Distinctive sleep complaints and polysomnographic findings in antibody subgroups of autoimmune limbic encephalitis.

INTRODUCTION: Sleep disturbances are being increasingly recognized in association with autoimmune encephalitis (AIE). We investigated the prevalence of sleep-related symptoms and polysomnographic features of patients with AIE and the long-term outcomes in these patients in a multi-center, prospective study from Turkey. METHODS: We prospectively evaluated patients with definite AIE in a common database including demographics, AIE-related and sleep-related symptomatology. Maximum and latest modified Rankin scores (mRS) and Liverpool Outcome Score (LOS) were noted. RESULTS: Of 142 patients, 87 patients (61.3%) fulfilled the criteria for definite AIE (mean age, 46.8+18.8 years; 51.7% women; mean disease duration, 21.0+38.4 months). 78.9% of patients had at least one or more new onset or worsened sleep-related symptomatology: insomnia (55.3%), excessive daytime sleepiness (EDS, 28.0%), sleep apnea (18.7%), REM sleep behavior disorder (RBD, 17.3%), restless legs syndrome (10.7%) and oneiric stupor (9.3%). Sleep efficiency, N3 and REM sleep were decreased and N1 sleep was increased in patients with Ab[+] AIE. LOS points were highest in those with insomnia and sleep apnea, and lowest in those with EDS, RBD and oneiric stupor. RBD and sleep apnea were more common in anti-LG1 Ab[+] group than anti-NMDAR Ab[+] group. Index of periodic leg movements was highest in anti-LG1 Ab[+] group. Patients with EDS and oneiric stupor had more common memory problems. Maximum and latest mRS scores were positively correlated with EDS and oneiric stupor. EDS, RBD and oneiric stupor were negatively correlated with LOS points. CONCLUSION: Our study emphasizes the presence and importance of early diagnosis of sleep disturbances in AIE in regard to their deteriorative influences on disease prognosis.

Rapid eye movement dependency is associated with increased inflammatory activity in obstructive sleep apnea syndrome.

OBJECTIVE: Rapid eye movement (REM)-dependent obstructive sleep apnea syndrome (OSAS) is a specific subtype of OSAS having some phenotypic characteristics like a preference for a younger age, female gender, and milder severity. Such favorable features could make it possible to consider an overall benign course for this phenotype. However, accumulating data introduced its association with several cardiometabolic and vascular disorders recently. The primary objective of this study was to address the disease from the inflammation perspective and evaluate the potential inflammatory status in this variant via two accessible blood parameters: platelet distribution width (PDW) and systemic immune-inflammation index (SII). The secondary aim was to investigate whether this status, together with other disease characteristics, demonstrates consistency under different definitions of REM-dependent OSAS published previously. PATIENTS AND METHODS: The medical records of 35 patients with mild-to-moderate REM-dependent OSAS, 35 age- and sex-matched patients with REM-independent OSAS, and 25 non-OSA controls were retrospectively analyzed. Baseline features, polysomnographic characteristics, PDW, and SII were compared between the groups. Secondly, the analyses were repeated using different definitions of REM-dependent OSAS. Bivariate analyses were performed, and a multiple stepwise regression model was applied to adjust for body mass index (BMI) and cardiovascular risk (CVR) factors.  RESULTS: Mean PDW and SII were increased in patients with REM-dependent OSAS as compared to non-OSA controls (p = .022 and .029). The significance remained stable after adjustment for BMI and CVRs and was consistent according to different definitions. The Comparison of patients with REM-independent OSAS and non-OSA controls, as well as the two different subtypes of OSAS, did not yield significance. CONCLUSION: Based on the current findings, patients with REM-dependent OSAS appear to be susceptible to inflammation and should be carefully monitored for the negative consequences of that issue. To our knowledge, this study is the first to evaluate SII and PDW in REM-dependent OSAS.

Predictive value of red cell distribution width for overlap syndrome in obstructive sleep apnea.

Purpose: Obstructive sleep apnea syndrome (OSAS) and chronic obstructive pulmonary disease (COPD) are prevalent disorders, and the concurrence so-called overlap syndrome (OVS) is not rare either. Early recognition of OVS is essential because this group is more prone to cardiovascular morbidities and requires effective multidisciplinary follow-up. This study aimed to evaluate RDW in patients with severe OSAS and investigate whether it can predict OVS. Patients and methods: 96 patients were retrospectively analyzed, of whom 66 were found to have severe OSAS alone and 30 OVS during diagnostic workups. Demographic, polysomnographic, and laboratory results, including RDW, were compared between groups. Multivariate logistic regression was used to determine independent associates of OVS. Results: Gender and body mass index (BMI) were similar, however, the mean age and RDW were higher in the OVS group (p:0.008, p:0.002). The increase in RDW remained significant after adjustment for age, BMI, and cardiovascular risk factors. An RDW value of >13.65% was shown to have a 78.3% sensitivity and 60% specificity for predicting OVS in severe OSAS (p:0.004). Conclusion: The results suggest that RDW can be a reliable indicator for diagnosing OVS in OSAS. It can help in identifying the subset of patients who would benefit from proper consultations and multidisciplinary follow-up, leading to appropriate treatment of each disease component and effective monitoring to prevent adverse cardiovascular outcomes.

The REM-sleep-related characteristics of narcolepsy: a nation-wide multicenter study in Turkey, the REMCON study.

INTRODUCTION: Narcolepsy type 1 (NT1) is caused by hypocretin deficiency, the pathophysiology of narcolepsy type 2 (NT2) has not been delineated. Except for the hypocretin deficiency and cataplexy, all clinical and laboratory features used in the diagnosis of NT2 are identical to those used for NT1. The aim of this study was to assess the rapid eye movement (REM) sleep-related characteristics in the patients with narcolepsy; the characteristics of REM sleep in polysomnography (PSG) and multiple sleep latency test (MSLT) recordings, the quantification of REM sleep without atonia (RSWA) and atonia index, and the analysis of rapid eye movements (REMs) during REM sleep. MATERIALS AND METHODS: This study was planned by the Sleep Medicine Study Group of the Turkish Neurology Society, and conducted in 11 centers in eight cities in Turkey. The analysis of RSWA was analyzed by reviewing all REM sleep periods on nocturnal PSG and MSLT recordings per standard criteria. The total duration of the increased muscle tone during REM sleep in the chin and bilateral leg electromyography (EMG) recordings was calculated as RSWA index. The REMs index was also investigated the relation to the RSWA. RESULTS: A total of 274 patients were involved; 147 patients (53.6%) were males and 127 patients (46.4%) were females; the mean age was 29.1 ± 12.0 years. The diagnosis of NT1 was made in 166 patients (60.6%), and 108 patients (39.4%) were diagnosed as having NT2. The mean Epworth sleepiness scale score was significantly higher in patients with NT1 than the patients with NT2 (P = 0.001). The diagnosis of REM sleep behavior disorder (RBD) was made in 19.3% of the patients with NT1 versus in 2.8% of the patients with NT2 (P < 0.001). The percentage of SOREMP in PSG recordings was significantly higher in patients with NT1 (37.1%) than those with NT2 (18.9%, P = 0.001). MSLT showed that the mean sleep latency was shorter in patients with NT1 compared to those with NT2 (P < 0.001). The total duration of REMs on electrooculography recordings was also significantly higher in patients with RSWA in comparison with the patients without RSWA (P = 0.002). Total duration of REMs was significantly and positively correlated with the duration of RSWA on chin-EMG and leg-EMG recordings (P = 0.001). ROC analyses showed an RSWA index of ≥2% for the RSWA on chin-EMG with a sensitivity of 86.7% and a specificity of 71.3% (P < 0.001). The REMs index ≥20% was associated with the presence of RSWA with a sensitivity of 70.0% and a specificity of 57.1% (P = 0.008). CONCLUSIONS: In this nation-wide study, we identified for the first time that the increase in REMs density during REM sleep may be a major correlate of the RSWA. Significant positive correlations were demonstrated between the total duration of REMs on electrooculography recordings and the mean durations of RSWA in both chin and leg EMG recordings. A REMs index of >20% was demonstrated to have a moderate sensitivity and specificity in the diagnosis of RSWA. As observed in chin RSWA index, REMs index also showed a significantly high association with RBD, in comparison to RSWA per standard criteria.

Screening Fabry's disease in chronic kidney disease patients not on dialysis: a multicenter study.

OBJECTIVES: Fabry's disease is an X-linked inherited, rare, progressive, lysosomal storage disorder, affecting multiple organs due to the deficient activity of α-galactosidase A (α-Gal A) enzyme. The prevalence has been reported to be 0.15-1% in hemodialysis patients; however, the information on the prevalence in chronic kidney disease not on dialysis is lacking. This study aimed to determine the prevalence of Fabry's disease in chronic kidney disease. METHODS: The patients older than 18 years, enclosing KDIGO 2012 chronic kidney disease definitions, not on dialysis, were enrolled. Dried blood spots on Guthrie papers were used to analyze α-Gal A enzyme and genetic analysis was performed in individuals with enzyme activity ≤1.2 µmol/L/h. RESULTS: A total of 1453 chronic kidney disease patients not on dialysis from seven clinics in Turkey were screened. The mean age of the study population was 59.3 ± 15.9 years. 45.6% of patients were female. The creatinine clearance of 77.3% of patients was below 60 mL/min/1.73 m2, 8.4% had proteinuria, and 2.5% had isolated microscopic hematuria. The mean value of patients' α-Gal A enzyme was detected as 2.93 ± 1.92 µmol/L/h. 152 patients had low levels of α-Gal A enzyme activity (≤1.2 µmol/L/h). In mutation analysis, A143T and D313Y variants were disclosed in three male patients. The prevalence of Fabry's disease in chronic kidney disease not on dialysis was found to be 0.2% (0.4% in male, 0.0% in female). CONCLUSION: Fabry's disease should be considered in the differential diagnosis of chronic kidney disease with unknown etiology even in the absence of symptoms and signs suggestive of Fabry's disease.

Combined surgical treatment for severe sleep apnoea, to improve BiPAP compliance.

Positive airway pressure (PAP) devices are used in the treatment of obstructive sleep apnoea syndrome (OSAS). In cases of PAP failure, many different surgical methods can be used for the treatment. The authors present an unusual case of a patient with Bi-level PAP (BiPAP)-intolerant severe OSAS who was treated with combined surgical methods. A 55-year-old man was treated with BiPAP due to OSAS; he was admitted to the clinic with nose stuffiness, respiratory distress and BiPAP adherence with tolerance and compliance problems. Septal deviation, concha hypertrophy, lateral pharyngeal band hypertrophy and Thornwaldt cyst were determined in the examination. Combined surgical methods were administered. The patient's apnoea hypopnoea index (AHI) was 72.8 in diagnostic polysomnography. Preoperative AHI was 7.3 and postoperative AHI was 2.3 while using BiPAP and, after the surgery, the BiPAP intolerance was eliminated. The authors suggest that a combination of different surgical methods would be an adjuvant treatment to increase BiPAP compliance.

Evoked potentials and disability in multiple sclerosis: A different perspective to a neglected method.

UNLABELLED: Evoked potentials and disability in multiple sclerosis: a different perspective to a neglected method. OBJECTIVE: Because evoked potentials (EP) are reflections of the functional integrity of sensory-motor systems, they are expected to reflect the abnormality in patients with disabilities and handicaps and also be in correlation with scales. This assumption was tested. METHODS: Patients with multiple sclerosis (MS) and myelopathy (M) and normal controls were investigated by EP, Multiple Sclerosis Walking Scale-12, timed 25-foot walk test and extended disability status scale (EDSS). EP results were converted to ordinal values, and correlations of these values with scales were calculated. Sensitivity and specificity analysis of EP parameters was also performed. RESULTS: Total EP scores revealed high rates of abnormality in both groups, but MS revealed a different correlation pattern from M. The SEP+MEP summed score showed high sensitivity and specificity for MS and this was also correlated with the MS-related disability-ambulation scales including EDSS. The most specific parameter was the minimum M latency in the MEP study. CONCLUSIONS: Four extremity recordings of EP with the use of more parameters than usual and ordinal expression of results seem to be benefical in MS. Although this study was cross sectional in nature, results indicated that EP might be useful in clinical follow up.

Acute motor-sensory axonal neuropathy with hyperreflexia in Guillain-Barré syndrome.

Guillain-Barré syndrome is an acute inflammatory autoimmune polyradiculoneuritis. Progressive motor weakness and areflexia are essential for its diagnosis. Hyperreflexia has rarely been reported in the early healing period of Guillain-Barré syndrome following Campylobacter jejuni infection in patients with acute motor axonal neuropathy with antiganglioside antibody positivity. In this study, we report a 12-year-old girl presenting with complaints of inability to walk, numbness in hands and feet, and hyperactive deep tendon reflexes since the onset of the clinical picture, diagnosed with acute motor-sensory axonal neuropathy type of Guillain-Barré syndrome.

Association of central pontin myelinolysis and extra-pontin myelinolysis in diabetic patient.

Central pontin myelinolysis and extra-pontin myelinolysis (CPM/EPM) implies the clinical picture after rapid recovery of hyponatremia or hyponatremia alone. SPM/EPM picture can occur without hyponatremia. Findings such as dysarthria, dysphagia, tetraparesia/plegy accompanies the clinical table. Our case was also diabetic, but there were no electrolyte imbalance in admission. The presence of diarrhea in medical history of the patient might indicate a short-term undetected electrolyte imbalance. In our diabetic case that has borderline metabolic status and lack of any detected electrolyte imbalance, we decided to present SPM/EPM table together with literature information.

Vascular Parkinsonism: a case of lacunar infarction localized to mesencephalic substantia nigra.

An 80-year-old caucasian man with acute onset parkinsonism is reported here. Lower limbs were predominantly involved and he did not show remarkable improvement with dopaminergic therapy. His MRI investigation showed an isolated lesion on the right side of the mesencephalon, consistent the localization of substantia nigra. The association of acute onset parkinsonism and this radiological finding is very rare in the literature.