Pseudotumor Cerebri Syndrome with Resolution After Discontinuing High Vitamin A Containing Dietary Supplement: Case Report and Review.

A 24-year-old non-obese, but slightly overweight, female presented with a two-week history of progressive severe headache associated with two days of blurry vision. Clinical exam was significant for bilateral papilledema and an enlarged blind spot on visual field testing. Contrast enhanced MRI head revealed no space occupying lesion. A lumbar puncture revealed an elevated opening pressure of 38 cm H2O with normal cerebrospinal fluid composition leading to a diagnosis of pseudotumor cerebri syndrome (PTCS). The patient lacked the typical risk factors of high body mass index or obvious antecedent medications; however, on subsequent questioning, she was chronically ingesting a high vitamin A containing weight loss dietary supplement (Thrive W® - Table 1), which we believe had caused intracranial hypertension. Discontinuation of the diet pill and treatment with acetazolamide led to marked improvement of her PTCS. This case highlights the fact that non-traditional products or medications with high vitamin A may cause pseudotumor cerebri, which treating physicians should assess for while dealing with non-obese PTCS patients.

Neuro-Ophthalmic Presentation of Neuro-Sweet Disease.

Acute febrile neutrophilic dermatosis (Sweet syndrome) is a systemic inflammatory condition usually associated with autoimmune or neoplastic processes and characterised by inflammatory dermatologic lesions such as erythematous plaques and papules associated with fever and leukocytosis. Neurological and ophthalmological involvement is rare. The authors describe an unusual case of Sweet syndrome associated with microscopic polyangiitis presenting with papilloedema, anterior uveitis, and skin rash. Years later, he developed acute posterior multifocal placoid pigment epitheliopathy. Treatment with immunosuppressive medications led to a relapsing remitting course with maximum benefit from use of steroids. The authors describe the difficulties in diagnosis and treatment of this rare case.

Bilateral Radiation Optic Neuropathy Following Concurrent Chemotherapy and Radiation in Glioblastoma.

Radiation optic neuropathy (RON) is an iatrogenic complication that causes severe, irreversible vision loss within months to years following radiation to lesions close to the visual pathway. The authors describe a case of RON in glioblastoma after radio-sensitisation with temozolomide with sequential involvement of both optic nerves. This case provides a timeline for clinical and imaging findings with RON and specifically resolution of nerve enhancement. The authors also highlight the potential of an increase in incidence of RON in glioblastoma with advances in survival seen with greater use of second-line chemotherapy and even re-radiation.

Progressive Multifocal Leukoencephalopathy: Recent Advances and a Neuro-Ophthalmological Review.

BACKGROUND: Progressive multifocal leukoencephalopathy (PML) is a severe often fatal opportunistic infection of the central nervous system caused by reactivation of a ubiquitous polyoma virus, JC virus. Although typically characterized by multifocal asymmetric subcortical white matter lesions, it may be monofocal and affect the cortical gray matter. Among the broad spectrum of clinical manifestations that occurs with PML, visual complaints are common. EVIDENCE ACQUISITION: Combination of representative personally observed cases of PML and comprehensive review of case series of PML from 1958 through 2014. RESULTS: Neuro-ophthalmic signs and symptoms were reported in approximately 20%-50% of patients with PML and can be the presenting manifestation in half of these. A majority of these presentations occur from damage to cerebral visual pathways resulting in visual field defects, cortical blindness, and other disorders of visual association. Given the decreased frequency of infratentorial and cerebellar involvement, ocular motility disorders are less common. CONCLUSIONS: Visual complaints occur in patients with PML and are often the presenting sign. Awareness of this condition is helpful in avoiding unnecessary delays in the diagnosis of PML and management of the underlying condition. Recent guidelines have established criteria for diagnosis of PML in the high-risk patient population and strategies to mitigate the risk in these populations.

Analysis of Faculty Gender and Race in Scholarly Achievements in Academic Neurology.

Background: Intersection of gender and race and/or ethnicity in academic medicine is understudied; we aim to understand these factors in relation to scholarly achievements for neurology faculty. Methods: Faculty from 19 US neurology departments completed a survey (2021-2022) to report rank, leadership positions, publications, funded projects, awards, and speaker invitations. Regression analyses examined effects of gender, race, and their intersectionality on these achievements. Women, Black/Indigenous/People of Color (BIPOC), and BIPOC women were comparator groups. Results: Four hundred sixty-two faculty responded: 55% women, 43% men; 31% BIPOC, 63% White; 21% BIPOC women, 12% BIPOC men, 36% White women, 31% White men. Men and White faculty are more likely to be full professors than women and BIPOC faculty. The number of leadership positions, funded projects, awards, and speaker invitations are significantly greater in White compared to BIPOC faculty. Relative to BIPOC women, the number of leadership positions is significantly higher among BIPOC men, White women, and White men. Publication numbers for BIPOC men are lower, number of funded projects and speaker invitations for White women are higher, and number of awards among White men and White women is higher compared to BIPOC women. Discussion: Our study highlights that inequities in academic rank, award number, funded projects, speakership invitations, and leadership roles disproportionately impacted BIPOC women. More studies are needed to evaluate gender and race and/or ethnicity intersectionality effects on faculty achievements, reasons for inequities, recognition, and potential solutions.

Dural puncture-induced intracranial hypotension causing diplopia.

BACKGROUND: Diplopia that occurs after an epidural spinal catheter has been placed for pain control has been attributed to sixth nerve palsy nerve palsy induced by intracranial hypotension. There is sparse information about the factors that confound diagnosis in this setting. METHODS: Review of 6 cases examined over a period of 5 years at a single tertiary care medical center. RESULTS: Six confounders to diagnosis were identified: 1) lack of awareness that an epidural spinal catheter was or had been in place; 2) delayed reporting of diplopia; 3) mild or inapparent ductional deficits; 4) lack of postural headache; 5) clinical features that suggested an alternative diagnosis; 6) neuroimaging features that did not allow exclusion of pachymeningitis. CONCLUSION: Clinicians should be aware of features that confound a diagnosis of dural puncture-induced intracranial hypotension as a cause of diplopia in the post-operative period when an epidural pain control system is or has been deployed. If these confounders are recognized and the correct diagnosis is reached, radiologists will be less likely to diagnose pachymeningitis and clinicians will be able to avoid lumbar puncture, which may exacerbate the condition.

New advancements in the management of Neuromyelitis Optica spectrum disease: literature review.

Neuromyelitis Optica spectrum disorder (NMOSD) is a relapsing autoimmune disease of the central nervous system (CNS) where aquaporin-4 water channels are the antigenic target of the disease. The spectrum of the disease involves regions of the CNS where the water channel is widely expressed including the spinal cord, the optic nerve, dorsal medulla, brainstem, and thalamus/hypothalamus. Management of NMOSD includes acute as well as long term treatment. Acute symptoms are typically treated with intravenous corticosteroids and/or plasma exchange while long-term treatment involves the use of immunosuppression/immune modulation. The year 2019 is thought to be the "year of the NMOSD" as three new medications became available for this devastating disease. In this review, FDA approved NMOSD medications are discussed.

Noncovalent surface grafting of uranium complexed cucurbit[5]uril oligomer onto palm shell powder: a novel approach for selective uranyl ion extraction.

Biomass - that is a new feather in your cap: palm shell powder was used as support for novel uranyl complexed cucurbit[5]uril oligomer to obtain a selective extractant for uranium. The key to the selectivity lies in the perfect environment of the two portals of cucurbit[5]uril for uranyl ion binding.

Fluorescent uranyl ion lidded cucurbit[5]uril capsule.

A novel fluorescent complex {(UO(2))(2)(CB5)}(NO(3))(4)·4HNO(3).3H(2)O (U2CB5) is obtained from cucurbit[5]uril (CB5) and uranyl nitrate under ambient temperature conditions. The crystal structure revealed that two uranyl ions are coordinated to the two open portals of CB5 giving a closed molecular capsule, which further connected through CB5 molecules to give two-dimensional frameworks. The U2CB5 complex was further investigated by NMR, FTIR and TGA techniques. The Fluorescence of uranyl ion was found to be enhanced due to complexation with cucurbituril.

Novel missense WFS1 variant causing autosomal dominant atypical Wolfram syndrome.

BACKGROUND: In contrast to the classic autosomal recessive Wolfram syndrome, Wolfram-like syndrome (WLS) is an autosomal dominant disease caused by heterozygous variants in the WFS1 gene. Here, we present deep phenotyping of a mother and son with a WFS1 variant NM_006005.3:c.2508 G > T, p. (Lys836Asn) detected with next-generation sequencing, which is novel at the nucleotide level. In this Greek family, the proband and mother had sensorineural hearing loss and mild non-progressive vision loss with optic nerve atrophy. An initial optic atrophy panel that did not test for WFS1 was unremarkable, but a broader inherited retinal dystrophy panel found the WFS1 variant. CONCLUSION: This study highlights the importance of including WFS1 sequencing in the evaluation of optic nerve atrophy to discover syndromic conditions.

MRI restricted diffusion in lymphomatous optic neuropathy.

Restricted diffusion in the optic nerve detected with MRI has been previously reported in infarction and inflammation but not in infiltrative neoplasm. We report a 44-year-old man with recently diagnosed non-Hodgkin B-cell lymphoma who developed an acute left optic neuropathy. MRI showed no evidence of brain parenchymal or meningeal lymphoma but did show restricted diffusion in the intraorbital portion of the affected optic nerve. Despite treatment with corticosteroid, standard chemotherapy, and orbital X-irradiation, visual function did not improve. The restricted diffusion persisted on a follow-up MRI performed 4 months after the onset, a phenomenon that is atypical for infarction. Perhaps, this persisting imaging abnormality in lymphomatous optic neuropathy reflects the dense cellularity of the neoplasm.

Identification of degradation impurity of TGR5 receptor agonist-ZY12201 by LC-MS technique during force degradation study.

Forced degradation study is a systemic characterization of degradation products of active pharmaceutical ingredient (API) at conditions which posses more harsh environment that accelerates degradation of API. Forced degradation and stability studies would be useful in selection of proper, packaging material and storage conditions of the API. These are also useful to demonstrate degradation pathways and degradation products of the API and further characterisation of the degradation products using mass spectrometry. TGR5 is a G protein-coupled receptor, activation of which promotes secretion of glucagon-like peptide-1 (GLP-1) and modulates insulin secretion. The potent and orally bioavailable TGR5 agonist, ZY12201, shows activation of TGR5 which increase secretion of GLP-1 and help in lowering blood glucose level in animal models. Hence it is necessary to establish and study degradation pathway and stability of API for better handling and regulatory approval. Force degradation studies of ZY12201 have shown presence of one oxidative impurity during oxidative degradation in HPLC analysis. The oxidized product is further characterized by LC-MS to elucidate structure of impurity and characterize its degradation pathway. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s42452-021-04660-y.

CT demonstration of dorsal midbrain hemorrhage in traumatic fourth cranial nerve palsy.

A 21-year-old man who suffered a traumatic brain injury from a motor vehicle accident recovered brain function except for an isolated left fourth cranial nerve palsy. Brain CT showed a focal hemorrhage in the right dorsal midbrain, directly in the brainstem path of what would become the left fourth cranial nerve. Although there has been previous imaging documentation of midbrain and cisternal hemorrhage in patients with isolated post-traumatic fourth cranial nerve palsy, this is the first report to show a large midbrain hemorrhage on CT. The mechanism is likely to be concussive impact of the dorsal midbrain on the tentorium cerebelli.

A Rare Case of Cerebellar Progressive Multifocal Leukoencephalopathy due to Idiopathic CD4 Lymphocytopenia.

Progressive multifocal leukoencephalopathy (PML) is a demyelinating disorder caused by reactivation of JC virus during a time of cell mediated immune suppression. One potential rare cause of PML is Idiopathic CD4 lymphocytopenia (ICL) in which there is an unexplained deficit of CD4 T cells. We present a case of cerebellar PML in the absence of known immunosuppression leading to the diagnosis of ICL.

Atypical MOG antibody disease presenting with typical multiple sclerosis lesions.

Myelin oligodendrocyte glycoprotein (MOG) antibody disease is an autoimmune disease of the central nervous system associated with a serological antibody against MOG, a glycoprotein expressed on the outer membrane of myelin. It is solely found within the central nervous system in the brain, optic nerves and spinal cord. MOG antibody disease falls within the neuromyelitis optica spectrum disorders (NMOSD), however clinical characteristics appear distinct from aquaporin-4 antibody related disease and multiple sclerosis. It has predilection for causing recurrent optic neuritis and transverse myelitis. Accurate diagnosis is important to determine long term prognosis and suitable treatment. We describe the case of a 42 year old woman previously labelled as MS who demonstrated a variable presentation of MOG antibody disease.