Just Accepted by International Journal of Neuroscience
Guillain-Barre´ syndrome following chickenpox: a case series P. Tatarelli, M. Garnero, V. Del Bono, M. Camera, A. Schenone, M. Grandis, L. Benedetti, C. Viscoli doi:10.3109/00207454.2015.1033621
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Abstract Guillain-Barre´ syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy, usually triggered by an infectious episode, mostly of viral origin. Varicella zoster virus (VZV) is a rare cause of GBS, mainly in case of latent infection reactivation. We report on 3 adult patients who developed GBS following chickenpox, after a short period of latency. They were promptly treated with intravenous immunoglobulin, and the first one with plasma exchange additionally. All the patients experienced almost complete clinical recovery. Our experience suggests that primary VZV infection constitutes a GBS triggering event.
© 2015 Informa Healthcare USA, Inc. This provisional PDF corresponds to the article as it appeared upon acceptance. Fully formatted PDF and full text (HTML) versions will be made available soon. DISCLAIMER: The ideas and opinions expressed in the journal’s Just Accepted articles do not necessarily reflect those of Informa Healthcare (the Publisher), the Editors or the journal. The Publisher does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of the material contained in these articles. The reader is advised to check the appropriate medical literature and the product information currently provided by the manufacturer of each drug to be administered to verify the dosages, the method and duration of administration, and contraindications. It is the responsibility of the treating physician or other health care professional, relying on his or her independent experience and knowledge of the patient, to determine drug dosages and the best treatment for the patient. Just Accepted have undergone full scientific review but none of the additional editorial preparation, such as copyediting, typesetting, and proofreading, as have articles published in the traditional manner. There may, therefore, be errors in Just Accepted articles that will be corrected in the final print and final online version of the article. Any use of the Just Accepted articles is subject to the express understanding that the papers have not yet gone through the full quality control process prior to publication.
Publisher: Informa Healthcare Journal: International Journal of Neuroscience DOI: http://dx.doi.org/10.3109/00207454.2015.1033621
scT rE ip tD
Guillain-Barré syndrome following chickenpox: a case series P. Tatarelli*1, M. Garnero*2, V. Del Bono1, M. Camera1, A. Schenone2, M. Grandis2, L. Benedetti3, C. Viscoli1 1
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DiNOGMI), University of Genoa and IRCCS San Martino-IST, Genoa, Italy Neurology Department, S. Andrea Hospital, La Spezia, Italy
*
CM Can EuP
3
Both authors equally contributed to the paper
*Corresponding Author: Email: [email protected]
Abstract
cSe pTt
eAd
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy, usually triggered by an infectious episode, mostly of viral origin. Varicella zoster virus (VZV) is a rare cause of GBS, mainly in case of latent infection reactivation. We report on 3 adult patients who developed GBS following chickenpox, after a short period of latency. They were promptly treated with intravenous immunoglobulin, and the first one with plasma exchange additionally. All the patients experienced almost complete clinical recovery. Our experience suggests that primary VZV infection constitutes a GBS triggering event. Keywords
Guillain-Barré syndrome
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
2
Division of Infectious Diseases, University of Genoa and IRCCS San Martino-IST, Genoa, Italy
VZV
Chickenpox
1
Background
scT rE ip tD
Case Reports
eAd
CM Can EuP
Case 1. A 47 year-old man developed progressive weakness and paresthesias involving the extremities 7 days after the onset of chickenpox rash. At the admission, acyclovir-based therapy was started (10 mg/kg/day intravenously). Neurological examination showed severe muscle weakness in all four limbs and tactile and vibratory sensory impairment with glove and sock distribution. Deep tendon reflexes were absent. Cerebrospinal fluid (CSF) examination showed increased protein level (2.513 mg/L) with normal cell count (0,8 cells/mm3) and glucose value. CSF polymerase chain reaction (PCR) for VZV-DNA was negative. Serologic tests were indicative of primary VZV infection (positive research for IgM antibodies, negative for IgG). Twenty-four hours after admission, treatment with intravenous immunoglobulin (IVIG; 0.4 g/kg/day for 5 days) was started. Electroneurography evidenced a demyelinating neuropathy. On the third day, the patient was transferred to the Intensive Care Unit due to respiratory failure. He underwent 5 cycles of plasma exchange were performed, with clinical improvement. After adequate rehabilitation therapy, an almost complete recovery of neurological function was observed; however, painful acral paresthesias persisted.
cSe pTt
Case 2. A 42 year-old woman was hospitalized for chickenpox while being treated with acyclovir (800 mg 5 times daily, orally). Six days after the onset of rash, she developed progressive fatigue, dysphonia and dysphagia. She had mild upper and lower limbs weakness and tendon hyporeflexia. In addition, she developed slurred speech, impaired swallow, facial amimia and Bell’s phenomenon, suggesting an atypical form of bilateral facial nerve palsy. CSF examination showed high protein concentration (655 mg/L) without pleocytosis (4 cell/mm3); CSF PCR for VZV-DNA was negative. VZV serologic tests confirmed a primary infection (IgM positivity, IgG negativity). IVIG treatment to the usual dosage was started 48 hours after the admission. Fifteen days after IVIG administration, symptoms gradually improved and the patient was transferred to a rehabilitation ward, from which she was finally discharged without neurological sequelae.
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Guillain-Barré syndrome (GBS) is an acute, monophasic, immune-mediated polyradiculoneuropathy. It is characterized by ascending muscle weakness, hyporeflexia, cranial nerve impairment and sensory changes. Usually, GBS follows an infectious illness. It is probably caused by production of antibodies against microorganisms, that cross-react with specific gangliosides and glycolipids distributed throughout the myelin. Varicella zoster virus (VZV) is a neurotropic herpes virus responsible of chickenpox and shingles, the latter expression of latent disease reactivation. VZV rarely can trigger GBS, usually during shingles. We describe 3 Italian patients with GBS following primary VZV infection admitted to our Infectious Diseases Unit during the last 12 months.
Case 3. A 35 year-old man developed urinary retention, weakness and numbness involving all the extremities 7 days after the onset of the chickenpox rash. Neurological examination showed tactile sensory impairment and muscle weakness worse in the lower limbs (3/5 proximally and 4/5 distally, according to MRC Scale for Muscle Strength). The MRI scan did not show spinal cord abnormalities. CSF examination showed high protein levels (1.037 mg/L) with normal cell count (0,8 cells/mm3) and glucose value; PCR for VZV-DNA was negative. Serum VZV IgM antibodies 2
eAd
CM Can EuP
scT rE ip tD
Discussion. Our report involves 3 Caucasians adults (mean age 41,3 years) with primary VZV infection, clinically and serologically documented, who developed symptoms and signs of acute polyradiculoneuropathy. GBS was diagnosed due to suggestive clinical presentation and typical CSF albumin-cytological dissociation. In one case, electroneurography documented demyelinating neuropathy. This case-series is characterized by short latency period between rash onset and the development of neurological symptoms (6,6 days on average). The course was complicated by acute respiratory failure in the first patient; nevertheless, it was favorable in all cases. Usually, the onset of GBS has a latency period of 2 weeks to 1 month after preceding infection, and shorten latency periods are associated with more severe illness. In our experience, the early IVIG treatment, and the additional plasmapheresis in the first case, have conceivably conditioned the favorable clinical outcome. VZV is a well-known neurotropic virus, responsible of several neurological syndromes (Table 1), usually characterized by detectable VZV-DNA in the CSF [1]. Since 1956, fewer than 50 cases of VZV-related GBS were reported, mostly in course of shingles. Only a few case reports have described the development of GBS following chickenpox, with demographic, clinical and laboratory features similar to those of our experience [2]. The causal mechanism of VZV-related GBS might be “molecular mimics”, as described for other well-known GBS triggering infections. The absence of CSF VZV-DNA and the ineffectiveness of acyclovir-based therapy in this case-series are consistent with GBS immune-mediated pathogenesis. Our experience suggests that primary VZV infection constitutes a GBS triggering event.
cSe pTt
The authors declare that they have no conflict of interest.
References
1. Rottenstreich A, Oz ZK, Oren I. Association between viral load of varicella zoster virus in cerebrospinal fluid and the clinical course of central nervous system infection. Diagn Microbiol Infect Dis. 2014 Jun;79(2):174-7.
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
were present while IgG were absent. Two days after admission, standard IVIG treatment was administered. After 6 days, progressive neurological improvement occurred, with resumption of spontaneous voiding and resolution of paresthesias and weakness. He was then discharged with normal neurological examination.
2. Cresswell F, Eadie J, Longley N, Macallan D. Severe Guillain-Barré syndrome following primary infection with varicella zoster virus in an adult. Int J Infect Dis. 2010 Feb;14(2):161-3.
3
Neurological complications associated with chickenpox and shingles. Chickenpox
Shingles
Encephalitis
Encephalitis
scT rE ip tD
Table 1.
Meningitis
Meningitis
Transverse myelitis
Transverse myelitis
Zoster ophthalmicus
Granulomatous cerebral angiitis
CM Can EuP
Ramsay-Hunt syndrome
cSe pTt
eAd
Post-herpetic nuralgia
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Reye’s syndrome
4
Guillain-Barre´ syndrome following chickenpox: a case series P. Tatarelli, M. Garnero, V. Del Bono, M. Camera, A. Schenone, M. Grandis, L. Benedetti, C. Viscoli doi:10.3109/00207454.2015.1033621
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Abstract Guillain-Barre´ syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy, usually triggered by an infectious episode, mostly of viral origin. Varicella zoster virus (VZV) is a rare cause of GBS, mainly in case of latent infection reactivation. We report on 3 adult patients who developed GBS following chickenpox, after a short period of latency. They were promptly treated with intravenous immunoglobulin, and the first one with plasma exchange additionally. All the patients experienced almost complete clinical recovery. Our experience suggests that primary VZV infection constitutes a GBS triggering event.
© 2015 Informa Healthcare USA, Inc. This provisional PDF corresponds to the article as it appeared upon acceptance. Fully formatted PDF and full text (HTML) versions will be made available soon. DISCLAIMER: The ideas and opinions expressed in the journal’s Just Accepted articles do not necessarily reflect those of Informa Healthcare (the Publisher), the Editors or the journal. The Publisher does not assume any responsibility for any injury and/or damage to persons or property arising from or related to any use of the material contained in these articles. The reader is advised to check the appropriate medical literature and the product information currently provided by the manufacturer of each drug to be administered to verify the dosages, the method and duration of administration, and contraindications. It is the responsibility of the treating physician or other health care professional, relying on his or her independent experience and knowledge of the patient, to determine drug dosages and the best treatment for the patient. Just Accepted have undergone full scientific review but none of the additional editorial preparation, such as copyediting, typesetting, and proofreading, as have articles published in the traditional manner. There may, therefore, be errors in Just Accepted articles that will be corrected in the final print and final online version of the article. Any use of the Just Accepted articles is subject to the express understanding that the papers have not yet gone through the full quality control process prior to publication.
Publisher: Informa Healthcare Journal: International Journal of Neuroscience DOI: http://dx.doi.org/10.3109/00207454.2015.1033621
scT rE ip tD
Guillain-Barré syndrome following chickenpox: a case series P. Tatarelli*1, M. Garnero*2, V. Del Bono1, M. Camera1, A. Schenone2, M. Grandis2, L. Benedetti3, C. Viscoli1 1
Department of Neuroscience, Rehabilitation, Ophthalmology, Genetics, Maternal and Child Health (DiNOGMI), University of Genoa and IRCCS San Martino-IST, Genoa, Italy Neurology Department, S. Andrea Hospital, La Spezia, Italy
*
CM Can EuP
3
Both authors equally contributed to the paper
*Corresponding Author: Email: [email protected]
Abstract
cSe pTt
eAd
Guillain-Barré syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy, usually triggered by an infectious episode, mostly of viral origin. Varicella zoster virus (VZV) is a rare cause of GBS, mainly in case of latent infection reactivation. We report on 3 adult patients who developed GBS following chickenpox, after a short period of latency. They were promptly treated with intravenous immunoglobulin, and the first one with plasma exchange additionally. All the patients experienced almost complete clinical recovery. Our experience suggests that primary VZV infection constitutes a GBS triggering event. Keywords
Guillain-Barré syndrome
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
2
Division of Infectious Diseases, University of Genoa and IRCCS San Martino-IST, Genoa, Italy
VZV
Chickenpox
1
Background
scT rE ip tD
Case Reports
eAd
CM Can EuP
Case 1. A 47 year-old man developed progressive weakness and paresthesias involving the extremities 7 days after the onset of chickenpox rash. At the admission, acyclovir-based therapy was started (10 mg/kg/day intravenously). Neurological examination showed severe muscle weakness in all four limbs and tactile and vibratory sensory impairment with glove and sock distribution. Deep tendon reflexes were absent. Cerebrospinal fluid (CSF) examination showed increased protein level (2.513 mg/L) with normal cell count (0,8 cells/mm3) and glucose value. CSF polymerase chain reaction (PCR) for VZV-DNA was negative. Serologic tests were indicative of primary VZV infection (positive research for IgM antibodies, negative for IgG). Twenty-four hours after admission, treatment with intravenous immunoglobulin (IVIG; 0.4 g/kg/day for 5 days) was started. Electroneurography evidenced a demyelinating neuropathy. On the third day, the patient was transferred to the Intensive Care Unit due to respiratory failure. He underwent 5 cycles of plasma exchange were performed, with clinical improvement. After adequate rehabilitation therapy, an almost complete recovery of neurological function was observed; however, painful acral paresthesias persisted.
cSe pTt
Case 2. A 42 year-old woman was hospitalized for chickenpox while being treated with acyclovir (800 mg 5 times daily, orally). Six days after the onset of rash, she developed progressive fatigue, dysphonia and dysphagia. She had mild upper and lower limbs weakness and tendon hyporeflexia. In addition, she developed slurred speech, impaired swallow, facial amimia and Bell’s phenomenon, suggesting an atypical form of bilateral facial nerve palsy. CSF examination showed high protein concentration (655 mg/L) without pleocytosis (4 cell/mm3); CSF PCR for VZV-DNA was negative. VZV serologic tests confirmed a primary infection (IgM positivity, IgG negativity). IVIG treatment to the usual dosage was started 48 hours after the admission. Fifteen days after IVIG administration, symptoms gradually improved and the patient was transferred to a rehabilitation ward, from which she was finally discharged without neurological sequelae.
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Guillain-Barré syndrome (GBS) is an acute, monophasic, immune-mediated polyradiculoneuropathy. It is characterized by ascending muscle weakness, hyporeflexia, cranial nerve impairment and sensory changes. Usually, GBS follows an infectious illness. It is probably caused by production of antibodies against microorganisms, that cross-react with specific gangliosides and glycolipids distributed throughout the myelin. Varicella zoster virus (VZV) is a neurotropic herpes virus responsible of chickenpox and shingles, the latter expression of latent disease reactivation. VZV rarely can trigger GBS, usually during shingles. We describe 3 Italian patients with GBS following primary VZV infection admitted to our Infectious Diseases Unit during the last 12 months.
Case 3. A 35 year-old man developed urinary retention, weakness and numbness involving all the extremities 7 days after the onset of the chickenpox rash. Neurological examination showed tactile sensory impairment and muscle weakness worse in the lower limbs (3/5 proximally and 4/5 distally, according to MRC Scale for Muscle Strength). The MRI scan did not show spinal cord abnormalities. CSF examination showed high protein levels (1.037 mg/L) with normal cell count (0,8 cells/mm3) and glucose value; PCR for VZV-DNA was negative. Serum VZV IgM antibodies 2
eAd
CM Can EuP
scT rE ip tD
Discussion. Our report involves 3 Caucasians adults (mean age 41,3 years) with primary VZV infection, clinically and serologically documented, who developed symptoms and signs of acute polyradiculoneuropathy. GBS was diagnosed due to suggestive clinical presentation and typical CSF albumin-cytological dissociation. In one case, electroneurography documented demyelinating neuropathy. This case-series is characterized by short latency period between rash onset and the development of neurological symptoms (6,6 days on average). The course was complicated by acute respiratory failure in the first patient; nevertheless, it was favorable in all cases. Usually, the onset of GBS has a latency period of 2 weeks to 1 month after preceding infection, and shorten latency periods are associated with more severe illness. In our experience, the early IVIG treatment, and the additional plasmapheresis in the first case, have conceivably conditioned the favorable clinical outcome. VZV is a well-known neurotropic virus, responsible of several neurological syndromes (Table 1), usually characterized by detectable VZV-DNA in the CSF [1]. Since 1956, fewer than 50 cases of VZV-related GBS were reported, mostly in course of shingles. Only a few case reports have described the development of GBS following chickenpox, with demographic, clinical and laboratory features similar to those of our experience [2]. The causal mechanism of VZV-related GBS might be “molecular mimics”, as described for other well-known GBS triggering infections. The absence of CSF VZV-DNA and the ineffectiveness of acyclovir-based therapy in this case-series are consistent with GBS immune-mediated pathogenesis. Our experience suggests that primary VZV infection constitutes a GBS triggering event.
cSe pTt
The authors declare that they have no conflict of interest.
References
1. Rottenstreich A, Oz ZK, Oren I. Association between viral load of varicella zoster virus in cerebrospinal fluid and the clinical course of central nervous system infection. Diagn Microbiol Infect Dis. 2014 Jun;79(2):174-7.
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
were present while IgG were absent. Two days after admission, standard IVIG treatment was administered. After 6 days, progressive neurological improvement occurred, with resumption of spontaneous voiding and resolution of paresthesias and weakness. He was then discharged with normal neurological examination.
2. Cresswell F, Eadie J, Longley N, Macallan D. Severe Guillain-Barré syndrome following primary infection with varicella zoster virus in an adult. Int J Infect Dis. 2010 Feb;14(2):161-3.
3
Neurological complications associated with chickenpox and shingles. Chickenpox
Shingles
Encephalitis
Encephalitis
scT rE ip tD
Table 1.
Meningitis
Meningitis
Transverse myelitis
Transverse myelitis
Zoster ophthalmicus
Granulomatous cerebral angiitis
CM Can EuP
Ramsay-Hunt syndrome
cSe pTt
eAd
Post-herpetic nuralgia
JAUc
Int J Neurosci Downloaded from informahealthcare.com by University of Otago on 07/06/15 For personal use only.
Reye’s syndrome
4
