Hopkins syndrome

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 following a severe asthma attack. It is exceptionally rare; 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 have occurred 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. [A case of Hopkins syndrome with onset at puberty.] "Rinsho Shinkeigaku". 1999;39(4):452-5. PMID 10391972] ^,Kurokawa T, et al. [An 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 9044403]

The 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. [An 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. [Hopkins 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 7625547]

As 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. [Neural 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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