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Biomedical subjects

Hannah R Briemberg

Publications and source records attributed to Hannah R Briemberg.

5 recordsLinked to original sources
PubMed2005

Interferon-alpha/beta-mediated innate immune mechanisms in dermatomyositis.

Dermatomyositis has been modeled as an autoimmune disease largely mediated by the adaptive immune system, including a local humorally mediated response with B and T helper cell muscle infiltration, antibody and complement-mediated injury of capillaries, and perifascicular atrophy of muscle fibers caused by ischemia. To further understand the pathophysiology of dermatomyositis, we used microarrays, computational methods, immunohistochemistry and electron microscopy to study muscle specimens from 67 patients, 54 with inflammatory myopathies, 14 with dermatomyositis. In dermatomyositis, genes induced by interferon-alpha/beta were highly overexpressed, and immunohistochemistry for the interferon-alpha/beta inducible protein MxA showed dense staining of perifascicular, and, sometimes all myofibers in 8/14 patients and on capillaries in 13/14 patients. Of 36 patients with other inflammatory myopathies, 1 patient had faint MxA staining of myofibers and 3 of capillaries. Plasmacytoid dendritic cells, potent CD4+ cellular sources of interferon-alpha, are present in substantial numbers in dermatomyositis and may account for most of the cells previously identified as T helper cells. In addition to an adaptive immune response, an innate immune response characterized by plasmacytoid dendritic cell infiltration and interferon-alpha/beta inducible gene and protein expression may be an important part of the pathogenesis of dermatomyositis, as it appears to be in systemic lupus erythematosus.

Adult↗
PubMed2005

Inflammatory neuropathies.

We discuss two of the most common of the acquired inflammatory neuropathies: Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, as well as their variants. We review their clinical presentation, electrophysiologic findings, and management, highlighting knowledge gained from the recent literature. Unfortunately, although treatments exist for both Guillain-Barre syndrome and chronic inflammatory demyelinating polyneuropathy, none are completely curative and all have significant potential side effects and/or expense. Better understanding of the underlying pathophysiology of these diseases is needed in order to develop more targeted therapies.

Guillain-Barre Syndrome↗
PubMed2004

Transthyretin amyloidosis presenting with multifocal demyelinating mononeuropathies.

We describe a patient with transthyretin amyloidosis who presented with multifocal mononeuropathies with features of demyelination on nerve conduction studies, a constellation of findings not previously described in amyloid polyneuropathy. Genetic testing revealed a valine122isoleucine mutation in the coding region of the transthyretin gene, a mutation that generally presents with late-onset cardiac amyloidosis. Our patient was also unusual in that she was 34 years old at the time of presentation and had no cardiac involvement.

Adult↗
PubMed2003

Neuromuscular complications of cancer.

Neuromuscular complications of cancer are common and can affect any component of the peripheral nervous system from peripheral nerve cell body to muscle. Perhaps the most common complication is a length-dependent symmetric axonal polyneuropathy that is often multifactorial in etiology, resulting from metabolic and treatment effects of the primary malignancy. However, neuromuscular disorders may also be the presenting complaint in many conditions, including disorders caused by malignant infiltration of nerve and disorders cause by paraneoplastic syndromes. Although many of the paraneoplastic conditions are poorly responsive to treatment, not all are, and one hopes that prompt diagnosis of the underlying malignancy will lead to improved patient outcome. Recognition of iatrogenic neuromuscular complications is also important to modify treatment protocols when possible and thus decrease the risk of long-term neurologic disability.

Biopsy↗