RESUMO
We evaluated 61 children with Guillain-Barré syndrome, 14 months to 14 years of age, admitted to the Hospital Nacional de Pediatria in Buenos Aires. According to the electrodiagnostic findings, they fit into two groups, those with acute motor axonal neuropathy (AMAN) (18 patients) and those with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (43 patients). Ninety percent of the children with AMAN resided in suburban or rural areas without running water, whereas half of the AIDP patients lived in a metropolitan district. Summer and winter months showed a higher incidence of both variants. Children with AMAN were younger, evolved more acutely, reached a higher maximum disability score, required assisted ventilation more often, had lower mean level of cerebrospinal fluid protein, improved more slowly, and had a poorer outcome 6 months and 12 months after onset. Electrophysiological findings in those with AIDP revealed a pattern of severe diffuse slowing in children 5 years old or younger and a multifocal pattern in children 6 years old or older. This difference was not reflected in the clinical picture. In contrast, AMAN showed a uniform pattern with normal sensory conduction, severely reduced compound muscle action potential amplitude, near normal conduction velocity, and early denervation. Epidemiological, clinical, electrodiagnostic, cerebrospinal fluid, and prognostic data indicate that these variants of Guillain-Barré syndrome should be regarded as different entities.
Assuntos
Síndrome de Guillain-Barré/epidemiologia , Síndrome de Guillain-Barré/fisiopatologia , Adolescente , Argentina/epidemiologia , Criança , Pré-Escolar , Demografia , Eletrodiagnóstico , Feminino , Síndrome de Guillain-Barré/induzido quimicamente , Humanos , Incidência , Lactente , Masculino , Dor , Prognóstico , Estações do AnoRESUMO
An 18-day-old child with a history of difficult breech delivery presented with wasting and weakness of C5-6-innervated muscles. The EMG examination performed the same day showed high-voltage polyphasic motor unit potentials without abnormal spontaneous activity. Both the EMG pattern and the clinical features suggest an injury taking place several weeks before delivery. This presumption seems confirmed when compared with findings in our series of 100 EMG examinations in 78 children with diagnosis of Erb's palsy. Because of both clinical and medicolegal implications, early EMG testing is advisable in all patients with congenital brachial paralysis.
Assuntos
Plexo Braquial/fisiopatologia , Paralisia/fisiopatologia , Eletromiografia , Feminino , Humanos , Recém-NascidoRESUMO
Two children aged 9 and 11 years suffered from left elbow sprain and right anterior tibial tuberosity cortical fracture respectively and were treated with plaster cast immobilization for about 30 days. They regained normal strength afterwards, but 9 and 2 months later developed insidious progressive weakness and wasting in the affected limb, mainly evident in the musculature surrounding the site of injury and sparing hand and foot muscles. Two to three years later the condition stabilized. Sensory abnormalities were not found. Electromyographic examination showed neurogenic pattern confined to the impaired extremity. The focal quality and the unusual disposition of muscle involvement suggest a correlation between trauma and/or immobilization and monomelic amyotrophy.
Assuntos
Moldes Cirúrgicos/efeitos adversos , Imobilização/efeitos adversos , Doença dos Neurônios Motores/etiologia , Entorses e Distensões/cirurgia , Fraturas da Tíbia/cirurgia , Adolescente , Criança , Articulação do Cotovelo , Eletromiografia , Potenciais Somatossensoriais Evocados , Seguimentos , Humanos , Masculino , Doença dos Neurônios Motores/fisiopatologia , Músculo Esquelético/fisiopatologia , Condução Nervosa , Fatores de TempoRESUMO
A 61 year old woman and her 58 year old brother presented with the clinical picture of late onset progressive bulbar and spinal muscular atrophy with family history of involvement in successive generations. The sister also had optic neuropathy and the brother developed diabetes mellitus and sex hormone abnormalities. Neurophysiological and histopathological studies showed a pattern of motor and sensory neuronopathy. There was no abnormal expansion of CAG repeats in the androgen receptor gene. This family seems to have a previously unrecognised entity with the bulbospinal neuronopathy phenotype.
Assuntos
Neuropatia Hereditária Motora e Sensorial/complicações , Neuropatia Hereditária Motora e Sensorial/genética , Atrofias Ópticas Hereditárias/complicações , Eletromiografia , Fasciculação/complicações , Fasciculação/fisiopatologia , Feminino , Neuropatia Hereditária Motora e Sensorial/diagnóstico , Humanos , Masculino , Pessoa de Meia-Idade , Músculo Esquelético/fisiopatologia , Linhagem , Fenótipo , Cromossomo XRESUMO
Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10% of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70% were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85%). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.
Assuntos
Encefalopatias/etiologia , Cocaína , Transtornos Relacionados ao Uso de Substâncias/complicações , Adolescente , Adulto , Alcoolismo/complicações , Coma/etiologia , Distonia/etiologia , Feminino , Humanos , Masculino , Parestesia/etiologia , Convulsões/etiologia , Tomografia Computadorizada por Raios XRESUMO
El consumo de cocaina ha adquirido proporciones epidemiológicas en los EEUU. Actualmente en nuestro país, sitio de paso de la droga, un 10 por ciento es retenida para su consumo, calculandose que un 80 por ciento de los drogadictos utilizan este alcaloide. Presentamos 13 paciente, 9 hombres y 4 mujeres con edades que oscilan entre 19 y 43 años que presentaron sintomatología por consumo de cocaína. Siete pacientes presentaron convulsiones y en uno de ellos se asociaron ausencias. Cuatro casos sufrieron accidentes cerebrovasculares, 2 de ellos isquémico y los otros hemorrágico, secundario a ruptura de malformaciones vasculares. Un paciente presentó fallas mnésicas severas y otro crisis distónicas paroxísticas. Todo menos uno consumían la cocaína por vía nasal y salvo 2 pacientes, el resto utilizaba otras drogas. El consumo concomitante de etanol era una práctica frecuente. La discontinuidad en el consumo del acaloide provó mejoría en las crisis epilépicas durante el período de seguimiento, salvo en el paciente con ausencias. El paciente con fallas mnésicas evidenció una marcada mejoría al cabo de 4 años de suspendida la cocaína. La paciente con crisis distónicas paroxísticas, al cabo de 2 años de haber suprimido la droga, persistía con el cuadro distónico. En 11 casos hubo una relación temporal entre el consumo de cocaína y la sintomatología neurológica, mientras que en los 2 restantes se debería al consumo crónico. Esto se debe a cambios farmacológicos y eventualmente estructurales en el sistema nervioso central. La frecuencia de estos casos, otrora excepcionales en nuestro medio, ha aumentado sensiblemente, obligando a considerar el consumo de este alcaloide en el enfoque diagnóstico de pacientes jóvenes con eventos neurológicos
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Cocaína , Manifestações Neurológicas , Transtornos Relacionados ao Uso de Substâncias/complicações , Alcoolismo/complicações , Coma , Distonia , Parestesia , Convulsões , Tomografia Computadorizada por Raios XRESUMO
Argentina is facing an increase in cocaine use by adolescents and young adults from every socioeconomic background. It is calculated that up to 10
of all cocaine passing through this country is locally sold and consumed. Nevertheless, local information describing common cocaine-related neurological events is scarce. From August 1988 to March 1993, 13 patients were evaluated with neurological disease associated with cocaine abuse. Among these 13 patients (Table 1), the mean age was 29; 70
were men. Patients most commonly used the nasal route (snorting). Concomitant abuse of other intoxicants, especially alcohol, was frequent (85
). The major neurological complications included one or more seizures (n = 7), ischemic stroke (n = 2) (Fig. 1-2), hemorrhagic stroke (n = 2) associated with arteriovenous malformation (Fig. 3a-b), memory disturbances (n = 1) and paroxysmal dystonia (n = 1). Psychiatric complaints were present in all patients. Mortality was not observed. There was no correlation between the appearance of complications and the amount of cocaine used, or prior experience with this drug. Only one of the 7 patients with seizures had a previous history of seizures. All had generalized tonic-clonic seizures, and one had concomitant absence episodes. Cocaine modulates central neurotransmitters and has direct cerebrovascular effects. The neurological complications appear to be related to cocaine hyperadrenergic effects, striatal dopaminergic receptor hypersensitivity and perhaps vasculitis. Structural changes in the brain of long-term cocaine abusers could explain the persistence of neurologic symptoms after drug withdrawl.
RESUMO
El consumo de cocaina ha adquirido proporciones epidemiológicas en los EEUU. Actualmente en nuestro país, sitio de paso de la droga, un 10 por ciento es retenida para su consumo, calculandose que un 80 por ciento de los drogadictos utilizan este alcaloide. Presentamos 13 paciente, 9 hombres y 4 mujeres con edades que oscilan entre 19 y 43 años que presentaron sintomatología por consumo de cocaína. Siete pacientes presentaron convulsiones y en uno de ellos se asociaron ausencias. Cuatro casos sufrieron accidentes cerebrovasculares, 2 de ellos isquémico y los otros hemorrágico, secundario a ruptura de malformaciones vasculares. Un paciente presentó fallas mnésicas severas y otro crisis distónicas paroxísticas. Todo menos uno consumían la cocaína por vía nasal y salvo 2 pacientes, el resto utilizaba otras drogas. El consumo concomitante de etanol era una práctica frecuente. La discontinuidad en el consumo del acaloide provó mejoría en las crisis epilépicas durante el período de seguimiento, salvo en el paciente con ausencias. El paciente con fallas mnésicas evidenció una marcada mejoría al cabo de 4 años de suspendida la cocaína. La paciente con crisis distónicas paroxísticas, al cabo de 2 años de haber suprimido la droga, persistía con el cuadro distónico. En 11 casos hubo una relación temporal entre el consumo de cocaína y la sintomatología neurológica, mientras que en los 2 restantes se debería al consumo crónico. Esto se debe a cambios farmacológicos y eventualmente estructurales en el sistema nervioso central. La frecuencia de estos casos, otrora excepcionales en nuestro medio, ha aumentado sensiblemente, obligando a considerar el consumo de este alcaloide en el enfoque diagnóstico de pacientes jóvenes con eventos neurológicos (AU)
Assuntos
Humanos , Masculino , Feminino , Adolescente , Adulto , Transtornos Relacionados ao Uso de Substâncias/complicações , Cocaína , Manifestações Neurológicas , Alcoolismo/complicações , Parestesia , Coma , Distonia , Tomografia Computadorizada por Raios XRESUMO
Una paciente de 52 años con una malformación de Arnold Chiari Tipo 1 (MAC T1) presentó tres episodios de apnea central aguda que requirieron asistencia ventilatoria mecánica. Los síntomas y signos neurológicos al ingreso sugerían una pobre recuperación clínica postoperatoria, al ser evaluados según criterios clínicos preoperatorios, establecidos como de valor predictivo. La evolución ulterior fue excelente y estuvo precedida de cambios favorables en los PESS realizados en el postoperatorio inmediato. Esta observación aislada, sustenta la necesidad de considerar a los PESS en la MAC T1, como un probable factor neurofisiológico predictivo de la evolución clínica postoperatoria en futuras investigaciones
Assuntos
Malformação de Arnold-Chiari/complicações , Insuficiência Respiratória/etiologia , Síndromes da Apneia do Sono/etiologia , Malformação de Arnold-Chiari/cirurgia , Malformação de Arnold-Chiari/diagnóstico , Insuficiência Respiratória/terapia , Potenciais Somatossensoriais Evocados , Cefaleia/etiologia , Espectroscopia de Ressonância Magnética , Síndromes da Apneia do Sono/tratamento farmacológico , Síndromes da Apneia do Sono/terapiaRESUMO
Una paciente de 52 años con una malformación de Arnold Chiari Tipo 1 (MAC T1) presentó tres episodios de apnea central aguda que requirieron asistencia ventilatoria mecánica. Los síntomas y signos neurológicos al ingreso sugerían una pobre recuperación clínica postoperatoria, al ser evaluados según criterios clínicos preoperatorios, establecidos como de valor predictivo. La evolución ulterior fue excelente y estuvo precedida de cambios favorables en los PESS realizados en el postoperatorio inmediato. Esta observación aislada, sustenta la necesidad de considerar a los PESS en la MAC T1, como un probable factor neurofisiológico predictivo de la evolución clínica postoperatoria en futuras investigaciones