Case Report: Subacute onset of the motor-sensory axonal neuropathy variant of Gullain-Barré syndrome after epidural anesthesia

Guillain-Barré syndrome (GBS) is an acute ascending peripheral neuropathy, caused by autoimmune damage of the peripheral nerves. GBS can be divided into three subtypes: acute inflammatory demyelinating neuropathy, acute motor axonal neuropathy, and the more rare type, acute motor and sensory axonal neuropathy (AMSAN). Reports of AMSAN with onset after epidural anesthesia and spinal surgery are extremely rare, and the linkage between development of GBS and neuroaxial anesthesia remains conclusively unconfirmed. We present a case in which the patient developed subacute motor and predominantly sensory neuropathy following epidural blockade. The case emphasizes the need of including AMSAN in differential diagnostic considerations to changes in motor and sensory function following epidural anesthesia, allowing accelerated rehabilitation and relevant alleviating therapy.

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Serum CXCL13 reflects local B-cell mediated inflammatory demyelinating peripheral neuropathy

Scientific Reports ◽

10.1038/s41598-019-52643-2 ◽

2019 ◽

Vol 9 (1) ◽

Cited By ~ 1

Author(s):

Young Hee Kim ◽

So Young Jang ◽

Yoon Kyung Shin ◽

Young Rae Jo ◽

Byeol-A. Yoon ◽

...

Keyword(s):

B Cell ◽

Axonal Neuropathy ◽

Serum Levels ◽

Demyelinating Neuropathy ◽

Acute Motor Axonal Neuropathy ◽

Charcot Marie Tooth Disease ◽

Chemokine Ligand ◽

Primary Demyelination ◽

Demyelinating Peripheral Neuropathy ◽

Inflammatory Milieu

Abstract Immune damages on the peripheral myelin sheath under pro-inflammatory milieu result in primary demyelination in inflammatory demyelinating neuropathy. Inflammatory cytokines implicating in the pathogenesis of inflammatory demyelinating neuropathy have been used for the development of potential biomarkers for the diagnosis of the diseases. In this study, we have found that macrophages, which induce demyelination, expressed a B-cell-recruiting factor CXC chemokine ligand 13 (CXCL13) in mouse and human inflammatory demyelinating nerves. The serum levels of CXCL13 were also higher in inflammatory demyelinating neuropathic patients but not in acute motor axonal neuropathy or a hereditary demyelinating neuropathy, Charcot-Marie-Tooth disease type 1a. In addition, CXCL13-expressing macrophages were not observed in the sciatic nerves after axonal injury, which causes the activation of innate immunity and Wallerian demyelination. Our findings indicate that the detection of serum CXCL13 will be useful to specifically recognize inflammatory demyelinating neuropathies in human.

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Finger drop sign—Characteristic pattern of distal weakness in Guillain-Barré Syndrome: A case report and review of the literature

SAGE Open Medical Case Reports ◽

10.1177/2050313x18773649 ◽

2018 ◽

Vol 6 ◽

pp. 2050313X1877364

Author(s):

Yong Chuan Chee ◽

Beng Hooi Ong

Keyword(s):

Case Report ◽

Axonal Neuropathy ◽

Good Prognosis ◽

Characteristic Pattern ◽

Guillain Barre Syndrome ◽

Demyelinating Neuropathy ◽

Limb Weakness ◽

Acute Motor Axonal Neuropathy ◽

Guillain Barré Syndrome ◽

Guillain Barré

Guillain-Barré Syndrome is an acquired acute autoimmune polyradiculoneuropathy that commonly presents with limb weakness and occasional cranial nerve, respiratory and autonomic involvement. Although the classic description of Guillain-Barré Syndrome is that of a demyelinating neuropathy with ascending weakness, predominant bilateral finger drop as presenting feature has rarely been reported. A characteristic pattern of weakness involving the extensor components of the fingers known as “finger drop sign” has been first described to be specific in acute motor axonal neuropathy form of Guillain-Barré Syndrome in the literature. We report a case of acute motor-sensory axonal neuropathy, which showed characteristic pattern of predominant finger extensor weakness, and provide a summary of all reported cases to date. While previous reports suggested that this is a sign that carries good prognosis, our case report suggested otherwise as the patient succumbed to respiratory and autonomic complications. Further studies are needed to evaluate the clinical significance of this peculiar sign.

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Development of Acute Inflammatory Demyelinating Polyneuropathy 11 Days after Spinal Surgery: A Case Report and Literature Review

Case Reports in Medicine ◽

10.1155/2021/6283076 ◽

2021 ◽

Vol 2021 ◽

pp. 1-9

Author(s):

Eiichi Kakehi ◽

Tadataka Kawakami ◽

Yukiko Ishikawa ◽

Takashi Matsuoka ◽

Naoki Nakagawa ◽

...

Keyword(s):

Cerebrospinal Fluid ◽

Literature Review ◽

Nerve Conduction Study ◽

Nerve Conduction ◽

Spinal Surgery ◽

Axonal Neuropathy ◽

Immunoglobulin Therapy ◽

Autonomic Nerve ◽

Acute Motor Axonal Neuropathy ◽

Study Results

Guillain–Barré syndrome (GBS) usually has a good prognosis; however, patients may develop sequelae without prompt treatment. We herein describe an 81-year-old woman who developed acute-onset excruciating thigh pain and weakness in her lower extremities after spinal surgery. We diagnosed acute inflammatory demyelinating polyradiculoneuropathy by a nerve conduction study, which showed findings of demyelination without cerebrospinal fluid analysis because of a spinal prosthesis. Although anti-GM1 and anti-GalNAc-GD1a antibodies were positive, the patient was clinically diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (a subtype of GBS), not acute motor axonal neuropathy. She recovered well with immunoglobulin therapy. A literature review of 18 cases revealed that unexplained weakness, areflexia, and numbness of the extremities after spinal surgery, a shorter time from spinal surgery to symptom onset to general GBS, abnormal nerve conduction study results, normal spinal imaging findings, and the development of atypical symptoms such as cranial and autonomic nerve syndrome and respiratory failure are useful for diagnosing GBS when cerebrospinal fluid examination cannot be performed after spinal surgery.

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HINT1 neuropathy in Norway: clinical, genetic and functional profiling

Orphanet Journal of Rare Diseases ◽

10.1186/s13023-021-01746-z ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Silvia Amor-Barris ◽

Helle Høyer ◽

Lin V. Brauteset ◽

Els De Vriendt ◽

Linda Strand ◽

...

Keyword(s):

Axonal Neuropathy ◽

Functional Characterization ◽

Treatment Strategies ◽

Central Nervous System Involvement ◽

Sensory Neuropathy ◽

Muscle Stiffness ◽

Compound Heterozygous ◽

Clinical Genetic ◽

Cellular Models ◽

New Variant

Abstract Background Autosomal recessive axonal neuropathy with neuromyotonia has been linked to loss of functional HINT1. The disease is particularly prevalent in Central and South-East Europe, Turkey and Russia due to the high carrier frequency of the c.110G > C (p.Arg37Pro) founder variant. Results In a cohort of 748 Norwegian patients with suspected peripheral neuropathy, we identified two seemingly unrelated individuals, compound heterozygous for a new variant (c.284G > A, p.Arg95Gln) and the most common pathogenic founder variant (c.110G > C, p.Arg37Pro) in the HINT1 gene. Probands presented with motor greater than sensory neuropathy of various onset, accompanied by muscle stiffness and cramps in the limbs. Furthermore, they displayed non-classical symptoms, including pain in the extremities and signs of central nervous system involvement. Haplotype analysis in both patients revealed a common chromosomal background for p.Arg95Gln; moreover, the variant was identified in Swedish carriers. Functional characterization in HINT1-knockout and patient-derived cellular models, and in HNT1-knockout yeast, suggested that the new variant is deleterious for the function of HINT1 and provided mechanistic insights allowing patient stratification for future treatment strategies. Conclusion Our findings broaden the genetic epidemiology of HINT1-neuropathy and have implications for molecular diagnostics of inherited peripheral neuropathies in Scandinavia.

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RFC1 AAGGG repeat expansion masquerading as Chronic Idiopathic Axonal Polyneuropathy

Journal of Neurology ◽

10.1007/s00415-021-10552-3 ◽

2021 ◽

Author(s):

Matteo Tagliapietra ◽

Davide Cardellini ◽

Moreno Ferrarini ◽

Silvia Testi ◽

Sergio Ferrari ◽

...

Keyword(s):

Care Center ◽

Axonal Neuropathy ◽

Tertiary Care ◽

Sensory Neuropathy ◽

Nerve Fibers ◽

Repeat Expansion ◽

Sural Nerve Biopsy ◽

Prevalence Odds Ratio ◽

Axonal Polyneuropathy ◽

Factor C

Abstract Background A biallelic intronic AAGGG repeat expansion in the Replication Factor C subunit 1 (RFC1) gene has been recently associated with Cerebellar Ataxia, Neuropathy, Vestibular Areflexia Syndrome, a disorder often presenting as a slowly evolving sensory neuropathy at the onset. “Chronic Idiopathic Axonal Polyneuropathy” (CIAP) is a common indolent axonal neuropathy of adulthood which remains without an identifiable cause despite thorough investigations. Methods We screened 234 probands diagnosed with CIAP for a pathogenic biallelic RFC1 AAGGG repeat expansion. Patients were selected from 594 consecutive patients with neuropathy referred to our tertiary-care center for a sural nerve biopsy over 10 years. Results The RFC1 AAGGG repeat expansion was common in patients with pure sensory neuropathy (21/40, 53%) and less frequent in cases with predominantly sensory (10/56, 18%, P < 0.001) or sensorimotor (3/138, 2%, P < 0.001) neuropathy. The mutation was associated with sensory ataxia (τb = 0.254, P < 0.001), autonomic disturbances (35% vs 8%, Prevalence Odds Ratio—POR 6.73 CI 95% 2.79–16.2, P < 0.001), retained deep tendon reflexes (score 18.0/24 vs 11.5/24, R = 0.275, P < 0.001). On pathology, we observed absent/scant regenerative changes (τb = − 0.362, P < 0.001), concomitant involvement of large (100% and 99%, n.s.), small myelinated (97% vs 81%, POR 7.74 CI 95% 1.03–58.4, P = 0.02) and unmyelinated nerve fibers (85% vs 41%, POR 8.52 CI 95% 3.17–22.9, P < 0.001). Cerebellar or vestibular involvement was similarly rare in the two groups. Conclusions This study highlights the frequent occurrence of the RFC1 AAGGG repeat expansion in patients diagnosed with CIAP and characterizes the clinical and pathological features of the related neuro(no)pathy.

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