Epileptic negative myoclonus in a newborn with hemimegalencephaly

PURPOSE: We report the case of a male newborn with Ohtahara syndrome and right hemimegalencephaly who presented epileptic negative myoclonus in the first days of life. METHODS: Prolonged polygraphic studies were performed, as well as MRI and a full clinical examination. RESULTS: EEG showed a constant and nonreactive pattern of burst suppression. There were several kinds of electro-clinical seizures (generalized myoclonia, short atonias, typical spasm and tonic spasms) at the beginning of the EEG's burst. The periods of EMG silence, lasting less than 300 ms, were associated with stereotyped EEG transients. CONCLUSIONS: Epileptic negative myoclonus can be observed also in neonatal age. The short transient impairment of motor function observed in the newborn seems linked to the slow component of spike-wave discharge, but its mechanism is still not clear.     

Histoplasmosis in two human immunodeficiency virus-positive immigrants to Italy: clinical features and management in the highly active antiretroviral therapy era

We report two cases of histoplasmosis occurring in human immunodeficiency virus-positive patients who immigrated to Italy, and focus our attention on the clinical features and therapeutic aspects, with particular emphasis on secondary prophylaxis. The patients had comparable human immunodeficiency virus baseline parameters, but had a completely different compliance over therapeutic regimens. The two patients were followed in two different city hospitals of our region, Padua and Verona, and the diagnosis was made on the basis of instrumental, histologic, and microbiologic findings. One of them was treated with corticosteroids because of nephrotic syndrome.     

The synthesis of glycoprotein Po and peripheral myelin protein 22 in sciatic nerve of male rats is modulated by testosterone metabolites

Glycoprotein Po (Po) and peripheral myelin protein 22 (PMP22) are two proteins playing a crucial physiological role in the maintenance of the multilamellar structure of peripheral myelin. We here demonstrate that the removal of circulating androgens by orchidectomy induces a significant decrease of the synthesis of Po and PMP22 in the rat sciatic nerve. In case of Po, this effect may be counteracted by the subsequent treatment with testosterone metabolites, dihydrotestosterone or 5alpha-androstan-3alpha,17beta-diol (3alpha-diol). Experiments have been consequently performed in order to evaluate the role of androgen receptor (AR) in the control of Po synthesis. In vivo treatment with flutamide (i.e., an antagonist of AR) induces a decrease of the synthesis of this myelin protein in the sciatic nerve of intact male rats confirming a role for this steroid receptor. On the contrary, PMP22 seems not to be under the control of AR, but a role for GABAA receptor may be proposed. This concept is based on the findings that: (a) only 3alpha-diol, which is able to interact with GABAA receptor, is effective in stimulating the synthesis of PMP22 in the sciatic nerve of castrated male rats, and (b) flutamide treatment is ineffective in decreasing the protein levels in intact male rats. The observations here reported clearly show similarities and dissimilarities with the effects exerted by other members of neuroactive steroid family, like for instance progesterone derivatives, which will be discussed in text.     

Localized cutaneous hyalohyphomycosis caused by a Fusarium species infection in a renal transplant patient

Fusariosis is a hyalohyphomycosis due to Fusarium species that mainly occurs in immunocompromised hosts. The clinical spectrum of Fusarium infection comprises localized and disseminated forms. A case of localized cutaneous fusariosis caused by Fusarium solani in a renal transplant patient is described, and the skin manifestations of the disease are discussed.     

Identification of the antibacterial component of an ethanolic extract of the Australian medicinal plant, Eremophila duttonii

Activity-guided fractionation was used to determine the antibacterial component of an ethanolic extract of the leaves of an Australian native medicinal plant, Eremophila duttonii F. Muell. (Myoporaceae). The extract, previously shown to have activity against Gram positive bacteria, was shown to have activity against additional Gram positive bacteria, including Clostridium perfringens, C. sporogenes and Listeria monocytogenes. Thin layer chromatography (TLC) was used to separate the extract into seven coloured fractions in visible light, one of which was shown by bioautography to contain antibacterial activity. Recovery of the component from the TLC plate and testing for antibacterial activity using a plate-hole diffusion assay supported this result. The purity of the component was verified by high-performance liquid chromatography and a time-kill experiment indicated that the purified component showed identical bactericidal activity to the whole extract. TLC spray reagents indicated that the component was a sterol, terpene or sugar but not a flavonoid, while the pigmented nature suggested a carotenoid.     

Papular xanthoma associated with angiokeratoma of Fordyce: considerations on the nosography of this rare non-Langerhans cell histiocytoxanthomatosis

BACKGROUND: Papular xanthoma (PX) is a rare normolipidemic non-Langerhans cell histiocytoxanthomatosis affecting both children and adults. OBJECTIVE: We describe an adult case of PX associated with angiokeratoma of Fordyce and review the literature in order to compare and discuss previous reports. METHODS: We studied the clinical, histopathological, immunocytochemical and ultrastructural findings. RESULTS: We report the findings of our case and compare our case with those described in the literature. CONCLUSIONS: Three clinical patterns of PX appeared to emerge in the review of the literature: a self-healing form, a persistent form and a progressive form. The progressive form of PX can be considered the same clinical entity that is also described as progressive nodular histiocytosis.     

Acute mediastinitis: a severe complication of transdermal therapy in a patient with ischaemic heart disease. A case report

Acute mediastinitis is an uncommon and potentially devastating infection. Generally, mediastinitis occurs as a postoperative infection following median sternotomy. We describe a case of acute mediastinitis in a patient with ischaemic heart disease, secondary to the spread of cutaneous infection of the chest wall in the application site of transdermal nitroglycerine patches.