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A 76-year-old male presented with a recurrent depressive episode, an unsteady gait and cognitive impairment. Extensive blood tests, including hemogram, biochemical tests, folic acid, vitamin B12, and thyroid hormone, showed normal results. With the exception of the unsteady gait, neurological examination was negative. Brian magnetic resonance imaging (MRI) showed the typical feature of central pontine myelinolysis (CPM); however, there was no history of alcoholism, liver transplantation, malnutrition or rapid correction of hyponatremia. The patient had taken venlafaxine to treat major depressive disorder for more than 20 years. After discontinuation of venlafaxine, the unsteady gait gradually resolved, and subsequent MRI revealed reduction of the lesions over 6 months. We discuss herein the possible correlation between chronic use of venlafaxine and CPM.

Background/aim Thalamus infarctions presented with various clinical findings are considered to be related to classical and variative infarction areas. In our study, we aimed to compare the sequela clinical findings of patients with isolated thalamus infarction according to anatomical areas. Materials and methods Seventy patients diagnosed with isolated thalamus infarction in our clinic between 2010 and 2020 were included in the study. The infarction areas of the patients were divided into groups by the radiologist, including the variative areas to the classical areas using magnetic resonance imaging. Neurological examinations were performed and recorded. Sequela clinical findings of the groups were compared. Results The mean age of all patients was 64.49 ± 13.75 (range between: 33–81) years, and the female ratio was 52.9% (n: 33). Inferolateral area infarction was detected most commonly. The most common complaints were sensory complaints (48.6%), speech disorders (20%), limb weakness (15.7%). There were no significant association between the neurological examination findings of classical and variative area infarctions of patients whose most common admission complaint is sensory deficits (p < 0.05), and significant signs of cognitive impairment were detected in the anterior area compared to other areas (p < 0.001). It can be considered that cognitive impairment we detected in the anterior area developed due to its associations. Conclusion In our study where sequela findings were evaluated, the absence of a significant difference in neurological examination findings can be explained by the decline of many acute clinical findings over time.

This paper presents the case of a 13-year-old female patient, operated in 2015 for left temporoparietal hydatid cyst and reoperated in 2016 for recurrence of the cerebral hydatid cyst. At admission, his GCS scores were E3V4M5. Neurological examination revealed a hemilatéral motor deficit estimated at 3/5 on right side without sensitive trouble. The CT scan showed a large left temporal cystic formation with an infected appearance associated with 4 cystic formations with calcified walls exerting a mass effect on the adjacent parechyma and the homolateral lateral ventricle responsible for a triventricular hydrocephalus. No primary focus was found in the lungs, liver, or other organs. In this article, we will discuss the management of complications related to hydatid cysts. Cerebral hydatid cyst is a rare condition, affecting mainly children. The diagnosis of cerebral hydatid cyst must be evoked in endemic countries in front of a symptomatology of intra-cranial hypertension.

Case reportG.F.M, 71 years old, male, farmer, presented a history of holocranial headache of a pulsatile type, more intense on waking, and associated with photophobia. The complaint lasted six months. He also reported episodes of tinnitus and unilateral hearing loss on the left. After conducting screening tests in the emergency department, communicating hydrocephalus and cystic lesions were found in the subarachnoid and intraparenchymal space. In the neurological examination, the positive data were bilateral papilledema and hearing loss in the left boné conduction. Given these findings, the racemous variant of neurocysticercosis and secondary hydrocephalus was suspected, causing intracranial hypertension. Thus, it was decided to start specific treatment with albendazole 15 mg/kg/day (two daily doses), associated with praziquantel 50 mg/kg/day (three daily doses), for 14 days. To reduce the inflammatory process of the lesions, it was proposed to keep the patient on dexamethasone 8 mg/day for 90 days. In addition to this scheme, topiramate 100 mg/day associated with acetazolamide 1 g/day was started, along with serial lumbar puncture to relieve intracranial pressure. ConclusionThe combination of modern diagnostic tests, use of antiparasitic drugs, optimization of anti-inflammatory treatments and minimally invasive neurosurgical procedures have improved results in patients with NCC. Even so, this is the helminth infection that most affects the CNS and represents a major public health problem in most parts of the world, since it remains a neglected pathology, like so many other parasites, given that it is susceptible to containment through simple preventive actions as well as eradication.

Epstein-Barr virus (EBV) has been associated with a plethora of neurological manifestations including polyneuropathy and polyradiculopathy. A 27-year-old man with a recent upper respiratory system infection presented with difficulty in walking. His neurological examination revealed reduced muscle strength in both proximal and distal lower limb muscles without sensory and autonomic signs. Needle electromyography showed abnormal spontaneous activity and reduced recruitment of motor units in muscles innervated by multiple lumbo-sacral roots. Cerebrospinal examination showed increased protein levels with normal cell counts. While spinal MRI was normal, whole-body CT and PET examination showed disseminated lymph node enlargement. Anti-EBV viral capsid antigen and anti-nuclear antigen IgG but not IgM was positive, whereas EBV PCR was negative in blood. Analysis of inguinal lymph node biopsy showed reactive lymphoid hyperplasia and EBV DNA. Leucine-rich glioma-inactivated protein 1 (LGI1) antibody was found in serum but not in CSF. All clinical, imaging, and electrophysiological findings improved following steroid and intravenous immunoglobulin treatment. These findings suggested the acute involvement of lumbo-sacral spinal roots and/or motor neurons. Purely motor polyradiculopathy has been reported in both EBV-positive and LGI1 antibody-positive patients, and EBV infection is known to precede different autoimmune manifestations. Whether EBV infection may trigger LGI1 autoimmunity and cause involvement of spinal motor roots and/or motor neurons needs to be further studied.

Among the most severe consequences of spinal hernia surgery (SHG) are spinal neurogenic bladder (SNGB) and impaired bowel movement. SNGB, in addition to its main manifestations in the form of violations of the accumulative function of the bladder and the function of emptying, is dangerous for the development of complications – vesicoureteral reflux, ascending infection of the urinary system and chronic kidney disease. Clinical manifestations of SNGB do not always accurately indicate a variant of bladder dysfunction. The authors presented variants of the results of urodynamic studies in children operated on for SHG SHG. The state of the detrusor – hyperactivity or atony and the sphincter – insufficiency or hyperactivity and the selection of a pathogenetically justified treatment option are described.

Craniocervical abnormalities in osteogenesis imperfecta (OI) such as basilar invagination or cervical kyphosis can cause severe neurological morbidity. These abnormalities may be more frequent in OI type V compared with other OI subtypes of similar disease severity, underlining the importance of screening in this group. Craniocervical abnormalities in osteogenesis imperfecta (OI) can cause severe neurological morbidity. Although radiological cranial base abnormalities in OI have been well described in the literature, there are limited data on these abnormalities in OI type V and their association with clinical sequelae. A retrospective case series on patients with craniocervical abnormalities in OI type V at our institution. Craniocervical abnormalities were present in 7 of 37 patients with OI type V (19%). For 5 patients (age at last follow-up: 5 to 26 years; 2 females), sufficient information was available for inclusion in the case series. All had genetically confirmed OI type V. Age range at diagnosis of the craniocervical abnormality was 1 day to 18 years. Basilar invagination was present in 3 patients; 2 had cervical kyphosis. Dysplasia of upper cervical vertebrae or base of skull was seen in 3 patients. The severity of the craniocervical abnormality did not clearly correlate with the severity of the OI phenotype. Three patients required surgical intervention (ages 7, 11, and 26 years) due to compression of the spinal cord or brainstem. Craniocervical abnormalities were detected incidentally or on screening in 3 patients, and only 2 had significant positive findings on neurological examination. A variety of craniocervical abnormalities are seen in OI type V including dysplasia of the cervical vertebrae. These cases highlight the importance of screening patients with OI type V with lateral skull and cervical spine x-rays throughout childhood and after skeletal maturity.

Neonatal sepsis is an important risk factor for lesions in the brain of preterm newborns (PTNB) and the most effective strategies to minimize its deleterious effects are early detection and intervention.To investigate the presence of neurological abnormalities in PTNBs after neonatal sepsis.This was a prospective cross-sectional study with 100 PTNBs selected at random, 50 of the study group (sepsis) and 50 of the control group (non-sepsis). The neurological evaluation protocol adopted was the Hammersmith Neonatal Neurological Examination (HNNE).The PTNBs of the sepsis group had total HNNE scores lower than expected for normality in 86% of the cases, and the non-sepsis group in 26% (The neurological dysfunctions after neonatal sepsis could be identified by clinical neonatal neurological evaluation.

Background: Reduced activity of lysosomal glucocerebrosidase is found in brain tissue from Parkinson’s disease patients. Glucocerebrosidase is also highly expressed in peripheral blood monocytes where its activity is decreased in Parkinson’s disease patients, even in the absence of GBA mutation. Objective: To measure glucocerebrosidase activity in cryopreserved peripheral blood monocytes from 30 Parkinson’s disease patients and 30 matched controls and identify any clinical correlation with disease severity. Methods: Flow cytometry was used to measure lysosomal glucocerebrosidase activity in total, classical, intermediate, and non-classical monocytes. All participants underwent neurological examination and motor severity was assessed by the Movement Disorders Society Unified Parkinson’s Disease Rating Scale. Results: Glucocerebrosidase activity was significantly reduced in the total and classical monocyte populations from the Parkinson’s disease patients compared to controls. GCase activity in classical monocytes was inversely correlated to motor symptom severity. Conclusion: Significant differences in monocyte glucocerebrosidase activity can be detected in Parkinson’s disease patients using cryopreserved mononuclear cells and monocyte GCase activity correlated with motor features of disease. Being able to use cryopreserved cells will facilitate the larger multi-site trials needed to validate monocyte GCase activity as a Parkinson’s disease biomarker.

Studies of the dental status of children with cerebral palsy (CP) indicate a high prevalence and intensity of damage to the hard tissues of the teeth. The risk of developing dental diseases is known to increase significantly as the severity of neurological symptoms increase. The purpose of the study was to assess the incidence of dental caries using the International Caries Detection and Assessment System (ICDAS II) criteria in children with organic diseases of the nervous system depending on the severity of motor impairment. A number of 122 children (mean age 8.8±3.7 years) with spastic forms of cerebral palsy were examined. They were divided into groups according to the Gross Motor Function Classification System – Expanded & Revised (GMFCS-ER). All patients underwent a neurological examination, and the state of dental caries was determined using the ICDAS II criteria. In children with cerebral palsy, lesions of the occlusal surfaces of the teeth predominate, lesions of the proximal surfaces appeared to be three times less, but more than three times higher than in healthy children. Higher intensity of the carious process and the frequency of deep cavities are observed in children with cerebral palsy with severe motor impairment, according to GMFCS-ER. Establishing the features of caries development in children with cerebral palsy depending on the severity of neurological symptoms according to the ICDAS II system is an essential factor in determining the direction of preventive measures that should be taken for this group of children.

Background Spastic Paraplegia type 7 (SPG7) is one of the most common autosomal recessive Hereditary Spastic Paraplegias (HSP); Spastic Paraplegias (SPGs) can present as hereditary ataxias. However, ataxia is frequently the symptom of presentation of many other hereditary/sporadic disorders, such as Multiple system atrophy type C (MSA-C), an α-synuclein sporadic neurodegenerative disorder, in which cerebellar ataxia is one of the main clinical features. Dopamine Transporter imaging (DAT-SCAN), associated with clinical features, can be a helpful tool in order to distinguish MSA-C from other causes of ataxia. Case-presentation We present the case of a 70-year-old man with gait difficulties over a period of 3 years and frequent backward/lateral falls. He also reported urinary urge incontinence, but no symptoms that are compatible with orthostatic hypotension. On neurological examination he showed ataxic gait, spasticity in the left lower limb and trunk and limb ataxia, especially on the left side. Mild hypokinesia was found in all 4 limbs, especially in the left foot. MRI revealed atrophy of the cerebellar hemispheres and vermis. DAT-SCAN imaging revealed bilateral nigro-striatal degeneration, which was compatible with a diagnosis of possible MSA-C. Considering the atypical disease course (the patient walked without any support after 3 years), we carried out a genetic investigation for Ataxia, and a mutation in SPG7 was found. Conclusions DAT-SCAN imaging, evaluated together with the clinical findings, can be useful for differentiating MSA from other possible causes of adult-onset Ataxia. Indeed, patients with MSA-C generally show a decreased uptake of dopamine transporters in DAT-SCAN imaging. Ours is the first case reported in the literature of a patient with SPG7 mutation with nigrostriatal degeneration and a clinical presentation of a possible MSA-C. Performing genetic investigations in patients with an atypical disease course is important to avoid MSA-mimicries. Identifying the correct diagnosis is important not only for prognostic reasons, but also for possible future genetic therapies.

Therapeutic blockade of tumour necrosis factor alpha (anti-TNF-α) is the mainstay treatment of several rheumatologic diseases. They are unfrequently associated with opportunistic infections and latent tuberculosis (TB) screening is paramount before immunosuppression. A 62-year-old man with psoriatic arthritis was under treatment with adalimumab for over 5 years. The screening for latent tuberculosis infection (LTBI) prior to the start of immunosuppression was negative, and a subsequent interferon gamma release assay 4 years later was also negative. He presented in the emergency department complaining of asthenia, anorexia, fever, night sweats and weight loss for over 5 months. He also complained of memory loss, despite his normal cognitive and neurological examination. After an exhaustive workup, he was diagnosed with a disseminated tuberculosis (cerebral, pulmonary and peritoneal) and treated accordingly. TB diagnosis remains challenging in certain situations. Latent TB screening tests may lack sensitivity and immunosuppressive therapy increases the risk of disseminated infection. We discuss the workup and management of a central nervous system disseminated TB related to adalimumab therapy.

Background : Pyomyositis is a subacute bacterial infection of the skeletal muscles. Its most common features are fever, muscle swelling, and focal pain. There have been insufficient data regarding pyomyositis in healthy infants. Case report : A one-month-old boy presented with an impairment of his left arm movement. He was well-nourished and not under any apparent distress. His vital signs were within the normal limits but neurological examination revealed left forearm paralysis. Physical examination showed no abnormal findings in the region from the left shoulder joint to the fingertips. Considering these factors, an intracranial pathology was initially suspected. However, he developed a fever, regular tachycardia, and swelling in the left forearm. Magnetic resonance imaging revealed inflammation in the left forearm muscles. He was diagnosed with bacterial myositis and started on intravenous antibiotics. On the 17th day, he was discharged with oral antibiotic treatment, which was completed over 25 days without any sequelae nor relapse. Conclusion : Here we report the case of Japanese primary pyomyositis following one-day afebrile upper limb monoplegia in an infant. Even when infants exhibit afebrile symptoms, a bacterial infection should be suspected. J. Med. Invest. 68 : 372-375, August, 2021.

Purpose: To facilitate precise diagnosis and provide tailored treatment of postural anomalies in the pediatric population, appropriate assessments are essential. In light of the multicomponent structure of postural control, understanding underlying constructs of an assessment is valuable in selecting and interpreting assessments. This study investigates the construct validity of the Gaits and Stations variables in the Revised Physical and Neurological Examination of Subtle Signs, a measure used to evaluate standing postural control in youth with mild neurological deficits. Methods: Data were included from 350 healthy participants ages 10–19 years old. An exploratory factor analysis with varimax rotation was performed. Individual loadings of ≥0.4 were used for factor designation. Results: Three latent factors were identified and labeled, based on evidence, as dynamic stability, movement strategies/coordination, and underlying motor systems—musculoskeletal strength. Conclusions: These brief, easily administered Gaits and Stations measures of the Physical and Neurological Examination of Subtle Signs facilitate evaluation of three constructs of standing postural control relevant to youth with mild neuromotor impairments. This information will potentially assist in clinical practice to identify youth with postural control deficits and establish developmentally appropriate interventions and in research to refine understanding of pathology and the impact on components of postural control.

We report a child with multiple sclerosis who presented with sixth nerve palsy. She is a twelve-year-old Bahraini female presented to the ophthalmology department complaining of double vision. She also had imbalance and paraesthesia. Extraocular muscle examination showed restriction of abduction in the right eye. There was no change in vision. MRI showed right eye optic neuritis (ON) and demyelination which was indicative of multiple sclerosis (MS). Ocular coherence tomography (OCT) showed thinning of nerve fibres of both eyes which was consistent with subclinical optic neuritis. Neurological examination showed brisk knee jerk on the left side and incoordination of movement on the same side. Disability Status Scale (EDSS) showed a score of 3.0. She was given Solu-medrol 500 mg intravenously (IV) in addition to omeprazole 40 mg IV and Vitamin D3 (cholecalciferol) 50,000 IU cap weekly for 8 weeks and Neurorubine forte tablets (vitamin B1, 6, 12) once a day for 2 months. She became asymptomatic in her follow-up visits. Children with MS can present as sixth cranial nerve palsy. Clinicians should have a high index of suspicion for early diagnosis and treatment. In addition to MRI, OCT is a useful diagnostic tool for optic neuritis and MS especially in children.

Introduction: With the aim of improving the care of the premature newborn during their hospital stay, and their well-being in the transition from enteral to independent feeding, it is proposed to incorporate an assessment system within a Spanish Neonatal Unit. The translation of the Early Feeding Skills Assessment (EFSA) tool is presented, along with a study of its measurement properties. Patients and method: A total of 104 assessments were made on premature babies of less than 34 + 6 weeks of gestational age, admitted to the neonatal unit with total or partial feeding, including a normal neurological examination for their age and with physiological stability. Results: The EFSA 2010 tool achieved an acceptable value (0.76) as regards its internal consistency. The EFSA 2018 tool maintained an acceptable internal consistency value (0.751). As regards the reliability between two observers, the results showed a satisfactory and excellent reliability in 57.69% of the items in the EFSA 2010 tool, a property that improved in the EFSA 2018 tool (73.68%). Conclusions: The Spanish version of the EFSA tool is consistent and reliable for use as a tool for the assessment of oral abilities for feeding premature babies admitted into a Spanish Neonatal Unit. Resumen: Introducción: Con el propósito de mejorar la atención al recién nacido prematuro durante su estancia hospitalaria y su bienestar en la transición de la alimentación enteral a independiente, se propone incorporar un sistema de evaluación dentro de una unidad neonatal española. Presentamos la traducción al idioma español de la herramienta Early Feeding Skills Assessment y el estudio de sus propiedades métricas. Pacientes y métodos: 104 evaluaciones sobre bebés prematuros menores de 34 + 6 semanas de edad gestacional admitidos en la unidad neonatal con alimentación oral parcial o total, con examen neurológico normal para su edad y con estabilidad fisiológica. Resultados: La herramienta EFSA 2010 alcanza un valor aceptable (0,76) en cuanto a su consistencia interna. La herramienta EFSA 2018 mantiene un valor de consistencia interna aceptable (0,751). En cuanto a la fiabilidad entre dos evaluadores, los resultados muestran fiabilidad satisfactoria y excelente en un 57,69% de los ítems en la herramienta EFSA 2010, propiedad que mejora en la herramienta EFSA 2018 (73,68%). Conclusiones: La herramienta EFSA-VE es consistente y fiable para su uso como herramienta de valoración de las habilidades orales para la alimentación del bebé prematuro ingresado en una unidad neonatal española.

BackgroundSELENON (SEPN1)-related myopathy (SELENON-RM) is a rare congenital myopathy characterized by slowly progressive proximal muscle weakness, early onset spine rigidity and respiratory insufficiency. A muscular dystrophy caused by mutations in theLAMA2gene (LAMA2-related muscular dystrophy, LAMA2-MD) has a similar clinical phenotype, with either a severe, early-onset due to complete Laminin subunit α2 deficiency (merosin-deficient congenital muscular dystrophy type 1A (MDC1A)), or a mild, childhood- or adult-onset due to partial Laminin subunit α2 deficiency. For both muscle diseases, no curative treatment options exist, yet promising preclinical studies are ongoing. Currently, there is a paucity on natural history data and appropriate clinical and functional outcome measures are needed to reach trial readiness.MethodsLAST STRONG is a natural history study in Dutch-speaking patients of all ages diagnosed with SELENON-RM or LAMA2-MD, starting August 2020. Patients have four visits at our hospital over a period of 1.5 year. At all visits, they undergo standardized neurological examination, hand-held dynamometry (age ≥ 5 years), functional measurements, questionnaires (patient report and/or parent proxy; age ≥ 2 years), muscle ultrasound including diaphragm, pulmonary function tests (spirometry, maximal inspiratory and expiratory pressure, sniff nasal inspiratory pressure; age ≥ 5 years), and accelerometry for 8 days (age ≥ 2 years); at visit one and three, they undergo cardiac evaluation (electrocardiogram, echocardiography; age ≥ 2 years), spine X-ray (age ≥ 2 years), dual-energy X-ray absorptiometry (DEXA-)scan (age ≥ 2 years) and full body magnetic resonance imaging (MRI) (age ≥ 10 years). All examinations are adapted to the patient’s age and functional abilities. Correlation between key parameters within and between subsequent visits will be assessed.DiscussionOur study will describe the natural history of patients diagnosed with SELENON-RM or LAMA2-MD, enabling us to select relevant clinical and functional outcome measures for reaching clinical trial-readiness. Moreover, our detailed description (deep phenotyping) of the clinical features will optimize clinical management and will establish a well-characterized baseline cohort for prospective follow-up.ConclusionOur natural history study is an essential step for reaching trial readiness in SELENON-RM and LAMA2-MD.Trial registrationThis study has been approved by medical ethical reviewing committee Region Arnhem-Nijmegen (NL64269.091.17, 2017–3911) and is registered atClinicalTrial.gov(NCT04478981).

Background Colony-stimulating factor-1 receptor (CSF1R)-related leukoencephalopathy is a rapidly progressive neurodegenerative disease for which there is currently no cure. Hematopoietic stem cell transplantation (HSCT) has been proposed as a disease-modifying treatment. Objective The objective of this study was to determine the effect of HSCT on disease progression. Methods We collected all available clinical data from a cohort of 7 patients with CSF1R-related leukoencephalopathy who underwent HSCT at our institutions. Clinical data included detailed neurological examination by a board-certified neurologist, serial cognitive screens, formal neuropsychological evaluations, and serial brain magnetic resonance imaging (MRI). Results Our patients had an average disease duration of 27.6 months at the time of transplant, and we have 87 months of total posttransplant follow-up time (median, 11; range, 2-27). One patient died in the periprocedural period. The remaining patients showed a variable response to treatment, with 6 of 7 patients trending toward stabilization on motor examination, cognitive scores, and/or MRI abnormalities, especially with white matter lesion burden. Conclusions This is the largest series of patients with CSF1R-related leukoencephalopathy receiving HSCT. We conclude that HSCT can stabilize the disease in some patients. Variability in patient responsiveness suggests that measures of disease heterogeneity and severity need to be considered when evaluating a patient's candidacy for transplant. HSCT appears to be the first disease-modifying therapy for CSF1R-related leukoencephalopathy. This milestone may serve as a foothold toward better understanding the disease's pathomechanism, thus providing new opportunities for better disease-specific therapies. © 2021 International Parkinson and Movement Disorder Society.

Objective The aim of this study was to describe the surgical technique and outcomes of dogs with congenital thoracic vertebral body malformations (CTVBM) treated with unilateral vertebral distraction and stabilization with monocortical screws and polymethylmethacrylate (PMMA). Study Design Medical records of 10 client-owned dogs with CTVBM were retrospectively searched for signalment, history, neurological examination, neurological grade based on the modified Frankel scale (MFS), diagnostic method, CTVBM location, Cobb's angle, and vertebral canal angle (VCA), surgical procedure, complications and long-term follow-up. Results One dog suffered cardiac arrest 24 hours post-surgery and died. Median follow-up in nine dogs was 26.5 months (12–50 months). Cobb's angle improved from 33–83 to 10–46 degrees and the VCA ratio from 97–138 to 122–164 degrees. The MFS improved from 3 (n = 3)–4 (n = 7) to 4 (n = 2)–6 (n = 7). Seven dogs recovered full function. Nine dogs improved after surgery compared with preoperative status, recovering ambulation when lost or achieving normal neurological examination in seven cases. No complications were reported during the follow-up period (12–50 months). Conclusion Unilateral transthoracic vertebral distraction and stabilization with monocortical screws and PMMA for treatment of compressive myelopathy due to CTVBM achieved long-term neurological improvement in 9 of 10 dogs.

Background:There is international variability in whether neurological determination of death (NDD) is conceptually defined based on permanent loss of brainstem function or “whole brain death.” Canadian guidelines are not definitive. Patients with infratentorial stroke may meet clinical criteria for NDD despite persistent cerebral blood flow (CBF) and relative absence of supratentorial injury.Methods:We performed a multicenter cohort study involving patients that died from ischemic or hemorrhagic stroke in Alberta intensive care units from 2013 to 2019, focusing on those with infratentorial involvement. Medical records were reviewed to determine the incidence and proportion of patients that met clinical criteria for NDD; whether ancillary testing was performed; and if so, whether this demonstrated the absence of CBF.Results:There were 95 (27%) deaths from infratentorial and 263 (73%) from supratentorial stroke. Sixteen patients (17%) with infratentorial stroke had neurological examination consistent with NDD (0.55 cases per million per year). Among patients that underwent confirmatory evaluation for NDD with an apnea test, ancillary test (radionuclide scan), or both, ancillary testing was more common with infratentorial compared with supratentorial stroke (10/12 (85%) vs. 25/47 (53%), p = 0.04). Persistent CBF was detected in 6/10 (60%) patients with infratentorial compared with 0/25 with supratentorial stroke (p = 0.0001).Conclusions:Infratentorial stroke leading to clinical criteria for NDD occurs with an annual incidence of about 0.55 per million. There is variability in clinicians’ use of ancillary testing. Persistent CBF was detected in more than half of patients that underwent radionuclide scans. Canadian consensus is needed to guide clinical practice.

Patients in neurology clinics are sometimes not aware of the reason for the consultation, and we have called this circumstance the “Don’t know” sign (DKS). Our objective was to define this new sign and its modalities and to evaluate its prevalence and its diagnostic accuracy for cognitive impairment (CI) in comparison to other observation-based signs. A cross-sectional prospective study included all new outpatients evaluated by the authors at neurology consultation. We recorded observation-based signs. The Global Deterioration Scale (GDS) was used to assess the cognitive status of patients, based on clinical history, caregiver interview, and cognitive test results. We analyzed the prevalence and the diagnostic accuracy for CI of DKS, “head turning sign,” “attending with,” verbal repetition, and combinations, calculating sensitivity (Se), specificity (Sp), positive predictive value (PPV), and negative predictive value (NPV). We enrolled 673 consecutive patients (62% female) with a mean ± SD age of 59.3 ± 20.2 years. DKS was positive in 94 patients (14%) and was strongly associated with GDS score. DKS had a Se of 0.41, Sp of 0.98, PPV of 0.89, and NPV of 0.79 for CI diagnosis. The presence of at least two positive observation signs yielded a Se of 0.50, Sp of 0.97, PPV of 0.86, and NPV of 0.81. DKS is frequently observed in neurology outpatients. It has low sensitivity but high specificity and PPV for CI diagnosis. It does not require additional consultation time, and its use can be recommended in combination with other observation-based signs.

Objective Diagnostic errors are major contributors to preventable patient harm. We validated the use of an electronic health record (EHR)-based trigger (e-trigger) to measure missed opportunities in stroke diagnosis in emergency departments (EDs). Methods Using two frameworks, the Safer Dx Trigger Tools Framework and the Symptom-disease Pair Analysis of Diagnostic Error Framework, we applied a symptom–disease pair-based e-trigger to identify patients hospitalized for stroke who, in the preceding 30 days, were discharged from the ED with benign headache or dizziness diagnoses. The algorithm was applied to Veteran Affairs National Corporate Data Warehouse on patients seen between 1/1/2016 and 12/31/2017. Trained reviewers evaluated medical records for presence/absence of missed opportunities in stroke diagnosis and stroke-related red-flags, risk factors, neurological examination, and clinical interventions. Reviewers also estimated quality of clinical documentation at the index ED visit. Results We applied the e-trigger to 7,752,326 unique patients and identified 46,931 stroke-related admissions, of which 398 records were flagged as trigger-positive and reviewed. Of these, 124 had missed opportunities (positive predictive value for “missed” = 31.2%), 93 (23.4%) had no missed opportunity (non-missed), 162 (40.7%) were miscoded, and 19 (4.7%) were inconclusive. Reviewer agreement was high (87.3%, Cohen’s kappa = 0.81). Compared to the non-missed group, the missed group had more stroke risk factors (mean 3.2 vs 2.6), red flags (mean 0.5 vs 0.2), and a higher rate of inadequate documentation (66.9% vs 28.0%). Conclusion In a large national EHR repository, a symptom–disease pair-based e-trigger identified missed diagnoses of stroke with a modest positive predictive value, underscoring the need for chart review validation procedures to identify diagnostic errors in large data sets.

Pendahuluan: Stroke merupakan penyakit yang sering terjadi di masyarakat dan memiliki morbiditas dan mortalitas yang tinggi. Pada stroke terjadi hemiparesis kontralateral, tetapi pada kasus tertentu, hemiparesis kontralateral dapat disertai dengan gejala saraf kranial ipsilateral Ilustrasi Kasus: Pasien perempuan, berusia 23 tahun, datang dengan keluhan kelemahan anggota gerak kanan disertai kelopak mata kiri tidak bisa membuka, penglihatan ganda, dan bicara pelo. Dari pemeriksaan fisik didapatkan tekanan darah 130/100mmHg. Dari pemeriksaan neurologis didapatkan ptosis mata kiri, dilatasi pupil mata kiri, refleks cahaya langsung dan tidak langsung negatif pada mata kiri, refleks pupil akomodasi negatif pada mata kiri, parese nervus okulomotorius dan trokhlearis kiri, kelumpuhan otot wajah bagian bawah sisi kanan, dan deviasi lidah ke kanan saat protrusi. Selain itu, kekuatan otot ekstremitas superior adalah 3/5 dan inferior adalah 2/5, serta refleks Babinski dan Chaddok positif pada bagian kanan. Dari pemeriksaan rontgen thoraks didapatkan kardiomegali tanpa bendungan paru, dan dari CT scan kepala tanpa kontras didapatkan lesi iskemik di kapsula interna. Pasien didiagnosis hemiparesis alternans dan selama perawatan, pasien diberikan aspirin 80 mg satu kali perhari dan amlodipin 10 satu kali perhari. Diskusi: Pemeriksaan MRI tidak dilakukan pada pasien, tetapi pemeriksaan ini penting diusulkan untuk mengetahui struktur batang otak yang terlibat pada kondisi hemiparesis alternans yang dialami pasien Simpulan: Hemiparesis alternans superior atau Sindroma Weber dapat menyebabkan kematian, sehingga diagnosis dan tata laksana segera dibutuhkan untuk pasien hemiparesis alternans. Faktor risiko pada hemiparesis alternans akibat stroke dapat berupa multifaktorial dan perlu diinvestigasi Kata kunci: hemiparesis alternans superior, sindroma Weber, stroke

Extra time of sitting in front of a digital device is required for e-learning by children during Corona virus (COVID-19) pandemic which can lead to many ocular problems including digital eye strain (DES). In view of increased incidence of DES in children, multiple studies had been conducted in central India to assess the eye strain in children due to excessive online classes as a research project named "Digital eye strain among kids (DESK) study." This study DESK-3 aimed to report series of cases of acute acquired comitant esotropia (AACE) in children attending online classes during COVID-19 pandemic. Children aged 6-18 years with recent onset of esotropia of1-month duration without any similar history in past presented in month of July-August 2020 at a tertiary children eye care hospital were evaluated. Data collection included age and gender of child, presence or absence of diplopia, visual acuity, duration of smartphone use, duration of online classes, angle of deviation for near and distance and cycloplegic refraction. Total eight children of AACE were included in the study. The mean age of children was 12.5 ± 4.2 years. All eight were males. The mean duration of smartphone use was 4.6 + 0.7 hours per day. All children were attending online classes4 hours per day on smartphone of average size 5.5 inches. Five children were emmetropic, one myopic, one pseudomyopic and one hyperopic. The angle of deviation for near and distance were 48.1 ± 16.4 PD and 49.3 ± 15.9 PD respectively with normal ocular motility. Seven children complained of horizontal diplopia in all gazes. Neurological examination and CT scan of brain and orbit was normal in all patients. Two months before the lockdown only one case was identified as compared to eight during the lockdown. Prolonged near work during smart phone use for e-learning might lead to the development of AACE in children.

Resumen: Introducción: Ante la creciente demanda de atención neurológica y la importancia de la exploración neurológica para el diagnóstico, se propone una intervención educativa basada en simulación (IEBS) para el desarrollo de la competencia clínica en exploración neurológica (CCEN). Métodos: Estudio cuasiexperimental con 34 estudiantes de segundo año de Medicina, seleccionados mediante muestreo no probabilístico, excluyendo aquellos que tuvieran algún curso en neurología. Todos recibieron la IEBS, la cual consistió en una sesión de 60 minutos con un experto en neurología, 2 sesiones prácticas de 30 minutos cada una con un paciente estandarizado y posterior retroalimentación y una sesión general de retroalimentación de 60 minutos. Todos los participantes fueron evaluados antes y después de la IEBS utilizando la rúbrica de habilidades clínicas básicas en neurología (HCBN) que considera 9 aspectos de la exploración neurológica. Resultados: Ningún participante contaba con la CCEN antes de la IEBS y 9 la desarrollaron posteriormente. Utilizando la prueba de McNemar, la diferencia en la CCEN entre los grupos pretest y postest fue estadísticamente significativa (0% vs. 26,47%, p = 0,004). De los 9 apartados de la rúbrica, 8 mostraron una mejora significativa (p

Background Myotonic dystrophy (dystrophia myotonica [DM]) is an autosomal-dominant inheritance, and myasthenia gravis (MG) is an autoimmune disease characterized by weakness of skeletal muscles. Cases of both DM and MG are extremely rare and distinguishing DM and MG symptoms is challenging. Case presentation We herein report a 49-year-old woman presenting with subacute dyspnea and muscle weakness. She had previously been diagnosed with DM 24 years earlier. Computed tomography (CT) revealed an anterior mediastinal 32-mm solid mass that was suspected of being thymoma. The clinical features and neurological examination findings confirmed the diagnosis of thymoma-associated MG coexisting with DM. Intensive treatment for MG, including surgery, resulted in an improvement in some of her neurological symptoms. Conclusions The symptoms of DM usually progress slowly, so the sudden exacerbation of symptoms indicates the involvement of other factors. It is important to be aware of these associations, as an early diagnosis with proper treatment will result in a better outcome.

Introduction: Traumatic intracranial hemorrhages (TIH) have traditionally been managed in the intensive care unit (ICU) setting with neurosurgery consultation and repeat head CT (HCT) for each patient. Recent publications indicate patients with small TIH and normal neurological examinations who are not on anticoagulation do not require ICU-level care, repeat HCT, or neurosurgical consultation. It has been suggested that these patients can be safely discharged home after a short period of observation in emergency department observation units (EDOU) provided their symptoms do not progress. Methods: This study is a retrospective cross-sectional evaluation of an EDOU protocol for minor traumatic brain injury (mTBI). It was conducted at a Level I trauma center. The protocol was developed by emergency medicine, neurosurgery and trauma surgery and modeled after the Brain Injury Guidelines (BIG). All patients were managed by attendings in the ED with discretionary neurosurgery and trauma surgery consultations. Patients were eligible for the mTBI protocol if they met BIG 1 or BIG 2 criteria (no intoxication, no anticoagulation, normal neurological examination, no or non-displaced skull fracture, subdural or intraparenchymal hematoma up to 7 millimeters, trace to localized subarachnoid hemorrhage), and had no other injuries or medical co-morbidities requiring admission. Protocol in the EDOU included routine neurological checks, symptom management, and repeat HCT for progression of symptoms. The EDOU group was compared with historical controls admitted with primary diagnosis of TIH over the 12 months prior to the initiation of the mTBI protocols. Primary outcome was reduction in EDOU length of stay (LOS) as compared to inpatient LOS. Secondary outcomes included rates of neurosurgical consultation, repeat HCT, conversion to inpatient admission, and need for emergent neurosurgical intervention. Results: There were 169 patients placed on the mTBI protocol between September 1, 2016 and August 31, 2019. The control group consisted of 53 inpatients. Median LOS (interquartile range [IQR]) for EDOU patients was 24.8 (IQR: 18.8 – 29.9) hours compared with a median LOS for the comparison group of 60.2 (IQR: 45.1 – 85.0) hours (P < .001). In the EDOU group 47 (27.8%) patients got a repeat HCT compared with 40 (75.5%) inpatients, and 106 (62.7%) had a neurosurgical consultation compared with 53 (100%) inpatients. Subdural hematoma was the most common type of hemorrhage. It was found in 60 (35.5%) patients, and subarachnoid hemorrhage was found in 56 cases (33.1%). Eleven patients had multicompartment hemorrhage of various classifications. Twelve (7.1%) patients required hospital admission from the EDOU. None of the EDOU patients required emergent neurosurgical intervention. Conclusion: Patients with minor TIH can be managed in an EDOU using an mTBI protocol and discretionary neurosurgical consults and repeat HCT. This is associated with a significant reduction in length of stay.

Rosette-forming glioneural tumors (RGNTs) are rare. Here, we report a case of RGNT of the fourth ventricle in an 18-year-old female. The patient presented with a 4-month history of headache and dizziness. Neurological examination showed papilledema, impaired tandem gait, and right-sided dysmetria. Radiological images showed a posterior fossa lesion in the fourth ventricle with hydrocephalus. An emergent ventriculostomy was performed followed by gross total surgical resection of the lesion. Histopathological examination confirmed the diagnosis of RGNT. The patient developed posterior fossa syndrome postoperatively which improved on follow-up. Although rare, RGNT should be considered in the differential diagnoses of posterior fossa lesions in young patients. Given its benign course, surgical resection remains the treatment of choice.

Summary Background Paediatric inflammatory multisystem syndrome temporally associated with SARS-CoV-2 (PIMS-TS) is a new, rare, post-infectious complication of SARS-CoV-2 infection in children. We aimed to describe the 6-month outcomes of PIMS-TS. Methods This retrospective cohort study comprised children (aged Findings 46 children were included in this study. The median age at presentation was 10·2 years (IQR 8·8–13·3), 30 (65%) patients were male and 16 (35%) were female, 37 (80%) were from minority ethnic groups, and eight (17%) had pre-existing comorbidities. All patients had elevated markers of systemic inflammation at baseline. None of the patients died. By 6 months, systemic inflammation was resolved in all but one patient. 38 (90%) of 42 patients who had positive SARS-CoV-2 IgG antibodies within 6 weeks of admission remained seropositive at 6 months. Echocardiograms were normal in 44 (96%) of 46 patients by 6 months, and gastrointestinal symptoms that were reported in 45 (98%) of 46 patients at onset were present in six (13%) of 46 patients at 6 months. Renal, haematological, and otolaryngological findings largely resolved by 6 months. Although minor abnormalities were identified on neurological examination in 24 (52%) of 46 patients at 6 weeks and in 18 (39%) of 46 at 6 months, we found minimal functional impairment at 6 months (median Expanded Disability Status Scale score 0 [IQR 0–1]). Median manual muscle test-8 scores improved from 53 (IQR 43–64) during hospital admission to 80 (IQR 68–80) at 6 months, but 18 (45%) of 40 patients showed 6-min walk test results below the third centile for their age or sex at 6 months. PedsQL responses revealed severe emotional difficulties at 6 months (seven [18%] of 38 by parental report and eight [22%] of 38 by self report). 45 (98%) of 46 patients were back in full-time education (virtually or face to face) by 6 months. Interpretation Despite initial severe illness, few organ-specific sequelae were observed at 6 months. Ongoing concerns requiring physical re-conditioning and mental health support remained, and physiotherapy assessments revealed persisting poor exercise tolerance. Longer-term follow-up will help define the extended natural history of PIMS-TS. Funding None.

Background: Chronic low back pain is a serious health problem. Chronic pain affects negatively quality of life and should be treated as interest as personal handicapping issues. Objective: To discuss etiologies and risk factors of failed back surgery syndrome (FBSS) with evaluation of different management modalities to help lowering its incidence and upgrading patient’s outcome. Patients and methods: This was a cross sectional retrospective study on randomized group of patients (50 patients) performed between May 2014 and May 2018 at El Sheikh Zayed specialized hospital. They complained from lower back pain with or without legs pain and had done one or more lumbar surgeries. All patients were subjected to history taking and neurological examination (motor power, stretch signs - strait leg raising, femoral stretch- and bladder control). Radiographic documentation was for all patients including magnetic resonance imaging (MRI) lumbar spinal stenosis (LSS). Results: Forty-four patients (88%) were managed by surgical re-intervention after clinical evaluation including complain analysis and neurological assessment and correlating it with pre-operative radiological findings. Six patients (12%) were found to have no radiological findings correlated to their complaints. Conclusion: Failed back surgery syndrome acts like a malignant disease that we are unable to completely treat it, but we can control it. In our study, we can catch many factors which will help. Patient selection to be fit candidate for lumbar surgery is a main matter.

Aim. To estimate psychophysiological changes during workspace virtualization.Materials and Methods. We evaluated the psychophysiological profile of 10 healthy right-handed males aged 25 to 45 years before, during and after the working in a virtual reality (VR) headset. All participants had higher education, normal or corrected to normal vision, and were experienced computer users. Psychometric testing included a neurological examination, assessment of functional and feedback-related brain activity (reaction time, errors, and missed signals) and attention span, quantification of processed symbols in the 1st and 4th minutes of Bourdon test, analysis of short-term memory (10 words, 10 numbers and 10 meaningless syllables memorization) and spatial perception, and multi-channel electroencephalography recording in rest.Results. Deterioration of psychometric indicators after a cognitive load in a VR headset was documented only in the most difficult tasks: the number of errors increased by 93% in the brain performance test and by 65% in the attention distribution test. The analysis of electroencephalography data showed that the delta rhythm and theta1 rhythm activity decreased by 28 and 13%, respectively, after working in a VR headset as compared to baseline values, while alpha1 rhythm activity increased by 96%. Probably, the observed electroencephalography changes corresponded to the patterns of brain activation associated with cognitive load and the resulting fatigue.Conclusions. We developed a suitable approach for the psychometric testing before and after working in VR headset, which demonstrated general tolerance and acceptable subjective difficulties to VR load.

The α2 agonists act through the endogenous sleep-promoting pathways to exert their sedative effect. Dexmedetomidine produces a decrease in activity of the projections of the locus ceruleus to the ventrolateral preoptic nucleus. This increases GABAergic release in the tuberomammillary nucleus, producing a decrease in histamine release in cortical and sub cortical projections. All patients underwent a thorough pre-anaesthetic checkup which included history taking, general examination, systemic examination and local neurological examination. Routine investigations like Haemogram, blood sugar, blood urea, serum creatinine, Chest X-Ray, ECG were carried out for all patients. All patients were taught about pain scale regarding VAS scale during pre-operative visit. Mean blood pressure in group D was lower compared to group P since immediately after induction upto 20 minutes after induction, which were statistically significant. There was no statistically significant difference in mean blood pressure of the patients between the two groups after 20 minutes.

This study aimed to investigate the efficacy of the combination of neuron-specific enolase (NSE) measurement and initial neurological examination in predicting the neurological outcomes of patients with cardiac arrest (CA) by retrospectively analyzing data from the Korean Hypothermia Network prospective registry. NSE levels were recorded at 48 and 72 h after CA. The initial Full Outline of UnResponsiveness (FOUR) and Glasgow Coma Scale (GCS) scores were recorded. These variables were categorized using the scorecard method. The primary endpoint was poor neurological outcomes at 6 months. Of the 475 patients, 171 (36%) had good neurological outcomes at 6 months. The areas under the curve (AUCs) of the categorized NSE levels at 72 h, GCS score, and FOUR score were 0.889, 0.722, and 0.779, respectively. The AUCs of the combinations of categorized NSE levels at 72 h with categorized GCS scores and FOUR score were 0.910 and 0.912, respectively. Each combination was significantly higher than the AUC value of the categorized NSE level at 72 h alone (with GCS: p = 0.015; with FOUR: p = 0.026). Combining NSE measurement and initial neurological examination improved the prediction of neurological outcomes.

Background HCV‐related extra‐hepatic complications include peripheral neuropathies, with important prevalence and impact. A recent metanalysis of previous intervention trials concluded for insufficient data to support evidence‐based treatments for this complication. In this longitudinal study, we assessed for the first time prevalence and outcome of neuropathy in a cohort of patients with chronic HCV, before and after direct‐acting antiviral agent (DAA) treatment. Method Ninety‐four patients (mean age 58.5 ± 9.9, infection duration 22.2 ± 6.3 years) without systemic and metabolic diseases, underwent neurological examination and electroneurography studies before (T0) and 10.4 ± 1.7 months after the end of DAA therapy (T1), and cryoglobulins (CG) assessment. Muscle strength was evaluated by Medical Research Council (MRC) score; neuropathic pain, sensory function, disability, quality of life were assessed by validated questionnaires (DN4, NPSI, SSS, INCAT and Euro‐QoL). Results At T0, sensory‐motor neuropathy was detected in 22 patients (23%), reflexes were depressed in 32 (34%) with no association with infection duration, viral load, age, CG. Neuropathic pain (DN4 ≥4) was present in 37 patients (39%). At T1, out of the 22 patients with altered electroneurography, 3 had died or developed HCC, 4 showed normal electroneurography, and nerve amplitude parameters tended to improve in the whole group. Only 11 patients (12%) had depressed reflexes and 10 (11%) DN4 ≥4 (P

Background:Even though Epilepsy is a common neurological disorder globally, developing countries still have insufficient epidemiological data regarding epilepsy. Aim of the Work:We aimed to estimate the prevalence of epilepsy in primary and preparatory school students in New Damietta city, Damietta governorate, Egypt. Patients and Methods:A cross-sectional community-based study that was conducted over a period of six months from February to July 2020. All students from conventional schools were screened using a special validated Arabic translated questionnaire. Students with positive questionnaire findings were subjected to a detailed history, neurological examination, Electroencephalography [EEG], and Magnetic resonance imaging [MRI] whenever possible. Data were coded and analyzed. Result:Lifetime prevalence was 7.5/1000 in conventional schools. Male: Female ratio was 2:1 with statistical significance [95% Confidence Interval= 0.276 to 0.963]. Low socioeconomic class students had a statistically significant higher prevalence of epilepsy. Prenatal and neonatal insults, history of febrile convulsions, and family history of epilepsy were the risk factors with statistical significance. Generalized seizures were more frequent than focal and 62.2% of children were receiving treatment. EEG detected epileptiform discharges in 33.3% of students. Conclusion: The prevalence of overall epilepsy among primary and preparatory school students was similar to that of other Egyptian districts within the same age group. Being male and low socioeconomic status was associated with a high prevalence of epilepsy. The reported prevalence is similar to the prevalence of epilepsy in other Egyptian Governorates and other Arab countries, but lower than the prevalence in Upper Egypt and most developing countries.

Background Spontaneous intracranial hypotension (SIH) is characterized by positional headache caused by low CSF pressure, without any major traumatic event. Optimal treatment is still debated; epidural blood patch (EBP) is usually used after unsuccessful conservative treatment with variable efficacy and potentially severe complications. Although steroids have been reported to be beneficial, their effectiveness is still controversial, and more clinical evidence is needed. Case presentation A 37-year-old woman was admitted to the neurology department due to severe orthostatic headache with nausea over the last 5 days. No trauma history or spinal manipulation were mentioned. On arrival, neurological examination, brain CT, and laboratory investigation were normal. Intracranial hypotension was clinically suspected, and lumbar puncture revealed low opening pressure. Brain MRI demonstrated pachymeningeal gadolinium enhancement and distended and rounded dural venous sinuses, while cervicothoracic spine MRI revealed thoracic CSF leakage, leading to SIH diagnosis. The patient was treated with high-dose intravenous methylprednisolone, with complete clinical resolution within 24 h. Conclusions Our case, combined with literature evidence, supports the high-dose intravenous corticosteroids as a reasonable treatment option in selected cases, before trying EBP or surgical repair. Randomized clinical trials are needed, in order to optimize SIH patients’ outcomes.

Background Variable neurological manifestations and imaging findings have been described in children with severe hypernatremia. We aimed to describe the spectrum of neuroimaging changes in infants with severe hypernatremia. Methods This retrospective study included infants with severe hypernatremia (serum sodium >160 mEq/L), abnormal neurological examination, and an abnormal magnetic resonance imaging (MRI) of the brain over a period of 2 years in a tertiary care hospital. Relevant clinical data, including the feeding practices, clinical features, complications, and biochemical and radiological parameters, were entered in a structured pro forma. MRI findings were classified as vascular (hemorrhages and cerebral sinus venous thrombosis), osmotic demyelination syndrome (pontine and extrapontine myelinolyses), and white matter changes. Results The common clinical features in the neonates were poor feeding (n = 4) and decreased urine output (n = 4); the older infants presented with gastrointestinal losses (n = 5). All cases had dehydration with encephalopathy. The patterns of radiological injury were vascular (hemorrhages, n = 5 and venous thrombosis, n = 3), osmotic demyelination (n = 8), and white matter changes (n = 7). Coagulopathy was correlated with the vascular complications (r = 0.8, p Conclusion Characteristic neuroimaging findings are vascular changes in the form of venous thrombosis and hemorrhages, osmotic demyelination and white matter tract injury, and/or mostly combinations of these findings. Severe hypernatremia and resulting hyperosmolarity frequently cause neurological sequelae in neonates and infants.

POLR3-related disorders are rare hypomyelinating leukodystrophies associated with hypodontia. We present a female patient, who was referred to pediatric neurology at 2 years of age for tremor, low tone, and motor delays. In addition, she was noted to have a delay in her teeth eruption and myopia. Neurologic examination was significant for ataxic features and global developmental delay. Laboratory workup was unrevealing. MRI was significant for hypomyelination. Genetic testing confirmed a pathogenic variant of POLR3B. POLR3-related leukodystrophies should be considered in patients who present with hypotonia, ataxia, and hypodontia. There are many different subtypes of POLR-related leukodystrophies each with distinguishing phenotypic and radiographic features. Although MRI can be helpful in initial evaluation, genetic testing is needed for confirmatory diagnosis and to guide prognosis.

MEGDEL 3-methylglutaconic (MG) aciduria, deafness, encephalopathy, Leigh-like syndrome is an autosomal recessive disorder associated with infantile hypoglycemia, progressive psychomotor developmental delay, cerebellar atrophy with lesions in the basal ganglia, spasticity, dystonia, deafness, and transient liver problems, which typically occur in the first year of life. Other clinical presentations include failure to thrive, epilepsy, and optic nerve atrophy. The serine active site-containing 1 (SERAC1) mutation is localized at the mitochondria-associated membranes, which are responsible for encoding a phosphatidylglycerol remodeler essential for both mitochondrial function and intracellular cholesterol trafficking and is thus responsible for the disease. Diagnosis is confirmed by the elevation of and concentrations of 3-MG acid and 3-methylglutaric acid in the urine or by identification of bi-allelic SERAC1 pathogenic variants on molecular genetic testing. Different pathological variants of SERAC1 have been identified in MEGDEL syndrome to date. Here, we report a case of a child with MEGDEL syndrome due to SERAC1 mutation. The child presented with accidental finding by CT showing hypodensity on bilateral symmetric anterior putamen and caudate abnormal. Neurological examination was unremarkable. This report presents a new neuroimaging finding by CT of MEGDEL syndrome.

Background The rehabilitation of patients with chronic prosopagnosia that occurs following a stroke is a challenge for modern medicine. Dysfunction to the facial processing areas is permanent and standard rehabilitation brings only limited improvement. Therefore, therapists suggest reinforcing the compensatory strategies used by such patients such as voice, figure, and gait recognition to help with the identification of a particular person, which promotes their social functioning. New neurotechnologies, especially QEEG/ERPs, displays of functional brain impairment in prosopagnosia, may be helpful in developing an appropriate neurotherapy protocol and create the conditions for other forms of rehabilitation in such patients. The purpose of our study was twofold: 1) to evaluate QEEG/ ERPs shows of post-stroke functional impairment associated with prosopagnosia, 2) to construct a neurofeedback protocol based on these indices to sup- port the neuropsychological rehabilitation of the case study described herein. We present the case of a 23-year-old right-handed student of the Graphics Faculty of the Academy of Fine Arts, with chronic associative prosopagnosia after infection with SARS-CoV-2 followed by Covid-19 and a right hemisphere stroke. He was re- ferred in April 2021 for diagnosis and therapy at the Reintegration and Training Cen- ter of the Polish Neuropsychological Society (PTNeur). Six months earlier, in October 2020, the patient had been admitted to the Infectious Disease Hospital. COVID-19 was diagnosed based on coronavirus 2 (SARS-CoV-2) reverse transcrip- tion PCR (RT-PCR) on a nasopharyngeal swab. The neurological examination re- vealed muscle weakness on the left side of the body, slow and aprosodic speech, preserved comprehension, and acute left homonymous hemianopsia, as well as prosopagnosia and mirror symptom. The patient was sedated and mechanically ventilated for six days. The CT-scan showed foci in the posterior part of the superior temporal lobe and hyperintense changes in the blood supply area of the right middle cerebral artery. After 30 days of hospitalization, the patient was discharged from this hospital and referred to an outpatient rehabilitation center for five months. Ther- apy improved his general condition but did not remove the chronic prosopagnosia: a personal tragedy for the patient which prevented him from continuing his studies. He was diagnosed at the PTNeur Reintegration and Training Center within the next few weeks: (1). ophthalmologic examinations revealed no pathology; (2) neuropsy- chological testing confirmed the presence of chronic apperceptive prosopagnosia; (3) examination of event-related potentials (ERPs) revealed a large delay of the N170 wave, particularly on the right side, indicating a slowing of the rate of nerve impulses in early face processing and a cause of prosopagnosia. The patient was referred for rehabilitation: he participated in 20 sessions of individually tailored anodal transcranial direct current stimulation (tDCS) twice a week for ten weeks, and in parallel, for indi- vidualized Prosopagnosia Symbolic Art Therapy provided once a week for ten weeks. By the end of therapy, the patient was not only recognizing but also painting portraits of faces. He returned to college, finished and defended a master’s thesis in Artistic Drawing, in which facial presentation played an important role. Quantitative EEG (QEEG) and event-related potentials (ERPs) neuromarkers helped to understand the mechanism of prosopagnosia and to choose an individualized protocol, thus the appropriate application of tDCS in our patient, which accelerated the recovery of the ability to perform complex tasks and created the conditions for Symbolic Art Therapy. Modern medicine can successfully use such a management protocol in individuals with chronic prosopagnosia.

To correlate arterial umbilical cord gas (aUCG) and infant blood gas with severity of neurological injury. Retrospective single-site study of infants evaluated for therapeutic hypothermia. Clinical neurological examination and a validated MRI scoring system were used to assess injury severity. Sixty-eight infants were included. aUCG base deficit (BD) and lactate correlated with infant blood gas counterparts (r = 0.43 and r = 0.56, respectively). aUCG and infant pH did not correlate. Infant blood gas lactate (RADJ2 = 0.40), infant BD (RADJ2 = 0.26), infant pH (RADJ2 = 0.17), aUCG base deficit (RADJ2 = 0.08), and aUCG lactate (RADJ2 = 0.11) were associated with clinical neurological examination severity. aUCG and infant blood gas measures were not correlated with MRI score. Metabolic measures from initial infant blood gases were most associated with the clinical neurological examination severity and can be used to evaluate hypoxic-ischemic cerebral injury risk.

The purpose of this study was to demonstrate whether application of the so-called safe incision when performing calcaneal sliding osteotomies reduces the risk of sural nerve injury. Patients who underwent either medial or lateral sliding calcaneal osteotomies between 2010 and 2018 were analysed retrospectively. A thorough neurological examination was performed, and the location of the surgical wound and the type of wound closure were recorded. The European Foot and Ankle Surgery (EFAS) score and 12-item Short Form Survey (SF-12) were also documented. A total of 57 patients were included, of which 20 (35.1%) had a sural nerve injury. Five patients had a neurapraxia (8.8%), while 15 patients had a permanent injury (26.3%). Respecting the “safe incision” decreased sural nerve injury (p = 0.02). The type of osteotomy and closure was not significant. No significant differences were found in the functional tests between the different techniques, or between patients who presented sural nerve injury and those who did not. Sural nerve injury after calcaneal sliding osteotomies is higher than previously reported in the scientific literature, with an incidence of 35.1% (20/57 patients). Respecting the so-called safe zone (oblique incision that runs through the point that is > 1/3 of the distance from the tip of the lateral malleolus to the posteroinferior margin of the calcaneus) clearly decreases the incidence of sural nerve injury. Finally, the majority of patients remained asymptomatic despite the neurological injury.

Objectives: This study aimed to evaluate incidence, mode, and severity of injury, treatment options and to determine the outcome in pediatric age group patients with head injury. Patients & Methods: 120 pediatric patients with a head injury, with ages up to 15 years or less, were included. Detailed history, general and neurological examination including GCS were noted. The CT scans were conducted and the patients managed as per said protocol. Various surgical procedures were performed including craniotomies and evacuation of hematoma, the elevation of depressed fractures and repair of the dura, decompression craniotomies, and evacuation of Subdural collections and contusions. Results: 60.1% of patients were boys and 39.16% were girls. In the majority (63%) of the children, the ‘fall’ was reported. The majority of children (68.3%) in our study had a mild head injury with GCS 13 – 15. 35.83% patients had normal CT scans, and 23.33% had isolated skull fractures. 68.75% patients were with a severe head injury and had a poor outcome. A poor outcome was noted in patients having brain edema, subdural hematoma, and brain contusions. Conclusion: Head injury in the pediatric age group still continues to be a significant cause of mortality and morbidity. The outcome is directly being related to the severity of injury i.e., GCS. Falls and RTAs are the most common causes and preventive measures in different forms need to be addressed.

Craniopharyngioma (CPG) is a benign epithelial tumor that originates from Rathke's pouch. Meningiomas (MNG) are neoplasms of meningothelial cell. The patient is a 42-year-old female who presented with left-sided temporal headaches and visual acuity impairment. Neurological examination revealed bilateral peripheral visual field defects. Imaging of the brain showed a hypointense solid-cystic sellar lesion with suprasellar extension without calcification which enhanced with contrast. Also present was a second, contrast-enhancing extra axial lesion overlying the olfactory groove. The patient had no history of tumor surgery or radiation therapy. Both of the lesions were operated for resection and post operation status was uneventful. This study reports non-radiation related co-occurrence of a craniopharyngioma (CPG) and a meningioma (MNG). We reviewed published articles between 1966 and 2020, and found that although MNGs are the most commonly occurring brain tumors, the occurrence of MNG and CPG together with no relation to radiation is relatively rare, reported only 7 times in living patients. By analyzing the data and keeping in mind that there is no proven common genetic background and risk factor between them, their co-occurrence, shows the importance of further investigation to clear any possible relationship between the two other than mere coincidence.

Objectives: Acute Traumatic Subdural Hematoma (ASDH) is one of the most dangerous and challenging neurosurgical problems faced by neurosurgeons. Road traffic accidents (RTAs) and falls are the most common causes of ASDH. This study focused to evaluate the incidence, mode, and severity of the injury, treatment options, and determine outcomes in patients with ASDH. Material & Methods: Thirty patients with ASDH were studied over a period of three years. Detailed history, general and neurological examination including GCS were noted, CT scans were done and patients managed according to said protocol. Results: Twenty-three (76.66%) were male and Seven (23.33%) were female. The common mode of injury were road traffic accidents (66.66%) and falls (26.66%). Out of 30 patients, 53.33% had GCS 3-5. Overall, the mortality rate was 60%. 23.33% of patients survived with severe disability and 10% of patients showed moderate disability and good recovery. The mortality rate was higher in patients above 50 years of age. Conclusion: Acute traumatic subdural hematoma is still very fatal and has a high mortality. Early CT scanning emergency, surgery and good postoperative ICU care can play a role in improving the outcome in patients with ASDH.