Failure of IVIG in steroid-responsive autoimmune glial fibrillary acidic protein astrocytopathy: A case report.

OBJECTIVE: To advocate for high-dose steroids, not intravenous immunoglobulins (IVIG), as first-line treatment for Anti-glial fibrillary acidic protein (GFAP) associated meningoencephalomyelitis. BACKGROUND: A novel IgG antibody against GFAP was associated with relapsing autoimmune meningoencephalomyelitis. DESIGN/METHODS: Here, we present an investigational case report to highlight continuing challenges in diagnosing and managing Anti-GFAP associated meningoencephalomyelitis. RESULTS: Our 45-year-old Asian female presented to the emergency department with an acute onset low-grade fever and back pain associated with headaches, intermittent confusion, vision changes, and hand tremors. A review of systems identified no inciting factors. Past medical history was significant only for chronic Hepatitis B without significant viral load. Neurological exam was significant for decreased visual acuity, high-frequency hand tremor, and gait imbalance. Serum labs were within normal limits. Video electroencephalogram captured tremors without electrographical correlates. Cerebrospinal fluid analysis revealed lymphocytic leukocytosis, elevated protein, and reduced glucose. A wide range of infectious studies including bacterial, viral, and fungal cultures were negative. MRI brain and spine showed leptomeningeal enhancement. CT chest abdomen pelvis were negative. Patient continued to decline clinically, working diagnosis was possible paraneoplastic syndrome with pending laboratory results. She was given a five-day course of intravenous immunoglobulin as a therapeutic trial,hh however, her symptoms did not improve. A broader investigation with repeat lumbar puncture, imaging and serum laboratory failed to provide any additional information. She was treated symptomatically with minimal benefit. A trial of steroids was given with clinical improvement and continued stability. Paraneoplastic panels returned positive for high levels of Anti-GFAP antibody for confirmation of diagnosis. CONCLUSIONS: Autoimmune GFAP astrocytopathy is a rare cause of meningoencephalomyelitis that remains difficult to diagnose despite emerging laboratory studies. Our case adds to the limited literature by proposing that high-dose steroids, not IVIG, should be the first-line treatment. Further investigations are underway to assess implications of this finding in disease pathophysiology and management.

Symptomatic therapy in multiple sclerosis.

Multiple sclerosis is the most common disabling neurological disease of young adults. The ability to impact the quality of life of patients with multiple sclerosis should not only incorporate therapies that are disease modifying, but should also include a course of action for the global multidisciplinary management focused on quality of life and functional capabilities.

Functional treatments in multiple sclerosis.

PURPOSE OF REVIEW: This review focuses on recent advances in the understanding and management of symptoms and dysfunctions associated with multiple sclerosis (MS). RECENT FINDINGS: A broad spectrum of dysfunctions associated with MS are under investigation. Research published in the past year and a half addresses gait dysfunction, exercise training, fatigue, bowel/bladder and sexual dysfunction, and sleep disruption. Functional electrical stimulation and strength training have been validated for improvement in gait and motor function. Exercise training has been shown to benefit mood and quality of life scores and to reduce circulating inflammatory cytokine levels. Fatigue remains a challenging problem with incremental improvements in understanding of underlying causes and effective drug therapies offered by recent work. Treatment of bowel, bladder and sexual dysfunction utilizing a variety of modalities has been investigated with some progress. SUMMARY: In the absence of treatments to reverse neurologic injury due to MS, effective symptom management and functional improvement remain essential to mitigate disability and maintain quality of life. Basic research, as well as controlled clinical trials, in this realm offers promising insights and solutions.

Multiple sclerosis.

Multiple sclerosis is the most common disabling neurologic disease affecting young adults and adolescents in the United States. The first objective of this article is to familiarize nonspecialists with the cardinal features of multiple sclerosis and our current understanding of its etiology, epidemiology, and natural history. The second objective is to explain the approach to diagnosis. The third is to clarify current evidence-based treatment strategies and their roles in disease modification. The overall goal is to facilitate the timely evaluation and confirmation of diagnosis and enhance effective management through collaboration among primary physicians, neurologists, and other care providers who are confronted with these formidably challenging patients.