomalias en la resonancia magnetica (RM) cerebral, que afectaban particularmente a los ganglios de la base, y especialmente en secuencias de difusion, en lactantes con espasmos que recibian VGB en altas dosis (> 100 mg/kg/dia), y se ha observado la desaparicion de las lesiones tras la retirada de dicho tratamiento. Casos clinicos. Se presentan dos casos clinicos con inicio de una encefalopatia epileptica en el primer año de vida y crisis en forma de espasmos infantiles. Ambos recibieron tratamiento con distintos farmacos, entre ellos VGB hasta dosis de 200 mg/kg/dia. Con 11 y 28 meses de vida, respectivamente, aparecian imagenes en la RM cerebral con una marcada hiperintensidad de señal en secuencias ponderadas en T2 en ambos palidos, talamos, porcion posterior del tronco encefalico y nucleos dentados, que asociaban restriccion en secuencias de difusion. Ambos disponian de estudios previos de RM, sin alteraciones. Tras excluir una metabolopatia subyacente, se decidio la retirada de la VGB y tres meses despues, en una RM de control, se aprecio la total reversibilidad de dichas lesiones. Conclusiones. Deben evaluarse con cautela los hallazgos de la RM cerebral en lactantes que reciban VGB en altas dosis para el tratamiento de espasmos. Su aparente efecto citotoxico sobre los ganglios de la base podria simular metabolopatias/enfermedades mitocondriales. Conocer este efecto de la VGB y sus caracteristicas tipicas en la RM puede evitar pruebas innecesarias, como una biopsia muscular o un nuevo cribado metabolico.
Subject(s)
Anticonvulsants/therapeutic use , Brain/diagnostic imaging , Neuroimaging , Spasms, Infantile/diagnostic imaging , Vigabatrin/therapeutic use , Brain/pathology , Drug Resistance , Drug Resistant Epilepsy/diagnostic imaging , Drug Resistant Epilepsy/drug therapy , Drug Resistant Epilepsy/pathology , Humans , Infant , Magnetic Resonance Imaging , Male , Spasms, Infantile/drug therapy , Spasms, Infantile/pathologySubject(s)
Papilloma, Choroid Plexus/diagnosis , Humans , Infant , Magnetic Resonance Imaging , Meninges/pathologyABSTRACT
No disponible
Subject(s)
Humans , Female , Infant , Papilloma, Choroid Plexus , Vomiting/etiology , Cerebrospinal Fluid/cytology , Cerebrospinal Fluid , Magnetic Resonance Imaging/methods , Gadolinium , Calcinosis/complications , Calcinosis , Papilloma, Choroid Plexus/complications , Papilloma, Choroid Plexus/diagnosis , Papilloma, Choroid Plexus/therapy , Ultrasonography/trends , Hydrocephalus/complications , Diagnosis, DifferentialABSTRACT
El quiste óseo aneurismático es una lesión ósea benigna de etiopatogenia incierta. Se ha postulado que pudiera tratarse de una lesión reactiva a un traumatismo o a un tumor óseo primario benigno (tumor de células gigantes, condroblastoma, etc.) o maligno (osteosarcoma) que acaba produciendo cambios hemodinámicos locales. Existe controversia sobre su posible malignización. Presentamos un caso de evolución radiológica de osteosarcoma de bajo grado indistinguible de quiste óseo aneurismático (AU)
Aneurysmatic bone cysts are benign lesions of unknown origin. It has been postulated that they might occur in reaction to trauma or to a primary benign (giant cell tumor, chondroblastoma, etc.) or malignant (osteosarcoma) bone tumor that results in local hemodynamic changes. Their malignant transformation is controversial. We present a case of low grade osteosarcoma with a radiologic progression that was indistinguishable from that of an aneurysmatic bone cyst (AU)
Subject(s)
Humans , Female , Child , Bone Cysts, Aneurysmal , Osteosarcoma , Neoplasms, Bone Tissue , Giant Cell Tumors , Bone Cysts, Aneurysmal/diagnosis , Bone Cysts, Aneurysmal/pathology , Chondroblastoma , Shoulder/pathology , ShoulderABSTRACT
No disponible
Subject(s)
Humans , Female , Adolescent , Sarcoma, Ewing , Sarcoma, Ewing/drug therapy , Sarcoma, Ewing/radiotherapy , Lymphoma/complications , Lymphoma , Neuroblastoma , Neuroectodermal Tumors , Palliative Care/methods , Palliative Care/trends , Pelvic Bones , Central Nervous System/pathology , Central Nervous SystemABSTRACT
Aneurysmatic bone cysts are benign lesions of unknown origin. It has been postulated that they might occur in reaction to trauma or to a primary benign (giant cell tumor, chondroblastoma, etc.) or malignant (osteosarcoma) bone tumor that results in local hemodynamic changes. Their malignant transformation is controversial. We present a case of low grade osteosarcoma with a radiologic progression that was indistinguishable from that of an aneurysmatic bone cyst.
