Clinical course and outcome of an outpatient clinic population with myasthenia gravis and COVID-19.

INTRODUCTION/AIMS: Coronavirus disease-2019 (COVID-19) may have a more severe course in patients with myasthenia gravis (MG). We aimed to assess severity of the infection and factors contributing to its severity in a group of MG patients, most of whom were not hospitalized. METHODS: One hundred forty outpatients with MG followed between March 2020 and April 2021 were included in our study. Patients were asked to respond to a brief questionnaire in person, by telemedicine, or through electronic messages. RESULTS: Nineteen patients tested positive for COVID-19 by polymerase chain reaction. Two were asymptomatic. Of the 17 symptomatic patients, 11 had mild symptoms. They either had no treatment or received antivirals, antibiotics, and anticoagulants. Their myasthenia was well-controlled before infection and was unaffected by COVID-19. Three patients with moderate COVID-19 required hospitalization, but not intensive care, and had full recovery. Three other patients, the oldest in the cohort, had severe disease: One patient with a postsurgery myasthenic exacerbation before the infection needed intensive care without intubation, but recovered completely; two morbidly obese patients with comorbidities required intubation and died. Corticosteroids were increased in four of the six moderate/severely affected patients. Immunosuppressive (IS) agents were generally continued. Hydroxychloroquine (HCQ) for COVID-19 was used in one patient. DISCUSSION: Most patients had mild COVID-19 and all but two patients recovered. The design of the study made it possible to capture mild cases. Having well-controlled MG before infection and absence of comorbidities likely affected the course of the infection favorably. IS did not influence the progression.

Neurogenic heterotopic ossification in Guillain-Barre syndrome: a rare case report.

Neurogenic heterotopic ossification (NHO) is an abnormal development of bone in extra-skeletal tissues, related to neurological disease. NHO is frequently seen after traumatic brain injury or spinal cord injury. NHO may also occur as a rare complication of Guillain Barre Syndrome (GBS). Here, we present a 39 year old man with an acute onset of GBS who developed NHO around both hips two months after the disease onset. Our patient had a history of mechanical ventilation, incomplete tetraplegia and prolonged immobilisation. The pathogenesis of NHO is unclear. Various risk factors have been associated with the development of NHO; prolonged coma, long-term sedation, spasticity, degree of paralysis. NHO is a rare complication of GBS and physicians should be aware that it can develop especially in patients with severe paralysis and in need of mechanical ventilation. Pain and restriction of movements, especially in the hips, should bring NHO to the mind.

Prompt Response to Prednisone Predicts Benign Course in MuSK-MG.

BACKGROUND: The difficult course of patients with myasthenia gravis (MG) with anti-muscle-specific tyrosine kinase antibodies (MuSK) has been emphasized. However, no clear information is available on patients who have a benign course. METHODS: This study was aimed at comparing patients with favorable (minimal manifestations [MM] or better) and unfavorable outcomes to determine whether excellent response to corticosteroid (CS) treatment within 3 months (good response-3 months) has any predictive effect on the prognosis. RESULTS: Forty-six percent of 46 patients had a favorable outcome at year 3 and 54% at final follow-up. The major finding of this study was its high predictive value with good response-3 months. Those with good response-3 months had significantly more favorable outcome as compared to those without at year 3. The positive predictive value of good response-3 months was high (89% at year 3 and 84% at final follow-up). The negative predictive value diminished from 85% at year 3 to 67% at final follow-up due to increasing number of patients improving in the long run. Overall, 33% of the patients had a benign course with good response-3 months and no major exacerbations until the end of follow-up. CONCLUSIONS: Excellent response to CSs within 3 months appears to predict a favorable outcome in MuSK-MG.

Six cases during 2012-2015 and literature review of Chryseobacterium indologenes infections in pediatric patients.

Chryseobacterium indologenes is a widespread bacteria in the environment, especially hospitals, and a rarely reported human pathogen. The lowest frequency has been reported in children under 5 years of age. Clinical manifestations of C. indologenes include nosocomial pneumoniae, biliary tract infection, peritonitis, surgical wound infection, intravascular catheter-related bacteremia, cellulitis, and primary bacteremia. There is a knowledge gap in the management of C. indologenes infections, especially pertaining children, because of multiple antibiotic resistance and limited data in the literature concerning effective empirical treatment. In the published literature, a total of 16 cases of C. indologenes infections were reported in the pediatric age group. Herein, we present our experience in 6 children with C. indologenes infections. Early and prompt management of C. indologenes infections, particularly in children with mechanic ventilation, with polymicrobial infections, and under the age of 2 years, is of major importance because these factors seem to have a negative effect on the prognosis of infections caused by C. indologenes. Ciprofloxacin and TPM-SMX may be the best therapeutic choices for a combined initial empirical treatment of the patients.

Metabolic acidosis during the relapse treatment of idiopathic intracranial hypertension.

We herein present a 40-year-old female physician who was diagnosed with idiopathic intracranial hypertension (IIH) 4 years ago. In the last years, the patient was in remission without any medications. Since the onset of COVID-19 pandemic, she has been stressfully working in the high-risk area, therefore using personal protective equipment (N95 mask, protective clothing, goggles, and protective cap) during the day for extended periods. Her headaches recurred and the patient was diagnosed with a relapse of IIH; acetazolamide and afterward topiramate were initiated, with diet treatment. Symptomatic metabolic acidosis, which is otherwise a rare side effect of the IIH treatment and not seen in her first attack even with higher doses, developed during the follow-up, presenting with shortness of breath and chest tightening. The emerging problems of IIH diagnosis and management during the COVID-19 pandemic will be discussed.

Prevalence and characteristics of visual aura in idiopathic generalized epilepsy.

Some patients with idiopathic/genetic generalized epilepsy (IGE) experience visual aura, which can confuse the diagnosis. We sought to determine the frequency and characteristics of visual auras in IGE patients. Among the 176 IGE patients, 4 men and 7 women reported visual auras (mean age - 24 years). Syndromic diagnoses were juvenile myoclonic epilepsy in four, eyelid myoclonia with absences (EMA) in three, juvenile absence epilepsy in three, and other in one. Visual auras consisted of flashing lights, macropsia, illusional movements, and blindness. Eyelid myoclonia with absences was significantly more common in the group with visual aura (3 of 11 patients vs. 8 of 165 IGE patients; P=0.02). Furthermore, photosensitivity was found significantly more common in IGE patients with visual aura (90% vs 46% of the total IGE patients) (P=0.004). In conclusion, the visual auras do not exclude a diagnosis of IGE. The presence of visual aura in the EMA syndrome is also remarkable.

Autoimmune Encephalitis After Treatment of Hodgkin's Lymphoma with the Immune Checkpoint Inhibitor Nivolumab.

In recent years, by the usage of new immune therapeutic agents for cancer treatment, the neurologic adverse events began to be seen more frequently. Nivolumab, one of the immune checkpoint inhibitor, is a human IgG4 antibody that blocks programmed cell death protein 1 and is approved against metastatic melanoma, squamous cell lung cancer, renal cell carcinoma, and Hodgkin's lymphoma after failure of prior line of chemotherapy. Here, we present a 40-year-old patient developing encephalopathy after treatment of Hodgkin's lymphoma with the immune checkpoint inhibitor nivolumab. In literature, cases of autoimmune encephalitis after receiving combination therapy of immune checkpoint inhibitors ipilimumab and nivolumab were described before. As far as we know, this is the unique case of encephalopathy reported after monotherapy with nivolumab treatment used for Hodgkin's lymphoma.

Comparison of Conventional Methods, Automated Systems, and DNA Sequence Analysis Methods in the Identification of Corynebacterium afermentans and Corynebacterium mucifaciens Bacteria Isolated from Blood and Catheter Culture Samples.

The aim of this study is to compare different methods due to the difficulties in identifying coryneform bacteria to species level and to determine antibiotic resistance profiles. Isolates identified as Turicella otitidis (n:45) by VITEK 2 Compact and Corynebacterium mucifaciens (n:1) by matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS), isolated from blood and catheter cultures between 2015 and 2017 were included in the study. For identification of the isolates, conventional tests and 16S rDNA sequence analysis were performed. Antibiotic susceptibilities of the isolates were determined by Etest. The isolates identified as T. otitidis with VITEK 2 Compact could not be identified by MALDI-TOF MS and described as C. mucifaciens/Corynebacterium afermentans spp. by 16S rDNA sequence analysis. One isolate identified as C. mucifaciens by MALDI-TOF MS could not be identified with VITEK 2 Compact and described as C. mucifaciens by 16S rDNA sequence analysis and conventional methods. All isolates (n:45) described as C. mucifaciens/C. afermentans spp. by 16S rDNA sequence analysis were identified as C. afermentans subsp. afermentans with conventional methods. All 45 isolates identified as C. afermentans subsp. afermentans were resistant to penicillin, erythromycin, and clindamycin and were susceptible to vancomycin and daptomycin, whereas 31 (69%) were resistant to trimethoprim-sulfamethoxazole (TMP-SXT). The isolate identified as C. mucifaciens was susceptible to penicillin, vancomycin, daptomycin, and TMP-SXT; it was resistant to erythromycin and clindamycin. In this study, we reported 45 C. afermentans isolates misidentified as T. otitidis in routine laboratory processes. To our knowledge, this is the first study to include the highest number of C. afermentans blood isolates.

The effects of diffusion and perfusion MRI mismatch on the admission blood glucose and blood pressure values measured in the first 12 hours of acute stroke.

Patients with severe stroke and salvageable brain tissue at admission, who have higher glycaemic and blood pressure levels, may have a risk of iatrogenic hypoglycemia/iatrogenic hypotension. In this study, we examined the relationship between the presence of diffusion-weighted imaging (DWI)/perfusion-weighted imaging (PWI) mismatch, admission blood glucose level, and admission blood pressure level in patients who were admitted in the first 12 hours after onset. We studied 212 patients who were prospectively and consecutively registered to the stroke unit from 2006 to 2009. Correlations between mismatch and admission blood pressure level (ABPL) and admission blood glucose level (ABGL) were analyzed using multivariate logistic regression. Mismatch (P = .064, adjusted OR = 2.297, 95% CI, 0.953-5.536) was not associated with a high ABGL in the whole group. However, after excluding patients with diabetes mellitus (DM) (n = 67, 35%), mismatch (P = .033, adjusted OR = 3.801, 95% CI, 1.110-13.015), an impaired level of consciousness, use of anti-DM medication, glycated hemoglobin levels, and cardioembolic aetiology were independent predictors of a high ABGL. The presence of mismatch or proximal vessel occlusion was not associated with ABPL. Female sex (P = .048) and total anterior circulation stroke (P = .008) were independent predictors associated with a higher ABPL. We conclude that patients with hyperacute ischemic stroke with PWI/DWI mismatch are more likely to have hyperglycemia.

Clinical and microbiological characteristics of Pantoea agglomerans infection in children.

Pantoea agglomerans is an environmental Gram-negative bacterium that rarely is responsible for the infections in humans but it is often a causative factor of a number of occupational diseases. This study evaluated the clinical and microbiological characteristics and pathogenicity of P. agglomerans in children. We retrospectively reviewed microbiological test results for all children (1 month old to 18 years old) who were admitted to our pediatric hospital between January 2000 to June 2015 and had positive clinical specimen cultures for P. agglomerans. Isolates were identified using conventional tests and the BBL Crystal E/NF ID or MALDI-TOF MS systems. Antibiotic susceptibilities were evaluated using the Kirby-Bauer disc diffusion method. We identified fifteen positive cultures from 14 patients with confirmed infections. The positive specimens included pus, urine, tracheal aspirate, blood, and central venous line samples that yielded P. agglomerans. The median patient age was 8.8 years (range: 1.5 months to 16.5 years), and all patients had underlying comorbidities. Five patients had medical devices, and two devices were removed. The most common P. agglomerans infections involved wound infections (35.7%), pneumonia (21.4%), and urinary tract infections (21.4%). Three patients had concomitant infections (Enterococcus faecium, Pseudomonas aeruginosa, and Aspergillus fumigatus). Five patients had anemia. Three patients (21.4%) died, and all three had carbapenem-resistant P. agglomerans that was detected after the first week of hospitalization; two cases involved pneumonia, which was ineffectively treated. P. agglomerans infections may be life-threatening, especially in young patients with pneumonia. Hospital-acquired P. agglomerans may have different pathogenicity and clinical features, compared to community-acquired P. agglomerans, although further studies are needed to understand the drug-resistance patterns in this bacterium.

Reevaluation of the Critically Ill Patients With Nonconvulsive Status Epilepticus by Using Salzburg Consensus Criteria.

OBJECTIVE: We aimed to assess the usefulness of the Salzburg Consensus Criteria (SCC) for determining the prognosis of critically ill patients with nonconvulsive status epilepticus (NCSE). METHODS: We retrospectively reviewed consecutive patients with unconsciousness followed up in the intensive care unit (ICU). Three clinical neurophysiologists, one of them blinded to clinical and laboratory data, reevaluated all EEG data independently and determined NCSE according to SCC. The incidence of NCSE and ictal EEG patterns and their relationship to clinical, laboratory, neuroradiological, and prognostic findings were assessed. RESULTS: A total of 107 consecutive patients with mean age 68.2 ± 15.3 years (57 females) were enrolled in the study. Primary neuronal injury was detected in 59 patients (55.7%). Thirty-three patients (30.8%) were diagnosed as NCSE. While authors decided to treat 33 patients (30.8%), 32 patients (29.9%) had been treated in real-life evaluation. Clinical and EEG improvement were detected in 12 patients (11.3%) in real-life treatment group showing correlation with lack of intubation and ICU stay related to postsurgical event. Rate of mortality (45.8%) was high showing association with systemic-metabolic etiology, severity of coma and presence of "plus" modifiers in the EEG. CONCLUSION AND SIGNIFICANCE: Our findings suggest that SCC is highly compatible with clinical practice in the decision for treatment of patients with NCSE. The presence of "plus" modifiers in the EEG was found to be associated with mortality in these patients and was a significant marker for the high mortality rate.

Clinical Strains of Chryseobacterium and Elizabethkingia spp. Isolated from Pediatric Patients in a University Hospital: Performance of MALDI-TOF MS-Based Identification, Antimicrobial Susceptibilities, and Baseline Patient Characteristics.

Our objective was to evaluate the performance of matrix-assisted laser desorption ionization time-of-flight mass spectrometry (MALDI-TOF MS) for identification of the Chryseobacterium and Elizabethkingia spp. isolated from pediatric patients at Hacettepe University Hospital using 16S rRNA gene sequencing as the gold standard and to determine the antimicrobial susceptibility patterns of the isolates and baseline characteristics of patients. All stored Chryseobacterium and Elizabethkingia spp. isolated from various clinical specimens (sputum, blood, and urine) of pediatric patients at Hacettepe University Hospital between 2012 and 2016 were included in this study. Minimum inhibitory concentrations of 10 antimicrobial agents were determined by Etest for all isolates. To determine the baseline characteristics of patients, medical records of all patients were retrospectively reviewed. In total, 18 isolates of Chryseobacterium spp. (16 C. indologenes, 2 C. gleum) and 5 isolates of Elizabethkingia spp. (3 E. meningoseptica, 2 E. anophelis) were identified by 16S rRNA sequencing. MALDI-TOF MS correctly identified 19 (82.6%) isolates to the species level. The quinolones (ciprofloxacin and levofloxacin), trimethoprim/sulfamethoxazole and piperacillin/tazobactam showed the highest spectrum of activity against the overall collection of isolates. Cystic fibrosis (CF) was the underlying disease in 81.8% of patients. To our knowledge, this study includes the largest number of Chryseobacterium spp. isolated from clinical specimens of pediatric patients in Turkey. In this study, we also report the first clinical isolate of E. anophelis in Turkey. Since, the majority of strains were isolated from patients with CF; larger, prospective clinical studies are needed to establish whether chryseobacteria could be considered as an emerging opportunistic pathogen in patients with CF.

Delta Brush Pattern Is Not Unique to NMDAR Encephalitis: Evaluation of Two Independent Long-Term EEG Cohorts.

PURPOSE: Although its specificity has not previously been investigated in other cohorts, delta brush pattern (DBP) is increasingly reported in the EEGs of patients with anti- N-methyl-d-aspartate receptor (NMDAR) encephalitis. METHODS: We aimed to investigate the DBP in the EEGs of 2 cohorts; patients with change in consciousness for various causes monitored in the intensive care unit (ICU) (n = 106) and patients with mesial temporal lobe epilepsy (MTLE) with or without antineuronal antibodies (n = 76). RESULTS: These patients were investigated for the presence of DBP, defined as an EEG pattern characterized by delta activity at 1 to 3 Hz with superimposed bursts of rhythmic 12- to 30-Hz activity. Two investigators blindfolded for the clinical and immunological data independently analyzed the EEGs for recognition of this pattern. An EEG picture compatible with DBP was observed in 4 patients; only 1 of them (1.3%) belonged to the MTLE group. She did not bear any of the investigated autoantibodies and was seizure-free after epilepsy surgery. In the ICU group, there were 3 additional patients showing DBP with various diagnoses such as hypoxic encephalopathy, brain tumor, stroke, and metabolic derangements. All of them had died in 1-month period. CONCLUSIONS: Our results underlined that DBP is not unique to NMDAR encephalitis; it may very rarely occur in MTLE with good prognosis after surgery and second, in ICU patients who have high mortality rate. Therefore, the presence of this pattern should alert the clinician for NMDAR encephalitis but other possible etiologies should not be ignored.

Clinical and magnetic resonance imaging findings of HIV-negative patients with neurosyphilis.

There have been no serial studies about neuroradiological findings of neurosyphilis in the literature. There have been only case reports concerning HIV negative patients with neurosyphilis. We present 8 HIV negative neurosyphilis patients two of whom are women. The mean age of the patients was 48 years+/-12.37. Five of the 8 patients had general paresis, two optic atrophy and one multiple cranial neuropathies. The CSF findings were quite similar in 6 of 8 patients. In half of the patients cranial MRI showed mild cerebral atrophy. Nonspecific hyperintense small foci in 3 patients are thought to be related to syphilis. Hyperintensity involving bilateral medial and anterior temporal regions more prominent on the left side was seen in one of the patients with general paresis. This finding may be due to cytotoxic edema associated with status epilepticus and may mimic herpes simplex and other limbic encephalitides. Though not typical, certain MRI findings guides for the diagnosis of neurosyphilis.

Vascular Ischemia as a Cause of Transient Global Amnesia: A Patient Series.

INTRODUCTION: Epileptic, migrainous, and vascular pathologies may cause transient global amnesia (TGA); however, the mechanism of causation remains unclear. We investigated possible vascular causes of TGA. METHODS: We retrospectively evaluated the clinical and radiologic studies of 13 patients with TGA. On admission, patients underwent diffusion-weighted imaging (DWI) and intra- and extracranial magnetic resonance angiography (MRA); vascular risk factor profiles for diabetes, hypertension, and hyperlipidemia; electroencephalography; and neuropsychological tests. Seven patients underwent control DWIs 24 h after symptom onset. RESULTS: One patient had two punctiform acute infarcts in the left hippocampus, and one had a left pontine paramedian acute infarct. In the second patient, control DWI showed additional left hippocampal and right frontal acute infarcts. None of the patients had electroencephalographic evidence of epileptic activity. All patients except for one had at least one vascular risk factor. The second patient was shown to have paroxsysmal atrial fibrillation during follow-up. CONCLUSION: Minor posterior circulation ischemic stroke appears to cause TGA in some patients. Evaluations such as DWI and vascular risk factor assessment may be helpful in making the diagnosis.

Prepubertal anti-Musk positive myasthenia gravis with long remission.

Anti-MuSK positive myasthenia gravis (MuSK-MG) is rare prepuberty. We report a female patient with MuSK-MG starting at 3.5years with ptosis as the sole symptom for 2years. A brief period of generalization was followed by complete stable remission for 6years. Prolonged ocular symptoms and long remissions are not features of MuSK-MG, but are often seen in prepubertal onset MG. The patient then presented at age 12 with moderately severe symptoms which were almost confined to oculobulbar muscles and were unresponsive to pyridostigmine. She was dependent on corticosteroids and thymectomy did not seem to be effective. She was later noted to have tongue atrophy after a period without treatment. Our patient thus presented with features seen in many prepubertal patients, but the later course was quite typical of MuSK-MG.