Hostname: page-component-848d4c4894-ndmmz Total loading time: 0 Render date: 2024-05-10T22:06:01.934Z Has data issue: false hasContentIssue false
  • English
  • Français

Simulation of Brain Death from Fulminant De-efferentation

Published online by Cambridge University Press:  02 December 2014

Yael Friedman ,
Liesly Lee ,
John R. Wherrett ,
Peter Ashby  and
Stirling Carpenter
Yael Friedman
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Liesly Lee
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
John R. Wherrett
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Peter Ashby
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Stirling Carpenter
Affiliation:
Division of Neurology, University of Toronto, Toronto, Ontario Canada
Rights & Permissions [Opens in a new window]

Abstract

Core share and HTML view are not available for this content. However, as you have access to this content, a full PDF is available via the ‘Save PDF’ action button.
Background:

Guillain-BarrÈ syndrome (GBS) classically presents with a subacutely evolving areflexic paralysis, with typical laboratory findings of elevated cerebrospinal fluid protein and abnormal nerve conduction studies. There is now an increasing recognition of GBS variants that differ in clinical presentation, prognosis, electrophysiology and presumed pathogenesis. Fulminant cases of GBS have been reported in which a rapid deterioration evolves to a clinical state resembling ìbrain deathî.

Methods:

A retrospective analysis of two such cases of fulminant neuropathy are described, that includes the clinical course, electrophysiology and neuropathology where available.

Results:

We describe two patients that presented with a rapid course of neurological deterioration, lapsing into what resembled a ìclinically brain-deadî state that was subsequently ascribed to a fulminant polyneuropathy. Investigations (electrophysiological, pathological) and the clinical course suggested an axonal neuropathy.

Conclusion:

A fulminant neuropathy can result in a clinical state resembling ìbrain deathî through diffuse de-efferentation. Although generally attributed to aggressive demyelination with secondary axonal degeneration, a primary axonopathy can also lead to a similar clinical presentation.

Résumé:

RÉSUMÉ:Introduction:

La présentation classique du syndrome de Guillain-Barré (SGB) consiste en une paralysie aréflexique évoluant de façon subaiguë accompagnée d'anomalies biochimiques typiques, soit une augmentation des protéines dans le liquide céphalorachidien et des anomalies de conduction nerveuse. On reconnaît maintenant qu'il existe des variantes du SGB qui diffèrent par leur présentation clinique, leur pronostic, les constatations électrophysiologiques et la pathogenèse présumée. Des cas fulminants de SGB chez qui une détérioration rapide évolue vers un état clinique ressemblant à la mort cérébrale ont été rapportés.

Méthodes:

Nous présentons une analyse rétrospective de deux cas de neuropathie fulminante, leur évolution clinique, ainsi que les constatations électrophysiologiques et neuropathologiques si elles étaient disponibles.

Résultats:

Nous décrivons deux patients qui ont consulté pour une détérioration neurologique rapide, les patients ayant éventuellement sombré dans un état qui ressemblait au point de vue clinique à la mort cérébrale, attribuée ultérieurement à une polyneuropathie fulminante. L'investigation électrophysiologique et anatomopathologique et l'évolution clinique suggéraient qu'il s'aggissait d'une neuropathie axonale.

Conclusions:

Une neuropathie fulminante peut induire un état clinique ressemblant à la mort cérébrale par de-efférentation diffuse. Bien qu'attribuée généralement à une démélinisation agressive avec dégénéressence axonale secondaire, une axonopathie primaire peut aussi entraîner une telle présentation clinique.

Type
Case Report
Information
Canadian Journal of Neurological Sciences , Volume 30 , Issue 4 , November 2003 , pp. 397 - 404
Copyright
Copyright © The Canadian Journal of Neurological 2003

References

1. Haymaker, W, Kernohan, JW. The Landry-Guillain-Barré syndrome. A clinicopathologic report of fifty fatal cases and a critique of the literature. Medicine (Baltimore) 1949; 28:59–141.Google Scholar
2. Asbury, AK, Arnason, BGW, Adams, RD. The inflammatory lesion in idiopathic polyneuritis. Its role in pathogenesis. Medicine (Baltimore) 1969; 58:173–215.Google Scholar
3. Prineas, JW. Pathology of the Guillain-Barré syndrome. Ann Neurol 1981; 9(Suppl):6–19.Google Scholar
4. Ng, KKP, Howard, RS, Fish, DR, et al. Management and outcome of severe Guillain-Barré syndrome. QJ Med 1995; 88:243–250.Google Scholar
5. Lawn, ND, Wijdicks, EF. Fatal Guillain-Barré syndrome. Neurol 1999; 52:635.Google Scholar
6. Thomas, PK. The Guillain-Barré syndrome: no longer a simple concept. J Neurol 1992; 239:361–362.CrossRefGoogle ScholarPubMed
7. Bolton, CF. The changing concepts of Guillain-Barré Syndrome. N Engl J Med 1995; 333:1415–1416.Google Scholar
8. Feasby, TE, Gilbert, JJ, Brown, WF, et al. An acute axonal form of Guillain-Barré polyneuropathy. Brain 1986; 109:1115–1126.Google Scholar
9. McLeod, JG. Electrophysiological studies in the Guillain-Barré syndrome. Ann Neurol 1981; 9:20–27.CrossRefGoogle ScholarPubMed
10. Brown, WF, Feasby, TE. Conduction block and denervation in Guillain-Barré polyneuropathy. Brain 1984; 107:219–239.Google Scholar
11. Brown, WF, Snow, R. Patterns and severity of conduction abnormalities in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 1991; 54:768–774.Google Scholar
12. Hall, SM, Hughes, RAC, Atkinson, PF, McColl, I, Gale, A. Motor nerve biopsy in severe Guillain-Barré syndrome. Ann Neurol 1992; 31:441–444.Google Scholar
13. Cros, D, Triggs, WJ. There are no neurophysiologic features characteristic of “axonal” Guillain-Barré syndrome. Muscle Nerve 1994; 17:675–677.Google Scholar
14. McKhann, GM, Cornblath, DR, Ho, T, et al. Clinical and electrophysiological aspects of acute paralytic disease in children and young adults in northern China. Lancet 1991; 338:593–597.CrossRefGoogle Scholar
15. McKhann, GM, Cornblath, DR, Griffin, JW, et al. Acute motor axonal neuropathy: a frequent cause of acute flaccid paralysis in China. Ann Neurol 1993; 33:333–342.Google Scholar
16. Griffin, JW, Li, CY, Ho, TW et al. Guillain-Barré syndrome in northern China: the spectrum of neuropathologic changes in clinically defined cases. Brain 1995; 118:577–595.Google Scholar
17. Griffin, JW, Li, CY, Ho, TW, et al. Pathology of the motor-sensory axonal Guillain-Barré syndrome. Ann Neurol 1996; 39:17–28.Google Scholar
18. Carroll, WM, Mastaglia, FL. “Locked-in coma” in postinfectious polyneuropathy. Arch Neurol 1979; 36:46–47.Google Scholar
19. Kotsoris, H, Schleifer, L, Menken, M, Plum, F. Total locked-in state resembling brain death in polyneuropathy. Ann Neurol 1984; 16:150.Google Scholar
20. Al-Din, ASN, Jamil, AS, Shakir, R. Coma and brains stem areflexia in brain stem encephalitis (Fisher’s syndrome). Br Med J 1985; 291:535–536.Google Scholar
21. Drury, I, Westmoreland, BF, Sharbrough, FW. Fulminant demyelinating polyradiculoneuropathy resembling brain death. Electroencephalogr Clin Neu ophysiol 1987; 67:42–43.Google Scholar
22. Coad, NR, Byren, AJ. Guillain-Barré syndrome mimicking brainstem death. Anaesthesia 1990; 45:456–457.Google Scholar
23. Hassan, T, Mumford, C. Guillain-Barré syndrome mistaken for brain stem death. Postgrad Med J 1991; 67:280–281.Google Scholar
24. Martí-Massó, JF, Suárez, J, López de Munain, A, Carrera, N. Clinical signs of brain death simulated by Guillain-Barré syndrome. J Neurol Sci 1993; 120:115–117.Google Scholar
25. Tan, AKY, Chee, MWL. Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination. J Neurol Sci 1995; 134:203–206.Google Scholar
26. Fuller, GN, Jacobs, JM, Lewis, PD, Lane, RJM. Pseudoaxonal Guillain-Barré syndrome: severe demyelination mimicking axonopathy. A case with pupillary involvement. J Neurol Neurosurg Psychiatry 1992; 55:1079–1083.Google Scholar
27. Kanda, T, Hayashi, H, Tanabe, H, Tsubaki, T, Oda, M. A fulminant case of Guillain-Barré syndrome: topographic and fibre size related analysis of demyelinating changes. J Neurosurg Psychiatry 1989; 52:857–864.Google Scholar
28. Bohlega, SA, Stigsby, B, Haider, A, et al. Guillain-Barré syndrome with severe demyelination mimicking axonopathy. Muscle Nerve 1997; 20:514–516.Google Scholar
29. Berciano, J, Figols, J, Garcia, A, et al. Fulminant Guillain-Barre syndrome with universal inexcitability of peripheral nerves: a clinicopathological study. Muscle Nerve 1997; 20:846–857.Google Scholar
30. Vargas, F, Hilbert, G, Gruson, D, et al. Fulminant Guillain-Barre syndrome mimicking cerebral death: case report and literature review. Intensive Care Med 2000; 26:623–627.Google Scholar
31. Bakshi, N, Maselli, R, Gospe, SM, et al. Fulminant demyelinating neuropathy mimicking cerebral death. Muscle Nerve 1997; 20:1595–1597.Google Scholar
32. Hughes, R, McGuire, G. Neurological disease and the determination of brain death: the importance of a diagnosis. Crit Care Med 1997; 25: 1923–1924.Google Scholar
33. Feasby, TE, Hahn, AF, Brown, WF, et al. Severe axonal degeneration in acute Guillain-Barre syndrome: evidence of two different mechanisms? J Neurol Sci 1993;116: 185–192.Google Scholar
34. Ho, TW, Li, CY, Cornblath, DR, et al. Patterns of recovery in the Guillain-Barre syndromes. Neurology 1997a; 48:695–700.Google Scholar
35. Ho, TW, Hsieh, S-T, Nachamkin, I, et al. Motor nerve terminal degeneration provides a potential mechanism for rapid recovery in acute motor axonal neuropathy after Campylobacter infection. Neurology 1997b; 48:717–724.Google Scholar