Concomitant spinal dural arteriovenous fistula and nodular fasciitis in an adolescent: case report

Background Spinal dural arteriovenous fistula (SDAVF) usually occurs during the 4th to 6th decades of life, and adolescent SDAVF is rarely reported. SDAVF arising around a tumor is also rare, and reported tumors are mostly schwannoma and lipoma. Case presentation We reported a 16-year-old male presented with progressive weakness and numbness of lower limbs for 3 months. A SDAVF was found, which was fed by right radicular arteries from segmental artery at L2 level and drained retrogradely into perimedullary veins. A concomitant spinal extradural nodular fasciitis at right L1/L2 intervertebral foramen was also noted. The SDAVF was completely obliterated by endovascular treatment and the tumor was debulked. The patient recovered well after the procedures. Conclusions Our case report suggests SDAVF can occur in adolescent. The concomitant presence with a nodular fasciitis indicates that although it usually arises in subcutaneous tissue but can rarely form on the dura of spine.

Guillain-Barré syndrome associated with COVID- 19 vaccination: a case report

Several reports and studies are being conducted to this day based on the safety profile of COVID-19 vaccines. COVID-19 vaccination inducing GBS is a rare adverse effect and is likely to be causal. Though, there are reports concerning the relation between coronavirus infections and GBS, the pathogenic mechanism and relevant factors behind COVID-19 vaccines inducing GBS are still not being corroborated so far. Guillain-Barre syndrome is the principal cause of acute flaccid paralysis with a prevalence rate of 2 in 100, 000 people per year. We illustrate a 55 years old female patient who presented with acute onset paraesthesia and progressive weakness of bilateral lower limbs and gait imbalance of 5 days duration to the Hospital during the first week of September. Her symptoms occurred within 2 weeks of the first dose of the ChAdOx1-n-CoV-19 (Covishield) vaccine proving a major possibility of vaccine-induced neurological adverse effect as she didn't have any likely significant history of illness or allergies in the past rather than type 2 diabetes mellitus. This report aims to highlight the incidence and to ruminate upon this matter while evaluating any GBS cases in the current eras of the COVID-19 pandemic and vaccination.

The Bilateral Sacral Origin of a Spinal Dural Arteriovenous Fistula: A Case Report

: Spinal dural arteriovenous fistulas (SDAVFs) are characterized by an abnormal connection between a spinal radicular artery and a perimedullary vein, mainly fed by a radicular artery at the nerve root sleeve. Here, we describe the case of a 40-year-old woman, presenting with progressive weakness of the lower extremities and the sphincter. Thoracic magnetic resonance imaging (MRI) showed spinal cord edema and signal voids on the dorsal surface of the cord. Spinal angiography demonstrated a SDAVF with a nidus at the sacral level; the feeder of the arteriovenous fistula was a lateral sacral artery, as a branch of the internal iliac artery. The lateral sacral artery was subselectively catheterized, and SDAVF was embolized with 25% n-butyl cyanoacrylate (NBCA) glue (glue: lipiodol ratio, 1:3). After embolization, no definite residual connection was visualized between the arterial and venous systems.

A Complex Phenotype of a Patient with Spastic Paraplegia Type 4 Caused by a Novel Pathogenic Variant in the SPAST Gene

Hereditary spastic paraplegias (HSPs) are rare neurological disorders caused by degeneration of the corticospinal tract. Among the 79 causative genes involved in HSPs, variants in <i>SPAST</i> on chromosome 2p22, which encodes the microtubule-severing protein spastin, are responsible for spastic paraplegia type 4 (SPG4), the most common form of HSPs. SPG4 is characterized by a clinically pure phenotype that is associated with restricted involvement of the corticospinal tract; however, it is often accompanied by additional neurological symptoms such as epilepsy and cognitive impairment. There are few reports regarding the clinical course and treatment of epilepsy associated with SPG4. We describe a 21-year-old male patient with progressive weakness and spasticity of the lower limbs since infancy, which was complicated by epilepsy and cognitive impairment. Magnetic resonance imaging of the brain showed right hippocampal atrophy before the onset of epilepsy. Genetic analysis revealed a novel missense variant (NM_014946.4:c.1330G&#x3e;C, p.Asp444His) in the <i>SPAST</i> gene. At the age of 13, the patient developed focal epilepsy, characterized by focal onset seizures that were preceded by a sensation of chest tightness. Carbamazepine, levetiracetam, and zonisamide were ineffective in controlling the seizures; however, the use of lacosamide in combination with lamotrigine and valproate was highly effective in improving the seizure symptoms and led to the patient being seizure free for at least 2 years. In conclusion, the missense variant in <i>SPAST</i> may cause a complex SPG4 phenotype accompanied by epilepsy and cognitive impairment, suggesting that the clinical manifestations of this condition do not confine to the motor system.

Characteristics of COVID and post COVID polyneuropathies in adults and pediatrics: an Egyptian sample

Background The aim of this study is to describe the different forms of polyneuropathy associated with coronavirus disease 2019 (COVID-19) as a secondary neurological complication for (COVID-19) and the outcome from different therapeutic regimens in adults and pediatrics in first and second waves of the pandemic. Case presentation This study was conducted on 42 patients, they were divided into two groups, group (A) and group (B) in first and second waves respectively. Twenty-five patients presented by ascending weakness preceded by fever, dry cough and respiratory distress, electromyography (EMG) and nerve conduction (NC) studies done and confirmed the clinical diagnosis of demyelinating polyneuropathy. Eight patients presented by acute flaccid quadriparesis, more severe in upper limbs preceded by fever and diarrhea diagnosed as acute axonal polyneuropathy. Five patients presented by severe fatigue and progressive weakness of both lower and upper limbs, they developed fever and cough 10 days after the neurological symptoms. EMG and NC done and confirmed clinical diagnosis of polyneuropathy of demyelinating with secondary axonal picture. Four patients presented 30 to 40 days after their recovery form corona virus infection with gradual progressive weakness of both upper and lower limbs over 2 to 3 months duration, mainly the proximal muscles of lower limbs were affected with areflexia. EMG and NC done and confirmed the diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Conclusion We should gain a better understanding of the underlying pathophysiology and therapeutic options of polyneuropathies related to COVID-19, which will have an impact on the treatment of the COVID related respiratory failure presenting with neuropathy.

Nitrous Oxide Poisoning Complicated by Syringomyelia: a Case Report

Background: Nitrous oxide is a colorless, aromatic, inert organic gas with low toxicity and is currently abused in many recreational areas.Nitrous oxide abuse can lead to vitamin B12 deficiency, which can lead to neurological damage such as spinal neuropathy and peripheral neuropathy. Its abuse has not been associated with syringomyelia.Case presentation: We described the case of a 21-year-old young girl who presented with progressive weakness in both legs and numbness in four limbs after excessive recreational inhalation of nitrous oxide and her imaging findings were considered syringomyelia.Conclusion: Nitrous oxide abuse may be associated with syringomyelia, and that patients with nitrous oxide abuse should be further performed cervical and thoracic MRI to determine whether they have syringomyelia and give timely treatment.

Sensitivity to change and responsiveness of lowering to the ground and rising from the ground evaluation in Duchenne muscular dystrophy: one-year longitudinal observation

BACKGROUND: The progressive weakness of Duchenne muscular dystrophy (DMD) interferes with performance. This study investigated the sensitivity to change and the responsiveness of sitting and standing from the ground in patients with DMD. AIM: The aim was to assess the sensitivity to change and the responsiveness of lowering to/ rising from the ground, in three, six, nine, and twelve month-evaluation intervals and to define the most suitable reevaluation intervals for ambulatory patients with DMD. METHOD: This is an observational, longitudinal study. Recordings of 28 patients performing lowering to/ rising from the ground were analyzed. Sensitivity to change was assessed using effect sizes and standardized response means. Responsiveness was assessed using minimal detectable changes (MDC) and minimal clinically important differences (MCID). RESULTS: In the lowering to the ground, significant sensitivity to change was found in higher than 6 months reassessment intervals. In the rising from the ground, significant sensitivity to change was observed in higher than 9 reassessment intervals. MDC and MCID varied from 1.0 to 1.6 points and from 0.5 to 2.5 seconds when lowering to the ground and from 1.3 to 2.6 points and from 5.0 to 28.0 seconds when rising from the ground. CONCLUSION: Patients should be reassessed after nine months from the lowering to and rising from the ground. Increments of 2.0 points and/or 2.5 seconds (or higher) in the score of lowering to the ground assessment denote clinically relevant changes. Increments of 3 points (or higher) in rising from the ground assessment are clinically relevant.

A 52-Year-Old Man With Progressive Weakness and Incontinence

Here we report a challenging case of a 52-year-old man presenting with subacute constipation, urinary retention, impotence, absent Achilles reflexes, and hypoesthesia in S2-S5 dermatomes. We review the clinical decision-making as the symptoms evolved and diagnostic testing changed over time. Once the diagnosis is settled, we discuss the sign and symptoms, additional diagnostic tools, treatment options and prognosis.

Successful Management of Parsonage–Turner Syndrome with Steroids in the Post-acute Weakness Phase: A Case Report

Introduction: Parsonage–Turner Syndrome (PTS) is a rare disease of the brachial plexus of unclear aetiology. The limited data available typically describes involvement of branches of brachial nerves. The authors present a case of PTS with a rare combination of unilateral brachial plexus, phrenic nerve, and recurrent laryngeal nerve injuries. They also highlight successful treatment with pharmacological intervention despite several months’ delay in diagnosis. The 35-year-old female presented with acute onset of severe left shoulder pain followed by severe progressive weakness of the left shoulder muscles, progressive weakness of her voice, nasal regurgitation of fluids, paroxysmal bouts of coughing, and exertional dyspnoea at rest. The symptoms remained undiagnosed for about 10 months. A clinical diagnosis of exclusion of PTS was finally made, and treatment with steroids, neurotropic drugs, and physiotherapy was started. The patient has recovered significantly since then and continues to improve. Conclusion: The authors presented a case of PTS with a rare combination of brachial plexus, recurrent laryngeal nerve, and phrenic nerve injuries. This case was also remarkable for the significant improvement in her symptoms with treatment, despite the delay in diagnosis. This bears evidence that steroids and adjuvant therapy is useful even months after onset of the disease.