Progressive systemic sclerosis: rare localization of the maxillofacial district

The authors focus on a case of scleroderma, or progressive systemic sclerosis. Scleroderma is a disease of unknown cause, characterized by an abnormal synthesis of connective tissues that causes a sclerosis of the skin involving organs and systems at various levels. It may appear in different forms and may involve, although not frequently, the maxillofacial district. The authors examined a 21-year-old woman who had been diagnosed with systemic progressive sclerosis in 1994. The patient showed a strong limitation of oral aperture, evidenced by an electrognathographic test, and osseous changes. Panoramic and full-mouth intraoral radiographs showed a complete agenesis of coronoid processes on both sides, whereas the angles and the rising branches of the mandibular and periodontal ligaments appeared normal. The patient was diagnosed as having maxillofacial localization of scleroderma with involvement of coronoid processes bilaterally.     

A prospective study of long-term intrathecal morphine in the management of chronic nonmalignant pain

OBJECTIVE: To examine in a prospective manner the long-term safety and efficacy of chronic intrathecal morphine in patients with severe, nonmalignant pain refractory to less invasive modalities. METHODS: Forty patients with severe, chronic nonmalignant pain poorly managed by systemic medications were identified as candidates for intraspinal trial of morphine. Thirty participants reported successful pain relief during trial and were implanted with an intraspinal delivery system. Standardized measures of pain and functional status were assessed before treatment was begun and at defined intervals during the subsequent 24 months. Intrathecal opioid use and pharmacological and device-related complications were also monitored. RESULTS: The participants had a mean age of 58 +/- 13 years and a mean pain duration of 8 +/- 9 years. Fifty-three percent of the study participants were women. Pain type was characterized as mixed neuropathic-nociceptive (15 of 30 patients, 50%), peripheral neuropathic (10 of 30 patients, 33%), deafferentation (4 of 30 patients, 13%), or nociceptive (1 of 30 patients, 3%). Forty-seven percent of the patients were diagnosed with failed back surgery syndrome. Significant improvement over baseline levels of visual analog scale pain was measured at each follow-up examination after implant. Overall, 50% (11 of 22 patients) of the population reported at least a 25% reduction in visual analog scale pain after 24 months of treatment. In addition, the McGill Pain Questionnaire, visual analog scale measures of functional improvement and pain coping, and several subscales of the Chronic Illness Problem Inventory showed improvement throughout the follow-up period. Pharmacological side effects were managed medically by morphine dose reduction, addition of bupivacaine, or replacement of morphine with hydromorphone. Device-related complications requiring repeat operations were experienced by 20% of the patients. CONCLUSION: Continuous intrathecal morphine can be a safe, effective therapy for the management of severe, nonmalignant pain among a carefully selected patient population and can result in long-term improvement in several areas of daily function.     

Epidemiology of systemic sclerosis

Systemic sclerosis is a family of disorders most appropriately considered in the category of the connective tissue diseases. Two major forms are recognized (diffuse cutaneous and limited cutaneous involvement subtypes), each with distinctive clinical and serologic findings as well as natural history. Scleroderma is characterized epidemiologically by several distinctive features. From a demographic viewpoint, the disease spares children and its incidence increases steadily with age among adults. If occurs much more frequently in women, especially during the child-bearing years, and most often and most severely in young black women, though there is no overall prominent racial predilection. The annual incidence approaches 20 per million population, and may be considerably underestimated. Both incidence and mortality have increased during the past several decades, but these changes are most likely a result of improved case detection rather than a true increase in incidence. Prevalence studies have not been undertaken, but 500 per million population may be a reasonable estimate. Family and genetic studies suggest a weak genetic predisposition, but several strong HLA associations with scleroderma-specific serum autoantibodies are evident. Certain environmental agents may be implicated in pathogenesis of scleroderma and closely related systemic illnesses associated with cutaneous fibrosis. The widespread pathologic process in systemic sclerosis leads to vascular insufficiency and fibrosis, which diminishes the reserve function of many organ systems. The result is considerable disability, especially affecting hand function, and a significant reduction in life span, with an overall 10-year survival from first physician diagnosis of under 70%. Further epidemiologic studies should take full advantage of established and newly proposed subsets of patients with homogeneous clinical, laboratory, serologic, and natural history features. The environment-host interactions noted here must be fully explored, especially in early untreated disease, where primary rather than secondary mechanisms are most likely to be operative.     

Transient tetraparesis after intrathecal and high-dose systemic methotrexate

The copper-containing enzyme superoxide dismutase (SOD) is a key enzyme in suppressing the amounts of superoxide anion radicals. Ceruloplasmin, the copper-transporting protein in plasma, also possesses an important redox capacity. In this study the levels of copper and ceruloplasmin as well as SOD-activity and ceruloplasmin oxidative activity were analyzed in order to throw some light on possible defects in copper mechanisms in patients diagnosed with Alzheimer's disease (AD). The study included 44 patients with AD and their healthy age- and gender-matched controls. No difference of significance was seen when comparing the copper or ceruloplasmin concentration in plasma of AD patients to that of their paired controls. The SOD activity in red blood cells was significantly lower in the patients than in their controls (p = 0.019). The ceruloplasmin oxidative activity in plasma of Alzheimer's patients was greatly reduced as compared to that of age- and gender-matched controls and the difference was highly significant (p = 0.0005). Ceruloplasmin activity and SOD activity were not found to be intrinsically correlated. It was postulated that reduced oxidative activity of ceruloplasmin in plasma might be either a cause or a consequence of AD and that reduced SOD activity might further add to the oxidative disturbances in AD due to defective ceruloplasmin activity.     

Interstitial pneumonia preceding systemic sclerosis

Spontaneous intramural dissection of the oesophagus has been recognized as a separate clinical entity between a transmural oesophageal rupture (Boerhaave syndrome) and an oesophageal mucosal tear (Mallory-Weiss syndrome). It is important to differentiate this condition from myocardiac infarction, dissecting aneurysm and other acute surgical conditions. Conservative management is usually thought to be adequate. We report a case of spontaneous intramural oesophageal dissection, in which the symptom of dysphagia did not improve with the conservative management and an endoscopic incision of the septum between true and false lumens using a needle-type diathermy knife was done safely and effectively.     

Progressive sensorineural hearing impairment in systemic vasculitides

OBJECTIVES: A large series of patients with various forms of systemic vasculitis were evaluated to analyze the prevalence of progressive sensorineural hearing loss (PSNHL), its characteristics and evolution, and the effects of different therapies. METHODS: A total of 673 patients were questioned about the presence of subjective audiovestibular disturbances. Of those, 80 subjects complained of subjective audiological disturbances and underwent oto-rhino-laryngological and audiovestibular evaluation. Those patients with progressive hearing impairment were selected and studied carefully. RESULTS: A PSNHL was observed in 14 patients. The hearing loss was bilateral and asymmetrical in most subjects. It was usually sensorineural, with a cochlear lesion. Unsteadiness was the most frequent vestibular symptom and canal paresis or palsy was noted in most patients. Systemic corticosteroids and cyclophosphamide were useful treatments; in unresponsive patients, satisfactory results were obtained with methotrexate and plasma exchange. CONCLUSIONS: PSNHL is a rare complication of systemic vasculitis, but occasionally is one of the presenting symptoms. Its clinical evolution is variable, but timely clinical assessment and treatment can positively affect prognosis.     

Gallbladder motility in systemic sclerosis

A gas circuit that was capable of passing continuous or pulsatile flows via a 350 L Collins chain-compensated gasometer was built and evaluated. Various turbine volume transducers and dry gas meters were tested with gas compositions and flows that mimicked: a) inspired pulsatile flow over the physiological range and, b) mixed expirate being withdrawn from a Douglas bag. We found the Collins gasometer to be very accurate throughout its elevation, but its mixing fan is not required and atmospheric air should be left to saturate and the added water vapour calculated. Dry gas meters can be accurate to within 1% when calibrated (60 to 150 L/min), but require at least 25 L to be passed through them. The Morgan Ventilometer is an extremely reproducible device (coefficient of variation 0-0.2%, n = 60), but an increase in calibration syringe rate will elevate the calibration factor and reduce the percentage accuracy (one unit increase in calibration factor reduces accuracy by 0.6 - 1.0%). The optimal calibration syringe rate appears to be 30 - 45 b/min. Entrainment through the attached respiratory tubing can also alter the validity of the Ventilometer's calibration procedure.     

Anorectal dysfunction in systemic sclerosis

Melioidosis varies from subclinical infection to fulminating disease with multiple organ involvement. Although endemic in South-East Asia and northern Australia, it may be imported into temperate zones. It is the wide spectrum of manifestation that makes it 'the great imitator'. The purpose of this pictorial essay is to familiarize the radiologist with the range of radiological manifestations of this disease. Intrathoracic findings include pulmonary nodules, consolidation, necrotizing lesions, pleural effusion, pleural thickening and mediastinal abscess. Abscess in almost all organ systems can be seen in the septicaemic and suppurative infections. Both clinical and radiological findings are non-specific. A high index of suspicion is required to focus the search for bacteriological confirmation. A prompt diagnosis is essential, as melioidosis is often rapidly fatal.     

Skeletal muscle pathology in systemic sclerosis

OBJECTIVE: Systemic sclerosis (SSc) is characterized by abnormal deposition of collagen in the skin and by visceral involvement. Muscle weakness is a relatively frequent complication of SSc, although severity varies. We studied muscle pathology in patients with SSc with progressive muscle involvement. METHODS: We performed histochemical and immunohistochemical investigations to detect neural cell adhesion molecule (NCAM). RESULTS: Five of the 6 cases of SSc expressed NCAM in atrophic angulated fibers (some fibers stained heavily with oxidative enzymes). CONCLUSION: Neurogenic involvement in SSc is more frequent than reported.     

Adenosine deaminase in progressive systemic sclerosis

The aim of the present study was to confirm the increase of adenosine deaminase (ADA) activity in progressive systemic sclerosis (PSS), described by Sasaki & Nakajima, and to compare plasma ADA activity of patients in different stages of the disease. Enzyme activity was measured with a colorimetric assay. The 48 patients were subdivided into 3 groups: subgroup 1 (n = 10), disease still limited to the skin; subgroup 2 (n = 21), involvement of the skin and oesophagus; and subgroup 3 (n= 17), involvement of the skin and multiple internal organs. ADA levels were highest in subgroup 3. However, the difference with respect to subgroup 2 did not reach statistical significance. Subgroup 1 was different from controls and subgroups 2 and 3 (p<0.001). Our results confirm that ADA activity is increased in PSS, and that this finding is observed even in the early stages of the disease process. We speculate that the increase in ADA, a well-known marker of T-cell activation, might be an indicator of disease activity in PSS, in the beginning as well as during phases of exacerbation in later stages of the disease.     

The intrathecal, polyspecific and oligoclonal immune response in multiple sclerosis

Sexual assault on children and adolescents has become a common topic of study, but there has been little research into the specific characteristics of the population of male victims. A national survey representative of school-age adolescents in France enabled us to study 465 adolescents reporting sexual assault (121 boys, 344 girls; mean age 15.4, SD 2.5 years). Girls were shown to be more frequently affected by certain medicopsychological symptoms: nightmares, multiple somatic complaints and some items concerning mood disorders. On the other hand, behavioural symptoms were much more frequently expressed in boys, in particular: repeated suicide attempts, running away, fits of violence and substance use. Boys presenting these symptoms should be questioned as a matter of routine concerning a history of sexual assault.     

Antiphospholipid syndrome associated with progressive systemic sclerosis

Hemifacial spasm is a neurological disorder due to abnormal hyperactivity of the facial nerve. The most common cause of hemifacial spasm is a neuro-vascular conflict in the cerebellopontine angle between a vascular loop and the root of the facial nerve (96% of cases). Tumors are the cause of hemifacial spasm in only 1% of cases). The authors present their results in 100 patients who underwent microvascular decompression for essential hemifacial spasm between 1990 and 1995. They used microsurgical and endoscopic procedures by a minimal retrosigmoid approach in all cases. The most common offending vessels were the posterior inferior cerebellar artery (70%), the vertebral artery (41%) and the anterior inferior cerebellar artery (28%). An aberrant vein was found in 2 cases. There were 38% of multiple artery-nerve conflicts. Physiopathology of hemifacial spasm is explained by two principal theories: in the ephaptic theory, hyperactivity and an abnormal nervous impulse pathway are due to a short demyelinated area on the nerve trunk caused by the offending vessel, inducing short circuiting between adjacent nerve fibers. In the nuclear theory, hyperactivity of the facial nerve is due to an abnormal and automatic activity of the facial nerve nucleus itself, induced by the vessel. The authors used pre and postoperative electromyographic tests and intraoperative electromyographic tests. Their results tend to prove the nuclear theory. Ninety per cent of the patients had a good result, with a mean follow-up time of 30 months in 60 cases. In 82% of the cases, there was a total recovery after a single procedure. There was no mortality and no facial palsy. Hearing loss occurred in less than 5%.