Atypical Neurological Manifestations Research Articles

Late-onset Parry-Romberg Syndrome with Atypical Neurological Manifestations: A Case Report (P11-11.015)

Late-onset Parry-Romberg Syndrome with Atypical Neurological Manifestations: A Case Report (P11-11.015)

Thrombotic thrombocytopenic purpura presenting as stroke mimics with normal diffusion-weighted MRI

BackgroundThrombotic thrombocytopenic purpura (TTP) is a rare and fatal thrombotic microangiopathy-based hematologic disease. Stroke has been reported as atypical neurological manifestations of TTP in some cases.Case presentationA 65-year-old male presented with typical acute ischemic stroke symptoms including sudden-onset dysarthria, right-sided facial paralysis and hemiplegia. However, his CT and MRI scans were negative without showing any new ischemic lesions. He was diagnosed with TTP with severe thrombocytopenia, mild anemia, increased LDH, and low ADAMTS-13 activity. The symptoms and positive signs were rapidly resolved after administrating the plasma exchange therapy.ConclusionClinicians should consider the possibility of TTP when a patient presents with acute stroke-like symptoms and thrombocytopenia, especially in an emergency room, either with or without new stroke lesions on the brain CT and MRI.

Informal gold miners with mercury toxicity: Novel asymmetrical neurological presentations

Mercury is a highly toxic heavy metal that may cause neurological, respiratory, gastrointestinal and dermatological illnesses. Previously described neurological manifestations of mercury toxicity are symmetrical, and include a pancerebellar syndrome, generalised seizures and encephalopathy. Mercury is used in the gold mining process, and in artisanal or illicit gold mining, often without necessary protection. Here we describe the cases of two artisanal gold miners from western Johannesburg, South Africa, who presented with atypical neurological manifestations of mercury toxicity. Patient 1 presented with focal seizures, an asymmetrical cerebellar syndrome and an acute encephalopathy. Patient 2 had unilateral cerebellar ataxia. Both patients had toxic mercury levels, with no other cause identified for their symptoms. Patient 1 responded well to chelation therapy, but patient 2 refused admission and further medical treatment. The neurological manifestations of mercury toxicity are typically symmetrical, whereas our two patients presented with markedly asymmetrical features. It is important to maintain a high index of suspicion for mercury poisoning, even in patients with atypical and unilateral or asymmetrical presentations. A prompt diagnosis and the commencement of early chelation therapy have the potential to produce good outcomes.

Deciphering the Role of Schwann Cells in Inflammatory Peripheral Neuropathies Post Alphavirus Infection

Old world alphaviruses (e.g., chikungunya) are known to cause severe acute and chronic debilitating arthralgia/arthritis. However, atypical neurological manifestations and, in particular, unexpected cases of acute inflammatory Guillain-Barre syndrome (GBS) have been associated with the arthritogenic alphaviruses. The pathogenesis of alphavirus-associated GBS remains unclear. We herein addressed for the first time the role of Schwann cells (SC) in peripheral neuropathy post-alphaviral infection using the prototypical ONNV alphavirus model. We demonstrated that human SC expressed the recently identified alphavirus receptor MxRA8 and granting viral entry and robust replication. A canonical innate immune response was engaged by ONNV-infected SC with elevated gene expression for RIG-I, MDA5, IFN-β, and ISG15 and inflammatory chemokine CCL5. Transcription levels of prostaglandin E2-metabolizing enzymes including cPLA2α, COX-2, and mPGES-1 were also upregulated in ONNV-infected SC. Counterintuitively, we found that ONNV failed to affect SC regenerative properties as indicated by elevated expression of the pro-myelinating genes MPZ and MBP1 as well as the major pro-myelin transcription factor Egr2. While ONNV infection led to decreased expression of CD55 and CD59, essential to control complement bystander cytotoxicity, it increased TRAIL expression, a major pro-apoptotic T cell signal. Anti-apoptotic Bcl2 transcription levels were also increased in infected SC. Hence, our study provides new insights regarding the remarkable immunomodulatory role of SC of potential importance in the pathogenesis of GBS following alphavirus infection.

Association des manifestations neurologiques rares à West Nile virus chez un patient immunocompétent

BackgroundNeuro-invasive disease is the most dreaded complication of West Nile virus infection. We report the case of an immunocompetent patient who developed a meningeal syndrome associated with cerebellar ataxia, facial diplegia and hemiparesis caused by West Nile virus infection with a favourable outcome. ObservationA 42-year-old man was admitted for a febrile meningeal syndrome and confusion associated with cerebellar ataxia. The patient had developed facial diplegia, left hemiparesis with worsening cerebellar syndrome. He was diagnosed with neuro-invasive West Nile virus infection given the positive West Nile virus serologies in serum and cerebrospinal fluid as well as West Nile virus polymerase chain reaction in serum and urine. The outcome was favourable. ConclusionBecause of the increasing prevalence of West Nile virus, this infection should be suspected in the event of atypical neurological manifestations even in immunocompetent patients.

NEUROVASCULAR HYPERCOAGULABILTY IN COVID-19-A PICTORAL ESSAY

Acute stroke is a medical emergency during the COVID-19 pandemic as COVID-19 being a hypercoagulable state. COVID-19 present mostly with respiratory system related symptoms, while others present with atypical gastrointestinal, cardiovascular, or neurological manifestations. Here we present a series of three patients with COVID-19 that presented with acute stroke [1]. Methods: Hospital PACS was searched for COVID RTPCR positive patients who presented with weakness. Patients who had imaging suggestive of stroke and PCR-conrmed COVID-19 infection were included in the study. Informed consent was obtained.

Kawasaki Disease with Peripheral and Facial Gangrene: A Case report and review of literature

With the onset of the SARS-CoV-2 pandemic, Kawasaki Disease (KD) has come to the fore with its many atypical manifestations. Atypical clinical neurological, ophthalmological, musculoskeletal, gastrointestinal and pulmonary manifestations in a febrile child with raised markers should prompt the clinician to think of Kawasaki disease. Peripheral gangrene is a rare atypical manifestation of KD reported in infancy. We present a three-and-a-half-year-old boy with extensive gangrene all four limbs and face along with purpura fulminans. He was successfully treated with two doses of intravenous immunoglobulin (IVIG) and infliximab, with no residual gangrene. This case highlights that very severe forms of Kawasaki disease require IVIG, pulse steroids as well as infliximab for adequate control and complete resolution of the disease.

Atypical neurological manifestations in Wernicke’s encephalopathy due to hyperemesis gravidarum

ABSTRACT Objectives: Hyperemesis gravidarum is known to induce nutritional, water and electrolyte deficiencies which can be fatal if not treated urgently. Thiamine deficiency may lead to a constellation of neurological symptoms that include Wernicke encephalopathy. Moreover, Wernicke encephalopathy is typically manifested as ocular paresis, ataxia and confusion. Methods: Retrospective review of 6 women who developed neurological abnormalities following hyperemesis gravidarum and were treated with varying dosage of parenteral thiamine. Results: Five women developed atypical neurological symptoms, namely, slurred speech, visual loss, seizure and aggressive behaviour while one woman developed typical clinical triad of Wernicke encephalopathy after hyperemesis gravidarum. Magnetic Resonance Imaging (MRI) scans revealed abnormalities suggestive of Wernicke encephalopathy in three women only. All women improved after parenteral thiamine administration during hospital stay and had a complete neurological recovery during 2 months follow up. Discussion: Wernicke encephalopathy may not be necessarily associated with the typical neurological triad and may not have noticeable hyperintensity signal in dorsomedial thalami, mammillary bodies, hippocampus and periaqueductal region during magnetic resonance imaging. Atypical neurological signs and symptoms following hyperemesis gravidarum would invariably respond immediately to appropriate dosage of parenteral thiamine. A lower loading dosage of thiamine (100 mg thrice daily) appeared adequate for management in women with normal MRI scans.

COVID-19 and Stroke

Most patients with COVID-19 present with constitutional and respiratory symptoms and some with atypical gastrointestinal, cardiovascular, or neurological manifestations. Recent studies suggest that there are neurologic manifestations of COVID-19, including acute cerebrovascular disease (CVD). The aim of this study is to find out any evidence of COVID-19 related stroke. Radiologic studies of the patients admitted to the emergency department (ED) of our center from March 11 Th to June 10 Th 2020, with acute stroke symptoms and of whom the acute cerebrovascular disease is confirmed by Computed Tomography (CT) and/or Magnetic Resonance Imaging (MRI) are searched retrospectively. CT Angiography (CTA) and MR Angiography (MRA) studies obtained for stroke management, searched for acute thromboembolism. We noticed some radiologic evidence of acute cerebrovascular disease in 56 patients of 528 patients admitted with immediate neurological symptoms. 11 (19.64 %) of these patients who were not diagnosed before, proved to be simultaneous COVID-19 infection with laboratory tests and/or thorax CT. It was noteworthy that these 11 patients presented with an acute cerebrovascular event supported by neurological and radiological findings instead of the well-known constitutional or respiratory symptoms of COVID 19 infection. 45 (80.35 %) patients were negative for COVID-19 infection. CT /or MR Angiography demonstrated carotid or intracranial major arterial thromboembolism in 5 (11.1 %) of the non-COVID-19 patients and 5 (45.4 %) of the simultaneous COVID-19 disease diagnosed ones. COVID19 positive 5 patients presented with acute internal carotid artery (ICA) or major ICA branch thrombosis at the first stage of COVID-19 infection, rather than a complication of the serious lung disease or a component of multiorgan disfunction related COVID-19. Acute cerebrovascular disease symptoms bringing patients to the ED instead of the respiratory symptoms, aroused high suspicion of the direct neuropathy and early coagulopathy effect of the virus. COVID- 19 disease, itself thought to be a great risk factor for stroke alone. Even in initial cases and in cases where COVID-19 infection do not show a severe and fatal course, stimulation in the coagulation cascade in the early stages, increased the risk of acute stroke.

Serum glutamate decarboxylase antibodies and neurological disorders: when to suspect their association?

To explore different neurological manifestations with suspicion of being associated to serum glutamate decarboxylase antibodies (GAD-Abs) in order to better characterize anti-GAD neurological syndromes. Observational retrospective study including all patients for whom GAD65-Abs titers in serum were requested by the Neurology Department at La Paz University Hospital between 2015 and 2019. GAD-Abs were measured by ELISA. Demographic data, neurological symptoms, comorbidity with diabetes mellitus (DM) or with another autoimmune disease, and GAD-Abs titers were studied. Stiff-person syndrome, ataxia, encephalitis, and epilepsy were considered typical anti-GAD neurological syndromes and were compared to other atypical manifestations. A total of 173 patients (51.7% men, mean age 51.62) were included. A progressive increase in requests of serum GAD-Abs has occurred over the last 5 years, especially in patients with atypical neurological manifestations. GAD-Abs were found in the serum of 22 patients (12.7%); of those, 15 (68.18%) suffered a typical anti-GAD syndrome. Presence of DM or another organ-specific autoimmune disease was predictive of GAD-AB seropositivity (p < 0.001). 6.6% of requested patients with an atypical syndrome had GAD-Abs, but serum levels were significantly lower than those found in patients with a typical syndrome (706.67 vs 1430.23 UI/mL; Mann-Whitney U, p = 0.034), and were finally diagnosed with another neurological disease. Serum GAD-Abs were infrequently found in patients with clinical phenotypes other than those classically described as anti-GAD disorders, and with very low titers. In typical anti-GAD syndromes, there is a high comorbidity with DM and with other autoimmune diseases, and high serum GAD-Abs levels are usually present.

A Noteworthy Case Report of Neuroborreliosis in an Unvaccinated Pediatric Patient.

IntroductionLyme disease typically presents with viral-like symptoms and a pathognomonic rash. With disease progression, symptoms of nervous system involvement usually include facial nerve palsy and meningitis, but other atypical neurologic manifestations have less commonly been documented.Case ReportA six-year-old male presented with prolonged fevers, rash, headache, and non-specific neurologic symptoms. The diagnosis of neuroborreliosis with meningitis and polyradiculitis was confirmed with laboratory evaluation and lumbar puncture.ConclusionNeuroborreliosis is a disseminated form of Lyme disease. While meningitis is a common sign, the presentation of polyradiculitis in children is rare and can lead to misdiagnosis and delay in treatment.

Atypical Neurological Manifestations of COVID-19.

The novel coronavirus (SARS-CoV-2), belonging to a group of RNA-enveloped viruses and believed to be transmitted by aerosol route, is a worldwide pandemic. Many studies have described typical clinical manifestations such as fever, cough, fatigue, diarrhea, and nasal congestion. However, to our knowledge, there are minimal studies on the neurological manifestations in SARS-CoV-2 positive patients. Our review aims to identify the various neurological manifestations in SARS-CoV-2 positive patients, which could be an added advantage in the early diagnosis and prevention of further complications of the nervous system.

COVID-19 presenting as stroke

ObjectiveAcute stroke remains a medical emergency even during the COVID-19 pandemic. Most patients with COVID-19 infection present with constitutional and respiratory symptoms; while others present with atypical gastrointestinal, cardiovascular, or neurological manifestations. Here we present a series of four patients with COVID-19 that presented with acute stroke. MethodsWe searched the hospital databases for patients that presented with acute stroke and concomitant features of suspected COVID-19 infection. All patients who had radiographic evidence of stroke and PCR-confirmed COVID-19 infection were included in the study. Patients admitted to the hospital with PCR- confirmed COVID-19 disease whose hospital course was complicated with acute stroke while inpatient were excluded from the study. Retrospective patient data were obtained from electronic medical records. Informed consent was obtained. ResultsWe identified four patients who presented with radiographic confirmation of acute stroke and PCR-confirmed SARS-CoV-2 infection. We elucidate the clinical characteristics, imaging findings, and the clinical course. ConclusionsTimely assessment and hyperacute treatment is the key to minimize mortality and morbidity of patients with acute stroke. Stroke teams should be wary of the fact that COVID-19 patients can present with cerebrovascular accidents and should don appropriate personal protective equipment in every suspected patient. Further studies are urgently needed to improve current understandings of neurological pathology in the setting of COVID-19 infection.

Spontaneous intracranial hypotension: key features for a frequently misdiagnosed disorder.

Spontaneous intracranial hypotension (SIH) is a rare neurological condition caused by low cerebrospinal fluid (CSF) volume, most commonly due to a CSF leak. The most common presenting symptom is an orthostatic headache, but some patients may present with atypical neurological manifestations such as cranial nerve palsies, an altered mental status, and movement disorders, which complicate the clinical diagnosis. Therefore, the diagnosis is based on the combination of clinical signs and symptoms, neuroimaging, and/or a low cerebrospinal fluid pressure. In this review, we describe the wide variety of neurological manifestations and complications seen in patients with SIH as well as the most common features described on imaging studies, including both subjective and objective measurements, in order to lead the clinician to a correct diagnosis. The prompt and correct management of patients with SIH will help prevent the development of life-threatening complications, such as subdural hematomas, cerebral venous thrombosis, and coma, and avoid unnecessary invasive procedures.

The changing clinico-demographic profile of dengue infection in children: a hospital-based study from eastern India

Background: Many parts of India, including the eastern regions, are now endemic for the dengue infection with increased recognition of atypical neurological manifestations apart from the classical clinical features.Methods: This prospective study was conducted in the department of paediatrics in a tertiary care teaching hospital in eastern India from July 2019 to November 2019 to determine the changing trends of the clinical features in the dengue patients of this region in the recent years among paediatric populations. All the serologically confirmed dengue patients admitted during this period satisfying the inclusion criteria were enrolled in the study population and classified as per new WHO-2009 classification into: dengue fever without warning signs (DF), dengue fever with warning signs (DFWS) and severe dengue (SD). Detailed history, clinical and laboratory parameters were recorded and analysed for all children.Results: Out of the total of 110 cases, 16 cases (14.55%) were DF, 80 cases (72.73%) were DFWS and 14 cases (12.73%) were SD. The commonest age group affected (63.64 %) was between 4-&lt;10 years. The male: female ratio of cases was 1.68:1. Besides classical clinical manifestations, we observed CNS involvement in nine cases (8.18%). Among them, five patients had dengue encephalitis, two patients had dengue encephalopathy, one patient had hypokalemic paralysis and one patient had Guillain-Barré syndrome. All the patients were treated as per standard guidelines.Conclusions: Clinician must be aware of such association during dengue outbreak because early diagnosis and appropriate supportive care can reverse this potentially fatal disease.

Neurological manifestations of cytomegalovirus infection with immune reconstitution syndrome in patients with poorly-controlled HIV

Cytomegalovirus reactivation is a complication of advanced HIV disease, with the main manifestation being retinitis. Other neurological manifestations are quite rare. The immune reconstitution syndrome has been reported, mainly as a uveitis. We present three cases of AIDS-associated cytomegalovirus disease with atypical neurological manifestations possibly as CMV related immune reconstitution syndrome. Three HIV positive patients, all with recent changes in their antiretroviral therapy regimen, presented with various neurological manifestations - painful polyradiculopathy, multiple ischemic infarcts and ventriculoencephalitis. Symptoms developed within 4 to 6 weeks of regimen change. CMV was diagnosed in each of the cases through cerebrospinal fluid polymerase chain reaction (CSF PCR), with supporting evidence from neuroimaging. Treatment with ganciclovir resulted in clinical improvement in all cases. New onset neurological deficits after initiation or change of ART should raise the suspicion for cytomegalovirus immune reconstitution inflammatory syndrome. Early diagnosis and prompt treatment of neurological CMV may achieve good outcomes with resolution of neurologic signs and symptoms.

Multi-systemic melioidosis: a clinical, neurological, and radiological case study from Hainan Province, China

BackgroundMelioidosis is a tropical disease caused by Burkholderia pseudomallei (B. pseudomallei). It can infect any organ system and lead to multiple abscesses. A few studies reported that central nervous system (CNS) is also involved.We present a diabetic patient with multi-systemic melioidosis that affected the CNS, thorax, and spleen. The aim was to study the clinical and radiological features of melioidosis and enhance understanding of the disease.Case presentationA 38-year-old male presented with cough and expectoration mixed with blood for several days. Chest computed tomography (CT) showed a patchy opacity in his left lung, and multiple low-density lesions in his spleen. After 10 days of antibiotics treatment, his clinical symptoms improved and he was discharged from the hospital. But 8 months later, the patient experienced sudden onset of left limb weakness and seizure and was re-admitted to the hospital. Brain CT indicated a low-density lesion over the right frontal lobe, and magnetic resonance imaging (MRI) indicated a well-enhanced lobulated lesion with multiple diffusion restriction areas in the lesion. He had a neuronavigation-guided open surgery but no malignancy was found. B. pseudomallei was cultured from the operative samples. After 4 months of systemic and intraventricular antibiotic administration treatment, he recovered complete consciousness with left hemiparesis.ConclusionsMulti-systemic melioidosis may present atypical clinical, neurological, and radiological manifestations. It is extremely important to accurately diagnose before treatment is selected. CNS melioidosis in early stage manifests similar symptoms to malignancy or stroke. It might mislead to a false diagnose. Diffusion weighted imaging (DWI) can help in differentiate abscesses from cystic tumours.

Study of neurological manifestations in patients of falciparum malaria

Background: Several types of neurological manifestations are associated with falciparum malaria. Cerebral malaria is one of the most dreaded complications. A significant number of survivors are left with disabling neurological sequelae, most of which are self-limiting. Neurological involvement is more frequent with falciparum malaria because of its unique characteristics leading to micro-vascular involvement.Methods: Present study was conducted at department of General Medicine, VIMSAR, Burla (Odisha). This observational study included in total 110 patients, who were confirmed cases of falciparum malaria. Clinical profile including detailed neurological examination with relevant investigations was done. Spectrum of neuropsychiatric manifestations were observed.Results: Out of 110 patients male and female ratio was 1:1 (M 55 and F 55). The mean age of presentation in male was 36.09 + 14.85 yrs. and in female was 31.85 + 14.00 yrs. The major presenting symptoms were fever, headache, vomiting, altered sensorium, altered behaviour and convulsions in their decreasing order of frequency. On neurological evaluation, diminished level of consciousness was found in 56.4% cases. 14% cases had GCS score ≤ 7 and 63% had GCS score ≥ 11. Convulsion was found in 31% cases, cranial neuropathy in 10.9%, cerebellar dysfunction in 12.7%, neck rigidity in 22.7%, speech abnormality in 3.6%, peripheral neuropathy in 8.2%, fundoscopic changes suggestive of retinal involvement in 44.5% cases. Atypical manifestation like; Intracerebral haemorrhage, subarachnoid haemorrhage, cortical venous sinus thrombosis were also found in few cases. 32.72% patients had Neuropsychiatric manifestations at discharge in the form of focal neurological deficits, psychosis, depression, speech disorder, memory loss, cerebellar abnormality &amp; peripheral neuropathy.Conclusions: Neurological manifestations in falciparum malaria patients are common and encompass a wide spectrum of clinical presentation. In an endemic area, falciparum malaria should be kept as a differential diagnosis in patients presenting with atypical neurological manifestations.

Spontaneous Spinal Subarachnoid Haemorrhage: A Rare Complication of Dengue Fever

A 37-year-old woman presented with a short history of fever and bilateral lower limb weakness. She also had impaired sensory function up to T4 spine level and lax anal tone. Laboratory investigations confirmed dengue infection with mild thrombocytopenia. MRI of the spine showed a spinal subarachnoid haemorrhage from the level of T4 till T9. Despite medical and surgical interventions, her lower limb weakness persists. A high index of suspicion is needed to recognise dengue-related neurological complications. This diagnosis should be considered in any patients from dengue endemic areas presenting with acute febrile illness with atypical neurological manifestations.

Neurological manifestations of atypical celiac disease in childhood.

Various typical and atypical neurological manifestations can be seen as the initial symptoms of celiac disease (CD). We suggest that gluten toxicity is the most suspicious triggering risk factor for probable pathophysiological pathways of neurological involvement in atypical CD. The medical charts of 117 patients diagnosed with atypical CD were retrieved from a tertiary center in Ankara, Turkey. Eight patients reported as having neurologic manifestations as initiating symptoms were evaluated in detail. The initial neurological manifestations of CD in our study included atypical absence, which was reported first in this study, generalized tonic-clonic seizures, complex partial seizures, severe axial hypotonia and down phenotype, multifocal leukoencephalopathy, mild optic neuritis, attention deficit hyperactivity disorder, and short duration headaches. Seizures mostly emphasizing atypical absence could be the initial presentation manifestation of CD, first described in this literature. Gluten toxicity could be one of the most powerful triggering factors for developing epilepsy in CD. Learning disorders such as attention deficit hyperactivity disorder, short duration headaches, mild optic neuritis, encephalopathy, and DS could also be the initial neurological manifestations of atypical CD. A gluten-restricted diet may improve neurological complaints, epileptic discharges, and neuropsychiatric symptoms. All we found may be a small part of the full range of neurological disorders of unknown origin related to CD. Clinical suspicion should be the rule for accurate diagnosis of the disease.