Demographic Disparities in the Incidence, Clinical Characteristics, and Outcome of Posterior Reversible Encephalopathy Syndrome in the United States.

Objectives: To estimate age-specific, sex-specific, and race-specific incidence of posterior reversible encephalopathy syndrome (PRES) in the United States.

Methods: We conducted a retrospective cohort study using the State Inpatient Database of Florida (2016-2019), Maryland (2016-2019), and New York (2016-2018). All new cases of PRES in adults (18 years or older) were combined with Census data to compute incidence.

New-Onset Refractory Status Epilepticus (NORSE) as a Recurrence of Anti-Neuronal Nuclear Antibody 2 (ANNA-2) Encephalitis After Immune Checkpoint Inhibition Therapy.

Paraneoplastic encephalitis from anti-neuronal nuclear antibody 2 (ANNA-2), usually associated with breast cancer, can cause seizures. We report a case of recurrent paraneoplastic encephalitis due to ANNA-2 presenting with new-onset refractory status epilepticus (NORSE) one month after receiving checkpoint inhibitors therapy. A 69-year-old female was diagnosed with opsoclonus myoclonus syndrome (OMS) secondary to ANNA-2, which led to a diagnosis of breast cancer.

Neuro-Ophthalmic Complications in Patients Treated With CTLA-4 and PD-1/PD-L1 Checkpoint Blockade.

Background: In recent years, CTLA-4 and PD-1/PD-L1 checkpoint inhibitors have proven to be effective and have become increasingly popular treatment options for metastatic melanoma and other cancers. These agents work by enhancing autologous antitumor immune responses. Immune-related ophthalmologic complications have been reported in association with checkpoint inhibitor use but remain incompletely characterized.

Onabotulinumtoxin Type A reconstitution with preserved versus preservative-free saline in chronic migraine (B-RECON). A randomised, double-blind trial.

Introduction: Onabotulinumtoxin type A (BoNTA) is manufactured as powder that requires reconstitution with normal saline prior to injection. Previous literature has suggested that preserved saline (PS) exerts a local anaesthetic effect, and reduces the procedure discomfort when used in reconstitution in lieu of preservative-free saline (PFS). However, this was mainly studied in the aesthetics indications of BoNTA, and never in its use for the treatment of chronic migraine.

Artificial Intelligence to Detect Papilledema from Ocular Fundus Photographs.

Background: Nonophthalmologist physicians do not confidently perform direct ophthalmoscopy. The use of artificial intelligence to detect papilledema and other optic-disk abnormalities from fundus photographs has not been well studied.

Methods: We trained, validated, and externally tested a deep-learning system to classify optic disks as being normal or having papilledema or other abnormalities from 15,846 retrospectively collected ocular fundus photographs that had been obtained with pharmacologic pupillary dilation and various digital cameras in persons from multiple ethnic populations.

Seven-Year Experience From the National Institute of Neurological Disorders and Stroke-Supported Network for Excellence in Neuroscience Clinical Trials.

Importance: One major advantage of developing large, federally funded networks for clinical research in neurology is the ability to have a trial-ready network that can efficiently conduct scientifically rigorous projects to improve the health of people with neurologic disorders.

Observations: National Institute of Neurological Disorders and Stroke Network for Excellence in Neuroscience Clinical Trials (NeuroNEXT) was established in 2011 and renewed in 2018 with the goal of being an efficient network to test between 5 and 7 promising new agents in phase II clinical trials. A clinical coordinating center, data coordinating center, and 25 sites were competitively chosen.

OnabotulinumtoxinA wear-off in chronic migraine, observational cohort study.

Introduction: The current standard-of-care protocol for OnabotulinumtoxinA (BoNTA) injections consists of fixed-site injections every 12 weeks. This pattern is based on clinical practice and extrapolated from BoNTA injections for other, non-migraine-related indications. It is unclear if this protocol is optimal for chronic migraine.

Further Evidence that Onabotulinum Toxin is a Viable Treatment Option for Pediatric Chronic Migraine Patients.

Introduction Chronic migraine is particularly devastating. It affects school work, extracurricular activities, and quality of life, including relationships with other family members, and can also influence the mental health of both the migraineurs and family members. According to the International Classification of Headache Disorders, 3rd edition (ICHD-3), chronic migraine is defined as 15 or more headache days per month for greater than three months, where at least on eight days per month, there are features of migraine headache.

Headache in Idiopathic Intracranial Hypertension: Findings From the Idiopathic Intracranial Hypertension Treatment Trial.

Objective: To characterize the phenotype, headache-related disability, medical co-morbidities, use of symptomatic headache medications, and headache response to study interventions in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT).

Methods: Patients with untreated IIH and mild vision loss enrolled in the IIHTT and randomized to acetazolamide (ACZ) and weight loss or placebo (PLB) and weight loss had prospective assessment of headache disability using the Headache Impact Test-6 (HIT-6) questionnaire. Subjects with headache at the baseline visit were assigned a headache phenotype using the International Classification for Headache Disorders version 3 beta (ICHD-3b).

CSF pressure, papilledema grade, and response to acetazolamide in the Idiopathic Intracranial Hypertension Treatment Trial.

Previous reports suggest an association between the degree of optic nerve head edema and CSF pressure (CSFp) in idiopathic intracranial hypertension (IIH). We hypothesized that CSFp would be associated with Frisén papilledema grade (FPG) and other clinical features, and that FPG would modify the CSFp response to acetazolamide in participants in the Idiopathic Intracranial Hypertension Treatment Trial (IIHTT). In the IIHTT, eligible patients underwent lumbar puncture (LP) prior to enrollment and were randomly assigned to one of two treatment groups: acetazolamide plus supervised diet or placebo plus supervised diet.

IgG4-related ophthalmic disease.

IgG4-related disease is a fibro-inflammatory condition with tendency to form tumors with inflammatory infiltrate with IgG4 rich plasma cells and elevation of IgG4 level in serum, which may affect virtually every organ and tissue in the organism. IgG4-related ophthalmic disease may present as dacryoadenitis, myositis, other orbital tissues, hypophysitis or pachymeningitis causing cranial neuropathies. The diagnosis of IgG4-related disease is based on a typical clinical scenario, supportive laboratory data, expected radiological characteristics and distinct histopathological and immunohistochemical features.

Transient ocular motor nerve palsies associated with presumed cranial nerve schwannomas.

Background: Cranial nerve schwannomas are radiologically characterized by nodular cranial nerve enhancement on magnetic resonance imaging (MRI). Schwannomas typically present with gradually progressive symptoms, but isolated reports have suggested that schwannomas may cause fluctuating symptoms as well.

Methods: This is a report of ten cases of presumed cranial nerve schwannoma that presented with transient or recurring ocular motor nerve deficits.

Impairment of habituation of the auditory brain stem response in migrainous vertigo.

Objectives: We evaluated the auditory brain stem response (ABR) in migrainous vertigo (MV).

Methods: Four subjects who met clinical criteria for definite MV and 4 subjects with non-vertiginous migraine (NVM) underwent ABR testing while asymptomatic and within 16 hours of a symptomatic episode. Four control subjects were also tested.

IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil.

Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning.

Quantitative differences in the immunomodulatory effects of Rebif and Avonex in IFN-β 1a treated multiple sclerosis patients.

Interferon-β (IFN-β) is a current effective treatment for multiple sclerosis (MS) and exerts its therapeutic effects by down-modulating the systemic immune response and cytokine signaling. In clinical practice there are several formulations of interferon including a low dose of IFN-β 1a formulation of 30 μg IM once weekly (Avonex) and a high dose formulation of 44 μg SC three times weekly (Rebif). Recent studies suggest that Rebif is more efficacious compared to Avonex in preventing relapses and decreasing MRI activity in relapsing remitting MS (RRMS) patients.

Weight gain and recurrence in idiopathic intracranial hypertension: a case-control study.

Objective: To determine whether weight gain is associated with recurrence in idiopathic intracranial hypertension (IIH).

Methods: Medical records of adult patients with IIH seen between 1993 and 2009 at 2 university hospitals were reviewed to identify those with and without recurrence. Patients with documented height and weight at presentation and at subsequent visits were studied.

Nonvestibulocochlear cranial nerve schwannomas.

Nonvestibulocochlear cranial nerve schwannomas traditionally have been managed by surgical excision. Although debulking surgery is still considered the first treatment option for larger tumors, stereotactic radiosurgery is now preferred for smaller tumors because of its high tumor control rate and low treatment-related morbidity. Furthermore, an initial period of radiologic and clinical observation following the diagnosis should be strongly considered for smaller tumors because some may not grow or may grow at a slow rate.

Interferon-beta treatment in multiple sclerosis attenuates inflammatory gene expression through inducible activity of the phosphatase SHP-1.

Interferon-beta is a current treatment for multiple sclerosis (MS). Interferon-beta is thought to exert its therapeutic effects on MS by down-modulating the immune response by multiple potential pathways. Here, we document that treatment of MS patients with interferon beta-1a (Rebif) results in a significant increase in the levels and function of the protein tyrosine phosphatase SHP-1 in PBMCs.

Stereotactic radiosurgery in two cases of presumed fourth cranial nerve schwannoma.

A 47-year-old woman and a 45-year-old man with gradually progressive fourth cranial nerve palsy underwent stereotactic radiosurgery for presumed fourth cranial nerve schwannomas with the gamma knife at a marginal tumor dose of 14 and 13 Gy, respectively. In one patient, the ocular misalignment disappeared; in the other patient, it stabilized. MRI showed shrinkage of the tumors.

Late onset optic neuropathy in methylmalonic and propionic acidemia.

Purpose: To describe 3 cases of late-onset bilateral optic neuropathy with visual dysfunction in patients with organic acidemia.

Design: Retrospective case series.

Methods: A total of 3 subjects, a 16-year-old male with methylmalonic acidemia (MMA), a 21-year-old male with MMA, and a 20-year-old female with propionic acidemia (PA), are included in this series.

SHP-1 deficiency and increased inflammatory gene expression in PBMCs of multiple sclerosis patients.

Recent studies in mice have demonstrated that the protein tyrosine phosphatase SHP-1 is a crucial negative regulator of cytokine signaling, inflammatory gene expression, and demyelination in central nervous system. The present study investigates a possible similar role for SHP-1 in the human disease multiple sclerosis (MS). The levels of SHP-1 protein and mRNA in PBMCs of MS patients were significantly lower compared to normal subjects.

Ophthalmoplegia, proptosis, and lid retraction caused by cranial nerve hypertrophy in chronic inflammatory demyelinating polyradiculoneuropathy.

A 53-year-old woman with long-standing chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) developed progressive proptosis, lid retraction, and ophthalmoplegia. MRI showed enlarged, enhancing cranial nerves that initially gave rise to diagnostic confusion with Graves disease or orbitocavernous mass lesions. This report further documents that CIDP may cause hypertrophy of ocular motor and trigeminal nerves with imaging features that suggest alternative causes.