Neurology International

The development of hydrocephalus after severe traumatic brain injury (TBI) is an under-recognized healthcare phenomenon and can increase morbidity. The current study aims to characterize post-traumatic hydrocephalus (PTH) in a large cohort. Patients were prospectively enrolled age 16–80 years old with Glasgow Coma Scale (GCS) score $\le$8. Demographics, GCS, Injury Severity Score (ISS), surgery, and cerebrospinal fluid (CSF) were analyzed. Outcomes were shunt failure and Glasgow Outcome Scale (GOS) at 6 and 12-months. Statistical significance was assessed at p < 0.05. In 402 patients, mean age was 38.0 ± 16.7 years and 315 (78.4%) were male. Forty (10.0%) patients developed PTH, with predominant injuries being subdural hemorrhage (36.4%) and diffuse axonal injury (36.4%). Decompressive hemicraniectomy (DHC) was associated with hydrocephalus (OR 3.62, 95% CI (1.62–8.07), p < 0.01). Eighteen (4.5%) patients had shunt failure and proximal obstruction was most common. Differences in baseline CSF cell count were associated with increased shunt failure. PTH was not associated with worse outcomes at 6 (p = 0.55) or 12 (p = 0.47) months. Hydrocephalus is a frequent sequela in 10.0% of patients, particularly after DHC. Shunt placement and revision procedures are common after severe TBI, within the first 4 months of injury and necessitates early recognition by the clinician. Full article

A BACKROUND: Multiple sclerosis (MS) is a complex chronic disease of the central nervous system (CNS). Body mass index (BMI), a component of metabolic syndrome (MetS), is considered among the risk factors for MS. However, its role in MS remains ambiguous. OBJECTIVE: To examine the impact of BMI on the age of onset in patients with relapsing-remitting MS (RRMS) in a Greek cohort. METHODS: Data from 821 Greek patients with RRMS were collected. The BMI values were considered as quartiles. Comparisons for the demographic characteristics between the quartiles were made by Pearson’s chi-square test for the categorical variables and by ANOVA for the continuous variables. An overall p-value was calculated corresponding to trend for association. In case of significant association, further post-hoc analysis was performed in order to identify differences in demographic characteristics between specific BMI quartiles groups. Linear regression analyses were used to assess the relationship between BMI and age at onset of MS. RESULTS: Comparisons of participant characteristics by quartiles of BMI revealed that participants with the highest BMI had an older age of disease onset. Results from linear regression analysis showed that with each increase of 1 BMI unit, the age of RRMS onset increases by 0.255 (95% CI 0.136 to 0.374) years, p < 0.001. CONCLUSIONS: Patients with higher BMI, as a parameter of MetS, exhibit increased age of RRMS onset. Our results may present an alternative personalized approach for diagnosis, prognosis, and/or prevention of RRMS. Full article

A 32-year-old man presented with complete third nerve palsy. Brain magnetic resonance imaging revealed a possible sporadic oculomotor nerve schwannoma in the left cavernous sinus. Interestingly, the patient’s ptosis was fluctuating and eased immediately after application of ice over his eyelid. The ice pack test (IPT) is a simple and fast method that can help diagnose myasthenia gravis (MG) in patients with ptosis. Additional diagnostic investigations (antibody assays and repetitive stimulation) excluded associated MG in the patient. Tumor treatment with Gamma Knife radiosurgery was planned. This case highlights the finding that IPT can also be positive in neurogenic (non-myasthenic) ptosis, but its usefulness in other disorders associated with muscle weakness and fatigability remains questionable. Full article

Serotonin-norepinephrine reuptake inhibitors (SNRIs) inhibit the presynaptic neuronal uptake of serotonin and norepinephrine and prolong the effects of the monoamines in the synaptic cleft within the central nervous system, leading to increased postsynaptic receptor activation and neuronal activities. Serotonin-norepinephrine reuptake inhibitors can have multiple clinical indications, including as the first-line agents for the management of depression and anxiety, and as analgesics in the treatment of chronic pain. The effects of reuptake inhibition of norepinephrine and serotonin are often dose-dependent and agent-dependent. There are five FDA-approved serotonin-norepinephrine reuptake inhibitors (desvenlafaxine, duloxetine, levomilnacipran, milnacipran and sibutramine) currently being marketed in the United States. As the COVID-19 pandemic significantly increased the incidence and prevalence of anxiety and depression across the country, there are significantly increased prescriptions of these medications perioperatively. Thus, anesthesiologists are more likely than ever to have patients administered with these agents and scheduled for elective or emergency surgical procedures. A thorough understanding of these commonly prescribed serotonin-norepinephrine reuptake inhibitors and their interactions with commonly utilized anesthetic agents is paramount. There are two potentially increased risks related to the continuation of SNRIs through the perioperative period: intraoperative bleeding and serotonin syndrome. SNRIs have some off-label uses, more new indications, and ever-increasing new applications in perioperative practice. This article aims to review the commonly prescribed serotonin-norepinephrine reuptake inhibitors and the current clinical evidence regarding their considerations in perioperative anesthesia and analgesia. Full article

We present a case of a patient with positive N-methyl-D-aspartate receptor (NMDAR) IgG antibodies in their serum and cerebrospinal fluid (CSF) associated with neuroborreliosis. Clinically, the patient presented with symptoms of confusion, as well as behavioral and speech impairments. Regardless of antibacterial treatment, no significant improvement was achieved. Methylprednisolone provided a marked improvement in the patient’s clinical signs and CSF findings. The screening did not reveal any underlying neoplasm. Taking into account the marked clinical improvement after treatment with glucocorticosteroids, we suggest that NMDAR encephalitis is a possible autoimmune complication in neuroborreliosis patients requiring additional immunotherapy. Full article

Within the neuropsychological assessment, clinicians are responsible for ensuring the validity of obtained cognitive data. As such, increased attention is being paid to performance validity in patients with multiple sclerosis (pwMS). Experts have proposed batteries of neuropsychological tests for use in this population, though none contain recommendations for standalone performance validity tests (PVTs). The California Verbal Learning Test, Second Edition (CVLT-II) and Brief Visuospatial Memory Test, Revised (BVMT-R)—both of which are included in the aforementioned recommended neuropsychological batteries—include previously validated embedded PVTs (which offer some advantages, including expedience and reduced costs), with no prior work exploring their utility in pwMS. The purpose of the present study was to determine the potential clinical utility of embedded PVTs to detect the signal of non-credibility as operationally defined by below criterion standalone PVT performance. One hundred thirty-three (133) patients (M age = 48.28; 76.7% women; 85.0% White) with MS were referred for neuropsychological assessment at a large, Midwestern academic medical center. Patients were placed into “credible” (n = 100) or “noncredible” (n = 33) groups based on a standalone PVT criterion. Classification statistics for four CVLT-II and BVMT-R PVTs of interest in isolation were poor (AUCs = 0.58–0.62). Several arithmetic and logistic regression-derived multivariate formulas were calculated, all of which similarly demonstrated poor discriminability (AUCs = 0.61–0.64). Although embedded PVTs may arguably maximize efficiency and minimize test burden in pwMS, common ones in the CVLT-II and BVMT-R may not be psychometrically appropriate, sufficiently sensitive, nor substitutable for standalone PVTs in this population. Clinical neuropsychologists who evaluate such patients are encouraged to include standalone PVTs in their assessment batteries to ensure that clinical care conclusions drawn from neuropsychological data are valid. Full article

Background: Ulnar nerve compression at the elbow level is the second-most common entrapment neuropathy. The aim of this study was to use shear-wave elastography for the quantification of ulnar nerve elasticity in patients after ulnar nerve decompression with anterior transposition and in the contralateral non-operative side. Method: Eleven patients with confirmed diagnosis and ulnar nerve decompression with anterior transposition were included and examinations were performed on an Aixplorer^TM ultrasound system (Supersonic Imagine, Aix-en-Provence, France). Results: We observed significant differences at 0-degree (p < 0.001), 45-degree (p < 0.05), 90-degree (p < 0.01) and 120-degree (p < 0.001) elbow flexion in the shear elastic modulus of the ulnar nerve in the operative and non-operative sides. There were no statistically significant differences between the elasticity values of the ulnar nerve after transposition at 0-degree elbow flexion and in the non-operative side at 120-degree elbow flexion (p = 0.39), or in the ulnar nerve after transposition at 120-degree elbow flexion and in the non-operative side at 0-degree elbow flexion (p = 0.09). Conclusion: Shear-wave elastography has the potential to be used postoperatively as a method for assessing nerve tension noninvasively by the estimation of mechanical properties, such as the shear elastic modulus. Full article

Liver and renal involvement is a rare event in Sjögren’s syndrome. Sjögren’s syndrome is characterized by the progressive immune-mediated destruction of epithelial tissues of the salivary and lacrimal glands. Sensory ganglionitis, accompanied by T-cell invasion, occurs in patients with Sjögren’s syndrome, resulting in sensory neuropathy of the face or limbs. Patients are assessed by quantitative sensory testing. A 76-year-old woman presented with numbness of her left face and was subsequently diagnosed with Sjögren’s syndrome and primary biliary cirrhosis, and was found to have renal failure. Detection of her serum anti-Ro/SSA antibody was strongly positive. Shirmer’s test or a salivary volume in the gum test also showed positive results. Her somatosensory disturbance severity was higher in the trigeminal area than in the forearm, suggesting that the trigeminal nerve is more susceptible than other parts of the nervous system in patients with Sjögren’s syndrome and primary biliary cirrhosis. A simple sensory test could be performed during regular check-ups, as sensory deficits might develop after patients are diagnosed with Sjögren’s syndrome and primary biliary cirrhosis. Full article

The monoamine hypothesis of depression attributes the symptoms of major depressive disorders to imbalances of serotonin, noradrenaline, and dopamine in the limbic areas of the brain. The preferential targeting of serotonin receptor (SERT) by selective serotonin reuptake inhibitors (SSRIs) has offered an opportunity to reduce the range of these side effects and improve patient adherence to pharmacotherapy. Clozapine remains an effective drug against treatment-resistant schizophrenia, defined as failing treatment with at least two different antipsychotic medications. Patients with schizophrenia who display a constellation of negative symptoms respond poorly to antipsychotic monotherapy. Negative symptoms include the diminution of motivation, interest, or expression. Conversely to the depressive symptomology of interest presently, supplementation of antipsychotics with SSRIs in schizophrenic patients with negative symptoms lead to synergistic improvements in the function of these patients. Fluvoxamine is one of the most potent inhibitors of CYP1A2 and can lead to an increase in clozapine levels. Similar increases in serum clozapine were detected in two patients taking sertraline. However, studies have been contradictory as well, showing no such increases, which are worrying. Clinicians should be aware that clozapine levels should be monitored with any coadministration with SSRIs. Full article

Background: We report the case of a patient presenting with orofacial tardive dyskinesia (TD), following administration of a first-generation antipsychotic (Loxapine). Intervention: Four weeks of repeated sessions of mindfulness-based cognitive therapy (MBCT) and mindfulness-based stress reduction (MBSR) protocols were administered, with TD hetero-quantified before and during each session via the Abnormal Involuntary Movement Scale (AIMS). Results: The dyskinesia ameliorated quantitatively and qualitatively (1) during each session, and (2) at resting conditions in the long term. During some sessions, after which patients’ compliance was auto-evaluated as maximal, complete arrest of the TD was observed. Hypothesis and Conclusion: We suggest mindfulness meditation as a novel adjunctive therapeutic approach for tardive dyskinesia, and invite for further clinical and neurological investigations. Full article

Background: Multiple sclerosis is a progressive neurodegenerative disease that affects myelin in the central nervous system. It is complex and unpredictable and occurs predominantly in young adults, causing increasing disability and a significantly lower quality of life. Recent studies investigated how rehabilitation training through the use of a robotic exoskeleton can influence walking recovery in patients with a serious neurological disease. Aim: The purpose of this study was to analyze the first approach of a multiple sclerosis patient to a robotic exoskeleton for the lower limbs, in order to assess the effectiveness of the protocol on walking ability, adaptability of the device, level of appreciation, variations in parameters related to walking, and fatigue perception. Methods: This study was conducted on a 71-year-old male diagnosed with primary progressive multiple sclerosis since 2012, with an EDSS score of 6. The patient underwent a cycle of 10 sessions of treatment with the exoskeleton for the lower limbs, the UAN.GO, lasting 1 h 30 min. Pre- and post-treatment evaluations were carried out with the 6 min walking test, the Fatigue Severity Scale, the Short Form-36 Health Survey, and a Likert scale for review. During each session, blood pressure, heart rate, and peripheral saturation were monitored; in addition, the perception of fatigue by the Borg scale was studied. Result: A comparison between the initial and final evaluations showed improvements in the walked distance at 6 MWT (T₀ = 53 m/T₁ = 61 m). There was a positive trend in saturation and heart rate values collected during each session. Further improvements were found by the Borg scale (T₀ = 15/T₁ = 11). Discussion: The data collected in this case report show promising results regarding the treatment of multiple sclerosis patients with the UAN.GO exoskeleton, with benefits on both motor performance and vital parameters. Full article

SARS-CoV-2 infection in children produces mild respiratory symptoms or no symptoms at all in most cases. Some pediatric patients develop a severe complication associated with high mortality, the multisystem inflammatory syndrome in children (MIS-C). In both scenarios, there are reports of neurological manifestations. This article aims to review the cases of pediatric patients with severe neurological issues and a coexisting positive SARS-CoV-2 test. A literature search was performed between March 2020 and May 2021. The results included the data from 41 studies, with 159 children with severe neurological manifestations, within an age range from 24 h to 17 years. The neurological disorders included 38 cases with stroke, 32 with encephalitis, 22 with encephalopathy, and 10 with Guillain–Barre syndrome. Sixty-five out of 159 cases with severe neurological manifestations were diagnosed with MIS-C. Direct neuroinvasion and the exaggerated immune response in some patients seem to be the most critical factors triggering these manifestations. Further research in the ongoing pandemic is needed to elucidate the precise mechanism. Full article

Guillain-Barré syndrome (GBS) is an immune-mediated demyelinating disorder which attacks the peripheral nervous system. Antecedent infection or vaccine administration are known to precipitate the onset of this disorder. Its typical presentation leads to a symmetric, rapidly progressive, ascending paresis with associated sensory deficits and impaired reflexes. We present a rare case of a bi-facial diplegia variant of GBS, within four weeks of the COVID-19 vaccination. Due to its chronology, clinical manifestations, and cerebrospinal fluid (CSF) findings, we propose this case to be a rare complication of the COVID-19 vaccination. Full article

Depression is the most prevalent psychiatric disorder in the world, affecting 4.4% of the global population. Despite an array of treatment modalities, depressive disorders remain difficult to manage due to many factors. Beginning with the introduction of fluoxetine to the United States in 1988, selective serotonin reuptake inhibitors (SSRIs) quickly became a mainstay of treatment for a variety of psychiatric disorders. The primary mechanism of action of SSRIs is to inhibit presynaptic reuptake of serotonin at the serotonin transporter, subsequently increasing serotonin at the postsynaptic membrane in the serotonergic synapse. The six major SSRIs that are marketed in the USA today, fluoxetine, citalopram, escitalopram, paroxetine, sertraline, and fluvoxamine, are a group of structurally unrelated molecules that share a similar mechanism of action. While their primary mechanism of action is similar, each SSRI has unique pharmacokinetics, pharmacodynamics, and side effect profile. One of the more controversial adverse effects of SSRIs is the black box warning for increased risk of suicidality in children and young adults aged 18–24. There is a lack of understanding of the complexities and interactions between SSRIs in the developing brain of a young person with depression. Adults, who do not have certain risk factors, which could be confounding factors, do not seem to carry this increased risk of suicidality. Ultimately, when prescribing SSRIs to any patient, a risk–benefit analysis must factor in the potential treatment effects, adverse effects, and dangers of the illness to be treated. The aim of this review is to educate clinicians on potential adverse effects of SSRIs. Full article

Introduction: Dystonia is a movement disorder substantially affecting the quality of life. Botulinum Neurotoxin (BoNT) is used intramuscularly as a treatment for dystonia; however, not all dystonia patients respond to this treatment. Deep brain stimulation (DBS) is an established treatment for Parkinson’s disease (PD) and essential tremor, but it can help in dystonia as well. Objectives: We studied a total of 67 dystonia patients who were treated with DBS over a period of 7 years to find out the long-term efficacy of DBS in those patients. First, we calculated patient improvement in post-surgery follow-up programs using the Global Dystonia Severity scale (GDS) and Burke–Fahn–Marsden dystonia rating scale (BFMDRS). Secondly, we analyzed the scales scores to see if there was any statistical significance. Methods: In our study we analyzed patients with ages from 38 to 78 years with dystonia who underwent DBS surgery between January 2014 and December 2020 in four different centers (India, Kuwait, Egypt, and Turkey). The motor response to DBS surgery was retrospectively measured for each patient during every follow-up visit using the GDS and the BFMDRS scales. Results: Five to 7 years post-DBS, the mean reduction in the GDS score was 30 ± 1.0 and for the BFMDRS score 26 ± 1.0. The longitudinal change in scores at 12 and 24 months post-op was also significant with mean reductions in GDS and BFMDRS scores of 68 ± 1.0 and 56 ± 1.0, respectively. The p-values were <0.05 for our post-DBS dystonia patients. Conclusions: This study illustrates DBS is an established, effective treatment option for patients with different dystonias, such as generalized, cervical, and various brain pathology-induced dystonias. Although symptoms are not completely eliminated, continuous improvements are noticed throughout the post-stimulation time frame. Full article

Angelman syndrome is a rare genetic disease affecting the central nervous system and neurobehavioral development causing severe mental, linguistic, and physical disabilities. The purpose of this review was to analyze the most recent evidence regarding the rehabilitation of subjects affected by this syndrome. The review was carried out in accordance with the preferred reporting items for systematic reviews and meta-analyses. A total of 3661 studies were identified in the databases. Once the inclusion/exclusion criteria were applied, 15 studies were considered for the paper’s preparation. The level of evidence of the studies was established according to the criteria of the Oxford Center for Evidence-Based Medicine—Levels of Evidence. From the selected studies, five rehabilitative approaches emerged: physiotherapy, applied behavioral analysis, toilet training, microswitch-cluster technology, and augmentative and alternative communication. Although the studies did not have a high level of evidence, the reported results appear to be encouraging and pave the way for further studies. It seems that individualized and multidisciplinary rehabilitation interventions help to improve patients’ autonomy and quality of life. In some studies, the caregivers’ role was fundamental to identify preferences and long-term improvements. Further studies on larger populations and with better methodological quality are needed to confirm the results. Full article

Purpose of Review: This is a comprehensive review of the literature regarding the use of paliperidone in the treatment of schizophrenia and schizoaffective disorder. It covers the background and presentation of schizophrenia and schizoaffective disorder, as well as the mechanism of action and drug information for paliperidone. It covers the existing evidence of the use of paliperidone for the treatment of schizophrenia and schizoaffective disorder. Recent Findings: Schizophrenia and schizoaffective disorder lead to significant cognitive impairment. It is thought that dopamine dysregulation is the culprit for the positive symptoms of schizophrenia and schizoaffective disorder. Similar to other second-generation antipsychotics, paliperidone has affinity for dopamine D₂ and serotonin 5-HT_2A receptors. Paliperidone was granted approval in the United States in 2006 to be used in the treatment of schizophrenia and in 2009 for schizoaffective disorder. Summary: Schizophrenia and schizoaffective disorder have a large impact on cognitive impairment, positive symptoms and negative symptoms. Patients with either of these mental illnesses suffer from impairments in everyday life. Paliperidone has been shown to reduce symptoms of schizophrenia and schizoaffective disorder. Full article

Most people with MS experience cognitive deficits especially in attention, memory, information processing, and executive functions, negatively impacting on their quality of life. Cognitive variables of short-term memory, logical memory, and verbal fluency in 65 patients with multiple sclerosis (MS) were analysed in conjunction with sociodemographic variables such as sex, age, and educational level that might influence disease progression. We found that psychoeducational variables exerted a significant effect on the cognitive status of patients with MS. Thus, when considering sex, age, educational level, and type of MS (SPMS or RRMS), tests for between-subject effects revealed statistically significant differences in all three cognitive variables. In addition, we found that the type of MS and time since onset also generated significant cognitive differences. Our study shows that educational achievement or level is a protective factor against the disease, acting as a source of intellectual enrichment that promotes cognitive reserve in patients with MS. Further longitudinal studies assessing disease progression and prognosis in patients with MS would be useful in order to determine the specific importance of these variables in such patients and in strategies that could enhance their performance in neuropsychological assessment tasks. Full article