CASE REPORT OF ADULT-ONSET CHARCOT MARIE TOOTH TYPE X

Charcot-Marie-Tooth (CMT) or Hereditary Motor and Sensory Neuropathy (HMSN) is the most common hereditary peripheral nerve disease with progressive chronic weakness, muscle atrophy, and sensory disturbances. There are several types and subtypes of CMT with their respective clinical manifestations. In this article, we reported a patient with of CMT type X. A 43-year-old male patient was referred to a neurology clinic with weakness in both limbs for 2 years, accompanied by tingling and sensory disturbance in both hands and feet. There are several of his family members who had similar complaints. Lumbosacral magnetic resonance imaging (MRI) examination revealed mild nucleus pulposus herniation. Electroneuromyography (ENMG) examination revealed demyelinating sensory motor polyneuropathy. Histopathological examination of nerve biopsy showed demyelination of the sural nerve. It is hard to make a diagnosis of CMT, because it requires high suspicion from clinicians once encounter a suspected case and also need to supported by sophisticated equipment such as electrophysiological examinations, nerve biopsy examinations, and genetic examinations. It is vital for clinicians for being able to diagnose CMT correctly and provide treatment as soon as possible in order to maintain the patients’ quality of life.

The Role of Monoamine Oxidase B Inhibitors in the Treatment of Parkinson’s Disease – An Update

Parkinson's disease (PD) is a progressive neurodegenerative disease characterised by reduced dopamine level in the substantial nigra. This may lead to typical motor features such as bradykinesia, resting tremors and rigid muscles; as well as non-motor symptoms such as neuropsychiatric symptoms, sleep disorders, autonomic dysfunction, and sensory disturbances. Inhibitors of MAO-B are used to alleviate symptoms by reducing monoamine oxidase-catalysed degradation of dopamine; hence, preserving functional levels of dopamine. The very first MAO-B used therapeutically was selegiline, followed by rasagiline, its indane derivative which has superior efficacy and selectivity. Both inhibitors can be used as monotherapy or in combination with other anti-Parkinson drugs. Safinamide, a reversible MAO-B inhibitor that utilises both dopaminergic and non-dopaminergic mechanisms, was recently approved by the European Medicines Agency (EMA) (2015) and U.S. FDA (2017) as an add-on therapy for patients with mid- or late-stage Parkinson’s disease. Furthermore, MAO-B inhibitors were found to be associated with potential neuroprotective and disease modifying effects. However, evidence of their efficacy and role in PD models are scarce and warrants further investigation.

Cervical Cord Compression With Lower Limbs Sensory Disturbance Presentation: Three Case Reports and Literature Review

Background: Lower limb sensory disturbance presentation can be a false localizing cervical cord compressive myelopathy (CSM). It may lead to delayed or missed diagnosis, resulting in the wrong management plan, especially in the presence of concurrent lumbar lesions.Case presentation:Three Asian patients with lower limb sensory disturbances presentation were treated ineffectively in the lumbar. Magnetic resonance imaging (MRI) showed cervical disc herniation and cervical level spinal cord compression. Anterior cervical discectomy surgery and zero-p interbody fusion were performed. After operations, imagings showed that the spinal cord compression were relieved, and the lower limbs sensory disturbances were also relieved. Three-months follow-up after operation showed good recovery.Conclusions:These three cervical cord compression cases of lower limb sensory disturbance presentation were easily misdiagnosed with lumbar spondylosis. Anterior cervical discectomy and fusion operation had a good therapeutic effect. Therefore, cases that present with lower limb sensory disturbance, but in a non-radicular classical pattern, should always alert a suspicion of a possible cord compression cause at a higher level.

A case of red nucleus and hillock syndrome

Th. Dosuzkov in Revue v neurologii i psychiatrii, 1928, no. In addition to the presence of semi-paresis, sensory disturbances, pain, intentional tremor, the author indicates cerebellar disorders on the same side, changes in local setting reflexes on both sides, and on the part of the psyche, euphoria and mild dementia.

Detailed Analysis of Neurological Symptoms and Sensory Disturbances Due to Chronic Arsenic Exposure in Toroku, Japan

As a result of population growth and the development of tube wells, humans’ exposure to arsenic has increased over the past few decades. The natural course of organ damage secondary to arsenic exposure is not yet well understood. In Toroku, Japan, an arsenic mine was intermittently operated from 1920 to 1962, and residents were exposed to high concentrations of arsenic. In this paper, we analyzed 190 consecutive residents for whom detailed records of neurological symptoms and findings were obtained from 1974 to 2005. All participants were interviewed regarding the presence of general, skin, hearing, respiratory, and neurological symptoms. Neurological symptoms were classified into extremity numbness or pain, constipation, dyshidrosis, sensory loss, and muscle atrophy. Superficial and vibratory sensation was also evaluated. More than 80% of participants experienced extremity numbness, and numbness was the most common neurological symptom. Numbness was associated with superficial sensory disturbance, and was correlated with the subsequent development of other neurological symptoms, including autonomic and motor symptoms. No previous studies have investigated the natural course of chronic arsenic intoxication; thus, these data serve as a guide for detecting early symptoms due to arsenic exposure.

Case Report: Abnormal ECG and Pantalgia in a Patient With Guillain–Barré Syndrome

Background: Guillain–Barré syndrome (GBS) is an acute immune-mediated disorder in the peripheral nervous system (PNS) characterized by symmetrical limb weakness, sensory disturbances, and clinically absent or decreased reflexes. Pantalgia and dysautonomia, including cardiovascular abnormalities, are common findings in the spectrum of GBS. It is usually challenging to distinguish GBS-related electrocardiogram (ECG) abnormities and chest pain from acute coronary syndrome (ACS) in patients with GBS due to the similar clinical symptom and ECG characteristics. Here, we present a case of GBS complicating ACS.Case Summary: A 37-year-old woman with a 2-month history of GBS presented to the emergency department due to pantalgia. The ECG showed a pattern of transitional T-wave inversion in the leads I, aVL, and V2 through V4 and shortly returned to normal, which appeared several times in a short time, but lab testing was unremarkable. Then, a further coronary computed tomography angiography (CTA) revealed the presence of critical stenosis of the left anterior descending artery, leading to the diagnosis of ACS. During the follow-up, she suffered from a non-ST-elevation myocardial infarction and accepted revascularization of the left anterior descending artery in the second week after discharge.Conclusion: Guillain–Barré syndrome could accompany chest pain and abnormalities on ECG. Meanwhile, it is essential to bear in mind that “GBS-related ECG abnormalities and chest pain” is a diagnosis of exclusion that can only be considered after excluding coronary artery disease, especially when concomitant chest pain, despite being a common presentation of pantalgia, occurs.

Discrepancy in the Usage of GFAP as a Marker of Satellite Glial Cell Reactivity

Satellite glial cells (SGCs) surrounding the neuronal somas in peripheral sensory ganglia are sensitive to neuronal stressors, which induce their reactive state. It is believed that such induced gliosis affects the signaling properties of the primary sensory neurons and is an important component of the neuropathic phenotype leading to pain and other sensory disturbances. Efforts to understand and manipulate such gliosis relies on reliable markers to confirm induced SGC reactivity and ultimately the efficacy of targeted intervention. Glial fibrillary acidic protein (GFAP) is currently the only widely used marker for such analyses. However, we have previously described the lack of SGC upregulation of GFAP in a mouse model of sciatic nerve injury, suggesting that GFAP may not be a universally suitable marker of SGC gliosis across species and experimental models. To further explore this, we here investigate the regulation of GFAP in two different experimental models in both rats and mice. We found that whereas GFAP was upregulated in both rodent species in the applied inflammation model, only the rat demonstrated increased GFAP in SGCs following sciatic nerve injury; we did not observe any such GFAP upregulation in the mouse model at either protein or mRNA levels. Our results demonstrate an important discrepancy between species and experimental models that prevents the usage of GFAP as a universal marker for SGC reactivity.

A 50-Year-Old Patient with Guillain–Barré Syndrome after COVID-19: A Case Report

Severe acute respiratory syndrome coronavirus 2, or SARS-CoV-2, causes acute respiratory disease (coronavirus disease 2019; COVID-19). However, the involvement of other mechanisms is also possible, and neurological complications are being diagnosed more frequently. Here, we would like to present a case of a Polish patient with Guillain–Barré syndrome (GBS), after a documented history of COVID-19: A 50-year-old man, 18 days after the onset of COVID-19 symptoms, had progressive quadriparesis preceded by 1-day sensory disturbances. Based on the clinical picture, the results of diagnostic work-up including a nerve conduction study (ENG) that revealed a demyelinating and axonal sensorimotor polyneuropathy, and cerebrospinal fluid (CSF) analysis that showed albumin–cytological dissociation, an acute inflammatory demyelinating polyneuropathy was confirmed, consistent with GBS. Upon a therapeutic plasma exchange (TPE), the patient’s condition improved. The presented case of GBS in a patient after mild COVID-19 is the first case in Poland that has supplemented those already described in the global literature. Attention should be drawn to the possibility of GBS occurring after SARS-CoV-2 infection, even when it has a mild course.

CHARACTERISTICS OF PATIENTS USING FIBRINOLYTIC MEDICINE - ALTEPLASE TREATING ACUTE ISCHEMIC STROKE

Objective: To investigate the characteristics of patients using fibrinolytics medicine - Alteplase in the treatment of acute ischemic stroke.Subjects and methods: A retrospective cross-sectional and non-interventional study on 52 stroke patients using Alteplase at Can Tho City General Hospital from April 2019 to October 2020.Results: Male accounted for 53.8%, average age 58.31 years old. Risk factors included a history of hypertension (53.8%), diabetes (21.8%), stroke (6.4%), atrial fibrillation (2.6%), and smoking.(15.4%). Common clinical symptoms included weakness/hemiplegia (88.5%), speech disorder (84.6%), facial paralysis (82.7%), and sensory disturbances (71.2%) and oculomotor limitation (23.1%). Complications of extracranial hemorrhage (17%), progressive ischemic stroke (9.7%), allergies (4.8%), new embolism (2.4%) and no cerebral hemorrhage patient. The average NIHSS score at admission was 11.8 ± 7.1 points, decreasing at the time of 24 hours and discharge, respectively, 5.8 ± 5.3 and 4.2 ± 5, 5 points. The difference was statistically significant between the study times.Conclusion: The average age of patients was 58.31 years old, mainly men. Hypertension, diabetes, and smoking were the most common risk factors. Weakness/hemiplegia, speech disorder, facialparalysis, and sensory disturbances were common symptoms. The NIHSS score decreased gradually at 24 hours after admission and at discharge compared with the time of admission.