Nora Daniel B, Gomes Irenio, El Ammar Gibran, Nunes Magda L
Servi o de Neurologia, Hospital São Lucas, Pontifícia Universidade Católica do Rio Grande do Sul, Porto Alegre, RS, Brasil.
Arq Neuropsiquiatr. 2003 Jun;61(2B):494-8. doi: 10.1590/s0004-282x2003000300033. Epub 2003 Jul 28.
Hopkins syndrome is a motor neuron disease which leads to a flaccid paralysis affecting one or more limbs resembling poliomyelites. It follows an asthmatic attack and the prognosis is poor. All the 34 related cases occured after 13 months of age and there is no report in South America. Our objective is to describe a case of Hopkins Syndrome in Brazil affecting a patient younger than 1 year.
Male 4 months-old infant, started presenting wheezing that turned into respiratory failure which required mecanical ventilation. Three days later he initiated with loss of strength in the lower limbs (LL). The patient was dismissed from hospital better of his respiratory complains but still paraparetic. Five months later, the patient returned with another episode of severe bronchospasm. At that time, he presented with flaccid paralysis, arreflexia and atrophy of the LL. There were no upper limbs and sensory abnormalities. The patient undergone to lumbar puncture, spinal MRI and SEPs which were all normal. MUSCLE BIOPSY: type grouping. EMG and NCS were in keeping with motor neuron disease affecting just the lumbosacral region.
Hopkins syndrome should be included in the differential diagnosis of any flaccid paralysis when it is associated with an asthma attack.
霍普金斯综合征是一种运动神经元疾病，可导致弛缓性麻痹，影响一个或多个肢体，类似于小儿麻痹症。它继发于哮喘发作后，预后较差。所有34例相关病例均发生在13个月龄之后，南美洲尚无相关报道。我们的目的是描述巴西一例1岁以下患者患霍普金斯综合征的病例。
一名4个月大的男婴，开始出现喘息，随后发展为呼吸衰竭，需要机械通气。三天后，他开始出现下肢无力。患者出院时呼吸症状有所改善，但仍为双下肢轻瘫。五个月后，患者再次出现严重支气管痉挛发作。此时，他出现弛缓性麻痹、反射消失和下肢萎缩。上肢及感觉无异常。患者接受了腰椎穿刺、脊髓磁共振成像和体感诱发电位检查，结果均正常。肌肉活检：肌纤维类型分组异常。肌电图和神经传导速度检查结果符合仅累及腰骶部的运动神经元疾病。
当弛缓性麻痹与哮喘发作相关时，霍普金斯综合征应列入鉴别诊断。
