Hopkins syndrome
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Hopkins syndrome, sometimes called acute post-asthmatic amyotrophy, is an acute attack of muscle weakness that arises in a small number of patients with asthma, usually after a severe asthma episode. It is exceptionally rare, and there are not more than a few dozen cases reported in the medical literature, worldwide. The syndrome resembles poliomyelitis; it characteristically produces acute flaccid paralysis with atrophy and fasciculations, usually of one or two limbs. The illness usually arises as the patient is recovering from the asthma attack; most cases occur in children. The cause of Hopkins syndrome has not been established, but its association with asthma exacerbations (usually with a respiratory infection as a trigger) has led to suspicion that the initial viral insult that causes the respiratory infection is also implicated in the subsequent paralysis. Herpes simplex virus type I DNA has been found in the cerebrospinal fluid of at least one patient diagnosed with Hopkins syndrome.Kyllerman MG, et al. PCR diagnosis of primary herpesvirus type I in poliomyelitis-like paralysis and respiratory tract disease. Pediatr Neurol. 1993;9(3):227-9. PMID 8394714 In several cases, anti-viral antibody titers for echovirus, enterovirus, coxsackievirus and poliovirus types 1, 2 and 3 were specifically sought; all were negative.Okayama A, et al. case of Hopkins syndrome with onset at puberty. Rinsho Shinkeigaku. 1999;39(4):452-5. PMID 10391972,Kurokawa T, et al. adult case of recurrent myelopathy presenting with monoplegia following asthmatic attacks. Fukuoka Igaku Zasshi. 2000;91(3):85-9. PMID 10826222 There is one reported case in which Mycoplasma pneumoniae infection was found in the patient.Acharya AB, Lakhani PK. Hopkins syndrome associated with Mycoplasma infection. Pediatr Neurol. 1997;16(1):54-5. PMID 9044403The syndrome appears to involve the spinal cord: specifically, the anterior horn cells subserving the affected muscles are often damaged. The evidence for anterior horn cell involvement comes from radiological Kurokawa T, et al. adult case of recurrent myelopathy presenting with monoplegia following asthmatic attacks. Fukuoka Igaku Zasshi. 2000;91(3):85-9. PMID 10826222,Nakano Y, et al. syndrome: oral prednisolone was effective for the paralysis. No To Hattatsu. 2001;33(1):69-73. PMID 11197900,Arita J, et al. Hopkins syndrome: T2-weighted high intensity of anterior horn on spinal MR imaging. Pediatr Neurol. 1995;13(3):263-5. PMID 8554668 and electromyographical studies.Acharya AB, Lakhani PK. Hopkins syndrome associated with Mycoplasma infection. Pediatr Neurol. 1997;16(1):54-5. PMID 9044403 In one case, a biopsy of an affected muscle "revealed scattered atrophic fibers, indicating lesions in the anterior horn cells of the spinal cord".Mizuno Y, et al. Poliomyelitis-like illness after acute asthma (Hopkins syndrome): a histological study of biopsied muscle in a case. Brain Dev. 1995;17(2):126-9. PMID 7625547As the illness is rare, no treatments have been subjected to a randomized controlled trial. Acyclovir,Kyllerman MG, et al. PCR diagnosis of primary herpesvirus type I in poliomyelitis-like paralysis and respiratory tract disease. Pediatr Neurol. 1993;9(3):227-9. PMID 8394714 steroids, and therapeutic plasma exchange have been tried; one report suggests that the latter is more effective than steroidal therapy.Kira J. damage associated with allergic diseases: pathomechanism and therapy. Rinsho Shinkeigaku. 2003;43(11):756-60. PMID 15152457 The prognosis for recovery of function of the affected limbs is generally considered to be poor.
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