Hopkins syndrome
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182517973
2008-01-06T12:51:20Z
Encephalon
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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 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 [[fasciculation]]s, 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.<ref name=Kyllerman>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</ref> In several cases, anti-viral antibody titers for [[echovirus]], [[enterovirus]], [[coxsackievirus]] and [[poliovirus]] types 1, 2 and 3 were specifically sought; all were negative.<ref name=Okayama>Okayama A, et al. [A case of Hopkins syndrome with onset at puberty.] ''Rinsho Shinkeigaku''. 1999;39(4):452-5. PMID 10391972</ref><sup>,</sup><ref name=Kurokawa>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</ref> There is one reported case in which ''[[Mycoplasma pneumoniae]]'' infection was found in the patient.<ref name=Acharya>Acharya AB, Lakhani PK. Hopkins syndrome associated with Mycoplasma infection. ''Pediatr Neurol''. 1997;16(1):54-5. PMID 9044403</ref>
The syndrome appears to involve the [[spinal cord]]: specifically, the [[anterior horn cell]]s subserving the affected muscles are often damaged. The evidence for anterior horn cell involvement comes from [[Radiology|radiological]] <ref name=Kurokawa>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</ref><sup>,</sup><ref name=Nakano>Nakano Y, et al. [Hopkins syndrome: oral prednisolone was effective for the paralysis.] ''No To Hattatsu''. 2001;33(1):69-73. PMID 11197900</ref><sup>,</sup><ref name=Arita>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</ref> and [[electromyograph]]ical studies.<ref name=Acharya>Acharya AB, Lakhani PK. Hopkins syndrome associated with Mycoplasma infection. ''Pediatr Neurol''. 1997;16(1):54-5. PMID 9044403</ref> In one case, a [[biopsy]] of an affected muscle "revealed scattered atrophic fibers, indicating lesions in the anterior horn cells of the spinal cord".<ref name=Mizuno>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</ref>
As the illness is rare, no treatments have been subjected to a [[randomized controlled trial]]. [[Acyclovir]],<ref name=Kyllerman>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</ref> [[steroid]]s, and therapeutic [[plasma exchange]] have been tried; one report suggests that the latter is more effective than steroidal therapy.<ref name=Kira>Kira J. [Neural damage associated with allergic diseases: pathomechanism and therapy.] ''Rinsho Shinkeigaku''. 2003;43(11):756-60. PMID 15152457</ref> The prognosis for recovery of function of the affected limbs is generally considered to be poor.
== References ==
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[[Category:Neurological disorders]]