Serum and CSF immunological findings were analysed in 37 patients with amyotrophic lateral sclerosis (ALS). ALS patients had significantly higher mean values of serum IgG and complement component C4 and significantly lower mean value of total haemolytic titre of complement (THC) compared with normal controls. Incidence of immune complexes (ICs) was significantly higher in sera of ALS patients than in normal controls. There was no significant difference regarding mean serum levels of IgM, IgA, and complement components C3 and Factor B between patients and controls. The blood-brain barrier (BBB) damage was found in 46% of patients. Intrathecal IgG synthesis was detected in six patients (16%). These results support the hypothesis of immune system involvement in ALS.

The effect of right ventricular pacing on left ventricular relaxation was studied in 13 patients (age 62 +/- 3 years), with the atrial sensing ventricular pacing mode (VDD). A control group of similar age (64 +/- 4 years) consisted of 11 patients with atrial pacing (AAI). The timing of events was determined in both groups at similar R-R intervals (921 +/- 77 ms vs 967 +/- 37 ms). The loading conditions as estimated by peak systolic wall stress (afterload) and end-diastolic left ventricular dimensions (preload) were approximately the same in both groups. The ratio of late to early filling velocities were similar in both groups. Dominant changes were: increased preejection period (142 +/- 13 ms vs 95 +/- 15 ms); and higher velocities of isovolumic relaxation flow (60 +/- 34 cm/s vs 25 +/- 4 cm/s) in patients with ventricular pacing. The isovolumic relaxation time was longer in patients with VDD pacing (127 +/- 14 ms vs 108 +/- 12 ms). Anterior systolic interventricular septal motion (paradoxal motion) was recorded in nine patients with VDD pacing and in none of the patients with AAI pacing. Isovolumic relaxation flow was detected during atrial pacing in five (45%) patients and in 13 (100%) patients during atrial sensing ventricular pacing, indicating asynchronous left ventricular relaxation. This data shows that VDD pacing compared to atrial pacing resulted in an altered activation pattern of the left ventricle, associated with delayed onset, asynchronous contraction with interventricular septal motion abnormalities and prolonged asynchronous left ventricular relaxation with abnormal motion manifested by the presence of isovolumic relaxation flow.

Neurofi bromatosis-1 (NF1) is one of the most common hereditary multisystemic disorders. The disease manifests a variety of characteristic features that include: hyperpigmentary abnormalities of the skin (café-au-lait macules, freckles in the axillae, and iris Lisch nodules) and growth of benign peripheral nerve sheath tumors (neurofi bromas) in the skin. Associated extracutaneous clinical features include: skeletal abnormalities, neurological, cardiovascular, endocrine and other malformations. NF1 is caused by mutation in the neurofi bromatosis-1 gene, which codes for the protein neurofi bromin. The inheritance of NF1 follows an autosomal dominant trait, although about 50% of patients present with new ("de novo") mutations, and represent the fi rst member of their family. No difference in the severity of the disease can be found in patients with familial mutations versus those with new mutations. We present a 78-year-old female patient with an extreme cutaneous form of neurofi bromatosis who reported no affected family member. Apart from skin problems, she had no major health issues in childhood and adolescence, but in recent decades she had frequent headaches, occasional abdominal pain, and vision and hearing impairment. About 10 to 14 days before admission, she developed a severe cough, shortness of breath, and chest and abdominal pain. On examination, the patient of short stature (hight: 152 cm, weight: 49 kg) presented with thousands of soft nodules dispersed over the whole body, except on extensor sides of thighs and lower legs; the nodules varied in color from skin-colored, livid erythematous, to brown-grey; the nodules on the abdomen were moist, partly bleeding from the base, and accompanied by an unpleasant odor. Her feet were also densely covered by dark purple lumps, with dystrophic changes of the toe nails that were thickened, frayed, and yellowish. The skeletal abnormalities included: short stature, severe osteoporosis and osteosclerosis of the head bone structure; degenerative arthropathc-spondylotic changes of the thoracolumbar spine segment with signs of diffuse skeletal hyperostosis; pronounced degenerative changes of the lumbar spine. CT scans of the head, chest and abdomen showed the following abnormalities: fl attening of the paraventricular gyri and reduction of brain parenchyma with hypodensity of the white matter in terms of cortical atrophy; periventricular bilateral small post-ischemic microvascular brain lesions of varying chronicity; in the parenchyma of the upper left lung lobe the apical presence of small areas of pleural effusion with consequent subatelectic region; distended stomach and a small inner wall herniation; hypotrophic right kidney; atherosclerotic lesions of the abdominal aorta; low grade infrarenal kinking of the abdominal aorta. Pathohistological analysis of biopsy specimen taken from the nodule corresponded with cutaneous neurofi broma. Consultative examinations of various specialists pointed to the existence of the following comorbidities: obstructive respira...

We present a 46-year-old non-atopic HIV-negative woman from Doboj, Republic of Srpska, Bosnia and Herzegovina, who was referred to the Department of Dermatovenereology, Clinical Center Banja Luka, Republic of Srpska, Bosnia and Herzegovina, with a 3-month long history of an erythematous, large indurated infl amed area on the upper arm. The condition was asymptomatic, immediately following surgical excision of a small tumor. After exlusion of pulmonary blastomycosis and other organ involvement, the diagnosis of primary inoculation cutaneous blastomycosis was made based on clinical presentation and histopathological fi ndings. Histopathology revealed thick-walled, rounded, budding yeasts with broad-based buds that stained pink with periodic acid-schiff (PAS) staining. Itraconazole therapy was initiated at a dose of 2x100 mg/day. After three months of therapy, the dose of itraconazole was increased to 2x200 mg/day during the next three months, and then the dose was reduced to 2x100 mg. Blastomycosis is an uncommon, chronic granulomatous and suppurative mycosis caused by Blastomyces dermatitidis, which belongs to the group of main endemic systemic mycoses and in the great majority of cases represents a primary pulmonary disease. Few sporadic cases have been reported in Europe. There are three forms of blastomycosis: primary cutaneous, pulmonary and disseminated. B. dermatitidis has rarely been isolated from the environment. Wood debris or land close to rivers or subject to fl ooding are considered to be the natural substrate. The fungus can grow in sterile soil in the laboratory, and it is believed that humans get infected by inhaling spores from a saprophytic source. Primary cutaneous blastomycosis is very rare and it is found in farmers and laboratory workers. Human to human transmission does not normally occur. The diagnosis of the skin lesions is made by direct microscopy of skin samples (e.g., pus, scrapings) with 10% potassium hydroxide mount and confi rmed by culture or biopsy. Histopathological analysis provides identifi cation of all the dimorphic fungi. However, this can be complicated by the fact that in some cases they can be morphologically atypical or sterile. In the tissues, B. dermatitidis produces characteristic thick-walled, rounded, refractile, and spherical budding yeasts with broad-based buds. Of the available antimycotic drugs, itraconazole 200 mg/day is probably the most effective, but at least 400 mg/day is redommended initially.

Imiquimod is a local immunomodulator with antiviral effects. Vulvar intraepithelial neoplasia is a chronic precancerous condition of the skin of the vulva, with different malignant potential and clinical course. The aim of the paper was to determine therapeutic effects of Imiquimod in treating different types and grades of vulvar intraepithelial neoplasms. The study enrolled 17 patients with vulvar pre-cancerous conditions of different grade and histological type. The patients were treated with combined medical therapy oral systemic immunomodulatory and antiviral drug-inosine acedoben dimepranol and 5% Imiquimod cream locally applied to the lesion area using cotton swabs. Complete remision (CR) had 41.18% of patients, partial remission (PR) was seen in 47.06%, and 11.76% of patients had no response (NR). Out of these patients, response distribution for usual type was: CR 80%, 20% NR, and for differentiated type the response distribution was: 8.3% NR, 66.67% PR, while 25% of patients had CR. The use of imiquimod for conservative treatment of vulvar intraepithelial neoplasia is a beneficial alternative to surgical treatment. The best results of imiquimod treatment are achieved in younger patients with usual type of vulvar neoplasia, while the treatment effects are limited to partial response in older patients with differentiated VIN.