Erdheim-Chester Disease Masquerading as CLIPPERS.

Objectives: To present 4 patients with Erdheim-Chester disease (ECD) based on clinical, radiologic, histopathologic, and molecular genetic findings who had enhancing brainstem lesions and were initially believed to have chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).

Methods: Case series.

Results: Although patients with ECD can demonstrate clinical and imaging features similar to CLIPPERS, refractoriness to corticosteroids, lack of fulfillment of specific MRI criteria (i.

18 F-FDG Brain PET/MRI in Granulomatous Primary Angiitis of the Central Nervous System.

An 18-year-old man presented with encephalopathy, headache, tremor, and left hemiparesis. 18 F-FDG brain PET/MRI revealed pronounced hypometabolism in the right cerebral hemisphere corresponding to extensive T2/FLAIR signal abnormality, with accompanying miliary enhancement and microhemorrhages in this region. The differential diagnosis favored an autoimmune or inflammatory origin, rather than an infectious or neoplastic etiology.

Frequency, characteristics, predictors and treatment of relapsing myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD).

Background: Myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) may have a monophasic or relapsing disease course. To date, factors that may predict a relapsing disease course remain largely unknown and only limited data exist regarding the efficacy of different utilized immunotherapy regimens at preventing or reducing relapses.

Objectives: To assess the characteristics, predictors, and immunotherapy of relapsing MOGAD.

18 F-FDG Brain PET/MRI in Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids.

An 89-year-old man presented with progressive gait disturbance, diplopia, and ataxia. Initial brain MRI demonstrated T2/FLAIR hyperintense signal abnormality in the pons extending along the middle cerebellar peduncles into the cerebellum, with associated punctate, patchy, and linear enhancement on postcontrast imaging. Initially, this was attributed to brainstem encephalitis; however, sarcoidosis, histiocytosis, and paraneoplastic/autoimmune encephalitis remained on the differential.

Teaching NeuroImage: Unilateral Primary Angiitis of the CNS.

A 48-year-old woman was referred with an 18-year history of focal-onset seizures. She also reported years-long slowly progressive right-sided weakness that was corroborated on examination. Repeated brain MRIs over 15 years showed multifocal left hemispheric T2 fluid-attenuated inversion recovery-hyperintense lesions with patchy enhancement and microhemorrhages, no diffusion restriction, and a left cerebellar infarct (Figure 1, A-F).

Identifying specific myelopathy etiologies in the evaluation of suspected myelitis: A retrospective analysis.

Background: Myelopathies require prompt etiologic diagnosis. We aimed to identify a specific myelopathy diagnosis in cases of suspected myelitis to highlight clinicoradiologic differences.

Methods: In this retrospective, single-centre cohort of subjects with suspected myelitis referred to London Multiple Sclerosis (MS) Clinic between 2006 and 2021, we identified those with MS and reviewed the remaining charts for etiologic diagnosis based on clinical, serologic, and imaging details.

Prolonged Corticosteroids Without Maintenance Immunotherapy for Treatment of Anti-LGI1 Encephalitis: Analysis of Outcomes and Relapse Rate.

Objectives: To analyze outcomes and relapse rate of patients with anti-LGI1 encephalitis referred to the London Health Sciences Centre Autoimmune Neurology Clinic, where prolonged (≥3 months) corticosteroids without steroid-sparing maintenance immunotherapy are the typical treatment approach.

Methods: Retrospective chart review.

Results: Eighteen patients with anti-LGI1 encephalitis were identified.

Predictors of overlapping autoimmune disease in Neuromyelitis Optica Spectrum disorder (NMOSD): A retrospective analysis in two inner-city hospitals.

Background: The coexistence of Neuromyelitis Optica spectrum disorder (NMOSD) with other autoimmune diseases (AD-NMOSD) presents worse clinical outcomes and healthcare costs than NMOSD alone (NMOSD-only). NMOSD and other autoimmune diseases also have a higher prevalence and morbidity in Black. We aim to compare clinical features and treatment responses in NMOSD patients with and without overlapping autoimmunity in a predominantly Black cohort.

Testing for Antibodies Against Aquaporin-4 and Myelin Oligodendrocyte Glycoprotein in the Diagnosis of Patients With Suspected Autoimmune Myelopathy.

Autoimmune myelopathies are immune-mediated disorders of the spinal cord that can cause significant neurologic disability. Discoveries of antibodies targeting aquaporin-4 (AQP4-IgG) and myelin oligodendrocyte glycoprotein (MOG-IgG) have facilitated the diagnosis of autoimmune myelopathies that were previously considered to be atypical presentations of multiple sclerosis (MS) or idiopathic, and represent major advancements in the field of autoimmune neurology. The detection of these antibodies can substantially impact patient diagnosis and management, and increasing awareness of this has led to a dramatic increase in testing for these antibodies among patients with suspected autoimmune myelopathy.

Pearls & Oy-sters: Gait Instability, Jaw Dystonia, and Horizontal Diplopia in a Woman With Anti-Ri Antibodies and Breast Cancer.

A 40-year-old woman was admitted for 6 months of progressive gait disturbance, lower limb-predominant weakness, stiffness, falls, jaw dystonia, horizontal diplopia, and weight loss. Neurologic examination revealed horizontal gaze paresis, limited jaw opening with palpable masseter hypertrophy, and spastic paraparesis with sustained clonus and upgoing plantar responses. MRI revealed T2-hyperintense signal abnormalities in the dorsal pons, medulla, and upper cervical cord central gray matter extending to C3, without gadolinium enhancement.

The concurrence of multiple sclerosis and glioblastoma.

Introduction: Glioblastoma rarely coincides with multiple sclerosis. Although registries have reported a higher proportion of brain tumors-most of which are glial-these events appear to be underreported. The relative contribution of JC virus (an oncogenic virus) and disease modifying therapies that may facilitate JC virus neurotropism and tumor-specific immune evasion remain unknown.

Optical coherence tomography of patients with Parkinson's disease and progressive supranuclear palsy.

Objectives: To determine if Parkinson's disease (PD) and progressive supranuclear palsy (PSP) differed on retinal measurements using optical coherence tomography (OCT).

Patients And Methods: In a prospective, controlled, cross-sectional cohort study, we recruited patients with PD or PSP for more than three years, as well as control subjects. We measured peripapillary retinal nerve fiber layer (RNFL) thickness and macular volume using spectral-domain OCT.

SPARC expression by cerebral microvascular endothelial cells in vitro and its influence on blood-brain barrier properties.

Background: SPARC (secreted protein acidic and rich in cysteine) is a nonstructural, cell-matrix modulating protein involved in angiogenesis and endothelial barrier function, yet its potential role in cerebrovascular development, inflammation, and repair in the central nervous system (CNS) remains undetermined.

Methods: This study examines SPARC expression in cultured human cerebral microvascular endothelial cells (hCMEC/D3)-an in vitro model of the blood-brain barrier (BBB)-as they transition between proliferative and barrier phenotypes and encounter pro-inflammatory stimuli. SPARC protein levels were quantified by Western blotting and immunocytochemistry and messenger RNA (mRNA) by RT-PCR.

The Spectrum of Psychiatric Pathology in a Patient with Genetically Verified Huntington's Disease.

Psychiatric and behavioral disturbances are common in Huntington's disease (HD) and contribute significantly to its morbidity and mortality. We herein present the case of a 43-year-old woman with genetically verified HD, whose deteriorating psychiatric condition necessitated multiple inpatient psychiatric hospitalizations and featured a clinical spectrum of neuropsychiatric disturbances classically associated with HD. This paper reviews the literature concerning Huntington's psychopathology and provides an illustrative case example of its clinical nature.

The Role of Therapeutic Hypothermia After Traumatic Spinal Cord Injury--A Systematic Review.

Background: Traumatic spinal cord injury (SCI) is a devastating neurologic entity characterized by a primary insult followed by a secondary pathologic cascade that propagates further injury. Hypothermia has an established clinical role in preventing SCI after cardiac arrest and thoracoabdominal aortic aneurysm repair, yet its emergence as a potential neuroprotectant after spinal cord trauma remains experimental. There are currently no pharmacologic interventions available to prevent secondary mechanisms of injury after spinal cord trauma.

Metastatic Adenocarcinoma of Unknown Origin Presenting as Small Bowel Perforation: A Case Report and Literature Review.

Metastatic malignant tumors that originate from occult primaries are defined as "cancers of unknown origin." We herein present the case of a 59-year-old man who presented with small bowel perforation secondary to metastatic adenocarcinoma of an unknown primary site. Imaging exhibited two pulmonary nodules, neither of which was dominant, along with mediastinal and retroperitoneal lymphadenopathy.

Spectral-domain optical coherence tomography of retinal nerve fiber layer thickness in NMO patients.

Background: Neuromyelitis optica (NMO) is a demyelinating syndrome of the central nervous system. NMO might be underdiagnosed at early stages when patients have not yet developed the full spectrum of disease. The aim of this study was to analyze the retinal nerve fiber layer (RNFL) with optical coherence tomography (OCT) and to compare RNFL measurements between NMO patients, patients with relapsing-remitting multiple sclerosis (RRMS), and healthy controls to determine whether differences in RNFL thickness could be an early diagnostic marker for NMO.

Retinal nerve fiber layer thickness in benign multiple sclerosis.

Background/objective: Retinal nerve fiber layer (RNFL) thickness has been linked to brain atrophy in multiple sclerosis (MS). However, little is known about retinal atrophy in 'benign' MS. We compared RNFL thickness in benign MS with healthy controls.