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   Table of Contents - Current issue
Coverpage
January-March 2022
Volume 39 | Issue 1
Page Nos. 1-59

Online since Thursday, March 31, 2022

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REVIEW ARTICLE  

Neurophysiological testing in the diagnosis of amyotrophic lateral sclerosis p. 1
David Burke
DOI:10.4103/nsn.nsn_199_21  
Neurophysiological testing plays a very important role in the diagnosis of amyotrophic lateral sclerosis (known in the British world as motor neuron disease). As specified in the Awaji criteria, electromyography is critical for defining the neurogenic changes due to involvement of the lower motor neuron (LMN), and it can do so for muscles that are not involved clinically or are so only minimally. Demonstrating LMN involvement can be enhanced by the judicious use of neuromuscular ultrasound and imaging, particularly whole-body magnetic resonance imaging. There is a gap with involvement of the upper motor neuron (UMN), with promising procedures yet to be adopted widely. Reflex function can be used to demonstrate hyperreflexia and sometimes that paresis is at least partly of UMN origin. Protocols using transcranial magnetic stimulation can demonstrate enhanced excitability of interneuronal circuits in motor cortex and thereby pathology involving the UMN. The motivation behind studies using these and other techniques is to be able to make the diagnosis before the disease has spread significantly from its site of onset, when the clinical deficit is still minor.
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ORIGINAL ARTICLES Top

Could we Predict Respiratory Failure in Amyotrophic Lateral Sclerosis? p. 8
Ceren Cetin Akkoc, Şeyma Aykaç, Fikret Bademkıran, İbrahim Aydoğdu, Sezai Taşbakan
DOI:10.4103/nsn.nsn_210_21  
Introduction: Respiratory complications are important in the prognosis of amyotrophic lateral sclerosis (ALS). The aim of this study was to determine the electrophysiological findings that may predict respiratory failure. Methods: According to the Awaji electrodiagnostic criteria, 30 patients with ALS who were diagnosed with definite or probable ALS without respiratory failure were included in the study. Nerve conduction studies, needle electromyography (EMG), and single-breath count tests were performed in all patients. In addition, the pulmonary function tests, swallowing EMG, and arterial blood gas analysis of the patients were recorded and evaluated. The patients were followed until respiratory failure developed. Results: As a result of 18 months of follow-up, 26 of 30 patients developed respiratory failure. When the contribution of the accessory respiratory muscles to the respiratory effort before the development of respiratory failure was evaluated clinically and electrophysiologically, it was observed that the most common muscles involved in the respiratory effort were sternocleidomastoid (SCM), trapezius, and rectus abdominis. Before the development of respiratory failure, the latest neurogenic EMG findings were seen in the SCM (50% cases), trapezius (20% cases), and thoracic paraspinal muscles (17% of cases), respectively. It was thought that this finding could be an important early electrophysiologic marker in predicting the development of respiratory failure in ALS cases. Conclusions: To sum up, the presence of neurogenic changes in certain muscles in needle EMG and demonstration of the contribution of certain accessory respiratory muscles in respiration can be used as an electrophysiological marker to predict the development of respiratory failure.
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Tremor and myoclonus is common in immune-mediated and hereditary polyneuropathies p. 14
Damla Cetinkaya Tezer, Ayşegül Gündüz, Melih Tütüncü, Mehmet Ali Akalın, Nurten Uzun Adatepe, Feray Karaali Savrun, Meral Erdemir Kızıltan
DOI:10.4103/nsn.nsn_99_21  
Objective: Our goal was to identify the frequency and types of involuntary movements in immune mediated and hereditary polyneuropathies. Methods: In this prospective study, we included all consecutive patients with immune mediated or hereditary polyneuropathy between January 2017 and November 2019. The presence and type of the involuntary movements were determined by the clinical examination and multichannel surface electromyography. Results: We identified 23 (48.9%) patients with involuntary movements among 47 patients with immune mediated or hereditary polyneuropathy in the study period. All patients with an involuntary movement had postural tremor with accompanying action and/or rest tremor. Short duration and high-amplitude myoclonus was accompanying in 18 (38.3%) patients. The demographic and clinical characteristics and features of nerve conduction studies were similar between patients with and without involuntary movements. Discussion: Tremor and myoclonus were frequent in our cohort. Postural tremor was the most frequent subtype. There was no significant relationship between myoclonus or tremor and clinical/electrophysiological features. Conclusion: For assessing movement disorders in polyneuropathies not only clinical examination, but also electrophysiological studies such as multichannel surface electromyography should be used.
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The Electroencephalographic Evolution of Electrical Status: Is it Possible to Diagnosis ESES from 180 Seconds of Sleep? p. 21
Habibe Koc Ucar, Ebru Arhan, Kürşad Aydın, Tuğba Hirfanoğlu, Ayşe Serdaroğlu
DOI:10.4103/nsn.nsn_136_21  
Purpose: Electrical status epilepticus during slow sleep (ESES) is an electroclinical syndrome with a specific electroencephalogram (EEG) pattern characterized by epileptic seizures, cognitive decline, and behavioral problems. The EEG pattern is defined by the percentage of the spike-wave index (SWI) in nonrapid eye movement (NREM) sleep without a clear cut-off value. The purpose of this study is to determine the significance of SWI calculation in the first 180 s of the NREM sleep stage. Methods: Patients with tonic seizures and those with SWI levels of <50% were excluded from the study. One hundred patients were enrolled in the study (typical ESES: 85; atypical ESES: 15). EEG findings were evaluated according to the following points: 1-ESES type: atypical ESES for SWI between 50% and 85% or typical ESES for ≥85%; 2-SWI calculation methods: Short method and long conventional method; 3-SWI percentage and spike frequency (SF). Results: A moderate correlation was determined between spike-wave percentage (SWP) and SF (r = 0.628; P < 0.001). A strong positive correlation was determined between the short method and long conventional method (r = 0.888; P < 0.001). In multivariate logistic regression with the SWI short method and the number of spikes in the first 180 s of NREM, only the SWI short method was found to predict typical ESES regardless of other factors (odds ratio: 1.18; P = 0.001). The optimal predictive value of the SWI short method for predicting typical ESES was >85, with sensitivity of 81.2%, and specificity of 73.3% (+PV: 94.5%, −PV: 40.7%; AUC ± SE = 0.850 ± 0.05; P < 0.001). Conclusion: Evaluating EEG epileptiform activities with objective and reproducible well-defined measurements such as SWP and SF allows for the comparison of different patient groups. We think that a shorter method for diagnosing ESES would potentially provide increased cost savings and patient comfort.
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Evaluation of the relationship between epileptic seizures and type of parenchymal lesion in patients with cerebral venous thrombosis p. 28
Yasemin Dinc, Aylin Bican Demir, Mustafa Bakar, İbrahim Bora
DOI:10.4103/nsn.nsn_139_21  
Introduction: Epileptic seizures occur in approximately 35%–40% of patients with cerebral venous thrombosis (CVT). The relationship between parenchymal lesions and epileptic seizures in CVT has been investigated, but the most associated types of parenchymal lesions have not been determined. This study, therefore, aimed to identify high-risk groups. Methods: A total of 159 patients were diagnosed as having CVT between 2015 and 2021 at our tertiary center. The risk factors for epileptic seizures after CVT were determined. Results: A total of 159 patients who were diagnosed with having CVT, 109 (68.5%) females and 50 (31.5%) males, were included in this study. The mean ages of the women and men were 41.20 ± 14.15 years and 43.60 ± 16.30 years, respectively. We found that superior sagittal sinus involvement (P = 0.019), sigmoid sinus involvement (P = 0.010), cortical vein involvement (P < 0.001), parenchymal lesion (P < 0.001), and the postpartum period (P = 0.003) increased the risk of epileptic seizures. When the significant variables associated with epileptic seizures in the patients were analyzed using binary logistic regression, the most significant variable was found to be the presence of parenchymal lesions. Conclusion: We found that the most significant variable for epileptic seizures after CVT was parenchymal lesions. Juxtacortical hemorrhages and nonhemorrhagic venous infarcts were the most common causes of epileptic seizures. CVT is a heterogeneous group of diseases caused by multiple aetiologies and may show ethnic and racial differences. For this reason, more precise information can be obtained with multi-center prospective studies in our population.
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Periodic legs movements in sleep persisting after positive airway pressure titration is significantly associated with cardiovascular diseases p. 35
Aygul Mahmudova, Hatice Kurucu, Gulcin Benbir Şenel, Derya Karadeniz
DOI:10.4103/nsn.nsn_149_21  
Purpose: Obstructive sleep apnea syndrome (OSAS) and periodic limb movement disorder in sleep (PLMS) are highly prevalent diseases of sleep and both are associated with a negative cardiovascular outcome. The purpose of this study was to investigate the presence of PLMS in patients with OSAS in positive airway pressure (PAP) titration and examine its relation to cardiovascular diseases. Materials and Methods: All patients who were diagnosed as having OSAS after one-night polysomnographic (PSG) recording during a 1-year period and were been treated successfully through PAP titration PSG were investigated retrospectively. Results: Out of 460 medical files investigated, 300 patients were included in the study (204 males, 68%) with a mean age of 54.2 ± 10.6 years. A comparison between patients with a PLMS index >15/h and PLMS index <15/h revealed that cardiac diseases (P = 0.040), hypertension (HT) (P = 0.006), and hyperlipidemia (P = 0.019) were all more common if patients had a PLMS index >15/h. Furthermore, a significant correlation was detected between the PLMS index and cardiac diseases (ß = 39.908, P = 0.012) and HT (ß = 32.884, P = 0.021). Conclusions: The risk of cardiovascular diseases is markedly increased in patients with OSAS with PLMS, and this was positively correlated with the PLMS index. Physicians should be aware that PLMS persisting after PAP titration might prevent the efficiency of PAP therapy in OSAS, especially in preventing cardiovascular complications.
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The effect of risk factors on the clinical course and treatment of older patients with coronavirus disease 2019 p. 40
Ulkuhan Duzgun, Ali Rıza Sonkaya, Bilgin Öztürk, Oktay Sarı, Eray Serdar Yurdakul, Ümit Savaşçı, Deniz Doğan, Ömer Karadaş
DOI:10.4103/nsn.nsn_114_21  
Introduction: Coronavirus disease 2019 (COVID-19) is known to have higher morbidity and mortality rates, parallel to the increased risk factors in the elderly. We aimed to define the risk factors related to mortality and morbidity in older patients hospitalized with COVID-19 disease in this study. Materials and Methods: This retrospective cross-sectional study included patients aged ≥65 years who were hospitalized with a confirmed diagnosis of COVID-19. We analyzed their demographic data, clinical findings, comorbidities, laboratory and radiologic findings, treatment protocols, and outcomes. Results: A total of 58 patients were included in the study. A total of eight (13.8%) patients died during the clinical follow-up and treatment, and 50 (86.2%) patients were discharged. The most common comorbidities among all patients were hypertension (HT) (69%) and diabetes mellitus (39.7%). The most common symptoms include fever (51.7%), cough (44.8%), and dyspnea (43.1%), and the most common neurologic findings were headache (27.6%) and impaired consciousness (27.6%). Intensive care unit admission was significantly higher among patients with comorbidities of HT, cerebrovascular disease, atrial fibrillation (AF), and chronic obstructive pulmonary disease. The rate of death was significantly higher in patients with a history of smoking, cerebrovascular disease, AF, and HT. Although there was a statistically significant positive correlation between the death rate and leukocyte, neutrophil, C-reactive protein, lactate dehydrogenase, D-dimer, interleukin-6, and procalcitonin levels, a negative correlation was observed in lymphocyte levels. Conclusion: Age-related comorbid conditions, especially HT, cerebrovascular disease, and AF, caused increased morbidity and mortality rates in older patients with COVID-19.
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Evaluation of brain death due to methanol intoxication p. 48
Tugce Mengi, Hüseyin Özkök, Özlem Öner, Erdem Yaka, Bilgin Cömert, Ali Necati Gökmen
DOI:10.4103/nsn.nsn_128_21  
Objective: Intoxication may mimic brain death and cause brain death. In the literature, brain death due to methanol intoxication is limited to case reports. In this report, patients with methanol intoxication who had findings of imminent brain death were evaluated. Materials and Methods: The study population consisted of patients with methanol intoxication treated in the adult intensive care unit (ICU) between October 2014 and October 2020. The records in the hospital automation system of patients with methanol intoxication were evaluated retrospectively. According to the outcomes, the patients were divided into two groups: survivors and patients who had imminent brain death. Results: Eighteen patients with methanol intoxication were investigated. The brainstem reflexes disappeared in seven patients who were in a coma. The imminent brain death rate of patients with methanol intoxication was 39%. Patients who had imminent brain death had lower Glasgow Coma Scale scores during ICU admission and a higher ratio of pathologic neuroimaging findings due to methanol intoxication (P < 0.05). According to the clinical criteria and ancillary tests, four patients were declared brain dead. The brain death rate of patients with methanol intoxication was 22%. One of four patients with brain death was an organ donor. The liver was transplanted from our donor. Conclusion: Treatment should be initiated immediately in cases of methanol intoxication. Patients who do not respond well to treatment should be followed closely in terms of brain death. Based on published data and our personal experience, organ donations can be performed after appropriate investigations in brain death cases due to methanol intoxication.
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LETTERS TO EDITOR Top

A patient with glucose transporter type 1 deficiency syndrome: Paroxysmal choreoathetosis and cerebral positron-emission tomography findings p. 53
Zuhal Yapici, Pinar Topaloğlu, Cuneyt Turkmen, Mefkure Eraksoy, Sameer Zuberi
DOI:10.4103/nsn.nsn_127_21  
Glucose transporter type 1 deficiency syndrome (GLUT-1 DS) is an inborn error of metabolism that results in defective glucose transport and consequently a reduced supply of glucose to the brain. Here, we describe a patient with a molecularly proven GLUT-1 mutation who presented with severe paroxysmal choreoathetosis. Different regional changes involving bilateral mesial temporal lobes were revealed using positron-emission tomography (PET). Several cases of GLUT-1 DS have been studied from the point of view of hyperkinetic movement disorders rather than epilepsy and ataxia. It is usual for these patients to first present with dystonia, choreoathetosis, parkinsonism, and paroxysmal exercise-induced dyskinesia, including dystonic, choreoathetotic, and ballistic movements. F18-fluorodeoxyglucose PET revealed hypometabolism in bilateral mesial temporal lobes along with the cerebellar cortex, confirming an impaired glucose metabolism effect on the area responsible for the extrapyramidal movement disorders.
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A rare paraneoplastic finding of the breast cancer and chronic inflammatory demyelinating polyneuropathy p. 56
Basak Elcin Ates, Kezban Aslan-Kara, Melek Ergin, Hacer Bozdemir
DOI:10.4103/nsn.nsn_123_21  
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REVIEWERS Top

Reviewers for the Year: 2021 p. 59
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