Epstein-Barr Virus Encephalitis Presenting As Acute Disseminated Encephalomyelitis: A Challenging Diagnostic Dilemma.

Epstein-Barr virus (EBV) is a common human herpesvirus associated with a wide range of clinical manifestations, primarily affecting the lymphoid system. However, central nervous system (CNS) involvement, although rare, can occur and present a diagnostic challenge, particularly in immunocompetent individuals. We present a case of a 28-year-old healthy female who initially presented with a flu-like illness, her symptoms rapidly progressed, leading to neurological deficits, and altered mental status. The patient's diagnostic workup, including a viral panel and various antibodies, failed to provide a conclusive diagnosis. However, lumbar puncture revealed significant abnormalities in cerebrospinal fluid (CSF), including elevated white blood cell count and elevated CSF protein. Neuroimaging studies demonstrated non-specific findings in subcortical white matter, pontomedullary junction, and extended spinal cord lesion. Tragically, the patient's condition rapidly worsened, with diffuse cerebral edema observed on repeat imaging, leading to the patient's demise even after conventional treatment. CSF analysis, performed at an apex lab, unexpectedly returned positive for EBV PCR, indicating a diagnosis of EBV encephalitis or EBV-associated acute disseminated encephalomyelitis (ADEM). This case highlights the challenges encountered in diagnosing EBV-associated CNS manifestations, especially in immunocompetent individuals, where these presentations are exceedingly rare. The atypical clinical course, negative initial laboratory investigations, and absence of specific radiological findings further complicated the diagnostic process. Early recognition and consideration of infectious etiologies, including EBV, in patients presenting with unexplained encephalitis or ADEM-like symptoms, are essential for timely intervention and optimal patient outcomes.

Cerebral Autosomal Dominant Arteriopathy With Subcortical Infarcts and Leukoencephalopathy (CADASIL): A Rare Cause of Transient Ischemic Attack.

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is increasingly recognized as an inherited and autosomal dominant arteriopathy of the cerebral vasculature, which is commonly misdiagnosed due to its different modes of presentation. It is characterized by variable manifestations of ischemic episodes, migraine with aura, cognitive deficits, and psychiatric disturbances. CADASIL is caused by a genetic mutation in the NOTCH3 gene, which is present on chromosome 19. The diagnosis of CADASIL can be made by personal and family history, skin biopsy, and magnetic resonance imaging (MRI) of the head showing high-intensity signal lesions, microbleeds, and white matter changes. There are currently no disease-modifying therapies available for CADASIL, and management focuses on reducing risk factors such as diabetes and hypertension and control of symptoms. We present a rare cause of transient ischemic attack (TIA) in a young female who was later diagnosed with CADASIL and aim to highlight rare and inherited causes of TIA and strokes in younger patients.

A Case Report of Wernicke's Encephalopathy Disguised As Limbic Encephalitis: A Clinical Puzzle.

Wernicke's encephalopathy (WE) is the presence of neurological symptoms in the central nervous system caused by thiamine (Vitamin B1) deficiency. It is an acute clinical condition characterized by confusion, ataxia, and ophthalmoplegia triad. WE is most commonly observed in chronic alcohol users, while it can also present in non-alcoholics. We present a 33-year-old man with alcohol-induced WE who presented with altered mental status and fever. His initial diagnosis was skewed towards bacterial meningitis and limbic encephalitis, but MRI findings were consistent with WE. The patient responded promptly to intravenous (IV) thiamine infusion, and his mental status changed significantly. Repeat EEG in 15 days shows complete recovery with normal brain wave activity. Untreated WE is a significant cause of permanent neurological morbidity and mortality, easily preventable. High suspicion of WE should always be entertained, especially when patients have a known history of alcohol use. Early initiation of IV thiamine could prevent the consequences. Hence, it is essential to raise awareness of WE to take measures without delay and reduce mortality and morbidity with an improved prognosis.

Ophthalmoparesis and Bilateral Ptosis as a Rare Manifestation of Todd's Phenomenon: Case Report and Review.

Todd's paresis or phenomenon (TP) is a focal weakness in a part of the body after a seizure. Seizure is an abrupt change in behavior caused by the cerebral cortex's electrical hyper-synchronization of neuronal networks. After the seizure, there is usually a transition period from the ictal state to the pre-seizure baseline level of awareness and function, referred to as the postictal period. Postictal symptoms include many systems, including sensory, motor, and psychosis. This phenomenon is named after Robert Bentley Todd, who first described it. Todd's paresis can be confused with other conditions, most commonly a stroke. Postictal ocular manifestation may be accompanied by aphasia or hemiplegia, but isolated gaze palsy is rarely reported. We are reporting a rare and first known isolated ophthalmoparesis and ptosis as postictal findings with no structural abnormalities present in imaging studies and complete resolution over three weeks on its own as an atypical postictal phenomenon. Patients with an underlying structural abnormality of the brain are more susceptible to Todd's phenomenon. Unusual manifestations of Todd's phenomenon are rare but clinically relevant and are decisive in therapeutic decision-making. Our patient presents a rare manifestation of Todd's phenomenon as ptosis and ophthalmoparesis in an elderly male with no underlying structural brain abnormalities that resolved within three weeks. Further research into the causes is needed to distinguish it from a stroke.

COVID-19-Associated Autoimmune Disease: A Rare First Case Report of Acute Motor Axonal Neuropathy Variant of Guillain-Barre Syndrome in a Woman Patient in New York City.

Novel outbreaks with COVID-19 can cause multiple systemic manifestations, including autoimmune disease. Among all the infections, respiratory complications are the most apparent symptoms. Guillain-Barre syndrome (GBS) is an acute immune-mediated polyradiculoneuropathy often related to previous infectious exposure. GBS emerged as a potentially severe complication of coronavirus disease 2019 (COVID-19) since its declaration as a global pandemic. We report the first case of COVID-19-induced acute motor axonal neuropathy variant of Guillain-Barre syndrome (GBS) from New York, USA. Our patient was a 66-year-old woman who had recently tested positive for COVID-19 and presented with bilateral upper and lower extremity weakness. Electromyogram studies showed acute demyelinating polyradiculoneuropathy. She was diagnosed with an acute motor axonal neuropathy variant of GBS. She was successfully treated with intravenous immunoglobulins (IVIGs) with marked improvement. In six months, she regained her strength back to normal. Whether GBS incidence in COVID-positive patients is based on molecular mimicry or anti-ganglioside antibodies is unclear. Physicians should be aware of GBS as a potentially serious complication associated with COVID-19. Further investigations and trials should be conducted better to understand the mechanism of GBS in patients of COVID-19.

A Rare Case of Coronavirus Disease 2019 Vaccine-Associated Cerebral Venous Sinus Thrombosis Treated with Mechanical Thrombectomy.

BACKGROUND Vaccine-related thrombosis and thrombocytopenia syndrome (TTS) is a rare life-threatening syndrome reported after vaccination against COVID-19. CASE REPORT We describe a case of 56-year-old postmenopausal, obese woman with hypothyroidism and hyperlipidemia, who presented to the Emergency Department (ED) with fluctuating mental status and left-side weakness for 5 days. She received her first and second dose of mRNA-1273 vaccine (Moderna) at 12 and 8 weeks, respectively, prior to presentation. She was found to have multiple hemorrhages and infarcts on a computed tomography (CT) scan of the head. She was intubated in the ED for airway protection and mechanically ventilated. Magnetic resonance angiogram and venogram showed multiple infarcts in right frontal, parietal, and left parietal lobes, along with occlusion of left-side transverse sinus, sagittal sinuses, and left internal jugular vein, suggesting cerebral venous sinus thrombosis (CVST). Despite anticoagulation, her clinical condition continued to worsen, and she was referred for emergent endovascular thrombectomy. Her clinical condition improved after thrombectomy, and she was discharged on warfarin. At 4-month follow-up, she was able to walk with an assistive device and able to carry out activities of daily living with assistance. She is planned for further work-up for hypercoagulable state at follow-up. CONCLUSIONS This case highlights the occurrence of vaccine-related thrombosis 3 months after vaccine administration. Only 2 cases of TTS have been reported so far after mRNA-1273 vaccination (Moderna). To the best of our knowledge, this is the first reported case of CVST presenting 3 months after the first dose of COVID-19 mRNA-1273 vaccine (Moderna).

Cerebellar Vertigo as an Unspecific Initial Presentation of Creutzfeldt-Jakob Disease.

BACKGROUND Creutzfeldt-Jakob disease (CJD) is a human prion disease characterized by severe and rapidly progressive fatal neurodegeneration. Currently, there is no cure for CJD, and death from CJD usually occurs within 1 year from the onset of the symptom, and the median survival time is 6 months. CASE REPORT The patient was a 63-year-old woman who presented to the hospital reporting having vertigo for the past 1 week and involuntary muscle contraction resulting in slow repetitive movement and abnormal posture for the past 3 days. A physical examination at the time of admission revealed unsteady gait, dystonia, and dysmetria of the left upper limb. There was no nystagmus on examination. Electroencephalography done on the same day showed focal epileptiform discharge on bilateral temporal lobes, which were more on the right side than the left. It also showed mild diffuse cerebral slowing. Cerebrospinal fluid analysis showed positive for RT-QulC, T tau protein, and 14-3-3. A diagnosis of CJD was made based on clinical course, imaging, and cerebrospinal fluid analysis. CONCLUSIONS The diagnosis of CJD can be suspected based on clinical signs and symptoms and can be confirmed after performing MRI, EEG, and lumbar puncture. Therefore, it is important to recognize vertigo as an unspecific symptom of CJD so that a timely diagnosis can be made and unnecessary procedures can be avoided.

COVID-19 and the Brain: Acute Encephalitis as a Clinical Manifestation.

Central nervous system (CNS) viral infections result in the clinical syndromes of aseptic meningitis or encephalitis. Although the primary target of coronavirus disease 2019 (COVID-19) is the respiratory system, it is increasingly being recognized as a neuropathogen. The hallmark clinical feature is altered mental status, ranging from mild confusion to deep coma. Most patients with encephalopathy or encephalitis are critically ill. We present a case of COVID-19-related encephalitis who presented with acute delirium and new-onset seizures. The patient responded well to treatment with intravenous immunoglobulins and rituximab.

Acute Abdomen with Ileus: A Heralding Presentation of Neuroleptic Malignant Syndrome.

The pathophysiology of neuroleptic malignant syndrome (NMS) with use of psychotropic drugs is still unclear. Although a rare event with an incidence of 0.02-3.2%, when not promptly recognized and managed, it carries a high mortality (10-20%) and morbidity rate. Presentation can be either typical, with muscle rigidity and hyperpyrexia, or atypical, the latter posing diagnostic and early management challenges in clinical practice. Our patient presented with delayed fever and ileus, making early diagnosis difficult. We propose that NMS be considered an alternate diagnosis in patients using psychotropic medications and manifest ileus and delayed fever, especially after other differentials have been excluded.

Perioperative Management of Neurological Conditions.

Perioperative care of the patients with neurological diseases can be challenging. Most important consideration is the management and understanding of pathophysiology of these disorders and evaluation of new neurological changes that occur perioperatively. Perioperative generally refers to 3 phases of surgery: preoperative, intraoperative, and postoperative. We have tried to address few commonly encountered neurological conditions in clinical practice, such as delirium, stroke, epilepsy, myasthenia gravis, and Parkinson disease. In this article, we emphasize on early diagnosis and management strategies of neurological disorders in the perioperative period to minimize morbidity and mortality of patients.

Caveolin-1 deficiency increases cerebral ischemic injury.

Caveolins (Cav), the principal structural proteins of the caveolar domains, have been implicated in the pathogenesis of ischemic injury. Indeed, changes in caveolin expression and localization have been reported in renal and myocardial ischemia. Genetic ablation of the Cav-1 gene in mice was further shown to increase the extent of ischemic injury in a model of hindlimb ischemia. However, the role of Cav-1 in the pathogenesis of cerebral ischemia remains unknown. Immunoblot and immunofluorescence analyses of rat brains subjected to middle cerebral artery occlusion revealed marked increases in endothelial Cav-1 and Cav-2 protein levels. To directly assess the functional role of caveolins in the pathogenesis of cerebral ischemic injury, we next investigated the effects of cerebral ischemia in caveolin knockout (KO) mice. Interestingly, Cav-1 KO mice showed a marked increase of cerebral volume of infarction, as compared with wild-type and Cav-2 KO mice. Immunofluorescence analyses showed an increased number of proliferating endothelial cells in wild-type ischemic brains, as compared with Cav-1 KO ischemic brains. Immunoblot analyses of wild-type ischemic brains showed an increase in endothelial nitric oxide synthase protein levels. Conversely, the protein levels of endothelial nitric oxide synthase remained unchanged in Cav-1 KO ischemic brains. TUNEL analysis also showed increased apoptotic cell death in Cav-1 KO ischemic brains, as compared with wild-type ischemic brains. Our findings indicate cerebral ischemia induces a marked increase in endothelial Cav-1 and Cav-2 protein levels. Importantly, genetic ablation of the Cav-1 gene in mice results in increased cerebral volume of infarction. Mechanistically, Cav-1 KO ischemic brains showed impaired angiogenesis and increased apoptotic cell death.

EDG receptors as a potential therapeutic target in retinal ischemia-reperfusion injury.

LPA (lysophosphatidic acid) specific endothelial differentiation gene (EDG) receptors have been implicated in various anti-apoptotic pathways. Ischemia of the brain and retina causes neuronal apoptosis, which raises the possibility that EDG receptors participate in anti-apoptotic signaling in ischemic injury. We examined the expression of EDG receptors in a model of retinal ischemia-reperfusion injury and also tested LXR-1035, a novel analogue of LPA, in the rat following global retinal ischemic injury. Rats were subjected to 45 or 60 min of raised intraocular pressure. Animals were sacrificed at 24 h post-ischemia and retinal tissue was stained for EDG receptors. In separate experiments, animals were randomized to receive LXR or saline vehicle by intravitreal injection 24 h prior to ischemia. The degree of retinal damage was assessed morphologically by measuring the thickness of the inner retinal layers as well as functionally by electroretinography (ERG). We found that the normal retina has a baseline expression of the LPA receptors, EDG-2 and EDG-4, which are significantly upregulated in the inner layers in response to ischemia. Animals pretreated with LXR-1035 had dose-dependent, significant reductions in histopathologic damage and significant improvement in functional deficits compared with corresponding vehicle-controls, after 45 and 60 min of ischemia. These results suggest that LPA receptor signaling may play an important role in neuroprotection in retinal ischemia-reperfusion injury.