European Federation Of Neurological Societies Research Articles

Clinical characteristics of children with Borrelia-related peripheral facial palsy and utility of cerebrospinal fluid testing.

To describe the aetiology and clinical characteristics of acute peripheral facial palsy (PFP) in children and investigate the utility of the European Federation of Neurological Societies (EFNS) criteria for diagnosing Borrelia-related PFP (BPFP) based on cerebrospinal fluid (CSF) testing and the Centers for Disease Control and Prevention (CDC) criteria based on serology. We retrospectively identified children aged <18 years diagnosed with acute PFP between 2014 and 2020. We used the EFNS criteria as the gold standard and the CDC criteria for diagnosing BPFP. Out of 257 children with PFP, 93 (36%) fulfilled the EFNS or CDC criteria for BPFP. We found a discrepancy between the EFNS criteria with CSF testing and the CDC without CSF testing in 27 (14%) of the 190 children with available data. Of the 37 children with PFP and ≥2 symptoms of fever, fatigue, nausea/vomiting or meningeal symptoms, 31 (84%) fulfilled the EFNS criteria for BPFP. Borrelia is a common cause of PFF in children, and its prevalence is higher in children with systemic symptoms. Also, CSF testing did not have decisive management implications in most cases. Therefore, clinical evaluation and Borrelia serology could be the initial steps in the diagnosis of PFP in children.

Modern aspects of diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy in children

Analysis of demographic, clinical, laboratory, electrophysiological and neuroimaging data and pathogenetic therapy of pediatric patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients (n=30) were observed in a separate structural unit of the Russian Children's Clinical Hospital of the Russian National Research Medical University named after. N.I. Pirogova Ministry of Health of the Russian Federation in the period from 2006 to 2023. The examination was carried out in accordance with the recommendations of the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society on the Management of CIDP (2021). All patients received immunotherapy, including intravenous immunoglobulin (IVIG) (n=1), IVIG and glucocorticosteroids (GCS) (n=17, 56.7%), IVIG+GCS+plasmapheresis (n=12, 40.0%). Alternative therapy included cyclophosphamide (n=1), cyclophosphamide followed by mycophenolate mofetil (n=1), rituximab (n=2, 6.6%), azathioprine (n=3), mycophenolate mofetil (n=2, 6.6%). In all patients, there was a significant difference between scores on the MRCss and INCAT functional scales before and after treatment. At the moment, 11/30 (36.6%) patients are in clinical remission and are not receiving pathogenetic therapy. The median duration of remission is 48 months (30-84). The longest remission (84 months) was observed in a patient with the onset of CIDP at the age of 1 year 7 months. Early diagnosis of CIDP is important, since the disease is potentially curable; early administration of pathogenetic therapy provides a long-term favorable prognosis.

Case report: Successive ipsilateral and contralateral laryngeal nerve palsy as probable manifestation of neuroborreliosis

Neuroborreliosis is part of advanced stage of Lyme disease and often characterized by damage to the cranial and/or peripheral nerves. Involvement of one or both recurrent nerves is rare. Diagnosis is often difficult and based on a set of clinical manifestations, biological arguments, and cerebrospinal fluid (CSF) analysis. A 70-year-old man was referred to our Voice Clinic with a 3-month history of dysphonia caused by right vocal fold paralysis (VFP) without any cutaneous symptoms of tick bite or erythema migrans in the previous weeks and normal initial radiological examination (neck and thorax CT). Methylprednisolone had already been prescribed but without any clinical improvement. Late biological investigation 3 months after initial symptoms of VFP showed high IgG (93 U/mL; reference <10 U/mL) against Borrelia burgdorferi (BB), which was confirmed by two immunoblot markers (VIsE, p39 antigens). Therefore, a possible manifestation of Lyme disease with involvement of the right inferior laryngeal nerve was suspected, namely Lyme neuroborreliosis. However, given the spontaneous recovery of the patient after 7 months without any adapted antimicrobial regimen treatment, the diagnosis of neuroborreliosis was not confirmed by a lumbar puncture. Nineteen months later, the patient presented again for the same symptomatology but as left VFP. High IgG (68 U/mL) and IgM (>6, reference <0.90) levels against BB were confirmed by immunoblot. Subsequently, lumbar puncture was performed and revealed IgG against BB at 46.1 UA/mL (reference<5.5 UA/mL) in the CSF, with an extremely high IgG intrathecal synthesis antibody index (281.33, positive if > 1.5). Intrathecal antibody synthesis is the gold standard for Lyme neuroborreliosis demonstrating a specific immune response to BB in the central nervous system, but with the limitation of persistence for years after eradication. Our patient did not exhibit pleocytosis in the CSF. Therefore, two criteria of the European Federation of Neurological Societies (EFNS) guidelines are fulfilled for possible neuroborreliosis. Doxycycline treatment led to rapid recovery in less than 8 weeks and normal mobility of the left vocal fold. Because of this very uncommon clinical presentation with two successive episodes of VFP for no other obvious reason and serological evidence from the serum and CSF during the second episode, we consider it possible that the first episode of VFP could also have been a manifestation of neuroborreliosis. This case is the first report of possible relapse of laryngeal palsy successively on the right, and then the left side as a manifestation of Lyme neuroborreliosis.

Enteroviral central nervous system infections in patients with Lyme neuroborreliosis

Patients with Lyme neuroborreliosis (LNB) are rarely tested for the presence of neurovirulent viruses other than tick-borne encephalitis virus (TBEV); however, such coinfections could be of clinical importance. The aim of the study was to search for the presence of neurotropic viruses in a LNB patients.Fourteen patients admitted with signs and symptoms of neuroinfection who were eventually diagnosed to have LNB (according to the guidelines of the European Federation of Neurological Societies) were subjects of the study. Sera and cerebrospinal fluid (CSF) collected at the time of initial presentation were tested for viral pathogens most common in our geographical area: human enteroviruses (EV), herpes simplex virus type 1 and 2, varicella-zoster virus, Epstein-Barr virus, cytomegalovirus, human herpesvirus type 6, human adenoviruses, and TBEV using PCR/RT-PCR and serological assays. RNA and DNA-based metagenomic next-generation sequencing (mNGS) was used to detect other viral pathogens.EV was detected in CSF from two (14 %) LNB patients and viral loads were similar (220 and 270 copies/ml). The mMGS analysis were performed on CSFs from 10 patients and generated a total 213,750,885 NGS reads, 0.05 % of which were viral. However, none of potential pathogens fulfilled the criteria for positive viral detection by mNGS.Using a number of PCR/RT-PCR assays and mNGS we identified EV infection in two out of 14 LNB patients. The possible co-occurrence of enterovirus and Lyme neuroborreliosis infections may warrant further research.

Pediatric-Onset Chronic Inflammatory Demyelinating Polyneuropathy: A Multicenter Study

BackgroundTo evaluate the clinical features, demographic features, and treatment modalities of pediatric-onset chronic inflammatory demyelinating polyneuropathy (CIDP) in Turkey. MethodsThe clinical data of patients between January 2010 and December 2021 were reviewed retrospectively. The patients were evaluated according to the Joint Task Force of the European Federation of Neurological Societies and the Peripheral Nerve Society Guideline on the management of CIDP (2021). In addition, patients with typical CIDP were divided into two groups according to the first-line treatment modalities (group 1: IVIg only, group 2: IVIg + steroid). The patients were further divided into two separate groups based on their magnetic resonance imaging (MRI) characteristics. ResultsA total of 43 patients, 22 (51.2%) males and 21 (48.8%) females, were included in the study. There was a significant difference between pretreatment and post-treatment modified Rankin scale (mRS) scores (P < 0.05) of all patients. First-line treatments include intravenous immunoglobulin (IVIg) (n = 19, 44.2%), IVIg + steroids (n = 20, 46.5%), steroids (n = 1, 2.3%), IVIg + steroids + plasmapheresis (n = 1, 2.3%), and IVIg + plasmapheresis (n = 1, 2.3%). Alternative agent therapy consisted of azathioprine (n = 5), rituximab (n = 1), and azathioprine + mycophenolate mofetil + methotrexate (n = 1). There was no difference between the pretreatment and post-treatment mRS scores of groups 1 and 2 (P > 0.05); however, a significant decrease was found in the mRS scores of both groups with treatment (P < 0.05). The patients with abnormal MRI had significantly higher pretreatment mRS scores compared with the group with normal MRI (P < 0.05). ConclusionsThis multicenter study demonstrated that first-line immunotherapy modalities (IVIg vs IVIg + steroids) had equal efficacy for the treatment of patients with CIDP. We also determined that MRI features might be associated with profound clinical features, but did not affect treatment response.

The influence of osteopathic correction on liquorodynamics in patients with tension-type headache

Introduction. Tension-type headache (TTH) is known to be the most common type of headache in all age groups. The guidelines of the European Federation of Neurological Societies, the Italian Guidelines for Primary Headaches and the Italian Consensus Conference on Pain in Neurorehabilitation report that non-pharmacological therapies are valid adjunctive treatments for TTH. Previous studies have shown that the use of general osteopathic treatment in patients with TTH is accompanied by a significant decrease in the severity of pain syndrome and asthenic condition. We did not find any scientific publications devoted to the objectification of the results of osteopathic correction in TTH using magnetic resonance imaging (MRI).The aim of the study was to objectify the results of osteopathic correction by assessing changes in the liquor dynamics of the posterior cranial fossa in patients with tension-type headache.Materials and methods. The study was conducted from December 2020 to December 2021 at the clinic of the Center for New Medical Technologies, Novosibirsk. There were under the observation 10 patients with an established diagnosis of TTH aged from 18 to 55 years, 4 men, and 6 women. All patients before the start of treatment and after the course completion were assessed for their osteopathic status and underwent high-field MRI 3T of the brain with the calculation of the posterior cranial fossa restriction index (CFRI). CFRI reflects the state of liquorodynamics at the level of the skull base and shows the level of freedom in the relationship between fluid spaces and brain tissues. Study participants received a course of osteopathic correction, which included 3–4 procedures with an interval of 5–7 days. The observed patients did not receive any other therapy during the study period.Results. The examined patients were most characterized by regional biomechanical disorders (RBD): head (9); neck, structural component (5); thoracic, visceral component (5); dura mater region (9). In terms of severity, mild RBD prevailed (1 point). After treatment, patients have a decrease of the detection frequency of major regional somatic dysfunctions (SD). Statistically significant differences (p&lt;0,05) were obtained in the SD incidence of head region; neck region, structural component; thoracic, visceral component; dura mater region. A statistically significant (p&lt;0,05) increase in the mean CFRI from 30,22±0,63 to 31,78±0,73 % was found after the treatment.Conclusion. The results of the high-field MRI with the study of CFRI allow to quantitatively assess the changes of the cerebrospinal fluid dynamics in patients with tension-type headache, and it can be used as an objective criterion for the osteopathic correction results and the therapy clinical effectiveness. The study should be continued with a more representative sample.

European Federation of Neurological Societies: parent to the European Academy of Neurology.

The European Academy of Neurology (EAN) is a vigorous organization of great importance for all neurologists and for our patients. But how did neurology get organized at the European level? That is the topic of this article. Most important sources are memories and documents of the author, who was a moving force in developing the European Federation of Neurological Societies (EFNS), one of the two parents of the EAN. All European national neurological societies and the World Federation of Neurology were involved in a difficult political interaction resulting in the EFNS. Organizational and administrative development was the initial task. Scientific panels led by a scientific committee, teaching courses for young neurologists, teaching courses in middle and eastern Europe and successful congresses were developed. The purchase of headquarters as well as the creation of a fully owned scientific journal (European Journal of Neurology) were important and financially beneficial. The EFNS also promoted the formation of the European Brain Council and of the patient organization European Federation of Neurological Associations. All these elements have continued after fusion with the European Neurological Society to form the EAN. The very successful development of the EFNS has largely been carried on into the EAN.

Prevalence and associated factors with peripheral neuropathies in the general population in the rural area of Adjohoun in Benin

BackgroundPeripheral neuropathies (PN) are a group of neurological conditions related to damage to the peripheral nervous system. Due to their wide diversity, few studies in sub-Saharan Africa have explored their epidemiology in general population. Our objective was to study the prevalence and associated factors with PN in general population in Adjohoun in Benin. MethodsA cross-sectional study has been conducted from February to March 2019 and included people aged ≥ 18 years old living in Adjohoun, Benin. Following a screening phase for PN (using World Health Organization questionnaire for major neurological diseases), a neurologist made a diagnosis after in-depth clinical examinations completed in some cases by electroneuromyography. The EFNS (European Federation of Neurological Societies) 2010 criteria was used for chronic inflammatory demyelinating polyneuropathy diagnosis. Data such as age, occupation, consanguinity, alcohol consumption, diabetes, hypertension were collected. Association between independent variables and PN were investigated using multivariable logistic regression models. ResultsIn total, 1 655 participants were included, mean age 41.4 ± 16.7 years; 64.8 % are female. The overall prevalence of PN was 6.9 % (95 %CI: 5.7 %-8.2 %). The main types of PN were: polyneuropathies 4.8 % (95 %CI: 3.8 %-5.9 %); polyradiculoneuropathies 1.6 % (95 %CI: 1.0 %-2.2 %). Factors independently associated with PN were age ≥ 40 years (adjusted Odds Ratio aOR = 19.6; 95 %CI: 8.2–46.3), diabetes (aOR = 1.8; 95 %CI: 1.08–2.99) and hypertension (aOR = 1.6; 95 %CI: 1.02–2.5). ConclusionThe prevalence of PN was high in the rural commune of Adjohoun in Benin. Actions on its modifiable associated factors such as diabetes and hypertension could help reduce the proportion of PN and their potential harmful consequences.

European Projects for Patients with Dementia and Their Caregivers.

As we all know there is no treatment that can stop or delay the progression of dementia. The treatment we use is only symptomatic. EFNS (European Federation of Neurological Societies) recommendations for dementia prevention by Sorbi et al. (2012) concluded that there is no treatment, no lifestyle, which could have an effect on prevention or delay of onset of different forms of dementia until today. The future studies in prevention must recruit younger people, larger sample, and for longer period. The last 10years we have run, in collaboration with organizations in different European countries, many projects in order to support patients with neurodegenerative diseases, mainly patients with dementia and their caregivers. The first project was a 2-year prospective cohort study of antidementia drug non-persistency in mild-to-moderate Alzheimer's disease (AD) in Europe: predictors of discontinuation and switch in the ICTUS (Impact of Cholinergic Treatment USe) study, an FP5 project with 1380 patients. Five studies were published. The second project was DESCRIPA study, an FP5 project to DEvelopment of Screening guidelines and clinical CRIteria for Predementia Alzheimer's disease, with 881 patients with mild cognitive impairment (MCI). LLM (Long Lasting Memories) and VRADA (A virtual reality application for the exercise of dementia and Alzheimer patients) are two projects that include body and cognitive exercise for health for the elderly and patients with mild cognitive impairment. The next is the RECAGE (REspectful Caring for the AGitated Elderly) project (Horizon 2020), a prospective cohort study for coping with behavioral and psychological symptoms of dementia. With six European universities we finished a very interesting FP6 project, the AddNeuroMed one, which gives even now information about the progression of normal elderly MCI and AD patients, in collaboration with other consortia. A very interesting Innovative Medicines Initiative (IMI) project about digital biomarkers was entitled Remote Assessment of Disease and Relapse-Alzheimer's Disease (RADAR project). The main goal of this project was the development and validation of technology-enabled, quantitative and sensitive measures of functional decline in people with early-stage AD. A running project is an Erasmus+ one in the higher education field, "Genetic counseling in European universities: The case of neurodegenerative diseases" (GECONEU project). The target of this study is to develop an online course for university students focusing on genetic counseling, and support people and society to better understand the aims of genetic testing and the usefulness of genetic counseling by involving students in an innovative learning and teaching setting. AD-gaming, BRIDGE, iCONNECT (Intergenerational CONtact between studeNts and people with dEmentia through CreaTive education), E.L.So.M.C.I (English Lessons with the Use of Songs for People with Mild Cognitive Impairment), Games4CoSkills, and De-Sign are all Erasmus+ projects that aim to improve the quality of life of patients with MCI or dementia. Story2remember, Dementia right, ASPAD (Augmentation of the Support of Patients suffering from Alzheimer's Disease and their caregivers), INFOCARE (Supporting Informal Caregivers of People with Dementia), S.IN.CA.L.A (Supporting Informal Carers: A Whole-Family and Life course Approach), and PIA (Peer support workers as an Innovative force in Advocacy in dementia care) are all Erasmus+ projects for training and supporting caregivers of patients with dementia.

Socio-demographic Characteristics of Tick Bite and Erythema migrans not associated with the Diagnosis of Neuroboreliosis

Introduction Lyme neuroborreliosis (LNB) is a tick-borne infection caused by bacteria Borrelia burgdorferi sensu lato that accounts for 10-15% of all Lyme borreliosis cases in Europe. LNB can present with a variety of neurological manifestations. We aimed to describe the typical anamnestic, clinical and laboratory features of patients diagnosed with LNB and to describe the differences between paediatric and adult cases. Additionally, we assessed the factors associated with definite LNB. Methods We retrospectively evaluated data of patients with suspected LNB had undergone lumbar puncture and were admitted to the Infectious diseases department of University hospital Bratislava, Slovakia, between September 2019 and May 2022. Patients were divided into three categories according to the diagnostic criteria of European Federation of Neurological Societies: A) cases with definite LNB, B) cases with possible LNB, C) non-LNB controls. Results In total, 139 patients were included in the analysis. 32 individuals were classified as definite LNB, 23 as possible LNB and 84 as non-LNB controls. 55.5% were females and 35.3% were children aged&lt;18 years. 56.3% of patients with definite LNB reported a history of tick bite, and 21.9% a history of erythema migrans (EM). Peripheral facial nerve palsy (PFNP) was the most common clinical symptom in patients with definite LNB (65.6%), followed by headache (50.0%), fever (21.9%) and radicular pain (18.8%). In a univariate and multivariable analyses neither history of tick bite nor history of EM were significantly associated with definite LNB. Factors independently associated with definite LNB in multivariable analyses were (i) age &lt; 18 years (aOR 7.89, 95% CI 2.00-31.03, p &lt; 0,003), (ii) female gender (OR 6.34; 95% CI 1.66-24.17, p &lt; 0.007), and (iii) facial nerve palsy (OR 10.54; 95% CI 2.41-55.19, p &lt; 0.002). Conclusion We found that peripheral facial nerve palsy is the strongest predictor of definite LNB, and that the children&lt;18 years and females in our study were more likely to be diagnosed with LNB. Our study also suggests that anamnestic data on history of tick bite and EM contribute little to the diagnosis of LNB and that the examination of CSF is essential for the diagnosis of LNB.

Acute Cognitive Dysfunction in a Patient With Traumatic Brain Injury: A Clinical Vignette.

Acute Cognitive Dysfunction in a Patient With Traumatic Brain Injury: A Clinical Vignette.

Cerebrospinal fluid markers of inflammation and brain injury in Lyme neuroborreliosis- a prospective follow-up study.

The purpose of this study was to evaluate levels and kinetics of cerebrospinal fluid (CSF) markers of inflammation and brain injury in patients with Lyme neuroborreliosis (LNB). Adult patients with clinically suspected LNB were enrolled, in a prospective clinical study in the South East of Sweden. Patients were classified according to the European Federation of Neurological Societies' guidelines. Definite cases of LNB were re-examined one month later including a repeat CSF investigation. Routine laboratory parameters were investigated along with CSF levels of neurodegenerative markers glial fibrillary acidic protein (GFAp), total tau (t-tau) and neurofilament light protein (NFL), as well as neuroinflammatory markers soluble triggering receptor expressed on myeloid cells 2 (sTREM2), YKL-40 and CXCL13. Non-LNB served as controls. An additional comparison group consisted of spinal anesthesia subjects (SAS) without known central nervous system conditions. CSF levels of sTREM2 and CXCL13 were elevated in definite LNB patients at diagnosis compared with non-LNB patients (p<0.001) and SAS (p≤0.01). In addition, CSF levels of sTREM2, YKL-40 and CXCL13 rapidly declined in at follow-up after antibiotic treatment. In contrast, CSF levels of GFAp and t-tau did not differ across LNB groups, and did not change after treatment. Although in a limited number of LNB patients, the results indicate a predominance of microglial and neuroinflammatory involvement rather than parenchymal CNS injury in CSF at diagnosis of LNB with a prompt decline after antibiotic treatment. The findings provide pathogenetic insights and may be of value in differential diagnosis of CSF findings.

Role of skin punch biopsy in diagnosis of small fiber neuropathy-A review for the neuropathologist.

Over the last three decades, skin punch biopsy has become the gold standard for diagnosis of small fiber neuropathies, including autonomic neuropathies commonly seen in diabetics, patients with HIV, and children with hereditary sensory autonomic neuropathies and toxin-induced neuropathy. Clinical, biochemical, electrophysiological tests are inconclusive, making it difficult to diagnose and initiate treatment. A skin punch biopsy is easy to perform in the outpatient clinic, is highly sensitive, and provides an objective diagnosis. Importantly, it helps avoid performing invasive nerve biopsy in patients with small fiber neuropathy, thereby preventing complications such as non-healing of the biopsy site, which is common in these patients. Secondly, the greatest advantage of skin punch biopsies is that they can be repeated any number of times, unlike a nerve biopsy, and are useful to evaluate disease progression and therapeutic response. More recently, its use has been expanded to the diagnosis of large fiber neuropathies, inherited demyelinating neuropathies, etc., obviating the need for a nerve biopsy. The European Federation of Neurological Societies has published guidelines for evaluation to ensure uniformity with regard to the site of biopsy, processing, and quantification. The evaluation of the skin biopsy involves morphometric assessment of the intraepidermal nerve fiber density using PGP 9.5 immunostained sections by bright-field microscopy. This review focuses on the practical aspects of skin punch biopsy and its utility for the practicing pathologist.

Retrospective Evaluation of Various Serological Assays and Multiple Parameters for Optimal Diagnosis of Lyme Neuroborreliosis in a Routine Clinical Setting.

ABSTRACTLaboratory diagnosis of Lyme neuroborreliosis (LNB) is challenging, and validated diagnostic algorithms are lacking. Therefore, this retrospective cross-sectional study aimed to compare the diagnostic performance of seven commercial antibody assays for LNB diagnosis. Random forest (RF) modeling was conducted to investigate whether the diagnostic performance using the antibody assays could be improved by including several routine cerebrospinal fluid (CSF) parameters (i.e., leukocyte count, total protein, blood-CSF barrier functionality, and intrathecal total antibody synthesis), two-tier serology on serum, the CSF level of the B-cell chemokine (C-X-C motif) ligand 13 (CXCL13), and a Borrelia species PCR on CSF. In total, 156 patients were included who were classified as definite LNB (n = 10), possible LNB (n = 7), or non-LNB patient (n = 139) according to the criteria of the European Federation of Neurological Societies using a consensus strategy for intrathecal Borrelia-specific antibody synthesis. The seven antibody assays showed sensitivities ranging from 47.1% to 100% and specificities ranging from 95.7% to 100%. RF modeling demonstrated that the sensitivities of most antibody assays could be improved by including other parameters to the diagnostic repertoire for diagnosing LNB (range: 94.1% to 100%), although with slightly lower specificities (range: 92.8% to 96.4%). The most important parameters for LNB diagnosis are the detection of intrathecally produced Borrelia-specific antibodies, two-tier serology on serum, CSF-CXCL13, Reibergram classification, and pleocytosis. In conclusion, this study shows that LNB diagnosis is best supported using multiparameter analysis. Furthermore, a collaborative prospective study is proposed to investigate if a standardized diagnostic algorithm can be developed for improved LNB diagnosis.IMPORTANCE The diagnosis of LNB is established by clinical symptoms, pleocytosis, and proof of intrathecal synthesis of Borrelia-specific antibodies. Laboratory diagnosis of LNB is challenging, and validated diagnostic algorithms are lacking. Therefore, this retrospective cross-sectional study aimed to compare the diagnostic performance of seven commercial antibody assays for LNB diagnosis. Multiparameter analysis was conducted to investigate whether the diagnostic performance using the antibody assays could be improved by including several routine (CSF) parameters. The results of this study show that LNB diagnosis is best supported using the detection of intrathecally produced Borrelia-specific antibodies, two-tier serology on serum, CSF-CXCL13, Reibergram classification, and pleocytosis. Furthermore, we propose a collaborative prospective study to investigate the potential role of constructing a diagnostic algorithm using multiparameter analysis for improved LNB diagnosis.

Pregabalina vs gabapentyna w leczeniu bólu neuropatycznego u pacjentów onkologicznych

Neuropathic pain affects an average of 7–10% of the population and is more often in elderly people and in women. It is a chronic pathological pain, resistant to analgesics from the first step of the analgesic ladder (paracetamol, metamizole, non-steroidal anti-inflammatory drugs). Neuropathic pain may be caused by: infectious diseases, trauma, ischemia, metabolic diseases, neurological diseases, toxic, consequences of damage to the nervous system resulting from the treatment of neoplastic disease, pressure, inflammation, as well as deficiencies. The American Academy of Neurology, Special Interest Group on Neuropathic Pain, and the European Federation of Neurological Societies recommend gabapentin/pregabalin and antidepressants as first choice in the treatment of neuropathic pain. In neuropathic pain accompanying neoplastic disease, due to its mixed nature, i.e., the coexistence of receptor pain, opioid analgesics are always used in the treatment: tramadol for moderate pain and strong opioids for high pain. The following article describes the differences in the pharmacokinetics and pharmacodynamics of gabapentin and pregabalin.

Clinical and long-term characteristics of the subtypes of chronic inflammatory demyelinating polyneuropathy

To clarify the clinical and long-term characteristic of each subtype of chronic inflammatory demyelinating polyneuropathy (CIDP). We evaluated data from 30 consecutive CIDP patients who met the criteria proposed by the European Federation of Neurological Societies and the Peripheral Nerve Society. Patients were classified as having typical CIDP (t-‍CIDP) (10/30, 33%), multifocal acquired demyelinating sensory and motor (MADSAM) (12/30, 40%), DADS (4/30, 13%), sensory CIDP (3/30, 10%) or motor CIDP (1/30, 3%). Nerve conduction studies showed more prolonged distal motor latencies/F-wave latencies and slower motor nerve conduction in the typical CIDP group than in the MADSAM group. Intravenous immunoglobulin (IVIg) was effective in 80% (8/10) of t-‍CIDP, 100% (12/12) of MADSAM, 100% (4/4) of DADS, and 100% (3/3) of sensory CIDP cases. Maintenance therapy with immunoglobulin was administered in patients with t-‍CIDP (5/10, 50%), MADSAM (9/12, 75%), DADS (1/4, 25%), and sensory CIDP (2/3, 67%). There were no patients with CIDP, in whom CIDP subtype was transformed from the initial diagnosis during five years of follow-up. Percentage of MADSAM was the most common phenotype in our cohort of CIDP patients, and IVIg/immunoglobulin maintenance was effective for MADSAM as well as t-‍CIDP in contrast to findings from the previous reports.

Clinical and neurophysiological patterns of early presenting symptoms in acute onset chronic inflammatory demyelinating polyradiculoneuropathy

The phenotypes of chronic inflammatory demyelinating polyneuropathy (CIDP) include an acute-onset phenotype (A-CIDP) with an evolution time of less than eight weeks from the onset of symptoms. This entity can be confused with Guillain-Barre syndrome of the acute inflammatory demyelinating variety (AIDP), delaying the start of treatment. To analyze the clinical and electrophysiological differences between A-CIDP, classic CIDP and AIDP, in order to identify factors that may help in the early differential diagnosis. A cross-sectional study was carried out with patients seen at the neuromuscular disease clinic of the National Institute of Neurology and Neurosurgery with a diagnosis of CIDP according to the criteria of the European Federation of Neurological Societies and Peripheral Nerve Society. Patients with CIDP <8 weeks were categorized as A-CIDP and were compared with patients diagnosed with classic CIDP and AIDP. Clinical, paraclinical and electrophysiological variables were obtained and analyzed. Significant differences in history of infection, cranial nerve involvement and dysautonomia were observed between A-CIDP and AIDP. Electrophysiological recordings reported significant differences in motor nerve conduction velocity and sural nerve recordings, being lower in the A-CIDP group. A history of infection, cranial nerve involvement and dysautonomia are important parameters to take into account for the differential diagnosis of these entities. Electrophysiological analysis is similar between A-CIDP and CIDP. The differential diagnosis between these types of demyelinating polyneuropathy must be based on clinical assessment.

Pharmacological and non-pharmacological management of spinocerebellar ataxia: A systematic review.

Spinocerebellar ataxias (SCA) comprise a rare, genetic subgroup within the degenerative ataxias and are dominantly inherited, with up to 48 recognized genetic subtypes. While an updated review on the management of degenerative ataxia is published recently, an evidence-based review focussed on the management of SCA is lacking. Here, we reviewed the pharmacological and non-pharmacological management of SCA by conducting a systematic review on Medline Ovid and Scopus. Of 29,284 studies identified, 47 studies (pharmacological: n = 25; non-pharmacological: n = 22) that predominantly involved SCA patients were included. Twenty studies had a high risk of bias based on the Cochrane's Collaboration risk of bias tool. As per the European Federation of Neurological Societies 2004 guideline for therapeutic intervention, the remaining 27 studies were of Class I (n = 4) and Class II (n = 23) evidence. Only two therapies had Level A recommendations for the management of ataxia symptoms: riluzole and immediate in-patient neurorehabilitation. Ten therapies had Level B recommendations for managing ataxia symptoms and require further investigations with better study design. These include high dose valproate acid, branched-chain amino acid, intravenous trehalose; restorative rehabilitation using cycling regimen and videogame; and cerebellar stimulations using transcranial direct current stimulation and transcranial magnetic stimulation. Lithium and coaching on psychological adjustment received Level B recommendation for depressive symptoms and quality of life, respectively. Heterogeneous study designs, different genotypes, and non-standardized clinical measures alongside short duration and small sample sizes may hamper meaningful clinical translation. Therefore, rating of recommendations only serve as points of reference.

Focused Neuromuscular Ultrasound Approach for the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy.

Previous ultrasonographic studies of individuals with chronic inflammatory demyelinating polyneuropathy (CIDP) have shown nerve enlargement at several sites. This prospective study compares only the bilateral median and ulnar nerves of individuals with CIDP with reference values to determine the clinical usefulness of this focused approach as a diagnostic tool. The cross-sectional area, echogenicity, and vascularity of the bilateral median and ulnar nerves of 25 subjects with CIDP were measured using ultrasound. Nineteen had typical CIDP based on the European Federation of Neurological Societies and the Peripheral Nerve Society guidelines, whereas six had atypical CIDP and were diagnosed based on clinical impression. Focal nerve enlargement was found in at least one segment in all subjects. Subjects with typical CIDP had larger cross-sectional areas compared with subjects with atypical CIDP. A focused ultrasound study, involving only the median and ulnar nerves, is sensitive for the detection of nerve enlargement in CIDP. Measuring the cross-sectional area of the median and ulnar nerves is clinically feasible and may help establish the diagnosis of CIDP.

Misdiagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): A two years follow-up study.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder involving peripheral nerves, characterized by sensory alteration and weakness with gradual progression over years leading to disability. Diagnosis is obtained through clinical history, neurological examination, serum and liquoral biomarkers and neurophysiology. Neurophysiological criteria proposed by European Federation of Neurological Societies and Peripheral Nerve Society (EFNS/PNS) are used for the diagnosis allowing high sensitivity and specificity.