Isolated abducens nerve palsy: Comparison of microvascular and other causes.

OBJECTIVE: Abducens nerve paralysis is the most common ocular motor neuropathy. In this article, we aimed to compare the causes of isolated abducens nerve palsy in terms of demographic, clinical features, and prognosis. METHODS: Thirty-six isolated abducens nerve palsy patients were prospectively enrolled in the study. The demographic, clinical features, and prognosis compared in two etiological groups as microvascular and other causes. RESULTS: The most common etiology was microvascular, which was seen in 16/36 (44.4%) patients. Mean clinical recovery time was 2.5±1.3 months (range, 10 days-6 months). When etiological groups were compared as microvascular and other causes, the mean age of the microvascular group was significantly higher (62.8±13.3 vs. 44.5±16.4, p=0.001). Diabetes mellitus was seen significantly higher in the microvascular group than other causes group (p=0.001), but no significant difference was observed in terms of other atherosclerotic risk factors (p>0.05). The fasting blood glucose and hemoglobin A1c value were significantly higher in the microvascular group (p=0.02 and p=0.02, respectively). There was no significant difference in terms of clinical improvement and clinical recovery times between groups (p>0.05). CONCLUSION: There is no difference between microvascular group and other causes in terms of clinical outcome, while the mean age and presence of diabetes were higher in the microvascular group. The presence of diabetes should be questioned in cases with isolated abducens nerve palsy.

Autonomic dysfunction in patients with COVID­19.

PURPOSE: Autonomic dysfunction in patients with viral infections has been described before. In this study, we aimed to evaluate autonomic functions in patients with the coronavirus infectious disease 2019 (COVID-19). METHODS: In this cross-sectional study, we compared 112 patients who had recovered from COVID-19 and 106 healthy controls. Symptoms of autonomic dysfunction were assessed with the SCOPA-AUT scale. RESULTS: Pupillomotor, urinary and sudomotor subscores of SCOPA-AUT scale were significantly higher in the COVID-19 patient group (p = 0.03, p = 0,006, p = 0.0001, respectively). There were no significant difference in terms of gastrointestinal, cardiovascular, sexual subscores and total SCOPA-AUT scores between the patient and control groups. The presence of fatigue symptom in the acute phase of COVID-19 increased the total SCOPA-AUT score by 2.2 points (p = 0.04) whereas the presence of smell loss (OR = 5.82, p = 0.01) and dyspnea (OR = 5.8, p = 0.03) were significant risk factors for pupillomotor dysfunction. The urinary, cardiovascular, sexual subscores and the total score of SCOPA-AUT scale were positively correlated with the age of the patient group. CONCLUSION: Our study suggests that many patients might have prolonged symptoms of autonomic dysfunction after the acute phase of COVID-19 that might worsen the clinical recovery.

Headache in idiopathic/genetic epilepsy: Cluster analysis in a large cohort.

OBJECTIVE: The link between headache and epilepsy is more prominent in patients with idiopathic/genetic epilepsy (I/GE). We aimed to investigate the prevalence of headache and to cluster patients with regard to their headache and epilepsy features. METHODS: Patients aged 6-40 years, with a definite diagnosis of I/GE, were consecutively enrolled. The patients were interviewed using standardized epilepsy and headache questionnaires, and their headache characteristics were investigated by experts in headache. Demographic and clinical variables were analyzed, and patients were clustered according to their epilepsy and headache characteristics using an unsupervised K-means algorithm. RESULTS: Among 809 patients, 508 (62.8%) reported having any type of headache; 87.4% had interictal headache, and 41.2% had migraine. Cluster analysis revealed two distinct groups for both adults and children/adolescents. In adults, subjects having a family history of headache, ≥5 headache attacks, duration of headache ≥ 24 months, headaches lasting ≥1 h, and visual analog scale scores > 5 were grouped in one cluster, and subjects with juvenile myoclonic epilepsy (JME), myoclonic seizures, and generalized tonic-clonic seizures (GTCS) were clustered in this group (Cluster 1). Self-limited epilepsy with centrotemporal spikes and epilepsy with GTCS alone were clustered in Cluster 2 with the opposite characteristics. For children/adolescents, the same features as in adult Cluster 1 were clustered in a separate group, except for the presence of JME syndrome and GTCS alone as a seizure type. Focal seizures were clustered in another group with the opposite characteristics. In the entire group, the model revealed an additional cluster, including patients with the syndrome of GTCS alone (50.51%), with ≥5 attacks, headache lasting >4 h, and throbbing headache; 65.66% of patients had a family history of headache in this third cluster (n = 99). SIGNIFICANCE: Patients with I/GE can be clustered into distinct groups according to headache features along with seizures. Our findings may help in management and planning for future studies.

An Unusual Cause of Internal Carotid Artery Dissection: Mobile Phone Misuse.

The use of mobile phones has become an indispensable part of our lives, especially due to widespread use of the internet. We report the case of a 38-year-old male patient who developed internal carotid artery dissection after talking on the phone between her left shoulder and ear by laterally flexing the neck for 20 minutes. In addition to many positive effects of technology that facilitate the daily life, the development of neurological deficits may be observed with widespread use of mobile phones. Misuse of mobile phone should be considered in patients with carotid artery dissection.

The assessment of cognitive functions in patients with isolated cerebellar infarctions: A follow-up study.

The role of the cerebellum on cognitive functions have been well-defined; however, the information related to the progress in time process is limited. In this study, we aimed to evaluate the cognitive function of patients with isolated cerebellar infarction in both the acute stage and the follow-up period. Twenty-three patients with isolated cerebellar infarction and 22 healthy control were examined through an extensive neuropsychological assessment battery. The patients were evaluated in the acute stage and at least six months after the stroke in the follow-up period. There were no significant differences between the patients and the controls regarding age (52.2 ± 7.0 and 54.9 ± 6.6, p = 0.184) and gender (Female/Male: 6/17 and 7/15, p = 0.672). There was no statistically significant difference between patients with right cerebellar infarction and left cerebellar infarction in terms of cognitive functions. Verbal fluency, attention, and verbal and non-verbal episodic memory scores were significantly lower in patient group in the acute stage when compared to the control group. When the follow-up evaluation was compared to acute stage, it was revealed that patients had recovered in all areas; however, less improvement was seen in word reading time. Our results support that lesions of the cerebellum affect cognitive functions in the acute stage. However, the improvement was demonstrated in all cognitive functions in the follow-up period.

Cervical vestibular-evoked myogenic potentials in patients with essential tremor.

Although different neuroanatomical structures and pathways are emphasized as possible explanations for essential tremor (ET), there is still an ongoing debate. This study aimed to assess the role of brainstem and reflex pathways with cervical vestibular-evoked myogenic potentials (VEMP) in patients with ET. This prospective study included 34 patients with ET and 25 healthy controls. Cervical VEMP was performed in both groups and latencies, inter-peak latency intervals, peak-to-peak amplitudes and asymmetry ratios were recorded. There was statistically no significant difference between the groups in terms of age (38.9 ± 14.9 years vs. 38.9 ± 14.9 years, p = 0.673) and gender (female to male ratio: 14/11 vs. 20/14, p = 0.828). Right N1 latency and right N1-P1 interval were significantly longer in the patient group (p < 0.05). There was a significant positive correlation between the duration of disease and the right N1-P1 interval (p < 0.05). There was no significant difference between the patient and control groups in terms of bilateral P1 latency, left N1 latency, left N1-P1 interval, and bilateral N1 and P1 amplitudes (p˃0.05). Cervical VEMP may reveal the involvement of brainstem and associated reflex pathways in ET.

Restless Legs Syndrome in Multiple Sclerosis.

INTRODUCTION: The co-existence of Restless Legs Syndrome/Willis-Ekbom Disease (RLS/WED/WED) and multiple sclerosis (MS) is a common condition. For this reason, we aimed to evaluate the effects of RLS/WED and its relationship with MS. METHODS: We evaluated the clinical features of 102 patients diagnosed with MS who were in follow-up between 2010 and 2015 in outpatient clinic. All the patients were evaluated for RLS/WED according to the revised International Restless Legs Syndrome Study Group (IRLSSG) diagnostic criteria. The Expanded Disability Status Scale (EDSS), Beck Depression Inventory, Beck Anxiety Inventory and Fatigue Severity Scale scores of all the patients were recorded. The IRLSSG 2003 severity scale was used to determine the degree of RLS/WED. RESULTS: RLS/WED was detected in 30.4% (n=31) of the patients (MS-RLS/WED+), but not in 69.6% (n=71) (MS-RLS/WED-). The mean EDSS score of the MS-RLS/WED+ patients were 3.2±2.1 while the MS-RLS/WED-patients were 2.0±1.6. The incidences of depression, moderate or severe anxiety, fatigue and intestinal and bladder dysfunction in the MS-RLS/WED+ patients were significantly higher. Regarding to RLS/WED complaints, 32.2% were mild, 35.4% were moderate, 19.3% were severe and 12.9% were very severe. When the MS subgroups were evaluated the highest RLS/WED severity score was found in the secondary progressive MS group. In the patients with pyramidal symptoms and intestinal and bladder dysfunction, the mean RLS/WED severity was significantly higher. The mean RLS/WED severity score was also significantly higher in those with depression and anxiety. The RLS/WED severity was significantly correlated with the number of pyramidal attacks and the EDSS score. CONCLUSION: Restless legs syndrome is a cause of depression, anxiety and fatigue and has negative effects on MS patients. Therefore, after the diagnosis of MS, the RLS/WED symptoms and signs should be determined, as soon as possible, in addition to the other MS symptoms. The treatment of this condition should be started early.

Endocan: A Novel Predictor of Endothelial Dysfunction in Silent Brain Infarction.

Silent brain infarction (SBI) has been considered as a subclinical risk factor for symptomatic possible future stroke. We investigated the association between serum inflammatory markers and SBI. Patients (n = 54) diagnosed with SBI as the study group and 52 individuals as the control group were included in this study. Silent brain infarction is defined as a hyperintense lesion that was ≥3 mm in 1 dimension on fluid-attenuated inversion recovery T2-weighted magnetic resonance image, if the patient had normal neurological examination or had an abnormality that was not consistent with the brain lesion locations, after being evaluated by a neurologist. Serum endocan levels (P = .036) and high-sensitivity C-reactive protein (hsCRP; P = .022) were significantly higher in patients with SBI than the controls. Pentraxin 3, erythrocyte sedimentation rate, white blood count, lymphocyte, monocyte, neutrophil, low-density lipoprotein, and triglyceride levels were not significantly different when comparing the groups with and without SBI. There was a significant correlation (r = -0.196; P = .16) between hsCRP and endocan levels in the SBI group. Endocan, a novel biomarker of endothelial pathology, was significantly increased in patients with SBI and may be useful to predict the future risk of stroke.

The simple and fast swallowing function assessment in acute stroke patients.

OBJECTIVE: The dysphagia associated pulmonary aspiration is one of the important reasons for mortality and morbidity in stroke. In this study, we evaluated the early swallowing functions of the acute ischemic stroke patients, and tried to choose the right way to start feeding with simple tests. METHODS: Seventy-three inpatients with acute stroke were included in this study. Age, gender, type of stroke, NIHSS and RANKIN scores, risk of aspiration and feeding route were recorded for all the subjects. Dysphagia was evaluated with the bedside clinical evaluation of swallowing function score (BDS) tests. These BDS tests are the assessment of dysphagia with neurological examination score (DSNE) and the bedside water drinking test (BWT) and the Swallowing score (SS) ratio (combining BWT and DSNE scores). All tests to evaluate swallowing were planned to be carried out 24 hours after the last known time of the patient's healthy and 48 hours after hospitalized. The tests were performed in awake patients who were able to manage to cooperate at the scheduled time. In addition, stroke patients were evaluated quantitatively using the Gugging Swallowing Screen (GUSS) test for dysphagia and compared with BDS tests. All patients were evaluated for aspiration pneumonia seven days after admission. If the patients had drowsiness or were unable to cooperate, they were not included in this study. RESULTS: Seventy-three (26F/47M) patients were included in this study if they were conscious and the Glasgow coma scale was above 10 points. When only BDS tests were performed, we decided that 74% (n=54) of the patients could be fed by the oral route, 13.7% of the patients could be fed only by NG route (n=10) and the patients who had the worst BWT and DSNE scores preferred to be feed with PEG route (11% of all the patients, n=8). In 41.1% of the patient (n=30) established the risk of aspiration on referral clinic and 23.3% of the patients (n=17) developed aspiration pneumonia in the clinical follow-up. When 30.1% (22) of the patients had dysphagia with GUSS test, 23.3% (n=17) of the patients were dysphagic with DSNE and 30.1% of the patients (n=22) were dysphagic with BWT and 22% (n=16) of the patients were moderate-severe, 11% (n=8) of the patients were mild dysphagic with the SS ratio. CONCLUSION: These BDS tests concluded are fast and reliable methods for evaluating the dysphagia and risk of aspiration pneumonia without laborious and very few clinically applicable methods, such as endoscopic or video fluoroscopy, in patients who are hospitalized with stroke.

Wernicke's Encephalopathy: Similar and Different Aspects of the Three Cases.

Wernicke encephalopathy is a neurological disorder, clasically characterized by altered consciousness, ophtalmoparesis, and ataxia results from tiamin deficiency. It is frequently associated with chronic alcohol abuse; however, many conditions which lead to thiamine deficiency such as gastric surgery, hyperemesis, parenteral nutrition, malnutrition may also be the cause. WE is a life-threatening condition that requires early diagnosis and rapid initiation of intravenous thiamin therapy. In patients, findings of characteristic examinations may not always be seen; therefore, imaging methods are of great importance. In this article, we aimed to emphasize the similar and different aspects of three WE cases with typical and atypical imaging findings.

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.

An uncommon neurological manifestation of chronic lymphocytic leukemia: Longitudinally extensive transverse myelitis.

We describe a 50-year-old male patient who was admitted to the emergency department with complaints of fever and fatigue that had suddenly started two weeks ago. In the laboratory evaluation, a white blood cell count of 131.000/mm3 was detected. The patient was hospitalized and developed fecal incontinence on the first day of hospitalization. Detailed neurological examination revealed the patient had tetraparesis. Long segment high signal intensity was observed on spinal MRI. Flow cytometry examination of the CSF and biopsy findings of the bone marrow were compatible with Chronic Lymphocytic Leukemia (CLL). The patient's MRI appearances resolved after treatment. The tetraparesis resolved partially. There was no motor deficit in upper extremities and the patient was able to walk without aid or rest for 100 m. Clinical manifestation of central nervous system (CNS) involvement in CLL is heterogeneous and therefore may be difficult to pinpoint. We have described an uncommon occurrence of CNS involvement in CLL.

Non-convulsive status epilepticus in two patients with tuberous sclerosis.

Tuberous sclerosis (TSC) is an autosomal dominantly inherited genetic disorder that chiefly affects the central nervous system, along with the other multiple systems. While phenomenology and symptom severity may vary greatly from one individual to another, the most common neurological presentation is epilepsy, which may be refractory in a considerable number of patients. Convulsive SE is seen frequently in TSC patients due to the high ratio of refractory seizures in well-studied cohorts. Status epilepticus (SE) is a life-threating condition and requires urgent medical care. Non-convulsive status epilepticus (NCSE) is an epileptic state with no convulsive seizures but impaired consciousness and corresponding electrophysiological findings. Due to its heterogeneity of clinical features, it is generally hard to recognize, and thus difficult to treat promptly. The relationship between TSC and NCSE is a relatively less emphasized issue in the literature. Here, we present two cases of TSC with NCSE with a view to increasing clinicians' awareness of the association between refractory epilepsy and NCSE.

Novel SNARE Complex Polymorphisms Associated with Multiple Sclerosis: Signs of Synaptopathy in Multiple Sclerosis

Background: It is well known that axonal degeneration plays a role in disability in patients with multiple sclerosis, and synaptopathy has recently become an important issue. Aims: To investigate the possible roles of selected synaptic and presynaptic membrane protein genetic polymorphisms (VAMP2, SNAP-25, synaptotagmin, and syntaxin 1A) in patients with multiple sclerosis. Study Design: Case-control study. Methods: A total of 123 patients with multiple sclerosis and 192 healthy controls were included. The functional polymorphisms of specific SNARE complex proteins (VAMP2, synaptotagmin XI, syntaxin 1A, and SNAP-25) were analyzed by polymerase chain reaction. Results: Significant differences were detected in the genotype and allele distribution of 26-bp Ins/Del polymorphisms of VAMP2 between patients with multiple sclerosis and control subjects; Del/Del genotype and Del allele of VAMP2 were more frequent in patients with multiple sclerosis (p=0.011 and p=0.004, respectively). Similarly, Ddel polymorphism of SNAP-25 gene C/C genotype (p=0.059), syntaxin 1A T/C and C/C genotypes (p=0.005), and synaptotagmin XI gene C allele (p=0.001) were observed more frequently in patients with multiple sclerosis. CC, syntaxin rs1569061 1A gene for 33-bp promoter region TC haplotypes, and synaptotagmin XI gene were found to be associated with an increased risk for multiple sclerosis (p=0.012). Similarly, GC haplotype for rs3746544 of SNAP-25 gene and rs1051312 of SNAP-25 gene were associated with an increased risk for multiple sclerosis (p=0.022). Conclusion: Genetic polymorphisms of SNARE complex proteins, which have critical roles in synaptic structure and communication, may play a role in the development of multiple sclerosis.

A rare condition mimicking stroke: Diabetic uremic encephalopathy.

Uremic encephalopathy (UE) is a metabolic disorder associated with acute or chronic renal failure. It is characterized by the acute or subacute onset of reversible neurological symptoms and specific imaging findings. It is uncommon for uremic encephalopathy to be associated with acute bilateral lesions of the basal ganglia in diabetic uremic patients, and this can be seen most often in Asian patients. Here, we report a patient with diabetic uremic encephalopathy and bilateral basal ganglia lesions who developed acute onset dysarthria. The clinical and magnetic resonance brain imaging findings resolved after hemodialysis treatment.

Alien hand syndrome and migraine with aura: A case report.

BACKGROUND: Alien Hand Syndrome (AHS) is an uncontrollable, involuntary, but in appearance, purposeful motor control disorder of the upper extremity. CASE REPORT: A 42-year-old male patient was admitted to our clinic complaining of involuntary motor activity in his right hand. He had a previous history of migraine with visual aura. The uncontrollable motor control disorder was compatible with Alien Hand Syndrome, which was appearing immediately after the visual aura and before the beginning of headache. CONCLUSION: Alien Hand Syndrome is usually observed with anterior cerebral artery infarction, midline tumors, trauma and several neurodegenerative diseases, but is rarely seen in paroxysmal conditions such as migraine with aura.

Olfactory dysfunction in multiple sclerosis.

BACKGROUND: Multiple sclerosis (MS) is a common chronic neurological disease that causes disability. MS can have various clinical manifestations, one of which is olfactory dysfunction. In clinical practice, olfactory disturbances are usually underdiagnosed. The aim of our study is to assess olfactory function and its relationship with MS disease duration, disability and cognition. METHOD: We assessed 31 MS patients and 24 healthy controls matched in sex and age at our MS outpatient clinic of the Istanbul Education and Research Hospital Neurology Department. Each subject was interviewed to obtain demographic data. The Connecticut Chemosensory Clinical Research Center (CCCRC) olfactory test and Montreal Cognitive Assessment (MOCA) were applied to each participant. RESULTS: The CCCRC test scores of the MS patients were lower than those in the control group (p < 0.05). Patients with a longer disease duration and more frequent attacks had lower CCCRC scores (p < 0.05). The Expanded Disability Status Scale scores had no correlation with the CCCRC test scores. The MOCA score was positively correlated with all domains of the olfactory test scores. CONCLUSION: This study supports the presence of olfactory dysfunction in early stages of MS and the correlation of cognitive impairment with olfactory dysfunction, even in the early stages of the disease in young and less physically- disabled patients. The assessment of olfaction may be helpful as a surrogate method for tracking disease progression in patients over time.