Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination

Tan, A. K. Y.; Chee, Michael W.L.

doi:10.1016/0022-510x(95)00235-8

Cited by 18 publications

(12 citation statements)

References 10 publications

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“…58,61,63,67 Evolution into a state like brain death observed here had already been recognized. 14,44,70 General autopsy findings, including panacinar emphysema and micronodular cirrhosis with globules positive to the PAS-diastase and specific immunoperoxidase staining for AAT, suggest that our patient had had an AAT deficiency. 69 Unfortunately this diagnosis was not clinically suspected and confirmatory tests, such as direct measurement of serologic AAT or its phenotype, were not done.…”

Section: Discussionmentioning

confidence: 76%

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

et al. 1997

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The pathological basis of nerve inexcitability in GuillainBarré syndrome has not been established with certainty. We report the clinicopathological findings in a 67-year-old patient with fulminant GuillainBarré syndrome who died 18 days after onset. Three serial electrophysiological studies revealed nerve inexcitability. Antibodies to Campylobacter jejuni were present but there was no antiganglioside reactivity. Spinal root sections revealed extensive and almost pure macrophage-associated demyelination with occasional presence of T lymphocytes and neutrophil leukocytes. Conversely, in femoral, median, and sural nerves the outstanding lesion was axonal degeneration, with some denuded axons remaining. Unmyelinated fibers, posterior root ganglia, and dorsal columns were preserved. Endoneurial postcapillary venules showed plump endothelial cells with loss of their tight junctions. We conclude that both primary demyelination and axonal degeneration secondary to inflammation account for nerve inexcitability. Our findings lend support to the hypothesis of increased endoneurial pressure as the cause of wallerian degeneration in nerve trunks. The Guillain-Barré syndrome (GBS) is an acute or subacute evolving paralytic disease of unestablished etiology with characteristic pathological features of macrophage and lymphocytic infiltration of peripheral nerve with myelin destruction. 2,3 Nerve conduction studies reveal the typical findings of an acute multifocal demyelinating polyradiculoneuropathy consisting of proximal or distal conduction block and slowing of conduction. 1,9,16,39,46,62 In a small proportion of GBS cases, however, one or more peripheral nerves are inexcitable. 1,62,72,73 Because of the scarcity of detailed autopsy studies, 5,[18][19][20]75 the pathological basis of nerve inexcitability in GBS is controversial, three explanations being possible: distal demyelination with conduction block, secondary wallerian degeneration following demyelination, or primary and severe axonopathy. 5,10,[18][19][20]49,72,73,75 Serial electrophysiological studies have shown that conduction block, the physiological hallmark of demyelination, often precedes the appearance of axonal degeneration. 72,73 Exceptionally inexcitable nerves may be an early and inaugural electrophysiologic feature which has been related to axonal degenera-

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Section: Discussionmentioning

confidence: 76%

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

et al. 1997

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“…Polyneuropathy is sometimes absent from the initial clinical picture 4. The outcome of lumbar puncture and the investigations are therefore all-important in this case 6,7. In some patients, a pathological analysis with nerve biopsy has been performed 3,8–10.…”

Section: Discussionmentioning

confidence: 98%

Long-term impact after fulminant Guillain-Barré syndrome, case report and literature review

Rougé¹,

Lemarié²,

Gibot³

et al. 2016

IMCRJ

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A 47-year-old man was admitted to the intensive care unit a few hours after presenting to emergency department with acute diplopia and dysphonia. Swallowing disorders and respiratory muscular weakness quickly required invasive ventilation. On day 3, the patient was in a “brain-death”-like state with deep coma and absent brainstem reflexes. Electroencephalogram ruled out brain death diagnosis as a paradoxical sleep trace was recorded. Cerebrospinal fluid analysis, electrophysiologic studies, and a recent history of diarrhea led to the diagnosis of Campylobacter jejuni-related fulminant Guillain-Barré syndrome (GBS) mimicking brain death. The outcome was favorable after long Intensive Care Unit and inpatient rehabilitation stays, despite persistent disability at 9 years follow-up. This case and the associated literature review of 34 previously reported fulminant GBS patients emphasize the importance of electrophysiological investigations during clinical brain-death states with no definite cause. Fulminant GBS has a worse outcome than “standard” GBS with higher rates of severe disability (about 50%). Long-term physiotherapy and specific rehabilitation programs appear essential to improve recovery.

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“…Two patients showed poor recovery, with permanent disabling weakness. The others could walk with persistent minor problems [4][5][6][7][8][9][10][11][12][13][14][15][16]. Our patient recovered with residual weakness in the lower limbs.…”

Section: Discussionmentioning

confidence: 99%

Fulminant Guillain Barré Syndrome (GBS) Refractory to Intravenous Immunoglobulin (IVIG) Treated with Therapeutic Plasma Exchange

Gupta¹

2017

HTIJ

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A 53 year old man was brought to the Neurology Intensive Care Unit, on a ventilator with a Glasgow Coma Scale (GCS) of 5T (E-Eye opening, V-Verbal response, M-Motor response, T-Intubated-E 4 V T M 1 ) which later worsened to 2T (E 1 V T M 1 ). On detailed evaluation, a diagnosis of Fulminant Guillain Barré Syndrome (Hughes score 5) was made and Intravenous Immunoglobulin (IVIG) was instituted. No response was noted even seven days after IVIG administration. Plasma exchange was initiated. A total of 14 exchanges were performed over a period of 13 weeks wherein 21.8 liter of plasma was exchanged. Patient started showing signs of improvement by the 6th week of illness. By the 19 th week of illness, he could be completely weaned off the ventilator. At the time of discharge (27 th week of illness), the power in his upper limbs was 4 and 2 in his lower limbs. Hughes score was 4 and GCS was 15 (E 4 V 5 M 6 ). This case illustrates the significant role of plasma exchange in the management of Fulminant Guillain Barré Syndrome.

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Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination

Cited by 18 publications

References 10 publications

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

Long-term impact after fulminant Guillain-Barré syndrome, case report and literature review

Fulminant Guillain Barré Syndrome (GBS) Refractory to Intravenous Immunoglobulin (IVIG) Treated with Therapeutic Plasma Exchange

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Fulminant Guillain-Barré syndrome with quadriplegia and total paresis of motor cranial nerves as a result of segmental demyelination

Cited by 18 publications

References 10 publications

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

Fulminant Guillain–Barré Syndrome with universal inexcitability of peripheral nerves: A clinicopathological study

Long-term impact after fulminant Guillain-Barr&eacute; syndrome, case report and literature review

Fulminant Guillain Barré Syndrome (GBS) Refractory to Intravenous Immunoglobulin (IVIG) Treated with Therapeutic Plasma Exchange

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Long-term impact after fulminant Guillain-Barré syndrome, case report and literature review