This article reports the diagnosis and treatment of a case of Diabetic ketoacidosis with Guillain-Barré syndrome. Diabetic ketoacidosis (DKA) is the most common acute diabetes mellitus, often diabetes and infecti...This article reports the diagnosis and treatment of a case of Diabetic ketoacidosis with Guillain-Barré syndrome. Diabetic ketoacidosis (DKA) is the most common acute diabetes mellitus, often diabetes and infection, insulin withdrawal or interruption of the history of triggers, with hyperglycemia, ketoacidosis, and acid poisoning as the main symptoms, rapid onset of ill-ness, and serious illness. Guillain-Barré syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with frequent respiratory or gastrointestinal tract infections and low clinical incidence before 1 - 3 weeks. This case was characterized by a headache with vomiting acute onset, a relatively clear diagnosis of diabetic ketoacidosis, a symmetrical decrease in muscle strength in the extremities, and recovery of consciousness after aggressive correction of environmental disturbance in electrolytes, but very poor muscle strength in the extremities, protein-cell segregation in cerebrospinal fluid (CSF). Considering Guillain-Barré syndrome, the patient’s muscle strength gradually recovered after treatment with proglobulin shock. At present, the pathogenesis of the two is not clear, but because of its rapid progress, clinicians should raise awareness of diabetic ketoacidosis combined with Guillain-Barré syndrome, early diagnosis, and active treatment. Inform consent has been obtained from the patient for this report.展开更多
Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-...Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-Barré syndrome and a 90-year-old male patient with facial nerve palsy admitted to our hospital. Both patients experienced recovery from early Guillain-Barré syndrome and peripheral facial nerve palsy after receiving intravenous infusion of NaCl with KCl solution and taking vitamin C.展开更多
Background: Seasonal variation & gender preponderance have not been adequately studied in Guillain Barre Syndrome (GBS). University of Health Sciences, Rohtak is the tertiary referral facility for a large part of ...Background: Seasonal variation & gender preponderance have not been adequately studied in Guillain Barre Syndrome (GBS). University of Health Sciences, Rohtak is the tertiary referral facility for a large part of North West India. We conducted a prospective study to investigate differences in GBS incidence between males and females & across different seasons of the year. Methods:65 clinically diagnosed GBS patients, aged 5 - 70 years, referred for nerve conduction, Fwave & EMG studies for 3 years. Results: 64.61% were males while 35.38% were females. Maximum patients were in the age group 5 - 20 years (46.15%). The highest incidence of GBS (41.53%) were seen in the summer months;19 (29.23%) in the spring season, 11 (16.92%) in winter season and 8 (12.30%) in rainy season. 5 patients had diarrhoea while 12 patients had flu like syndrome 1 - 2 weeks before the onset of GBS. Conclusion: The peak seasonal clustering noted by us in the summer months was consistent significantly with other Asian studies. The age and sex distribution of GBS in our series, which showed children & minor preponderance with peak incidence in 5 - 20 years age followed by another in the age group 21 - 40 years, is different from most studies which report a second peak after 50 years of age.展开更多
BACKGROUND Bortezomib is a first-line drug approved for patients with multiple myeloma (MM) and has significantly increased their overall survival. However, bortezomib-induced peripheral neuropathy (PN) remains a sign...BACKGROUND Bortezomib is a first-line drug approved for patients with multiple myeloma (MM) and has significantly increased their overall survival. However, bortezomib-induced peripheral neuropathy (PN) remains a significant side effect that has led to its discontinuation in some patients. Guillain-Barré syndrome (GBS) is recognized as an immune-mediated PN characterized by the involvement of multiple nerve roots and peripheral nerves and albuminocytologic dissociation in cerebrospinal fluid (CSF) tests. Intravenous immunoglobulin (IVIG) and plasmapheresis are effective. CASE SUMMARY A 45-year-old man diagnosed with stage III MM (λ type) was treated with bortezomib and dexamethasone. Fourteen days after the second course, he complained of intense burning sensation in the lower limbs and hands, loss of tactile sensation, and pain in the distal area of both thighs and in the distal part of both wrist joints. Neurological examination revealed absence of knee and ankle reflexes. CSF examination revealed albuminocytologic dissociation. Nerve conduction studies indicated sensory nerve action potential amplitudes, conduction velocity decrease, and F wave latency prolongation. He was diagnosed as MM complicated with GBS. Subsequently, he was treated with high-dose IVIG (400 mg/kg/d for five days). His symptoms fully resolved without relapse at the 6-month follow-up. CONCLUSION Our case highlights the differential diagnosis and management of complications after bortezomib treatment in MM.展开更多
To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined ...To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.展开更多
We reviewed 100 cases with Guillain－ Barré syndrome (GBS) from 1980 to 1999, and found that the features of GBS in electrophysiological classification, age, area, seasonal distributions, and in preceding illness...We reviewed 100 cases with Guillain－ Barré syndrome (GBS) from 1980 to 1999, and found that the features of GBS in electrophysiological classification, age, area, seasonal distributions, and in preceding illness in northwestern China are different in some aspects from those in Europe and North America or in northern China. The demyelinating pattern appeares as a major subtype not only in different age groups, but also in different test times after symptom onset.展开更多
The present study examined 24 children with acute Guillain-Barre syndrome using magnetic resonance imaging （MRI） plain scans and fat-suppressed enhanced Tl-weighted imaging （T1WI） scans. Axial MRI plain scans cent...The present study examined 24 children with acute Guillain-Barre syndrome using magnetic resonance imaging （MRI） plain scans and fat-suppressed enhanced Tl-weighted imaging （T1WI） scans. Axial MRI plain scans centering on the medullary conus were positive in nine patients （38%）. These displayed variable thickening involving the cauda equina with isointensity on T1WI and isointensity or slight hyperintensity on T2WI. False negatives were obtained in patients with cervical and cranial nerve symptoms. Contrast enhancement of T1WI with fat suppression was positive in all patients in the cauda equina with varied thickening and enhancement centering on the medullary conus. Five patients （36%） were positive in the cervical nerves and 3 patients （50%） were positive in the cranial nerves. These patients had corresponding cervical and cranial nerve symptoms, respectively. Patients with serious clinical symptoms in the lower limbs exhibited obvious involvement of the cauda equina by MRI. Statistical analysis revealed a positive correlation between the extent of enlargement of the cauda equina, centering on the medullary conus, and cerebrospinal fluid protein concentration.展开更多
The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during ...The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.展开更多
Campylobacter jejuni infection has been implicated in the pathogenesis of Guillain-Barré syndrome (GBS) due to production of humoral immune response against neural antigens. A case-control study was performed in ...Campylobacter jejuni infection has been implicated in the pathogenesis of Guillain-Barré syndrome (GBS) due to production of humoral immune response against neural antigens. A case-control study was performed in a tertiary care teaching hospital for the estimation of anti-ganglioside antibodies in GBS patients and their controls. Blood samples were collected from 59 GBS cases, 58 neurological controls (NC) and 60 non-neurological control (NNC) patients for automatic estimation of IgG and IgM antibodies to seven gangliosides using EUROLineScan software. Antibodies of IgG class for GM1 were highly significant in GBS (p = 0.000) and NC (p = 0.031) compared to NNC. However GBS group was not significant (p = 0.413) compared to NC. For GM2 ganglioside, GBS and NC groups were significant (p = 0.000) compared to NNC, but GBS group was not significant (p = 0.999) compared to NC. For GM3 ganglioside, GBS and NC groups were significant (p = 0.000) compared to NNC;but GBS group was insignificant (p = 0.858) compared to NC with similar trend for all other ganglioside antibodies. When IgM class of antibodies was evaluated for GM1, GBS group was not significant (p = 0.604) whereas NC group was significant (p = 0.000) compared to NNC. GBS group was not significant compared to NC (p = 0.011). The trend was the same for GM2 antibodies. For GM3, GBS group was significant (p = 0.010) and NC was near significant (p = 0.055) compared to NNC. However GBS group was not significant (p = 0.808) compared to NC. No groups were significant (p > 0.05) in relation to the remaining gangliosides except for GQ1b where GBS group (p = 0.001) and NC group were significant (p = 0.000) compared to NNC. GBS group was also significant (p = 0.001) compared to NC and NNC. Anti-gangliosides antibodies were present in highly significant levels in the GBS group, though they were also present in the non-paralytic neurological control patients compared to the non-neurological control group.展开更多
BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is med...BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE： To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING： A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS： Venous blood samples were obtained from 6 healthy donors, aged 6-12 years （all routine blood examination items were normal）, and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A （rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form） was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS： Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups： control （2 μg/mL phytohemagglutinin）, 2μg/mL phytohemagglutinin ＋ 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine （3H-TdR） method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay （ELISA）. The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES： The following parameters were measured： rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS： T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group （P 〈 0.05）. The ratio of hTLIA＋ CD3＋ T cells to CD3＋ T cells in acute GBS children was significantly greater than the control group （P 〈 0.01 ）. Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased （P 〈 0.05）. CONCLUSION： In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.展开更多
Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dys...Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.展开更多
The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavir...The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.展开更多
Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Gui...Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.展开更多
Study Objectives: Guillain-Barre syndrome (GBS) is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that often follows an infection. The outcome and prognosis of GBS depend on many ...Study Objectives: Guillain-Barre syndrome (GBS) is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that often follows an infection. The outcome and prognosis of GBS depend on many factors such as the etiology, clinical features, neurophysiology and immunological parameters. The aim of this study was to assess the factors (clinical, investigatory tools, and therapies) that may affect the outcome of patients with GBS. Patients and methods: this was an analytical observational study that was conducted at Ain Shams university hospitals and Kobri Elkoba Military Hospital including twenty patients with the diagnosis of Guillain Barre Syndrome in the duration from 2016 to 2018. This study included twenty patients with the diagnosis of GBS within two weeks from onset of neurologic symptoms, whom their diagnosis based on the established clinical criteria and verified by investigations. Patients were selected from both genders and aged from 18 to 65 years old. Nerve conduction studies and electromyography were performed within two weeks from admission. Various lines of treatment such as plasma exchange (PE), intravenous immunoglobulins (IVIG) or both were used during the period of admission in hospital. Outcome was assessed by the Hughes functional score (F-score), that was applied to the patients on admission, at end of 4 weeks from onset of neuropathy and at the end of 8 weeks. The final outcome at the end of 8 weeks was classified as follow: Group I: good prognosis (0 - 2) on the Hughes functional score (15 patients) and Group II: poor prognosis (3 - 6) on the Hughes functional score (5 patients). Results: the age of the study population ranged from 18 to 65 years with mean of 36.10 ± 16.08 years. Fifteen (75%) patients were males and 5 (25%) patients were females. There was no statistically significant difference found between poor and good prognosis regarding gender. The most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Most patients (75%) had a good outcome at end of study period. It was found that the different line of treatment administered (plasma exchange or IVIG or both) was not associated with poor or good outcome. The patients who needed mechanical ventilation had significantly poor prognosis. Conclusion: the most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Ascending pattern of weakness was more common than descending pattern in this study population and was not related to prognosis. High Hughes score at admission was associated with poor outcome at 8 weeks.展开更多
Introduction: Guillain-Barre Syndrome (GBS) is an acute-onset autoimmune-mediated neuropathy. Guillain-Barre Syndrome can be divided into three subtypes: acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP...Introduction: Guillain-Barre Syndrome (GBS) is an acute-onset autoimmune-mediated neuropathy. Guillain-Barre Syndrome can be divided into three subtypes: acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). About 20% of patients with GBS develop respiratory failure and require mechanical ventilation. We are presenting a variant of GBS (Miller Fisher Syndrome, or MFS), which has been confirmed by nerve conduction studies along with the triad of ophthalmoplegia, ataxia, and areflexia. The objective of this study is to present a rare case of chemotherapy-induced GBS. Important clinic findings: A 25-year-old gentleman with acute lymphocytic leukemia on active chemotherapy treatment presented with lower limb weakness. This weakness started after his fifth chemotherapy session. After the sixth chemotherapy, he developed complete paralysis of the left lower limb. Later, he developed right lower limb paralysis. He was also complaining of eye dryness and incomplete closure of both eyes. While inpatient, he developed upper-limb weakness. His chemotherapy consisted of MESNA, cyclophosphamide, doxorubicin, vincristine, cyorabine, and methotrexate. He had ptosis and ophthalmoplegia in the left abducent and right oculomotor regions. He had bilateral facial nerve palsy. He was hypotonic with power grade 3 in the upper limbs and grade 0 in the lower limbs with areflexia. His sensation was intact in the upper limbs but lost in the lower limbs. His planter reflexes were mute. Diagnoses and Management: Intravenous immunoglobulins were given for 5 days. A nerve conduction study showed severe demyelinating sensorimotor polyradoculoneuropathy with secondary axonal loss. The triad of ataxia, ophthalmoplegia, and areflexia was consistent with MFS. The patient improved over the course of the hospital stay but did not reach full recovery. Conclusion: Although GBS is uncommon, it must be taken into account when making a differential diagnosis for any patient presenting with progressive weakness. Drug history is important in all GBS cases.展开更多
文摘This article reports the diagnosis and treatment of a case of Diabetic ketoacidosis with Guillain-Barré syndrome. Diabetic ketoacidosis (DKA) is the most common acute diabetes mellitus, often diabetes and infection, insulin withdrawal or interruption of the history of triggers, with hyperglycemia, ketoacidosis, and acid poisoning as the main symptoms, rapid onset of ill-ness, and serious illness. Guillain-Barré syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with frequent respiratory or gastrointestinal tract infections and low clinical incidence before 1 - 3 weeks. This case was characterized by a headache with vomiting acute onset, a relatively clear diagnosis of diabetic ketoacidosis, a symmetrical decrease in muscle strength in the extremities, and recovery of consciousness after aggressive correction of environmental disturbance in electrolytes, but very poor muscle strength in the extremities, protein-cell segregation in cerebrospinal fluid (CSF). Considering Guillain-Barré syndrome, the patient’s muscle strength gradually recovered after treatment with proglobulin shock. At present, the pathogenesis of the two is not clear, but because of its rapid progress, clinicians should raise awareness of diabetic ketoacidosis combined with Guillain-Barré syndrome, early diagnosis, and active treatment. Inform consent has been obtained from the patient for this report.
文摘Guillain-Barré syndrome is a rare but fatal autoimmune disease of unknown origin. Infectious disease is the most common etiology of Guillain-Barré syndrome. We had a 75-year-old female patient with Guillain-Barré syndrome and a 90-year-old male patient with facial nerve palsy admitted to our hospital. Both patients experienced recovery from early Guillain-Barré syndrome and peripheral facial nerve palsy after receiving intravenous infusion of NaCl with KCl solution and taking vitamin C.
文摘Background: Seasonal variation & gender preponderance have not been adequately studied in Guillain Barre Syndrome (GBS). University of Health Sciences, Rohtak is the tertiary referral facility for a large part of North West India. We conducted a prospective study to investigate differences in GBS incidence between males and females & across different seasons of the year. Methods:65 clinically diagnosed GBS patients, aged 5 - 70 years, referred for nerve conduction, Fwave & EMG studies for 3 years. Results: 64.61% were males while 35.38% were females. Maximum patients were in the age group 5 - 20 years (46.15%). The highest incidence of GBS (41.53%) were seen in the summer months;19 (29.23%) in the spring season, 11 (16.92%) in winter season and 8 (12.30%) in rainy season. 5 patients had diarrhoea while 12 patients had flu like syndrome 1 - 2 weeks before the onset of GBS. Conclusion: The peak seasonal clustering noted by us in the summer months was consistent significantly with other Asian studies. The age and sex distribution of GBS in our series, which showed children & minor preponderance with peak incidence in 5 - 20 years age followed by another in the age group 21 - 40 years, is different from most studies which report a second peak after 50 years of age.
基金Supported by National Natural Science Foundation of China,No.81660038
文摘BACKGROUND Bortezomib is a first-line drug approved for patients with multiple myeloma (MM) and has significantly increased their overall survival. However, bortezomib-induced peripheral neuropathy (PN) remains a significant side effect that has led to its discontinuation in some patients. Guillain-Barré syndrome (GBS) is recognized as an immune-mediated PN characterized by the involvement of multiple nerve roots and peripheral nerves and albuminocytologic dissociation in cerebrospinal fluid (CSF) tests. Intravenous immunoglobulin (IVIG) and plasmapheresis are effective. CASE SUMMARY A 45-year-old man diagnosed with stage III MM (λ type) was treated with bortezomib and dexamethasone. Fourteen days after the second course, he complained of intense burning sensation in the lower limbs and hands, loss of tactile sensation, and pain in the distal area of both thighs and in the distal part of both wrist joints. Neurological examination revealed absence of knee and ankle reflexes. CSF examination revealed albuminocytologic dissociation. Nerve conduction studies indicated sensory nerve action potential amplitudes, conduction velocity decrease, and F wave latency prolongation. He was diagnosed as MM complicated with GBS. Subsequently, he was treated with high-dose IVIG (400 mg/kg/d for five days). His symptoms fully resolved without relapse at the 6-month follow-up. CONCLUSION Our case highlights the differential diagnosis and management of complications after bortezomib treatment in MM.
基金the Rockefeller Foundation in the United States (Grant no. HS 9430).
文摘To explore the risk factors for Guillain-barre syndrome. Methods Case-control study design was used in 51 cases of Guillain-barre syndrome,and 51 matched controls.All of the 51 cases in this study had been examined by electrophysiology. Serum IgG antibodies specific for C. jejuni were determined in all the subjects by ELISA. Each case and control were interviewed using an ad hoc questionnaire, including his/her demographic information,onset of the illness, their personal hygiene and so on. Results The study showed that Guillain-barre syndrome was associated with a few factors, such as polio vaccine immunization before onset of illness (OR=7.27), no hand washing after defecation and before meals (OR=6.15). Infection of C. jejuni was strongly associated with the illness (OR=9.5,P<0.001). Conclusion It is suggested that occurrence of Guillain-barre syndrome may correlate to infection of C. jejuni and poor personal hygiene in children.
文摘We reviewed 100 cases with Guillain－ Barré syndrome (GBS) from 1980 to 1999, and found that the features of GBS in electrophysiological classification, age, area, seasonal distributions, and in preceding illness in northwestern China are different in some aspects from those in Europe and North America or in northern China. The demyelinating pattern appeares as a major subtype not only in different age groups, but also in different test times after symptom onset.
文摘The present study examined 24 children with acute Guillain-Barre syndrome using magnetic resonance imaging （MRI） plain scans and fat-suppressed enhanced Tl-weighted imaging （T1WI） scans. Axial MRI plain scans centering on the medullary conus were positive in nine patients （38%）. These displayed variable thickening involving the cauda equina with isointensity on T1WI and isointensity or slight hyperintensity on T2WI. False negatives were obtained in patients with cervical and cranial nerve symptoms. Contrast enhancement of T1WI with fat suppression was positive in all patients in the cauda equina with varied thickening and enhancement centering on the medullary conus. Five patients （36%） were positive in the cervical nerves and 3 patients （50%） were positive in the cranial nerves. These patients had corresponding cervical and cranial nerve symptoms, respectively. Patients with serious clinical symptoms in the lower limbs exhibited obvious involvement of the cauda equina by MRI. Statistical analysis revealed a positive correlation between the extent of enlargement of the cauda equina, centering on the medullary conus, and cerebrospinal fluid protein concentration.
文摘The recommended therapy for chronic hepatitis C (CHC) infection is the combination of a Pegylated interferon and Ribavirin. Almost all such patients on combination therapy experience one or more adverse events during the course of treatment. Significant neurological side effects are rare. A few cases of Bell's Palsy, chronic inflammatory demyelinating polyneuropathy and even one case of acute demyelinating polyneuropathy with atypical features for Guillain-Barre syndrome (GBS) associated with Interferon therapy have been reported but no report of GBS with typical features has been published. We present a case report of typical GBS associated with Peginterferon alfa-2a and Ribavirin used for treatment of CHC infection.
文摘Campylobacter jejuni infection has been implicated in the pathogenesis of Guillain-Barré syndrome (GBS) due to production of humoral immune response against neural antigens. A case-control study was performed in a tertiary care teaching hospital for the estimation of anti-ganglioside antibodies in GBS patients and their controls. Blood samples were collected from 59 GBS cases, 58 neurological controls (NC) and 60 non-neurological control (NNC) patients for automatic estimation of IgG and IgM antibodies to seven gangliosides using EUROLineScan software. Antibodies of IgG class for GM1 were highly significant in GBS (p = 0.000) and NC (p = 0.031) compared to NNC. However GBS group was not significant (p = 0.413) compared to NC. For GM2 ganglioside, GBS and NC groups were significant (p = 0.000) compared to NNC, but GBS group was not significant (p = 0.999) compared to NC. For GM3 ganglioside, GBS and NC groups were significant (p = 0.000) compared to NNC;but GBS group was insignificant (p = 0.858) compared to NC with similar trend for all other ganglioside antibodies. When IgM class of antibodies was evaluated for GM1, GBS group was not significant (p = 0.604) whereas NC group was significant (p = 0.000) compared to NNC. GBS group was not significant compared to NC (p = 0.011). The trend was the same for GM2 antibodies. For GM3, GBS group was significant (p = 0.010) and NC was near significant (p = 0.055) compared to NNC. However GBS group was not significant (p = 0.808) compared to NC. No groups were significant (p > 0.05) in relation to the remaining gangliosides except for GQ1b where GBS group (p = 0.001) and NC group were significant (p = 0.000) compared to NNC. GBS group was also significant (p = 0.001) compared to NC and NNC. Anti-gangliosides antibodies were present in highly significant levels in the GBS group, though they were also present in the non-paralytic neurological control patients compared to the non-neurological control group.
基金Supported by:the Program of the Key Laboratory of Health Department of Jilin Province, No.2006079the Fortieth National Post-Doctoral Scientific Foundation,No. 20060400893
文摘BACKGROUND： Human tumor necrosis factor-like molecule 1A （hTL1A） is a strong T helper cell type 1 （Thl） co-stimulator. Guillain-Barre syndrome （GBS） is an autoimmune disorder of the nervous system, which is mediated by Thl cells. OBJECTIVE： To determine hTL1A expression in peripheral blood T lymphocytes of acute GBS children and the effects of hTL1A on secretion of interferon-γ. DESIGN, TIME AND SETTING： A randomized, controlled, neuroimmunological in vitro study was performed at the Central Laboratory of First Hospital of Jilin University, China from November 2005 to November 2007. MATERIALS： Venous blood samples were obtained from 6 healthy donors, aged 6-12 years （all routine blood examination items were normal）, and 6 additional children with acute GBS, aged 6-12 years. The GBS children fell ill within 1 week and were not treated with hormones or immunoglobulin Purified recombinant human soluble tumor necrosis factor-like molecule 1A （rhsTL1A, 1 mg/mL, relative molecular mass 22 000, 6× His tag, soluble form） was supplied by the Central Laboratory of First Hospital of Jilin University, China. METHODS： Peripheral blood mononuclear cells were isolated from healthy donors using the standard Ficoll gradient centrifugation and were incubated in 96-well culture plates. The cells were assigned to the following groups： control （2 μg/mL phytohemagglutinin）, 2μg/mL phytohemagglutinin ＋ 25, 100 and 400 ng/mL rhsTL1A. T cell proliferation was quantified using the tritiated thymidine （3H-TdR） method. Serum interferon-γ levels in acute GBS children were detected by enzyme-linked immunosorbent assay （ELISA）. The ratio of hTL1A-positive T cells to CD3-positive T cells in peripheral blood of acute GBS children was determined using flow cytometry. Following in vitro pre-activation of peripheral blood mononuclear cells by 2 μg/mL phytohemagglutinin, the peripheral blood mononuclear cells were treated with 400 ng/mL exogenous rhsTLIA. Finally, peripheral blood mononuclear cell-secreted interferon-γlevels were measured by ELISA. MAIN OUTCOME MEASURES： The following parameters were measured： rhsTLIA stimulation index to stimulate proliferation of T cells; the serum interferon-γ levels in acute GBS children; the ratio of hTL1A-positive cells to CD3-positive cells; the levels of interferon-γ secreted by peripheral blood mononuclear cells in acute GBS children, as well as rhsTL1A-stimulated interferon-γ levels. RESULTS： T cell proliferation assay revealed that the stimulation index in each rhsTL1A group was greater than the control group. The stimulation index of the 400 ng/mL rhsTL1A group was the greatest. Serum interferon-γ levels in acute GBS children were significantly greater than the control group （P 〈 0.05）. The ratio of hTLIA＋ CD3＋ T cells to CD3＋ T cells in acute GBS children was significantly greater than the control group （P 〈 0.01 ）. Phytohemagglutinin stimulated peripheral blood mononuclear cells to a greater extent than 400 ng/mL rhsTL1A in the acute GBS group, and the secreted interferon-γ levels were significantly increased （P 〈 0.05）. CONCLUSION： In T cells pre-activated with 2 μg/mL phytohemagglutinin, proliferation was effectively increased with 400 ng/mL rhsTL1A treatment. Expression of hTLIA was increased in activated T cells from peripheral blood of acute GBS children, followed by increased interferon-γ secretion. These mechanisms are considered to be part of the pathological process that induces the secretion of inflammatory cytokines in GBS syndrome.
文摘Background: Guillain-Barre syndrome (GBS) is an autoimmune disorder characterized by a heterogeneous group of pathological and clinical entities. It is associated with ascending areflexic paralysis, some autonomic dysfunction and respiratory failure in severe cases and ultimately death if not promptly diagnosed and treated. It may be preceded by an antecedent event in about two-third of cases. This could be an upper respiratory tract infection, viral illness, recent history of vaccination, pregnancy, cancer or even trauma. The condition is exceedingly rare in pregnancy and only few cases have been reported in literature. Case Report: This is a case of a 28-year-old Gravida 3, Para 1+1 and Estimated Gestational Age of 30 weeks and 4 days. There was a history of upper respiratory tract infection eight weeks prior to presentation which spontaneously resolved. On examination, she was a young woman, anxious, weak, afebrile, not pale, the neck could not hold the head upright and there was bilateral non tender pitting pedal oedema extending to her mid-shin. There were no cranial nerve deficits and no sign of meningeal irritation. There were normal muscle bulk with global hypotonia and flaccid quadriparesis, Power was 3/5. The proximal groups of muscles were more affected than the distal parts. Reflexes were diminished globally with plantar flexor response. She had immunoglobulin as treatment. Conclusion: In a low resource setting like ours it is important to have a high index of suspicion of GBS when an apparently healthy gravid woman presents with progressive weakness of the limbs.
文摘The severe acute respiratory syndrome coronavirus 2 infection typically presents with respiratory symptoms. Additionally, there are a number of less frequent neurological manifestations of infection with the coronavirus disease 2019 (COVID-19) with case reports suggesting an association with the Guillain-Barre syndrome. Most patients present with the typical upper respiratory symptoms in association with these neurological symptoms. We present a case of an unvaccinated gentleman with none of the typical respiratory symptoms of COVID-19 who presented with the Guillain Barre syndrome and myalgia. His symptoms settled following treatment with intravenous immunoglobulins. This case highlights the importance of testing for COVID-19 in patients without typical symptoms but who present with neurological illness and supports the use of intravenous immunoglobulin therapy.
文摘Background: The association of Guillain-Barre syndrome and cerebral sinus thrombosis is uncommon. Case Presentation: We report a 37-year-old patient hospitalized in medical ICU for respiratory distress following a Guillain-Barre syndrome. He had symptomatic treatment in addition to plasma exchange. In the presence of clonic movements, a brain venography magnetic resonance showed a thrombophlebitis of the left lateral sinus, and hence a low-molecular-weight heparin treatment was begun. Immunological, thrombophilia and serological tests were negative. After a favorable evolution, he was transferred to the neurology department. Conclusion: The combination of a Guillain-Barre syndrome and a cerebral sinus thrombosis would suggest a common process. A rigorous investigation, including the use of imaging, is necessary in front of any unusual clinical sign during a GBS.
文摘Study Objectives: Guillain-Barre syndrome (GBS) is an acute-onset, monophasic immune-mediated disorder of the peripheral nervous system that often follows an infection. The outcome and prognosis of GBS depend on many factors such as the etiology, clinical features, neurophysiology and immunological parameters. The aim of this study was to assess the factors (clinical, investigatory tools, and therapies) that may affect the outcome of patients with GBS. Patients and methods: this was an analytical observational study that was conducted at Ain Shams university hospitals and Kobri Elkoba Military Hospital including twenty patients with the diagnosis of Guillain Barre Syndrome in the duration from 2016 to 2018. This study included twenty patients with the diagnosis of GBS within two weeks from onset of neurologic symptoms, whom their diagnosis based on the established clinical criteria and verified by investigations. Patients were selected from both genders and aged from 18 to 65 years old. Nerve conduction studies and electromyography were performed within two weeks from admission. Various lines of treatment such as plasma exchange (PE), intravenous immunoglobulins (IVIG) or both were used during the period of admission in hospital. Outcome was assessed by the Hughes functional score (F-score), that was applied to the patients on admission, at end of 4 weeks from onset of neuropathy and at the end of 8 weeks. The final outcome at the end of 8 weeks was classified as follow: Group I: good prognosis (0 - 2) on the Hughes functional score (15 patients) and Group II: poor prognosis (3 - 6) on the Hughes functional score (5 patients). Results: the age of the study population ranged from 18 to 65 years with mean of 36.10 ± 16.08 years. Fifteen (75%) patients were males and 5 (25%) patients were females. There was no statistically significant difference found between poor and good prognosis regarding gender. The most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Most patients (75%) had a good outcome at end of study period. It was found that the different line of treatment administered (plasma exchange or IVIG or both) was not associated with poor or good outcome. The patients who needed mechanical ventilation had significantly poor prognosis. Conclusion: the most common electrophysiological subtype was demyelinating followed by axonal neuropathy. Ascending pattern of weakness was more common than descending pattern in this study population and was not related to prognosis. High Hughes score at admission was associated with poor outcome at 8 weeks.
文摘Introduction: Guillain-Barre Syndrome (GBS) is an acute-onset autoimmune-mediated neuropathy. Guillain-Barre Syndrome can be divided into three subtypes: acute inflammatory demyelinating poly-radiculo-neuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). About 20% of patients with GBS develop respiratory failure and require mechanical ventilation. We are presenting a variant of GBS (Miller Fisher Syndrome, or MFS), which has been confirmed by nerve conduction studies along with the triad of ophthalmoplegia, ataxia, and areflexia. The objective of this study is to present a rare case of chemotherapy-induced GBS. Important clinic findings: A 25-year-old gentleman with acute lymphocytic leukemia on active chemotherapy treatment presented with lower limb weakness. This weakness started after his fifth chemotherapy session. After the sixth chemotherapy, he developed complete paralysis of the left lower limb. Later, he developed right lower limb paralysis. He was also complaining of eye dryness and incomplete closure of both eyes. While inpatient, he developed upper-limb weakness. His chemotherapy consisted of MESNA, cyclophosphamide, doxorubicin, vincristine, cyorabine, and methotrexate. He had ptosis and ophthalmoplegia in the left abducent and right oculomotor regions. He had bilateral facial nerve palsy. He was hypotonic with power grade 3 in the upper limbs and grade 0 in the lower limbs with areflexia. His sensation was intact in the upper limbs but lost in the lower limbs. His planter reflexes were mute. Diagnoses and Management: Intravenous immunoglobulins were given for 5 days. A nerve conduction study showed severe demyelinating sensorimotor polyradoculoneuropathy with secondary axonal loss. The triad of ataxia, ophthalmoplegia, and areflexia was consistent with MFS. The patient improved over the course of the hospital stay but did not reach full recovery. Conclusion: Although GBS is uncommon, it must be taken into account when making a differential diagnosis for any patient presenting with progressive weakness. Drug history is important in all GBS cases.
