Skip to main content
SpringerLink
Log in

An uncommon case of amyotrophic lateral sclerosis with isolation of a virus from the CSF

  • Original Investigations
  • Published:
Journal of Neurology Aims and scope Submit manuscript

Summary

An atypical case of amyotrophic lateral sclerosis (ALS) is described, characterized by early manifestation, a long lasting course with asymmetry of the lesions, absence of bulbar symptoms in the presence of an otherwise very advanced symptomatology, and constant signs of an inflammatory reaction in the CSF which was the reason to initiate extensive virological studies, including procedures for virus isolation. A virus, belonging to the TbE complex of arbovirus group B (tick-borne flaviviruses), was finally isolated from the CSF. About 70% of the ALS cases in Hamburg/W. Germany, examined for antibodies, appearently had contact with this virus. The antibody pattern found made it possible to explain this exceptional case.

Zusammenfassung

Ein atypischer Fall von myatrophischer Lateralsklerose wurde beschrieben, dessen Besonderheit auf einer Frühmanifestation, auf einer länger dauernden Asymmetrie der Initialsymptome, auf dem Fehlen einer bulbären Symptomatik bei sonst sehr fortgeschrittener Symptomatik, auf einem ungewöhnlich langen Verlauf und auf konstanten Zeichen einer entzündlichen Reaktion im Liquor beruht. Die konstante Liquor-Pleocytose war Anlaß, ausgedehnte virologische Untersuchungen einzuleiten, die im Versuch einer Virusisolierung gipfelten. Es wurde schließlich aus dem Liquor ein Virus isoliert, welches dem TbE-Komplex der Arbovirus-Gruppe B (tick-borne Flaviviren) zugehört. Etwa 70% einer Gruppe von 19 ALS-Patienten aus Hamburg hatten mit diesem Virus offenbar Kontakt. Das Antikörpermuster des Falles ermöglichte eine Erklärung seiner Besonderheiten.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Similar content being viewed by others

References

  • Allen, L. V., Dermott, E., Conoly, J. H., Hurwitz, L. J.: A study of a patient with the amyotrophic form of Creutzfeldt-Jakob-disease. Brain 94, 715–724 (1971)

    Article  CAS  PubMed  Google Scholar 

  • Assmus, H., Thiele, R. M.: Gibt es extrem chronische Verläufe bei der myatrophischen Lateralsklerose? Nervenarzt 44, 609–610 (1973)

    CAS  PubMed  Google Scholar 

  • Blinzinger, K. H.: Comparative electron microscopic studies of several experimental group B arbovirus infections of the murine CNS (CEE virus, Zimmern virus, Yellow fever virus). Ann. Inst. Pasteur 123, 497–519 (1972)

    CAS  Google Scholar 

  • Bodechtel, G.: Die nukleären Atrophien — ein postenzephalitisches Zustandsbild? Dtsch. Z. Nervenheilk. 158, 439–446 (1948)

    Article  CAS  Google Scholar 

  • Boman, K., Meurman, T.: Prognosis of amyotrophic lateral sclerosis. Acta neurol. scand. 43, 489–498 (1967)

    Article  CAS  PubMed  Google Scholar 

  • Bonduelle, M.: Amyotrophic lateral sclerosis. Handbook of clinical neurology. P. J. Vinken, G. W. Bruyn, eds. Vol. 22, 281–338. Amsterdam-Oxford: North-Holland Publ. Comp. 1975

    Google Scholar 

  • Brody, J. A.: Chronic sequelae of tick-borne enzephalitis and Vilyuisk enzephalitis. NINDB Monograph No. 2. Slow, latent and temperate virus infections (eds. C. J. Gibbs, D. C. Gajdusek), pp. 111–113. Washington D.C.: US Gov. Print. Office 1965

    Google Scholar 

  • Bunina, T. L.: Les insertions intracellulaires dans la sclerose amyotrophique laterale familiale congenitale. Zh. Nevropat. Psikhiat. 62, 1293–1299 (1962)

    CAS  Google Scholar 

  • Burg, D., Schmitt, W.: Beitrag zur Klinik und Prognose der amyotrophischen Lateralsklerose. Fortschr. Neurol. Psychiat. 41, 639–652 (1973)

    CAS  Google Scholar 

  • Casals, J.: Immunological characterization of Vilyuisk human encephalomyelitis virus. Nature (Lond.) 200, 339–341 (1963)

    Article  CAS  PubMed  Google Scholar 

  • Catalano, L. W.: Herpesvirus hominis antibody in multiple sclerosis and amyotrophic lateral-sclerosis. Neurology (Minneap.) 22, 473–478 (1972)

    PubMed  Google Scholar 

  • Colmant, H. J.: Die chronisch-progressive Bulbärparalyse. Hdb. spez. path. Anat. Bd. 13/2 B, S. 2571–2584. Berlin-Heidelberg-New York: Springer 1968

    Google Scholar 

  • Denk, H., Kovac, W.: Zur Entwicklung des morphologischen Bildes der experimentellen FSEM (Frühsommer-Encephalomyelitis) der weißen Maus. Acta neuropath. (Berl.) 7, 62–69 (1966a)

    Article  Google Scholar 

  • Denk, H., Kovac, W.: Zur experimentellen Frühsommer-Encephalomyelitis (FSEM) der weißen Maus. Lokalisation der encephalitischen Läsionen. Acta neuropath. (Berl.) 7, 162–174 (1966b)

    Article  Google Scholar 

  • Doherty, P. C., Reid, H. W.: Louping-ill encephalomyelitis in the sheep. II. Distribution of virus and lesions in nervous tissue. J. comp. Path. 81, 531–536 (1971)

    Article  CAS  PubMed  Google Scholar 

  • Doherty, P. C., Rennie, J. C., Smith, W.: The effect of concurrent scrapie on louping-ill encephalitis in the mouse. Res. vet. Sci. 13, 146–149 (1972)

    CAS  PubMed  Google Scholar 

  • Erbslöh, F.: Atrophierende Prozesse. In: Differentialdiagnose neurologischer Krankheitsbilder (ed. G. Bodechtel), 3. Aufl. Stuttgart: Thieme 1974

    Google Scholar 

  • Erbslöh, F., Kunze, K., Recke, B., Abel, M.: Die myatrophische Lateralsklerose. Dtsch. med. Wschr. 93, 1131–1141 (1968)

    PubMed  Google Scholar 

  • Gibbs, C. J., Gajdusek, D. C.: Kuru — a prototype subacute infectious disease of the nervous system as a model for the study of amyotrophic lateral sclerosis. In: Motor neuron diseases (eds. F. H. Norris, L. T. Kurland), pp. 269–279. New York-London: Grune & Stratton 1969

    Google Scholar 

  • Gibbs, C. J., Gajdusek, D. C.: Experimental subacute spongiform virus encephalopathies in primates and other laboratory animals. Science 182, 67–68 (1973)

    Article  PubMed  Google Scholar 

  • Gibbs, C. J., Gajdusek, D. C., Asher, D. M., Alpers, M. P., Beck, E., Daniel, P. M., Matthews, W. B.: Creutzfeldt-Jakob disease (spongiform encephalopathy). Transmission to the chimpanzee. Science 161, 388–389 (1968)

    Article  PubMed  Google Scholar 

  • Henner, K., Hanzal, F.: Die tschechoslowakische Zeckenencephalitis und die sog. Rošňava-Enzephalitis. Psychiat. Neurol. med. Psychol. (Lpz.) 12, 161–169 (1960)

    CAS  PubMed  Google Scholar 

  • Huang, A. S., Baltimore, D.: Defective viral particles and viral disease processes. Nature (Lond.) 226, 325–327 (1970)

    Article  CAS  PubMed  Google Scholar 

  • Janzen, R.: Erkrankung — Krankheit — Reaktionsform, Entität. Dtsch. med. Wschr. 93, 1463–1467 (1968)

    CAS  PubMed  Google Scholar 

  • Kuimov, D. T., Dubov, A. V.: Amyotrophic lateral sclerosis as a vernal encephalitis syndrome: 20th Anniversary of the discovery and research of Soviet scientists on vernal encephalitis. Zh. Nevropat. Psikhiat. 58, 282–287 (1958)

    CAS  Google Scholar 

  • Kurland, L. T.: Amyotrophic lateral sclerosis. In: Slow, latent and temperate virus infections (eds. C. J. Gibbs, D. C. Gajdusek, M. Alpers), pp. 13–22. NINDB Monograph No. 2. Washington D.C.: US Gov. Print. Office 1965

    Google Scholar 

  • Likar, M., Dane, D. S.: An illness resembling acute poliomyelitis caused by a virus of the RSSE-Louping-ill group in Northern Ireland. Lancet 1958 II, 456–458

    Article  Google Scholar 

  • Müller, W. K., Schaltenbrand, G.: Arboviruserkrankungen in Mitteleuropa. Stuttgart: Thieme 1975

    Google Scholar 

  • Müller, W. K.: Slow virus infections of the nervous system. In: Scientific foundations of neurology (eds. McD. Critchley, J. L. O'Leary, B. Jennett), pp. 459–468. London: Heinemann 1973

    Google Scholar 

  • Müller-Jensen, A., Bernhardt, W.: Unsere Erfahrungen bei der myatrophischen Lateralsklerose. Nervenarzt 44, 143–149 (1973)

    PubMed  Google Scholar 

  • Myrianthopoulos, N. C., Smith, J. K.: Amyotrophic lateral sclerosis with progressive dementia and with pathologic findings of the Creutzfeldt Jakob syndrome. Neurology (Minneap.) 12, 603–610 (1962)

    CAS  PubMed  Google Scholar 

  • Poskanzer, D. C., Cantor, H. M., Kaplan, G. S.: The frequency of preceding poliomyelitis in amyotrophic lateral sclerosis. In: Motor neuron diseases (eds. F. H. Norris, L. T. Kurland), pp. 286–290. New York-London: Grune & Stratton 1969

    Google Scholar 

  • Protas, I. I., Votyakov, V. I.: Concerning the nosological interrelationships between lateral amyotrophical sclerosis and tick-borne encephalitis. Zh. Nevropat. Psikhiat. 70, 1124–1129 (1970)

    CAS  Google Scholar 

  • Richling, E.: Virusmeningoencephalitis in Austria. 1. Epidemiological features. Bull. Wld Hlth Org. 12, 521–532 (1955)

    CAS  Google Scholar 

  • Verlinde, J. D., van Tongeren, H. A. E., Pattyn, S. R., Rosenzweig, A.: Virus meningoencephalitis in Austria. 3. Pathogenic and immunological properties of the virus. Bull. Wld Hlth Org. 12, 565–569 (1955)

    CAS  Google Scholar 

  • Zilber, L. A., Bajdakova, Z. L., Gardas, A. N., Konovalov, N., Bunia, T. L., Barabadze, E. M.: Über die mögliche Virusätiologie der amyotrophischen Lateralsklerose. Vop. Virus. 520–528 (1962)

  • Zilber, L. A., Bajdakova, Z. L., Gardas, A. N., Konovalov, N., Bunia, T. L., Barabadze, E. M.: Study of the etiology of amyotrophic lateral sclerosis. Bull. Wld Hlth Org. 29, 449–456 (1963)

    CAS  Google Scholar 

  • Zilkha, K. J.: In: Discussion on motor neuron disease. Proc. roy. Soc. Med. 55, 1028–1029 (1962)

    CAS  PubMed  Google Scholar 

  • Zlotnik, I., Carter, G. B., Grant, D. P.: The persistence of Louping-ill virus in immuno-suppressed Guinea-pigs. Brit. J. exp. Path. 52, 395–407 (1971)

    CAS  PubMed  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Clinic of Neurology, University Hospital Hamburg-Eppendorf, Germany

    W. K. Müller, F. Hilgenstock, L. v. d. Kuip & F. Leupe

  2. Laboratory of Medical Microbiology of the State University, Leiden, The Netherlands

    W. K. Müller, F. Hilgenstock, L. v. d. Kuip & F. Leupe

Authors
  1. W. K. Müller
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. F. Hilgenstock
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. L. v. d. Kuip
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. F. Leupe
    View author publications

    You can also search for this author in PubMed Google Scholar

Additional information

We thank the Prinses-Beatrix-fonds for generous support.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Müller, W.K., Hilgenstock, F., v. d. Kuip, L. et al. An uncommon case of amyotrophic lateral sclerosis with isolation of a virus from the CSF. J. Neurol 211, 11–23 (1975). https://doi.org/10.1007/BF00312460

Download citation

  • Received:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00312460

Key words

Search

Navigation