Brainstem encephalitis: etiologies, treatment, and predictors of outcome.

Brainstem encephalitis (BE) is an uncommon condition. We sought to characterize clinical presentations, etiologies, response to treatment, and predictors of outcome. We performed a retrospective review of non-HIV infected patients diagnosed with BE at Johns Hopkins Hospital (January 1997-April 2010). We characterized clinical and paraclinical features, and used regression models to assess associations with poor outcome. BE was diagnosed in 81 patients. An etiology was identified in 58 of 81 (71.6%) of cases, most of which were confirmed or probable inflammatory/autoimmune conditions. Of the remaining 23 cases in which a specific diagnosis remained undefined, clinical presentation, CSF, neuroimaging studies, and outcomes were similar to the inflammatory/autoimmune group. Brain biopsy identified a specific diagnosis in 7 of 14 patients (50%). Fifteen patients (18.5%) either died or had a poor outcome. In multivariate logistic regression models, a higher CSF protein (per 5 mg/dl, OR = 1.11, 95% CI: 1.03-1.20), a higher CSF glucose (per 5 mg/dl, OR = 1.36, 95% CI: 1.09-1.70), and higher serum glucose (per 5 mg/dl, OR = 1.27, 95% CI: 1.06-1.52) were independently associated with increased odds of poor outcome. Inflammatory and non-infectious conditions accounted for most cases of BE. Higher CSF protein and glucose were independently associated with poor outcome. In immunocompetent patients with BE of undefined etiology despite extensive investigation, a trial of immunosuppressive treatment may be warranted, though deterioration clinically or on magnetic resonance imaging should prompt a brain biopsy.

HIV-associated opportunistic infections of the CNS.

Survival in people infected with HIV has improved because of an increasingly powerful array of antiretroviral treatments, but neurological symptoms due to comorbid conditions, including infection with hepatitis C virus, malnutrition, and the effects of accelerated cardiovascular disease and ageing, are increasingly salient. A therapeutic gap seems to exist between the salutary effects of antiretroviral regimens and the normalisation of neurological function in HIV-associated neurocognitive disorders. Despite the advances in antiretroviral therapy, CNS opportunistic infections remain a serious burden worldwide. Most opportunistic infections can be recognised by a combination of characteristic clinical and radiological features and are treatable, but some important challenges remain in the diagnosis and management of HIV-associated opportunistic infections.

Peripheral nerve toxic effects of nitrofurantoin.

OBJECTIVE: To investigate the role of skin biopsy in nitrofurantoin peripheral neuropathy. DESIGN: We describe the clinical features and skin biopsies of 2 cases of non-length-dependent small-fiber neuropathy/ganglionopathy attributable to nitrofurantoin. SETTING: Clinical evaluation and skin biopsies were performed at a tertiary teaching hospital in Baltimore, Maryland. PATIENTS: A 59-year-old woman with disabling generalized dysesthesia and a 53-year-old woman with progressive burning pain in the perineum and extremities. MAIN OUTCOME MEASURES: Slow or incomplete recovery and possibly irreversible damage. RESULTS: The neuropathy was neither dose dependent nor associated with impaired renal function. Results from nerve conduction studies were normal. Skin biopsies revealed distinctive morphologic changes with clustered terminal nerve swellings without evidence of nerve fiber degeneration. CONCLUSIONS: These distinct morphologic changes associated with nitrofurantoin have not been previously reported to our knowledge. Skin biopsy appears to be helpful in confirming the diagnosis in these patients.

Neuromuscular complications in HIV: effects of aging.

There has been speculation that chronic HIV infection is a condition of accelerated aging that may lead to early onset of disease in multiple organ systems. The neuromuscular disorders of HIV, in particular distal symmetric polyneuropathy and myopathies, are also seen in the general population among older patients. As the HIV-infected population ages, there may be deleterious synergistic effects of age and chronic HIV infection on the brain, peripheral nerve, and muscle. In this review, we explore commonalities between the clinical features and putative mechanisms of neuromuscular disorders and HIV.

Severe Guillain-Barré syndrome following head trauma.

Fulminant forms of Guillain-Barré syndrome (GBS) present as acute onset tetraparesis and areflexia with absent brainstem reflexes, simulating brain death. Head trauma as an antecedent to fulminant GBS has been infrequently reported, and recognizing an association between GBS and head trauma may be crucial for patient management. Consequently, we report a patient with fulminant GBS with mixed demyelinating and axonal features preceded by a closed head injury, and discuss the possible pathophysiological mechanisms.