Acute Onset Of Neurological Deficits Research Articles

Clinicopathological and pedigree investigation of a novel spinocerebellar neurological disease in juvenile Quarter Horses in North America.

In 2020, a novel neurologic disease was observed in juvenile Quarter Horses (QHs) in North America. It was unknown if this was an aberrant manifestation of another previously described neurological disorder in foals, such as equine neuroaxonal dystrophy/equine degenerative myeloencephalopathy (eNAD/EDM). To describe the clinical findings, outcomes, and postmortem changes with Equine Juvenile Spinocerebellar Ataxia (EJSCA), differentiate the disease from other similar neurological disorders, and determine a mode of inheritance. Twelve neurologically affected QH foals and the dams. Genomic DNA was isolated and pedigrees were manually constructed. All foals (n = 12/12) had a history of acute onset of neurological deficits with no history of trauma. Neurological deficits were characterized by asymmetrical spinal ataxia, with pelvic limbs more severely affected than thoracic limbs. Clinicopathological abnormalities included high serum activity of gamma-glutamyl transferase and hyperglycemia. All foals became recumbent (median, 3 days: [0-18 days]), which necessitated humane euthanasia (n = 11/12, 92%; the remaining case was found dead). Histological evaluation at postmortem revealed dilated myelin sheaths and digestion chambers within the spinal cord, most prominently in the dorsal spinocerebellar tracts. Pedigree analysis revealed a likely autosomal recessive mode of inheritance. EJSCA is a uniformly fatal, rapidly progressive, likely autosomal recessive neurological disease of QHs <1 month of age in North America that is etiologically distinct from other clinically similar neurological disorders. Once the causative variant for EJSCA is validated, carriers can be identified through genetic testing to inform breeding decisions.

AN UNFORESEEN CATASTROPHIC CONSEQUENCE OF ENOXAPARIN: A RARE CASE OF ACUTE QUADRIPLEGIA AND APHASIA SECONDARY TO SPINAL EPIDURAL HEMATOMA

SESSION TITLE: Medical Student/Resident Critical Care Posters SESSION TYPE: Med Student/Res Case Rep Postr PRESENTED ON: October 18-21, 2020 INTRODUCTION: Spontaneous spinal epidural hematomas (SSEH) are a rare but catastrophic consequence of anticoagulation that causes an acute onset of neurological deficits. Here we present the first case of a patient presenting with quadriplegia and aphasia due to SSEH. CASE PRESENTATION: A 60 year-old African-American woman with a history significant for hypertension and prior DVT was admitted after a syncopal event. On presentation, she was found to be in respiratory distress with hypotension due to massive pulmonary embolism accompanied by lower extremity DVT. She required tissue plasminogen activator (tPA) administration followed by therapeutic anticoagulation with enoxaparin. On day 3 of hospitalization she acutely developed quadriparesis and aphasia. An MRI of the cervical spine showed fluid in the posterior paraspinal musculature from the midline skull base to C5 and in the right posterior paraspinal musculature from C7 into the upper thoracic spine with moderate cord compression. These findings were consistent with a spontaneous spinal epidural hematoma. The patient underwent urgent temporary IVC filter placement. Anticoagulation was reversed with protamine sulfate and the patient was taken to the OR for hematoma evacuation and cervical spine decompression. Post-intervention, the patient had a full neurological recovery, and was discharged without complications on post operative day 7. DISCUSSION: SSEH is a rare entity that if not diagnosed and treated promptly can lead to detrimental outcomes including death. It is characterized by an accumulation of blood in the vertebral epidural space that compresses the spinal cord or spinal nerve roots. It is typically attributed to coagulopathies and arteriovenous malformations. Standard therapy is decompressive laminectomy, with only 50% of patients exhibiting full neurological recovery. To our knowledge, this is the first case of a patient presenting with quadriplegia and aphasia due to a spontaneous cervical spine epidural hematoma from anticoagulation. The added complexity of this patient, in which she also had a lower extremity DVT that required IVC filter placement and reversal of her anticoagulation for surgical decompression, adds to the distinctiveness of this case. CONCLUSIONS: Due to the broad nature of clinical presentation of SSEH, ranging from severe neck pain to aphasia and quadriplegia, it is of utmost importance for clinicians to quickly diagnose and treat this devastating condition. After identification, resources and care teams must rapidly be mobilized in order for evacuation and decompression. In patients who experience this rare disorder due to therapeutic anticoagulation with enoxaparin and who have a deep venous thrombosis, rapid placement of IVC filter followed by protamine reversal and emergent laminectomy and decompression within 12 hours of symptom onset can potentially allow full recovery of neurological deficits. Reference #1: Spinal epidural hematoma. J Am Acad Orhop Surg. 2010 Aug; 18(8):494-502. Reference #2: Spontaneous Spinal Epidural Hematoma in a Patient on Rivaroxaban: Case Report and Literature Review. J Emerg Med. 2017 Oct;53(4):536-539. Reference #3: Spontaneous spinal epidural hematoma: the importance of preoperative neurological status and rapid intervention. J Clin Neurosci. 2015; 22(1) 123 DISCLOSURES: No relevant relationships by Shahrzad Abdollahi, source=Admin input No relevant relationships by Hadiatou Barry, source=Web Response no disclosure on file for Jennifer Frabizzio; No relevant relationships by Rajesh Patel, source=Web Response No relevant relationships by Andrew Tran, source=Web Response

Nomogram to predict risk for early ischemic stroke by non-invasive method.

Stroke is the acute onset of neurological deficits and is associated with high morbidity, mortality, and disease burden. In the present study, we aimed to develop a scientific, nomogram for non-invasive predicting risk for early ischemic stroke, in order to improve stroke prevention efforts among high-risk groups. Data were obtained from a total of 2151 patients with early ischemic stroke from October 2017 to September 2018 and from 1527 healthy controls. Risk factors were examined using logistic regression analyses. Nomogram and receiver operating characteristic (ROC) curves were drawn, cutoff values were established. Significant risk factors for early ischemic stroke included age, sex, blood pressure, history of diabetes, history of genetic, history of coronary heart disease, history of smoking. A nomogram predicting ischemic stroke for all patients had an internally validated concordance index of 0.911. The area under the ROC curve for the logistic regression model was 0.782 (95% confidence interval [CI]: 0.766–0.799, P < .001), with a cutoff value of 2.5. The nomogram developed in this study can be used as a primary non-invasive prevention tool for early ischemic stroke and is expected to provide data support for the revision of current guidelines.

Ligamentum Flavum Cyst With Acute Onset Motor Deficit: A Literature Review and Case Series.

Ligamentum flavum cysts have been rarely described in the literature and are one of the rare causes of neural compression and canal stenosis. Very few cases of their association with neurologic deficits are reported to date, and association with acute onset weakness is even rarer. We report our experience with 3 cases of ligamentum flavum cyst that presented with acute onset weakness and also present a comprehensive literature review on lumbar ligamentum flavum cysts reported to date. All 3 patients had symptoms of severe neurogenic claudication and presented to us with acute onset of motor weakness in lower limbs. Ligamentum flavum cyst was located in the midline in 2 cases and laterally in 1 case. We performed excision of the cyst and decompression with fusion in 2 cases and decompression alone in 1 case. All 3 cases had significant improvement in their neurologic status postoperatively. Histopathological examination confirmed ligamentum flavum cyst in all 3 cases. We performed a PUBMED and EMBASE database search using the MeSH (Medical Subject Headings) terms "ligamentum flavum" and "cysts" for articles published to April 2019. We could identify 7 studies describing 20 cases of lumbar ligamentum flavum cysts with motor weakness in the literature. Only 1 case had been described with an acute onset of weakness. Ligamentum flavum cysts should remain in the differential diagnosis of a patient who has symptoms of lumbar canal stenosis and presents with acute onset of neurological deficits. Such patients have a good improvement with surgery.

MELAS: Mitochondrial Encephalomyopathy, Lactic Acidosis and Stroke-Like Episodes

Mitochondrial disease is caused by a deficiency in the energy supply to cells due to mitochondrial dysfunction. Mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) is a mitochondrial disease that presents with stroke-like episodes such as acute onset of neurological deficits and characteristic imaging findings. Stroke-like episodes in MELAS have the following features: 1) neurological deficits due to localization of lesions in the brain, 2) episodes often accompany epilepsy, 3) lesions do not follow the vascular supply area, 4) lesions are more often seen in the posterior brain than in the anterior brain, 5) lesions spread to an adjacent area in the brain, and 6) neurological symptoms often disappear together with imaging findings, but later relapse. About 80% of patients with MELAS have an A-to-G transition mutation at the nucleotide pair 3243 in the dihydrouridine loop of mitochondrial tRNA^Leu(UUR), which causes the absence of posttranscriptional taurine modification at the wobble nucleotide of mitochondrial tRNA^Leu(UUR) and disrupts protein synthesis. However, the precise pathophysiology of stroke-like episodes is under investigation, with possible hypotheses for these episodes including mitochondrial angiopathy, mitochondrial cytopathy, and neuron-astrocyte uncoupling. With regard to treatment, L-arginine and taurine have recently been suggested for relief of clinical symptoms.

Prevalence of Significant Carotid Stenosis and Other Risk Factors in Patients with Acute Ischemic Stroke in Yaounde, Cameroon

The prevalence and incidence of cardiovascular disease have been increasing in sub Saharan Africa for the last past twenty years. One of the most common manifestations of cardiovascular disease is stroke. Very few studies have addressed the major causes of ischemic stroke in this region. The objective of this study was to determine the prevalence of significant extra cranial carotid stenosis in a population of patients with acute ischemic stroke. Patients and methods: we conducted a prospective study at the University Hospital Centre of Yaounde from January 2013 to October 2013. We included all consenting patients with acute onset of neurological deficit with a CT scan of the head showing an ischemic stroke. We excluded all patients with hemorrhagic stroke on CT scan, or who did not have a head CT scan. The study was approved by the National ethics committee. Results: During that period, 35 patients met the criteria for acute ischemic stroke from which 51.4% were women with a sex ratio of 0.94. The mean age in this series was 66.6 years. Thirty five patients over 76 had an ischemic stroke on the CT scan, with a prevalence of 46%. Hypertension was present in 21 (60%) cases and it was a major risk factor. Only 3 patients had a carotid stenosis between 50-75%, there was no patient with a stenosis above 75%. Elevated cholesterol was found in 3.3% of patients while 30 (86%) patients had an abnormal electrocardiogram: 9 patients were found to have a tachyarrhythmia and 6 atrial fibrillation. There was no intracardiac clot even though the echocardiogram was abnormal in 26 (77%) patients. Concerning the CT scan of the head results, hypo densities were found in 26 (74.3%) patients and the CT was normal in 9 (9.9%) patients with a sudden neurological deficit. Conclusion: From this pilot study, we have found that carotid stenosis in association with hypercholesterolemia and arteriosclerosis may not be a major risk factor for ischemic stroke in our environment, while age, hypertension and arrhythmia are the most important risk factors for this disease in Cameroon. Further and larger study is needed to confirm this preliminary finding.

Early Diagnosis of Spontaneous Spinal Epidural Hematoma with Echo-Planar Gradient-Echo T2*-Weighted MR Imaging

Spontaneous spinal epidural hematoma (SSEH) is a rare idiopathic condition that leads to the acute onset of neurological deficits, which can have catastrophic consequences if not recognized early. It is important to make an early precise diagnosis. Spinal epidural hematoma has been increasingly recognized since the advent of magnetic resonance imaging (MRI). However, T1- and T2-weighted gradient-echo sequences are relatively less sensitive to the magnetic susceptibility effects of hemorrhage. Echo-planar gradient-echo T2*-weighted MR imaging (T2* MRI) is sensitive to these magnetic susceptibility effects and is commonly used for the detection of hemorrhage. We reported that the case of a 76-year-old man who presented with tetra paresis had an early diagnosis of spontaneous spinal epidural hematoma early diagnosed by T2* MRI.

Demyelinating disease masquerading as a surgical problem: a case series.

IntroductionWe report three cases of demyelinating disease with tumor-like presentation. This information is particularly important to both neurosurgeons and neurologists who should be aware that inflammatory demyelinating diseases can present as a mass lesion, which is indistinguishable from a tumor, both clinically and radiologically, especially when there is no evidence of temporal dissemination of this disease.Case presentationThe first patient was a 42-year-old Malay woman who developed subacute onset of progressive quadriparesis with urinary incontinence. Magnetic resonance imaging of her spine showed an intramedullary lesion at the C5-C7 level. She was operated on and biopsy was suggestive of a demyelinating disease. Retrospective history discovered two episodes of acute onset of neurological deficits with partial recovery and magnetic resonance imaging of her brain revealed demyelinating plaques in the centrum semiovale.The second patient was a 16-year-old Malay boy who presented with symptoms of raised intracranial pressure. A computed tomography brain scan revealed obstructive hydrocephalus with a lesion adjacent to the fourth ventricle. An external ventricular drainage was inserted. Subsequently, a stereotactic biopsy was taken and histopathology was reported as demyelination. Retrospective history revealed similar episodes with full recovery in between episodes.The third case was a 28-year-old Malay man who presented with acute bilateral visual loss and confusion. Magnetic resonance imaging of his brain showed a large mass lesion in the right temporoparietal region. Biopsy was consistent with demyelinating disease. Reexamination of the patient revealed bilateral papillitis and not papilledema. Visual evoked potential was prolonged bilaterally. In all three cases, lumbar puncture for cerebrospinal fluid study was not carried out due to lack of patient consent.ConclusionsThese cases illustrate the importance of considering a demyelinating disease in the differential diagnosis of a mass lesion. Critical analyses of clinical presentations coupled with good physical examination are vital in assisting clinicians to reach the correct diagnosis.

Acute hemorrhagic leukoencephalitis (Hurst disease): A case report and review of recent literature

Acute hemorrhagic leukoencephalitis (AHLE) is a rare fulminant demyelinating disorder. It is considered to be the most severe form of acute disseminated encephalomyelitis. It is associated with a high rate of mortality or severe neurological deficits, with only few reports of recovering patients. We report a 49-year-old female patient who presented with acute onset of neurological deficits, due to a right thalamic hemorrhage together with intraventricular bleeding. There was a continued clinical deterioration. In follow-up CT and MRI scans bilateral confluent white matter lesions were seen, together with T2 * hypointense lesions indicating microbleeds. A stereotactic biopsy was carried out and the diagnosis of AHLE was suggested. In acute post infectious encephalitides (and especially the severe form) early diagnosis, administration of treatment is of prime importance for outcome in a patient with Hurst encephalitis.

Vascular Encephalopathy Associated With Bacterial Endocarditis in Four Dogs

Vascular encephalopathy is a commonly encountered clinical complication of infectious endocarditis in humans, but it has been infrequently reported in dogs. A series of four dogs with bacterial endocarditis that subsequently developed acute onset of neurological deficits is described. Clinical signs, diagnostic test results, and outcomes for each case are presented and compared with the human clinical syndrome.

Epidermoid cyst of the sphenoid sinus with extension into the sella turcica presenting as pituitary apoplexy: case report

Background Epidermoids of the central nervous system are rare tumors. They are usually found in the fourth decade of life and most commonly off midline in the cerebellopontine angle. We report here a rare case of an epidermoid arising from the sphenoid sinus with extension into the sella and adjacent structures with acute onset of neurological deficit. The significance of the clinical presentation resembling pituitary apoplexy and magnetic resonance imaging (MRI) findings is noted. Case Description A 25-year-old man presented with acute severe headache, diplopia, and decreased visual acuity. Examination revealed right-sided ptosis and paresis of the third cranial nerve on the right side. Computed tomography and MRI were suggestive of a slow-growing sphenoid sinus mass with extension into the sella. The sublabial transsphenoidal approach was used to remove the mass under direct visualization. The patient's neurological status improved to baseline both subjectively and objectively after the operation. Conclusion Epidermoids, although rare, should be considered as part of the differential diagnosis when evaluating lesions of sphenoid sinus or sellar origin.

Arterial dissection and stroke following child abuse: case report and review of the literature

Arterial dissection and stroke following trauma are seen frequently. Although similar mechanisms occur for trauma and child abuse, no reports of stroke have been observed in the literature. Two patients successively presented to our institution with acute onset of neurological deficit and stroke. Further evaluation disclosed child abuse as an underlying condition leading to the strokes. Child protection services were contacted immediately after admission of the patients and further measures were taken. Recognition of potential cases of child abuse is crucial. Early intervention and cessation of interaction between the aggressor and the child are important measures to be taken in all situations when there is strong evidence of suggestive examination of child abuse.