Neurol. Int., Volume 12, Issue 3 (December 2020) – 12 articles

The jugular foramen and the hypoglossal canal are both apertures located at the base of the skull. Multiple lower cranial nerve palsies tend to occur with injuries to these structures. The pattern of injuries tend to correlate with the combination of nerves damaged. Case Report: A 28-year-old male was involved in an AVP injury while crossing the highway. Exam showed a GCS of 15 AAOx3, with dysphagia, tongue deviation to the right, uvula deviation to the left and a depressed palate. Initial imaging showed B/L frontal traumatic Sub-Arachnoid Hemorrhages (tSAH), Left Frontal Epidural Hematoma and a Basilar Skull Fracture. On second look by a trained Neuroradiologist c At 3 month follow up, patient’s tongue normalized to midline and his dysphagia resolved. Discussion: Collette-Sicard syndrome is a rare condition/syndrome characterized by unilateral palsy of CN: IX, X, XII. This condition has been rarely described as a consequence of blunt head trauma. In most cases, the condition is self-limiting with patients regaining most to all of their neurological functions within 6 months. Nerve traction injuries and soft tissue edema compressing the cranial nerves are the leading two hypothesis. In conclusion, injuries with focal neurological deficits which were not apparent on initial imaging should be reviewed by relevant experts with concomitant knowledge of the patient’s history. Full article

Hemichorea–hemiballism (HC–HB) is a spectrum of involuntary flinging and flailing, non-patterned, irregular movements involving one side of the body. A rare dysfunction of glucose metabolism leading to a state of non-ketotic hyperglycemia (NKH) is thought to be a cause of these symptoms. In previous case studies, imaging findings have been in the basal ganglia as hyperintense lesions on magnetic resonance imaging (MRI) or hyperdensities on computerized tomography (CT). This case is unique due to abnormal findings in the MRI T2/fluid-attenuated inversion recovery (FLAIR) sequence in areas not previously reported—the thalamus and midbrain/pons. As in other NKH cases, the patient improved both clinically and radiologically. In patients with uncontrolled diabetes and abnormal movements, monitoring of blood glucose is imperative as it can lead to recognition of HC–HB. Other etiologies, including stroke, neoplasm, demyelination, and inflammatory processes, have uncertain prognoses with unfavorable outcomes. The prognosis for NKH is usually favorable, and thus important to identify. Full article

Parkinson’s disease (PD) is a common neurodegenerative disorder that leads to significant morbidity and disability. PD is caused by a loss of dopaminergic, cholinergic, serotonergic, and noradrenergic neurons in the central nervous system (CNS), and peripherally; the syndromic parkinsonism symptoms of movement disorder, gait disorder, rigidity and tremor are mostly driven by the loss of these neurons in the basal ganglia. Unfortunately, a significant proportion of patients taking levodopa, the standard of care treatment for PD, will begin to experience a decrease in effectiveness at varying times. These periods, referred to as “off episodes”, are characterized by increased symptoms and have a detrimental effect on quality of life and disability. Istradefylline, a novel adenosine A2A receptor antagonist, is indicated as a treatment addition to levodopa/carbidopa in patients experiencing “off episodes”. It promotes dopaminergic activity by antagonizing adenosine in the basal ganglia. This review will discuss istradefylline as a treatment for PD patients with off episodes. Full article

Multiple sclerosis (MS) is a prevalent and debilitating neurologic condition characterized by widespread neurodegeneration and the formation of focal demyelinating plaques in the central nervous system. Current therapeutic options are complex and attempt to manage acute relapse, modify disease, and manage symptoms. Such therapies often prove insufficient alone and highlight the need for more targeted MS treatments with reduced systemic side effect profiles. Ozanimod is a novel S1P (sphingosine-1-phosphate) receptor modulator used for the treatment of clinically isolated syndrome, relapsing–remitting, and secondary progressive forms of multiple sclerosis. It selectively modulates S1P1 and S1P5 receptors to prevent autoreactive lymphocytes from entering the CNS where they can promote nerve damage and inflammation. Ozanimod was approved by the US Food and Drug Administration (US FDA) for the management of multiple sclerosis in March 2020 and has been proved to be both effective and well tolerated. Of note, ozanimod is associated with the following complications: increased risk of infections, liver injury, fetal risk, increased blood pressure, respiratory effects, macular edema, and posterior reversible encephalopathy syndrome, among others. Further investigation including head-to-head clinical trials is warranted to evaluate the efficacy of ozanimod compared with other S1P1 receptor modulators. Full article

Objective: to find the most up-to-date evidence of the effectiveness and safety of supervised physical therapy in polymyositis/dermatomyositis patients. Methods: a systematic review of the literature in the main scientific databases was carried out. We searched for randomized controlled trials concerning supervised physical therapy and polymyositis/dermatomyositis. The PICOS method was used for the formulation of the clinical query. Methodological quality and the level of evidence of the included studies were assessed using the modified Jadad scale and the Oxford Centre for Evidence-Based Medicine Levels of Evidence guide, respectively. Results: a total of 2591 articles were found. By applying the inclusion/exclusion criteria, six randomized controlled clinical trials were admitted to the final phase of the review. The compared approaches concerned supervised exercise programs based on strategies of muscle strengthening or aerobic work. Following these exercises, an increase in the maximum rate of oxygen consumption, a decrease in creatine phosphokinase levels, an enhancement in the patient’s aerobic performance and an improvement in the quality of life indexes were registered. The methodological quality of the included studies ranged from 3 to 4.5. All the studies were classified as presenting an evidence level of 2b. Conclusions: supervised physical therapy in polymyositis/dermatomyositis is an effective, safe and free-of-contraindications tool to be used both in the acute and in the established phases of the pathology. However, further and higher-quality studies are necessary to confirm those findings, to clarify the timing of exercise delivery and to guide the choice towards different types of muscle contraction exercises. Full article

Neurological disorders, including Parkinson’s disease (PD), have increased in prevalence and are expected to further increase in the coming decades. In this regard, PD affects around 3% of the population by age 65 and up to 5% of people over the age of 85. PD is a widely described, physically and mentally disabling neurodegenerative disorder. One symptom often poorly recognized and under-treated by health care providers despite being reported as the most common non-motor symptom is the finding of chronic pain. Compared to the general population of similar age, PD patients suffer from a significantly higher level and prevalence of pain. The most common form of pain reported by Parkinson’s patients is of musculoskeletal origin. One of the most used combination drugs for PD is Levodopa-Carbidopa, a dopamine precursor that is converted to dopamine by the action of a naturally occurring enzyme called DOPA decarboxylase. Pramipexole, a D2 dopamine agonist, and apomorphine, a dopamine agonist, and Rotigotine, a dopamine receptor agonist, have showed efficacy on PD-associated pain. Other treatments that have shown efficacy in treating pain of diverse etiologies are acetaminophen, Nonsteroidal anti-inflammatory drugs (NSAIDs), and cyclooxygenase-2 (COX-2) inhibitors. Opioids and opioid-like medications such as oxycodone, morphine, tramadol, and codeine are also commonly employed in treatment of chronic pain in PD. Other opioid related medications such as Tapentadol, a central-acting oral analgesic with combined opioid and noradrenergic properties, and Targinact, a combination of the opioid agonist oxycodone and the opioid antagonist naloxone have shown improvement in pain. Anticonvulsants such as gabapentin, pregabalin, lamotrigine, carbamazepine and tricyclic antidepressants (TCAs) can be trialed when attempting to manage chronic pain in PD. The selective serotonin and noradrenaline reuptake inhibitors (SNRIs) also possess pain relieving and antidepressant properties, but carry less of the risk of anticholinergic side effects seen in TCAs. Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has been shown in multiple studies to be effective against various types of PD associated pain symptoms. Massage therapy (MT) is one of the most common forms of complementary and alternative medicine. Studies have shown that pressure applied during MT may stimulate vagal activity, promoting reduced anxiety and pain, as well as increasing levels of serotonin. In a survey study of PD patients, rehabilitative therapy and physical therapy were rated as the most effective for pain reduction, though with only temporary relief but these studies were uncontrolled. Yoga has been studied for patients with a wide array of neurological disorders. In summary, PD pathology is thought to have a modulating effect on pain sensation, which could amplify pain. This could help explain a portion of the higher incidence of chronic pain felt by PD patients. A treatment plan can be devised that may include dopaminergic agents, acetaminophen, NSAIDs, opioids, antidepressants, physical therapies, DBS and other options discussed in this review. A thorough assessment of patient history and physical examination should be made in patients with PD so chronic pain may be managed effectively. Full article

Having served as editor-in-chief for Neurology International since its inception back in 2009, I am very pleased to see the journal grow and expand, with a major development now taking place with new ownership by MDPI, a world leader in Open Access Journal publication [...] Full article

Late-onset Pompe disease (LOPD) is an autosomal recessive lysosomal storage disease. Clinical features include skeletal muscle deficiency and diaphragm weakness. Clinical management relies on supportive treatment and mechanical ventilation in patients with chronic respiratory failure. M mode ultrasound and sniff tissue Doppler imaging can be used to assess and follow diaphragm function. Full article

Introduction: Gait velocity in spastic patients after stroke is both a life quality and mortality predictor. However, the precise biomechanical events that impair a faster velocity in this population are not defined. This study goal is to find out which are the gait parameters associated with a higher velocity in stroke patients with spastic paresis. Methods: The registries of a Gait analysis laboratory were retrospectively analyzed. The inclusion criteria were: trials of adult stroke patients with unilateral deficits. The exclusion criteria were: trials when patients used an external walking device, an orthosis, or support by a third person. Of the 116 initial patients, after the application of the exclusion criteria, 34 patients were included in the cohort, all with spatiotemporal, static and dynamic kinematic and dynamometric studies. Results: There was a correlation of velocity with cadence, stride length of the paretic (P) limb, stride length, and time of the P and non-paretic (NP) limb, double support time, all the parameters related to hip extension during stance phase, knee flexion during swing phase, and parameters related to ankle plantarflexion during stance phase. Conclusions: The main gait analysis outcomes that have a correlation with speed are related to the formula velocity = step length × cadence or are related to stance phase events that allow the anterior projection of the body. The only swing phase outcome that has a correlation with speed is knee flexion. More studies are needed from gait analysis laboratories in order to point out the most relevant goals to achieve with gait training in spastic stroke patients. Full article

Ependymoblastoma is an uncommon, exceedingly malignant brain neoplasm that adversely influences children’s quality of life. Ependymoblastoma represents a subtype of primitive neuroectodermal tumors, categorized as grade IV, according to the 2007 World Health Organization (WHO) classification of central nervous system tumors. Ependymoblastomas are often located in the supratentorial zone and often associated with the ventricular system. Histopathological sections of the tumor revealed uniform, primitive, small blue cells, with multi-layered rosettes, accompanied by abundant mitoses. The clinical and imaging features of ependymoblastomas are not specific, which can result in misdiagnosis as other brain neoplasms. In this paper, we described the identification of a fourth-ventricular ependymoblastoma that was misdiagnosed as pilocytic astrocytoma, despite the utilization of advanced magnetic resonance imaging (MRI) protocols. Full article

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