Hopkins' syndrome in the differential diagnosis of flaccid paralysis in children: clinical and neurophysiological features. Case report
Nora, D.B.; Gomes, I.; El Ammar, G.; Nunes, M.L.
Arquivos de Neuro-Psiquiatria 61(2b): 494-498
2003
ISSN/ISBN: 0004-282X PMID: 12894293 Document Number: 564243
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.