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Sural nerve pathology in ALS patients: a single-centre experience

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Abstract

Amyotrophic lateral sclerosis (ALS) is a progressive degenerative disease of upper and lower motor neurons. Sensory involvement is thought not to be a feature of ALS. We reviewed 17 cases of sural nerve biopsies performed in a large cohort of ALS patients referred to our centre over a 23-year period. More than two-third of biopsies revealed a variable degree of axonal loss. In one case, pathological findings suggested the concomitant presence of an inherited neuropathy, subsequently confirmed by genetic evaluation. In another case, pathological and neurographic data were similar to those of an inflammatory demyelinating neuropathy, but the clinical course corroborated the diagnosis of ALS. Our data confirm that sensory nerve involvement may be found in ALS patients. This finding should prompt physicians to carefully investigate a possible alternative diagnosis, but does not exclude the possibility that the patient may have ALS.

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References

  1. Haverkamp LJ, Appel V, Appel SH (1995) Natural history of amyotrophic lateral sclerosis in a database population. Validation of a scoring system and a model for survival prediction. Brain 118:707–719

    Article  PubMed  Google Scholar 

  2. Brooks BR, Miller RG, Swash M, Munsat TL, World Federation of Neurology Research Group on Motor Neuron Diseases (2000) El Escorial revisited: revised criteria for the diagnosis of amyotrophic lateral sclerosis. Amyotroph Lateral Scler Other Motor Neuron Disord 1:293–299

    Article  PubMed  CAS  Google Scholar 

  3. Hammad M, Silva A, Glass J, Sladky JT, Benatar M (2007) Clinical, electrophysiologic, and pathologic evidence for sensory abnormalities in ALS. Neurology 69:2236–2242

    Article  PubMed  CAS  Google Scholar 

  4. Oey PL, Vos PE, Wieneke GH, Wokke JH, Blankestijn PJ, Karemaker JM (2002) Subtle involvement of the sympathetic nervous system in amyotrophic lateral sclerosis. Muscle Nerve 25:402–408

    Article  PubMed  Google Scholar 

  5. Desai J, Swash M (1999) Extrapyramidal involvement in amyotrophic lateral sclerosis: backward falls, retropulsion. J Neurol Neurosurg Psychiatry 67:214–216

    Article  PubMed  CAS  Google Scholar 

  6. Lloyd CM, Richardson MP, Brooks DJ, Al-Chalabi A, Leigh PN (2000) Extramotor involvement in ALS: PET studies with the GABA(A) ligand [(11)C]flumazenil. Brain 23:2289–2296

    Article  Google Scholar 

  7. Pugdahl K, Fuglsang-Frederiksen A, de Carvalho M, Johnsen B, Fawcett PR, Labarre-Vila A, Liguori R, Nix WA, Schofield IS (2007) Generalised sensory system abnormalities in amyotrophic lateral sclerosis: a European multicentre study. J Neurol Neurosurg Psychiatry 78:746–749

    Article  PubMed  CAS  Google Scholar 

  8. Isaacs JD, Dean AF, Shaw CE, Al-Chalabi A, Mills KR, Leigh PN (2007) Amyotrophic lateral sclerosis with sensory neuropathy: part of a multisystem disorder? J Neurol Neurosurg Psychiatry 78:750–753

    Article  PubMed  Google Scholar 

  9. Wechsler IS, Brock S, Weil A (1929) Amyotrophic lateral sclerosis with objective and subjective (neuritic) sensory disturbances A clinical and pathologic report. Arch Neurol Psychiatry (Chic) 21:299–310

    Google Scholar 

  10. Sabatelli M, Zollino M, Luigetti M, Del Grande A, Lattante S, Marangi G, Lo Monaco M, Madia F, Meleo E, Bisogni G, Conte A (2011) Uncovering ALS phenotypes: clinical features and long-term follow-up of UMN-D ALS. Amyotroph Lateral Scler 12:278–282

    Article  PubMed  Google Scholar 

  11. Sommer CL, Brandner S, Dyck PJ, Harati Y, LaCroix C, Lammens M, Magy L, Mellgren SI, Morbin M, Navarro C, Powell HC, Schenone AE, Tan E, Urtizberea A, Weis J, Society Peripheral Nerve (2010) Peripheral Nerve Society Guideline on processing and evaluation of nerve biopsies. J Peripher Nerv Syst 15:164–175

    Article  PubMed  Google Scholar 

  12. Weis J, Katona I, Müller-Newen G, Sommer C, Necula G, Hendrich C, Ludolph AC, Sperfeld AD (2011) Small-fiber neuropathy in patients with ALS. Neurology 76: 2024–2029

  13. Joint Task Force of the EFNS, the PNS. European Federation of Neurological Societies/Peripheral Nerve Society (2010) Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society—First Revision. J Peripher Nerv Syst 15:1–9

    Article  Google Scholar 

  14. O’Sullivan SS, McCarthy A, Mullins GM, McNamara B, Sweeney BJ (2006) ALS in a patient with hereditary neuropathy with liability to pressure palsy. Neurology 67:2260

    Article  PubMed  Google Scholar 

  15. Canali E, Chiari A, Sola P, Fioravanti V, Valzania F, Pentore R, Nichelli P, Mandrioli J (2010) Rapidly progressive amyotrophic lateral sclerosis in a young patient with hereditary neuropathy with liability to pressure palsies. Amyotroph Lateral Scler 11:335–336

    Article  PubMed  Google Scholar 

  16. Bhatt A, Farooq MU, Aburashed R, Kassab MY, Majid A, Bhatt S, Naravetla B, Dhaliwal G (2008) Hereditary neuropathy with liability to pressure palsies and amyotrophic lateral sclerosis. Neurol Sci 30:241–245

    Article  Google Scholar 

  17. Devigili G, Uçeyler N, Beck M, Reiners K, Stoll G, Toyka KV, Sommer C (2011) Vasculitis-like neuropathy in amyotrophic lateral sclerosis unresponsive to treatment. Acta Neuropathol 122:343–352

    Article  PubMed  Google Scholar 

  18. Collins MP, Dyck PJ, Gronseth GS, Guillevin L, Hadden RD, Heuss D, Léger JM, Notermans NC, Pollard JD, Said G, Sobue G, Vrancken AF, Kissel JT, Society Peripheral Nerve (2010) Peripheral Nerve Society Guideline on the classification, diagnosis, investigation, and immunosuppressive therapy of non-systemic vasculitic neuropathy: executive summary. J Peripher Nerv Syst 1:176–184

    Article  Google Scholar 

  19. Rajabally YA, Jacob S (2008) Chronic inflammatory demyelinating polyneuropathy-like disorder associated with amyotrophic lateral sclerosis. Muscle Nerve 38:855–860

    Article  PubMed  Google Scholar 

  20. Camdessanché JP, Belzil VV, Jousserand G, Rouleau GA, Créac’h C, Convers P, Antoine JC (2011) Sensory and motor neuronopathy in a patient with the A382P TDP-43 mutation. Orphanet J Rare Dis 6:4

    Article  PubMed  Google Scholar 

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Correspondence to Marco Luigetti.

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Luigetti, M., Conte, A., Del Grande, A. et al. Sural nerve pathology in ALS patients: a single-centre experience. Neurol Sci 33, 1095–1099 (2012). https://doi.org/10.1007/s10072-011-0909-5

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  • DOI: https://doi.org/10.1007/s10072-011-0909-5

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