Prion Disease.

Prion diseases are a phenotypically diverse set of disorders characterized by protease-resistant abnormally shaped proteins known as prions. There are three main groups of prion diseases, termed sporadic (Creutzfeldt-Jakob disease [CJD], sporadic fatal insomnia, and variably protease-sensitive prionopathy), genetic (genetic CJD, fatal familial insomnia, and Gerstmann-Straussler-Scheinker syndrome), and acquired (kuru, variant CJD, and iatrogenic CJD). This article will review the pathophysiology, genetics, clinical presentations, and diagnostic challenges in patients with prion disease.

Human Chronic Necrotizing Granulomatous Meningoencephalitis: A Novel Case Report.

Necrotizing and granulomatous meningoencephalitis are common central nervous system diseases known to affect canines. To date, necrotizing granulomatous meningoencephalitis has yet to be described in humans. Current studies of presumed pathogenesis and possible treatment options have only been described in canines.

Diagnostic Approach to Chronic Meningitis.

Chronic meningitis is defined as cerebrospinal fluid pleocytosis that persists for at least 4 weeks without spontaneous resolution. The differential diagnosis of chronic meningitis is broad, encompassing 4 main categories, including infectious, autoimmune, neoplastic, and idiopathic. Up to one-third of cases have no discernible cause, making chronic meningitis a diagnostic dilemma for many clinicians.

Herpesvirus Infections of the Nervous System.

Purpose Of Review: This article reviews the spectrum of neurologic disease associated with human herpesvirus infections.

Recent Findings: As more patients are becoming therapeutically immunosuppressed, human herpesvirus infections are increasingly common. Historically, infections with human herpesviruses were described as temporal lobe encephalitis caused by herpes simplex virus type 1 or type 2.

Mutation in KIF5A c.610C>T Causing Hereditary Spastic Paraplegia with Axonal Sensorimotor Neuropathy.

Hereditary spastic paraplegias (HSP) are a rare heterogeneous group of inherited neurodegenerative diseases characterized by progressive lower extremity spasticity and weakness. Mutations of the kinesin family member 5A (KIF5A) gene lead to a spectrum of phenotypes ranging from spastic paraplegia type 10 to Charcot-Marie Tooth Disease type 2. We report the second known case of a mutation in the KIF5A gene at c.

Chronic Autoimmune Meningoencephalitis and Periodic Fever Syndrome Treated with Anakinra.

We report the case of a 63-year-old male diagnosed with chronic autoimmune mediated meningoencephalitis and periodic cyclical fever syndrome. After 2 years of failed conventional treatment and recurrent hospitalizations, anakinra treatment (an interleukin-1 [IL-1] receptor antagonist) was trialed which resulted in full recovery. As evidenced by this case, anakinra can be used to treat chronic autoimmune meningitis, which can often be refractory to treatment.

A rare case of rapidly progressive dementia with elevated RT-QuIC and negative 14-3-3 and tau proteins.

Creutzfeldt-Jakob disease (CJD) is characterized by rapidly progressing dementia with death usually occurring within 6 months. There is no verified disease-specific pre-mortem diagnostic test besides brain biopsy. We describe a 66 y old previously high functioning male who presented with a 5 month history of rapidly progressive dementia.

Evaluation and treatment of chronic meningitis.

Chronic meningitis is defined as an inflammatory cerebrospinal fluid (CSF) profile that persists for at least 1 month. The presentation often includes headache, nausea, vomiting, cranial neuropathies, symptoms of elevated intracranial pressure, or focal neurologic deficits. The most common etiologies of chronic meningitis fall into 3 broad categories: infectious, autoimmune, and neoplastic.

Progressive multifocal leukoencephalopathy in patients with multiple sclerosis.

Purpose Of Review: Progressive multifocal leukoencephalopathy (PML) is a rare opportunistic infection of the central nervous system. It has been recently associated with selective immunosuppression in patients with multiple sclerosis. This review describes the pathogenesis, clinical presentation, diagnosis, and treatment of natalizumab-associated PML.

Chronic and subacute meningitis.

Purpose Of Review: This article describes the background, clinical presentation, diagnosis, and treatment of selected etiologies of subacute and chronic meningitis. Key diagnostic considerations when evaluating a patient presenting with chronic inflammation of the CNS are discussed, and several specific infectious, neoplastic, and autoimmune etiologies are reviewed in detail.

Recent Findings: With recent advancement in serologic and CSF diagnostic testing, specific infectious, neoplastic, or autoimmune etiologies of chronic meningitis can be identified.

Neurologic emergencies in patients who have cancer: diagnosis and management.

The nervous system can be significantly affected by cancer. Neurologic symptoms are present in 30% to 50% of oncologic patients presenting to the emergency department or in neurologic consultation at teaching hospitals. Evaluation and treatment require collaborative effort between specialties.

Neuroinfectious diseases in pregnancy.

Pregnancy is associated with impaired T-cell-mediated immunity putting the woman at risk for infectious diseases not typically seen in immunocompetent individuals. When maternal infection is severe, and when a primary herpesvirus infection is acquired during pregnancy, there is a risk of fetal loss, maternal death, neonatal death, or congenital abnormalities from intrauterine infection. In this review, the authors emphasize specific neurologic infectious diseases that occur in pregnancy, the safety of the antimicrobial therapy of these diseases during pregnancy, as well as the congenital and neonatal sequelae of infection.