Severe axonal polyneuropathy with onset in the postpartum period

We report two patients who presented severe polyneuropathy in the postpartum period. Electrophysiological studies evidenced an axonal process which was associated with proximal demyelination in the second patient. In both cases, a peripheral nerve biopsy showed severe axonal Wallerian-like degeneration and no feature of demyelination. The first patient had a dramatic loss of myelinated fibres, and severe disability persisted for several months. These two patients are different from cases of acute or chronic inflammatory demyelinating polyradiculoneuropathy previously reported in relation with pregnancy.     

Neurogenic muscle hypertrophy in radiculopathy

The course of radiculopathy is sometimes associated with weakness and wasting of muscles. Very rarely in such cases, however, is hypertrophy of muscle fibres observed. Three cases are presented of sciatica with enlarged calves caused by hypertrophy of type 1 or types 1 and 2 muscle fibres. In light of the literature and the results obtained, an attempt is made to explain the cause of rare clinical symptoms and draw attention to diagnostic and therapeutic difficulties.     

Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe): presentation with a puzzling penile plaque*

Objective Report of two cases of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), with typical penile lesions. Differential diagnosis of persistent penile plaques. Background Following remission of the generalized eruption of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), it is not unusual to see a single remaining lesion localized on the penis. Since Sulzberger et al. (Sulzberger MB, Witten VH, Hunt JA. Puzzling persistent penile plaques. Arch Dermatol 1956:73:101-109) reviewed dermatoses presenting with puzzling persistent penile plaques in 1955, further dermatoses have evolved as important differential diagnoses. Much controversy has arisen as to the justification of exudative discoid and lichenoid dermatosis (Sulzberger-Garbe) as a disease entity. Conclusions Diagnosis of most cases of persistent plaques of dermatitis on the penis is possible with a careful examination of the entire skin, and a thorough history to discover past lesions. The possibility of malignancy makes a biopsy mandatory. Exudative discoid and lichenoid chronic dermatosis (Sulzberger-Garbe) typically responds well to systemic steroids. Intrale-sional triamcinolone acetonide has been demonstrated to be effective in the treatment of a persistent penile plaque in exudative discoid and lichenoid dermatosis (Sulzberger-Garbe), and may be helpful in discontinuing or reducing systemic therapy to a level compatible with long-term treatment.     

Nerve biopsy findings in Niemann-Pick type II (NPC)

The severe infantile form of Niemann-Pick disease type II was diagnosed in a 4-year-old girl and confirmed by demonstrating in cultured skin fibroblasts a deficiency of low-density lipoprotein-stimulated cholesterol ester synthesis of < 5% of normal. Electrodiagnostic studies revealed changes of a predominantly demyelinating motor and sensory polyneuropathy. Light microscope and ultrastructural examination of a peroneal nerve biopsy showed unique changes. Compacted myelin sheaths were disproportionately thin with marked globular irregularities in single teased nerve fibres and evidence of chronic demyelination. The majority of axons were preserved but axonal spheroids and cytoskeletal abnormalities akin to neuroaxonal dystrophy were noted. Membrane-bound multilobulated lysosomal inclusions of floccular and electron-dense material were present in Schwann cells (SC), endoneurial fibroblasts, macrophages, pericytes and endothelial cells. SC of myelinated fibres were stuffed with whorls of concentric osmiophilic membranous profiles and electron-lucent material. The findings are diagnostic and differ from those of classical Niemann-Pick disease.     

Should punch biopsies be used when high-grade disease is suspected at initial colposcopic assessment? A prospective study

The reliability and applicability of colposcopically directed cervical punch biopsy was assessed in a sample of 170 paired punch and large loop excision of cervical transformation zone (LLETZ) specimens obtained from previously untreated women who had been selected for treatment on the basis of cytology and/or colposcopic findings and in whom the entire cervical transformation zone was visible. A single punch biopsy was taken immediately before the LLETZ, and all the specimens were reviewed by a single pathologist. Nine (5.3%) punch biopsies were inadequate. In terms of whether or not there was cervical intraepithelial neoplasia (CIN), the chance-corrected kappa analysis rated overall agreement as poor (kappa = 0.21, 95% confidence limits 0.02-0.39), whereas in terms of histologic grade, it was fair to moderate (kappa = 0.32, 95% confidence limits 0.23-0.42). Punch biopsy tended to underestimate the disease. The sensitivity and specificity of colposcopically directed punch biopsy for the detection of high-grade CIN was 74% and 91%, respectively, with positive- and negative predictive values of 97% and 48%, respectively. Two microinvasive and two intraepithelial glandular lesions were missed on punch biopsy. Punch biopsy should be avoided when high-grade disease is suspected.     

Carcinoma of the papilla of vater: Are endoscopic appearance and endoscopic biopsy discordant?

Carcinoma of the papilla of Vater is classified as periampullary cancer representing 5% of all gastrointestinal tract malignancies. Early and accurate diagnosis is important for those patients with a tumor of the papilla, as the prognosis is more favorable than in other periampullary neoplasms. Endoscopically obtained biopsies from suspicious papillae can detect an early tumor, although even for skilled pathologists it is often difficult to differentiate carcinomas from noninvasive lesions on the basis of forceps biopsies. The purpose of this study was to assess the preoperative diagnostic accuracy of duodenoscopy appearance and biopsy in all cases with suspicion of tumor. Thirty patients with suspicion of carcinoma of the papilla of Vater and with final diagnosis established by pancreatoduodenectomy were included in this retrospective study. In each case, a comparison was made between endoscopic biopsy and duodenoscopic appearance. Duodenoscopic appearance sensitivity and accuracy for malignancy were 86% and 83%, respectively, whereas endoscopic biopsy sensitivity and accuracy were 65% and 67%, respectively. Although preoperative diagnosis of carcinoma of the papilla of Vater is useful for making therapeutic decisions, the diagnostic value of the endoscopic appearance was superior to endoscopic biopsy in this series. Presented at the 2003 American Hepato-Pancreato-Biliary Association Congress, Miami, Florida, February 27-March 3, 2003. Supported by FADA-CAPES/PROP 200J (M.L.D.).     

Pathological effects on mice by gambierol, possibly one of the ciguatera toxins

Gambierol was isolated from Gambierdiscus toxicus, which causes ciguatera fish poisoning. The acute toxicological effects induced in mice by synthesized gambierol were studied. The lethal doses were about 80 μg/kg by i.p. and i.v., and 150 μg/kg by p.o. The main injury by this toxin was observed in the lung, and secondary in the heart, resulting in systemic congestion. Another toxic effect was seen in the stomach, inducing hypersecretion and ulceration. With survival from the severe stage during the initial 3 h, recovery was favorable, especially after 4 days. Additional effects were not evident during 1-week post-administration observation.     

慢性非特异性溃疡性结肠炎中医研究述评

本文通过对古典医籍及近１０年文献有关慢性非特异性溃疡性结肠炎的资料分析，认为本病的证候特点与中医病名“休息痢”相符，病机归纳为脾虚为发病之本，湿热为致病之标，血瘀为局部病理变化，治疗方面得出了健脾益气化湿、活血化瘀解毒、托疮祛腐生肌等方法是治疗本病之关键的结论，提出分期治疗本病的思想，认为提高近期治愈率、降低复发率及做好癌前监测是今后研究的努力方向。     

少见肺部原发性恶性肿瘤七例报告

报告我院１９８０年－１９９３年诊治的７例少见肺部原发性恶性肿瘤，包括来源于间叶组织的２种肉瘤，来源于间胚叶软组织肿瘤，来源于气管粘膜腺体上皮的肿瘤，１例由原始生细胞向胚外组织分化而成的内胚窦瘤，全部病例经病理活组织检查证实，重点讨论诊断的体会。     

Human immunodeficiency virus (HIV)-related chronic relapsing inflammatory demyelinating polyneuropathy with multifocal unusual onion bulbs in sural nerve biopsy

Summary This is obviously the first report on a case with a spontaneous sensu strictu relapsing variant of human immunodeficiency virus (HIV)-related polyneuropathy. Its manifestation preceded LAS. Intrathecal HIV-antibodies developed between the most severe third and fourth episode. Analysis of sural nerve biopsy was consistent with a multifocally accentuated chronic inflammatory demyelination, characterized by unusual onion bulb-like Schwann cell formations with irregular voluminous layers, electron density, aggregation of filaments, multiple indented nuclei, and numerous enclosed collagen pockets. A direct or immune-mediated indirect specific influence on Schwann cell morphology by HIV might be discussed. Virus-like particles and ultrastructural markers of HIV were not detectable.