Patients affected by a new variant of endemic pemphigus foliaceus have autoantibodies colocalizing with MYZAP, p0071, desmoplakins 1-2 and ARVCF, causing renal damage.

BACKGROUND: We have previously reported that about 30% of patients affected by a new variant of endemic pemphigus foliaceus (EPF) in El Bagre, Colombia (termed El Bagre-EPF or pemphigus Abreu-Manu) have systemic compromise. In the current study, we focused on studying autoreactivity to the kidney and its pathological correlations. AIM: To investigate patients with El Bagre-EPF for renal compromise. METHODS: We performed a case-control study, enrolling 57 patients with El Bagre-EPF and 57 controls from the endemic area, matched by age, sex, race, work activity, demographics and comorbidities. We took skin and renal biopsies; performed direct and indirect immunofluorescence, immunohistochemistry (IHC), confocal microscopy, immunoblotting, direct and indirect immune electron microscopy; and tested kidney function in all living patients. We also used IHC to study seven kidney autopsy samples. RESULTS: Of the 57 patients, 19 had autoantibodies to kidney, with polyclonal reactivity (P < 0.01). Most cases were positive along the basement membrane of the proximal tubules, but in some cases there was also positivity against the glomeruli and/or mixed patterns. Fifteen patients had increases in serum urea and creatinine compared with controls (P < 0.01). The autoantibodies colocalized with commercial antibodies to desmoplakins I and II, p0071, armadillo repeat gene deleted in velo-cardio-facial syndrome (ARCVF) and myocardium-enriched zonula occludens-1-associated protein (MYZAP) (P < 0.01). All of the kidney disease autopsies showed alterations, mostly in the vessels. CONCLUSION: We demonstrate for the first time that one-third of patients with El Bagre-EPF have polyclonal autoantibodies to kidney. The kidneys showed a mixed histological pattern resembling lupus nephritis, with a diffuse proliferative Class IV (G) global diffuse pattern in active lesions, and additional interposition of membranoproliferative glomerulonephritis.

Temporal pleomorphic xanthoastrocytoma with glycogen accumulation--case report.

Pleomorphic xanthoastrocytoma (PXA) is a rare superficial glioma that predominates in the young and has good prognosis. A long history of repeated seizures is commonly associated with PXA, which is frequently observed in neuroimaging scans as a solid-cystic, contrast-enhancing lesion. We report a case in which PXA diagnosis was favored by its histological features, such as pleomorphic multinucleated giant cells, with disproportionately few mitoses and necrotic areas. An eye-catching feature was widespread, pale-staining, circumscribed deposits in the cytoplasm of tumor cells, which turned out to be glycogen upon histochemical and electron-microscopical examination. The stored material was strongly PAS-positive and digested by diastase, and had a finely granular ultrastructural appearance. No evidence of lipid droplets was found on oil-red-O staining. The tumor was immunoreactive for glial fibrillary acidic protein and vimentin. Many cells were positive for CD34 on the external membrane, a feature which has been described in chronic CNS lesions associated with epilepsy. Intracytoplasmic immunostaining for EGFR was observed in most tumor cells, which might have favored neoplastic proliferation. Nuclear immunolabeling for p53 protein was rare and does not support a major role for p53 mutation in PXA tumorigenesis. Intracellular accumulation of glycogen in glial tumors is uncommon and may originate from abnormalities in carbohydrate metabolic pathways.

Classification of the uptake hydrogenase-positive (Hup+) bean rhizobia as Rhizobium tropici.

Phenotypic and genetic characterization indicated that Hup+ bean rhizobial strains are type IIA and type IIB Rhizobium tropici. The Hup+ strain USDA 2840, which did not cluster with either of the two types of R. tropici in a restriction fragment length polymorphism analysis, had electrophoretic patterns of PCR products generated with primers for repetitive extragenic palindromic and enterobacterial repetitive intergeneric consensus sequences similar to those of three reference strains of R. tropici type IIA. The Hup+ strain USDA 2738, which clustered with the reference strain of R. tropici IIB in a restriction fragment length polymorphism analysis, had electrophoretic patterns of PCR products generated with primers for repetitive extragenic palindromic and enterobacterial repetitive intergeneric consensus sequences more closely resembling those of the reference strains of R. tropici type IIA than those of type IIB. DNA amplification with the Y1 and Y2 primers to generate a portion of the 16S rDNA operon was useful to distinguish R. tropici type IIA strains from other bean rhizobial strains. The phylogenetic position of the type IIA strain of R. tropici USDA 2840, determined from the partial 16S rDNA sequence, indicated a more distant relationship with the type IIB strain of R. tropici CIAT899 than with the as yet unnamed rhizobial species of Leucaena leucocephala, TAL 1145. Therefore, we suggest that it may be appropriate either to separate R. tropici types IIA and IIB into two different species or to identify TAL 1145 to the species level as a third type of R. tropici.

Uptake Hydrogenase (Hup) in Common Bean (Phaseolus vulgaris) Symbioses.

Strains of Rhizobium forming nitrogen-fixing symbioses with common bean were systematically examined for the presence of the uptake hydrogenase (hup) structural genes and expression of uptake hydrogenase (Hup) activity. DNA with homology to the hup structural genes of Bradyrhizobium japonicum was present in 100 of 248 strains examined. EcoRI fragments with molecular sizes of approximately 20.0 and 2.2 kb hybridized with an internal SacI fragment, which contains part of both bradyrhizobial hup structural genes. The DNA with homology to the hup genes was located on pSym of one of the bean rhizobia. Hup activity was observed in bean symbioses with 13 of 30 strains containing DNA homologous with the hup structural genes. However, the Hup activity was not sufficient to eliminate hydrogen evolution from the nodules. Varying the host plant with two of the Hup strains indicated that expression of Hup activity was host regulated, as has been reported with soybean, pea, and cowpea strains.

[Spinal cord cysticercosis: report of 2 cases, review of the literature, and comments on its pathogeny]. / Cisticercose medular. Relato de dois casos, revisão da literatura e comentários sobre a patogenia.

A case of spinal intramedullary cysticercosis in a 13-year-old Brazilian female and a case of spinal leptomeningeal infestation by cysticercosis in a 51 year-old Brazilian female are presented. A review of 95 published cases of medullar cysticercosis since 1856 shows the incidence of this condition. Extramedullary forms are explained by the downward migration of larvae from the cerebral to the spinal subarachnoid space and most larvae are expected to be stopped in the upper portions of the spinal canal due to peculiarities of the anatomy of the spinal cord.

[Pure spinal leptomeningeal cysticercosis]. / Cisticercose espinhal leptomeníngea pura.

An adult female patient presented with an exclusively spinal leptomeningeal infestation by cysticercosis (restricted to the cauda equina), verified in surgery. The rôle of the vertebral vein system in the spread of cysticercosis larvae is discussed. A commentary on the possibility that cysticercosis of the basal cisterns may be due to ascending migration of primarily spinal cysticerel, as originally proposed by Isamat de la Riva, is stated.

Cisticercose espinhal leptomeníngea pura / Pure spinal leptomeningeal cysticercosis

Relato de um caso com verificaçäo cirúrgica de cisticercose espinhal, forma leptomeníngea restrita à cauda eqüina, em paciente adulto. E discutido o papel do sistema venoso vertebral na infestaçäo dos espaços subaracnóideos na neurocisticercose, originando formas clínicas primariamente espinhais

[Lumbosacral neoplastic radiculopathy]. / Radiculopatia neoplásica lombossacral.

Lumbar-disc protrusions (LDP) constitute well-defined syndromes on clinical and anatomical grounds, and neurosurgeons are prone to rely upon the clinical signs to identify the level of disc protrusion when a "typical" case is found. Sometimes, non-contrasted computerized tomographic (CT) scans centered on the L5-S1, L4-L5 and L3-L4 interspaces and spine roentgenograms are the only special ancillary means in presurgical evaluation. We report three patients from our series, in which neoplastic spinal disease presented as classic LDP (one patient with a cauda equina schwannoma, and two with metastatic carcinoma). The cases were chosen because they posed special problems to the referred radiodiagnostic routine. Systematic CT-evaluation of the sacrum and conus medullaris zone is recommended in every patient with lumbosacral radiculopathy, and intrathecal contrast should be employed in patients with unreliable findings or normal CT-scans.

An arachnoid cyst extending above and below the foramen magnum.

A case of progressive bulbar and long tract symptoms with hydrocephalus of eleven years duration in a 23-year-old man, in whom an arachnoid cyst of the cisterna magna cerebellomedullaris was disclosed at operation.

So-called tethered cervical spinal cord.

A case is reported of the so-called Tethered Cervical Spinal Cord Syndrome presenting as multiradicular pain on an upper limb in a 45-year old woman, in whom spina bifida occulta (C6) was associated with an intramedullary lipoma, intradural fibrous adhesions and the fusion of the sixth and seventh cervical vertebrae. Surgical release of the tether was ineffective, and the patient underwent a posterior rhizotomy for pain relief. The clinical and maldevelopmental aspects of this most uncommon form of spinal cord tethering are discussed briefly.