Dados do Trabalho

Título

SINDROME DE HOPKINGS: A CASE REPORT

Apresentação do caso

A previously asthmatic 10-year-old girl, with irregular treatment, presented to the emergency with a severe acute exacerbation. She didn’t respond to any initial medication and needed invasive ventilation for 5 days. After withdrawal of sedation, asymmetric paraplegia was noted associated with pain, areflexia in all but right achilles which has hyporeflexia, and Babinski sign on the right. Her upper limbs had minimum weakness and readily symmetric reflexes with a positive Hoffman sign. A head CT revealed a left arachnoid cyst at the posterior fossa causing minimal mass effect against cerebellum, pons, and medulla oblongata. Therefore, a lumbar puncture was contraindicated. A needle electroneuromyography showed asymmetric motor axonal neuropathy/neuronopathy and a spinal cord MRI revealed enhancement of ventral roots of the cauda equina. Considering the gravity of the symptoms, we gave human immunoglobulin 2g/kg for 5 days. Later, a somatosensory evoked potential in the legs revealed reduced amplitude on the left, corroborating the head CT finding, which we attributed, after discussing with neurosurgeons, that it was a previously non-symptomatic lesion. She improved her arms’ strength but was discharged using wheelchairs and referred to rehabilitation.

Discussão

Hopkins syndrome (HS) is a rare condition, with almost 40 cases reported worldwide. It is characterized by acute flaccid paralysis of one or more limbs, usually asymmetrically, due to lesion of the anterior horn of spinal cord subsequently a severe asthma exacerbation. Sensitivity and urinary-rectum sphincters are preserved. Children are most susceptible, and the etiology is still not well understood. It is suggested that neurotropic viruses, corticosteroids immunosuppression, neurotoxic drugs such as xanthines and neuromuscular blockers, or cross-reactivity between an allergen and the peripheral nervous system.
Symptoms develop when bronchospasm begins remission, evolving to paralysis within hours a days. There is no definite treatment and patients must initiate rehabilitation as soon as possible. Residual deficits usually persist but fortunately, episodes of HS rarely recur. There have been some reports of clinical improvement after intravenous immunoglobulin and prednisolone.

Comentários finais

Albeit rare, HS must be on the radar for differential diagnosis in a child with asymmetric acute flaccid paralysis following severe respiratory distress caused by asthma exacerbation.

Palavras Chave

Flaccid, asthma, paralysis, rare diseases

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Não