Neurology International

For certain clinical circumstances, the differentiation between cerebellar medulloblastoma and brainstem glioma is essential. We aimed to evaluate the role played by the apparent diffusion coefficient (ADC) values in the differentiation between cerebellar medulloblastomas and brainstem gliomas in children. The institutional review board approved this prospective study. Brain magnetic resonance imaging (MRI), including diffusion-weighted imaging (DWI) and ADC, was assessed in 32 patients (median age: 7.0 years), divided into two groups, a medulloblastoma group (group 1, n = 22) and a brainstem glioma group (group 2, n = 10). The Mann–Whitney U test was utilized to compare tumor ADCmax, ADCmin, ADCmean, and ADCsd values, and their ratios with the parenchyma values between the two groups. Receiver operating characteristic (ROC) curve analysis and the Youden index were used to calculate the cut-off value, along with the area under the curve (AUC), sensitivity, and specificity. The median ADCmax, ADCmin, and ADCmean values were significantly higher in group 2 than in group 1 (p < 0.05). The median ratios of ADCmin and ADCmean to the parenchyma were significantly higher in group 2 than in group 1 (p < 0.05). The ROC analysis showed that the AUC for the ADCmean ratio was the highest among these parameters, at 98.2%. The ADCmean tumor to parenchyma ratio was a significant and effective parameter for the differentiation between pediatric medulloblastomas and brainstem gliomas. Full article

A 70-year-old man presented to the emergency department with blood hypotension associated to a sudden paraplegia and thermalgesic analgesia. He had an history of colic and prostatic adenocarcinoma, hypertension and non-dialyzed Chronic Kidney Disease (CKD) related to an idiopathic membranous glomerulonephritis type 1 discovered 9 years ago. Magnetic resonance imaging confirmed a diagnosis of Spinal Cord Infarction (SCI). Few months later, he presented a blurred vision due to central Retinal Vein Occlusion (RVO), which was improved by Anti-VEGF therapy. This is the first reported case of a concomitance of retinal vascular event and SCI highlights the links between the central nervous system and retinal vascularization despite separate involvement of the two events in the arterial and venous systems. Additionally, CKD worsened the risk of cardiovascular incidents by induced oxidative stress, thrombophilia, chronic inflammation, and endothelial dysfunction. SCI occurrence indicates severe vascular dysfunction and elevates the risk of additional vascular disorders. Full article

Nitrous oxide (N2O) is a weak anesthetic gas that was first used in 1844 in the field of dental anesthesia. However, currently, N2O is being abused for entertainment purposes in the form of N2O-filled balloons, called funky balls, which can cause many adverse effects, especially nervous system injury. This study aimed to investigate the detailed clinical and subclinical features associated with N2O intoxication. We retrospectively reviewed 47 patients diagnosed with N2O intoxication, from May 2018 to July 2019, and collected demographic data, clinical and laboratory tests, and spinal cord magnetic resonance imaging (MRI) findings. The mean time of funky ball use was 8.8 months, with a mean use of 36.3 balls per day. All patients presented with superficial sensory disorders. Reductions in muscle strength, decreased vibration sensation, and decreased or lost tendon reflexes were the most common clinical signs of N2O intoxication. Romberg sign and Lhermitte sign were observed in 39 patients (83%) and 21 patients (44.7%), respectively. Spinal cord lesions on MRI were observed in 32 patients (68.1%), which mostly presented with an inverted V sign. The total duration of N2O use, the number of days of using N2O per week, and the presence of Lhermitte sign (P<0.05) were significantly different between patients with and without spinal cord lesions on MRI. Serum levels of homocysteine and vitamin B12 were significantly different between the time of admission and discharge (P<0.05). Our study indicated that the days of using N2O per week was significantly associated with Spinal Cord Injuries (SCI) on MRI. According to the Receiver Operating Characteristic (ROC) curve analysis, a cutoff days of using per week value of 2.5 days could predict SCI with a sensitivity of 81.3%, a specificity of 73.3%, and an area under the ROC curve (AUC) of 0.813. Changes in the serum levels of homocysteine and vitamin B12 were effective markers for the evaluation of treatment response. Full article

Endovascular Embolization (EVE) of aneurysms is a very effective and efficient treatment modality. Nevertheless, a few complications have been reported after EVE of aneurysms. Our study therefore evaluated the safety and efficacy of Low-profile Visible Intraluminal Support (LVIS) stentassisted EVE for intracranial Dissecting Aneurysms (DAs). We conducted a retrospective study to identify patients with DAs who were treated with LVIS stent from July 2015 to September 2018. The DAs were categoried into ruptured and unruptured. The arteries harbouring the aneurysm were identified in all cases. LVIS device stent assisted coil EVE treatment modality was utilized to treat all the patients. Surgical safety, immediate surgery outcome, recurrence rate and imaging follow-up results of all patients were analysed. The Glasgow Outcome Scale (GOS) score of all patients where assessed during discharge. Cerebral angiography of all patients were reevaluated on scheduled visits from three months up to one year after their operations. A total of Six DA patients were identified during our analysis. Four of the cases were ruptured DAs while two cases were unruptured. The DAs originated from the Internal Carotid Artery (ICA) in two cases, while in the remaining four cases, the DAs originated from vertebral artery (VA). Stents and coils were successfully implanted in all six patients. The DAs were embolized satisfactorily and the parent arteries were patent immediately after the operations. We obsereved 5 points GOS score in four cases and 4 points in two cases. No aneurysmal recurrece, no stent collapse or displacement was obsereved in all cases during follow-ups. Our study suggests that, LVIS stent-assisted EVE is simple, safe and effective in the treatment of DAs. Full article

In this review, we focus on summarizing everything that is known about the neurological effects of Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-COV-2). It has been shown that Coronavirus Disease 2019 (Covid-19) may result in neuromuscular disorders or damage to nerves outside of the brain and spinal cord, which may lead to weakness, numbness, and pain. Published literature has stated that SARS-COV-1 may infect the central nervous system and due to its similarities to SARS-COV-2, we suspect that SARS-COV-2 has the same potential. We conclude that Covid-19 has neurological manifestations. Further research should be done in this field to understand the full extent of this virus. Full article

Parkinson’s disease (PD) has the second highest prevalence among neurodege - nerative diseases. In Colombia, PD population dynamics are currently unknown. Health records offer a unique resource to study frequency and multi-morbidity of chronic diseases. The aim of this research is to estimate prevalence and staging using administrative data (AD) provided by Health Maintenance Organizations (HMOs). A cross-sectional study was conducted using 2015 AD from two Colombian HMOs (4.312.928 beneficiaries, 9.01% of the affiliated Colombian population). PD prevalence and severity was estimated by age and sex. Prevalence was adjusted to WHO demographics. Age-adjusted PD prevalence was 205.89 per 100.000 inhabitants. Prevalence increment of 62.13% was found between those aged ≥40 years and those aged ≥50 years. Similarly, each extra decade (50-80+) represented an increment of 83.65%, 80.95%, and 35.10%. Between 40 and 89 years, males exhibited a significantly higher PD prevalence compared to females. Advanced PD was more frequent as age increased from 3.77% in the group between 40 to 49 years to 25.86% in those older than 89 years. More common related comorbidities were arterial hypertension, diabetes, and psychiatric disorders; the first two increased their frequency with age, and the last one maintained its prevalence across all age groups. AD sets are useful to estimate the prevalence and staging of PD. Prevalence of PD in Colombia is higher in men and increases with age, as well as disease severity. Full article

We examined pathogenesis and clinical features of three hemichorea-hemiballism (HCHB) cases. We studied their age, magnetic resonance imaging results, vascular risk factors, management, and outcomes. One man and two women (aged 74-86 years) demonstrated acute onset of HCHB, lasting for at least several months. Patients had one or more vascular risk factors, including hypertension and diabetes. All patients presented subacute or old infarction in the basal ganglia with contralateral symptoms. We administered clonazepam (0.5-1 mg/day), haloperidol (0.375-0.75 mg/day), or both as necessary and observed symptom-control. Vascular lesions in the basal ganglia were a contributing factor. Symptoms were controlled using pharmacotherapy with gamma-aminobutyric acid-agonist (clonazepam) or anti-dopaminergic (haloperidol) medication. Full article

Attention deficit hyperactivity disorder (ADHD) is one of the most common neurobehavioural in the children. Genetic factor is known one of the factors which contributed in ADHD development. VNTR polymorphism in 3’UTR exon 15 of DAT1 gene and exon 3 of DRD4 gene are reported to be associated in ADHD. In this study we examine the association of ADHD with VNTR polymorphism of DAT1 and DRD4 gene in Indonesian children. Sixty-five ADHD children and 70 normal children (6- 13 years of age), were included in the study, we matched by age and gender. ADHD was diagnosed by DSM-IV. We performed a casecontrol study to found the association between ADHD and VNTR polymorphism of DAT1 and DRD4 genes. The 10-repeat allele of DAT1 and 2-repeat allele of DRD4 were higher in Indonesian children. Although the frequency of these allele was higher, but it was similar both in ADHD and control groups. Neither DAT1 nor DRD4 gene showed showed significant difference in genotype distribution and frequency allele between both groups (p > 0.05). No association between ADHD and VNTR polymorphism of DAT1 and DRD4 genes found in Indonesian children. This data suggest that DAT1 and DRD4 do not contribute to etiology of ADHD in Indonesian children. Further studies are needed to clarify association between VNTR polymorphism of DAT1 and DRD4 genetic with ADHD of Indonesian children in larger sample size and family based study. Full article

Lyme disease related central and peripheral nervous system manifestations can occur in isolation or together. Radiculitis or inflammation of the nerve root can be seen 3-5% of the time in acute neuroborreliosis affecting the PNS with a typical presentation and meningitis affecting the CNS is usually seen 1% of the time. The appropriate diagnosis and management of neuroborelliosis can be challenging and require meticulous medical approaches. Herein we present a unique case of Lyme disease with neurologic manifestations including both radiculitis and meningitis due to its atypical and challenging clinical presentation and management with updated literature review. Full article

Ischemic stroke is one of the most common cause of mortality and disability in the modern world. Still, therapeutic options remains modest. Aim of the study was to present dynamics of inflammatory factors expression (C reactive protein, procalcitonin, interleukin 10) in patients after ischemic stroke. Our study included 101 patients divided in thrombolised and non-thrombolised groups. Inflammatory factors concentration in serum was determinate at admission, 24, 48 hours and seven days after the initial onset, while neurological assessment was measured at the admission, 24 hours, seven days and three months after the initial onset using National Institute of Health Stroke Scale and Rankin Scale. Certain pattern was observed in dynamics of inflammatory factors: intensive increase in first and second day after the stroke, followed by decrease till day seven in both groups. Additionally, thrombolised group showed significant neurological improvement. Although well investigated, the role of inflammatory factors in the ischemic stroke still stays controversial. High association of C reactive protein and interleukin 10 values suggest potential prognostic role in patients follow-up, while the role of procalcitonin values still remains unclear. Full article

Sleep is responsible for several functions required for homeostasis. REM sleep could be a rearrangement period where limits of certain functions can be moved to a new state of balance. This study proposes that dopaminergic deficit may be responsible for the circadian dysregulation that occur with neurodegeneration and therefore a restitution of REM sleep and an improvement in Parkinson disease’s symptoms can be achieved with the controlled use of dopamine agonists during the night. Twenty parkinsonian patients underwent to a onemonth study of subcutaneous nocturnal apomorphine treatment at the beginning of each REM stage. This therapeutic approach led to a significant benefit for patients in all of the 3 UPDRS scores. The mean change from baseline in the MDS-UPDRS Part I, II and III was significantly greater in the apomorphine vs. placebo group. In the UPDRS Part I total score was 0.8 (95% confidence interval [CI]: 1.612, -0.012) and 3.3 (95% CI: 4.732, 1.867) for the placebo and apomorphine groups, respectively (difference between groups: 2.5, 95% CI: 3.454, 1.545; P = 0.002). For UPDRS Part II total score, the mean change was 1.3 (95% CI: 2.692, - 0.09) and 4.6 (6.916, 2.28). Difference between groups: 3.3, 95% CI: 4.752, 1.847; P = 0.013. In UPDRS Part III was 1.1 (95% CI: 2.425, -0.225) and 5.5 (95% CI: 8.808, 2.191). Difference between groups: 4.4, (95% CI: 6.321, 2.478; P = 0.012). We can conclude that sleep alteration in PD can be improved by stimulation of D2 receptors. The symptomatic benefits obtained due to restoration of REM functions were significant. Full article

Myotonic dystrophy (DM) is a complex multisystem disease with specific clinical and electrodiagnostic findings. Myotonia can be seen in the distal and proximal muscle groups in upper and lower limbs. There is no established guideline to demonstrate the sensitivity of muscles in the diagnosis of myotonic dystrophy. The aims of this study are to describe common electrodiagnostic findings in patients with DM; and to assess the electrodiagnostic sensitivity of muscles in the diagnosis of DM. In this retrospective study, patients’ age, sex, nerve conduction study findings including common upper and lower limbs nerve functions, and needle examination findings were collected and analyzed. A descriptive analysis (with percentage) was performed on the data obtained from the charts. NCS analysis showed more than half of patients had normal sensory and motor NCS findings. In 11 over 12 patients, sensory NCSs were within normal limits. Only one patient showed abnormal sensory responses. The most common abnormal NCS findings were decreased amplitude with normal latency and normal conduction velocity. The needle analysis showed distal muscles including first dorsal interosseous, abductor policies brevis, tibialis anterior, medial gastrocnemius and peroneal longus muscles are more sensitive in detecting myotonic discharges than proximal muscles including deltoid, triceps, vastus medialis and vastus lateralis. Our findings showed sensory nerve responses were usually within normal limits. The most common NCS abnormality showed decreased motor nerve amplitudes. The needle test showed myotonic discharges were more prominent in the distal muscles in upper and lower limbs. Full article

Prognostic factors and long-term treatment response of interferon β-1a s.c tiw has not been studied in a real-life clinical cohort in Finland. The aim of the paper was to evaluate long-term treatment response, prognostic clinical factors and adherence among interferon β-1a s.c tiw treated patients in Finland. A retrospective review of medical records was performed. Confirmed relapsing multiple sclerosis patients treated with interferon β-1a s.c tiw 22μg or 44μg as their first treatment, from 1996 to 2010 in Western Finland, were included. Longitudinal generalized linear regression models were applied to assess risk of disability progression, using Expanded Disability Status Scale (EDSS), during the treatment period. Odd’s ratios with 95% confidence intervals (95% CI) were calculated for risk factors: gender, age at diagnosis, treatment delay, dose, baseline EDSS and EDSS change in one year. Kaplan-Meier was applied to study median time to discontinuation. Mean duration of treatment in 293 cases was 2.9 years (min 0.04, max 13.5). EDSS increase vs. no increase in one-year carried a significant risk for long-term disability progression (1.20, 1.08-1.33). Older age, defined by a 10-year increase in age at diagnosis (1.43, 1.07 -1.91) and one-year delay to treatment start showed an increased risk for disability progression (1.05, 0.99-1.11), but gender (0.66, 0.38-1.15) or initial dose (1.00, 0.45-2.25) showed no risk. Treatment was stopped in 37% due to disease activation at median of 1.7 years, and in 25% due to side effects at 9.3 months. Our results show that young age, a short delay to treatment start and slower disability progression were identified as factors for better outcome among cases with interferon β-1a s.c tiw as their first disease modifying treatment. Full article

A 69-year-old man who had been bedridden in nursing home because of a 5-year history of progressive supranuclear palsy (PSP) was admitted due to aspiration pneumonia. Besides neck dystonia in extension, he showed “alternating flexed–extended posturing”, in which the arm was flexed on one side and extended on the other. Magnetic resonance imaging of the brain revealed global cerebral atrophy that predominantly affected the cortex and midbrain. The mechanisms of complex posturing in late-stage PSP may sometimes be related to decortication and decerebration as well as dystonia, and “alternating flexed–extended posturing” might be one of the phenotypes of pathological progression in PSP. Full article

One of the best characterized autoimmune encephalitis is the Anti-Nmethyl-D-aspartate receptor (NMDAR) encephalitis, which may occur in the presence of cancer. First- and second-line immunotherapy and oncological investigations are suggested. We present here a case of an 18-year-old female who was our first patient suffering from Anti-NMDAR encephalitis more than 9 years ago. She was satisfactorily treated with intravenous immunoglobulins and high dose steroid therapy. After more than one year the patient had a relapse. First-line immunotherapy was repeated; however, a complete recovery was achieved only after plasmapheresis. Afterwards, she continued maintenance immunotherapy with steroids for two years and with Azathioprine for about five years associated to regular oncological assessment. In the last years our therapeutical approach of Anti-NMDARencephalitis has significantly changed. Nevertheless, established treatment guidelines are still missing and the role of long-term maintenance immunotherapy is largely unexplored. In addition, oncological revaluation might be indicated in selected patients. Full article

Ocular manifestations of Lyme disease (LD) remain a rare feature of the disease, but it may present a wide range of clinical presentations with different combinations. LD related optic neuritis or cranial nerve (CN) six palsy have been reported in the literature. However, this is the first case report of simultaneous involvement of CN 2 and CN 6 in a patient with LD. The diagnosis of LD can be challenging and initial laboratory tests can be a false negative. It is paramount important to repeat the diagnostic test if clinical suspicious is ongoing. With this case, we aim to increase awareness of clinicians for possible ocular manifestations of LD and its complex diagnostic process. Full article

Neuropsychiatic systematic lupus erythematosus (NPSLE) is a form of SLE involves the inflammation and/or thrombotic event in the nervous system. Patients with NPSLE are likely to have a positive antiphospholipid antibody (aPL), therefore are at higher risk of recurrent ischemic stroke. The management of NPSLE with aPLrelated stroke is rather different from the traditional ischemic stroke. One must treat it with anticoagulation and immunosuppressive therapy. The present case is a 47-yearold Taiwanese female with NPSLE and positive aPL, presented with a recurrent MCA ischemic stroke. Initial laboratory results showed significantly elevated levels of anti-ANA, anti-dsDNA, anti-cardiolipin, and decreased complement levels. Due to multiple contraindications for tPA, she was treated with antiplatelet, anticoagulation, steroid pulse therapy, and plasmapheresis during the hospitalization. Despite treatments, her stroke progressed to multi-focal lesions, involving the ACA, MCA, and basal ganglion. On follow up of her brain CT scan showed tissue edema and suspicious for subfalcine herniation. Responding to this clinical deterioration, we stopped warfarin and started mannitol. Eventually, her condition improved and was transferred to the rehabilitation program. Currently, there is no unified guideline regarding the secondary prevention of ischemic stroke in NPSLE with aPL patients. Additionally, previously reported use of steroid pulse therapy and plasmapheresis can potentially harm the patient. Clinicians must be cautious when treating such patient. Full article

In this article, we discuss the clinical approach to patients with dropped head syndrome and identify the various neuromuscular causes of dropped head syndrome including muscle, neuromuscular junction, peripheral nerve and motor neuron etiologies. We aim to increase awareness of recognition the entity of dropped head syndrome and factors that may predict response to immunomodulating therapy in dropped head syndrome. Full article