Increased mortality following Guillain-Barré syndrome: A population-based cohort study.

BACKGROUND AND PURPOSE: Guillain-Barré syndrome (GBS) may be fatal in the acute phase but also affect long-term prognosis due to irreversible sequelae and secondary medical complications. We determined the short-term, intermediate, and long-term mortality of GBS compared to the general population. METHODS: Individual-level data from nationwide registries were linked in this matched cohort study of all first-time hospital-diagnosed GBS patients in Denmark between 1987 and 2016 and 10 individuals from the general population, matched on age, sex, and index date. We used Cox regression analysis to calculate matched mortality hazard ratios (HRs) following GBS, assessing short-term (0-6 months), intermediate (>6 months-4 years), and long-term (>4 years) mortality. RESULTS: We identified 2414 patients with GBS and 23,909 matched individuals from the general population. Short-term mortality was 4.8% (95% confidence interval [CI] = 4.0-5.8) and 0.8% (95% CI = 0.7-0.9) for GBS patients and general population members, respectively, resulting in an HR of 6.6 (95% CI = 4.0-5.8). Intermediate mortality was 7.6% (95% CI = 6.5-8.9), compared with 5.8% (95% CI = 5.5-6.1) for general population members, corresponding to an HR of 1.5 (95% CI = 1.3-1.8). After the first 4 years, long-term mortality showed similar results for GBS patients and general population members (HR = 1.1, 95% CI = 0.9-1.2). CONCLUSIONS: During the first 6 months after GBS hospital admission, GBS was associated with a 6.6-fold increased mortality as compared with the background population of the same age. Mortality remained increased for approximately 4 years following GBS, and then leveled off to a similar long-term mortality rate.

Triggers of Guillain-Barré Syndrome: Campylobacter jejuni Predominates.

Guillain-Barré syndrome (GBS) is a rare immune-mediated acute polyradiculo-neuropathy that typically develops after a previous gastrointestinal or respiratory infection. This narrative overview aims to summarise and discuss current knowledge and previous evidence regarding triggers and pathophysiology of GBS. A systematic search of the literature was carried out using suitable search terms. The most common subtypes of GBS are acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor axonal neuropathy (AMAN). The most common triggers of GBS, in three quarters of cases, are previous infections. The most common infectious agents that cause GBS include Campylobacter jejuni (C. jejuni), Mycoplasma pneumoniae, and cytomegalovirus. C. jejuni is responsible for about a third of GBS cases. GBS due to C. jejuni is usually more severe than that due to other causes. Clinical presentation of GBS is highly dependent on the structure of pathogenic lipo-oligosaccharides (LOS) that trigger the innate immune system via Toll-like-receptor (TLR)-4 signalling. AIDP is due to demyelination, whereas in AMAN, structures of the axolemma are affected in the nodal or inter-nodal space. In conclusion, GBS is a neuro-immunological disorder caused by autoantibodies against components of the myelin sheath or axolemma. Molecular mimicry between surface structures of pathogens and components of myelin or the axon is one scenario that may explain the pathophysiology of GBS.

Neuro-COVID Requires Comprehensive Work-up.

Finsterer J, Scorza FA. Neuro-COVID Requires Comprehensive Work-up. Indian J Crit Care Med 2021;25(8):956-957.

Ocrelizumab in a case of refractory chronic inflammatory demyelinating polyneuropathy with anti-rituximab antibodies.

Rituximab (RTX), a chimeric anti-CD20 monoclonal antibody, has demonstrated good efficacy as treatment in patients with resistant chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), but it is highly immunogenic due to its structure. Ocrelizumab (OCR) is a humanized anti-CD20 antibody, with higher tolerability and a lower immunogenic profile compared to RTX. We present a case of refractory CIDP effectively treated with OCR, switched from RTX after the development of anti-drug antibodies. A 25-year-old man was admitted to our clinic for the onset of distal upper and lower limb weakness and numbness, with electrodiagnostic criteria of CIDP. After several attempted standard CIDP treatments, RTX was introduced due to poor control of clinical relapses. Unfortunately, the patient developed a high anti-drug antibody titer after RTX infusion, with no control of disease. OCR was started as an off-label treatment, resulting in partial recovery from the last recurrence and achieving good prevention of new relapses with no adverse events. We suggest that OCR should be considered as another therapeutic option in refractory CIDP. In the literature, this is the first case of CIDP treated with OCR, demonstrating good efficacy for its anti-CD20 effect and better tolerability because of its lower immunogenicity.

[Small Tick - Big Danger].

INTRODUCTION: We report a 23-year-old patient who presented to the general practitioner due to persisting headache, fever, and vomiting. In the further course, a tetraparesis dominated on the right side, dysphagia and dysarthria occurred, and a general tonic-clonic seizure. Further examinations confirmed tick-borne encephalomyelitis as well as polyradiculitis. After two months of rehabilitation, neuropsychological as well as focal-neurological deficits persisted in the unvaccinated patient.

Asynchronous combined central and peripheral demyelination (CCPD) in a girl with anti-MOG positivity: A case report and review of the literature.

The occurrence of combined central and peripheral demyelination (CCPD) is rare, data are limited to small case and cohort studies, mainly concerning adults. In few patients positivity to anti MOG antibody is reported, thus widening the spectrum of anti-MOG associated disorders (MOGAD). We describe a 7-year-old girl with optic neuritis followed 8 years later by peripheral demyelination, with fluctuating anti-MOG antibody positivity at cell-based assay. From the review of the literature, MOGAD-CCPD appear very rare in childhood, especially with asynchronous course. Clinicians should keep this possibility in mind to better define diagnosis in atypical demyelination syndromes, with therapeutical implications.

Isolated neurobrucellosis-characteristic clinical and laboratory features.

To report various neurological syndromes, CSF findings, imaging and diagnostic methods used in neurobrucellosis patients admitted in our Neurology department over a period of 6 years. Case records of patients admitted to our department from August 2014 to May 2020 were searched for neurobrucellosis and data were obtained. A total of 19 patients were diagnosed as neurobrucellosis over a period of 6 years. Ten patients had chronic meningitis, five had VIII nerve involvement, one had optic neuritis, two had acute meningitis, one had subacute meningitis, four had myelopathy, five had polyradiculitis and two had spondylodiscitis. CSF was abnormal in 17 patients. Neutrophilic pleocytosis was seen in 12 patients who included nine patients with chronic symptomatology. Brain imaging was abnormal in three chronic meningitis patients. One had diffuse meningeal enhancement, another had hydrocephalus while the third patient had meningeal enhancement with basal exudates and contrast enhancement of bilateral VIII nerve. One of the patients of acute meningitis had hydrocephalus while the other one had bilateral T2/FLAIR hyperintensities with enhancement of meninges and leptomeningeal vessels. Elevated antibody titers only in serum was seen in six patients while elevated antibody titers only in CSF was seen in seven patients. Four patients had elevated antibody titers in both serum and CSF. CSF culture was positive in three patients. Neurobrucellosis is a rare clinical complication of brucellosis but may pose a problem in diagnosis as it can mimic tuberculosis. Involvement of VIII nerve and neurophilic pleocytosis in CSF despite chronic symptomatology can be diagnostic clues favoring neurobrucellosis.

SARS-CoV-2 vaccinations reduce the prevalence of post-COVID Guillain-Barre syndrome.

Guillain-Barre Syndrome (GBS) has been repeatedly reported as a neurological complication of COVID-19 (post-COVID GBS [PCG]). Whether the introduction of SARS-CoV-2 vaccines reduced the prevalence of PCG is unknown. This narrative review aimed to compare the number of published PCG cases between the second half of 2020 (no vaccination available) with those of the first half of 2021 (vaccination available). A total of 124 articles reported 300 patients with PCG between January 2020 and June 2021. The ages ranged from 7 to 94y. There was male dominance. The latency between the onset of COVID-19 and the onset of PCG ranged from -10 to 90d Acute, inflammatory, demyelinating polyneuropathy was diagnosed in 171 patients, acute, motor axonal neuropathy in 24, and acute, motor, and sensory axonal neuropathy in 16 patients. Regarding treatment, 241 patients received immunoglobulins, 28 patients' plasmaphereses, and 7 patients' steroids. Artificial ventilation was required in 59 patients. Full recovery was achieved in 42 cases, partial recovery in 163 cases, and 17 patients died. The number of published PCG patients fell from 192 in the second half of 2020 to 75 patients in the first half of 2021. It is concluded that the prevalence of PCG has decreased since the introduction of SARS-CoV-2 vaccines. SARS-CoV-2 vaccinations have a positive effect on the prevalence of PCG.

Lumbosacral polyradiculitis associated with brucellosis.

INTRODUCTION: Brucellosis is a bacterial disease caused by different species of Brucella. Neurobrucellosis is one of the complications of brucellosis and polyradiculopathy is an uncommon manifestation. CASE REPORT: In this article we report a 29-year-old male patient diagnosed with neurobrucellosis who presented with subacute lower limbs weakness and inability to walk in the last 50 days. The patient declared usage of non-pasteurized dairy products in his past medical history. Diagnosis was confirmed by the LP of CSF and serological tests. Although PCR for Brucella was negative, Wright, Coombs Wright and 2ME tests were reported positive in both CSF and serum. MRI and EMG were also performed that highlighted polyradiculopathy. After six months treatment, complete clinical recovery along with elimination of nerve root enhancement in MRI by injection in the lumbosacral region was seen. CONCLUSION: Neurobrucellosis is a serious manifestation of Brucellosis that can have many side effects. Therefore, clinicians must pay attention to the neurological manifestations of this disease, but also reduce the effects of this disease by accelerating the start of treatment.

Guillain-Barre syndrome 15 days after COVID-19 despite SARS-CoV-2 vaccination.

Guillain-Barre syndrome (GBS) following a SARS-CoV-2 vaccination has been repeatedly observed but GBS following a SARS-CoV-2 infection in a vaccinated patient has not been reported. A 69yo female developed paresthesias of both lower and upper limbs which were followed by progressive muscle weakness. The history was positive for a mild SARS-CoV-2 infection 15 days earlier. COVID-19 occurred despite a first dose of a vector-based SARS-CoV-2 vaccine, 40 days prior to onset of GBS. Since CSF investigations and nerve conduction studies were indicative of GBS, subtype acute, inflammatory, demyelinating polyneuropathy (AIDP), immunoglobulins (30 g/d) were started. After two weeks complete recovery was achieved. In conclusion, SARS-CoV-2 infections may develop despite full vaccination and may be complicated by GBS. Since no other trigger of GBS could be identified, a causal relation between the SARS-CoV-2 infection and GBS was suspected.

Autoimmune polyradiculitis due to combination immunotherapy with ipilimumab and nivolumab for the treatment of metastatic melanoma.

Immune checkpoint inhibitors, also known as immunotherapy, have revolutionised the treatment of metastatic melanoma, but are frequently associated with immune-related adverse events (irAEs) affecting a variety of organ systems. Here, we present a case of a patient with metastatic melanoma, being treated with combination ipilimumab-nivolumab, who developed a foot drop. MRI demonstrated enhancement of the nerve roots of the cauda equina. The patient had other irAEs, which warranted cessation of immunotherapy and the introduction of corticosteroids, and this also resulted in improvement in the patient's lower limb symptoms and MRI appearances. This confirmed an autoimmune polyradiculitis - a rare irAE.

Manual push technique, an alternative route of subcutaneous immunoglobulin administration in chronic inflammatory demyelinating polyradiculoneuropathy: A proof-of-concept study.

OBJECTIVE: Subcutaneous immunoglobulin (SCIg) administered through infusion pump has been reported as effective in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients. In this study we evaluate an alternative technique of SCIg administration, based on the delivery of lower volumes administered daily using manual push technique (MPT) in 10 CIDP patients. METHODS: In this randomized, controlled, two-arm, crossover clinical trial, CIDP patients were randomly assigned 1:1 to receive SCIg either by MPT or pumps for 4 consecutive months with crossover to the other. The primary objective was to assess whether MPT had the same effectiveness as pumps. The secondary objectives were to assess whether MPT resulted in greater plasma IgG levels and improved quality of life (QoL). RESULTS: Ten patients (mean age = 48.3) were enrolled. No significant changes were observed in the efficacy parameters (INCAT, MRC, R-ODS, and GS scales). A positive mean variation of 5.4 % in plasma IgG levels in the group treated with MPT was observed at the end of MPT periods. Treatment interference, which is one of the dimensions of the Life Quality Index, showed a significant improvement in the MPT periods. CONCLUSION: In CIDP patients, the MPT technique was as effective as pump infusion, allowed comparable, slightly increases plasma IgG levels, and also improved the QoL.

Guidelines for diagnosis and treatment in neurology - Lyme neuroborreliosis.

Lyme borreliosis is the most common tick-borne infectious disease in Europe. A neurological manifestation occurs in 3-15% of infections and can manifest as polyradiculitis, meningitis and (rarely) encephalomyelitis. This S3 guideline is directed at physicians in private practices and clinics who treat Lyme neuroborreliosis in children and adults. Twenty AWMF member societies, the Robert Koch Institute, the German Borreliosis Society and three patient organisations participated in its development. A systematic review and assessment of the literature was conducted by the German Cochrane Centre, Freiburg (Cochrane Germany). The main objectives of this guideline are to define the disease and to give recommendations for the confirmation of a clinically suspected diagnosis by laboratory testing, antibiotic therapy, differential diagnostic testing and prevention.

Other central nervous system infections: cytomegalovirus, Mycobacterium tuberculosis, and Treponema pallidum.

Human immunodeficiency virus (HIV)-infected individuals are particularly susceptible to several central nervous system infections: human cytomegalovirus, which may cause encephalitis, ventriculitis, polyradiculitis, or polyradiculomyelitis; Mycobacterium tuberculosis, which can cause meningitis or space-occupying lesions; and Treponema pallidum subspecies pallidum (T. pallidum), which affects the meninges, cerebrospinal fluid, cranial nerves, and vasculature in early neurosyphilis, and additionally the brain and spinal cord parenchyma in late neurosyphilis. Central nervous system cytomegalovirus infection is seen in HIV-infected individuals with very advanced immunosuppression. Its prognosis is poor and optimal therapy has not been determined. Tuberculous meningitis has a high mortality in those also infected with HIV, especially in the developing world, and better therapies are urgently needed. As the rates of syphilis increase in the developed world, neurosyphilis and in particular ocular syphilis are increasingly reported. The likelihood of all three of these central nervous system infections is decreased in individuals who receive potent antiretroviral therapy.