63 research outputs found
Psychiatric manifestations of multiple sclerosis and acute disseminated encephalomyelitis
It is unusual for acute disseminated encephalomyelitis and multiple sclerosis to present as purely psychiatric disorders. We report five patients with such demyelinating diseases and symptoms of psychosis, depression or anxiety. The importance of excluding demyelination as the basis for these psychiatric disturbances is emphasized, especially in the presence of unexplained neurologic findings. The possible relationship between psychiatric symptoms and demyelinating disorders is explored
Rozsiane zapalenie mózgu i rdzenia kręgowego oraz stwardnienie rozsiane; dwie różne choroby - spojrzenie krytyczne
Podjęcie leczenia immunomodulacyjnego bezpośrednio po wystąpieniu
pierwszego izolowanego zespołu objawów (CIS, clinically
isolated syndrome), sugerującego stwardnienie rozsiane
(SM, sclerosis multiplex), powinno być poprzedzone diagnostyką
różnicową z wykluczeniem rozsianego zapalenia mózgu i rdzenia
kręgowego (DEM, disseminated encephalomyelitis). Przebieg kliniczny,
właściwości genetyczne, obraz histopatologiczny oraz
wyniki badań obrazowych wskazują, że DEM i SM są odrębnymi
jednostkami chorobowymi. Ostre i nawracające DEM częściej dotyczy
dzieci, ale może również wystąpić u osób dorosłych. Przebieg
DEM jest wieloobjawowy. W porównaniu z SM częściej występują
gorączka, zaburzenia świadomości, zaburzenia poznawcze,
afazja i objawy oponowe. Rzadko stwierdza się obecność
prążków oligoklonalnych w płynie mózgowo-rdzeniowym. Rezonans
magnetyczny (MRI, magnetic resonance imaging) jest najlepszą
metodą obrazowania ośrodkowego układu nerwowego
wykorzystywaną w diagnostyce różnicowej DEM i SM. W przypadku
DEM występuje wiele ognisk demielinizacji w istocie białej.
Zmiany umiejscawiają się także we wzgórzu i jądrach podstawy.
W początkowej fazie choroby są zwykle bardziej rozległe niż
w przypadku SM i wzmacniają się po podaniu gadoliny. W obrazie
MRI stwierdza się występowanie ognisk demielinizacyjnych obejmujących co najmniej trzy segmenty rdzenia kręgowego oraz
zapalenie nerwów wzrokowych (NMO, neuromyelitis optica). Niekiedy
NMO towarzyszy obecność przeciwciał przeciw akwaporynie
4, ale bywają także stwierdzane w SM oraz DEM. W większości
przypadków NMO jest składową DEM, a nie SM i przypomina
„orientalną” lub „wzrokowo-rdzeniową” postać SM
Elevated ectodomain of type 23 collagen is a novel biomarker of the intestinal epithelium to monitor disease activity in ulcerative colitis and Crohn's disease
BACKGROUND: Impaired intestinal epithelial barrier is highly affected in inflammatory bowel disease. Transmembrane collagens connecting the epithelial cells to the extracellular matrix have an important role in epithelial cell homeostasis. Thus, we sought to determine whether the transmembrane type 23 collagen could serve as a surrogate marker for disease activity in patients with Crohn's disease and ulcerative colitis. METHODS: We developed an enzyme-linked immunosorbent assay to detect the ectodomain of type 23 collagen (PRO-C23) in serum, followed by evaluation of its levels in both acute and chronic dextran sulfate sodium colitis models in rats and human inflammatory bowel disease cohorts. Serum from 44 Crohn's disease and 29 ulcerative colitis patients with active and inactive disease was included. RESULTS: In the acute and chronic dextran sulfate sodium-induced rat colitis model, the PRO-C23 serum levels were significantly increased after colitis and returned to normal levels after disease remission. Serum levels of PRO-C23 were elevated in Crohn's disease (p < 0.05) and ulcerative colitis (p < 0.001) patients with active disease compared to healthy donors. PRO-C23 differentiated healthy donors from ulcerative colitis (area under the curve: 0.81, p = 0.0009) and Crohn's disease (area under the curve: 0.70, p = 0.0124). PRO-C23 differentiated ulcerative colitis patients with active disease from those in remission (Area under the curve: 0.75, p = 0.0219) and Crohn's disease patients with active disease from those in remission (area under the curve: 0.68, p = 0.05). CONCLUSION: PRO-C23 was elevated in rats with active colitis, and inflammatory bowel disease patients with active disease. Therefore, PRO-C23 may be used as a surrogate marker for monitoring disease activity in ulcerative colitis and Crohn's disease
Carnitine palmitoyl transferase type 2 defi ciency -case report and review of the literature
ABSTRACT -Carnitine palmitoyl transferase (CPT) defi ciency is a relatively rare disease of fatty acid oxidation inherited autosomal recessively. CPT2 defi ciency presents frequently in adults with rhabdomyolysis and myoglobinuria triggered most oft en by prolonged exercise. Carnitine is required for the transfer of longchain fatty acids from the cytoplasm to the mitochondrial matrix for their oxidation. Strenuous exercise is known to increase serum creatine kinase (CK) in nearly all healthy people and can be elevated oft en over ten times the upper limit of normal. Rhabdomyolysis can be of inherited etiology (disorders of glycogenolysis, fatty acid oxidation, mitochondrial respiratory chain pathways) or acquired (trauma, compartment syndrome, drugs, caff eine, toxins, infections, infl ammatory muscle diseases, and exertion). Here we present a female patient with CPT2 defi ciency diagnosed aft er recurrent rhabdomyolysis upon physical exertion and carbohydrate-restrictive diet. With the implementation of dietary measures and lifestyle changes that included more frequent but shorter interval exercise and avoidance of inappropriate physical exertion, the patient had a normal neurological status with only slightly elevated CK levels. Th is example illustrates the importance of careful monitoring of patients with increased levels of CK, even when there are no evident clinical, histopathologic or electromyoneurography (EMNG) indicators of myopathy
Inflammatory bowel disease and pregnancy
Upalne bolesti crijeva često zahvaćaju žene generativne dobi. Često se postavlja pitanje utjecaja trudnoće na prirodni tijek bolesti, utjecaja same bolesti na tijek i ishod trudnoće te sigurnosti primjene lijekova u trudnoći. Žene s upalnom bolesti crijeva u pravilu mogu očekivati normalan tijek trudnoće te rađanje zdravog novorođenčeta. Aktivnost bolesti prilikom koncepcije najvažniji je čimbenik koji utječe na tijek i ishod trudnoće. Terapija koja se uobičajeno koristi za liječenje upalnih bolesti crijeva sigurna je za primjenu u trudnoći. Na temelju prikaza slučaja bolesnice s prvom manifestacijom ulceroznog kolitisa u trudnoći prikazati ćemo utjecaj bolesti na tijek i ishod trudnoće, utjecaj trudnoće na prirodni tijek bolesti te sigurnost primjene lijekova za liječenje upalnih bolesti crijeva u trudnoći.Inflammatory bowel diseases often affect women during their reproductive age. The questions regarding the effect of pregnancy on the natural course of inflammatory bowel disease, effect of inflammatory bowel disease on the course and outcome of pregnancy and safety of medications used to treat inflammatory bowel disease during pregnancy arise quite often. In general, women with inflammatory bowel disease can expect to have a normal pregnancy with delivery of a healthy newborn. The most important factor influencing the effect of disease on the course of pregnancy is disease activity at conception. Medications routinely used for the treatment of inflammatory bowel diseases can be used safely during pregnancy. Acase of first presentation of ulcerative colitis in pregnancy is presented with review of disease course during pregnancy, influence of disease on pregnancy course and outcome and safety of medications used to treat inflammatory bowel diseases during pregnancy
Intramedullary non-specific inflammatory lesion of thoracic spine: A case report
<p>Abstract</p> <p>Background</p> <p>There are several non-neoplastic lesions which mimick intramedullary spinal cord neoplasm in their radiographic and clinical presentation. These can be classified as either infectious (TB, fungal, bacterial, parasytic, syphilis, CMV, HSV) and non-infectious (sarcoid, MS, myelitis, ADEM, SLE) inflammatory lesions, idiopathic necrotizing myelopathy, unusual vascular lesions and radiation myelopathy. Although biopsy may be indicated in many cases, an erroneous diagnosis of intramedullary neoplasm can often be eliminated pre-operatively.</p> <p>Case description</p> <p>the authors report a very rare case of intramedullary non-specific inflammatory lesion of unknown origin, without signs of infection or demyelinization, in a woman who showed no other evidence of systemic disease.</p> <p>Conclusions</p> <p>Intramedullary lesions that mimick a tumor can be various and difficult to interpret. Preoperative MRI does not allow a certain diagnosis because these lesions have a very similar signal intensity pattern. Specific tests for infective pathologies are useful for diagnosis, but histological examination is essential for establishing a certain diagnosis. In our case the final histological examination and the specific tests that we performed have not cleared our doubts regarding the nature of the lesion that remains controversial.</p
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Superior MRI outcomes with alemtuzumab compared with subcutaneous interferon β-1a in MS
To describe detailed MRI results from 2 head-to-head phase III trials, Comparison of Alemtuzumab and Rebif Efficacy in Multiple Sclerosis Study I (CARE-MS I; NCT00530348) and Study II (CARE-MS II; NCT00548405), of alemtuzumab vs subcutaneous interferon β-1a (SC IFN-β-1a) in patients with active relapsing-remitting multiple sclerosis (RRMS).
The impact of alemtuzumab 12 mg vs SC IFN-β-1a 44 μg on MRI measures was evaluated in patients with RRMS who were treatment-naive (CARE-MS I) or who had an inadequate response, defined as at least one relapse, to prior therapy (CARE-MS II).
Both treatments prevented T2-hyperintense lesion volume increases from baseline. Alemtuzumab was more effective than SC IFN-β-1a on most lesion-based endpoints in both studies ( < 0.05), including decreased risk of new/enlarging T2 lesions over 2 years and gadolinium-enhancing lesions at year 2. Reduced risk of new T1 lesions ( < 0.0001) and gadolinium-enhancing lesion conversion to T1-hypointense black holes ( = 0.0078) were observed with alemtuzumab vs SC IFN-β-1a in CARE-MS II. Alemtuzumab slowed brain volume loss over 2 years in CARE-MS I ( < 0.0001) and II ( = 0.012) vs SC IFN-β-1a.
Alemtuzumab demonstrated greater efficacy than SC IFN-β-1a on MRI endpoints in active RRMS. The superiority of alemtuzumab was more prominent during the second year of both studies. These findings complement the superior clinical efficacy of alemtuzumab over SC IFN-β-1a in RRMS.
NCT00530348 and NCT00548405.
The results reported here provide Class I evidence that, for patients with active RRMS, alemtuzumab is superior to SC IFN-β-1a on multiple MRI endpoints.Dr. Havrdova was supported by the Czech Ministry of Education, PRVOUK-P26/LF1/4. The CARE-MS studies were funded by Sanofi Genzyme and Bayer HealthCare Pharmaceuticals
A pilot study of transrectal endoscopic ultrasound elastography in inflammatory bowel disease
BACKGROUND:
Using standard diagnostic algorithms it is not always possible to establish the correct phenotype of inflammatory bowel
disease which is essential for therapeutical decisions. Endoscopic ultrasound elastography is a new endoscopic procedure
which can differentiate the stiffness of normal and pathological tissue by ultrasound. Therefore, we aimed to investigate
the role of transrectal ultrasound elastography in distiction between Crohn's disease and ulcerative colitis. ----- METHODS:
A total 30 Crohn's disease, 25 ulcerative colitis, and 28 non-inflammatory bowel disease controls were included. Transrectal
ultrasound elastography was performed in all patients and controls. In all ulcerative coltis patients and 80% of Crohn's
disease patients endoscopy was performed to assess disease activity in the rectum. ----- RESULTS:
Significant difference in rectal wall thickness and strain ratio was detected between patients with Crohn's disease and
controls (p = 0.0001). CD patients with active disease had higher strain ratio than patients in remission (p = 0.02). In
ulcerative colitis group a significant difference in rectal wall thickness was found between controls and patients with
active disease (p = 0.03). A significant difference in rectal wall thickness (p = 0.02) and strain ratio (p = 0.0001) was
detected between Crohn's disease and ulcerative colitis patient group. Crohn's disease patients with active disease had a
significantly higher strain ratio compared to ulcerative colitis patients with active disease (p = 0.0001). ----- CONCLUSION:
Transrectal ultrasound elastography seems to be a promising new diagnostic tool in the field of inflammatory bowel
disease. Further study on a larger cohort of patients is needed to definitely assess the role of transrectal ultrasound
elastography in inflammatory bowel disease
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