RESUMO
Guillain-Barré's syndrome in childhood can follow an atypical course, increasing the challenges in diagnosis and decisions regarding immunomodulatory treatment. Here, we report the case of on a 13-year-old boy with acute onset Guillain-Barré's syndrome progressing over 40 days to a very severe, locked-in-like syndrome despite intensive immunomodulatory treatment. After a plateau phase lasting 3 months and characterized by fluctuating signs of ongoing inflammatory disease activity, we were prompted to perform repeated and maintenance immunomodulatory treatment, which resulted in a continuous and nearly complete recovery of function. Atypical features at disease onset, the severe "total" loss of all peripheral and cranial nerve function, and an apparent late response to treatment give rise to reviewing the dilemmas of diagnosis and treatment in such severe and protracted courses of Guillain-Barré syndrome.
Assuntos
Síndrome de Guillain-Barré , Fatores Imunológicos/farmacologia , Síndrome do Encarceramento , Adolescente , Morte Encefálica , Progressão da Doença , Síndrome de Guillain-Barré/complicações , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/tratamento farmacológico , Humanos , Síndrome do Encarceramento/diagnóstico , Síndrome do Encarceramento/tratamento farmacológico , Síndrome do Encarceramento/etiologia , MasculinoAssuntos
Eletroencefalografia , Parada Cardíaca/complicações , Síndrome do Encarceramento/diagnóstico , Estado Vegetativo Persistente/diagnóstico , Córtex Pré-Frontal/fisiopatologia , Estimulação Transcraniana por Corrente Contínua , Adulto , Diagnóstico Diferencial , Parada Cardíaca/fisiopatologia , Humanos , Síndrome do Encarceramento/etiologia , Síndrome do Encarceramento/fisiopatologia , Masculino , Estado Vegetativo Persistente/etiologia , Estado Vegetativo Persistente/fisiopatologiaRESUMO
BACKGROUND: Lumbar puncture is a common procedure that can be safely performed in most patients. Certain populations may have increased risk for complications following lumbar puncture, but the significance of basilar invagination is often underappreciated. CASE DESCRIPTION: A 45-year-old woman with basilar invagination received multiple lumbar punctures in the workup of acute meningitis. Preprocedural computed tomography was obtained. Following lumbar puncture, the patient developed locked-in syndrome. Magnetic resonance imaging obtained several days later demonstrated severe compression and infarction of the medulla and inferior cerebellum by the odontoid process and ectopic cerebellar tonsils. The patient was transferred but at this point, surgical decompression was not possible. She did not regain significant neurologic function. CONCLUSIONS: Basilar invagination is a risk factor for devastating neurologic complications following lumbar puncture. Awareness of this complication and prompt recognition of its occurrence may prevent future morbidity of lumbar puncture in patients with basilar invagination.
Assuntos
Infartos do Tronco Encefálico/diagnóstico por imagem , Síndrome do Encarceramento/diagnóstico , Bulbo/irrigação sanguínea , Bulbo/diagnóstico por imagem , Meningite Pneumocócica/diagnóstico , Platibasia/diagnóstico por imagem , Complicações Pós-Operatórias/diagnóstico , Punção Espinal/efeitos adversos , Infartos do Tronco Encefálico/etiologia , Cerebelo/anormalidades , Cerebelo/diagnóstico por imagem , Feminino , Humanos , Síndrome de Klippel-Feil/complicações , Síndrome de Klippel-Feil/cirurgia , Síndrome do Encarceramento/diagnóstico por imagem , Síndrome do Encarceramento/etiologia , Imageamento por Ressonância Magnética , Meningite Pneumocócica/complicações , Pessoa de Meia-Idade , Processo Odontoide/anormalidades , Processo Odontoide/diagnóstico por imagem , Platibasia/complicações , Complicações Pós-Operatórias/diagnóstico por imagem , Complicações Pós-Operatórias/etiologia , Fusão Vertebral , Streptococcus pneumoniae , Tomografia Computadorizada por Raios XRESUMO
Persons in the completely locked-in state (CLIS) suffering from amyotrophic lateral sclerosis (ALS) are deprived of many zeitgebers of the circadian rhythm: While cognitively intact, they are completely paralyzed, eyes mostly closed, with artificial ventilation and artificial nutrition, and social communication extremely restricted or absent. Polysomnographic recordings in eight patients in CLIS, however, revealed the presence of regular episodes of deep sleep during night time in all patients. It was also possible to distinguish an alpha-like state and a wake-like state. Classification of rapid eye movement (REM) sleep is difficult because of absent eye movements and absent muscular activity. Four out of eight patients did not show any sleep spindles. Those who have spindles also show K-complexes and thus regular phases of sleep stage 2. Thus, despite some irregularities, we found a surprisingly healthy sleep pattern in these patients.
Assuntos
Esclerose Lateral Amiotrófica/fisiopatologia , Ritmo Circadiano/fisiologia , Síndrome do Encarceramento/fisiopatologia , Sono/fisiologia , Adulto , Esclerose Lateral Amiotrófica/diagnóstico , Esclerose Lateral Amiotrófica/terapia , Movimentos Oculares/fisiologia , Feminino , Humanos , Síndrome do Encarceramento/diagnóstico , Síndrome do Encarceramento/terapia , Masculino , Pessoa de Meia-Idade , Polissonografia/métodos , Respiração Artificial/métodos , Sono de Ondas Lentas/fisiologia , Adulto JovemRESUMO
Locked-in syndrome (LIS) is a state of quadriplegia and anarthria with preserved consciousness, which is generally triggered by a disruption of specific white matter fiber tracts, following a lesion in the ventral part of the pons. However, the impact of focal lesions on the whole brain white matter microstructure and structural connectivity pathways remains unknown. We used diffusion tensor magnetic resonance imaging (DT-MRI) and tract-based statistics to characterise the whole white matter tracts in seven consecutive LIS patients, with ventral pontine injuries but no significant supratentorial lesions detected with morphological MRI. The imaging was performed in the acute phase of the disease (26 ± 13 days after the accident). DT-MRI-derived metrics were used to quantitatively assess global white matter alterations. All diffusion coefficient Z-scores were decreased for almost all fiber tracts in all LIS patients, with diffuse white matter alterations in both infratentorial and supratentorial areas. A mixture model of two multidimensional Gaussian distributions was fitted to cluster the white matter fiber tracts studied in two groups: the least (group 1) and most injured white matter fiber tracts (group 2). The greatest injuries were revealed along pathways crossing the lesion responsible for the LIS: left and right medial lemniscus (98.4% and 97.9% probability of belonging to group 2, respectively), left and right superior cerebellar peduncles (69.3% and 45.7% probability) and left and right corticospinal tract (20.6% and 46.5% probability). This approach demonstrated globally compromised white matter tracts in the acute phase of LIS, potentially underlying cognitive deficits.