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The electrodiagnostic findings in peripheral neuropathy associated with monoclonal gammopathy.

The electrodiagnostic findings in 51 patients with monoclonal serum proteins of different etiologies and peripheral neuropathies are analyzed and the findings in an additional 10 patients with multiple myeloma are discussed. The nine patients with monoclonal gammopathy of undetermined significance had electrodiagnostic patterns consistent with either predominant axonal degeneration or demyelination. Almost all 15 patients with osteosclerotic myeloma had evidence of a demyelinating neuropathy. The 27 patients with primary systemic amyloidosis almost always displayed evidence of an axonal neuropathy with superimposed carpal tunnel syndrome in some cases. The 10 patients with multiple myeloma had heterogeneous findings. Electrodiagnostic studies can aid in classifying these patients and suggest the likelihood of a specific etiology.

Amyloidosis↗

Peripheral neuropathy in osteosclerotic myeloma: clinical and electrodiagnostic improvement with chemotherapy.

A patient with a severe remote-effect polyneuropathy and other paraneoplastic features of osteosclerotic myeloma improved dramatically with melphalan and prednisone treatment. Serial electrodiagnostic studies provided an objective means of following the response to therapy and documented the improvement. We believe this represents the first reported patient with multifocal osteosclerotic myeloma and a myelomatous polyneuropathy responding to melphalan and prednisone.

Adult↗

Morbilliviruses and human disease.

Morbilliviruses are a group of viruses that belong to the family Paramyxoviridae. The most instantly recognizable member is measles virus (MV) and individuals acutely infected with the virus exhibit a wide range of clinical symptoms ranging from a characteristic mild self-limiting infection to death. Canine distemper virus (CDV) and rinderpest virus (RPV) cause a similar but distinctive pathology in dogs and cattle, respectively, and these, alongside experimental MV infection of primates, have been useful models for MV pathogenesis. Traditionally, viruses were identified because a distinctive disease was observed in man or animals; an infectious agent was subsequently isolated, cultured, and this could be used to recapitulate the disease in an experimentally infected host. Thus, satisfying Koch's postulates has been the norm. More recently, particularly due to the advent of exceedingly sensitive molecular biological assays, many researchers have looked for infectious agents in disease conditions for which a viral aetiology has not been previously established. For these cases, the modified Koch's postulates of Bradford Hill have been developed as criteria to link a virus to a specific disease. Only in a few cases have these conditions been fulfilled. Therefore, many viruses have over the years been definitely and tentatively linked to human diseases and in this respect the morbilliviruses are no different. In this review, human diseases associated with morbillivirus infection have been grouped into three broad categories: (1) those which are definitely caused by the infection; (2) those which may be exacerbated or facilitated by an infection; and (3) those which currently have limited, weak, unsubstantiated or no credible scientific evidence to support any link to a morbillivirus. Thus, an attempt has been made to clarify the published data and separate human diseases actually linked to morbilliviruses from those that are merely anecdotally associated.

Animals↗

Clinical course of bone metastasis from prostatic cancer following endocrine therapy: examination with bone x-ray.

X-ray findings of bone metastatic lesions from 81 cases of stage D2 prostatic cancer were examined before and following endocrine therapy. Untreated lesions were classified into five types; osteoblastic (15%), mixed, but mainly osteoblastic (31%), mixed, but mainly osteolytic (17%), osteolytic (10%), and undetermined with a positive bone scan (27%). Patients with two mixed types had a tendency of widely speeded areas of metastasis and elevated serum prostatic acid phosphatase. Temporal enlargement of sclerotic lesion immediately after the start of therapy did not indicate disease progression. In many cases, changes from osteolytic to osteoblastic patterns were noticed in the same lesion regardless of the effects of endocrine therapy. Remodeling to the sclerotic pattern appeared as curative changes. From these findings, it was concluded that the natural course of bone lesions showed a tendency to change from the osteolytic to osteoblastic type and relapse was often accompanied by an increase of the osteolytic type lesion. Evaluation of therapeutic effects based on remodeling, changes in number and areas of lesions, and the appearance of new lesion correlated well with prognosis.

Aged↗

The relationship between the degree of chronic middle ear inflammation and tympanic bulla pneumatization in the pig as animal model.

The relationship between the degree of chronic middle ear inflammation and pneumatization was investigated in the pig as an animal model, since its tympanic bulla closely resembles the human mastoid air cell system. Ten piglets (sire: Landrace-Hampshire crossbreed; dam: Duroc) were used for this experiment. Four ears of two animals served as the normal control group and 16 ears of eight animals were the experimental group. In this latter group, otitis media was induced by injecting glycerin into the middle ear clefts 1 months after birth, and the degree of inflammation was varied by administering or withholding antibiotics (cefamandole and dibekacin) and adjusting the dosage regimen. The animals were sacrificed 6 months after birth and examined for the relationship between the degree of chronic middle ear inflammation present and tympanic bulla pneumatization. Various degrees of inflammation were successfully induced by injecting the antibiotics: the more severe the inflammation found, the greater was the inhibition of pneumatization. Findings demonstrated that the degree of inhibition of pneumatization produced was directly proportional to the severity of chronic middle ear inflammation.

Animals↗

A sign for the early detection of medullary sclerosis.

The radiographic image of a normal bone has a continuous radiopaque boundary of variable thickness and a relatively radiolucent interior. Normally, the endosteal edge of this boundary line is well defined. However, this edge may be obscured in diseases that induce medullary new bone formation. The use of this sign for the early detection of medullary sclerosis is demonstrated by specific examples.

Female↗

Anterior chest wall involvement in patients with pustulosis palmoplantaris.

With the aim of determining the frequency and radiographic features of anterior chest wall involvement in patients with pustulosis palmoplantaris, a questionnaire was sent to 107 patients. Ninety-three patients returned the questionnaire, five of whom were excluded from further analysis due to the appearance of psoriatic lesions. Twenty-five (28%) of the remaining 88 patients reported pain and/or swelling of joints or bones in the anterior chest wall. All were examined radiographically, using tomography, and a group of 20 patients without anterior chest wall complaints were examined similarly. Sixteen of the patients with, but none of the patients without, complaints were found to have arthro-osteitis of the anterior chest wall, consisting of diffuse sclerosis of the manubrium sterni in one patient, localized sclerosis in seven patients, and sequelae of arthritis of the sternoclavicular, upper sternocostal and/or manubriosternal joint in eight patients.

Adult↗

Bone island (enostosis): clinical significance and radiologic and pathologic correlations.

This study was undertaken to explain the increased scintigraphic activity of some bone islands by correlating the clinical, radiographic, scintigraphic, and histopathologic findings. The material for the study consisted of six patients with bone islands who had undergone histopathologic examinations of the lesions. Based on histopathologic-radiologic correlations, we postulate that the osteoblastic activity of a bone island makes it appear "hot" on scintigraphy, although other factors such as the size of the lesion may play some role in this phenomenon. In addition, we tried to find a logical algorithm for the radiologic evaluation and management of sclerotic lesions that look like bone islands but exhibit increased uptake of radiopharmaceuticals on bone scan examination. We emphasize that the morphology of the lesion, as demonstrated on the radiologic examination, rather than its degree of activity on the bone scan is a guide to the correct diagnosis.

Adult↗

A new lethal sclerosing bone dysplasia.

A neonate is described with a lethal sclerosing bone dysplasia associated with prenatal fractures and craniofacial abnormalities including microcephaly, exophthalmos, hypoplastic nose and mid-face, small jaw and nodular hyperplasia of the gums. Parental consanguinity suggests that an autosomal recessive mutation is the likely aetiology.

Bone Diseases, Developmental↗

Computed tomographic assessment of vertebral bone mineral in childhood.

Quantitative computed tomography (QCT) was used to assess trabecular bone mineral concentration in the vertebrae of 132 children, 45 with suspected disorder of bone mineralisation, 54 with thalassaemia and 37 controls. The range for bone mineral concentration in controls, expressed as equivalent K2HPO4 concentrations, was 90-190 mg cm-3. Abnormally low values were seen in all untreated children with idiopathic juvenile osteoporosis, 3/9 steroid recipients, and three patients with osteogenesis imperfecta. Abnormally high values were seen in 10/14 chronic renal failure patients. Comparison of the single and dual-energy methods showed that the single energy method, which has a lower radiation dose and is less prone to error from movement artifact, is satisfactory in most paediatric applications.

Bone Diseases↗

Scintigraphic and radiographic patterns of skeletal metastases in breast cancer: value of sequential imaging in predicting outcome.

OBJECTIVE: To determine whether temporal changes in scintigraphic and bone radiographic findings have prognostic significance in patients with skeletal metastases from breast cancer. DESIGN: Clinical information and films were retrospectively reviewed in 101 randomly selected patients with skeletal metastases. Images from sequential bond scans and bone radiographs were correlated with survival after detection of the metastases. RESULTS: Time to detection of skeletal metastases and the length of time for which patients were classified as radiologically stable after development of skeletal metastases correlated with survival (r = 0.843; r = 0.821, respectively). Failure to develop a radiographically and scintigraphically stable pattern after treatment was associated with significantly decreased survival compared with the rest of the patients (mean survival 2.1 +/- 1.3 years vs 4.3 +/- 2.3 years; p < 0.001). Scintigraphic regression of metastases was associated with significant survival benefit and longer stabilization of disease compared to all other patterns (mean survival 5.0 +/- 2.7 years of regressive disease vs 3.7 +/- 1.9 years for stable disease and 2.2 +/- 1.3 years for progressive disease; p < 0.001). CONCLUSIONS: Sequential scintigraphic and radiographic imaging is useful in breast cancer patients not only for detection of metastases and monitoring of treatment effect, but also because these studies provide valuable prognostic information.

Adult↗

Paget's disease of bone: review with emphasis on radiologic features, Part I.

Distinctive clinical, radiologic and pathologic features are seen in Paget's disease of bone. These distinct features can be divided into three phases; initial phase, midphase, and late phase. The clinical features may vary from patients being asymptomatic (involving a single bone) to patients having severe, multiple bone involvement with systemic disease. Radiologically, there are unique features which can differentiate Paget's disease from other bone diseases. The radiologic features follows the histologic findings on light microscopy. The correlation of the different phases with the clinical, radiologic and pathologic features of Paget's disease allows for early accurate diagnosis and treatment.

Adult↗

Metaphyseal sclerosis in patients with chronic renal failure.

We reviewed radiographs of the hands and wrists of 33 patients with immature skeletons and chronic renal disease. Various radiographic manifestations of renal osteodystrophy were seen, including osteopenia in 23 patients (70%), subperiosteal resorption in 20 (61%), distal tuft resorption in 14 (42%), sclerosis of vertebral bodies in 2 (6%), and soft-tissue calcification in 1 (3%). We also noted that 13 patients (39%) exhibited metaphyseal sclerosis adjacent to the growth plates. Five of these 13 showed persistent sclerosis years after the growth plates had fused. None of the patients showed other radiographic changes of rickets, and there was no correlation between the serum calcium, phosphorus, or aluminum levels and the presence of metaphyseal sclerosis. Neither was there any association with the underlying cause of renal failure, method of treatment, presence of a transplant, or type of dialysis. We view this finding as another manifestation of renal osteodystrophy. The importance of distinguishing it from other sclerotic lesions is discussed.

Adolescent↗

Radiological appearances following limb replantation: a report of 5 cases.

Plain radiographs of the limbs were reviewed after replantation procedures following traumatic amputation in 5 patients at the wrist (2), mid-forearm (2) and knee (1). Following upper limb replantation, rapid development of osteopaenia was initially noted in the juxta-articular regions and metaphyses of the tubular bones of the hand. These changes were followed by diaphyseal cortical bone loss with evidence of subperiosteal, intracortical and endosteal bone resorption. Juxta-articular erosions developed at the margins of the small joints in the hand in all cases. Gradual resolution of bone changes was noted in 3 of the 4 cases where follow-up exceeded 5 years. Following lower limb replantation, there was no evidence of bone loss; however, severe neuropathic joint disease developed within 18 months and progressed over the following 9 years. We suggest that the radiographic changes observed after upper limb replantation reflect regional hyperaemia secondary to neurovascular damage, whilst the changes in the lower limb reflect a similar process in a limb subjected to weight bearing.

Adult↗

Osteosclerotic myeloma with polyneuropathy and hypocalcemia.

A case is presented of a 46-year-old man with multifocal osteosclerotic bone lesions, peripheral polyneuropathy and hypocalcemia. Histologic examination of a bone marrow biopsy disclosed a multiple myeloma. Immunoelectrophoresis revealed a small M-component identified as IgG-lambda. Osteosclerotic myeloma lacking any osteolytic lesions seems to be very rare and shows several different features as compared with classical myeloma. A review of the current literature suggests that multiple myeloma is not a uniform disease but rather a group of clinical syndromes characterized by the special properties of their proliferating plasma cell clones.

Bone Marrow↗

Clinical heterogeneity in the tricho-dento-osseous syndrome.

The tricho-dento-osseous syndrome (TDO syndrome) involves morphologic abnormalities of hair, teeth, and skeleton. Clinical findings of the TDO syndrome are excessively curly (fuzzy) hair, enamel hypoplasia, and skeletal findings of a generalized pattern of osseous sclerosis. We report an autosomal dominant syndrome with similar hair and teeth morphology, but with a skeletal dysplasia consisting of sclerosis and thickening of the calvarium with long bones that show subtle undertubulation but no sclerosis.

Adult↗