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Background Although rare, Guillain-Barré Syndrome (GBS) has a high economic burden, with consequences for families and society. This study aimed to estimate the total cost of GBS, per individual and per variant of the disease, as well as its effect on household income, from the perspective of patients. Methods This was a cost-of-illness study from the perspective of patients and their families, with a time horizon from disease onset to 6 mo after discharge. The total cost of GBS was estimated by bottom-up microcosting, considering direct and indirect costs. Results The median cost of GBS per individual was US$1635.5, with direct costs accounting for 64.3% of this amount. Among the variants analyzed, acute motor sensory axonal neuropathy (US$4660.1) and acute inflammatory demyelinating polyneuropathy (US$2017.0) exhibited the highest costs compared with acute motor axonal neuropathy (US$1635.5) and Miller Fisher Syndrome (US$1464.8). The costs involved compromise more than 20% of the household income of 22 (47.8%) patients. Conclusions This study demonstrated how costly GBS can be. It is hoped that decision-makers will analyze these results with a view to improving the structure of healthcare services.

Objective: To analyze patients with Guillain Barre Syndrome (GBS) requiring intensive care unit (ICU) admission, their course, response to treatment and outcome. Methods: A retrospective study conducted at ICU of The Children’s Hospital Lahore in 194 children with diagnosis of GBS from June 2018 to May 2020. Demographic profile, clinical features, treatment, duration of mechanical ventilation, length of ICU stay and outcome was noted. Results: Mean age of children was 5.95 ± 3.1 years with male predominance 136 (70 %). Major proportion were in range of 1-5 years 93(47.9%). Mean duration of ICU stay was 21.3±34.4 days. Out of 194 patients 125(64%) received IVIGs while 112 (57.7%) required mechanical ventilation because of rapidly progressive weakness within 24 hours of admission and those who remained on mechanical ventilation for longer period showed AMSAN (Acute motor sensory axonal neuropathy) type of GBS ( p value

A case of Miller Fisher and acute motor sensory axonal neuropathy (AMSAN) variant Guillain-Barré (MFS/AMSAN-GBS) overlap syndrome is presented. The neurological presentation of the overlap syndrome was preceded by an upper respiratory tract infection. Eventually, severe weakness of bulbar and limb muscles, areflexia, ophthalmoplegia, ataxia, and respiratory insufficiency developed. The electroneuromyography revealed symmetrical axonal polyneuropathy which was dominant in both upper limbs. Although a panel of anti-ganglioside antibodies including anti-GQ1b was negative, immediate treatment with intravenous immunoglobulin resulted in dramatic response.

Guillain-Barré syndrome (GBS) in an immune mediated disease that affects peripheral nerves with possible life-threatening complications. GBS has multiple subtypes including acute inflammatory demyelinating polyradiculoneuropathy (AIDP), acute motor axonal neuropathy (AMAN) and acute motor sensory axonal neuropathy (AMSAN), which can make GBS difficult to diagnose. GBS commonly presents after viral infections such as influenza virus, campylobacter jejuni, and zika virus. GBS commonly presents with a prolonged clinical course leading to increased morbidity among affected patients. It is not surprising that COVID-19 has been connected with multiple cases of GBS, which may alter the recovery course for several patients post-COVID. In this report, we present a case of 69-year-old-female who presented with progressive motor weakness and loss of sensation in her extremities after testing positive for antibodies to COVID-19 one-month prior to presentation. Her presentation and treatment of GBS in the setting of COVID-19 is an example of one of the many COVID-19 complications and sheds light on the prolonged recovery course that we may experience as clinicians in the wake of this pandemic.

International audience; Background: We describe a case of severe Guillain-Barre syndrome (GBS) associated with chronic active hepatitis C and mixed cryoglobulinemia (MC). To our knowledge, this association between GBS and hepatitis C virus (HCV) infection has been rarely reported.Case presentation: A 56-year-old man developed symmetrical muscle weakness in all extremities, areflexia and sensorial disorder followed by acute respiratory failure associated with chronic active hepatitis C, which was confirmed by the presence of anti-HCV antibodies in the serum and persistence of HCV RNA viral load for more than 6 months. Chronic hepatitis C was further complicated by type 3 MC. Electromyography showed peripheral nerve injury (mainly in axon). A severe acute motor sensory axonal neuropathy (AMSAN) was diagnosed. After treatment with intravenous immunoglobulin and plasma exchange followed by antiviral therapy by direct-acting antiviral agent, patient showed progressive recovery and was transferred 3 months after his first admission to a rehabilitation center.Conclusions: Our case reported a severe GBS associated with HCV infection and MC. EMG classified for the first time the subtype of GBS (severe AMSAN) correlated with severe clinical form. HCV infection should be screened in high-risk patients to prevent silent progression of the chronic hepatitis C and its potentially severe extra-hepatic manifestations.

Background Guillain–Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. Methods This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann–Whitney U tests, as appropriate. Results A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25–53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). Conclusion The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.

This study aimed to explore the correlation of elevated glucose levels in the blood and cerebrospinal fluid with the progression and short-term prognosis of Guillain-Barr�� syndrome (GBS). The medical records of 982 patients who were diagnosed with GBS in 31 representative tertiary hospitals, located in 14 provinces in southern China, were collected and retrospectively reviewed. Patients were grouped according to the levels of fasting plasma glucose (FPG) and cerebrospinal fluid (CSF) glucose, as well as the concentration of blood hemoglobinAlc (HbA1c). The Hughes grade scale was used to quantify functional outcomes. Compared to patients with normal FPG and CSF glucose levels, those in the high FPG and high CSF glucose groups were characterized by a higher proportion of severe patients (HFGS ��� 3) at admission (58.8 vs. 73.1, P = 0.000; 57.6 vs. 71.2, P = 0.000), at nadir (67.4 vs. 83.0, P = 0.000; 66.2 vs. 80.4, P = 0.000), and at discharge (29.8 vs. 46.3, P = 0.000; 26.4 vs. 45.0, P = 0.000). Patients in the high HbA1c group also had more severe disability at admission (74.6 vs. 56.1, P = 0.005) and at nadir (80.3 vs. 64.3, P = 0.012) compared to the normal HbA1c group. Moreover, elevated levels of FPG and CSF glucose were significantly correlated with more severe disability at admission, at nadir, and at discharge. The present study showed that elevated glucose levels in the blood and cerebrospinal fluid were associated with the severity and short-term prognosis of GBS. chicTR-RRc-17,014,152. GBS, Guillain-Barr�� syndrome; FPG, fasting plasma glucose; CSF, cerebrospinal fluid; HFGS, Hughes Functional Grading Scale; HbA1c, hemoglobin A1c. DM, diabetes mellitus; NCS, nerve conduction study; AIDP, acute inflammatory demyelinating polyneuropathy; AMAN, acute motor axonal neuropathy; AMSAN, acute motor sensory axonal neuropathy; MV, mechanical ventilation.

Background: The clinical spectrum of coronavirus disease 2019 (COVID-19), due to severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection, may be quite wide, including neurological symptoms. Among them, para-infectious or post-infectious neurological syndromes (PINS), caused by an inflammatory response against the central and/or peripheral nervous system, have been reported. The aim of this paper is to illustrate the functional and neurophysiological recovery in a series of subjects with COVID-19-related PINS who underwent intensive neurorehabilitation.Materials and Methods: Five patients with PINS associated with COVID-19 were evaluated at baseline and followed up for 6 months. Three of them had polyradiculoneuropathy and two patients had myelitis. The onset of the neurological syndromes was temporally associated with the SARS-CoV-2 infection. After completing the acute neurological treatments in the intensive care unit, patients underwent a personalized multidisciplinary rehabilitation program. An in-depth clinical, functional, and electrophysiological assessment was carried out at baseline and at 3- and 6-month follow-ups.Results: Among patients with polyradiculoneuropathy, the electrophysiological evaluation at baseline disclosed an acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in two patients and an acute motor and sensory axonal neuropathy (AMSAN) in the third patient. At follow-up, the electrophysiological features improved in one subject with AIDP and were stable in the remaining two cases. The functional assessment after neurorehabilitation showed global recovery and full independence in walking and in activities of daily life in one patient and mild improvement in the other two cases. Of the two subjects with myelitis, the baseline electrophysiological examination showed a prolonged central motor conduction time, which returned to normal in one patient, whereas it improved but remained pathological in the other patient at follow-up. The neurorehabilitation led to a substantial functional improvement in both subjects.Discussion and Conclusions: This is the first study to describe clinical and electrophysiological aspects along with medium-term outcome in patients with COVID-19-related neurological manifestations who underwent an intensive rehabilitation program. The functional outcome following neurorehabilitation in patients with PINS related to SARS-CoV-2 infection is variable. In our small case series, subjects with polyradiculoneuropathy had a poorer recovery compared to patients with myelitis. The clinical course largely paralleled the follow-up electrophysiological findings.

Acute Motor Sensory Axonal Neuropathy (AMSAN) is a rare and severe variant of Guillain-Barré syndrome (GBS) that has a prolonged recovery course. GBS is often suspected due to ascending muscle weakness, sensation difficulties, respiratory compromise, and antecedent diarrhea. The diagnosis of GBS is supported by cerebrospinal fluid analysis showing albuminocytologic dissociation. Electromyogram and nerve conduction study confirm the diagnosis and allow for further classification by variant. Treatment involves either IV immune globulins or plasmapheresis, and patients typically recover. However, depending on the variant and severity, patients may ultimately require prolonged mechanical ventilation with tracheostomy. In these cases, they may continue to have persistent muscle and sensation abnormalities requiring long-term care. We present a unique case of a 38-year-old female patient with decade-long use of lithium for bipolar disorder that presented with acute lithium toxicity. Though she was ultimately diagnosed with AMSAN, the Syndrome of Irreversible Lithium-Effectuated Neurotoxicity (SILENT) may have also contributed to her persistent neurological sequelae.

Tuberculosis being a global pandemic causes an array of neurological presentations ranging from tuberculoma, meningitis, radiculomyelitis, brain abscess, and so on.Association of Guillain-Barre syndrome with tuberculosis has been reported five to six times in the past. The authors report a case of a young female with tubercular meningitis on antitubercular therapy and steroids who went on to develop acute areflexic quadriparesis and diagnosed as a case of acute motor sensory axonal neuropathy variety of acute inflammatory demyelinating polyneuropathy (AIDP) who responded positively to plasmapheresis. The authors present the first case of an association between tubercular meningitis and subsequent development of AIDP.

Background: Guillain-Barré syndrome (GBS) is the commonest cause of acute flaccid paralysis worldwide, with an incidence of 0.6-4 per 100.000 inhabitants per year. It affects all age groups and carries an incapacity burden of up to 20%. The aims of this study were to evaluate the demographic and clinical presentation, hospital care and outcome with different modalities of treatment in adult patients suffering from GBSadmittedina tertiary care hospital in Dhaka.Methods: This observational study was done in the Neurology department of a tertiary care hospital in Dhaka.The studystarted in July-2011 and the first fiftypatients suffering from GBSwereenrolledconsecutively. Subjects included in this study were>18years of age.All the patients were interviewed and clinically examined. Relevant data were documented in a structured questionnaire. Nerve conduction Study (NCS) and Cerebrospinal fluid (CSF) study results were also documented. All of patients were watched for respiratory insufficiency and those who developed respiratory paralysis were transferred to ICU for respiratory assistance. According to clinical and electrophysiological criteria, the patients were classified into different variants of GBS. Patients were treated with intravenous immunoglobulin (IVIg), plasmapheresis, supportive care and outcome was observed. The patient who recovered and were discharged were later followed up on out –patientbasis for one month Results: The commonest age group affected was 31-50 yrs with male preponderance .The mean age of study subjects was 46years. In most of the patient their weakness progressed for 3-5 days. Clinical evidence of a preceding infection was present inforty-six percent of the patientsbutcausative organism was not identified.Clinical presentations were quadriparesis in 84 % of patients, cranial nerve palsy in 48% of patients. Severe respiratory involvement requiring mechanical ventilation developed in 10% of patientsafter admission. CSF study was done and only10 subjects displayed typical findings of albumino cytological dissociation. NCS wasabnormal in all study subjects and revealed Acute Motor Sensory Axonal Neuropathy (AMSAN) in58%of study population. Among the fifty patients 23received plasmapheresis and another 9 receivedstandard IVIg therapy. Of these patients 20(88%) and 7 (77.6%) patients made significant recovery, respectively. Residual neurodeficit persisted in 6 of the remaining patients who received treatment in the form of only physiotherapy at the end of one month follow-up.Conclusion: Rapidly progressive quadriparesis with cranial nerve involvement was the commonest presentation. In this group of patients NCS proved to be a sensitive study for early diagnosis of patients with GBS in both diabetic and non-diabetic population. The axonal variety of GBS was more common than the demyelinating type. Although not statistically significant, the better response to plasmapheresis seemedencouraging. Again the patients who received IVIg or plasmapheresis early in the course of disease had faster recovery as compared to patients who received only supportive line of treatment.Birdem Med J 2017; 7(1): 38-42

Background Guillain–Barré syndrome (GBS) is an immune-mediated disorder of the nervous system that shows acute or subacute onset. It is also known as Landry's paralysis. It is characterized by muscle weakness of legs and arms, limb paresthesias, and total or relative areflexia. Acute motor sensory axonal neuropathy (AMSAN) is a distinct subtype of GBS. It is not only a rare but severe variant that involves axonal degeneration in motor and sensory nerve fibers and has a prolonged recovery course. Case description A 60-year-old male presented to the emergency department having complaints of weakness, numbness, and tingling sensation in feet for the last fortnight, which ascended gradually towards the calves. He was observed to have a sensory disorder in hands, but not flaccid paralysis. The patient history and nerve conduction studies were indicative of AMSAN variant of GBS. Discussion In patients of AMSAN, the reduction of sural nerve amplitude is more pronounced as compared to acute inflammatory demyelinating polyneuropathy (AIDP) patients. In case of our patient, the electrophysiological feature indicated a more than 50% decrease in SNAP. A marked reduction in sensory nerve action potential and compound muscle action potential with only slightly decreased conduction velocities is a requirement for the diagnosis of axonal neuropathies, which is the trait seen in the reported case. How to cite this article Saggar SK, Thaman RG, Mohan G. Acute Motor Sensory Axonal Neuropathy: A Variant of Guillain–Barré Syndrome—A Rare Case Report. MEI's Curr Trends Diagn Treat 2020;4(2):110–111.

Intramedullary Spinal Cord Metastases (ISCMs) are rare, especially in squamous non-small cell lung cancer simultaneous with Acute Motor and Sensory Axonal Neuropathy (AMSAN); diagnosing these phenomena may be challenging. We report a 56-year-old man presenting with rapidly symmetrical progressive ascending weakness and paresthesia, especially in lower extremities, urinary and fecal incontinence, without any pain or respiratory symptoms. Thoracic spine MRI with and without gadolinium revealed an enhancing intramedullary lesion at the T3-T5 level. Chest CT discovered subpleural pulmonary lesion. The result lung biopsy was consistent with poorly differentiated squamous cell carcinoma, and electrodiagnosis noted severe axonal loss in motor and sensory studies with significant membrane instability and neurogenic findings in electromyography. After 8 months, the patient received just one cycle of chemotherapy and died 6 months later. AMSAN was associated with ISCM of nonsmall cell lung cancer in this case, who has not been previously reported in the literature.

An 85-year-old Japanese female was admitted with sudden onset of quadriparesis with areflexia. Preceding infection was not present. IgG anti-GT1b antibodies were prominently positive in serum. Nerve conduction study results suggested Guillain-Barre syndrome (GBS) classified as acute motor sensory axonal neuropathy (AMSAN). While intravenous immunoglobulin (IVIg) was started, bulbar palsy and respiratory failure progressed and the condition deteriorated. Although mechanical ventilation was required, second IVIg course led to gradual improvement of quadriparesis and bulbar palsy. In the present case with elderly-onset disease, the levels of anti-GT1b antibodies were elevated, which is relatively rare in GBS. It was suggested that anti-GT1b antibodies may be related to the development of axonal GBS with bulbar palsy.

Systemic lupus erythematosus (SLE) is an autoimmune disease that involves multiple organs and is generally treated by immunosuppressive agents. Acute motor-sensory axonal neuropathy (AMSAN) is a variant of Guillain-Barré syndrome. The standard therapies are intravenous immunoglobulin or plasmapheresis. An association between AMSAN and SLE is rarely reported. Herein, we describe a case of a 72-year-old man who presented with rapidly progressive paraparesis, dysesthesia, and joint pain with morning stiffness. Initially, he was diagnosed with AMSAN. Intravenous immunoglobulin was given without significant improvement. Subsequent studies indicated the diagnosis of SLE. Therefore, the patient was treated with intravenous methylprednisolone, cyclophosphamide, and then plasmapheresis. At 3 months, he improved from bedridden to wheelchair-bound. Our case demonstrates AMSAN as a rare initial manifestation that can lead to significant disability.

Background Guillain–Barré syndrome (GBS) is a monophasic disease characterized by acute polyradiculoneuropathy. Aim This study investigated cardiovascular complications in patients with GBS and their relationship with outcomes. Design and methods We included 96 patients, who were diagnosed with GBS according to Brighton case definitions. All enrolled patients were evaluated according to a predetermined algorithm, which included nerve conduction studies, cerebrospinal fluid analysis, electrocardiography, 2D echo, cardiac markers and autonomic function testing. Results We enrolled a total of 96 patients. The mean age of patients was 35.75 ± 17.66 years. Furthermore, 54.2% of patients developed cardiovascular complications, of which changes in electrocardiography (ECG) findings (50%), hypertension (28.12%), labile hypertension (12.5), tachycardia (26.04), bradycardia (13.54%) and a fluctuating heart rate (HR) (11.46) were common. Other cardiovascular complications seen in GBS patients were increased pro-BNP (26.04%), raised troponin T levels (3.12%), acute coronary syndrome (2.08%), heart failure (2.08%) and abnormal 2D echo findings (8.33%). The results of the univariate analysis revealed that a history of preceding infection, a Medical Research Council sum score, neck muscle weakness, facial nerve involvement, bulbar involvement, respiratory failure, cardiovascular complications, autonomic dysfunction, acute motor sensory axonal neuropathy subtype and baseline Hughes score were significantly (P Conclusion A considerable number of patients with GBS developed cardiovascular complications and it needs attention.

We report three females who developed Guillain–Barre Syndrome in postpartum period (within 6 weeks of delivery) and were admitted in the Neurological Rehabilitation Department for rehabilitation after the initial diagnosis and treatment in the Department of Neurology. The first case, axonal variant (acute motor axonal neuropathy [AMAN]) had worst presentation at the time of admission, recovered well by the time of discharge. The second case, acute motor sensory axonal neuropathy variant and the third case, AMAN variant presented at the late postpartum period. Medical treatment was sought much later due to various reasons and both the patients had an incomplete recovery at discharge. Apart from their presentations, rehabilitation management is also discussed in some detail.

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

Background We describe a case of Guillain-Barre syndrome (GBS) associated with hemorrhagic fever with renal syndrome. To our knowledge, only five cases of GBS associated with Hantavirus infection have been reported so far. Case presentations A 62-year-old man presented intermittent fever, chill and oliguria. According to remarkable leukocytosis, atypical lymphocytes, thrombocytopenia and former dwelling in hemorrhagic fever-endemic area, he was suspected as hemorrhagic fever with renal syndromeand certified with positive Hantavirus IgG. Later, the patient had symmetrical flaccid paralysis of all extremities. Electromyography showed peripheral nerve injury (mainly in axon). The patient was diagnosed as having acute motor sensory axonal neuropathy (AMSAN). After immunoglobulin infusion, patient showed progressive recovery and was transferred 3 weeks after his first admission to a rehabilitation center. Conclusions Our case was the 6th reported case of GBS associated with hemorrhagic fever with renal syndrome. Moreover, we for the first time classified the subtype of GBS (AMSAN) based on the electrophysiology characteristics. GBS should be suspected in patients who are already diagnosed as hemorrhagic fever with renal syndrome when delayed symmetrical limb paralysis occurs. Until recent now, GBS was only reported in hemorrhagic fever patients in Europe and Asia, which termed as hemorrhagic fever with renal syndrome.

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days.

Diagnosing concomitant transverse myelitis (TM) and Guillain-Barré syndrome (GBS) can be challenging. We report a case of an elderly patient presenting with acute sensory and motor disturbances in the four limbs, associated with urinary retention, ophthalmoparesis, facial weakness, and dysarthria. Electrodiagnostic studies were consistent with acute motor sensory axonal neuropathy (AMSAN), and imaging showed a longitudinally extensive tumefactive contrast-enhancing hyperintense spinal cord lesion extending from T6 to the cone. Concomitant AMSAN and TM have not been previously reported in the elderly. Comorbid TM and other GBS variants have been previously reported. Intravenous methylprednisolone, plasma exchange, cyclophosphamide, or combination therapies are usually used, although there are no randomized controlled studies regarding treatment choices.

Background Guillain–Barre syndrome (GBS) is a monophasic disease, characterized by acute, polyradiculoneuropathy. The study was aimed to asses cardiovascular complications in GBS and its relationship with outcome. Material and methods 101 patients were included in the study according to Brighton case definition. All enrolled patients were evaluated as per predetermined algorithm, which included Nerve conduction studies, Cerebrospinal fluid analysis, routine investigations, ECG, 2D Echo, cardiac markers and autonomic function testing. The study protocol was approved by institutional ethical committee. Results The 96 patients were enrolled in the study. Mean age of patients was 35.75±17.66. 60.4% males and 39.6% were females. Cardiovascular complications were present in 54.2% of patients. The electrocardiogram changes were present in 50%, hypertension in 28.12%, tachycardia in 26.04%, bradycardia in 13.54% and 11.46% patients had fluctuating heart rate. Raised Pro BNP(b-type natriuretic peptide) and Troponin T were present in 26.04% and 3.12% patients respectively. Acute coronary syndrome occurred in 2.08% and heart failure in 2.08% patients. Echo abnormality was found in 8.33% of patients. On univariate analysis, history of preceding infection, Medical Research Counsel sum score, neck muscles weakness, facial nerve involvement, bulbar involvement, respiratory failure, cardiovascular complications, autonomic dysfunction, Nerve Conduction Study (Acute Motor Sensory Axonal Neuropathy), baseline Hughes score, were significantly (P Conclusion Fair number of patients of GBS developed cardiovascular complications and these complications were associated with poor clinical outcome.

Guillain–Barre syndrome is characterized by acute progressive weakness, areflexia and maximal motor disability that occur within 4 weeks of onset. Its clinical and pathological spectra range extends from classical acute inflammatory demyelinating polyneuropathy to axonal variants with (acute motor sensory axonal neuropathy) or without (acute motor axonal neuropathy) sensory involvement. Moreover, several clinical variants (Fisher syndrome, ataxic Guillain–Barre syndrome and pharyngeal–cervical–brachial weakness) have been proposed. The association of clinical characteristics of Guillain–Barre syndrome with specific infections and the presence of antiganglioside antibodies also has been reported. Guillain–Barre syndrome now is recognized as a heterogeneous disorder with various clinical manifestations and recent neurophysiological and pathological findings have led to its reclassification. This review summarizes current concepts about the clinical spectrum of Guillain–Barre syndrome and its variants and ab...

This paper reports a case of a Jamaican young woman who experienced flaccid quadriparesis and bulbar weakness over a three-week period after a gastrointestinal illness. Nerve conduction studies confirmed an axonal type neuropathy consistent with the acute motor-sensory axonal neuropathy variant of the Guillain-Barre syndrome. Recovery, although evident, was slow and was augmented after a course of intravenous immunoglobulin. The patient was discharged from hospital after three months but was re-admitted one week later and eventually succumbed to complications of the illness. This case serves as a reminder that Guillain-Barre syndrome is now the most common cause of acute flaccid paralysis and should be considered early in all patients presenting with flaccid quadriparesis.

Systemic lupus erythematosus is a multisystemic, autoimmune, inflammatory disorder predominantly affecting young females. Its onset may be abrupt or insidious, presenting with a broad range of clinical and immunological features. We report an unusual case of elderly-onset systemic lupus erythematosus in a woman initially diagnosed with discoid lupus, and subsequently admitted to hospital due to a progressive psycho-motor deficit. Electrophysiological measurements suggested a diagnosis of acute motor sensory axonal neuropathy. Unusual clinical features and negative serology led to diagnostic uncertainty. This case report offers information on the course of the disease through the entire chain of the health care delivery (from primary to tertiary). Despite the efforts of the hospital staff, it was not possible to save the life of the woman.

Background: Guillain-Barre Syndrome (GBS) is an acute monophasic demyelinating neuropathy characterized by progressive motor weakness of limbs with areflexia. Aim: To study the clinical pattern and outcome of children with Guillain-Barre syndrome. Materials and Methods: It was a cross-sectional study conducted in a pediatric unit of tertiary care hospital over a period of 18 months. We assessed the clinical manifestations, results of electro-diagnostic tests, functional status, treatment instituted and outcome of 20 children diagnosed with GBS. Results: Of the 20 (male to female ratio = 2.3:1) children studied, all had motor weakness, 5 (25%) had sensory loss, 4 (20 %) had cranial nerve palsies and 4 (20%) had autonomic disturbances. Respiratory paralysis was found in 7 (35%) children requiring assisted ventilation. Antecedent illness preceding GBS was recorded in 50% children. The GBS subtype distribution as per electrodiagnostic studies was as follows: acute motor axonal neuropathy (AMAN) in 7 (38.9%), acute motor sensory axonal neuropathy (AMSAN) in 4 (22.2%), acute inflammatory demyelinating polyradiculoneuropathy (AIDP) in 4 (22.2%) and both axonal and demyelinating neuropathy in 3 (16.7%). Intravenous immunoglobulins (IVIG) constituted the treatment given in majority of the patients. Plasmapharesis was performed in one child in view of poor response to IVIG. Complete recovery was observed in 14 children and the remaining 3 children experienced only incomplete recovery. Conclusion: Male preponderance and presence of antecedent illness in a majority of subjects was observed in our study. Regardless of the severity of illness at admission and electrophysiological subtypes, a majority achieved full recovery. Intravenous Immunoglobulin and supportive care form the cornerstone of management in childhood GBS.

We performed electrophysiological and serological testing within 15 days of symptom onset on 369 patients with Guillain-Barre Syndrome (GBS) enrolled in a trial comparing plasma exchange, intravenous immunoglobulin, and both treatments. Patients were classified into five groups by motor nerve conduction criteria; 69% were demyelinating, 3% axonal, 3% inexcitable, 2% normal, and 23% equivocal. Six of 10 (60%) patients with axonal neurophysiology had had a preceding diarrheal illness compared with 71 of 359 (20%) in other groups. Antiganglioside GM1 antibodies were present in a higher proportion of patients with axonal physiology or inexcitable nerves than other patients. The number dead or unable to walk unaided at 48 weeks was greater in the group with initially inexcitable nerves (6 of 12, 50%) compared with the rest (52 of 357, 15%), but was not significantly different between the axonal (1 of 10, 10%) and demyelinating (44 of 254, 17%) groups. Sensory action potentials and clinical sensory examination were both normal in 53 of 342 (16%) patients, and these "pure motor GBS" patients were more likely than other GBS patients to have IgG antiganglioside GM1 antibodies and to have had preceding diarrhea but had a similar outcome. The axonal group was more likely than other groups to have normal sensory action potentials. The outcomes in response to the three treatments did not differ in any subgroup (including patients with pure motor GBS or preceding diarrhea) or any neurophysiological category.

Guillain-Barre syndrome (GBS) is one of the classic diseases in neurology with an easily recognized clinical pattern of acral paresthesias, ascending generalized weakness, and areflexia. This syndrome has typical presentations and several variants, as well as variable electrodiagnostic features, and treatment modalities including the selection of immunomodulating agents. This chapter reviews the current views of the pathogenesis and clinical presentations of GBS, its electrodiagnostic features, differential diagnosis, treatment and prognosis. A review of GBS variants follows the discussions of classical GBS.

BACKGROUND: Guillain-Barre syndrome (GBS) is a well known entity that has many infectious agents reported as antecedent events. The spectrum of GBS includes acute inflammatory demyelinating polyneuropathy (AIDP), acute motor axonal neuropathy (AMAN), acute motor sensory axonal neuropathy (AMSAN), and some other variants like Miller-Fisher syndrome (MFS). ----- METHODS: Patient with AMAN variant of GBS after severe bilateral pneumonia and ARDS due to the novel pandemic H1N1 influenza A virus is presented. ----- RESULTS: 28-year-old white female was admitted to our Intensive Care Unit during the influenza pandemic because of severe ARDS due to bilateral pneumonia. The course of the disease was complicated with the new onset tetraplegia due to the AMAN variant of GBS. Treatment with plasma exchange was conducted and the patient had satisfactory recovery. ----- CONCLUSION: We report a case of AMAN variant of GBS associated with proven H1N1 influenza A infection. This virus has not been reported previously as the agent of antecedent infection that induced this disorder. Risk factors for other causes of ICU neuromuscular weakness are usually present in the ICU patients and should not be the reason for reluctance in active quest for GBS. Once the diagnosis of GBS is established or suspected the treatment with plasma exchange or intravenous immune globulin is indicated.

Background: The diagnostic yield of various neurophysiological criteria may vary in different subforms of Guillain-Barre syndrome (GBS), whose prevalence varies in different geographical areas. Aims: To evaluate the sensitivity of various neurophysiological criteria in different clinical subtypes of GBS, and their relationship with severity, duration and outcome. Methods: Consecutive patients with GBS underwent detailed clinical evaluation. Severity was graded on a scale from 0 to 10. Motor and sensory nerve conductions and F wave studies were performed. The diagnostic sensitivity of Albers et al (set 1), Cornblath (set 2), Ho et al (set 3), Dutch GBS study group (set 4), Italian GBS study group (set 5) and Albers and Kelly (set 6) criteria were evaluated and correlated with clinical subtypes of GBS, duration, severity and outcome. Results: There were 51 patients. Mean disability was 6.8; 34 patients were bedridden and five needed a ventilator. Clinical presentation was pure motor in 31, motorsensory in 18 and pure sensory in two patients. The sensitivity of nerve conduction study in the diagnosis of GBS was highest in set 1 (88.2%) followed by set 3 (86.3%) and set 4 (82.4%) and lowest in set 2 (39.2%). The diagnostic yield of sets 1, 3 and 4 were also higher than sets 2, 5 and 6 in different clinical subtypes of GBS. As per Ho et al, patients could be categorised into acute inflammatory demyelinating polyradiculoneuropathy (44 (86.3%)), acute motor axonal neuropathy (4 (7.8%)) and acute motor sensory axonal neuropathy (3 (5.9%)). One (2%) patient died, 22.4% had complete, 57.1% partial and 18.4% poor recovery at 3 months. Outcome was related to severity of illness and compound muscle action potential (CMAP) amplitude. Conclusion: The sensitivity of different neurophysiological criteria in the diagnosis of Indian GBS patients varied from 39.2% to 88.2%. The outcome was related to severity of illness and CMAP amplitude.

Guillain Barre Syndrome (GBS) is an acute inflammatory demyelinating polyradiculoneuropathy of autoimmune nature, characterized by rapidly progressive, symmetric weakness and areflexia. Acute motor sensory axonal neuropathy is a variant of GBS with motor paralysis, hyporeflexia/areflexia, and sensory loss. Alcohol produces central nervous system toxicity as its principal side effects. Alcoholic neuropathy is a mixed sensory and motor disorder affecting large and small fibers to varying degrees. In contrast to the classic, slowly progressive polyneuropathy in alcoholic patients, acute forms, clinically mimicking GBS, are rare. This is a case of a 37 year old chronic alcoholic lady presenting with subacute onset weakness of both lower limbs whose laboratory data indicate neuropathy of acute motor sensory axonal pattern. We emphasize that acute alcoholic neuropathy has to be distinguished from GBS and other forms of acute polyneuropathy by using clinical, laboratory, and electrophysiological data for therapeutic and prognostic concerns.

Background: The Guillain Barre' syndrome (GBS) is a heterogeneous disease with various subtypes, the prevalence of which would depend on the geographic region. Recognition of these subtypes is of clinical importance since each subtype has an independent pathogenesis and different type of pathology and prognosis. Objectives: To study the various subtypes of GBS using the various published electrophysiological criteria. Design: Retrospective descriptive study. Materials and Methods: In a tertiary care hospital setting, the study compared the various published criteria for demyelination in GBS. The charts of 115 consecutive patients referred for electrodiagnostic evaluation to the Electromyography laboratory between July 2000 and June 2006 were reviewed. Results: Of the 115 patients, 51 (44.4%) patients had axonal forms of GBS and 44 (38.2%) patients had acute inflammatory demyelinating polyradiculoneuropathy (AIDP). Applying the various published criteria, the number of patients categorized under the AIDP subtype ranged between 23.4% and 67.2%. Conclusion: In this study 44% patients had axonal forms of the disease, 38.2% patients had AIDP subtype and 17% remained unclassified. The most sensitive criteria to identify AIDP were the criteria proposed by Albers and colleagues and the Dutch group.

Acute peripheral nervous system diseases leading to paralysis in children are rare in Europe and the USA, whereas epidemics of a Guillain-Barré-like syndrome occur annually among children in rural parts of northern China. To clarify the features of this disorder 36 patients, aged 15 months to 37 years (median 7) with this syndrome were investigated; 91% were from rural areas. In 47%, a prodromal illness was reported in the preceding 4 weeks. Leg weakness and resistance to neck flexion were the earliest symptoms. The weakness ascended rapidly and symmetrically to affect the arms and respiratory muscles, with maximum weakness occurring a mean of 6 days after onset of weakness. Bulbar weakness occurred in 61% of patients, but only 1 had extraocular paresis. Respiratory assistance was needed by 31% of patients. Tendon reflexes were lost as weakness developed. 42% of patients had raised concentrations of protein in the cerebrospinal fluid, and the mean cell count was 3 cells/microliters (range 0-12/microliters). Electrodiagnostic studies in 22 patients showed severe reductions in motor evoked amplitudes from distal stimulation. Sensory action potentials were normal. Electromyography revealed denervation potentials in limb muscles. The distinctive epidemiological, clinical, and neurophysiological characteristics of this illness suggest that the disorder is different from both Guillain-Barré syndrome and poliomyelitis. The neurophysiological findings support the hypothesis that the disorder is a reversible distal motor nerve terminal or anterior horn cell lesion.