Neuropathy associated with “benign” anti-myelin-associated glycoprotein IgM gammopathy: Clinical,immunological, neurophysiological pathological findings and response to treatment in 33 cases

We studied 33 patients presenting with a peripheral neuropathy associated with non-malignant anti-myelin-associated glycoprotein (MAG) IgM monoclonal gammopathy (MG) in an attempt to delineate their clinical, immunological, electrophysiological and pathological characteristics; we also reviewed our experience concerning long-term follow-up and therapy. Peripheral neuropathy associated with non-malignant anti-MAG IgM MG was observed mostly in males (sex ratio 7.2), and mean age at onset was 67 years (range 46–81). A predominantly sensory pattern was noted in more than 80% of cases, although some patients were affected by a predominantly motor peripheral neuropathy. Although disease progression was slow in most cases, 45% of patients suffered severe disability, and in 2 cases, the patient's death appeared to stem directly from the neuropathy. The electrophysiological findings were indicative of a demyelinating process in 90% of cases, and electron microscopic examination of nerve biopsy specimens demonstrated widening of the myelin lamellae in more than 95% of cases. Most of our patients showed a disappointing response to steroids and chemotherapy or plasma exchanges. Intravenous immune globulin, evaluated in 17 patients, had a transient, mostly subjective effect in 35% and led to a clear-cut improvement in 24% of cases. We did not observe any correlation between the severity of the clinical picture and the anti-sulphoglucuronyl paragloboside antibody titre; in individual cases, clinical improvement occurred without lowering of IgM levels. Although the severity and the rate of progression may greatly vary from patient to patient, the combination of clinical, electrophysiological and pathological features delineates a characteristic pattern in peripheral neuropathy associated with non-malignant anti-MAG IgM MG.     

Extrinsic compression of the renal artery by diaphragmatic crus

Extrinsic compression of the renal artery due to a fibromuscular band originating from the diaphragm was encountered in a 26-year-old patient who had systemic hypertension associated with stenosis and kinking of one of her renal arteries. After surgical decompression, the renal artery assumed a normal expansion with disappearance of hypertension. Six other cases of extrinsic compression of the renal artery have been found in the literature. Surgical treatment is mandatory in all cases because the mechanism that causes the lesion makes percutaneous transluminal angioplasty illusory.     

The Intubation Difficulty Scale (IDS): Proposal and Evaluation of a New Score Characterizing the Complexity of Endotracheal Intubation

Background: A quantitative scale of intubation difficulty would be useful for objectively comparing the complexity of endotracheal intubations. The authors have developed a quantitative score that can be used to evaluate intubating conditions and techniques with the aim of determining the relative values predictive factors of intubation difficulty and of the techniques used to decrease such difficulties.

Methods: An Intubation Difficulty Scale (IDS) was developed, based on parameters known to be associated with difficult intubation. It was then evaluated prospectively in a group of 311 consecutive prehospital intubations and 315 intubations in an operating room. In the operating room, the IDS was compared with two other parameters: the time to completion of intubation and the visual analog scale (VAS). Time was measured by an independent observer. Operators in both groups completed a checklist regarding the conditions of intubation.

Results: There is a good correlation between the IDS scale and the VAS assessment of difficulty and time to completion of intubation. VAS and time to completion have a significant but lesser correlation to each other. Comparison of IDS with operator-assessed subjective categorical impression of difficulty by Kruskall-Wallis was statistically significant.     

Cervicofacial Rhytidectomy: The Superficial Plane

During the past 20 years, numerous techniques have been described to improve the results of facelifting as well as the duration of its rejuvenating effect. These increasingly aggressive techniques have increased the operating time, the potential morbidity of the operation, and the duration of convalescence. No one can ensure that this evolution in sophistication of rhytidectomy techniques give longer lasting results. Therefore, a simple technique applied under the appropriate indications gives good and predictable results.     

Adverse events in phase one studies: a study in 430 healthy volunteers

Summary All the clinical, laboratory and electrocardiographic adverse events detected during 24 Phase I studies in the same unit over a 5 y period are reported here. 430 healthy male volunteers were involved, corresponding to 5488 days of follow-up.The overall incidence of adverse events was 13.5%, with a significant difference between active drug (15.3%) and placebo (7.4%) treatments. There were 69 distinct types of adverse events. Headache was the most frequent symptom (2%). There were severe adverse events in 20 cases (0.36%), with an incidence of 20/430 per subject (4.6%). There were no deaths or life-threatening events.Although the main objective of Phase I studies is to determine the maximum dose tolerated, cause-effect relationships with adverse events are hard to establish, because of the frequency of adverse events with placebo, and because of the limited number of subjects included such studies.     

Eighteen-month Coronal Caries Incidence in North Carolina Older Adults

In this longitudinal study of a random sample of North Carolinians over the age of 65 and living in their homes, 325 blacks and 280 whites were examined and interviewed 18 months after baseline examinations. Coronal caries incidence was greater among whites than blacks. The increment due to teeth becoming root fragments were similar for both races; however, there were more newly crowned teeth among whites. Newly crowned surfaces were not used as part of the caries increment in logistic regression models to investigate potential risk predictors. For blacks, caries development over the 18-month period was associated with a higher lactobacillus score and more coronal caries at baseline, more previously filled coronal surfaces, and lack of active membership in clubs or other groups. For whites, having no self-reported tooth sensitivity, having a lower socioeconomic index score, taking antihistamine medications at baseline, and having the perception of more problems after the age of 40 than before were all associated with the development of coronal caries.     

Autosomal dominant centronuclear myopathy]

In a family 6 members in 3 generations were affected by centronuclear myopathy (CNM) of autosomal dominant inheritance. The apparent onset was in the early forties and the disease progressed slowly. Limb weakness was predominant. Strabismus was present in 5 cases and calves hypertrophy in 3. Serum creatinine kinase was always within the normal range. In one case myotonic bursts were found at electromyography. In 2 cases brain stem auditory evoked potential studies demonstrated abnormal prolongation of interpeak latencies I-III and favoured subclinical nervous system involvement. Muscular biopsies showed typical features of centronuclear myopathy with 50 to 80% central nuclei. In two cases immunocytochemical labelling of dystrophin showed staining in the sarcoplasm in favour of an arrest in the morphogenesis of developing myofiber. Others families with autosomal dominant CNM in the literature and also some sporadic adult cases had similar clinical features.     

Simultaneous occurrence of acute myelogenous leukemia and seminoma of the testis

Summary Simultaneous tumors are rarely encountered during the course of acute leukemias. We report on a case of seminoma of the testis that occurred during the evolution of acute myelogenous leukemia. To our knowledge, this stimultaneous association has not previously been described, but a causal relationship was not apparent in the present case. The likelihood of a common carcinogenesis existed, but direct exposure to carcinogens could not be established. Although the results of a physical examination and echography were normal at the time of diagnosis, we cannot exclude the presence of microscopic cancer of the testis. Since the dissemination pattern of seminoma is usually slower than that observed in this case and the disease remains limited to the lymph nodes for long periods following dissemination, the rapid development of the present case might have been attributable to the immunosuppression and the scrotal sepsis that occurred during the induction therapy. Immunosuppression might have stimulated the progression of a primary microscopic seminoma and the development of metastasis, whereas the scrotal sepsis and inflammation might have favored the occurrence of metastasis through bypass of the lymphatic barrier.