Acute Inflammatory Demyelinating Polyradiculoneuropathy Research Articles

Guillain-Barré syndrome: History, pathogenesis, treatment, and future directions.

Since its description by Guillain, Barré, and Strohl in 1916, Guillain-Barré syndrome (GBS) has attracted a large literature. The author reviews the history of research into its pathogenesis and treatment to highlight promising avenues for future research. This is a nonsystematic personal review. Since the early 1900s, the clinical picture of GBS has been illustrated in multiple series culminating in the ongoing International Guillain-Barré Syndrome study of 2000 patients. In the 1950s and 1960s, the inflammatory nature of the commonest form, acute inflammatory demyelinating polyradiculoneuropathy (AIDP), was described. In the 1990s, two axonal forms, acute motor-sensory axonal neuropathy and acute motor axonal neuropathy, were recognized. In the 1990s and early 2000s, these forms were shown to be due to antibodies against Campylobacter jejuni glycans cross-reacting with glycolipids on axonal membranes. The pathogenesis of AIDP remains unknown, but T-cell responses to the compact myelin proteins, P2 and P0, which cause experimental autoimmune neuritis, suggest that T cells are important. Randomized controlled trials in the 1970s and 1980s showed no benefit from corticosteroids. Trials in the 1980s showed benefit from plasma exchange and in the 1990s from intravenous immunoglobulin. Future research should seek biomarkers to identify subgroups with different treatment responses, define the true natural history of the disease with population-based epidemiological studies, study the pathology in autopsies early in the disease, seek causative antibodies and confirm autoimmune T-cell responses in AIDP, and expand treatment trials to include anti-T-cell agents.

The sural-sparing pattern in clinical variants and electrophysiological subtypes of Guillain-Barré syndrome.

Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency.

Electrophysiologic Findings in Pediatric Guillain Barre Syndrome

Objective: To describe electrophysiologic findings in the pediatric GuillianBarre syndrome (GBS).&#x0D; Study design: Comparative prospective study.&#x0D; Place and Duration of Study: Department of Neurology, The Children’s Hospital and Institute of Child Health, Lahore Pakistan, from Jun to Dec 2015.&#x0D; Methodology: Children below 18 years of age, presenting in medical emergency/neurology OPD and fulfilling the clinical case definition of GBS were included for the study. Electrophysiologic studies were performed within 24 hours of admission in all patients.&#x0D; Results: Out of 83 patients with GBS, 59% were male and 80% were between 3-12 years of age. According to the electrophysiological findings, acute inflammatory demyelinating polyradiculoneuropathy (AIDP) was the most common subtype followed by acute motor axonal neuropathy (AMAN). Reduced CMAP and absent F-response were the most common electrophysiologic findingspresented in 70% and 57.8% of patients respectively. However, absent F-response was not specific for any subtype (p&gt;0.05). Prolonged motor DL, reduced NCV, temporal dispersion and abnormal F-wave latency were characteristicelectrophysiologic features of demyelination (p≤0.001). However, prolonged motor DL and absent F-wave occurred early in the course of disease while reduced NCV and temporal dispersion observed later.&#x0D; Conclusion: Electrophysiologic studieswere useful in making the appropriate diagnosis to initiate immunotherapy, particularly during first week after onset of weakness when albuminocytologic dissociation may not be present.

An Atypical Clinical Presentation of Acute Inflammatory Demyelinating Polyradiculoneuropathy

Acute inflammatory demyelinating polyradiculoneuropathy (AIDP), also known as Guillain-Barre syndrome (GBS), is an autoimmune disease that is typically present as a progressive ascending weakness, with subtle or no sensory findings, and reduced or absent deep tendon reflexes. In this case report I am presenting a patient who presented with an atypical clinical feature of AIDP in the form of a descending rather than ascending weakness, highlighting the importance of considering AIDP as a potential diagnosis in such atypical presentations.

Sympathetic skin response (SSR) in pediatric Guillain-Barré syndrome.

In the present study, sympathetic skin response (SSR) characteristics were explored in children with Guillain-Barré syndrome (GBS) and the value of early diagnosis and prognostic evaluation in GBS complicated by autonomic dysfunction (AD) was evaluated. A total of 25 children with GBS and 30 healthy controls (HCs) were enrolled in this prospective study. SSR findings for the two groups were compared. SSR and nerve conduction study (NCS) results were compared among patients with GBS, and differences in clinical characteristics between the groups with abnormal and normal SSR were analyzed. Within the GBS group, six patients (24%) required mechanical ventilation, 17 patients (66.7%) had AD, 18 patients (72%) had an abnormal SSR, and 13 patients (52%) had AD combined with SSR abnormalities. There was a statistically significant difference in SSR latency in the lower limbs between the GBS group and HCs (P < 0.05). There was no statistically significant difference between SSR and NCS results in the acute phase of GBS (P > 0.05), and there was no significant difference in the rate of AD or in Hughes functional grade at nadir between the groups with abnormal and normal SSR (P>0.05). However, there was a statistically significant difference between the results of SSR and NCS tests during the recovery phase (P = 0.003). Abnormal SSR mainly occurred in cases of the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) subtype. In addition, SSR was abnormal in all pediatric GBS patients with poor prognosis 1 month after onset of symptoms. Two-thirds of the children with GBS have AD. SSR could be used for early diagnosis and follow-up of GBS and may also be helpful in evaluating disease severity and short-term prognosis.

Comparison of ulnar, median, and sural sensory nerve conduction studies between demyelinating and axonal forms of Guillain-Barré Syndrome

Background: Guillain-Barré syndrome (GBS) is an important neurological disease that can cause disability. Axonal GBS and acute inflammatory demyelinating polyradiculoneuropathy (AIDP) are two important subgroups.&#x0D; Methods: Patients with clinical and electrodiagnostic features compatible with GBS were included in this retrospective study. The patients were divided into two groups neurophysiologically as Axonal GBS and AIDP. Medical research council (MRC) scores of the patients' muscles, median / ulnar / posterior tibial / peroneal / sural nerve conduction study findings were included in the analyses. Sural sparing pattern was considered as the absence of median/ulnar compound nerve action potential (CNAP) and present sural nerve CNAP (SS-M/SS-U).&#x0D; Results: Twelve AIDP and 10 Axonal GBS patients were included in the study. MRC scores were not different between the two groups (p=0.895). CNAPs of the right median and ulnar nerves were smaller in AIDP patients than in Axonal GBS patients (p

A Perplexing Case of Borrelia Burgdorferi (P9-10.003)

<h3>Objective:</h3> To expose a potential Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) mimicker and understand the significance of diagnostic tests in a case of Bannwarth syndrome. <h3>Background:</h3> Lyme borreliosis is an infectious disease affecting the skin, nervous system, and joints transmitted by the bite of Ixodes Ricinus. Neurological manifestations occur in 3–15% of the cases. Bannwarth syndrome is reported in 3% of the CDC-verified cases of Lyme disease. It presents early within weeks to a few months of initial infection, mainly characterized by painful polyradiculoneuritis. <h3>Design/Methods:</h3> <h3>Case:</h3> A 67-year-old male presented with progressive bilateral upper extremity weakness, sparing the lower extremities with diffuse areflexia, facial diplegia, dysarthria, dysphagia, and diplopia. <h3>Results:</h3> Initial exam was remarkable for bilateral arm weakness with left-sided lower motor neuron facial palsy and dysarthria. Symptoms quickly worsened to facial diplegia and dysphagia with inability to manage secretions. Magnetic resonance imaging (MRI) brain showed hyperintensity with enhancement of the left cerebellum and cauda equina enhancement on MRI lumbar spine. Cerebrospinal fluid (CSF) analysis was remarkable for elevated protein (448 mg/dl), mild pleocytosis (8/mm³), and albumin cytological dissociation. Monoclonal protein IgG kappa was present in the gamma globulin region. History, exam and initial work-up led to a tentative diagnosis of pharyngeal-cervical-brachial variant of AIDP. He was treated with plasmapheresis with mild improvement. Initial Lyme serum and CSF serology were positive with a positive confirmatory CSF western blot. His inpatient electromyography exposed asymmetric, subacute cervical polyradiculopathy with signs of active axonal denervation and reinnervation changes with no acquired demyelination. The patient was started on intravenous Ceftriaxone and physical therapy with significant improvement. <h3>Conclusions:</h3> Lyme disease is a magnificent chameleon; hence it is crucial to keep it among the differential diagnosis. This case emphasizes the importance of EMG amongst diagnostic tools given it ultimately, in combination with Lyme testing, changed the diagnosis and management for the patient. <b>Disclosure:</b> Dr. Sahni has nothing to disclose. Dr. Samaniego has nothing to disclose. Dr. Gates has nothing to disclose.

Frequency, Age, Gender Distribution, and Seasonal Variation of Guillain-Barré Syndrome in a Province of Pakistan: A Retrospective Study

Characterized by the sudden onset of muscle weakness, Guillain-Barré Syndrome (GBS) is a rare autoimmune disorder which can progress to paralysis. GBS has different subtypes based on the clinical and electrophysiological characteristics, including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), axonal GBS (AMAN and AMSAN), and Miller Fisher Syndrome (MFS). Objective: To study frequency of the disease in Khyber Pakhtunkhwa. Methods: In this retrospective study, medical records of 39 patients diagnosed with GBS at Lady Reading Hospital Peshawar, Pakistan, were analyzed to determine the prevalence of GBS subtypes in the country. Results: The results showed that the most prevalent subtype of GBS was AMAN, accounting for 59% of cases, followed by AMSAN at 25.6%, and AIDP at 15.3%. The axonal variety made up 84.6% of the total GBS cases in this study. On average, the patients with AMSAN were 39.2 years old, while patients with AMAN and AIDP were relatively younger, with mean ages of 30 and 28 years, respectively. There was a male predominance in all subtypes except for AIDP, which showed equal distribution. Conclusions: These findings provide valuable information on the distribution of GBS subtypes in Peshawar, Pakistan, which may have implications for the diagnosis and management of GBS in the country. Additionally, the study's results can contribute to the global understanding of GBS epidemiology and may help improve the diagnosis and treatment of GBS patients worldwide.

A Retrospective Single-center Study of Presentation and Prognosis of Guillain-Barré Syndrome in Pediatric Patients

Background: Guillain-Barré syndrome (GBS) is a post-infectious immune-mediated peripheral neuropathy, progressing bilaterally and often symmetrically and affecting sensory and motor function. Most cases completely recover, but around 20% of cases may lead to complications, incomplete recovery, or even death. Objectives: This study aims to assess the prognosis of GBS in pediatric patients and possible associated conditions regarding recovery or prognosis. Methods: We investigated 71 cases of GBS admitted to Mofid Pediatric Hospital from March 2014 to March 2017. Demographic, clinical, and laboratory data were retrospectively recorded and analyzed. Two follow-up visits were performed after 1 to 3 and 5 to 8 years from onset, according to the GBS Disability Scale, and recovery of motor function was assessed during patients’ visits to the clinic. Results: We found 35 male and 36 female subjects with an average age of 6.17 ± 3.82 (range 0.9 up to 15 years old); cases were mostly presented with myalgia and weakness (78.9%) followed by headache, found in 5 patients (7%). Around 84.5% of patients had an upper respiratory infection as their antecedent infection. Fifteen cases of autonomic dysfunction were observed, and 15 patients had cranial nerve involvement. Most cases had the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) form of GBS on electrophysiologic tests. Analysis showed only axonal involvement was significantly correlated with poor prognosis (P-value

Miller Fisher Syndrome Induced by Chemotherapy in Known Case of Acute Lymphocytic Leukaemia: A Case Report

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.

Elevated monocyte to high-density lipoprotein cholesterol ratio correlates with clinical severity in acute inflammatory demyelinating polyradiculoneuropathy patients.

While monocyte to high-density lipoprotein cholesterol ratio (MHR) has been reported to be associated with nervous system lesions, the role of MHR has not been determined in patients with Guillain-Barré Syndrome (GBS). The purpose of our study was to explore the role of MHR in patients with GBS. A total of 52 GBS patients were involved in the study retrospectively, including patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), and acute motor sensory axonal neuropathy (AMSAN). We used Hughes Functional Grading Scale (HFGS) score to evaluate functional status in GBS patients. Among patients with different subtypes of GBS, MHR was significantly elevated in those with demyelination compared to patients without demyelination (p < 0.001); AIDP patients had an increased MHR compared with AMAN or AMSAN patients (p = 0.001; p = 0.013). There was a positive correlation between MHR and HFGS score (r = 0.463, p = 0.006) in AIDP patients, but not in AMAN or AMSAN. Multiple linear regression analysis revealed that MHR was independently associated with HFGS score (beta = 0.405, p = 0.013) in AIDP patients. Our study suggests that MHR as an inflammatory marker is elevated in patients with AIDP compared to AMAN or AMSAN patients, while MHR has a positive correlation with clinical severity in AIDP patients, suggesting that MHR may provide an additional information to reflect the pathophysiology of AIDP.

Children with Guillain-Barre Syndrome: A Comparison between AIDP and AMAN variants among Patients admitted in a Tertiary Care Hospital

Background: Guillain-Barre syndrome (GBS) is the leading cause of acute flaccid paralysis in children. This study was aimed to compare the clinical spectrum and shortterm outcome of children with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and acute motor axonal neuropathy (AMAN) subtypes of GBS in children. Methods: The study was a prospective cohort study done in a tertiary neurology hospital for 3 years. Children under 18 years of age fulfilling the Brighton diagnostic criteria for GBS were enrolled in the study. Based on the nerve conduction study, patients were subclassified as AIDP, AMAN, AMSAN, and others. Finally, a comparison was done in children with AIDP and AMAN subtypes. Results: A total of 102 children have fulfilled the Brighton diagnostic criteria of GBS during that study period. Among them, 83 children were included in the final analysis as NCS findings suggestive of AIDP and AMAN were found in 29(28.43%) and 54(52.94%) of cases respectively. No patient died in this cohort and follow-up was done at 3 months after discharge. A comparison of clinical data between the two groups revealed similar clinical features in most of the cases. The mean age difference between the two groups was statistically significant and AIDP was found to be more frequent in the 1-5 years age group. There was a significant association between gastroenteritis and AMAN subtypes. On symptom analysis, pain and tingling sensation were found predominantly in AMAN subtypes. Children having AMAN variants developed respiratory distress more than AIDP. Assisted ventilation were needed in 14.45% of cases and the majority of them were from the AMAN group. The mean duration of hospital stay and the mean disability scores at three months after discharge were significantly higher in the AMAN group. Conclusions: AMAN was the commonest GBS subtypes in children. AIDP was more frequent in the younger age group. Children with AMAN appeared to have higher short-term morbidity and slower recovery than those with AIDP. Bang. J Neurosurgery 2022; 11(2): 94-100

Outcome of Guillain-Barré syndrome following intravenous immunoglobulin compared to natural course.

Intravenous immunoglobulin (IVIg) is recommended in Guillain-Barré syndrome (GBS), but its efficacy may vary in different subtypes. We report the outcomes of patients with GBS following IVIg treatment compared to the natural course (NC). We also compare the effect of IVIg treatment in different subtypes of GBS. From a cohort of 528 GBS subjects, we have extracted 189 patients who received IVIg and compared their outcomes with 199 age- and peak disability-matched patients who did not receive IVIg, plasmapheresis, or corticosteroid. Disability was assessed using the 0-6 Guillain-Barré Syndrome Disability Scale (GBSDS). Clinical and neurophysiological subtypes were recorded. The primary outcome was functional disability at 6 months, which was categorized as complete (GBSDS ≤ 1), partial (GBSDS 2-3), or poor (GBSDS &gt; 3). The secondary outcomes were in-hospital death, duration of hospitalization, and mechanical ventilation. In-hospital death (2.6% vs. 2%, p=0.74) and 3-month poor recovery (20.7% vs. 18%) were similar in the IVIg and NC groups. At 6 months, however, a lesser proportion of patients in the IVIg group had poor recovery (2.2% vs. 8.3%, p=0.026). The outcomes of IVIg and NC were compared in 72 acute motor axonal neuropathy (AMAN) and 256 acute inflammatory demyelinating polyradiculoneuropathy (AIDP) patients. IVIg therapy did not alter the outcome in AMAN but resulted in a lesser proportion of poor recovery at 6 months in AIDP (0.8% vs. 6.6%, p=0.03). IVIg is beneficial in AIDP variants of GBS but not in the AMAN subtype. A customized treatment may be cost-effective until a randomized controlled trial is conducted in AMAN.

Natural course of Guillain-Barré syndrome.

In this retrospective study based on a registry, Kalita et al. [1] compared the outcomes of 189 patients with Guillain-Barré syndrome (GBS) who received intravenous immunoglobulin (IVIg), with 199 age- and peak disability-matched patients who did not receive any immunomodulation or plasmapheresis, thus representing the natural course of GBS. Numbers for in-hospital deaths and poor recovery at 3 months were similar between the two groups. At 6 months, 8.3% of the natural course subgroup, but only 2.2% of the IVIg group, had a poor outcome. After the patients had been divided into those with the acute motor axonal neuropathy (AMAN) phenotype and the acute inflammatory demyelinating polyradiculoneuropathy (AIDP) phenotype, only those with the AIDP phenotype had a better 6-month outcome with IVIg. This study could not have been done in any setting where IVIg or plasmapheresis, the two established treatment modalities for GBS, are readily available. In fact, no clinical trial in GBS with a placebo arm would receive an ethics commission's approval for being carried out. Thus, data like the ones presented in this study are very valuable for our understanding of GBS treatment. The International Guillain-Barré Syndrome Outcome Study (IGOS) is collecting data on GBS patients around the world in a registry. Thus, here is another chance to compare outcomes of patients with and without immunomodulatory treatment. In one report from the IGOS group on current practice of GBS treatment, 193 patients from Bangladesh were excluded from the analysis, because 83% of them were not treated [2]. A study from Bangladesh reported a high mortality (12%) in GBS patients [3]. This was mainly due to lack of ventilator support. In this cohort, 71% of patients are reported as not receiving treatment. Lack of treatment was not a risk factor for mortality; however, the type of treatment is not specified in the report. Together, these data support the notion that intensive care support is more crucial for the survival of GBS patients than the immunomodulatory treatment [4]. We need to consider that the natural course subgroup in the study by Kalita et al. did not renounce treatment out of their free will or for medical reasons. The mere reason was that these patients could not afford the treatment. The group for natural course was matched to the IVIg group by the authors according to age and peak disability. Thus, any questions on whether disability did, in the end, influence the decision on treatment cannot be answered by the present study. However, the authors might further analyze their registry for this question. Claudia Sommer: Conceptualization (equal); writing – original draft (equal); writing – review and editing (equal). Open Access funding enabled and organized by Projekt DEAL. C.S. is a member of the International Guillain-Barré syndrome Outcome Study (IGOS) consortium. Data sharing is not applicable to this article as no new data were created or analyzed in this study.

COVID-19 vaccine-related Guillain-Barré syndrome in the Liguria region of Italy: A multicenter case series

Background and purposeGuillain-Barré-Syndrome (GBS) can follow COVID-19 vaccination, with clinical and paraclinical features still to be precisely assessed. We describe a cohort of patients who developed GBS after vaccination with different types of COVID-19 vaccines. MethodsPatients with post-COVID-19 vaccination GBS, admitted to the six hospitals that cover the whole Liguria Region, Northwestern Italy, from February 1st to October 30th 2021, were included. Clinical, demographic, and paraclinical data were retrospectively collected. ResultsAmong the 13 patients with post-COVID-19 vaccination GBS (9 males; mean age, 64 year), 5 were vaccinated with Oxford-AstraZeneca, 7 with Pfizer-BioNTech, and one with Moderna. Mean time between vaccination and GBS onset was 11.5 days. Ten patients developed GBS after the first vaccination dose, 3 after the second dose. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) was the predominant GBS variant, mainly characterized by sensory involvement. Bilateral seventh cranial nerve involvement followed AstraZeneca vaccination in two cases. Three patients presented treatment-related fluctuations, and 4 mild symptoms that delayed treatments and negatively affected prognosis. Prognosis was poor (GBS-disability score, ≥3) in 5/13 patients, with a disability rate of 3/13. ConclusionsOur findings confirm that most post-COVID-19 vaccination GBS belong to the AIDP subtype, and occur after the first vaccine dose. Treatment-related fluctuations, and diagnosis-delaying, mild symptoms at onset are clinical features that affect prognosis and deserve particular consideration.

Review of Acute Inflammatory Demyelinating Polyradiculoneuropathy

On average, 97% of patients in North America and Europe diagnosed with Guillain-Barré syndrome have a specific subtype known as acute inflammatory demyelinating polyradiculoneuropathy (AIDP). AIDP is suspected to be a result of proinflammatory cytokines causing demyelination, specifically within the peripheral nervous system, in response to stress. Events that precede the onset of AIDP include gastrointestinal or respiratory infections, surgeries, and trauma. AIDP primarily presents with acute ascending paralysis and areflexia. Diagnosis of AIDP is confirmed by nerve conduction studies (NCSs) with electromyography (EMG) and lumbar puncture for cerebral spinal fluid (CSF) analysis. NCS/EMG is used to assess the peripheral nerve function, and serial examinations are often needed. CSF analysis is looking to identify albumino-cytological dissociation. Most commonly, intravenous immunoglobulin is used to treat AIDP with plasmapheresis being the second most common treatment. Prognosis is dependent on the remyelination process but overall is relatively good. More than 80% of patients can walk independently within one year of recovery.

Prognostic Significance of Serial Nerve Conduction in GB Syndrome.

We evaluated dynamic changes in neurophysiology of Guillain-Barré syndrome (GBS) at different time points and the role of demyelination and axonal burden in predicting outcome. Nerve conduction study (NCS) was done in 44 GBS patients at admission and at 1 and 3 months, and were categorized into acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), equivocal and in-excitable motor nerve (IMN). The demyelinating and axonal burden on motor NCS at admission, 1 and 3 months were computed and correlated with disability at 3 and 6 months. Disability was assessed using Clinical Grading Scale. Twenty-four (54.3%) had AIDP, 5 (11.4%) AMAN, 12 (27.3%) equivocal and 3 (6.8%) had IMN at admission. Maximum instability was noted in equivocal group; majority of whom became AIDP at three months. Neurophysiological subtypes at different time points did not correlate with 6 months disability, but demyelination burden at admission (r = -0.42; P = 0.005) and axonal burden at one month (r = 0.43; P = 0.04) correlated with six months disability. Inverse correlation of axonal burden at one and three months with disability suggests role of secondary axonal damage in predicting outcome. Repeat NCS at one month helps in categorizing GBS and also in prognostication.

COVID-19 Associated Guillain-Barré Section Syndrome- A Case Series

The outbreak of Coronavirus Disease-2019 (COVID-19) infection with associated severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) caused modified and compromised immune system that gave rise to various immune mediated disease. Various studies on both central and peripheral nervous system involvement has been reported. The common syndromes reported are meningoencephalitis, myelitis and Guillain-Barré syndrome (GBS) etc. This case series reports four cases (45 years old male, 35 years old female, 50 years old male and 65 years old male patients) presenting with the duration from onset of viral illness to neurologic manifestations ranging from 4 days to 60 days. One patient had a typical course of viral symptoms preceding GBS findings and two patient presented with GBS later. A patient was found to be IgG seropositive for SARS-CoV-2 and presented 2 months later of recovery from infection while one case had onset of weakness while having respiratory symptoms. These cases had Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) who presented with acute flaccid paralysis two to three weeks following COVID-19 infection. All the patients received Intravenous Immunoglobulin (IVIG) as treatment and showed significant improvement. It can be concluded that COVID-19 viral infection is probably related as a causal factor for immune mediated illness like GBS and early identification and treatment has good recovery.

Relationship between cerebrospinal fluid protein level and electrophysiologic abnormalities in the acute inflammatory demyelinating polyradiculoneuropathy variant of Guillain-Barré syndrome.

Objective: Guillain-Barré syndrome (GBS) is an autoimmune disease characterized by weakness in limbs or cranial nerve innervated muscles. Acute inflammatory demyelinating polyradiculoneuropathy (AIDP) is the most common variant. Electrophysiologic abnormalities and elevated cerebrospinal fluid (CSF) protein are frequently present in AIDP, but the relationship between these two parameters is not well known. We aimed to fill this gap by studying this relationship.Methods: This was a prospective cross-sectional study conducted for two years in the Department of Neurology, Dr. Ruth K. M. Pfau Civil Hospital, Karachi, Pakistan. All 90 adult patients with the AIDP variant of GBS were selected. Nerve conduction studies were performed to determine the degree of demyelination through the four electrophysiologic demyelination criteria. The CSF sample was sent to lab immediately after lumbar puncture. SPSS version 20.0 was used. The CSF protein level was measured with mean ±SD. Demyelination criteria were measured in frequency and percentages. Chi-square test was applied to a number of demyelination criteria and T-test/ANOVA was applied on mean CSF protein level.Results: We found a mean CSF protein of 37.41 mg/dl (±3.69) with one demyelination criterion, 81.87 mg/dl (±17.39) with two demyelination criteria, 119.75 mg/dl (±31.42) with three demyelination criteria, and 134.00 mg/dl (±42.87) with four demyelination criteria (P-value <0.001). Conclusion: This study demonstrates a significant relationship between CSF protein levels and degree of demyelination in the AIDP variant of GBS. This is an under-researched area in GBS and this study adds favorably to limited data in this regard.

Comparative Study between COVID Associated Guillan Barre Syndrome and Non-COVID Associated Guillan-Barré Syndrome

More than 12 million people have been infected with COVID-19 worldwide, with more than 500,000 deaths to date.1 Although COVID-19 research is rapidly evolving, new findings must be thoroughly scrutinised before any conclusions or treatment protocols are established or amended.2 Although COVID-19 is most usually associated with respiratory symptoms such as cough and dyspnea; it has recently been associated with a neurotropic presentation.3 Guillain-Barré Syndrome (GBS) is best described as an acute inflammatory polyradiculoneuropathy clinically characterized by areflexia and progressive weakness of arms and legs. Though, many rare variants of GBS have been described, the commonly observed subtypes such as Acute Motor Axonal Neuropathy (AMAN), Acute Motor Sensory Axonal Neuropathy (AMSAN) and Acute Inflammatory Demyelinating Polyradiculoneuropathy (AIDP) tend to fulfil the above-mentioned criteria.4 Recently, several case reports have suggested a relationship between the occurrence of Guillain-Barré syndrome (GBS) and a previous SARS-CoV-2 infection, which preceded the GBS onset by up to 4 weeks. Therefore, a post-infectious dysregulation of the immune system, triggered by SARS- CoV2, appears to be the most probable cause COVID-19 is a systemic disorder presenting typically with fever and respiratory symptoms but neurological manifestations such as acute cerebrovascular diseases, seizures, ageusia, anosmia meningitis, encephalitis and skeletal muscle involvement were soon reported.5 More recently, an increase in case reports of Guillain-Barré syndrome (GBS) in people infected with SARS-CoV-2 has prompted concerns about a possible link.