Hopkins syndrome

Hopkins syndrome
Specialty	Neurology

Hopkins syndrome is a neurological disorder. Its cause 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.

References

1. Kyllerman, MG; Herner, S; Bergström, TB; Ekholm, SE (1993). "PCR diagnosis of primary herpesvirus type I in poliomyelitis-like paralysis and respiratory tract disease". Pediatric Neurology. 9 (3): 227–9. doi:10.1016/0887-8994(93)90091-P. PMID 8394714.
2. Okayama, A; Hara, H; Shigeto, H; Yamada, T; Kira, J (1999). "A case of Hopkins syndrome with onset at puberty". Rinsho Shinkeigaku. 39 (4): 452–5. PMID 10391972.
3. Kurokawa, T; Taniwaki, T; Arakawa, K; Yamada, T; Kira, J (2000). "An adult case of recurrent myelopathy presenting with monoplegia following asthmatic attacks". Fukuoka Igaku Zasshi = Hukuoka Acta Medica. 91 (3): 85–9. PMID 10826222.
4. Acharya, AB; Lakhani, PK (1997). "Hopkins syndrome associated with Mycoplasma infection". Pediatric Neurology. 16 (1): 54–5. doi:10.1016/S0887-8994(96)00248-2. PMID 9044403.
5. Nakano, Y; Kohira, R; Yamazaki, H; Fujita, N; Fuchigami, T; Okubo, O; Harada, K (2001). "Hopkins syndrome: oral prednisolone was effective for the paralysis". No to Hattatsu. Brain and Development. 33 (1): 69–73. PMID 11197900.
6. Arita, J; Nakae, Y; Matsushima, H; Maekawa, K (1995). "Hopkins syndrome: T2-weighted high intensity of anterior horn on spinal MR imaging". Pediatric Neurology. 13 (3): 263–5. doi:10.1016/0887-8994(95)00181-E. PMID 8554668.
7. Mizuno, Y; Komori, S; Shigetomo, R; Kurihara, E; Tamagawa, K; Komiya, K (1995). "Poliomyelitis-like illness after acute asthma (Hopkins syndrome): a histological study of biopsied muscle in a case". Brain & Development. 17 (2): 126–9. doi:10.1016/0387-7604(94)00125-H. PMID 7625547. S2CID 12879859.
8. Kira, J (2003). "Neural damage associated with allergic diseases: pathomechanism and therapy". Rinsho Shinkeigaku. 43 (11): 756–60. PMID 15152457.