Skip to main content
SpringerLink
Log in

A clinical, epidemiological and genetic study of hereditary motor neuropathies in Benghazi, Libya

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

Summary

A 4-year-search for spinal muscular atrophies (hereditary motor neuropathies, HMN) in Benghazi, Libya, yielded a total of 24 patients, among whom 18 were index cases. This group comprised 6 acute infantile, 12 chronic childhood, and 3 each with adult-onset proximal, and distal forms of the disorder. Distal HMN constituted 12.5% of the total cases. The crude average annual incidence of acute infantile HMN was 0.3/100,000 total population and 1/12,500 births in Benghazi. The crude prevalence rates of chronic childhood, adult-onset proximal, and distal types of HMN were 2.3, 0.6, and 0.6/100,000 respectively. The segregation ratios, 0.26 for acute infantile HMN and 0.24 for chronic childhood HMN, suggested autosomal recessive inheritance. The consanguinity rates among parents of cases and the population did not differ significantly.

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

  1. Brandt S (1949) Hereditary factors in infantile progressive muscular atrophy. Am J Dis Child 78:226–230

    Google Scholar 

  2. Emery AEH (1971) Review. The nosology of the spinal muscular atrophies. J Med Genet 8:481–495

    Google Scholar 

  3. Emery AEH, Hausmanowa-Petrusewicz I, Davie AM, Holloway S, Skinner R, Borkowska J (1976) International collaborative study of the spinal muscular atrophies. I. Analysis of clinical and laboratory data. J Neurol Sci 29:83–94

    Google Scholar 

  4. Emery AEH, Davie AM, Holloway S, Skinner R (1976) International collaborative study of the spinal muscular atrophies. II. Analysis of genetic data. J Neurol Sci 30:375–384

    Google Scholar 

  5. Fried K, Mundel G (1977) High incidence of spinal muscular atrophy type I (Werdnig-Hoffmann disease) in the Karaite community in Israel. Clin Genet 12:250–255

    Google Scholar 

  6. Harding AE (1984) Inherited neuronal atrophy and degeneration predominantly of lower motor neurons. In: Dyck PJ, Thomas PK, Lambert EH, Bunge R (eds) Peripheral neuropathy, vol II. Saunders, Philadelphia, pp 1537–1556

    Google Scholar 

  7. Kurtzke JF (1984) Neuroepidemiology. Ann Neurol 16:265–277

    Google Scholar 

  8. Lander CM, Eadie MJ, Tyrer JH (1976) Hereditary motor peripheral neuropathy predominantly affecting the arms. J Neurol Sci 28:389–393

    Google Scholar 

  9. Magee KB, De Jong RN (1960) Neurogenic muscular atrophy simulating muscular dystrophy. Arch Neurol 2:677–682

    Google Scholar 

  10. O'Sullivan DJ, McLeod JG (1978) Distal chronic spinal muscular atrophy involving the hands. J Neurol Neurosurg Psychiatry 41:653–658

    Google Scholar 

  11. Pearn JH (1973) The gene frequency of acute Werdnig-Hoffmann disease (SMA type I). A total population survey in North-East England. J Med Genet 10:260–265

    Google Scholar 

  12. Pearn JH (1978) Incidence, prevalence, and gene-frequency studies of chronic childhood spinal muscular atrophy. J Med Genet 15:409–413

    Google Scholar 

  13. Pearn JH (1978) Genetic studies of acute infantile spinal muscular atrophy (SMA type I). An analysis of sex ratios, segregation ratios and sex influence. J Med Genet 15:414–417

    Google Scholar 

  14. Pearn JH (1978) Segregation analysis of chronic childhood spinal muscular atrophy. J Med Genet 15:418–423

    Google Scholar 

  15. Pearn JH (1980) Classification of spinal muscular atrophies. Lancet I:919–922

    Google Scholar 

  16. Pearn JH, Hudgson P (1979) Distal spinal muscular atrophy — a clinical and genetic study of 8 kindreds. J Neurol Sci 43:183–191

    Google Scholar 

  17. Pearn JH, Hudgson P, Walton JN (1978) A clinical and genetic study of spinal muscular atrophy of adult-onset. The autosomal recessive form as a discrete disease entity. Brain 101:591–606

    Google Scholar 

  18. Radhakrishnan K, Ashok PP, Sridharan R, Mousa ME (1985) Prevalence and pattern of multiple sclerosis in Benghazi, Northeastern Libya. J Neurol Sci 70:39–46

    Google Scholar 

  19. Radhakrishnan K, Ashok PP, Sridharan R, Mousa ME (1986) Descriptive epidemiology of motor neuron disease in Benghazi, Libya. Neuroepidemiology 5:47–54

    Google Scholar 

  20. Radhakrishnan K, El-Mangoush MA, Gerryo SE (1987) Descriptive epidemiology of selected neuromuscular disorders in Benghazi, Libya. Acta Neurol Scand 75:95–100

    Google Scholar 

  21. Winsor EJ, Murphy EG, Thompson MW, Reed TE (1971) Genetics of childhood spinal muscular atrophy. J Med Genet 8:143–148

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Neurology Unit, Department of Medicine, Arab Medical University, P.O. Box 13426, Benghazi, Libya

    K. Radhakrishnan, A. K. Thacker & J. C. Maloo

Authors
  1. K. Radhakrishnan
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. A. K. Thacker
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. J. C. Maloo
    View author publications

    You can also search for this author in PubMed Google Scholar

Rights and permissions

Reprints and permissions

About this article

Cite this article

Radhakrishnan, K., Thacker, A.K. & Maloo, J.C. A clinical, epidemiological and genetic study of hereditary motor neuropathies in Benghazi, Libya. J Neurol 235, 422–424 (1988). https://doi.org/10.1007/BF00314486

Download citation

  • Received:

  • Revised:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00314486

Key words

Search

Navigation