Acute-onset chronic inflammatory demyelinating polyneuropathy following AstraZeneca COVID-19 vaccine: a case report.

COVID-19 vaccination side effects have been increasingly reported, including new-onset autoimmune diseases such as chronic arthritis, thrombocytopenia, Guillain-Barré syndrome (GBS), and more recently chronic inflammatory demyelinating polyneuropathies (CIDP). Molecular mimicry and vaccine adjuvants appear to be important contributors to immune-mediated neuropathies. However, whether the link between the COVID-19 vaccine and these autoimmune disorders is coincidental or causal remains uncertain. We describe the ever-reported case of acute-onset CIDP following the Oxford/AstraZeneca vaccine in Tunisia. The patient is a 41-year-old man who presented with acute, worsening weakness of the four limbs. The symptoms appeared 15 days after his first dose of the AstraZeneca vaccine. The diagnosis of GBS was initially confirmed according to the clinical features, the albumino-cytological dissociation in the cerebrospinal fluid (CSF), and the electroneuromyography (ENMG) findings. Serum workup for all known infections associated with immune-mediated neuropathy was negative. The patient was treated with plasma exchange without initial improvement followed by aggravation of the symptomatology after an interval of four and a half months. Control ENMG showed signs of CIDP meeting the European Academy of Neurology/Peripheral Nerve Society (EAN/PNS) criteria of 2021. The patient was treated with maintenance intravenous immunoglobulin and oral corticosteroids. Neurological examination 3 months after discharge showed partial improvement. Worldwide, cases of demyelinating polyneuropathies post-COVID-19 vaccination are increasingly reported. The acute onset of CIDP might lead to a misdiagnosis of GBS. Awareness of this complication and distinction from GBS enables early relay with maintenance treatment to prevent relapses and severe complications. Post-COVID neuropathies are found to be more frequently linked to the AstraZeneca vaccine, however, temporal association does not confirm causal association.

Mild limb girdle muscular dystrophy R9 phenotype caused by novel compound heterozygous FKRP gene mutation.

Fukutin-related protein (FKRP) mutations cause a broad spectrum of muscular dystrophies, from a relatively mild limb-girdle muscular dystrophy type 9 (LGMDR9) to severe congenital muscular dystrophy (CMD). This study aims to report two siblings belonging to a non-consanguineous Tunisian family harboring a novel compound heterozygous FKRP variant and presenting a mild LGDMR9 phenotype. For mutation screening, massive parallel sequencing was performed, followed by Sanger sequencing and multiplex ligation-dependent probe amplification (MLPA) to validate the existence of the discovered variants. The absence of alpha-dystroglycan was determined by immunohistochemistry. Brain and thigh magnetic resonance imaging (MRI) were performed to detect thigh and brain abnormalities. The two siblings had a late age at onset and clinical examination showed that the pelvic girdles had a predominantly proximal and symmetrical distribution of weakness without cardiac or respiratory involvement. They both had a modified Gardner-Medwin Walton Scale mGMWS grade of 4 and a modified Rankin Scale (mRS) score of 1. The DNA sequencing revealed a novel deletion of exons 2 and 3 in one allele and a missense mutation c.1364C > A, which has been reported to be responsible for congenital muscular dystrophy and mental retardation on the second allele. The simultaneous presence of the two variations in the two cases suggests that the variants segregate with the pathophysiology.

Intrathecal B cell-related markers for an optimized biological investigation of multiple sclerosis patients.

In multiple sclerosis (MS) disease, the importance of the intrathecal B cell response classically revealed as IgG oligoclonal bands (OCB) in cerebrospinal fluid (CSF) was reaffirmed again in the recently revised diagnostic criteria. We aimed to optimize Laboratory investigation by testing the performance of new B cell-related molecules in CSF (Ig free light chains (FLCκ and λ) and CXCL13 (B-Cell Attracting chemokine1)) for MS diagnosis. 320 paired (CSF-serum) samples were collected from 160 patients with MS (n = 82) and non-MS diseases (n = 78). All patients benefited from IgG index determination, OCB detection, CSF CXCL13 and FLC (κ and λ) measurement in CSF and serum for metrics calculation (κ/λ ratio, FLC-related indexes, and κFLC-intrathecal fraction (IF)). CXCL13 and FLC metrics in CSF were higher in patients with MS and positive OCB. As expected, κFLC metrics-in particular, κFLC index and κFLC IF-had the highest accuracy for MS diagnosis. κ index showed the best performance (sensitivity 83% and specificity 91.7%) at a cut-off of 14.9. Most of the FLC-related parameters were positively correlated with IgG index and the level of CXCL13. In conclusion, the quantitative, standardizable, and technically simple CSF FLCκ metrics seem to be reliable for MS diagnosis, but could not replace OCB detection. CXCL13 appears to be an effective parameter reflecting the intrathecal B cell response. An optimized way for CSF testing combining the conventional and the new B cell-related parameters is proposed in this study.

Guillain-Barré syndrome following the first dose of Pfizer-BioNTech COVID-19 vaccine: case report and review of reported cases.

BACKGROUND: Since the SARS-CoV-2 pandemic has started in December 2019, millions of people have been infected all over the world. Vaccination is the most efficient tool to end this pandemic, but vaccine surveillance is necessary to identify side effects. Some studies have shown that neurological complications after COVID-19 vaccination are rare and dominated by demyelinating disease. CASE PRESENTATION: We present a case of a 67-year-old man who presented 7 days following his first dose of Pfizer-BioNTech COVID-19 vaccine a rapidly progressive ascending muscle weakness. The diagnosis of Guillain-Barré syndrome (GBS) was confirmed according to the clinical features, the albumino-cytological dissociation in the cerebrospinal fluid, and the electroneuromyography findings. The workup for all known infections associated with immune-mediated GBS was negative. The patient received treatment with intravenous immunoglobulin. Neurological examination 1 month after discharge showed full recovery and he regained his baseline functional status. CONCLUSIONS: As far as we know, this is the first reported case in Tunisia. Although extremely rare, neurologists should remain vigilant for acute inflammatory demyelinating polyradiculoneuropathy after COVID-19 vaccination.

Epidemiological and clinical profile of hypertensive octogenarian patients and factors associated with uncontrolled hypertension: observational study of 346 patients.

INTRODUCTION: hypertension (HTN) is the main risk factor for most morbidities of elderly subjects. The objective of this study was to identify the epidemiological and clinical characteristics of hypertension in octogenarians and to identify the factors associated with uncontrolled hypertension in this population. METHODS: we used data collected in the outpatient cardiology department of the University Hospital of Sfax between 15th April 2019 and 15th May 2019 as part of the National Tunisian Registry of Hypertension. We included in our study patients aged 80 years or more with hypertension. We described the epidemiological and clinical profile of this population, and we studied the associations between uncontrolled hypertension and socio-demographic, lifestyle, clinical and therapeutic factors using logistic regression models. RESULTS: we included 346 subjects (45.1% (n=156) male and 54.9% (n=190) female), with a mean age of 84.36 (SD 4.01) years. More than half of them had uncontrolled hypertension. Dyslipidemia was the most common cardiovascular risk factor found in 43.6 % (n=151) of patients followed by diabetes (35.5%, n=122). One-third of patients had a history of coronary artery disease and/or stroke. Renal failure and kalemia disorders were observed, respectively, in 12.1% (n=42) and 25.2% (n=40) of patients. In multivariate analysis, factors associated with uncontrolled hypertension (HTN) were male sex (adjusted odds ratio (aOR): 1.663, 95% confidence interval (CI): 1.045-2.647; p=0.032), diabetes (aOR: 1.66, 95%CI: 1.031-2.688; p=0.037,) and poor adherence to blood pressure (BP) medications (aOR: 1.960, 95%CI: 1.195-3.214; p=0.008). CONCLUSION: our results showed that more than half of octogenarian hypertensive patients did not reach the BP target and that poor adherence to BP medications was the main factor of uncontrolled HTN. In this population, the presence of other comorbidities and poor adherence to BP medications are very common. Systematic research for behaviors suggesting poor medication adherence should be a priority for physicians caring for these patients.

[Observational study of the prevalence of non-valvular atrial fibrillation and ischemic stroke and the factors associated with non-valvular atrial fibrillation in hypertensive patients: about 2887 cases]. / Prévalence de la fibrillation atriale non valvulaire et de l´accident vasculaire cérébral ischémique et facteurs associés à la fibrillation atriale non valvulaire chez les patients hypertendus: étude observationnelle à propos de 2887 patients.

INTRODUCTION: high blood pressure (HBP), atrial fibrillation (AF) and ischemic stroke are three public health problems. The purpose of this study was to determine the prevalence of AF and ischemic stroke in hypertensive patients and the factors associated with the occurrence of non-valvular AF in these patients. METHODS: we used data collected in the Department of Cardiology at the University Hospital of Sfax as a part of National Tunisian Registry of Hypertension. We examined the associations between different socio-demographic, clinical, paraclinical, and therapeutic variables and AF in hypertensive patients using logistic regression models. RESULTS: our sample consisted of 2887 patients with a sex ratio of 0.95. The average age of patients was 65 years (±11). AF was found in 230 patients (8%), while ischemic stroke was found in 152 patients (5.3%). Multivariate analysis highlighted that independent factors associated with an increase in the prevalence of AF were: advanced age (p = 0,001, Odds Ratio (OR) = 1,647, 95% confidence interval ( CI): 1,1227-2,213), the detection of left ventricular hypertrophy (LVH) on cardiac ultrasound (p= 0,004, OR= 2,140, 5%CI 9: 1,281-3,576), and left ventricular ejection fraction (LVEF) < 50% (p<0,001, OR=4,677, 95% CI: 2,715 and 8.057). CONCLUSION: this study confirms that there is a direct and independent relationship between high blood pressure, advanced age, LVH and AF. AF in patients with high blood pressure is a disease progression leading to an increased risk of ischemic stroke. Optimal high blood pressure control is a priority in hypertensive patients, especially in the elderly, aiming at preventing the risk of AF and ischemic stroke.

Smoldering multiple myeloma revealed by superior ophthalmic vein thrombosis.

Superior ophthalmic vein thrombosis is a rare entity. It is associated with significant morbidities. It may present with dramatic clinical signs. It is frequently secondary to cavernous sinus pathology and it can be a harbinger of cavernous sinus thrombosis. We report an unusual case of superior ophthalmic vein thrombosis, as the first manifestation of multiple myeloma. As far as we know, this is the first case described in the literature. Here we describe a patient presented with a painful, visual blur and a right-sided proptosis due to superior ophthalmic vein thrombosis. Appropriate medical workup was conducted, and smoldering multiple myeloma was diagnosed as the underlying cause. We further discuss the possible involved mechanisms.

Vertebral solitary bone plasmacytoma in a young adult with Trisomy 21: A case report.

Context: Solitary bone plasmacytoma (SBP) are rare lesions, accounting for less than 5% of all plasma cell proliferations. We describe a case of a 21-year-old female with Trisomy 21 presenting with cauda equina compression from an SBP. Findings: Solitary bone plasmacytoma (SBP) is a rare primary bone tumor. It is characterized by monoclonal proliferation of malignant plasma cells localized to a bone segment, without signs of systemic invasion. The vertebral location is the most common. It preferentially affects men during their 5th or 6th decade. Clinical relevance: We report the first association between solitary bone plasmacytoma and Trisomy 21.

[Five-word test calibration in a Tunisian population of healthy subjects]. / Étalonnage du test des cinq mots dans une population tunisienne de sujets sains.

INTRODUCTION: Five-word test (5WT) is a memory test to assess the verbal episodic memory. It measures the memory of subjects with memory impairment, in particular within the framework of the diagnosis of Alzheimer's disease, where it is sensitive and specific. The purpose of our study was to evaluate the effect of different sociodemographic parameters on subject's performance, to set standards relevant to Tunisian population and to compare our results to previous studies. METHODS: We report 5WT calibration in 315 normal subjects aged 40 to 90 years (169 men, 146 women), divided into four age groups (40-49,50-59,60-69 and 70 years) having three levels of education (I: primary, II:secondary and III:higher). We calculated the mean score (standard deviation) for the different scores: Total Score (TS), Total Weighted Score (TWS), Delayed Free Recall (DFR), Total Delayed Recall (TDR) and Total Free Recall (TFR). RESULTS: The average age of subjects was 57.29 years (11.02). Performances appeared to be better in youngest and better educated subjects, without any significant difference between the two sexes. Standards were calculated on the basis of age and levels of education. CONCLUSION: Five-word test allows rapid screening of patients in whom complementary neuropsychological assessment is essential for the diagnosis of cognitive disorders.