SUBACUTE SCLEROSING PANENCEPHALITIS (A Report on Two Cases)

作者:疯疯颠颠  于 2019-9-20 01:43 发表于 最热闹的华人社交网络--贝壳村

通用分类:英文分享|已有6评论

SUBACUTE SCLEROSING PANENCEPHALITIS (A Report on Two Cases)


Abstract

We report two cases of subacute sclerosing panencephalitis (SSPE) which is a rare, late neurological sequel, of measles infection acquired in childhood. The diagnosis was reached by adopting criteria defined by Dyken. Past history of measles was present in both the cases and they showed high titres of measles specific antibody in CSF and serum by quantitative ELISA.

KEY WORDS: SSPE, Measles

Introduction

Subacute sclerosing panencephalitis is a distinct degenerative neurological disorder [], which is preceded by measles infection in the distant past, often early in life. Very occasionally measles immunisation may be the preceding event []. The reported incidence of SSPE is 8 per million after measles infection and 0.7 per million doses of measles vaccine []. Dyken (1985) laid down definite criteria to diagnose SSPE (a] classical presentation and clinical findings that include intellectual deterioration and myoclonus, (b) EEG findings that show characteristically periodic slow bizarre complexes, (c) elevated CSF immunoglobulin levels, (d) elevated CSF measles specific antibody and (e) classical histopathological findings. Presence of any two criteria is considered enough for a diagnosis of SSPE, but serial (a) and (d) are considered the most important markers []. There are many reports of SSPE from India [], but detailed immunological work up has been done in only one []. Quantitative ELISA has not been used for immunological confirmation of SSPE in any earlier reports.

TABLE 1
FeaturesPatient 1Patient 2
Clinical Data
Sex and ageMale 8 yearsFemale 8 years
Past history of measlesAt 18 months of ageAt 7 years of age
Presenting complaintsGradual scholastic deteriorationGradual scholastic deterioration
Jerky movements left sideJerky movements whole body
Inability to walkRepeated falls
Loss of speechResponse in monosyllables Bowel, bladder incontinence
Duration of illness on admission2 months1½ years
Immunisation historyDPT and MeaslesOnly DPT
Clinical findingsAphasiaDysarthria
Spasticity three limbs, decorticate posturing (Fig. 1)Flaccid paralysis both lower limbs (Fig. 2)
MyoclonusMyclonus
Exaggerated tendon reflexesExaggerated tendon reflexes
Planters upgoingPlanters upgoing
Chorioretinitis
EEGBilateral epileptiform dischargeAbnormal record suggestive of generalised epilepsy and burst suppression
CSF
 AppearanceClearClear
 Protein50 mg/dl50 mg/dl
 Sugar54 mg/dl80 mg/dl
  (Random blood sugar)(98 mg/dl)(117 mg/dl)
  GlobulinsElevatedElevated specific
Measles antibody titre
(ELISA units per ml)
 Serum107.9175.9
 CSF77.7117.7

Discussion

SSPE is now considered a slow viral disease that is caused by measles virus that is defective in its genes involving envelope proteins like M,F and H (1), which are needed for budding of virus. As no extracellular expression of viral antigens occurs at cell surface immune destruction of infected cells can not occur. The defective virus is selected out in brain. Being unable to bud out, it spreads slowly within the network of CNS cells in the presence of normal immune response. The virus in SSPE has been shown to be more neurotropic than the regular wild virus [].

SSPE follows one to ten years after measles. In the two cases reported, the interval has been seven years to as less as six months. In the first case, measles and SSPE have followed despite measles immunisation pointing to vaccination failure. The onset of SSPE is insidious with slow progression. Our first patient had a rapid progression in just two months. Scholastic deterioration was present in both the patients. Myoclonus, reportedly the most important clinical finding (4) was present in both. EEG was not classical in our patients since the expected typical periodic complexes were absent. It is the experience of other authors that typical first EEG is found in only 79% of cases, strict periodicity tending to occur with progression of disease [].

Haemagglutination inhibition assays have been used by many to detect measles antibodies (6,9) and qualitative ELISA by some []. Many authors believed, mere demonstration of measles antibody in CSF is enough to clinch diagnosis of SSPE []. The patients of SSPE are known to have antibody titres 10–100 times higher than is expected in convalescent samples of sera []. High CSF antibody titres dictate intrathecal synthesis. We have put up atraumatically collected normal CSF samples as controls and found that none gave titres > 20 EU/ml (the cut off value for positivity in the kit used – Diamedix, USA). It is also known that CSF antibody levels may fluctuate during SSPE with periods of total absence []. Therefore antibody alone is not enough to confirm or refute a diagnosis of SSPE and the totality of clinical and other criteria are to be relied upon [].

SSPE is generally a fatal disease. Some may die in few months, others may remain in a decerebrate state for years. Focal retinitis occurs in a majority causing blindness []. We found chorioretinitis in our second patient. There are reports claiming isoprenazine (Inosiplex) prolongs life in SSPE [].

That measles infection gives rise to a life long immunity is well known. The fact that the virus may remain sequestered in brain to produce a late sequel of SSPE have raised questions about the efficacy of viral clearance by a normally functioning immune defence.


REFERENCES
1. Norrby E, Oxman MN. Measles virus. In: Fields BN, Knipe DN, editors. Field's Virology, 2nd edn. Raven Press; New York: 1990. pp. 788–792. []
2. Landrigan PJ, White JJ. Subcute sclerosing panencephalitis following measles vaccination. JAMA. 1973;223:1459–1462. [PubMed[]
3. Conolly JH, Allen IV, Hurwitz LJ, Millar HJD. Measles virus antibody and antigen in subacute sclerosing panencephalitis. Lancet. 1967;1:542–544. [PubMed[]
4. Dyken PR. Subacute sclerosing panencephalitis. Neurology Clinics. 1985;3:182–184. []
5. Saha V, John TJ, Mukandan P. High incidence of sclerosing panencephalitis in South India. Epide-mol Infect. 1990;104(1):151–156. [PMC free article] [PubMed[]
6. Thakare JP, Wadia RS, Deuskar NI. Subacute sclerosing panencephalitis cases in Pune. Neurology India. 1987;35:333–339. []
7. Lakshmi V, Malathy Y, Rao RR. Serodiagnosis of subacute sclerosing panencephalitis by enzyme linked immunosorbent assy. Indian J Paediatr. 1993;60:37–41. [PubMed[]
8. White DO, Fenner F. Persistent infections. In: White DO, Fenner F, editors. Medical Virology, 3rd ed. Academic Press, Inc; Orlando: 1986. pp. 193–197. []
9. Broor S, Pal SR, Banerjee AK. Subacute sclerosing panencephalitis in Chandigarh. Indian J Med Res. 1979;70:536–539. [PubMed[]
10. Jones CE, Dyken RP, Huttenlocher PR. Inosiplex in subacute sclerosing panencephalitis. Lancet. 1982;1:1034–1036. [PubMed[]

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发表评论 评论 (6 个评论)

2 回复 疯疯颠颠 2019-9-20 01:49
袁锦楣教授1967年的英文论文。居然现在还那么热!
2 回复 浮平 2019-9-20 12:31
这篇1994年发表的文章作者中 MKK RAO,* RK GUPTA,+ VC OHRI,# S NARAYAN,** and SANGEETA JOSHI, Dr++ 哪一位是袁锦楣教授?

这篇 references 提及的1967年的文章作者中 Conolly JH, Allen IV, Hurwitz LJ, Millar HJD. Measles virus antibody and antigen in subacute sclerosing panencephalitis. Lancet. 1967 哪一位是袁锦楣教授?
回复 疯疯颠颠 2019-9-21 04:49
浮平: 这篇1994年发表的文章作者中 MKK RAO,* RK GUPTA,+ VC OHRI,# S NARAYAN,** and SANGEETA JOSHI, Dr++ 哪一位是袁锦楣教授?

这篇 references 提及的1967年的文
请参阅北京大学第一医院神经科人物风采
回复 疯疯颠颠 2019-9-21 04:52
请参阅北京大学第一医院神经科人物风采

https://pkufh.com/Html/News/Articles/17233.html
回复 浮平 2019-9-21 06:10
疯疯颠颠: 请参阅北京大学第一医院神经科人物风采
再请问,这上面的文章和 references为什么没有一篇与这位袁教授相关?

是因为年代,语言的因素,还是其它?

想理解一下这个风采是本国认可的还是国际的,以便区别人物风采是主观的,墙内的,自吹的还是有一定国际范围,客观依据的。

比如,屠呦呦的人物风采就有国际认可的文章和奖项。
回复 疯疯颠颠 2019-9-27 03:22
浮平: 再请问,这上面的文章和 references为什么没有一篇与这位袁教授相关?

是因为年代,语言的因素,还是其它?

想理解一下这个风采是本国认可的还是国际的,以便
一个是诺贝尔的的得奖人,一个是国内神经科著名医生,两者无可比性!

facelist doodle 涂鸦板

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