Variants of Guillain-Barré syndrome: low incidence but high impact

Lehmann, Helmar C.; Hartung, Hans‐Peter

doi:10.1007/s00415-009-5253-9

Cited by 6 publications

(6 citation statements)

References 20 publications

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“…Nevertheless, the case described here did not meet the diagnostic criteria for GBS, as typical features, such as sensory-motor impairment of the limbs with hypo-areflexia and albuminocytologic dissociation, were missing. However, in recent years, the notion of GBS as a disease encompassing different neurological presentations has become increasingly accepted, since anti-GM1 antibodies has been related with a wider clinical spectrum than previously thought [4,5]. Interestingly, a GBS variant with prominent FP has been recently described [6].…”

Section: Dear Sirmentioning

confidence: 95%

Idiopathic bilateral facial palsy: is a causative role of anti-GM1 ganglioside and herpes simplex type 1 possible?

et al. 2011

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Bilateral facial nerve palsy (FP) is a rare clinical entity often idiopathic or secondary to several diseases [1]. A role of Herpes simplex virus type 1 (HSV-1) in idiopathic FP is under debate [2]. We report here on a case of bilateral FP associated with anti-GM1 ganglioside antibodies and cerebrospinal HSV-1 DNA, who recovered after therapy with intra-venous immunoglobulins (IVIg).A 68-year-old woman presented a sudden complete right sided FP that, despite treatment (methylprednisolone 40 mg/ day i.m.), became bilateral in 1 week. The clinical history was devoid of relevant pathological events. At day 10 from the onset of symptoms, a neurological examination showed bilateral complete lower motor neuron FP (House-Brackmann grading system V on right and IV on left side). Meanwhile, there was no evidence of motor or sensory impairment of other cranial nerves and limbs, and deep tendon reflexes were normal. Neurophysiology, which included nerve conduction studies, motor, somato-sensory and auditory evoked potentials, was normal. Bilateral stapedial muscle and blink reflexes were absent in keeping with the diagnosis of bilateral facial palsy. MRI showed signs compatible with inflammation in both the facial nerves ( Fig. 1). Routine blood investigations, including serological tests, paraneoplastic markers, CSF analysis and neuroimaging studies ruled out several known aetiologies. CSF nested PCR was positive for HSV-1 DNA and negative for other neurotropic viruses. To confirm the CSF positivity and the presence of an active infection, an aliquot of residual frozen CSF sample was thawed and used as inoculum on HSV-1 susceptible HEP cell lines. Though the freeze-thawing procedure may impair the viability of viral particles in biological samples, a cytopathic effect was observed after a 10-day culture (Fig. 2). However, no viral DNA was detected in cell lysates by nested PCR analysis from the same culture. Serum analysis revealed a past infection by HSV-1 since anti-HSV-1 IgG titre was 1:9,900 (v.n. \ 1:230) and the research for anti-HSV-1 IgM was negative.As Guillain-Barre' (GBS) and Miller Fisher syndrome are likely causes, a serum anti-GM1 and anti-GQ1b antibody (Ab) assay was performed by ELISA. Increased level of anti-GM1 IgG Ab was found (0.083 OD at a dilution 1:640) since absorbance was more than three standard deviations above the mean levels found in serum of normal subjects (normal values \0.050 OD). Anti-GQ1b Ab was negative. Anti-GM2 Ab assay was not performed. No Ab for Borrelia burgdoferi and Campylobacter jejuni was detected in the blood.IVIg therapy (400 mg/kg/day for 5 days) was immediately started, with a prompt improvement. Considering the amelioration of symptoms, the lack of evidence of active infection, and the current orientation for the treatment of facial palsy [3], we avoided the use of antiviral. After 6 months the patient showed a marked clinical recovery (House and Brackmann grade II, residual deficit more pronounced on the right side); serum anti-GM1 Ab was not detectable.

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Section: Dear Sirmentioning

confidence: 95%

Idiopathic bilateral facial palsy: is a causative role of anti-GM1 ganglioside and herpes simplex type 1 possible?

et al. 2011

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“…As pointed out by an editorial by us accompanying the case series of Susuki and colleagues [6], it is important to recognize the clinical features that support the concept that facial diplegia and limb paresthesia form indeed part of the GBS spectrum. In the careful analysis done by Susuki and colleagues, all patients had albuminocytologic dissociation in the CSF and in the majority of patients, demyelinating abnormalities were detectable in nerve conduction studies [9].…”

Section: Dear Sirsmentioning

confidence: 96%

Guillain–Barré syndrome variant with prominent facial diplegia, limb paresthesia, and brisk reflexes

et al. 2011

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“…Our patient showed all these features but her reflexes were preserved during the entire course of the illness. Patients with facial diplegia and hyperreflexia have been reported in the literature and regarded as having a GBS variant (4,14,15).…”

Section: Discussionmentioning

confidence: 99%

“…During the course of the illness, 24-60% of patients develop facial nerve paresis, almost all of which are bilateral (2). The clinical spectrum of GBS has expanded because many variants have been described in recent years (3,4,5,6,7,8). Facial diplegia with paresthesias (FDP) is characterized by prominent facial diplegia and distal limb paresthesia with no or only mild motor deficit, and is a localized form of GBS that can be distinguished by involvement of certain muscle groups or nerves (7,8).…”

Section: Introductionmentioning

confidence: 99%