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Introduction Osteoarthritis (OA) of the hip is a low-grade inflammatory disease of multiple etiology that has a limited conservative management and insufficiently explored. The application of botulinum toxin type A (BoNT-A) produces in the applied muscle a temporary, delimitable and reversible flaccid paralysis, which applied in a strategic way achieves to reduce mechanical stress and pain. Objective To evaluate the effect on pain perception, functionality and rigidity and changes in flexibility, internal and external rotation, before and 90 days after the application of BoNT-A. Methods Clinical, experimental, longitudinal, open study, with a total of 35 patients and 45 hips treated. BoNT-A of 500 U was applied in iliac, adductor brevis and longus muscles. Results The Wilcoxon test was used to compare the evaluation scores on days 0-90, observing the decrease in referred pain (p < 0.0001), rigidity (p < 0.002), improved perceived function (p < 0.001) and mobility arcs: flexion, internal and external rotation (p < 0.0001). Conclusions The treatment with BoNT-A provides a conservative and safe option for the management of symptoms and physical restraint caused by hip OA.

An infusion of 100cc of 0,2% potassium chloride was accidental performed through a thoracic epidural catheter, inserted to perioperative analgesia, to a 66years old man who was scheduled for right hemicolectomy, 48hours after surgery. Paresis of upper limbs, flaccid paralysis of lower limbs and a sensitive level at T8 was observed. An epidural lavage with an initial dose of 20cc of saline was slowly injected, followed for a saline infusion of 20cc per hour. Neurologic signs were totally reverted some hours later and 24hours after the incident the physical exam was normal. We reviewed the clinical presentation of the complication and its mechanisms, the more frequent clinical evolution, as well as treatment measures and strategies to prevent the incident.

Background Guillain-Barré syndrome (GBS) is the main cause of acute and subacute flaccid paralysis in western nations since the eradication of poliomyelitis. Objective The aim of this study is to investigate epidemiology and mortality characteristics of GBS in the north of Iran. Material and methods In this study, the hospital information system (HIS) was used to access each patient’s information. The final 174 cases were examined in terms of age, sex, place of residence, the year of referral, the month of referral, the season of referral, client city, accompanying background disease, and the type of GBS. Results The mean incidence rate in Guilan province was about 0.69 in 100,000 persons, and the case fatality rate was 10.34%. The most reported type of GBS was AIDP (33.90%), and the most common symptom was upper and lower limbs paresis in 65 cases (37%). Respiratory distress (P =

Patients with facial paralysis require a systematic zonal assessment. One frequently overlooked region is the effect of facial paralysis on nasal airflow. Patients with flaccid paralysis experience increased weight of the cheek and loss of muscle tone in the ala and sidewall; this significantly contributes to nasal valve narrowing and collapse. These specific findings are often not adequately corrected with traditional functional rhinoplasty-grafting techniques. Flaccid paralysis typically results in inferomedial displacement of the alar base, which must be restored with suspension techniques to fully treat the nasal obstruction. Multiple surgical options exist and are discussed in this article.

Introduction: Inflammatory demyelinating disease like combined central and peripheral demyelination (CCPD) could have varied clinical presentation depending upon the topographical distribution of neural involvement. Case presentation: A seven-year-old child had presented with fever followed by acute onset flaccid paralysis and urinary retention. Weakness in the lower limbs as reported was ascending and symmetric in nature, while no history of trauma, band-like sensation or altered sensorium were documented. Superficial and deep tendon reflexes of both the lower limbs were absent. Routine blood investigations had revealed neutrophilic leucocytosis only. Serum IgM antibody for scrub typhus was found positive. CSF study didn’t show cyto-protein dissociation. NCV had demonstrated absence of F wave and H reflex in the peripheral nerves of lower limbs. Anti-ganglioside antibody profiles were negative. Subsequent investigations including MRI brain and spinal cord had revealed acute onset CCPD. Conclusion: Acute onset combined central and peripheral demyelination in a child had presented as acute flaccid paralysis of the lower limbs and the condition was temporally association with scrub typhus.

Guillain–Barré syndrome (GBS) is an acute, immune-mediated inflammatory peripheral polyneuropathy characterized by ascending paralysis. Most GBS cases follow gastrointestinal or chest infections. Some patients have been reported either following or concomitant with head trauma, neurosurgical procedures, and rarely hemorrhagic stroke. The exact pathogenesis is not entirely understood. However, blood–brain barrier damage may play an essential role in triggering the autoimmune activation that leads to post-stroke GBS. Here, we present two cases of fulminant GBS following hemorrhagic stroke to remind clinicians to be aware of this rare treatable complication if a stroke patient develops unexplainable flaccid paralysis with or without respiratory distress.

Guillain-Barre syndrome (GBS) is a common cause of flaccid paralysis. Up to a third of cases result in respiratory failure requiring mechanical ventilation. We aim to study the possible predictors of respiratory compromise requiring mechanical ventilation in cases of GBS as well as their clinical outcome in the UAE population. A retrospective observational study was conducted between the periods of January 2009 till January 2019 in a tertiary center in Dubai. Cases were grouped according to their need for mechanical ventilation then compared for possible predicting factors. Up to 1-year follow-up was done to assess the clinical outcome. Out of the 82 cases that were included in the study, 64 (78%) were males with a mean age at presentation being 37 ± 14.4. Most of the cases presented with disability score of 4 (n= 33, 40.2%), being bed bound or wheelchair bound. Twenty cases (24.4%) required mechanical ventilation at presentation. Axonal type of nerve injury was present in 11 (55%) patients requiring intubation, in contrast to 17 (27.4%) patients not requiring intubation. Cases that required mechanical ventilation were older (P .028). They also had a reduced rate of recovery after 1 month (P .004), and more residual deficit at 6 months (P .003) and 12 months (P

Guillain Barre Syndrome (GBS) is usually a post-infectious autoimmune disease that manifests as acute ascending flaccid paralysis. The disease is usually uncommon. However, recently it was reported in a few COVID-19 cases before complete resolution of COVID symptoms. An association between olfactory-gustatory disturbances and sensory abnormalities is frequently observed in GBS with COVID-19. The electrophysiological studies usually reveal a demyelinating pattern. Respiratory involvement, as part of respiratory muscle paralysis or COVID-19 pneumonia, is associated with poor recovery in affected patients. Here, we present a case of a young man, pre-morbid healthy, who presented with GBS with mild COVID-19 infection. He successfully recovered after treatment with Intravenous immunoglobulin IVIg. How to cite this article:Dabas A, Kumar N. Guillain Barre Syndrome in a Young Man with SARSCoV-2: A Rare Association. J Adv Res Med 2021;8(1): 22-25. DOI: https://doi.org/10.24321/2349.7181.202104

INTRODUCTION: Acute Flaccid Myelitis (AFM) is a recently recognized pediatric condition characterized by flaccid paralysis that can cause lifelong deficits. Adhesive Capsulitis (AC) is a chronic condition that causes loss of passive range of motion in the shoulder due to joint contracture. CASE REPORT: This is a case report of a 15-year-old male who suffered from shoulder pain with progressive loss of passive range of motion after AFM, diagnosed with AC based on exam and radiology. After failing conservative management, the patient was successfully treated with ultrasound guided corticosteroid injection. DISCUSSION/CONCLUSION: AC is uncommonly reported in pediatric patients. However, secondary adhesive capsulitis is frequently associated with disorders of shoulder immobility in adults. This case contributes to the body of literature regarding pediatric frozen shoulder, highlights the successful use of ultrasound guided injections for management, and suggests a relationship between AFM and AC which may be beneficial for pediatric physiatrists in treating patients with shoulder pain or immobility related to this condition.

Background Acute flaccid myelitis (AFM) is an acute-onset anterior horn disease resulting in flaccid paralysis of extremities, trunk, facial, and cervical musculature in children following upper respiratory or gastrointestinal viral illness. Nerve transfer procedures have been shown to restore function. Objective To present a technical description of sciatic-to-femoral nerve transfers in 4 children with AFM. Methods Retrospective review of relevant cases was performed. Results A total of 4 cases are presented of young children with persistent quadriparesis in the setting of AFM, presenting between 4 and 15 mo following initial diagnosis. Electromyography showed denervation of muscles innervated by the femoral nerve, with sparing of the sciatic distribution. The obturator nerve was also denervated in all patients. We therefore elected to pursue sciatic-to-femoral transfers to restore active knee extension. These transfers involved end-to-end coaptation of a sciatic nerve fascicle to the femoral nerve motor branches supplying quadriceps muscles. Conclusion We present technical descriptions of bilateral sciatic-to-femoral nerve neurotization for the restoration of quadriceps function in 4 patients with AFM. The sciatic nerve fascicles are a reasonable alternative donor nerve for patients with proximal muscle paralysis and limited donor options in the lower extremity.

This article analyzes the literature related to flaccid paresis and paralysis of the upper extremities in children during the first months of life. This pathology is a heterogeneous group of diseases with different etiopathogenesis. There are various courses of flaccid paresis and paralysis of the upper extremities in children: damage to the spinal cord, brachial plexus, peripheral nervous system to the level of the brachial plexus, and isolated damage to peripheral nerves. According to the time of occurrence, flaccid paresis and paralysis can be divided into three groups: antenatal, intranatal, and postnatal pathology. The main mechanism of occurrence of this pathology is intranatal trauma. More rare causes of flaccid paresis and paralysis of the upper extremities are antenatal conditions of dysplastic and traumatic origin, postnatal damage to the peripheral nervous system due to trauma or infection. Congenital contractures of the upper extremities combined with flaccid paralysis are connected with genetically determined diseases of the lower motor neurons and congenital myopathies, intrauterine injuries of the brachial plexus peripheral nerves. This article discusses the issues of topical and differential diagnosis of this pathology, the clinical picture suitable for each period of the childs life, and the prognosis of the disease. This research will be useful not only for neurologists, but also for specialists of related specialties: orthopedists, physiotherapists, and neonatologists for making correct the diagnosis, providing adequate treatment, and predicting its results.

Neurologic manifestation of coronavirus disease 2019 (COVID-19) in children is evolving with time. We are reporting a young girl who presented to us with acute febrile illness followed by acute onset severe flaccid paralysis requiring prolonged intensive care unit stay and ventilator support. She was evaluated extensively and found to be positive for COVID serology, and neuroimaging revealed features of longitudinally extensive transverse myelitis (LETM) with enhancing cauda equina nerve roots, suggesting Guillain-Barré Syndrome (GBS). She failed to respond to immune suppressive therapy and needed plasma exchange for recovery. Like other common viral illnesses, COVID-19 can also act as a trigger for GBS-like illness and LETM, and we need to suspect these diagnoses in the cases with COVID-19 infection in compatible cases. This is probably the first pediatric case with concurrent GBS and LETM secondary to COVID-19 infection.

We report a 5-year-old male with a PDHA1 variant who presented with alternating hemiplegia of childhood and later developed developmental regression, basal ganglia injury and episodic lactic acidosis. Enzyme assay in lymphocytes confirmed a diagnosis of Pyruvate Dehydrogenase Complex (PDC) deficiency. His mother who was heterozygous for the same variant suffered from ophthalmoplegia, chronic migraine and developed flaccid paralysis at 36 years of age. PDHA1 is the most common genetic cause of PDC deficiency and presents with a myriad of neurological phenotypes including neonatal form with lactic acidosis, non-progressive infantile encephalopathy, Leigh syndrome subtype and intermittent ataxia. The presentations in our 2 patients contribute to the clinical heterogeneity of this neurogenetic condition.

Hematological malignancies rarely present with spontaneous haematomas (Lakhotia et al., 2015 [1]). Although cutaneous and mucous membrane bleeds do occur in chronic myeloid leukemia (CML) due to quantitative or qualitative platelet abnormalities, deep soft tissue bleeds are rare (Lakhotia et al., 2015 [1]). We report the case of a 49 year old man presenting with an acute hematoma of the left biceps brachii causing compartment syndrome of his left upper limb leading to flaccid paralysis. He underwent surgical evacuation of the hematoma and investigations revealed that he had CML with leukemic infiltration in the biceps brachii.

Background and Importance: Diffuse leptomeningeal glioneuronal tumor (DLGNT) represents a provisional entity in the 2016 World Health Organization classification of tumors; it is characterized by a widespread leptomeningeal growth and oligodendroglial-like cytology. To this day, 4 pediatric patients have been reported to present with an isolated spinal cord tumor in the absence of leptomeningeal dissemination. Gross total resection (GTR) was achieved in only 1 patient. We present the clinical and technical nuances of this unique type of tumor, as well as the second reported case of GTR in a patient with DLGNT. Clinical Presentation: A 4-year-old boy presented to the emergency department after an episode of flaccid paralysis of bilateral lower extremities. MRI showed an intramedullary spinal cord tumor centered at T8. The patient was taken to the operative room, where a laminectomy and tumor resection were performed; cystic and solid tumor components were identified. Pathology report was consistent with DLGNT. After achieving GTR, patient is free of recurrence after a 15-month follow-up. Conclusion: No standard treatment for DLGNT has been identified. Current literature report surgery and chemotherapy with variable success rates. DLGNT presenting as an isolated intramedullary tumor is an uncommon condition which progression appears to be halted when treated promptly. Identifying solid and cystic components of this tumor is crucial for achieving GTR.

Guillain-Barre syndrome (GBS) is an acute immune-mediated progressive predominantly motor symmetric polyradiculoneuropathy which causes demyelination and leads to weakness, ataxia and areflexia. There are a variety of forms of the syndrome; and despite being the most common cause of acute flaccid paralysis in children, it has a low incidence under 18 years old, and it is even rarer in children less than 2 years of age. Very few cases have been reported under 12 months of age. We describe a case of an 11-month-old male infant presenting with weakness and inability to ambulate who was diagnosed with GBS.

Background: Guillain-Barré syndrome (GBS) is an acute autoimmune polyneuroradiculopathy characterized by flaccid paralysis which may lead to respiratory failure requiring intensive care. Objective: The purpose of the present study was to explore the relationship between the fasting plasma glucose (FPG), hemoglobin A1c (HbA1c) and disease severity of GBS patients who are not known to have DM. Methodology: This cross-sectional study included adult GBS patients without having DM [age 35 (22-48) years, median (interqurtile range, IQR); 39 male 22 female] who were admitted to Neurology department, National Institute of Neurosciences and Hospital, Dhaka, Bangladesh from July 2018 to June 2019. Demographics, clinical data were noted and FPG, HbA1c were measured. Disease severity were assessed by the GBS disability scale ranging from 0 to 6 with increasing score reflecting increased disability. Results: Patients with more severe GBS (disability score ≥4, unable to walk) had higher frequency of elevated FPG >5.5 mmol/L (61.2%; 30/49) in comparison to those with less severe GBS (disability score ≤3, able to walk; FPG >5.5 mmol/L in 16.7%, 2/12; p=0.006). But distribution of HbA1c category was not different across the groups (disability score ≥4 vs. ≤3: HbA1c 6.4: 10% vs. 17%; p=0.296). Participants with elevated FPG were elder [elevated vs. normal FPG: 40 (28-54) vs. 25 (19-43) years; median (IQR), p=0.012] and had higher CSF glucose (p=0.002) than those with normal FPG, but there was no difference in respct of gender, MRC sum score, requirement of assisted ventilation, CSF protein, GBS subtypes and duration of hospital stay (p=not significant for all). Conclusions: Patients with severe GBS have higher frequency of elevated FPG but not HbA1c. An acute change in glucose metabolism may occur in GBS which needs further study. Journal of National Institute of Neurosciences Bangladesh, 2020;6(2): 96-100

Background: Acute flaccid myelitis (AFM) is an illness defined by rapid onset of flaccid paralysis in one or more limbs or bulbar muscles, with MRI findings of predominantly spinal cord gray matter abnormalities spanning one or more spinal segments following a viral illness. Individuals with AFM may require rehabilitation to promote recovery. Activity-based restorative therapy (ABRT) has previously been shown to result in positive outcomes in children with neurologic deficits related to AFM. Objectives: This study examined functional changes in a group of children with AFM who participated in ABRT in an inpatient setting. Methods: Retrospective chart review of children with AFM admitted to a single inpatient rehabilitation unit from 2014 to 2018. Children were assessed using the Functional Independence Measure for Children (WeeFIM), Manual Muscle Testing (MMT), Spinal Cord Independence Measure (SCIM), and the Physical Abilities and Mobility Scale (PAMS) as part of routine clinical care; the Modified Rankin Scale for Neurologic Disability was completed retrospectively. Results: Children showed significant improvements across all outcome measures, with effect sizes ranging from moderate to large. Significant change was also seen across all muscle groups on MMT, with effect sizes ranging from small to large. Consistent with previous reports, children demonstrated better function in distal than proximal muscle groups at both admission and discharge. Conclusion: Children with AFM who participated in ABRT increased muscle strength and made functional gains across all outcome measures. These results support the utility of rehabilitation in the long-term care of children with AFM and residual neurologic deficit.

Introdução: A Síndrome de Guillain-Barré (SGB) é a causa mais frequente de paralisia flácida aguda e subaguda desde a erradicação da poliomielite. Embora rara, é reconhecida como a principal causa de paralisia flácida entre pessoas internadas em terapia intensiva pediátrica por doenças neuromusculares agudas. Objetivo: Relatar um caso de paciente do sexo masculino, com 14 meses de idade, com diagnóstico provável de Síndrome de Guillain-Barré com neuropatia sensitivo motora, aguda, mielínica, com provável comprometimento axonal secundário, com rápida evolução e melhora. Descrição do caso: Foi admitido em hospital público maternoinfantil de referência para o Distrito Federal um paciente masculino, residente na Região Integrada de Desenvolvimento do Distrito Federal e Entorno. A criança tinha 14 meses de idade e 8,6kg, situação vacinal atualizada e desenvolvimentoneurospicomotor adequado para a idade, com quadro de paresia em membros inferiores, sem alterações cognitivas. Após 14 horas da admissão, diante do agravamento do quadro clínico e da dissociação albomino-citológica identificada pela análise de líquido cefalo-raquidiano foi iniciada imunoterapia (imunoglobulina humana endovenosa, 0,7g/kg/dia por três dias). Após 24 horas do início do tratamento, a criança apresentou melhora em seu estado geral. O paciente teve alta hospitalar após cinco dias de internação. Após 76 dias da alta, foi constatada melhora significativa no desenvolvimento neuropsicomotor, apesar de leve atraso em seu desenvolvimento até o momento. Conclusão: Diante da raridade de casos em crianças, é importante que os profissionais de saúde se mantenham sensíveis a captar e tratar os casos de maneira oportuna. Recomendamos ainda que os casos identificados sejam acompanhados cuidadosamente, afim de verificar se a SGB, esuas variantes, podem explicar transtornos de desenvolvimento à posteriori.

Guillain–Barré syndrome – an acute demyelinating autoimmune disease characterized by lesions of the peripheral nervous system and consequently peripheral paralysis, paresthesias and/or pain. Guillain–Barré syndrome is a predominant cause of acute flaccid paralysis, which may occur at any age. We present two clinical cases of flaccid paralysis with patients at the age of 7 and 5 years requiring verification of the diagnosis.

Background Hypokalemic periodic paralysis is a rare skeletal muscle channelopathy characterized by intermittent episodes of acute flaccid paralysis with associated hypokalemia. We present here the case of a first-onset hypokalemic periodic paralysis triggered by lumbar spinal surgery for tumor resection. Case Description A 37-year-old male without any known prior medical conditions presented with a first-onset attack of hypokalemic paralysis 1.5 days after lumbar spinal surgery for myxopapillary ependymoma. Initially, the patient presented paraparesis mimicking a spinal cord compression, and while en route for imaging there was an abrupt onset of flaccid paralysis with significant respiratory distress. The emergency blood tests revealed extreme hypokalemia with a serum potassium of 1.42 mm/L. The patient was transferred to the intensive care unit, intubated, sedated, and administered intravenous reperfusion with an infusion dose of 20 mEq/hour potassium in a solution of 5% mannitol. Following reperfusion, the patient recovered completely in 12 hours. Renal potassium hyperexcretion and hyperthyroidism were excluded by laboratory tests. The diagnosis was confirmed by genetic tests showing mutation of the CACNA1S gene. Conclusions To the best of our knowledge, this is the first described case with the first onset triggered by a neurosurgical intervention and the second case following any kind of surgery. Neurosurgeons should consider hypokalemic periodic paralysis when encountering a rapidly evolving tetraparesis, even in an apparently healthy patient.

Periodic paralyses (PPs) are a group of rare disorders characterized by episodic, sudden-onset, flaccid paralysis of skeletal muscles usually resulting in complete recovery after the attacks. PPs are caused by abnormal, mostly potassium-sensitive excitability of the muscle tissue. Hypokalemic and hyperkalemic periodic paralysis (HypoKPP and HyperKPP) have been described according to their characteristic phenotypes and the serum potassium level during the attacks of weakness. The T704M mutation on the SCN4A gene is the most common mutation in HyperKPP. Different mutations of the SCN4A gene have also been reported in some cases of HypoKPP. In this study, a large Turkish family carrying the T704M mutation on the SCN4A gene with HypoKPP disease was examined. A similar history was noted in a total of 17 subjects in the pedigree. SCN4A gene of the patients was sequenced with Sanger sequencing. In this study, this mutation was associated with a HypoKKP diagnosis for the first time in the literature. The symptoms of hallucination and diplopia seen in patients had also never been indicated in the literature before. This report expands the phenotypic variability of the T704M mutation, further confirming the lack of genotype-phenotype correlation in SCN4A mutations. © 2020 Elsevier Ltd

Ventral hernias represent the most common complication after abdominal surgery. Loss of domain and/or large ventral hernias in patients are especially challenging for surgeons to manage, but preoperative image-guided botulinum toxin injection has emerged as an effective adjunct to abdominal wall surgery. Loss of domain is caused by chronic muscle retraction of the lateral abdominal wall and leads to an irreducible protrusion of abdominal viscera into the hernia sac. Botulinum toxin can be used in the oblique muscles as a chemical component relaxation technique to aid abdominal wall reconstruction. Intramuscular botulinum toxin injection causes functional denervation by blocking neurotransmitter acetylcholine release resulting in flaccid paralysis and elongation of lateral abdominal wall muscles, increasing the rate of fascial closure during abdominal wall reconstruction, and decreasing recurrence rates. In total, 200–300 units of onabotulinumtoxinA (Botox®) or 500 units of abobotulinumtoxinA (Dypsort®) in a 2:1 dilution with normal saline is most commonly used. Botulinum toxin can be injected with ultrasonographic, EMG, or CT guidance. Injection should be performed at least 2 weeks prior to abdominal wall reconstruction, for maximal effect during surgery. At minimum, botulinum toxin should be injected into the external and internal oblique muscles at three separate sites bilaterally for a total of six injections. Although botulinum toxin use for abdominal wall reconstruction is currently not indicated by the Food and Drug Administration, it is safe with only minor complications reported in literature.

Differential diagnostics of multifocal motor neuropathy (MMN) has many difficulties associated with a number of factors: rare nature of disease, polymorphic clinical forms and a phenotypic picture similar to peripheral motor neuron diseases. Such diseases also include rare nosological forms: amyotrophic lateral sclerosis, hereditary myopathies and neuropathies; their general phenotypic picture has a form of progressive flaccid paralysis, age of the disease onset and the nature of its course. However, different pathogenesis of these diseases requires a differentiated approach to therapy. This article deals with differential diagnostics of multifocal motor neuropathy, gives examples of modern diagnostic criteria necessary for diagnosing multifocal motor neuropathy and analyzes a clinical case with an incorrect diagnosis of multifocal motor neuropathy.

Acetazolamide is an infrequently prescribed medication in the outpatient pediatric setting, as one of its major indications is for the treatment of glaucoma, which is a largely adult disease. Though incredibly rare, serious neurologic side effects such as stroke, flaccid paralysis, and coma can occur after its administration. This case presentation of acute flaccid paralysis with metabolic derangement underscores the unusual, yet possible, toxic-metabolic and neurologic sequelae that can occur in an already acidotic host (in this case, caused by gastroenteritis) with acetazolamide ingestion. Life-threatening conditions must always be ruled out in patients who present with encephalopathy, but a medication history was crucial in clinching this case's unifying diagnosis. While there are case reports highlighting the rare central nervous system toxicities associated with acetazolamide ingestion, to our knowledge, none exist in the pediatric literature.

Poliomyelitis-like illness is a common clinical manifestation of neurotropic viral infections. Functional loss and death of motor neurons often lead to reduced muscle tone and paralysis, causing persistent motor sequelae among disease survivors. Despite several reports demonstrating the molecular basis of encephalopathy, the pathogenesis behind virus-induced flaccid paralysis remained largely unknown. The present study for the first time aims to elucidate the mechanism responsible for limb paralysis by studying clinical isolates of Japanese encephalitis virus (JEV) and Chandipura virus (CHPV) responsible for causing acute flaccid paralysis (AFP) in vast regions of Southeast Asia and the Indian subcontinent. An experimental model for studying virus-induced AFP was generated by intraperitoneal injection of 10-day-old BALB/c mice. Progressive decline in motor performance of infected animals was observed, with paralysis being correlated with death of motor neurons (MNs). Furthermore, we demonstrated that upon infection, MNs undergo an extrinsic apoptotic pathway in a RIG-I-dependent fashion via transcription factors pIRF-3 and pIRF-7. Both gene-silencing experiments using specific RIG-I-short interfering RNA and in vivo morpholino abrogated cellular apoptosis, validating the important role of pattern recognition receptor (PRR) RIG-I in MN death. Hence, from our experimental observations, we hypothesize that host innate response plays a significant role in deterioration of motor functioning upon neurotropic virus infections. IMPORTANCE Neurotropic viral infections are an increasingly common cause of immediate or delayed neuropsychiatric sequelae, cognitive impairment, and movement disorders or, in severe cases, death. Given the highest reported disability-adjusted life years and mortality rate worldwide, a better understanding of molecular mechanisms for underlying clinical manifestations like AFP will help in development of more effective tools for therapeutic solutions.

Background: Complex ventral hernias following laparotomy present a unique challenge in that repair is hindered by the lateral tension of the abdominal wall. A novel approach to overcome this is the “chemical component separation” technique. Here, botulinum toxin A (BTA) is instilled into the muscles of the abdominal wall. This induces flaccid paralysis and effectively reduces tension in the wall, allowing the muscles to be successfully joined in the midline during surgery. We describe a method where a large incisional hernia was repaired using this technique and review the variations in methodology. Case report: A woman in her mid-40s developed a ventral hernia in the setting of a previous laparotomy for a small bowel perforation. Computed tomography (CT) of the abdomen demonstrated an 85 (Width) × 95 mm (Length) ventral hernia containing loops of the bowel. Pre-operative botulinum toxin A administration was arranged at the local interventional radiology department. A total of 100 units of BTA were instilled at four sites into the muscular layers of the abdominal wall under CT-fluoroscopic guidance. She underwent an open incisional hernia repair 4 weeks later, where the contents were reduced and the abdominal wall layers were successfully joined in the midline. There was no clinical evidence of hernia recurrence at 3-months follow-up. Conclusion: Low-dose BTA effectively facilitates the surgical management of large ventral incisional hernias. There is, however, significant variation in the dosage, concentration and anatomical landmarks in which BTA is administered as described in the literature. Further studies are needed to assess and optimise these variables.

Snakebites are common in India and the most common neurotoxic snakebites in India are due to Common krait (Bungarus caeruleus) and cobra (Naja naja). Severe envenomation may mimic brain death or a locked-in state with flaccid paralysis in a descending manner and total ophthalmoplegia. Usually, patients who receive timely antivenom and ventilator support recover completely without any sequalae. We are reporting two cases of krait bite with an unusually long period of flaccid paralysis, which required prolong ventilation. While case 1 required 10 days of mechanical ventilation followed by 5 days of non-invasive ventilation, case 2 required 11 days of mechanical ventilation followed by 5 days of non-invasive ventilation. Both the cases had delayed recovery and residual weakness at 3-month follow up. These case reports suggest that krait bite may cause prolong neuromuscular weakness in children, which has implications for both acute and chronic management.

Sjogren’s syndrome (SS) is common autoimmune diseases manifesting with various glandular and extra glandular features which are difficult to evaluate and manage. Hypokalemic flaccid paralysis may be the rare extra glandular clinical manifestation of SS. We present a case of acute flaccid paralysis which on evaluation turned out to be primary sjogren’s syndrome (pSS). We present a case of 30 year old young woman with no known comorbidities presented with acute flaccid paralysis. Her evaluation reveals severe hypokalemia with K+-1.6 meq/l, metabolic acidosis with pH-7.28 and urinary pH ?6.5. An initial diagnosis of hypokalemic periodic paralysis (HPP) secondary to distal renal tubular acidosis (dRTA) was made. She was later found to have sjogren’s syndrome on the basis of positive ANA & Anti-Ro (SS-A) Antibody and salivary gland biopsy. She was managed with potassium supplements, sodium bicarbonate and immunomodulators. Primary Sjogren’s syndrome usually associated with distal renal tubular acidosis which may cause hypokalemia leading to HPP, rarely which may be the initial presenting symptom in young women as in our case. Early diagnosis and treatment of these patients would lead to less complication and better quality of life. Keywords: Hypokalemic periodic paralysis, Sjogrens syndrome, Distal renal tubular acidosis.

Hypokalemic periodic paralysis (HypoPP) is a rare autosomal dominant disorder characterized by episodic flaccid paralysis with concomitant hypokalemia. More than half of patients were associated with mutations in CACNA1S that encodes the alpha-1-subunit of the skeletal muscle L-type voltage-dependent calcium channel. Mutations in CACNA1S may alter the structure of CACNA1S and affect the functions of calcium channels, which damages Ca2+-mediated excitation-contraction coupling. In this research, we identified and described a Chinese HypoPP patient with a novel frameshift mutation in CACNA1S [NM_000069.2: c.1364delA (p.Asn455fs)] by targeted sequencing. This study would expand the spectrum of CACNA1S mutations, further our understanding of HypoPP, and provided a new perspective for selecting effective treatments.

Guillain-Barré syndrome (GBS) represents the main cause of flaccid paralysis worldwide. Although most cases have a typical clinical presentation of symmetric ascending flaccid paralysis with areflexia or hyporeflexia, this disease may present as multiple clinical entities, therefore representing a diagnostic challenge for physicians, who should consider these variants when assessing neuropathies. The pharyngeal-cervical-brachial (PCB) variant of Guillain-Barré syndrome is defined by rapidly progressive oropharyngeal and cervicobrachial weakness associated with hyporeflexia or areflexia in the upper limbs. It is present in 3% of all Guillain-Barré syndrome cases and is characterized by axonal rather than demyelinating neuropathy. We present a pharyngeal-cervical-brachial variant case in a 55-year-old male who presented to the neurological emergency department with a three-day history of progressive and continuous dysarthria, dysphagia to solids, and tongue numbness, later developing paresthesia and weakness in the upper limbs. On physical examination, slight bilateral facial weakness, limited soft palate elevation, absent gag reflex, and limited tongue lateralization were found. Additionally, weakness was found bilaterally in the upper limbs and the flexor and extensor muscles of the neck with preserved muscle strength in the lower limbs. The patient presented upper limb hyporeflexia with lower limb hyperreflexia. A lumbar puncture was performed, revealing protein levels of 35 mg/dL and no cells in the cerebrospinal fluid. Nerve conduction studies reported acute motor and sensory axonal neuropathy (AMSAN). Management of the patient consisted of IgG administration and nasogastric tube insertion.

Apart from the usual differentials of transverse myelitis and cord compression, paraparetic GBS should be considered when sudden, flaccid paralysis of the lower limbs occurs, as prompt diagnosis and management can minimize sequel and unnecessary procedures. We do report a case wherein we managed a similar situation without the use of an immunomodulatory therapy.

Quarenta búfalos foram simultaneamente tratados com clorpirifós e triclorfom na dose recomendada para bovinos. Após uma semana, 19 animais apresentaram sinais clínicos caracterizados por apatia, diarreia, agressividade, desidratação e incoordenação motora, seguidos por paralisia flácida e decúbito lateral permanente. Todos os búfalos afetados morreram após um curso clínico de 1–4 dias. Redução da atividade da colinesterase sérica em três casos foi indicativa de exposição significativa a organofosforados (OPs). O exame patológico de três búfalos não revelou lesões macroscópicas e histológicas. Por cromatografia em camada delgada, resíduos de clorpirifós e traços de resíduos de triclorfon foram detectados em amostras de tecidos frescos. Os achados epidemiológicos, clínicos, patológicos e toxicológicos foram compatíveis com neuropatia tardia induzida por OPs, uma manifestação neurológica raramente descrita em animais domésticos. Forty 1-2-y-old water buffaloes were simultaneously treated with trichlorfon and chlorpyrifos products in the recommended dose for cattle. After a week, 19 animals started presenting clinical signs characterized by apathy, diarrhea, aggressiveness, dehydration, and motor incoordination, followed by flaccid paralysis and permanent lateral recumbency. All affected buffaloes died after a clinical course of 1–4 days. Reduction of serum cholinesterase activity in three cases was indicative of significant exposure to organophosphorus compounds (OPs). Pathological examination of three buffaloes revealed no gross and histological lesions. By thin layer chromatography, chlorpyrifos residues and trace of trichlorfon residues were detected in fresh tissue samples. The epidemiological, clinical, pathological, and toxicological findings were highly compatible with OPs-induced delayed neurotoxicity, a neurological manifestation rarely described in domestic animals.

Aim: Spinal cord injury (SCI)-related flaccid paralysis may result in a debilitating hyperlordosis associated with a progressive hip flexion contracture. The aim of this study was to evaluate the correction of hip flexion contractures and lumbar hyperlordosis in paraplegic patients that had a history of spinal cord injuries.Methods: A retrospective review was performed on 29 hips of 15 consecutive patients who underwent corrective surgeries for severe hip flexion deformity from 2006 to 2018. The mean age at surgery was 10.1 years (2.7 to 15.8), and the mean follow-up was 68 months (7 to 143). Relevant medical, surgical, and postoperative information was collected from the medical records and radiographs.Results: Improvements were seen in the mean hip flexion contracture (p < 0.001) with 100% hip correction at surgery and 92.1% at the latest follow-up. Mean lumbar lordosis decreased (p = 0.029) while the mean Cobb angle increased (p = 0.001) at the latest follow up. Functional score subdomains of the Spinal Cord Independence Measure, Functional Independence Measure, and modified Barthel activities of daily living (ADL) scores remained the same at the final follow-up.Conclusion: For paraplegic SCI patients, we found an association between treating the hip flexion contracture and indirect correction of their lumbar hyperlordosis. We recommend the surgeon carefully examine the hip pathology when managing SCI-related spinal deformities, especially increased lumbar lordosis.

A young lady, a known case of Sjogren’s syndrome, presented to our hospital with hypokalemic flaccid paralysis requiring ventilatory support. She was investigated and found to have severe hypokalemia and metabolic acidosis. During her hospitalization, she had severe persistent carpopedal spasm with normal ionized calcium. Following further evaluation, there was evidence of hypomagnesemia. The spasms were improved after her hypomagnesemia was corrected. This case report illustrate the role of hypomagnesemia in neurological abnormalities in hospitalized patients.

Background. Hypokalemic periodic paralysis is a rare neuromuscular disorder characterized by transient episodes of flaccid paralysis due to a defect in muscle ion channels. Most cases are hereditary, but it can be acquired. We present a case of acquired hypokalemic periodic paralysis associated with hyperthyroidism and renal tubular acidosis. Clinical Case. A 38-year-old female with a history of Graves’ disease presented to the emergency department with generalized weakness and associated nausea, vomiting, and weight loss. Examination was significant for diffuse weakness in all extremities. Labs showed hypokalemia, hyperthyroidism, and nonanion gap metabolic acidosis with a positive urine anion gap. She was treated for hypokalemic periodic paralysis and renal tubular acidosis. Potassium replacement, propranolol, methimazole, and sodium bicarbonate were initiated. Her potassium gradually corrected with resolution of her symptoms. Further investigation revealed a history of dry eyes, dry mouth, and recurrent dental carries. She had positive ANA, SS-A, and SS-B antibodies. She was diagnosed with Sjögren’s syndrome, which may have been associated with her Graves’ disease and thus contributed to both her RTA and hyperthyroidism. Conclusion. Early recognition and treatment of thyrotoxic periodic paralysis are important to prevent cardiac complications. Management includes potassium replacement with careful monitoring to prevent rebound hyperkalemia. The definitive treatment is to achieve euthyroid status.

Background Guillain-Barre syndrome (GBS) is a rare but serious disorder involving peripheral nerve inflammatory demyelination characterized by acute onset tetraparesis and areflexia. Generally, GBS is preceded by a bacterial or viral infection, and post-traumatic or postsurgical GBS is rarely seen. Case Description A 41-year-old man with severe craniocerebral gunshot injury and open depressed occipital bone fracture was operated urgently. Two weeks postoperatively, he suffered from sudden quadriparesis. He had flaccid paralysis of his bilateral muscle lower extremities (0/5), along with bilateral upper extremity weakness (2/5). Conclusions We report the first case, to our knowledge, with post-traumatic GBS after craniocerebral gunshot injury. We want to indicate the possibility of post-traumatic GBS in cases of unexplained quadriparesis or quadriplegia after trauma or surgery.

Hipokalemik periyodik paralizi (HPP), ağır egzersiz, diyet yapma veya yüksek karbonhidratlı yemekler ile tetiklenen ve ağrısız kas güçsüzlüğü ataklarıyla karakterize kaslarda iyon kanallarındaki bir defekt ile ilgili olan nadir görülen bir nöromüsküler bozukluktur. Biz bu olguda ani başlangıçlı güç kaybı şikayetiyle Acil Servis (AS)’e başvuran ve daha öncede benzer şikayetleri olan 37 yaşındaki bir erkek hastayı sunuyoruz. AS’te çalışılan rutin kan testlerinde serum potasyum seviyesi belirgin olarak düşük saptandı. Hipokaleminin diğer nedenleri dışlandı. Hastanın semptomları potasyum yerine koyma tedavisi ile 4 saat gibi kısa bir sürede tamamen düzeldi. Bu olgu, ani flask paralizisi ile başvuran genç hastalarda ayırıcı tanıda nadir olmasına rağmen HPP’nin düşünülmesi gerektiğini ve serum potasyum seviyelerine bakılarak kolayca belirlenebileceğini göstermektedir.

Recurrent GBS is a rare neurological condition in which patients develop similar symptoms of motor weakness after different preceding infections and suffer shorter intervals in between subsequent episodes of GBS. However, the majority of RGBS patients undergo full recovery.

Purpose To describe one institution's experience in the rehabilitation of children with acute flaccid myelitis (AFM). This study reviews the medical and rehabilitative course and functional outcomes of a cohort of children who underwent Activity Based Restorative Therapy (ABRT) at a single center. Methods Children with AFM presenting for rehabilitation between March 2005 and January 2017 were identified and a retrospective chart review was conducted. Changes in medical and functional status were assessed using multiple standardized instruments, as well as a chart review of medical progress. Results Thirty-one children with AFM treated at our institution in the study time period were identified. Of these, seventeen received inpatient treatment, and fourteen received solely outpatient interventions. Their medical and functional outcomes are described with use of standardized measures when available. Conclusions Children with flaccid paralysis due to AFM undergoing structured, comprehensive rehabilitation interventions, even when these are initiated long after paralysis onset, can make significant neurologic and functional gains. Recovery of function and prevention of comorbidities are the main therapeutic targets for interventions in this population.

Hypokalemic periodic paralysis (HypoPP) is a relatively rare but treatable disorder caused by mutations in the CACNA1S gene. HypoPP patients may experience paralytic episodes associated with hypokalemia and, infrequently, may develop late-onset proximal myopathy. The paralytic attacks are characterized by reversible flaccid paralysis and, in most cases, spare the respiratory muscles and heart. We report a case of CACNA1S periodic paralysis precipitated by vigorous exercise in a 14-year-old boy who presented with sudden-onset paralysis of both his upper and lower extremities. Laboratory evaluation revealed a markedly low serum potassium level. The patient’s symptoms resolved after correction of the potassium abnormality, and he was discharged with no neurological deficits. Although rare, HypoPP must be differentiated from other causes of weakness and paralysis so that proper treatment can be promptly initiated to ensure good outcomes.