Rare Neurological Complication Research Articles

Bing-Neel Syndrome – A Challenging Diagnosis: Case Report

Bing-Neel syndrome is a rare neurologic complication of Waldenström’s macroglobulinemia, characterized by infiltration of the central nervous system by clonal lymphoplasmacytes. We present a rare clinical case of a patient, who one year after the diagnosis of Waldenström’s macroglobulinemia, progressed with diverse neurologic presentation and cerebrospinal fluid involvement. The diagnosis was based on magnetic resonance imaging and flow cytometry detection of clonal B-cells in the cerebrospinal fluid. Bing-Neel syndrome should be considered in patients with neurologic symptoms and a history of Waldenström’s macroglobulinemia.

Neurological Complications of SARS-CoV-2 Infection in Children: A Systematic Review and Meta-Analysis

BackgroundKnowledge about neurological complications of COVID-19 in children is limited due to the paucity of data in the existing literature. Some systematic reviews are available describing overall clinical features of COVID-19 in children and neurological complications of COVID-19 in adults. But to the best of our knowledge, no systematic review has been performed to determine neurological manifestations of COVID-19.MethodsSix different electronic databases (MEDLINE, EMBASE, Web of Science, CENTRAL, medRxiv and bioRxiv) were searched for articles related to COVID-19 and neurological complications in children. Studies/case series reporting neurological manifestations of COVID-19 in patients aged ≤18 years, as well as case reports, as neurological complications appear to be rare. The pooled estimate of various non-specific and specific neurological manifestations was performed using a random effect meta-analysis.ResultsTwenty-one studies/case series and five case reports (3707 patients) fulfilled the eligibility criteria and were included in this systematic review, from a total of 460 records. Headache, myalgia and fatigue were predominant non-specific neurological manifestations, presenting altogether in 16.7% cases. Total of 42 children (1%) were found to have been reported with definite neurological complications, more in those suffering from a severe illness (encephalopathy—25, seizure—12, meningeal signs—17). Rare neurological complications were intracranial hemorrhage, cranial nerve palsy, Guillain–Barré syndrome and vision problems. All children with acute symptomatic seizures survived suggesting a favorable short-term prognosis.ConclusionNeurological complications are rare in children suffering from COVID-19. Still, these children are at risk of developing seizures and encephalopathy, more in those suffering from severe illness.

Multiple Cerebral and Cerebellar Infarctions following Russell's Viper (Daboia Russelii) Envenomation - A Case Report

Ischemic stroke is a rare neurological complication following SriLankan Russell's viper envenomation. We report a case of a 44-year-old person, who developed multiple cerebral and cerebellar infarctions following SriLankan Russell's viper envenomation. The patient developed both local and systemic envenomation and was treated with Snake Venom Anti-Serum I.P. (VinsBioproducts Ltd). On day six, this patient developed left-sided hemiparesis. CT scan of the brain showed bilateral cerebellar, bilateral occipital, and right-sideparietal lobe infarctions.

Rare Neurological Complication Due to Air Embolism Facilitated by Prone Position During PCNL Surgery and its Successful Management

Rare Neurological Complication Due to Air Embolism Facilitated by Prone Position During PCNL Surgery and its Successful Management

Respiratory Distress in SARS-CoV-2 without Lung Damage: Phrenic Paralysis Should Be Considered in COVID-19 Infection.

We describe an overweight COVID-19 patient with respiratory distress preceded by anosmia/dysgeusia with no lung injury shown on CT, angio-CT or ventilation/perfusion scans. Orthopnoea and paradoxical abdominal respiration were identified. Phrenic paralysis, demonstrated by examination of patient breathing, and on x-ray while standing breathing in and out, explained the respiratory distress. This is a rare and previously undescribed neurological complication of COVID-19 infection caused by vagus nerve injury.LEARNING POINTSPhrenic paralysis must be kept in mind as a rare neurological complication of COVID-19.Vagus nerve palsy is a neurological manifestation as anosmia and dysgeusia, that were already identified in the olfactory system of COVID-19 patients.

Perioperative Complications and Clinical Outcomes in Patients with Congenital Cyanotic Heart Disease Undergoing Surgery for Brain Abscess.

Background Brain abscess is a rare neurological complication in patients with congenital cyanotic heart disease (CCHD). Perioperative complications are high in patients with CCHD. We evaluated incidence of and risk factors for perioperative complications and their impact on clinical outcomes in patients with CCHD undergoing brain abscess surgery with monitored anesthesia care (MAC) or general anesthesia (GA). Methods In this single-center retrospective cohort study, data were collected from consecutive patients with CCHD who presented with brain abscess and underwent surgery from January 2006 to December 2018. Data regarding demographics, type of CCHD, signs and symptoms of brain abscess and CCHD, type and duration of surgery, details of anesthesia, perioperative complications, and clinical outcomes were collected. Chi-square test was used to analyze nonparametric data and student t -test for parametric data. Results Of the 402 patients with brain abscess, data of 34 patients with CCHD who underwent brain abscess surgery were analyzed. The mean age at presentation of brain abscess was 15.8 ± 10.8 years and duration of symptoms was 17.3 ± 15.5 days. The incidence of perioperative complications was 82.4% (28/34 patients). Seven patients (20.6%) developed perioperative cyanotic spells which led to cardiac arrest in 5 patients (14.7%) and death in 2 patients (5.9%). Patients on cardiac medications and with high heart rate had higher incidence of cyanotic spells and mortality. Technique of anesthesia did not affect cardiac and neurological outcome. Conclusions Perioperative complications are high after brain abscess surgery in patients with CCHD. Perioperative characteristics and outcomes were similar with MAC and GA techniques.

Subsultus Tendinum in a Child with Typhoid Fever

A 5-year-old male child with blood culture confirmed typhoid fever presented with twitching over the left scapular region. Contrast computerized tomography and electroencephalogram were normal. Following treatment with azithromycin and clonazepam, the twitching subsided. Subsultus tendinum, a rare neurological complication of typhoid fever, resolves spontaneously with treatment.

Posterior Reversible Encephalopathy Syndrome – A Rare Complication after Methylprednisolone

Posterior reversible encephalopathy syndrome (PRES) is a rare neurological complication. It can occur as a complication of hypertensive disease of pregnancy, autoimmune disorder or after immunosuppressant. Typical imaging features of PRES are bilateral symmetrical involvement of parietal and occipital lobe. Here, we reported a case of PRES after methylprednisolone.
 Bangladesh J Medicine Jan 2020; 31(1) : 41-42

Stroke in critical COVID-19 patients: a cautionary tale from the frontlines.

IntroductionAlthough Coronavirus Disease 2019 (COVID-19) is primarily a disease of the respiratory system in its transmission and clinical manifestations, physicians have also reported a tropism toward the nervous system.MethodsNeurological symptoms can occur as one of many systemic manifestations of a critical form of the disease or in isolation as the predominant presenting complaint.ResultsWe report a series of 6 patients who suffered significant cerebrovascular accidents while being treated for critical COVID-19 in the intensive care units of a quaternary care hospital in New York’s Hudson valley.ConclusionsThis series demonstrates how a relatively rare but catastrophic neurological complication can occur in patients with COVID-19 while they are being managed for their more common problems such as respiratory and renal failure.

Generation of retractive spine circuits in the process of vitamin B12 deficiency

Introduction. Subacute degeneration of the posterior and lateral spinal cord is a rare neurological complication of B12 avitaminosis. Aim. In this paper, we present the case of a 65-year-old man who, in the course of long-term vitamin B12 deficiency associated with atrophic gastritis, developed a severe set of neurological symptoms that are part of the retinal spinal cord degeneration with characteristic features in MR imaging of the cervical spine in the form of an inverted “V mark” ‘in axial images and typical localization in the spinal cord. Description of the case. After careful analysis of the syndrome and making an early diagnosis, parenteral vitamin B12 supplementation began, resulting in improved neurological status, laboratory parameters as well as regression of changes in magnetic resonance imaging. Conclusion Significant symptoms of this syndrome are paresthesia in the distal parts of the limbs, ataxia, spastic paresis.

SARS-CoV-2-Associated Acute Transverse Myelitis in Panama with Analysis of 38 Cases Reported Worldwide During COVID-19

Background: Acute transverse myelitis (ATM) is a rare neurological complication of viral infections and vaccinations. We report a 72-year-old man with SARS-CoV-2-associated cervicothoracic ATM in Panama, and analyze the results of a bibliographic investigation on myelitis during COVID-19.Methods: Systematic review of 38 patients with COVID-19-associated myelitis from 19 countries published between March and October 2020.Findings: All patients had typical features of ATM with acute onset of paralysis, sensory level, and sphincter deficits due to spinal cord lesions demonstrated by imaging. There were 21 men (55%) and 17 women (age range, 21-73 years; mean age, 49 years) with two age peaks at 29 and 58 years, excluding two pediatric cases. Longitudinally-extensive ATM involved cervicothoracic segments in 14 cases (42%), localized ATM lesions affected cervical (10 cases) and thoracic cord segments (7 cases). Acute disseminated encephalomyelitis (ADEM) occurred in 7 patients, mainly women (67%) with ages ranging from 27 to 64 years. Two patients had blindness due to myeloneuritis optica, and 2 had acute motor axonal neuropathy.Interpretation: Acute myelitis is an unexpectedly frequent neurological complication of COVID-19. Short latency from onset of infection to first neurological symptoms may indicate a direct neurotropic effect of SARS-CoV-2 during the initial infection, while longer latency of the post-infectious neurological complications may be mediated by the host’s response to the virus. The immune mechanisms causing ATM remain unknown. It is conceivable that common antigens inducing ATM may occur in both SARS-CoV-2 and in the ChAdOx1 nCoV-19 Chimpanzee adenovirus vaccine. (ClinicalTrials.gov Identifier: NCT04324606). Funding: None.Declaration of Interests: The authors declare no competing interests.

Posterior reversible encephalopathy syndrome after massive transfusion in a patient with postpartum vaginal hematoma

Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiological syndrome characterized by headache, potentially reversible neurological deficits. PRES has been described in various clinical settings, such as severe hypertension, eclampsia, and chemotherapy. Transfusion related PRES are rare. We report a patient with postpartum vaginal hematoma who manifested PRES after massive packed red blood cell transfusion confirmed by brain MRI and clinical features. A 34-year-old woman gravida 1, para 1 admitted for surgery of postpartum vaginal hematoma, rectum repair, sphincter repair. Twelve hours ago, she was delivered. After delivery her uterine contraction was poor and vaginal bleeding was persistent. On admission, her hematocrit was at 27.2%, and her hemoglobin was at 9.0 g/dl. She received 18 packed red blood cell transfusion and 10 packed fresh frozen plasma. Her hematocrit and hemoglobin increased 41.1% and 13.9 g/dl, and platelet increased up to 78,000/ul. After 2 days of the transfusion, she had a left eye ball deviation and 5 min prolonged confusion. Her blood pressure was normal, and no other contributing factors for PRES were found. Brain MRI showed regional T2 signal change in right occipital lobe including cortex, and small signal change in left occipital lobe. In this case, some relation may have existed between late postpartum eclampsia and PRES, but the pathogenesis remains unclear. Although blood transfusion is a common procedure with rare neurological complications, great caution should be taken with anemic patients because a rapid elevation in hemoglobin may precipitate PRES.

MON-137 A Case of Diabetic Striatopathy Initially Diagnosed as Intracerebral Hemorrhage

Diabetic striatopathy is a rare neurologic complication of uncontrolled diabetes, specifically associated with non-ketotic hyperglycemia. Though the exact pathophysiology is unclear, patients with this syndrome typically present with hemichorea-hemiballismus, but other sensorimotor disturbances may also be present. The diagnosis is made through unique radiographic findings on imaging of the basal ganglia, which may be misconstrued as an alternative diagnosis. The following is a case of diabetic striatopathy in patient with uncontrolled diabetes who was initially thought to have an intracerebral hemorrhage (ICH). A 45-year-old gentleman with a prior history of uncontrolled type 2 diabetes mellitus due to non-compliance, hypertension, and hyperlipidemia presented to the emergency department of an outside hospital with the chief complaint of left arm numbness and weakness, as well as left-sided tinnitus, for one week. His physical exam was significant for lateral strabismus of the left eye. His blood work on evaluation revealed profound hyperglycemia of 700 mg/dL. A computerized topography (CT) scan of the head revealed enhancement of the right basal ganglia. Due to initial concern for an ICH, he was subsequently transferred to a tertiary care center for further evaluation. However, upon a second review of imaging by radiology and neurology, it was determined that this was less likely to be an ICH given his otherwise normal neurologic exam. There was additionally no vasogenic edema on the CT scan. Given his profound hyperglycemia and his symptoms, the working diagnosis of diabetic striatopathy was established. With correction of his blood sugars, his symptoms improved and on follow-up magnetic resonance imaging (MRI) of the brain, his initial basal ganglia enhancement had resolved. This case highlights a rare but significant complication of hyperglycemia that is important to diagnose early to prevent possible permanent structural change and continuous symptoms. This syndrome should especially be considered in diabetic patients with hyperglycemia and abnormal findings on neuroimaging, to facilitate timely treatment via correction of hyperglycemia which can lead to resolution of symptoms.

Cauda Equina Syndrome in Ankylosing Spondylitis: Challenges in Diagnosis, Management, and Pathogenesis.

Cauda equina syndrome (CES) is a rare neurologic complication of longstanding ankylosing spondylitis (AS). It is unclear what causes CES, and no proven or effective therapy has been reported to date. We have encountered 6 patients with longstanding AS diagnosed with CES. We set about to study their features, review the literature, and generate hypotheses regarding pathophysiology, as well as to speculate on the possibilities of early recognition and prevention. We obtained permission from 6 patients with longstanding AS and CES to access their medical records and imaging studies for research purposes related to this paper. We collected and reviewed each patient's medical history, imaging studies, disease duration, past therapies especially those that relate to AS, laboratory data, as well as any treatment they received for CES and followup results of each case to the present time. The 6 cases of CES with AS have remarkable similarity to each other in that several decades of the disease had passed before neurologic symptoms and later signs appeared. All cases have fused spines and facet joints without spinal fractures, spinal stenosis, or disc herniation. CES is a rare yet debilitating neurologic complication of longstanding AS. The pathophysiology and treatments are far from clear. We postulate that chronic enthesitis of the vertebral column initiates the process that results in dural stiffening and formation of ectasias, causing downstream nerve root damage.

Rare Case of Ischaemic Stroke Following Cervical Transforaminal Injection.

We report a case of brainstem infarction following a C5–C6 cervical transforaminal injection, a rare and serious neurological complication of this procedure. Cervical transforaminal steroid injection is a common therapy for patients with persistent cervical radiculopathy not relieved by conservative treatment, and is effective in 65–70% of cases. Unfortunately, this procedure may lead to serious complications such as neurological damage. These complications are rare but potentially fatal, as reported in our case. Complications could be due to three mechanisms: the technique itself, the cervical vascular anatomy and the properties of the product (corticoids). The neurological complications can be diagnosed through brain MRI. This case report focuses on the importance of a risk/benefit evaluation when performing this medical procedure.LEARNING POINTSPhysicians should be better informed about severe complications following cervical transforaminal epidural steroid injections and their strict indications.Severe adverse events are rare but they can be disastrous.Non-particulate corticosteroid should be used.

Dengue: Uncommon Neurological Presentations of a Common Tropical Illness.

ABSTRACTDengue is a common arthropod-borne flavivirus and commonly manifests as fever, bleeding diathesis, and capillary leak syndrome. Neurological manifestations are uncommon except for encephalopathy. We have recently had the opportunity of observing two patients with rare neurological complications of dengue, transverse myelitis, and Guillain-Barré syndrome. Both the cases had good neurological recovery with steroid and intravenous immunoglobulin, respectively.How to cite this article: Singh H, Pannu AK, Bhalla A, Suri V, Kumari S. Dengue: Uncommon Neurological Presentations of a Common Tropical Illness. Indian J Crit Care Med 2019;23(6):274–275.

Clinical improvement of status epilepticus after delivery: A case report

Status epilepticus is a rare neurological complication of pregnancy, associated with significant maternal and fetal morbidity and mortality. Termination of pregnancy is not currently a management strategy. The clinical case was 35-year-old known epileptic presented with complaints of multiple convulsions of 2 days and loss of consciousness of a day. On examination, she was unconscious, with Glasgow Coma Scale 5/15, having repeated seizures, and blood pressure was normal. The assessment was status epilepticus and she was shortly admitted into the intensive care unit. She was eventually paralyzed and put on mechanical ventilation. She had a spontaneous delivery, subsequently improved, and was discharged on oral carbamazepine. Key aspects of this patient's management buttressed that status epilepticus can mimic eclampsia. That she significantly and progressively improved following delivery and that previous similar cases have been reported brings to bear that delivery might need to be considered as a management strategy for refractory status epilepticus.

Isolated third nerve palsy complicating Rickettsial fever: A rare neurological complication

Isolated third nerve palsy complicating Rickettsial fever: A rare neurological complication

344. Post Malaria Neurological Syndrome: A Rare Complication of Malaria

BackgroundPost malaria neurological syndrome (PMNS) is a rare neurological complication that can occur after recovery from malaria, usually following severe Plasmodium falciparum malaria. A total of 43 cases have been previously reported in the literature.MethodsWe report a patient with neurological symptoms following 1 month of clinical and microbiological resolution of severe falciparum malaria following treatment consistent with PMNS.ResultsA 24-year-old male presented with fever, confusion, dysarthria, and grand mal seizure. His recent medical history was significant for severe falciparum malaria with multiorgan dysfunction, including acute kidney injury after being noncompliant with antimalarial prophylaxis while working for the Peace Corps in Togo prior to falling ill. He fully recovered but still required dialysis, and returned to the United States. He presented to the hospital 1 month after his initial malaria infection. On physical examination, the patient was febrile to 38.9°C, lethargic and responsive only to painful stimuli. Signs of meningismus were absent. Computed tomography of head, abdomen and thorax were unrevealing. Magnetic resonance imaging of the brain revealed a nonspecific focus of signal abnormality in right internal capsule. Cerebral spinal fluid (CSF) analysis revealed WBC of 75/μL, with lymphocytic pleocytosis and elevated protein of 65 mg/dL. CSF bacterial and viral polymerase chain reaction pane and cryptococcal antigen were negative. CSF VDRL was nonreactive. HIV antigen/antibody serology was negative. Blood, CSF, and urine cultures were all negative for growth. Two malaria smears were negative. Initially, he was started on broad spectrum antibiotics, Acyclovir and Coartem which were discontinued following negative laboratory results, and a steroid taper was initiated. His mental status began improving on the second day of the initiation of the steroid taper, and he fully recovered by day 5 of steroid therapy.ConclusionIn patient with recent medical history of malaria who presents with neuropsychiatric symptoms, clinicians must have a high index of suspicion for PMNS.Figure 1.MRI of brain showing nonspecific focus of signal abnormality in the posterior limb of the right internal capsule.Disclosures All authors: No reported disclosures.

Acute myelitis associated with dengue infection

ObjectivesTo present a rare neurological complication of dengue fever. Patients and methodsA 24-year-old female presented with acute myelitis seven days after dengue fever onset. ResultsThe patient presented with intense fever. The day-7 examination revealed a paraparesis, T2 sensory level, and urinary retention. The patient complained of electric discharges in the four limbs. The sitting and standing positions were impossible. An MRI of the spinal cord performed on day 8 revealed diffuse medullar hyper intense lesions on T2-weighted sequences at the cervical and thoracic levels, with enhancement of the thoracic lesion after gadolinium injection. Laboratory tests revealed positive dengue antigen on day 5 and positive IgM/IgG on day 8. Treatment with intravenous pulse methylprednisolone was initiated. ConclusionDengue virus has not often been reported as a cause of myelitis. Physicians must be aware of this rare complication in patients living in or coming from endemic areas.