POEMS syndrome as an uncommon cause of papilledema.

BACKGROUND: Polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is an uncommon condition related to a paraneoplastic syndrome secondary to an underlying plasma cell disorder. Among the myriad of manifestations of the disease, ocular signs and symptoms are relatively prevalent, affecting about half of all patients with the disease. OBJECTIVE: To report the ocular manifestations of POEMS syndrome. CASE: A 47-year-old lady diagnosed to have POEMS syndrome presented with painless progressive visual diminution. Her color vision was impaired. There was bilateral papilloedema. CONCLUSION: POEMS syndrome should be considered among the differential diagnoses of all patients with a bilateral papilledema in which no other cause can be readily elucidated.

Horse chestnut (Aesculus hippocastanum L.) starch: Basic physico-chemical characteristics and use as thermoplastic material.

Starch isolated from non-edible Aesculus hippocastanum seeds was characterized and used for preparing starch-based materials. The apparent amylose content of the isolated starch was 33.1%. The size of starch granules ranged from 0.7 to 35 µm, and correlated with the shape of granules (spherical, oval and irregular). The chain length distribution profile of amylopectin showed two peaks, at polymerization degree (DP) of 12 and 41-43. Around 53% of branch unit chains had DP in the range of 11-20. A. hippocastanum starch displayed a typical C-type pattern and the maximum decomposition temperature was 317 °C. Thermoplastic starch (TPS) prepared from A. hippocastanum with glycerol and processed by melt blending exhibited adequate mechanical and thermal properties. In contrast, plasticized TPS with glycerol:malic acid (1:1) showed lower thermal stability and a pasty and sticky behavior, indicating that malic acid accelerates degradation of starch during processing.

Daytime sleepiness in Parkinson's disease: perception, influence of drugs, and mood disorder.

Parkinson's disease (PD) is associated with sleep complaints as excessive daytime sleepiness (EDS) and several factors have been implicated in the genesis of these complaints. Objective. To correlate the subjective perception of EDS with variables as the severity of the motor symptoms, medications, and the presence of depressive symptoms. Materials and Methods. A cross-sectional study, using specific scales as Epworth sleepiness scale (ESS), Beck depression inventory (iBeck) and Hoehn and Yahr (HY), in 42 patients with PD. Results. The patients had a mean age of 61.2 ± 11.3 years and mean disease duration of 4.96 ± 3.3 years. The mean ESS was 7.5 ± 4.7 and 28.6% of patients reached a score of abnormally high value (>10). There was no association with gender, disease duration, and dopamine agonists. Patients with EDS used larger amounts of levodopa (366.7 ± 228.0 versus 460.4 ± 332.25 mg, P = 0.038), but those who had an iBeck >20 reached lower values of ESS than the others (5.9 ± 4.1 versus 9.3 ± 4.8, P = 0.03). Conclusions. EDS was common in PD patients, being related to levodopa intake. Presence of depressed mood may influence the final results of self-assessment scales for sleep disorders.

Obstructive sleep apnea severity correlates with cellular and plasma oxidative stress parameters and affective symptoms.

Obstructive sleep apnea syndrome (OSAS) is considered a sleep-related respiratory disorder, characterized by repetitive episodes of complete (apnea) or partial (hypopnea) obstruction of airflow in the upper airway (UA) during sleep. The pathophysiology of upper airway obstruction in OSAS is multifactorial, leading to a chronic recurrent state of intermittent hypoxemia and reoxygenation during sleep, maintaining a state of oxidative stress, which seems to be the key to the pathophysiological manifestations of OSAS, and is associated with the development of a number of high morbidity-mortality systematic complications, such as obesity, type 2 diabetes, metabolic syndrome, and cardiovascular and neuropsychological diseases. This study is an open, cross-sectional, and comparative clinical trial, whose general objective was to assess the correlation between OSAS severity, oxidative stress markers, and the presence of affective symptoms (depressive and anxious) in OSAS patients. We studied 38 adult males, who had been diagnosed with OSAS by overnight polysomnography, between 18 and 60 years of age, divided into three groups: group 1-10 individuals with mild OSAS (AHI between 5 and 14.9/h), group 2-13 individuals with moderate OSAS (AHI between 15 and 30/h), and group 3-15 individuals with severe OSAS (AHI >30/h). All individuals were evaluated for level of subjective sleepiness using the Epworth Sleepiness Scale, for depressive and anxiety symptoms by the Hamilton Depression (HAM-D) and Anxiety (HAM-A) Scales, and for parameters of the oxidative stress state, measuring superoxide radical and serum nitrates and nitrites levels. There was a progressive and significant increase in the state of oxidative stress (p < 0.05), in the total score of depressive symptoms (p = 0.001) and in the overall score of anxiety symptoms (p = 0.004) directly proportional to the severity of apnea when comparing the mild group to the severe group. Positive correlations were identified between superoxide production and the apnea-hypopnea index (AHI) (r = 0.48), Epworth sleepiness score (r = 0.36), and Hamilton depression score (HAM-D) (r = 0.40); between serum nitrates and nitrites levels and SO(2) min (r = 0.44); and between the AHI and the HAM-D (r = 0.51) score and HAM-A (r = 0.40) score. Negative correlations were observed between the AHI and serum nitrates and nitrites levels (r = -0.42), between superoxide production and SO(2) min (r = -0.31), between serum nitrates and nitrites levels and HAM-D (r = -0.50) and HAM-A (-0.42) scores, and between SO(2) min and HAM-D (r = -0.48) and HAM-A (r = -0.40) scores. According to the results of this study, we can conclude that (1) individuals with OSAS show an increase in the production of superoxide radical and a decrease in serum nitrates and nitrites levels, which are objective signs of a state of oxidative stress. (2) The more severe the OSAS, the more fragmented the sleep and the greater the nocturnal hypoxemia, the more severe is the oxidative stress state and the greater is the incidence of daytime symptoms, especially sleepiness and depressive and anxiety symptoms. Future studies might explore the investigation of oxidative stress parameters as an alternative approach to anticipate symptoms, measure prognosis, and monitor OSAS progression or treatment response.

Plasmodium carmelinoi n. sp. (Haemosporida: Plasmodiidae) of the lizard Ameiva ameiva (Squamata: Teiidae) in Amazonian Brazil.

Plasmodium carmelinoi n. sp. is described in the teiid lizard Ameiva ameiva from north Brazil. Following entry of the merozoites into the erythrocyte, the young, uninucleated trophozoites are at first tear-shaped and already possess a large vacuole: with growth, they may assume an irregular shape, but eventually become spherical or broadly ovoid. The vacuole reduces the cytoplasm of the parasite to a narrow peripheral band in which nuclear division produces a schizont with 8-12 nuclei. At first the dark, brownish-black pigment granules are restricted to this rim of cytoplasm but latterly become conspicuously concentrated within the vacuole. The mature schizonts are spherical to ovoid and predominantly polar in their position in the erythrocyte. They average 5.4 x 4,9 microm (4.4 x 4.4 - 6.6 x 5,9 microm), shape index 1.1, n = 50: 8-12 merozoites are produced and measure approximately 2.0 x 1,0 microm. Mature gametocytes are also polar in position, and spherical to subspherical. The macrogametocytes measure 5.7 x 5,2 microm (4.4 x 4.0- 5.9 x 5,1 microm), shape index 1.1, n = 50 and, following staining by Giemsa's method, possess a compact, pink-staining nucleus and a clear blue, faintly stained cytoplasm. Microgametocytes are slightly larger, 6.0 x 5,0 microm (5.2 x 4.4 - 6.2 x 5,2 microm), shape index 1.2, n = 45. They stain an over-all pink colour due to the dispersed nuclear chromatin. The vacuoles in both the macro- and microgametocytes are considerably smaller than those of the schizonts and of ovoid or spindle shape: they contain most of the pigment granules. The sex ratio, as seen in an inicial intense infection, was 1 male to 2.2 females. Prevalence of infection was low (5%) but, due to the very low parasitaemia which may result in a failure to detect parasites, it is probably higher than this.

Trypanosoma (Megatrypanum) saloboense n. sp. (Kinetoplastida: Trypanosomatidae) parasite of Monodelphis emiliae (Marsupiala: Didelphidae) from Amazonian Brazil.

Trypanosoma (Megatrypanum) soloboense n. sp., is described in the Brazilian opossum Monodelphis emiliae (Thomas, 1912) from primary forest in the Salobo area of the Serra dos Carajás (6 degrees S, 50 degrees 18' W) Pará State, North Brazil. Two morphologically different trypomastigotes were noted. Slender forms, regarded as immature parasites, have a poorly developed undulating membrane adhering closely to the body: large, broad forms with a well developed membrane are considered to be the mature trypomastigotes and have a mean total length of 71.2 microm (62.4-76.2) and a width of 6.1 (5.0-8.0). Infections studied in two opossums were of very low parasitaemia. The large size of T. (M.) saloboense readily distinguishes it from the two previously described members of the subgenus Megatrypanum of neotropical marsupials, T. (M.) freitasi Régo et al., 1957 of Didelphis ozarae and D. marsupialis, and T. (M.) samueli Mello, 1977 of Monodelphis domesticus, which measure only 49.0-51.5 microm and 42.4 microm respectively. No infections were obtained in hamsters inoculated with triturated liver and spleen from one infected M. emiliae, or in laboratory mice inoculated with epimastigotes from a blood-agar culture. No division stages could be detected in the internal organs or the peripheral blood.

Production of cyclodextrins by CGTase from Bacillus clausii using different starches as substrates.

Cyclodextrins (CDs) are cyclic oligasaccharides composed by D-glucose monomers joined by alpha-1,4-D glicosidic linkages. The main types of CDs are alpha-, beta- and gamma-CDs consisting of cycles of six, seven, and eight glucose monomers, respectively. Their ability to form inclusion complexes is the most important characteristic, allowing their wide industrial application. The physical property of the CD-complexed compound can be altered to improve stability, volatility, solubility, or bio-availability. The cyclomaltodextrin glucanotransferase (CGTase, EC 2.4.1.19) is an enzyme capable of converting starch into CD molecules. In this work, the CGTase produced by Bacillus clausii strain E16 was used to produce CD from maltodextrin and different starches (commercial soluble starch, corn, cassava, sweet potato, and waxy corn starches) as substrates. It was observed that the substrate sources influence the kind of CD obtained and that this CGTase displays a beta-CGTase action, presenting a better conversion of soluble starch at 1.0%, of which 80% was converted in CDs. The ratio of total CD produced was 0:0.89:0.11 for alpha/beta/gamma. It was also observed that root and tuber starches were more accessible to CGTase action than seed starch under the studied conditions.

New species of Eimeria and Isospora (Protozoa: Eimeriidae) in Geochelone spp. (Chelonia: Testudinidae) from Amazonian Brazil.

Tetrasporocystic, dizoic oocysts of reptiles have been separated by some authors into the genera Eimeria, Choleoeimeria and Acroeimeria (Protozoa: Eimeriidae), based on the site and mode of development of their endogenous stages. The majority of Eimeria species have been, and still are, however, described on oocyst morphology alone. Four different oocysts with this basic morphology were encountered in the faeces of Brazilian tortoises, Geochelone carbonaria Spix, 1824 and are assigned to the genus Eimeria, with the view that they can readily be transferred to the genus Choleoeimeria or Acroeimeria if this is indicated by a future examination of their endogenous development. A morphological comparison distinguishes the oocysts from those of Eimeria spp., previously described in chelonids of the family Testudinidae, and the names E. amazonensis, E. carbonaria, E. carajasensis and E. wellcomei n. spp. are proposed. Coccidial infection appears to be common in G. carbonaria, with three of seven animals examined passing oocysts. Oocysts of Isospora rodriguesae n. sp. (Protozoa: Eimeriidae) are described in the faeces of Geochelone denticulata Linnaeus, 1766. They are morphologically very different from those of Isospora testudae, Davronov, 1985 in Testudo horsfieldi. Eimeria motelo Hurková et al., 2000, previously described in Geochelone denticulata from Peru, is here recorded in the some chelonid from Amazonian Brazil.

Natural and experimental infection of the lizard Ameiva ameiva with Hemolivia stellata (Adeleina: Haemogregarinidae) of the toad Bufo marinus.

Developmental stages of a haemogregarine in erythrocytes of the lizard Ameiva ameiva (Teiidae), from Pará State, north Brazil, were shown to be those of Hemolivia by the nature of the parasite's sporogonic cycle in the tick Amblyomma rotondatum. The type species, Hemolivia stellata Petit et al., 1990 was described in the giant toad Bufo marinus and the tick Amblyomma rotondatum, also from Pará State, and in view of the fact that A. ameiva and Bufo marinus share the same habitat and are both commonly infested by A. rotondatum, the possibility that the parasite of A. ameiva is H. stellata had to be considered. Uninfected lizards fed with material from infected ticks taken from B. marinus, and others fed with liver of toads containing tissue-cysts of H. stellata, were shown to subsequently develop typical Hemolivia infections, with all stages of the development similar to those seen in the naturally infected lizards. Conversely, a juvenile, uninfected toad became infected when fed with sporocysts of Hemolivia in a macerated tick that had fed on an infected A. ameiva and pieces of liver containing tissuecysts from the same lizard. The remarkable lack of host specificity shown by H. stellata, in hosts so widely separated as an amphibian and a reptile, is discussed.

MRI of medulloblastoma in adults.

Medulloblastoma has variable appearances on MRI in both children and adults. Adults are more likely to have heterogeneous cerebellar hemisphere tumours, and this is thought to be related to the greater prevalence of desmoplastic tumours in adulthood. Few studies have addressed the MRI features of adult medulloblastoma and the specific characteristics of desmoplastic and classic tumours have not been analysed. Our aim was to analyse the imaging characteristics of desmoplastic (DM) and classic (CM) medulloblastomas in adult. We retrospectively studied preoperative MRI of six men and three women, median age 33 years, range 23-53 years, with pathologically proved medulloblastomas. There were six (67%) with DM. The tumour was in the cerebellar hemisphere in eight patients (89%), including the three with CM, one of which was bilateral. All tumours were heterogeneous, giving predominantly low or isointense signal on T1- and isointense signal on T2-weighted images. Cystic or necrotic areas in all patients were particularly visible on T2-weighted images. Contrast enhancement was absent in one DM and varied from slight to intense in eight (three CM), homogeneous in one DM and patchy in seven. All tumours extended to the surface of the cerebellum and two had well-defined margins. MRI does not allow a clear distinction between DM and CM in adults.

[Multiple gliomas. Illustrative cases of 4 different presentations]. / Gliomas múltiplos: casos ilustrativos de quatro formas de apresentação.

Multiple gliomas are uncommon and may be classified according to: a) the time of presentation in early (at diagnosis) or late (during treatment); b) the characteristics of computed tomography or magnetic resonance imaging (CT/MRI) in multifocal (with evidence of spread) and multicentric (without evidence of spread). From 212 patients with histopathologic diagnosis of glioma evaluated from March/90 to September/99, 15 (7%) had multiple lesions. We describe 4 patients: early multicentric, late multicentric, early multifocal and late multifocal, with emphasis on characteristics of CT/MRI and possible differential diagnosis. The differential diagnosis of multiple lesions in the central nervous system includes mainly infectious/inflammatory diseases and metastasis, however multiple gliomas should always be considered, even in patients with known systemic cancer, as described by others. Considering that CT/MRI features are not definite, the diagnosis should always be confirmed by histopathologic examination.

Moyamoya disease. Report of three cases in Brazilian patients.

Moyamoya disease (MMD) is a chronic occlusive cerebrovascular disease of unknown etiology reported mainly in the Japanese. Most cases occur in children. The disease is rare in non-Oriental adults manifesting itself mostly as intracerebral hemorrhages. We describe MMD in 2 non-Oriental young adults and one adolescent that developed cerebral infarctions. The adults were medicated with aspirin and no medication was given to the adolescent. All patients did not deteriorate in a follow-up period from 1 to 4 years. Although rare, MMD is an important cause of stroke in young individuals and may well be underreported: only 18 patients have been reported till 1997 in Brazil. Neurologists should include MMD in differential diagnosis of ischemic and hemorrhagic strokes in young adults.

Clinical orodental abnormalities in Mexican children.

A total of 32,022 Mexican children (16,473 boys, 15,549 girls) were examined for several congenital oral and paraoral anomalies. The findings for commissural lip pits (boys 53.1, girls 52.4 per 1000) are less than those reported for adults. This may indicate that pits become accentuated with age. Fordyce granules were seen with a prevalence of 1.2 per 1000. This is in contrast to the reported 85.6% prevalence for the adult population, also possibly reflecting increased manifestation with increased age. Our data for exogenous tooth pigmentation show increased prevalence with age (group I [5 to 10 1/2 years], 9.8%, versus group II [10 1/2 to 14 1/2 years], 12.9%), possibly indicating decrease in attention to oral hygiene. The prevalence of talon cusp was found to be 0.6 per 1000, and for ankyloglossia 8.3 per 1000. Prevalence values for bifid tongue are reported for the first time, indicating one affected per 187 children examined. The prevalence of fissured tongue (15.7%) shows a statistically significant difference between boys (16.8%) and girls (14.5%). The prevalence of geographic tongue (1.9%) shows a marked difference between group I (2.2%) and group II (1.2%).