Presence of crystalline inclusions in the peripheral nerve of a patient with IgA lambda monoclonal gammopathy of undetermined significance

Association of a peripheral neuropathy with an IgA monoclonal gammopathy of undetermined significance (MGUS) is not commonly observed and is sometimes considered as coincidental. We present a case in which the nerve biopsy revealed the presence of crystalline inclusions in the endoneurium, a very unusual finding. A 75‐year‐old man complained of paresthesiae in both feet and unsteady gait for 6 months. He had no weakness, but deep tendon reflexes were absent and vibratory sensation distally diminished in both legs. An IgA lambda MGUS was evidenced in his serum at 10.2 g/L with 7% plasma cells in his bone marrow and no lytic lesion at skeletal examination. A superficial peroneal nerve biopsy was performed and showed numerous crystalline inclusions in the endoneurium. These were located in the cytoplasm of macrophagic histiocytes or free in the vicinity of nerve fibers. There was also a marked loss of myelinated nerve fibers and several “onion bulb” formations surrounding either isolated remyelinating fibers or small clusters of remyelinating fibers. Such crystalline inclusions have mainly been observed in the cytoplasm of plasma cells in cases of multiple myeloma, and correspond to non‐secreted IgA or IgG immunoglobulins with a kappa or rarely lambda light chain. Such inclusions have also been reported in the cytoplasm of the epithelial cells from corneal fragments, in patients with multiple myeloma or IgG MGUS, and in the tubular cells from the kidney of patients with multiple myeloma and a nephrotic syndrome. In the literature, there is only one very briefly mentioned case of neuropathy associated with a myeloma and with crystalline inclusions present in the epineurium. Thus, in dysglobulinemic neuropathy, nerve fibers can be damaged by three kinds of interstitial deposits, easily identified by immunohistochemistry and at ultrastructural examination: the well known amyloid fibrils, granulo‐fibrillar deposits and also crystalline inclusions.     

Coherence treatment of postmenopausal osteoporosis with growth hormone and calcitonin

Summary Fourteen women with postmenopausal osteoporosis, all having at least one vertebral crush fracture, were randomly assigned to two treatment arms, each lasting 24 months. The coherence treatment group (7 patients) was treated in the following sequence: human growth hormone (hGH) 7 IU subcutaneously daily for 2 months, followed by 3 months of salmon calcitonin (CT), 100 MRC units every other day. After a 3 month rest period, this sequence was repeated twice. The contrast group (7 patients) was treated intermittently with salmon CT given in the same time periods and at the same dose as in the coherence treatment group. Bone mass was measured every 4 months by neutron activation analysis for total body calcium (TBCa) and by single photon absorptiometry for bone mineral content (BMC) of the distal radius. Although there were no significant differences between the two groups (two-way ANOVA), the rate of change in TBCa in the coherence treatment group was significantly different from zero (F=3.8,P<.05) and was +2.3%/year. The increase in bone mass appeared to be sustained throughout the 2 year study, in contrast with previous studies where a plateau effect was observed with calcitonin given alone or continuously with growth hormone. No significant change was found in bone histomorphometric values measured before and after treatment in 4 patients from each group.     

Increased Dosage of DYRK1A and Brain Volumetric Alterations in a YAC Model of Partial Trisomy 21

A yeast artificial chromosome (YAC) transgenic murine model of partial trisomy 21 overexpressing five human genes—including DYRK1A, which encodes a serine threonine kinase involved in cell cycle control—has been shown to present an increase in brain weight. We analyzed this new phenotype by measuring total and regional brain volumes at different ages, using a 7 Tesla magnetic resonance imaging volumetric approach. Volumetric measurements showed a total volume increase of 13.6% in adult mice. Changes in brain morphogenesis were already visible at a very early postnatal stage (postnatal days 2–7). Region‐specific changes were characterized from postnatal day 15 to 5 months. These results, made it possible to define region‐specific effects of DYRK1A overexpression, with the strongest increase seen in the thalamus–hypothalamus area (24%). Anat Rec, 291:254–262, 2008. © 2008 Wiley‐Liss, Inc.     

Physical Activity, Physical Fitness, and Coronary Heart Disease Risk Factors in Youth: The Québec Family Study

Objective. The relationships between physical activity, fitness, and CHD risk factors were investigated in 342 males and 268 females 9–18 years of age.Methods. Daily energy expenditure, moderate to vigorous physical activity, inactivity, and television viewing time were estimated. Indicators of physical fitness included submaximal work capacity, quadriceps muscle strength, sit-ups, and the sum of six skinfolds. Risk factors included mean arterial blood pressure and fasting blood levels of triglycerides, LDL-cholesterol, HDL-cholesterol, and glucose.Results. Canonical correlations between activity and risk factor profiles range from 0.22 to 0.45, while those between fitness and risk factor profiles range from 0.34 to 0.55, indicating that 5 to 20% and 11 to 30% of the variance in the risk profile is explained by activity and fitness, respectively.Conclusion. The results suggest that both physical fitness and level of habitual physical activity are strongly associated with CHD risk factors in this sample of youth.     

Subungual Leukemia Cutis

A 79-year-old man had generalized humping of the nails and subungual tumors involving several fingers and the left big toe. Findings on histologic study of the tumor, together with the pattern on the peripheral blood smear, established the diagnosis of chronic lymphocytic leukemia. To the best of our knowledge, this is the first reported case of chronic lymphocytic leukemia involving the subungual area. The authors' observation emphasizes the importance of taking a biopsy specimen in the differential diagnosis of subungual tumors.     

Deathbed scenes as imagined by the young and experienced by the old

This study compares the deathbed scenes anticipated by students enrolled in university death education courses with the actual deathbed scenes experienced by elderly people with terminal cancer. Most students expected to live into old age and then die (a) at home, (b) with the companionship of loved ones, (c) quickly (usually in a day or less), and (d) without pain or other symptoms, while (e) remaining alert and lucid. Thoughts about an afterlife were seldom introduced. The students' concept of a deathbed scene worse than the expected usually involved the addition of pain or “lingering on.” Respondents found it difficult to improve on their expected deathbed scene because, in effect, they had already substituted “desired” for “most likely to happen.” Location of the deathbed scene in old age is consistent with the probable life expectancy of the respondents, but the easeful death (quick and asymptomatic) is at variance with observations made of terminally ill people. Comparisons are made with data from an ongoing study of deathbed scenes of patients receiving hospice care and from the National Hospice Study. A major purpose of this study is to introduce the deathbed scene as a situation deserving clinical and conceptual attention, and to make available a set of primary coding categories useful in developing a basic data base.     

Tight lens syndrome associated with a 24-hour disposable collagen lens: a case report

Tight lens syndrome occurred 8 hours after a 24-hour Bio-Cor bandage collagen lens (Bausch & Lomb, Inc.) was placed on the eye of a 19 year old female patient with a history of Stevens-Johnson syndrome and recurrent corneal abrasions. Because collagen bandage contact lenses share some physical and fitting properties with hydrophilic lenses, they should be used with caution in patients at high risk for a tight lens.     

Fixation of mandibular angle fractures with a 2.0-mm 3-dimensional curved angle strut plate.

PURPOSE: The aim of this study was to evaluate our experience and complication rate with the use of a 3-dimensional 2.0-mm curved angle strut plate for mandibular angle fracture fixation. PATIENTS AND METHODS: This was a retrospective evaluation of 37 patients with noncomminuted mandibular angle fractures fixated with a transorally placed curved 2.0-mm strut plate. Postoperative intermaxillary fixation was used in 5 patients for a mean period of 22 days. A nonchewing diet was prescribed for 6 weeks. Records were reviewed for demographic information, medical history, fracture characteristics, operative management, and complications. RESULTS: Two patients developed infections requiring plate removal and reapplication of fixation. Both of these patients had a molar in the fracture line that was left in place during the first operation. One patient developed a mucosal wound dehiscence without consequence. After a mean follow-up period of 10 weeks, 39.4% of patients with a postinjury/pretreatment inferior alveolar nerve deficit reported a return to normal sensation. All patients who developed a sensory deficit as a result of surgery reported full recovery of sensation. A persistent sensory deficit appeared to be related to fracture displacement. CONCLUSION: Fixation of noncomminuted mandibular angle fractures with a 2.0-mm curved angle strut plate was predictable. This plate is low in profile, strong yet malleable, facilitating reduction and stabilization at both the superior and inferior borders. Development of a postoperative infection appeared to be related to failure of removal of a molar in the fracture line. The infection rate of 5.4% found in this study compares favorably with that seen with reconstruction plates. Use of this plate did not appear to cause a permanent sensory deficit in this study.