RESUMO
Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.
Assuntos
Encefalopatias , Policondrite Recidivante , Corticosteroides , Adulto , Encéfalo/diagnóstico por imagem , Encefalopatias/etiologia , Feminino , Humanos , Imageamento por Ressonância Magnética , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológicoRESUMO
Relapsing polychondritis (RP) is a rare multisystemic autoimmune disorder characterized by the inflammation and destruction of cartilages, with preference for auricular, nasal and laryngotracheal cartilages. RP may also affect proteoglycan-rich structures, such as, blood vessels, eyes, kidneys, and heart. The central nervous system (CNS) is involved in less than 3% of patients. We report a 32-year-old female with RP associated with a progressive subacute encephalopathy characterized by behavioral disturbances, auditory and visual hallucinations. The EEG showed generalized slow activity and a mononuclear pleocytosis with increased protein was found in the cerebrospinal fluid. The brain magnetic resonance imaging showed multiple supra and infratentorial nodular inflammatory lesions. After initiating treatment with corticosteroids and cyclophosphamide, a significant improvement in chondritis and neurological status was observed.
Assuntos
Humanos , Feminino , Adulto , Policondrite Recidivante/complicações , Policondrite Recidivante/diagnóstico , Policondrite Recidivante/tratamento farmacológico , Encefalopatias/etiologia , Encéfalo/diagnóstico por imagem , Imageamento por Ressonância Magnética , CorticosteroidesRESUMO
Monomelic amyotrophy, also known as Hirayama disease, is a rare lower motor neuron syndrome due to localized lower motor neuron loss in the spinal cord at the cervical level. Clinically, monomelic amyotrophy is defined by the insidious onset of unilateral atrophy and weakness involving the hand and forearm, typically beginning in the second or third decade of life. We report 19-year-old man with a two years history of slowly progressive unilateral weakness and atrophy of his right-hand muscles. Neurological examination revealed weakness and atrophy in his intrinsic hand muscles, with sparing of the abductor pollicis brevis muscle. Also, mild atrophy of the ulnar aspect of the forearm was detected with sparing of the brachioradialis muscle. Electromyography showed active and chronic neurogenic changes affecting C8 and T1 myotomes, with mild chronic neurogenic changes on C7 myotome. Magnetic resonance imaging of his cervical spine revealed spinal cord atrophy involving C5 to C7 segments, associated with forward displacement of the posterior wall of the dura in cervical spine flexion. The clinical features associated with the imaging and electrophysiological findings support the diagnosis of monomelic amyotrophy.
Assuntos
Atrofias Musculares Espinais da Infância , Adulto , Vértebras Cervicais/diagnóstico por imagem , Eletromiografia , Mãos/diagnóstico por imagem , Humanos , Imageamento por Ressonância Magnética , Masculino , Atrofias Musculares Espinais da Infância/complicações , Atrofias Musculares Espinais da Infância/diagnóstico , Adulto JovemRESUMO
Monomelic amyotrophy, also known as Hirayama disease, is a rare lower motor neuron syndrome due to localized lower motor neuron loss in the spinal cord at the cervical level. Clinically, monomelic amyotrophy is defined by the insidious onset of unilateral atrophy and weakness involving the hand and forearm, typically beginning in the second or third decade of life. We report 19-year-old man with a two years history of slowly progressive unilateral weakness and atrophy of his right-hand muscles. Neurological examination revealed weakness and atrophy in his intrinsic hand muscles, with sparing of the abductor pollicis brevis muscle. Also, mild atrophy of the ulnar aspect of the forearm was detected with sparing of the brachioradialis muscle. Electromyography showed active and chronic neurogenic changes affecting C8 and T1 myotomes, with mild chronic neurogenic changes on C7 myotome. Magnetic resonance imaging of his cervical spine revealed spinal cord atrophy involving C5 to C7 segments, associated with forward displacement of the posterior wall of the dura in cervical spine flexion. The clinical features associated with the imaging and electrophysiological findings support the diagnosis of monomelic amyotrophy.
Assuntos
Humanos , Masculino , Adulto , Adulto Jovem , Atrofias Musculares Espinais da Infância/complicações , Atrofias Musculares Espinais da Infância/diagnóstico , Imageamento por Ressonância Magnética , Vértebras Cervicais/diagnóstico por imagem , Eletromiografia , Mãos/diagnóstico por imagemRESUMO
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.
Assuntos
Síndrome de Creutzfeldt-Jakob/diagnóstico , Proteínas 14-3-3/líquido cefalorraquidiano , Idoso , Autopsia , Eletroencefalografia , Evolução Fatal , Humanos , Imageamento por Ressonância Magnética , MasculinoRESUMO
Creutzfeldt-Jakob disease has a higher incidence in Chile than in other countries. The post mortem pathological characterization of brain tissue is necessary to reach a definitive diagnosis. We report a 73 years old man with a history compatible with of a rapidly progressive dementia, in which the first electroencephalographic study showed a pattern consistent with non-convulsive status epilepticus. Besides discarding this diagnosis, it was necessary to rule out other causes of rapidly progressive dementia such as Hashimoto encephalopathy. Finally, the sustained clinical deterioration with no response to anticonvulsants and corticosteroids, the imaging studies, a serial electroencephalographic monitoring study and the detection of 14-3-3 protein in cerebrospinal fluid were the keys to achieve the diagnosis of the disease.
Assuntos
Humanos , Masculino , Idoso , Síndrome de Creutzfeldt-Jakob/diagnóstico , Autopsia , Imageamento por Ressonância Magnética , Evolução Fatal , Proteínas 14-3-3/líquido cefalorraquidiano , EletroencefalografiaRESUMO
El objetivo de este estudio es la identificación de posibles factores de riesgo para el consumo de drogas entre jóvenes de enseñanza secundaria en la Región de Magallanes, lo que contribuiría al desarrollo de programas preventivos que logren una mayor efectividad y eficiencia social. La población estudiada estuvo constituida por el universo de estudiantes de 3° año de enseñanza media, de los colegios de esa región durante 1994 (1.270 jóvenes). Se aplicó un set de encuestas en forma autoadministrada, que indagaba por los datos personales, la relación con el colegio, consumo de sustancia por los padres y los amigos, varias dimensiones psicosociales y las características del consumo de drogas (cuando estaba presente). Se encontró asociación significativa con el consumo de drogas para las variables que se refieren a la relación del joven con el colegio, para el consumo de drogas por parte de los padres y de los amigos, para una mayor intensidad del hábito tabáquico y el consumo de bebidas alcohólicas, para variables psicosociales como el nivel de síntomas ansioso-depresivos, el nivel de estrés de los últimos meses y el apoyo social que el joven siente que sus padres le dan. La mayoría de los jóvenes considera que el consumo de drogas es dañino y esta creencia se asocia en forma inversa con el uso de sustancias. Sin embargo, la mayoría de los consumidores funciona con una disonancia cognitivo - conductual al respecto. La mayoría de los jóvenes ha dejado de consumir drogas por períodos y sólo una proporción muy pequeña señala que no fue capaz de hacerlo. Los resultados encontrados son relevantes para el diseño de programas preventivos. Se apreciaron importantes diferencias para varones y mujeres, lo que plantea la necesidad de incorporar una perspectiva de género en estos programas.