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E Simmons-O'Brien

Publications and source records attributed to E Simmons-O'Brien.

9 recordsLinked to original sources

Toxic neuropathy in patients with pre-existing neuropathy.

The authors report significant worsening of a pre-existing neuropathy in six patients who received "non-toxic" dosages of known neurotoxic agents. Before treatment, baseline total neuropathy score (TNS) averaged 9.5 (range 0 to 19). After chemotherapy (Taxol [125 to 175 mg/m(2) x 4]; vincristine [2 to 5 mg]; cisplatin [40 mg/m(2) x 8]; and thalidomide [60 g]), the TNS averaged 22 (range 13 to 29). The authors conclude that functionally disabling toxic neuropathy can occur in patients with pre-existing neuropathy at standard doses.

Adult↗

Thalidomide-induced neuropathy.

BACKGROUND: Thalidomide is effective for the treatment of some refractory dermatologic and oncologic diseases. Toxic neuropathy limits its use, as embryopathy can be avoided by contraceptive measures. OBJECTIVE: To describe the clinical, electrophysiologic, and pathologic features of thalidomide-induced peripheral neuropathy. METHODS: Clinical and electrophysiologic examinations were performed in seven patients with thalidomide-induced peripheral neuropathy. Thalidomide was used for graft-vs-host disease, pyoderma gangrenosum, and discoid lupus with dosages ranging from 100 to 1,200 mg/day for 5 to 16 months (cumulative dosages of 24 to 384 g). RESULTS: All seven patients had clinical and electrophysiologic evidence of a sensory more than motor, axonal, length-dependent polyneuropathy that presented as painful paresthesias or numbness. Sural nerve biopsies, done in three patients, showed evidence of Wallerian degeneration and loss of myelinated fibers. The symptoms, signs, and electrophysiologic data correlated with total cumulative dose of thalidomide. CONCLUSIONS: Thalidomide induces a dose-dependent sensorimotor length-dependent axonal neuropathy; it should be judiciously used with close neurologic monitoring.

Adult↗

Cronkhite-Canada syndrome: report of two cases, biopsy findings in the associated alopecia, and a new treatment option.

Two patients with Cronkhite-Canada syndrome (CCS) are reported, both of whom had diffuse alopecia, nail and skin changes, gastrointestinal polyposis, diarrhea, and wasting. A scalp biopsy was performed in one patient, and the specimen showed a marked noninflammatory loss of follicular units, miniaturization of the hair shafts, markedly dilated follicles, and a heavy deposition of glycosaminoglycans in the reticular dermis. This patient responded to prednisone therapy. The other patient was found to have elevated gastric acid levels and responded to ranitidine therapy. The conditions of both patients are now in remission two and six years later, respectively. Our patients have shown a temporally related remission of disease during treatment with prednisone and ranitidine, suggesting that each agent may be effective in CCS. However, randomized placebo-controlled trials are needed to prove the efficacy of these therapies. Further investigation of the histopathologic features of the associated alopecia may determine its cause.

Aged↗

Anti-Ro(SS-A) antibody positive Sjögren's/lupus erythematosus overlap syndrome.

Unlike anti-Ro(SS-A) antibody positive subacute cutaneous lupus erythematosus (SCLE) patients, anti-Ro(SS-A) antibody positive lupus patients with Sjögren's syndrome (SS/LE) appear to have a more guarded prognosis. Our studies indicate these SS/LE patients have an increased frequency of pulmonary, neurological, and renal disease compared to SCLE patients.

Adolescent↗

Significance of the anti-Ro (SS-A) antibody in evaluation of patients with cutaneous manifestations of a connective tissue disease.

The anti-Ro(SS-A) antibody is arguably the most important antibody determination except for antinuclear antibodies in evaluation of patients suspected of having lupus erythematosus. During the past 25 years, studies have established the importance of this antibody in the evaluation of patients with atypical lupus erythematosus, who have a photosensitive dermatitis as the presenting sign. The purpose of this review is to demonstrate the utility and the necessity of this antibody determination in the evaluation of all patients with cutaneous manifestations who are suspected of having a connective tissue disease.

Antibodies, Antinuclear↗

One hundred anti-Ro (SS-A) antibody positive patients: a 10-year follow-up.

To explore further the varied clinical expression of anti-Ro(SS-A) antibody positive patients and to determine the outcomes of these patients, we followed 100 anti-Ro(SS-A) antibody positive patients, originally seen at the Johns Hopkins Medical Institutions in 1982 and 1983, over a 10-year period. The results of this study indicate that anti-Ro(SS-A) antibody positive patients have a diverse clinical presentation and that the anti-Ro(SS-A) antibody response generally persists for years. Some of these patients appear to have a static disease process for years. However, 65% (51, including 13 deaths, of 78 patients) of the patients for whom we had follow-up data had a chronic (10 years or greater) progressive disease process. Black patients, in general, have an earlier onset of disease and may have a more severe disease than white patients. At least 25% of our anti-Ro(SS-A) antibody positive patients demonstrated a dynamic change in clinical presentation with the development of Sjögren syndrome and/or a progressive "rheumatoid-like" arthritis. Interstitial pulmonary disease, central nervous system disease, and vasculitic insults occur frequently in these patients. Renal disease occurred in 19 anti-Ro(SS-A) positive patients, and in 47% of these renal disease patients, no anti-DNA antibodies (dsDNA or ssDNA) were detected. Cutaneous manifestations are prominent in anti-Ro(SS-A) antibody positive patients with lupus. Photosensitivity and a malar dermatitis were the most common features. Twenty percent of lupus patients had discoid lesions, and 20% had SCLE lesions. Based on this study, we believe that anti-Ro(SS-A) antibody positive patients should be routinely evaluated for the emergence of systemic features. Since these systemic features are at least in part, if not solely, the result of inflammation, early treatment with steroids and/or immunosuppressive agents may minimize the damage and influence in a positive manner the significant morbidity and mortality observed in some anti-Ro(SS-A) antibody positive patients.

Adolescent↗

Hypopigmented mycosis fungoides.

BACKGROUND: Only five cases of purely macular hypopigmented mycosis fungoides have been published in the literature. These patients all had clinical evidence of disease 7 months to 10 years prior to histologic diagnosis, suggesting that this clinical presentation of mycosis fungoides is easily misdiagnosed. OBSERVATIONS: Three African-American males, ages 9, 15, and 22 years, were found to have mycosis fungoides on evaluation of skin biopsy specimens after initially being clinically diagnosed with, and treated for, pityriasis alba. All three had typical histopathologic changes and cell marker studies showing a relative loss of the CD7 antigen, which is normally present on the majority of T cells. One patient who was treated with methoxsalen plus UV light (PUVA) therapy had clearance of his disease clinically and histologically. CONCLUSION: From the literature, it would appear that macular hypopigmented mycosis fungoides is exceptionally rare. This perceived rarity may be inaccurate due to either relative underreporting, incorrect diagnoses, or both. From the eight patients now reported to date, it may be said that hypopigmented mycosis fungoides may be seen in nonwhite individuals and more often than not, has onset before age 20 years.

Adolescent↗