A Novel Case of Bifacial Diplegia Variant of Guillain-Barré Syndrome Following Janssen COVID-19 Vaccination

Guillain-Barré syndrome (GBS) is an immune-mediated demyelinating disorder which attacks the peripheral nervous system. Antecedent infection or vaccine administration are known to precipitate the onset of this disorder. Its typical presentation leads to a symmetric, rapidly progressive, ascending paresis with associated sensory deficits and impaired reflexes. We present a rare case of a bi-facial diplegia variant of GBS, within four weeks of the COVID-19 vaccination. Due to its chronology, clinical manifestations, and cerebrospinal fluid (CSF) findings, we propose this case to be a rare complication of the COVID-19 vaccination.

Relapse of Neuromyelitis Optica Spectrum Disorder Presented with Suspected Bacterial Meningomyelitis

Neuromyelitis optica spectrum disorder (NMOSD) is a rare inflammatory demyelinating disease. Anti-aquaporin-4 antibodies serve as a specific biomarker, while other factors including antecedent infection may also play a role in the development of NMOSD. Abnormal cerebrospinal fluid (CSF) findings such as leukocytosis with concentration >50/mm³ are one of the characteristics of NMOSD, but these were not specific for identifying other infective neurological diseases. Here we describe a rare case of NMOSD with CSF findings suggestive of bacterial meningomyelitis.

Role of Nerve Conduction Study in Polyneuropathy

Background: Polyneuropathy has many different causes. It is often very difficult to find out the cause. Nerve conduction study (NCS) can classify neuropathy as axonal and demyelinatig variety and direct the search for cause. Methodology: Purposively selected 80 patients from the department of Neurology Dhaka Medical College during the period of January 2009 to June 2010 were taken for NCS whose were compatible with polyneuropathy by history and clinical examination. Clinical, electrophysiological feature and pattern of polyneuropathy were analyzed. Results: Mean age of the patients was 34.5 ±6.8 and M: F was 1.8:1. Students, laborer and cultivators were the most affected people. 55% patients were acute cases and 35% patients were chronic Cases. 30% patient had no known risk factor for neuropathy 25% patient had antecedent infection, 15% had diabetes mellitus, 7.5% were exposed to drugs/toxins or solvents and 5% had family history of neuropathy. In clinical examination 37.5% patients were in motor type, 10% pure sensory type and 52.5% mixed sensorimotor type. In NCS47.5% were motor, 7.5% pure sensory 45% mixed sensorimotor type. Axonal were 47.5%, demyelinating 27.5% and 25% as mixed axonal and demyelinating type. Conclusion: NCS in polyneuropathy play critical role by classifying it as axonal or demyelinating and shorten the cause. Bangladesh J Medicine Jan 2020; 31(1) : 3-8

293 Autoimmune autonomic ganglionopathy: the NHNN experience

BackgroundAutoimmune Autonomic Ganglionopathy (AAG) is a rare, immune-mediated condition characterised by subacute pandysautonomia. 50% have auto-antibodies to the ganglionic nicotinic acetylcholine receptor (gAChR), affecting synaptic transmission at autonomic ganglia.MethodsWe describe 13 patients (5 female, median age 47) presenting with widespread autonomic failure, confirmed on cardiovascular, sudomotor and pupillometry testing at the National Hospital for Neurology and Neurosurgery (NHNN), and high gAChR antibody levels (>200 pm) measured by radioimmunoprecipitation assay at Oxford University.ResultsOf the 13 patients, 8 had other autoimmune conditions, 3 had antecedent infection and 3 were paraneoplastic. All had orthostatic hypotension, gastro-intestinal and urinary symptoms, 11 had documented pupillary abnormalities (9 mixed sympathetic and parasympathetic deficits, 2 subclinical sympathetic deficits), 11 had secretomotor dysfunction, 8 had generalised/partial anhidrosis, 8 had sexual dysfunction and 7 had evidence of small fibre dysfunction on neurophysiology.Ten received immunomodulatory treatment; 6 plasma exchange and 4 combination treatment including intravenous immunoglobulin, mycophenolate and rituximab. Earlier treatment was associated with greater clinical response.DiscussionPatients with AAG and high gAChR antibody levels have widespread dysfunction of the autonomic nervous system, which can respond dramatically to immunomodulation. Further research is needed to develop robust clinical biomarkers to guide treatment and monitor response.

Humoral immune response to campylobacter jejuni in patients with enterocolitis and Guillain-Barré syndrome

Campylobacter jejuni is one of the most important causes of diarrheal disease worldwide. In addition, it can cause neurological post-infectious sequels, such as Guillain-Barr? syndrome (GBS). Humoral immune response to C. jejuni was monitored in patients with C. jejuni enterocolitis, GBS patients and healthy persons, by ELISA. Statistical significance between patients with enterocolitis and healthy persons, as well as among GBS patients and healthy controls, was proven. Statistical significance in IgA among the examined groups was also noticed. The highest values of IgM were found in the patients with GBS, while the highest values of IgG were found in those with enterocolitis. C. jejuni is a significant cause of antecedent infection in GBS. ELISA techniques can be considered a reliable method in determining the presence of serum antibodies in patients with enterocolitis caused by C. jejuni, as well as in patients with GBS.

Anti-Borrelia burgdorferi Antibody Profile in Post-Lyme Disease Syndrome

ABSTRACTPatients with post-Lyme disease syndrome (PLDS) report persistent symptoms of pain, fatigue, and/or concentration and memory disturbances despite antibiotic treatment for Lyme borreliosis. The etiopathogenesis of these symptoms remains unknown and no effective therapies have been identified. We sought to examine the antiborrelia antibody profile in affected patients with the aim of finding clues to the mechanism of the syndrome and its relationship to the original spirochetal infection. Serum specimens from 54 borrelia-seropositive PLDS patients were examined for antibodies toBorrelia burgdorferiproteins p18, p25, p28, p30, p31, p34, p39, p41, p45, p58, p66, p93, and VlsE by automated immunoblotting and software-assisted band analysis. The presence of serum antibodies to the 31-kDa band was further investigated by examination of reactivity against purified recombinant OspA protein. Control specimens included sera from 14 borrelia-seropositive individuals with a history of early localized or disseminated Lyme disease who were symptom free (post-Lyme healthy group), as well as 20 healthy individuals without serologic evidence or history of Lyme disease. In comparison to the post-Lyme healthy group, higher frequencies of antibodies to p28 (P< 0.05), p30 (P< 0.05), p31 (P< 0.0001), and p34 (P< 0.05) proteins were found in the PLDS group. Assessment of antibody reactivity to recombinant OspA confirmed the presence of elevated levels in PLDS patients (P< 0.005). The described antiborrelia antibody profile in PLDS offers clues about the course of the antecedent infection in affected patients, which may be useful for understanding the pathogenic mechanism of the disease.

Colonic Malakoplakia in a Liver Transplant Recipient

Malakoplakia is a rare inflammatory condition seen in transplant patients. There are two previously reported cases of malakoplakia involving the gastrointestinal tract in liver transplant patients. The present paper reports a case of colonic malakoplakia in a 58-year-old woman, a liver transplant recipient who was receiving immunosuppressive drugs. She presented with chronic diarrhea while on tacrolimus. There was no history of antecedent infection. Colonoscopy showed patchy mucosal edema, but no discrete yellow plaques or nodules. The diagnosis was made by colon biopsies, which showed chronic inflammation with many histiocytes containing Michaelis-Gutmann bodies. Although rare, malakoplakia is one of many potential causes of diarrhea in a transplant patient. The present case indicates that malakoplakia may be associated with chronic diarrhea, even if there are no macroscopic lesions seen during colonoscopy.

Antibodies to ganglioside GM1 and Campylobacter jejuni in patients with Guillain-Barre syndrome

Guillain-Barre syndrome (GBS) is an acute immune mediated neuropathy, polyradiculoneuritis, characterized by rapid onset of symmetric extremity muscle paralysis, areflexia and albuminocytological dissociation in the cerebrospinal fluid (CSF). Recently, the heterogeneity of GBS has been noticed with definition of several GBS variants. The axonal GBS associated with anti-GM1 antibodies is the most important variant with the specific role of Campylobacter jejuni (CJ) in the induction of the disease. The role of our study was to determine the frequency of antecedent infection with CJ in the population of our patients with GBS, the association with anti-GM1 antibodies and the distribution of these antibodies within clinical forms of the disease. The diagnosis of GBS has been established in 17 patients according to clinical, electrophysiological and laboratory (CSF) criteria. The serum antibodies to 63 kDa flagellar protein isolated from CJ serotype 0:19 were determined by ELISA and Western blot and serum anti-GM1 antibodies by ELISA. In relation to the disability score two patients were ambulatory, five were ambulatory with support, seven were bedridden and two patients needed respirator. Five (29%) patients had pure motor, while 12 (71%) had sensorimotor GBS. The crania! nerves were involved in 11 (65%) and 9 (53%) patients had autonomic dysfunction. Electromyoneurography showed primary axonal, predominantly motor neuropathy in 6 (35%) and demyelinating sensorimotor neuropathy in 11 (65%) patients. The CSF protein content ranged from 0.47 to 3.88 g/L. The antecedent infection with CJ was shown by serum antibodies to CJ flagellar protein in 12 (71%) patients. Fifteen (88%) patients had IgG anti-GMI antibodies. Twelve (71%) patients had both antibodies. In relation to the clinical form, anti-CJ antibodies were found in 8 (73%) out of 11 patients with demyelinating GBS and in 4 (66.6%) out of b patients with axonal GBS. The high titer of anti-GM1 antibodies was found in all patients (100%) with axonal and in 9 (82%) out of 11 patients with demyelinating GBS. The association of IgG anti-CJ and IgG anti-GM1 antibodies was found in 4 (66.6%) out of b patients with axonal and in 8 (73%) out of 11 patients with demyelinating GBS. The main features of our patients with GBS were high frequency of antecedent infection with CJ, unusually frequent association with anti-GM1 antibodies, and equally frequent association of anti CJ and anti-GM1 antibodies in both, axonal and demyelinating GBS.

Campylobacter Species and Guillain-Barré Syndrome

SUMMARY Since the eradication of polio in most parts of the world, Guillain-Barré syndrome (GBS) has become the most common cause of acute flaccid paralysis. GBS is an autoimmune disorder of the peripheral nervous system characterized by weakness, usually symmetrical, evolving over a period of several days or more. Since laboratories began to isolate Campylobacter species from stool specimens some 20 years ago, there have been many reports of GBS following Campylobacter infection. Only during the past few years has strong evidence supporting this association developed. Campylobacter infection is now known as the single most identifiable antecedent infection associated with the development of GBS. Campylobacter is thought to cause this autoimmune disease through a mechanism called molecular mimicry, whereby Campylobacter contains ganglioside-like epitopes in the lipopolysaccharide moiety that elicit autoantibodies reacting with peripheral nerve targets. Campylobacter is associated with several pathologic forms of GBS, including the demyelinating (acute inflammatory demyelinating polyneuropathy) and axonal (acute motor axonal neuropathy) forms. Different strains of Campylobacter as well as host factors likely play an important role in determining who develops GBS as well as the nerve targets for the host immune attack of peripheral nerves. The purpose of this review is to summarize our current knowledge about the clinical, epidemiological, pathogenetic, and laboratory aspects of campylobacter-associated GBS.