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 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.[1] In several cases, anti-viral antibody titers for echovirus, enterovirus, coxsackievirus and poliovirus types 1, 2 and 3 were specifically sought; all were negative.[2],[3] There is one reported case in which Mycoplasma pneumoniae infection was found in the patient.[4]

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 [3],[5],[6] and electromyographical studies.[4] In one case, a biopsy of an affected muscle "revealed scattered atrophic fibers, indicating lesions in the anterior horn cells of the spinal cord".[7]

As the illness is rare, no treatments have been subjected to a randomized controlled trial. Acyclovir,[1] steroids, and therapeutic plasma exchange have been tried; one report suggests that the latter is more effective than steroidal therapy.[8] The prognosis for recovery of function of the affected limbs is generally considered to be poor.

[edit] References

  1. ^ a b 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
  2. ^ Okayama A, et al. [A case of Hopkins syndrome with onset at puberty.] Rinsho Shinkeigaku. 1999;39(4):452-5. PMID 10391972
  3. ^ a b 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
  4. ^ a b Acharya AB, Lakhani PK. Hopkins syndrome associated with Mycoplasma infection. Pediatr Neurol. 1997;16(1):54-5. PMID 9044403
  5. ^ Nakano Y, et al. [Hopkins syndrome: oral prednisolone was effective for the paralysis.] No To Hattatsu. 2001;33(1):69-73. PMID 11197900
  6. ^ 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
  7. ^ 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
  8. ^ Kira J. [Neural damage associated with allergic diseases: pathomechanism and therapy.] Rinsho Shinkeigaku. 2003;43(11):756-60. PMID 15152457