Experimental axonopathy induced by immunization with Campylobacter jejuni lipopolysaccharide from a patient with Guillain-Barré syndrome (original) (raw)

Painful sensory neuropathy and dysimmunity: is there a relation?

Journal of the Peripheral Nervous System, 2004

Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.

Chronic ataxic neuropathy initially diagnosed as ataxic variant of guillain barre syndrome

Journal of the Peripheral Nervous System, 2004

Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.

Demyelinating motor guillain-barre syndrome following rubella

Journal of The Peripheral Nervous System, 2004

Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.

Hyperreflexia in axonal Guillain–Barré syndrome subsequent to Campylobacter jejuni enteritis

Journal of the Neurological Sciences, 2002

We describe a patient with the acute motor axonal neuropathy (AMAN) form of Guillain -Barré syndrome (GBS), who showed generalized hyperreflexia. A 24-year-old man developed acute paralysis following Campylobacter jejuni enteritis. He showed exaggerated tendon reflexes with abnormal reflex spread to other segments, and was initially diagnosed as having post-infectious myelitis. Nerve conduction studies showed motor axonal degeneration (the AMAN pattern), and increased soleus H-reflex amplitudes. His serum was positive for IgG antibodies to gangliosides GM1b and GalNAc-GD1a. He was treated with plasmapheresis, resulting in rapid recovery. Hyperreflexia was still present 12 months after onset when muscle strength was completely normal. This case provides further evidence that patients with AMAN can develop increased motor neuron excitability, and possible mechanisms for the hyperreflexia are discussed. D

Acute painful autoimmune neuropathy: A variant of Guillain-Barré syndrome

Muscle & nerve, 2017

Introduction We present a painful small fibre neuropathy variant of Guillain-Barré syndrome. Methods characterized by antecedent infectious symptoms, hyporeflexia and albuminocytologic dissociation. Results Two patients received intravenous immunoglobulin, and subsequently improved. IgG antibodies in their acute phase sera strongly bound to murine small nerve fibres, and the binding disappeared during the convalescent phase. Serum transfer to a murine nociceptive model induced transient alteration in thermal pain responses. Discussion Our case series suggest that an acute transient immune response can be directed against small nerve fibres, and that patients so affected can exhibit features of Guillain-Barré syndrome. This article is protected by copyright. All rights reserved.

Axonal Guillain-Barré syndrome: concepts and controversies

The Lancet Neurology, 2013

Acute motor axonal neuropathy (AMAN) is a pure motor axonal subtype of Guillain-Barré syndrome (GBS) that was identifi ed in the late 1990s. In Asia and Central and South America, it is the major subtype of GBS, seen in 30-65% of patients. AMAN progresses more rapidly and has an earlier peak than demyelinating GBS; tendon refl exes are relatively preserved or even exaggerated, and autonomic dysfunction is rare. One of the main causes is molecular mimicry of human gangliosides by Campylobacter jejuni lipo-oligosaccharides. In addition to axonal degeneration, electrophysiology shows rapidly reversible nerve conduction blockade or slowing, presumably due to pathological changes at the nodes or paranodes. Autoantibodies that bind to GM1 or GD1a gangliosides at the nodes of Ranvier activate complement and disrupt sodium-channel clusters and axoglial junctions, which leads to nerve conduction failure and muscle weakness. Improved understanding of the disease mechanism and pathophysiology might lead to new treatment options and improve the outlook for patients with AMAN.

Increase of pro-inflammatory cytokines in chronic sensory ataxic neuropathies

Journal of The Peripheral Nervous System, 2004

Case Report: A 20-year-old man presented for evaluation following an episode of painless injury in which he almost amputated his thumb. Upon further questioning, the patient reported he rarely experienced physical (including visceral) pain and his mother provided support to his history recalling incidents occurring as a child. He does not experience itching either. His neurologic and general medical history was otherwise negative. His neurologic and general examination were unremarkable except for the inability to perceive painful (pinprick, pressure on Achilles' tendons), hot and cold stimuli over his entire body, with some relative sparing over his chest, abdomen and back. Nerve conduction studies and needle examinations were normal. Quantitative sensory testing revealed normal threshold to vibration, relatively elevated threshold to cold and insensitivity to heat and pain. Autonomic reflex screen and thermoregulatory sweat test were normal. Sural nerve biopsy showed normal myelinated and unmyelinated fibers. MRI of head and cervical spine, and extensive laboratory testing were unremarkable. Conclusion: This patient differs from any other case described in the literature of pain insensitivity or pain indifference. Suspecting a central pathology, we prescribed naltrexone and retested his heat-pain sensation after treatment. He perceived the probe as hotter. Unfortunately, he suffered unacceptable dysthymia from the medication and stopped it.

Guillain-Barre syndrome subtypes related to Campylobacter infection

Journal of Neurology, Neurosurgery & Psychiatry, 2011

Background: In Guillain-Barré syndrome (GBS) the diversity in electrophysiological subtypes is unexplained, but may be determined by geographical factors and preceding infections. Acute motor axonal neuropathy (AMAN) is a frequent GBS variant in Japan and one study proposed that in Japan Campylobacter jejuni infections exclusively elicit AMAN. In the Netherlands C. jejuni is the predominant type of preceding infection, yet AMAN is rare. This may indicate that not all Dutch GBS patients with C. jejuni infections have AMAN. Objective: To determine if GBS patients with a preceding C. jejuni infection in the Netherlands exclusively have AMAN. Methods: Retrospective analysis of preceding infections in relation to serial electrophysiology and clinical data from 123 GBS patients. C. jejuni-related cases were defined as having preceding diarrhea and positive C. jejuni serology. Electrophysiological characteristics in C. jejuni-related cases were compared with those in viral-related GBS patients. In addition, eight GBS patients from another cohort with positive stool cultures for C. jejuni were analysed. Results: Seventeen (14%) of 123 patients had C. jejuni-related GBS. C. jejuni patients had lower motor and higher sensory action potentials compared to viral-related cases. Nine (53%) C. jejuni patients had either AMAN or inexcitable nerves. However, three (18%) patients fulfilled the criteria for acute inflammatory demyelinating polyneuropathy (AIDP). Also, two (25%) of eight additional patients with a C. jejuni-positive stool sample had AIDP. Conclusion: In the Netherlands C. jejuni infections are strongly, but not exclusively associated with axonal GBS. Some patients with these infections fulfil current criteria for demyelination.