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.

Neutrophil­to­lymphocyte ratio as a marker of inflammation in restless legs syndrome during pregnancy.

PURPOSE: The pregnancy is accepted as an independent risk factor for restless legs syndrome/Willis-Ekbom disease (RLS/WED). The neutrophil-to-lymphocyte ratio (NLR) was recently reported in the pathophysiology of RLS/WED. In this report, we investigated the relationship between the presence of RLS/WED and the levels of NLR in pregnancy. METHODS: We included 268 pregnant women attending routine prenatal visits; 148 women had RLS/WED, and 120 women without RLS/WED were the control group. A pre-formed questionnaire was administered to all participants regarding demographic characteristics, habitual behaviors, detailed medical history and questions about RLS/WED including disease duration, severity, and family history. Laboratory investigations were performed in all participants regarding the complete blood count, NLR, fasting blood glucose, blood urea nitrogen, creatinine, ferritin, and total iron binding capacity. RESULTS: NLR was significantly higher in pregnant women with RLS/WED as compared to those without it (3.9±0.9 versus 3.5±1.1, p=0.039). Smoking was also significantly more common in pregnant women with RLS/WED (p=0.022). NLR significantly increased as the gestational period progressed, even after the adjustments for age, BMI, and smoking (p=0.035). Higher NLR in pregnant women with RLS/WED was especially prominent in the 3rd trimester, although the difference was not significant. CONCLUSION: These results may suggest that an increased inflammation demonstrated by the increased NLR, may, in part, play a role in higher prevalence of RLS/WED in pregnancy, especially in late gestational weeks (Tab. 2, Fig. 1, Ref. 34). Text in PDF www.elis.sk Keywords: neutrophil-to-lymphocyte ratio, restless legs syndrome/Willis-Ekbom disease, pregnancy, inflammation.

Progression of myoclonus subtypes in subacute sclerosing panencephalitis.

OBJECTIVES: Diagnostic criteria of subacute sclerosing panencephalitis (SSPE) include myoclonus, a well-recognized clinical feature. Here, we studied the electrophysiological features of myoclonus with regards to disease staging in SSPE patients. METHODS: We included 10 patients diagnosed with SSPE between 2010 and 2018, along with 21 healthy subjects. All participants had detailed electrophysiological evaluation including polymyographic analysis, blink reflex after trigeminal stimulation, auditory startle response, startle response after somatosensory stimuli, F-waves, and long-loop reflexes. Clinical findings were retrieved from the medical records. RESULTS: Patients were categorized into Gascon stage 2B (n = 5, 50%), 2A (n = 2, 20%), 3B (n = 2, 20%) and 4A (n = 1, 10%) at the time of electrophysiological evaluation. Two patients had cortical myoclonus, four had possible cortico-subcortical myoclonus, and four had brainstem myoclonus. Patients were categorized into Gascon stages 2a and 2b had possible cortico-subcortical myoclonus (85.7%). However, none of the patients with stage 3b or 4a had possible cortico-subcortical subtype but all had the brainstem subtype. CONCLUSION: Association was seen between subtypes of myoclonus and clinical staging in SSPE. This suggests that myoclonus in SSPE may primarily involve the cortex and cortico-subcortical structures such as the thalamus at earlier stages of disease, and then involve more caudal structures as the disease progresses.

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.

Electrophysiological investigation for autonomic dysfunction in patients with myasthenia gravis: A prospective study.

BACKGROUND AND PURPOSE: Myasthenia gravis (MG) is an autoimmune disorder of neuromuscular transmission. Autonomic dysfunction is not a commonly known association with MG. We conducted this study to evaluate autonomic functions in MG & subgroups and to investigate the effects of acetylcholinesterase inhibitors. METHODS: This study comprised 30 autoimmune MG patients and 30 healthy volunteers. Autonomic tests including sympathetic skin response (SSR) and R-R interval variation analysis (RRIV) was carried out. The tests were performed two times for patients who were under acetylcholinesterase inhibitors during the current assessment. RESULTS: The RRIV rise during hyperventilation was better (p=0.006) and Valsalva ratio (p=0.039) was lower in control group. The SSR amplitudes were lower thereafter drug intake (p=0.030). As much as time went by after drug administration prolonged SSR latencies were obtained (p=0.043).Valsalva ratio was lower in the AchR antibody negative group (p=0.033). CONCLUSION: The findings showed that both ocular/generalized MG patients have a subclinical parasympathetic abnormality prominent in the AchR antibody negative group and pyridostigmine has a peripheral sympathetic cholinergic noncumulative effect.

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.

The Relationship Between Serum Uric Acid Levels and Clinical Features in Essential Tremor.

INTRODUCTION: In this study, we aimed to investigate the effect of uric acid on the disease, its severity and progression in ET patients with partially co-clinical features with Parkinson's disease (PD). METHODS: Serum UA levels of 87 consecutive ET patients were measured and were matched according to age and sex with 87 healthy controls. Fahn-Tolosa-Marin scale was used for the severity of tremor. Sociodemographic characteristics, type of ET, duration of disease, and treatment modalities were evaluated. RESULTS: The mean uric acid level was calculated as 4.986±2.1458 mg/dL and 6.004±1.523 mg/dL in the patient and control groups, respectively (p≤0.005). The blood UA level of patients with sporadic (n: 61) ET was found to be lower than the familial ET (n: 26) (p≤0.005). The tremor severity of the family ET patients was lower than the sporadic ET. (n: 61) (p≤0.005). The mean blood UA level (4.429±1.216 mg/dL) in the patients with high total tremor severity scores (n: 48) was found lower than in the patients with low total tremor severity scores (n: 39) (5.673±2.106 mg/dL) (P=0.000). The serum UA level was significantly lower in the patients whose disease duration longer than 5 years than in patients whose duration of the disease was shorter than 5 years. 5.732±1.240 for ≥5 years; 6.438±0.286≤5 years) (P=0.001). CONCLUSION: We hypothesize that as a result of high antioxidant properties of high serum uric acid levels, it is a biomarker that can show disease risk and progression in patients with ET as well as PD.

Autonomic dysfunction in restless legs syndrome.

OBJECTIVE: Autonomic dysfunction in patients with RLS has been described in some domains; however, detailed studies on this subject are limited and report conflicting results. In this study, we aimed to evaluate autonomic functions electrophysiologically and clinically in patients with restless legs syndrome (RLS). METHODS: Fifty-two adult patients with RLS and 40 healthy controls were enrolled in this prospective study. Electrophysiological tests of sympathetic skin response (SSR) and RR interval variability (RRIV) analysis were performed, and the SCOPA-AUT questionnaire was applied to evaluate autonomic functions. RESULTS: There was no significant difference in terms of SSR results between patients and controls (p > 0.05). However, there were significant differences between the patient and control groups in terms of RRIV analyses at rest, deep breathing, and valsalva, and also valsalva ratio (p = 0.037, p = 0.049, p = 0.017, p = 0.020). The mean SCOPA-AUT total score was higher in the RLS group compared with the control group (20.7 ± 10 vs 14.2 ± 8; p = 0.003). Significant differences were found regarding gastrointestinal, urinary, and cardiovascular domains (p = 0.01, p = 0.007, p = 0.049); on the other hand, pupillomotor, thermoregulatory, and sexual function did not significantly differ (p > 0.05). CONCLUSION: Autonomic functions should be questioned in detail as well as motor and sensory symptoms of RLS, and care should be taken especially on cardiac dysfunction.

The yield of electroencephalography in syncope.

BACKGROUND AND PURPOSE: Syncope is defined as a brief transient loss of consciousness due to cerebral hypoperfusion. Although the diagnosis of syncope is based on a thorough history and examination, electroencaphalography (EEG) is also an important investigational tool in the differential diagnosis in this group of patients. In this study we aimed to identify the diagnostic value of EEG in patients with syncope. METHODS: We retrospectively examined EEG recordings of 288 patients with the diagnosis of syncope referred to the Cankiri State Hospital EEG laboratory, from January 2014 to January 2016. The EEG findings were classified into 6 groups as normal, epileptiform discharges (spike and sharp waves), generalized background slowing, focal slowing, hemispherical asymmetries, and low amplitude EEG tracing. The EEGs were separated according to gender and age. RESULTS: Total of 288 patients were included in this study, 148 were females (51.4%) and 140 (48.6%) were males. Among all the EEG reports, 203 (70.5%) were normal, 8 of them (2.8%) showed generalized background slowing and 7 (2.4%) demonstrated focal slow waves. Epileptiform discharges occured among 13 patients (4.5%). Hemispherical asymmetries were detected in 10 patients (3.5%) and low amplitude EEG tracing in 47 patients (16.3%). There was no significant difference between age groups in EEG findings (p=0.3). Also no significant difference was detected in EEG results by gender (p=0.2). CONCLUSION: Although the diagnosis of syncope, epilepsy and non-epileptic seizures is clinical diagnosis, EEG still remains additional method.

Intravenous Immunoglobulin Treatment for Recurrent Optic Neuritis.

INTRODUCTION: Recurrent optic neuritis neuritis (rON) is an autoimmune inflammatory condition of unknown cause. Intravenous immunoglobulin (IVIg) treatment is used for many autoimmune disorders; however we do not have any information about its effect in rON, other than case reports. We aimed to evaluate our patients with rON who were treated with IVIg. METHODS: Data from all our patients with rON with or without anti aquaporin4 (AQP4) seropositivity, seen between April 2011 and October 2015, who received IVIg treatment were retrospectively evaluated. RESULTS: Nine patients (all female) with rON had received IVIg. These patients were aged between 34 and 65 years, and had started receiving monthly IVIg from 6 to 58 months after onset of disease. In three out of nine rON patients serum AQP4 antibody were positive. Under current treatments the patients had continued to have attacks, therefore monthly IVIg was given in addition to the existing immunosuppressant drug. The follow up duration was between 6 to 31 months. Three patients, each suffered one relapse under IVIg treatment. Mean number of relapses in the year prior to treatment was 1.4±0.72, whereas it was 0.3±0.5 during the year after IVIg therapy. During follow-up with IVIg administration only one patient had fever and no other adverse events were reported. CONCLUSION: Monthly IVIg is well-tolerated and safe and it seems to be effective in rON as an add on treatment. However, since our study is a retrospective case series, future randomized controlled trials with IVIg are needed.

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.

Evaluation of the Tibial Nerve with Shear-Wave Elastography: A Potential Sonographic Method for the Diagnosis of Diabetic Peripheral Neuropathy.

Purpose To evaluate the value of shear-wave elastography (SWE) in the detection of diabetic peripheral neuropathy (DPN) of the tibial nerve. Materials and Methods This study was approved by the institutional review board, and written informed consent was obtained from all study participants. The study included 20 diabetic patients with DPN (10 men, 10 women), 20 diabetic patients without DPN (eight men, 12 women), and 20 healthy control subjects (nine men, 11 women). The tibial nerve was examined at 4 cm proximal to the medial malleolus with gray-scale ultrasonography and SWE. The nerve cross-sectional area (in square centimeters) and the mean nerve stiffness (in kilopascals) within the range of the image were recorded. Inter- and intrareader variability, differences among groups, and correlation of clinical and electrophysiologic evaluation were assessed with intraclass correlation coefficients, the Mann Whitney U test, and the Wilcoxon signed rank test. Results Between diabetic patients with and diabetic patients without DPN, mean age (60 years [range, 38-79 years] vs 61 years [range, 46-75 years], respectively), mean duration of diabetes (10 years [range, 1-25 years] vs 10 years [range, 2-26 years]), and mean body mass index (31.4 kg/m2 [range, 24.7-48.1 kg/m2] vs 29.8 kg/m2 [range, 22.9-44.0 kg/m2]) were not significantly different. Diabetic patients without DPN had significantly higher stiffness values on the right side compared with control subjects (P < .001). Patients with DPN had much higher stiffness values on both sides compared with both diabetic patients without DPN (P < .001) and healthy control subjects (P < .001). A cutoff value of 51.0 kPa at 4 cm proximal to the medial malleolus revealed a sensitivity of 90% (95% confidence interval [CI]: 75.4%, 96.7%) and a specificity of 85.0% (95% CI: 74.9%, 91.7%). Conclusion Tibial nerve stiffness measurements appear to be highly specific in the diagnosis of established DPN. The increased stiffness in subjects without DPN might indicate that the nerve is affected by diabetes. © RSNA, 2016 Online supplemental material is available for this article.

Neuromyelitis Optica and Neuromyelitis Optica Spectrum Disorder Patients in Turkish Cohort: Demographic, Clinical, and Laboratory Features.

BACKGROUND: Neuromyelitis optica (NMO) is an immune-mediated, chronic relapsing, inflammatory disease characterized by severe attacks of optic neuritis and myelitis. OBJECTIVE: To determine the demographic, clinical, and laboratory features; antibody status; and treatment modalities of patients with NMO and neuromyelitis optica spectrum disorders in a Turkish cohort from 11 centers. METHODS: A total of 182 patients were included in this study. Data on age at disease onset, sex, type of attacks, clinical presentation, analysis of cerebrospinal fluid, serum antiaquaporin-4 antibody status, annual progression index, and medical and family histories were collected. RESULTS: Mean age was 38.43±12.40 years (range, 13 to 75 y), and mean age at disease onset was 31.29±12.40 years (median, 29 y; range, 10 to 74 y). In NMO group, the rate of NMO immunoglobulin (Ig)G positivity was 62.5%. The annual progression index was significantly higher in the longitudinally extending spinal cord lesion. The mean Expanded Disability Status Scale score was higher in the late than early-onset NMO group. CONCLUSION: Our results revealed a lower rate of NMO IgG positivity, more severe disability in patients with NMO/neuromyelitis optica spectrum disorders presenting with either transverse myelitis or late-onset NMO, and no correlation between disability and NMO IgG status.

What Does One Sleep-Onset REM Period­During Either Nocturnal Polysomnography or Multiple Sleep Latency Test­Mean in Differential Diagnosis of Central Hypersomnias?

PURPOSE: The differentiation of narcolepsy without cataplexy from idiopathic hypersomnia is based on the number of sleep-onset rapid eye movement periods (SOREMPs) observed by multiple sleep latency test (MSLT) and nocturnal polysomnography. The main aim of this study was to investigate the utility of SOREMP in differential diagnosis of central hypersomnias. METHODS: The authors retrospectively evaluated consecutive 101 patients with a normal polysomnography other than the presence of SOREMP and/or REM without atonia and a latency of ≤8 minutes in MSLT. RESULTS: The authors classified patients as follows: 52 patients had at least 2 SOREMPs (narcolepsy group), 23 had no SOREMPs (idiopathic hypersomnia group), and 26 patients had only 1 SOREMP (intermediate group). In polysomnographic recordings, both mean sleep latency and REM latency were significantly shorter in the narcolepsy (P = 0.012, P < 0.001, respectively) and intermediate groups (P = 0.005 and P = 0.035, respectively) compared with the idiopathic hypersomnia group. In MSLT recordings, sleep latency was 2.7 ± 2.2 minutes in the narcolepsy group, 3.6 ± 1.4 minutes in the intermediate group, and 5.2 ± 2.7 minutes in the idiopathic hypersomnia group (P < 0.001). The mean REM latency and sleep stages SOREMPs arised from were similar between the narcolepsy and intermediate groups. CONCLUSIONS: To date, SOREMPs in MSLT and polysomnography remain the sole electrodiagnostic feature that discriminates narcolepsy without cataplexy from idiopathic hypersomnia. Different parameters or combined criteria are being increasingly investigated to increase the sensitivity and specificity of MSLT. The findings showed an altered instability of REM sleep not only in patients with 2 or more SOREMPs in MSLT but also in patients with one SOREMP.

A prospective study of Willis-Ekbom disease/restless legs syndrome during and after pregnancy.

OBJECTIVES: Willis-Ekbom disease/restless legs syndrome (WED/RLS) is the most common sleep-related movement disorder in pregnancy. We designed a prospective longitudinal study to investigate the correlates of WED/RLS during and after pregnancy. DESIGN: A total of 138 pregnant women with WED/RLS and a control group of 251 age-matched pregnant women were enrolled prospectively. A questionnaire was administered during a face-to-face interview at first evaluation during pregnancy and three months after delivery. RESULTS: Among all women in the first trimester, 15.6% were diagnosed with WED/RLS, whereas 32.8% of those in the second trimester and 38.8% of those in the third trimester were diagnosed with WED/RLS (p = 0.032). In regression analysis, later gestational age [p < 0.001; odds ratio (OR) 1.054] and previous history of WED/RLS (p = 0.001; OR 2.795) were positively correlated with the presence of WED/RLS, while ferritin levels (p = 0.001; OR 0.956) were negatively correlated with the presence of WED/RLS. Ferritin levels were also negatively correlated with the International RLS Study Group severity index (p = 0.041). Forty-eight patients (34.8%) experienced WED/RLS symptomatology after delivery. The ferritin levels were lower, and the mean number of pregnancies was higher, in women with residual WED/RLS (p = 0.008). CONCLUSION: Our survey showed that WED/RLS was more common in the second and third trimesters. Emergence of WED/RLS during the second trimester was strongly associated with residual WED/RLS. Lower ferritin levels were associated with both WED/RLS in pregnancy and residual WED/RLS after delivery. A higher number of pregnancies were also associated with a greater likelihood of having residual WED/RLS after delivery.

Median nerve stiffness measurement by shear wave elastography: a potential sonographic method in the diagnosis of carpal tunnel syndrome.

OBJECTIVES: To measure the median nerve (MN) stiffness by quantitative shear wave elastography (SWE) at the carpal tunnel inlet and to determine whether SWE can be used in the diagnosis of carpal tunnel syndrome (CTS). METHODS: The study included 37 consecutive patients (60 wrists) with a definitive diagnosis of CTS and 18 healthy volunteers (36 wrists). The MN cross-sectional area (CSA) by ultrasound and stiffness by SWE were studied. The difference between CTS patients and controls, and the difference among subgroups based on electrodiagnostic tests were studied by the Student's t test. Interobserver variability and ROC analysis were performed. RESULTS: The MN stiffness was significantly higher in the CTS group (66.7 kPa) when compared to controls (32.0 kPa) (P < 0.001), and higher in the severe or extreme severity group (101.4 kPa) than the mild or moderate severity group (55.1 kPa) (P < 0.001). A 40.4-kPa cut-off value on SWE revealed sensitivity, specificity, PPV, NPV and accuracy of 93.3%, 88.9%, 93.3%, 88.9% and 91.7%, respectively. Interobserver agreement was excellent for SWE measurements. CONCLUSIONS: Median nerve stiffness at the carpal tunnel inlet is significantly higher in patients with carpal tunnel syndrome, for whom shear wave elastography appears to be a highly reproducible diagnostic technique. KEY POINTS: • Clinical examination is important for diagnosis of carpal tunnel syndrome • Shear wave elastography (SWE) offers new clinical opportunities within diagnostic ultrasound • SWE is highly reproducible in evaluation of median nerve stiffness • Median nerve stiffness is significantly increased in carpal tunnel syndrome • Elastography could become useful in diagnosis of carpal tunnel syndrome.