Multiple system atrophy

The past year has seen advances in delineating the clinical features, natural history and imaging characteristics of multiple system atrophy. The initiating pathogenetic mechanisms remain unknown. However, any aetiological or pathophysiological hypothesis must consider not only neuronal loss and gliosis but also the recently described characteristic oligodendrolial cytoplasmic inclusions.     

Multiple system atrophy and visuospatial function.

An appropriate battery of tests of visuospatial function was given to 10 adult patients with multiple system atrophy, to 10 adult patients with idiopathic Parkinson's disease, and to 18 adult normal control Ss. Members of the 3 groups were matched for age, educational level, and Verbal IQ. Comparison of results showed that, unlike the patients with Parkinson's disease and the control Ss, patients with multiple system atrophy were impaired in their ability to discriminate between angles and to match angles, but showed no other visuospatial deficits. These results indicate a need for further study of this matter on a larger scale. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Multiple myeloma in young patients: clinical presentation and treatment approach

Multiple myeloma (MM) in patients younger than 40 or 30 years accounts for only 2% and 0.3% of all myelomas, respectively. The presenting clinical and laboratory features are similar to those observed in patients of all ages who have myeloma, except a higher proportion of young patients have only light-chain myeloma. Some very young patients, particularly those younger than 30 years, have multiple skeletal lesions with extramedullary spread and a small M-component with few bone marrow plasma cells. In young patients with MM, particularly in those with good prognostic features (that is, normal renal function or low beta2-microglobulin level) and also in those younger than 30 years, the survival is longer than that in series of patients of all ages with MM. Young patients with MM might benefit from early high-dose therapy followed by autologous or allogeneic stem cell rescue. The current status of autologous and allogeneic transplantation in MM is reviewed.     

Multiple system atrophy following chronic carbon disulfide exposure

Carbon disulfide toxicity is well characterized. The principal target organ is the nervous system, although cardiovascular, reproductive, ophthalmologic, and other effects are also recognized. The neurotoxicity manifests in three ways: encephalopathy, peripheral and cranial nerve dysfunction, and movement abnormalities. This report describes a case of olivopontocerebellar atrophy, a form of multiple system atrophy, developing in an adult after over 30 years of occupational exposure to carbon disulfide. The patient presented with the insidious onset of balance problems, impotence, and irritability, without tremor, cogwheel rigidity, bradykinesia, or changes in facial expression. Over the next few years severe ataxia developed, and the clinical diagnosis was confirmed with computed tomography and magnetic resonance imaging scans. The patient experienced multiple medical complications and died approximately 9 years after diagnosis. This case is consistent with a large body of clinical and experimental literature, much of it 50 years old, showing that carbon disulfide can cause movement disorders. It also serves as a reminder that movement disorders, ranging from parkinsonism to dystonia, are associated with a variety of toxic exposures such as manganese, carbon monoxide, 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine, and medications.     

Secondary hypertension and clinical genetics: usual presentation with unusual diagnosis

A transjugular central venous catheter was inadvertently sutured to the wall of the right atrium in a 63-year-old female during coronary bypass surgery. Using two nitinol Goose Neck snares via a transfemoral and a transjugular approach the catheter was severed into two pieces and retrieved percutaneously.     

The watermelon stomach: clinical presentation, diagnosis, and treatment

The watermelon stomach syndrome is an increasingly recognized cause of persistent acute or occult gastrointestinal bleeding, typically in elderly women. This disorder often presents with severe iron deficiency anemia, and a variety of associated conditions including autoimmune disease, cirrhosis, achlorhydria, and hypochlorhydria. Diagnosis is made by the characteristic endoscopic appearance of visible linear watermelon-like vascular stripes in the antrum. Histology confirms the vascular nature of this disorder, showing dilated and thrombosed capillaries in the lamina propria, associated with reactive fibromuscular hyperplasia. The optimum treatment of choice is not known. Several treatment options, including surgical antrectomy, and endoscopic photocoagulation with Nd:Yag laser, heater probe therapy, and bipolar electrocautery, have yielded excellent results. Pharmacological agents have also been used to treat selected numbers of patients, most of which comprise a small number of case reports.     

Neurological cases for MRCP: 2. Multiple system atrophy (MSA)

The effects of 3,5,3'-triiodothyronine (T3) levels on threshold, latency and duration of pentylenetetrazole-induced seizures were tested in rats treated with thyroxine (300 micrograms/kg.day, N = 9) or methimazole (60 mg/kg.day, N = 5) dissolved in drinking water. Compared to controls (N = 7), methimazole treatment reduced T3 levels (45.4 +/- 2.0 vs. 33.0 +/- 4.8 ng/dl) and increased seizure duration (36.2 +/- 22.4 vs. 289.6 +/- 24.4 s) and threshold (29.0 +/- vs. 45.5 mg/kg). Thyroxine treatment increased T3 levels (45.4 +/- 2.0 vs. 67.7 +/- 4.8 ng/dl), but had no significant effect on seizures.     

Consensus statement on the diagnosis of multiple system atrophy

We report the results of a consensus conference on the diagnosis of multiple system atrophy (MSA). We describe the clinical features of the disease, which include four domains: autonomic failure/urinary dysfunction, parkinsonism and cerebellar ataxia, and corticospinal dysfunction. We set criteria to define the relative importance of these features. The diagnosis of possible MSA requires one criterion plus two features from separate other domains. The diagnosis of probable MSA requires the criterion for autonomic failure/urinary dysfunction plus poorly levodopa responsive parkinsonism or cerebellar ataxia. The diagnosis of definite MSA requires pathological confirmation.     

Dengue virus infection: epidemiology, pathogenesis, clinical presentation, diagnosis, and prevention

OBJECTIVES: To investigate whether the isolated urinary bladder spontaneously releases substance P (SP) or bradykinin (BK), which can act as potent mediators of pain and inflammation of the urinary bladder, and whether peptidase inhibitors enhance peptide release. MATERIALS AND METHODS: Urinary bladder segments (2 x 10 x 0.8-1 mm) were isolated from guinea pigs and studied in vitro; tissue contraction was assessed using force-displacement transducers and the release of peptides by specific enzyme immunoassays. RESULTS: In the absence of any exogenous agonists, the inhibition of neutral endopeptidase and angiotensin-converting enzyme by phosphoramidon and captopril, respectively, increased the frequency and magnitude of spontaneous motility of isolated bladder strips. Phosphoramidon increased the net release of SP-like immunoreactivity (SP-LI) and captopril increased the net release of SP-LI and BK-LI, concomitant with contraction. Peptide-LI was recovered primarily from bladder mucosa and to a lesser degree from detrusor smooth muscle. Similarly, peptidase inhibitors primarily affected the bladder mucosa; phosphoramidon induced a fourfold increase in SP-LI and captopril induced a significant increase of SP-LI and BK-LI from the mucosa. Tissues contracted in response to peptidase inhibitors in the presence of atropine and indomethacin, but contraction was reduced significantly by in vitro capsaicin desensitization or removal of bladder mucosa. BK stimulated SP-LI release from mucosa but not detrusor. SP stimulated increased BK-LI release from mucosa and detrusor. CONCLUSIONS: These findings indicate the basal release of peptide-like immunoreactivity by isolated bladder and further support the concept that peptidases located in the bladder mucosa are important in terminating the effects of endogenous peptides.     

Value of sphincter electromyography in the diagnosis of multiple system atrophy

It is clinically important, to distinguish between idiopathic Parkinson's disease (IPD) and multiple system atrophy (MSA) not only because of the implications for prognosis but also because urinary incontinence is often an early troublesome feature of MSA and by making the correct neurological diagnosis inappropriate urological surgery may be avoided. Onuf's nucleus in the sacral cord is the location of the anterior horn cells innervating the sphincters, and it is among central nervous system sites affected by neuronal cell loss in MSA but not in IPD. A systematic analysis of motor units recorded from the sphincter looking for changes of chronic reinnervation has therefore been used to distinguish between these conditions. Sphincter electromyography (EMG) was carried out in 126 patients with suspected MSA with review of their case notes up to 2 years later. Of those in whom a diagnosis of MSA was made, 82% had had an abnormal sphincter EMG.     

Hepatic amebiasis: an unusual clinical presentation

BACKGROUND: Painful liver enlargement with fever are common signs of hepatic ambiasis. Exceptionally, atypical signs may also occur including symptoms suggesting renal sepsis. CASE REPORT: An 18-year-old woman from the New Caledonia was hospitalized in metropolitan France for suspected right-sided acute pyelonephritis. Urinalysis was normal and the kidney ultrasound suggested the need for an abdominal CT-scan which evidenced a voluminous 10-cm abscess pus. Serology for amebia was positive, confirming the diagnosis of hepatic amebic abscess. Outcome was rapidly favorable with intravenous anti-parasite treatment amebic abscess. Outcome was rapidly favorable with intravenous anti-parasite treatment and percutaneous drainage. DISCUSSION: Atypical signs of hepatic ambiasis may mislead diagnosis. The absence of a fetid odor at puncture helps guide diagnosis, confirmed by serology. Percutaneous drainage can hbe proposed for voluminous abscesses or if the risk of extrahepatic complications is eminent.     

Alpha-interferon-induced arthritis: clinical presentation treatment, and prevention

Carotenoid interactions during absorption and in postabsorptive metabolism have been observed in both human and animal studies. We reviewed the mutual interactions reported between lutein and beta-carotene and report new data on the postprandial beta-carotene and retinyl ester response in the triglyceride-rich plasma lipoprotein (TRL) fraction in volunteers after a single test meal with beta-carotene alone, or beta-carotene combined with lutein or lycopene. Results indicate an inhibitory effect of lutein on beta-carotene absorption, but apparently not on beta-carotene cleavage. In a comparative study with two beta-carotene/lutein ratios (2:1 and 1:2, respectively), this inhibitory effect of lutein was found to be most marked when lutein was the predominant carotenoid. In studies on plasma (serum) response also an inhibitory effect of beta-carotene on lutein response was observed.     

Primary hepatic lymphoma: unusual presentation and clinical course

Although primary hepatic lymphoma is rare, it should be considered in the differential diagnosis of a hepatic tumor, because it is usually associated with a favorable prognosis. This report describes an unusual case of primary hepatic lymphoma with an atypical presentation (only mild, right upper quadrant pain and no hepatomegaly) followed by acute fulminating hepatic failure, metabolic acidosis, followed by a rapidly fatal course. A review of the literature and discussion of the disease are also presented.     

多因素综合分析在OG风机在线监测诊断系统的应用

在线监测诊断系统预警准确率直接决定了在线监测诊断系统的生命力,也是目前在线监测诊断系统发展的难点,尤其是变速设备的预警准确率一直困扰着在线监测诊断系统的发展。分析OG风机运行中的变动因素以及影响OG风机振动、温度的因素,通过软件实现影响振动、温度的因素相互关联,提升在线监测诊断系统预警的准确率,进而提升在线监测诊断系统的使用率。多因素综合分析在OG风机在线监测诊断系统运用后,误报警率为零,突发故障为零,降低了设备管理人员的劳动强度。     

Lyme carditis: a clinical presentation and long time follow-up

1. We evaluated the effects of the dietary restriction of sodium chloride on blood pressure and systemic calcium metabolism in 19 in-patients with essential hypertension (11 men and 8 women, mean age 49.9 +/- 12.1 years). 2. All patients received a high-sodium diet (250 mmol/day) for 1 week, followed by a low-sodium diet (10 mmol/day) for another week. Intake of potassium (100 mmol/day) and of calcium (15 mmol/day) were kept constant throughout the study. 3. Sodium restriction significantly reduced the mean blood pressure (from 114.0 +/- 1.9 to 105.0 +/- 13.7 mmHg, P < 0.01). Urinary calcium excretion was significantly reduced (from 5.1 +/- 2.4 to 2.2 +/- 1.0 mmol/day, P < 0.01). 4. The change in mean blood pressure after sodium restriction was not correlated with a change in any parameter of calcium metabolism [whole blood ionized calcium, plasma intact parathyroid hormone, or 1,25-(OH)2 vitamin D3]. 5. Plasma renin activity during a regular sodium diet, an index of renin status, was significantly and inversely correlated with the change in blood pressure during sodium restriction, but not with any change in the parameters of calcium metabolism. 6. We conclude that sodium restriction reduces blood pressure and decreases urinary calcium excretion. However, we observed no significant role of extracellular calcium concentration or of calciotropic hormone concentration in the mechanism of sodium sensitivity.     

Multiple sclerosis, the great masquerader: an atypical ocular presentation

BACKGROUND: Multiple sclerosis (MS) is the most common chronic disease of the central nervous system and is pleomorphic in it presentations. The optic pathways are frequently involved, and the classic ocular abnormality is optic neuritis. METHODS: A case is presented of a 23-year-old woman in whom optic neuropathy developed and multiple sclerosis was diagnosed by neuroradioimaging, in spite of her lack of awareness of vision loss. RESULTS: The demyelinating lesions in MS may develop anywhere in the visual system and produce a variety of visual defects. Magnetic resonance imaging (MRI) is the most sensitive method for demonstrating these lesions. CONCLUSION: The cause of MS is unknown, but current opinion holds that autoimmunity--perhaps induced by viral infection--is likely to be implicated in its etiopathogenesis. Currently, no method for prevention of MS is known.