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.

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.

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.

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.

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.

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.

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.

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.