Progressive Cerebral Venous Thrombosis with Cranial Nerve Palsies in an Adolescent African Girl & Associated Diagnostic Pitfalls: A Rare Case Report.

Cerebral venous thrombosis (CVT) is a cerebrovascular disorder caused by complete or partial occlusion of the cerebral venous and sinus system. The etiology has been attributed to hypercoagulability and pro-thrombotic states, leading to raised intracranial pressures that often manifest as headaches and focal neurological deficits. However, the multifactorial nature of CVT can create a diagnostic conundrum for clinicians. We describe a unique case of a 16-year-old female who presented with convulsions, postictal confusion, and drowsiness followed by residual weakness of her extremities. She initially presented to the primary care center with headache, high-grade fever, and altered mental status and was empirically treated for pyogenic meningitis. The patient failed to improve with a week of antibiotics and was referred to the tertiary care center for urgent attention. On presentation, the patient developed VI and VII cranial nerve palsy. Subsequently, MRI images showed filling defects in the superior sagittal, right transverse, and sigmoid sinuses with right parietal gyral T1 hyperintensity and T2 hypo-intensity. She was diagnosed with septic CVT based on sinus venous thrombosis and venous infarction, probably secondary to meningococcal pneumonia. It can be challenging to distinguish between both conditions as their presentations overlap. Moreover, cranial nerve palsy is an infrequent manifestation of CVT, with unclear pathogenesis. We highlight the role of neuro-imaging in the early detection of CVT and bring to light the unfamiliar symptoms and a more varied clinical spectrum that may hinder the diagnosis in a limited-resource setting. Future research should be explicitly modeled to improve the diagnostic efficiency of CVT and improve outcomes in younger patient populations.

Wild honey poisoning: A case report from western Nepal.

Wild honey has been used for several purposes in South-Asia and Eastern Europe since long ago. One of the commonest is medicinal purposes, especially for gastrointestinal disorders (peptic ulcer disease, dyspepsia, and gastritis), hypertension, and an aphrodisiac (sexual stimulant). However, honey produced from the nectar of few rhododendron species contains a toxin known as grayanotoxin, which acts on the sodium channel and prevents its inactivation leading to vagal activation, causing hypotension and bradycardia. Here, we report a case of 55 years woman who reported to our Emergency Department with complaints of dizziness and vomiting as well as bradycardia and hypotension. Most of the cases of wild honey poisoning resolve within the first 24 h, but in our case, the symptoms persisted for 72 h. So, we must observe patients for 72 h as some cases may last longer. Timely management of the patient can prevent fatal complications.

Behavioral Problems in Fragile X Syndrome: A Review of Clinical Management.

Fragile X syndrome (FXS) is noted to be the leading cause of inherited intellectual disabilities and is caused by expansive cytosine-guanine-guanine (CGG) trinucleotide repeats in the fragile X mental retardation 1 gene (FMR1). FXS can display a wide range of behavioral problems in addition to intellectual and developmental issues. Management of these problems includes both pharmacological and non-pharmacological options and research on these different management styles has been extensive in recent years. This narrative review aimed to collate recent evidence on the various management options of behavioral problems in FXS, including the pharmacological and non-pharmacological treatments, and also to provide a review of the newer avenues in the FXS treatment.

Prevalence of Fabry Disease in Patients With Cryptogenic Strokes: A Systematic Review.

Fabry disease (FD) is an X-linked disorder involving multiple organs. Stroke is a serious and frequent complication of FD. Cryptogenic stroke is a common presentation of FD, especially in the young population. The etiology of cryptogenic stroke is highly variable and difficult to assess, frequently leaving patients without a primary diagnosis. We conducted a systematic review to investigate the pooled prevalence of FD among patients with cryptogenic stroke, or patients with FD in whom a stroke was the presenting condition. English-language studies involving humans published in the last 20 years were included in this systematic review. FD was more common in male patients and tended to present at an earlier age. The frequency of hemorrhagic and ischemic strokes in this population was similar to that in the general population. There was a high rate of stroke recurrence in the study sample, even among patients undergoing enzyme replacement therapy. We conclude that screening for FD in patients with cryptogenic stroke is low yield and not cost-effective. However, it may be worthwhile to screen for FD among patients with recurrent strokes.

Rituximab in the Management of Refractory Myasthenia Gravis and Variability of Its Efficacy in Anti-MuSK Positive and Anti-AChR Positive Myasthenia Gravis.

Myasthenia gravis affects the neuromuscular junction of the skeletal muscles. It results in muscle weakness involving skeletal muscles (diaphragm, extraocular muscles) and myasthenic crisis. Treatment options for myasthenia gravis management have expanded, including azathioprine, corticosteroids, plasma exchange, and tacrolimus. Unfortunately, a few cases of myasthenia gravis don't respond to conventional treatment modalities. Monoclonal antibodies, rituximab (RTX), are novel treatments that have garnered interest as of late due to their efficacy within the patient population presented with refractory form myasthenia gravis. This review aims to showcase how RTX is an effective treatment within different forms of myasthenia gravis. A limited review was performed using databases that include PubMed and Google Scholar. The following keywords were used: "myasthenia gravis," "rituximab," "monoclonal antibody," "anti-AChR antibody," and "refractory myasthenia." The review focused on case reports, human studies, or research surveys based on the inclusion criteria of human studies involving participants more than 18 years of age and published in English literature. Out of 69 articles, 14 were duplicates, and 29 were relevant and met the inclusion criteria. The findings from the study demonstrate that patients with refractory myasthenia gravis responded well to RTX treatment. Furthermore, RTX has been shown to decrease corticosteroid dependence, induce sustained remission, and have a favorable response to anti-MuSK antibody positive myasthenia gravis compared to anti-AChR antibody positive myasthenia gravis. This literature review suggests that patients with refractory myasthenia gravis can benefit from rituximab; however, it has a variable response in different forms of myasthenia gravis.

Bevacizumab and Sinus Venous Thrombosis: A Literature Review.

Pediatric glioma treatment can be confounded by eloquent anatomical location and pathologic and genetic characteristics. Current literature suggests that the vascular endothelial growth factor (VEGF) inhibitor bevacizumab has been linked to enhancing disease control; however, its safety and effectiveness are unknown. Bevacizumab has been linked with an increased incidence of intratumoral hemorrhage as well as arterial and venous thromboembolism. A rare adverse effect of chemotherapeutic treatment with bevacizumab is sinus venous thrombosis (SVT), with only a few cases reported to date. This review highlights the pathophysiology of bevacizumab, its rare and life-threatening side effect of SVT, and future recommendations.

Understanding Parinaud's Syndrome.

Parinaud's syndrome involves dysfunction of the structures of the dorsal midbrain. We investigated the pathophysiology related to the signs and symptoms to better understand the symptoms of Parinaud's syndrome: diplopia, blurred vision, visual field defects, ptosis, squint, and ataxia, and Parinaud's main signs of upward gaze paralysis, upper eyelid retraction, convergence retraction nystagmus (CRN), and pseudo-Argyll Robertson pupils. In upward gaze palsy, three structures are disrupted: the rostral interstitial nucleus of the medial longitudinal fasciculus (riMLF), interstitial nucleus of Cajal (iNC), and the posterior commissure. In CRN, there is a continuous discharge of the medial rectus muscle because of the lack of inhibition of supranuclear fibers. In Collier's sign, the posterior commissure and the iNC are mainly involved. In the vicinity of the iNC, there are two essential groups of cells, the M-group cells and central caudal nuclear (CCN) group cells, which are important for vertical gaze, and eyelid control. Overstimulation of the M group of cells and increased firing rate of the CCN group causing eyelid retraction. External compression of the posterior commissure, and pretectal area causes pseudo-Argyll Robertson pupils. Pseudo-Argyll Robertson pupils constrict to accommodation and have a slight response to light (miosis) as opposed to Argyll Robertson pupils were there is no response to a light stimulus. In Parinaud's syndrome patients conserve a slight response to light because an additional pathway to a pupillary light response that involves attention to a conscious bright/dark stimulus. Diplopia is mainly due to involvement of the trochlear nerve (IVth cranial nerve. Blurry vision is related to accommodation problems, while the visual field defects are a consequence of chronic papilledema that causes optic neuropathy. Ptosis in Parinaud's syndrome is caused by damage to the oculomotor nerve, mainly the levator palpebrae portion. We did not find a reasonable explanation for squint. Finally, ataxia is caused by compression of the superior cerebellar peduncle.

The Influence of Coronavirus Disease-2019 (COVID-19) On Parkinson's Disease: An Updated Systematic Review.

BACKGROUND: COVID-19 has affected global communities with multiple neurological complications in addition to other critical medical issues. COVID-19 binds to the host's angiotensin-converting enzyme 2 (ACE2) receptors, which are expressed in the neurons and glial cells, acting as an entry port to the central nervous system (CNS). ACE2 receptors are abundantly expressed on dopamine neurons, which may worsen the prognosis of motor symptoms in Parkinson's disease (PD). SARS-CoV-2 may lead to an indirect response via immune-mediated cytokine storms and propagate through the CNS leading to damage. In this systematic review, we aim to provide thorough analyses of associations between COVID-19 and neurological outcomes for patients with PD. METHODS: Using PRISMA statement 2020, a systematic review was conducted to isolate confirmed COVID-19 patients and analyze the PD-associated neurological outcomes using the following databases: PubMed, Science Direct, Google Scholar, and Cochrane databases. The following keywords were used "COVID19, SARS-CoV-2, Parkinson's disease, Pandemic, Mortality." A modified Delphi process was employed. RESULTS: Of the 355 studies located during the initial round of screening, 16 were included in the final synthesis. Of PD patients who tested positive for SARS-CoV-2, worsening motor symptoms and other viral-associated symptoms were reported. These symptoms included bradykinesia, tremors, gait disturbances, delirium and dementia, and severe spasms of arms and legs. Encephalopathy was presented in 2 of the included studies. Increased mortality rates were identified for hospitalized patients due to COVID-19 and PD as compared to other patient groups. CONCLUSION: Patients with PD may experience substantial worsening of symptoms due to COVID 19. Given the novelty of neurological-viral associations, clinical studies in the future ought to explore the disease severity and neurological outcomes in COVID-19 positive patients with PD as compared to non-PD patients, in addition to understanding the role of ACE2 in increased vulnerability to contracting the infection and as a treatment modality.

Medical Decision Making: Hyposphagma Prior to Intravenous Tissue Plasminogen Activator in Acute Ischemic Stroke.

The decision to administer intravenous tissue plasminogen activator (IV tPA) is based on standard exclusion and inclusion criteria, which include laboratories, imaging, and time of last known well. When patients present with a clinical scenario that is not addressed in these standards, the decision to administer IV tPA is more complex. We present a case of a patient with an acute stroke syndrome that also included acute subconjunctival hemorrhage (i.e., hyposphagma). We provide the medical decision making that occurred prior to the administration. Ultimately, the finding of hyposphagma should not disqualify eligible patients from receiving IV tPA.

Unusual case of bilateral caudate infarcts following pituitary apoplexy.

IMPORTANCE: Cerebral ischemia due to pituitary apoplexy is very rare. It may be caused by vasospasm or direct compression of cerebral vessels by the expanding mass. Bilateral caudate infarcts also are very rare. To our knowledge, this is the first case report that presents pituitary apoplexy causing compression of bilateral anterior cerebral artery branches and leading to bilateral caudate infarcts. OBSERVATIONS: An 81-year-old woman with a pituitary macroadenoma presented with circulatory shock due to pituitary apoplexy. Neurological examination revealed new asymmetric quadriparesis with chronic bilateral visual disturbance. On brain magnetic resonance imaging, she was found to have watershed infarcts in the anterior cerebral artery-middle cerebral artery and middle cerebral artery-posterior cerebral artery watershed zones in addition to bilateral caudate infarcts. CONCLUSIONS AND RELEVANCE: Pituitary apoplexy can cause compression of bilateral anterior cerebral arteries from the expanding mass and lead to bilateral caudate infarcts. It is important to understand the pathophysiology of cerebral ischemia in pituitary apoplexy to improve management.

Macroglossia associated with brainstem injury.

BACKGROUND: Macroglossia has been reported in patients undergoing posterior fossa neurosurgical procedures and is thought to be as a result of venous engorgement from intubation or mechanical positioning during these prolonged procedures. METHODS: We report three patients who developed macroglossia and dysautonomia of central neurogenic origin following brainstem injury. RESULTS: The three patients developed macroglossia and dysautonomia with wide hemodynamic fluctuations in the setting of posterior fossa injury of the lower brainstem structures, necessitating tracheostomy placement. Macroglossia was managed with dexamethasone and there was complete resolution of dysautonomia while treated with beta-blockers and gabapentin. CONCLUSIONS: Neurointensivists should be aware of macroglossia with dysautonomia complicating brainstem injury, which may have perilous consequences in the setting of cerebral edema or intracranial hypertension.