Hopkins' syndrome: a case report and review of the literature

Abstract

Hopkins' syndrome is a motor neuron disease which leads to a flaccid paralysis due to anterior horn cell damage and normal sensation affecting one or more limbs resembling poliomyelitis. It occurs less than one week aft er an asthma attack and its prognosis is poor. Although there has been no satisfactory explanation for the etiology; drug neurotoxicity, steroids and viruses (enteroviruses, Mycoplasma and Herpes simplex) have been implicated. Here, a rarely seen male patient having Hopkins' syndrome was described. A five year old male with persistent asthma, allergic rhinitis and atopic dermatitis presented to us with left lower extremity (LLE) flaccid paralysis and hyporefl exia. Three weeks earlier, he was treated for an exacerbation of asthma with-7-day course of oral prednisone, fluticason and albuterol nebules with improvement. However, he had another attack one dav prior to flaccid paralysis. His weakness began with pain and paraesthesia in that morning. He then started to limp and was unable to stand or walk on that left leg a couple of hours later. Physical examination revealed flaccid and areflexic LLE on admission. Plantar reflexes, sensation, hip movements on both lower extremities and sphincter tone were normal. Several months later, he walked with a limp and had persistent LLE weakness and muscle atrophy. CBC, routine biochemistry, CRP, ESR, CPK, toxicology screen and urine analvsis were within normal. lgM to EBV, CMV and PCR for enteroviruses were negative. Specific IgE was positive for dust mite. CSF showed normal findings. MRI of spine/pelvis/cranium and both extremities was normal. EMG revealed anterior horn cell damage. Hopkins' syndrome should be kept in mind in the differential diagnosis of any flaccid paralvsis associated with an asthma attack.