Chronická zápalová demyelinizačná polyneuropatia (CIDP) je získaná, imunitne sprostredkovaná neuropatia, spôsobená zápalom periférnych nervov a nervových koreňov. Jedná sa o najčastejšiu chronickú autoimunitnú polyneuropatiu, ktorá je stále poddiagnostikovaným ochorením. Ak sa dlhodobo nelieči alebo je liečená nesprávne, môže viesť k závažnému zneschopneniu s narušením jemnej motoriky, chôdze a celkovej mobility pacienta. CIDP býva asociovaná s viacerými ochoreniami ako sú diabetes mellitus, monoklonálne gamapatie, infekcia HIV, malignity či viaceré systémové ochorenia. V poslednej dobe pribúda referencií, že prevalencia CIDP má tendenciu byť vyššia u diabetikov, najmä u pacientov vo vyššom veku. Diagnostika CIDP u pacienta s diabetom je náročná, pretože superponované axonálne poškodenie pri možnej diabetickej neuropatii môže zakryť typické demyelinizačné elektrofyziologické nálezy. Na druhej strane diabetická polyneuropatia môže spôsobiť zvýšenie hladiny proteínov v likvore. Vo vysvetlení asociácie týchto dvoch ochorení existuje stále viacero kontroverzií. Stále nemáme adekvátny diagnostický nástroj pre jasné definovanie CIDP u diabetika. Výzvou pre neurológov je práve identifikácia potenciálnych biomarkerov CIDP u diabetického pacienta, pretože CIDP je liečiteľné ochorenie.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated neuropathy caused by inflammation of peripheral nerves and nerve roots. It is the most common chronic autoimmune polyneuropathy, which is still considered underdiagnosed. If it remains untreated or improperly treated for a long time, it can lead to severe disability with impairment of the patient‘s fine motor skills, walking, and general mobility. CIDP may be associated with several diseases such as diabetes mellitus, monoclonal gammopathy, HIV infection, malignancies, or several systemic diseases. Recently, there have been several references that the prevalence of CIDP tends to be higher in diabetics, especially in older patients. Diagnosing CIDP in a patient with diabetes is challenging, because superimposed axonal damage in possible diabetic neuropathy can obscure typical demyelinating electrophysiological findings. On the other hand, diabetic polyneuropathy can cause elevated protein in cerebrospinal fluid. There are still many controversies in explaining the association of these two diseases. We still do not have an adequate diagnostic tool to clearly define CIDP in diabetic patients. The identifying a potential biomarkers of CIDP in diabetic patients is a challenge for neurologists, as CIDP is a treatable disease.
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INTRODUCTION: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory central nervous system (CNS) disorder, chiefly involving the brainstem, especially the pons. The diagnosis is challenging, requires careful exclusion of alternative diagnoses and a targeted therapeutic approach. CLIPPERS is known to respond well to corticosteroids, but the treatment needs to be long-term and can cause significant side-effects. Moreover, subsequent corticosteroid withdrawal often leads to a relapse. It has been suggested that anti-CD20 molecules could benefit several antibody-mediated CNS inflammatory diseases, including CLIPPERS. CASE REPORT: This paper describes two cases of CLIPPERS. The first demonstrates the benefit of early introduction of corticosteroids with side effects in cases of long-term use. The second demonstrates the efficacy of ocrelizumab (anti-CD20 molecule) in a severe course of CLIPPERS. CONCLUSION: These two cases bring attention to this rare, often misdiagnosed but treatable disease.
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- hormony kůry nadledvin terapeutické užití MeSH
- lidé MeSH
- magnetická rezonanční tomografie * MeSH
- nemoci centrálního nervového systému * komplikace diagnóza farmakoterapie MeSH
- pons MeSH
- progrese nemoci MeSH
- steroidy terapeutické užití MeSH
- zánět MeSH
- Check Tag
- lidé MeSH
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- kazuistiky MeSH
BACKGROUND: Despite the high prevalence of depression and anxiety in chronic pain conditions, current knowledge concerning emotional distress among painful diabetic polyneuropathy (pDSPN) and other diabetes mellitus (DM) sufferers is limited. METHODS: This observational multicentre cohort study employed the Hospital Anxiety and Depression Scale, the Beck Depression Inventory II and the State-Trait Anxiety Inventory to assess symptoms of depression and anxiety in several groups with diabetes, as well as in a control group. The study cohort included 347 pDSPN patients aged 63.4 years (median), 55.9% males; 311 pain-free diabetic polyneuropathy (nDSPN) patients aged 63.7 years, 57.9% males; 50 diabetes mellitus (DM) patients without polyneuropathy aged 61.5 years, 44.0% males; and 71 healthy controls (HC) aged 63.0 years, 42.3% males. The roles played in emotional distress were explored in terms of the biological, the clinical (diabetes-, neuropathy- and pain-related), the socio-economic and the cognitive factors (catastrophizing). RESULTS: The study disclosed a significantly higher prevalence of the symptoms of depression and anxiety not only in pDSPN (46.7% and 60.7%, respectively), but also in patients with nDSPN (24.4% and 44.4%) and DM without polyneuropathy (22.0% and 30.0%) compared with HCs (7.0% and 14.1%, p < 0.001). Multiple regression analysis demonstrated the severity of pain and neuropathy, catastrophic thinking, type 2 DM, lower age and female sex as independent contributors to depression and anxiety. CONCLUSIONS: In addition to the severity of neuropathic pain and its cognitive processing, the severity of diabetic polyneuropathy and demographic factors are key independent contributors to emotional distress in diabetic individuals. SIGNIFICANCE: In large cohorts of well-defined painless and painful diabetic polyneuropathy patients and diabetic subjects without polyneuropathy, we found a high prevalence of the symptoms of depression and anxiety, mainly in painful individuals. We have confirmed neuropathic pain, its severity and cognitive processing (pain catastrophizing) as dominant risk factors for depression and anxiety. Furthermore, some demographic factors (lower age, female sex), type 2 diabetes mellitus and severity of diabetic polyneuropathy were newly identified as important contributors to emotional distress independent of pain.
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- deprese epidemiologie MeSH
- diabetes mellitus 2. typu * komplikace epidemiologie MeSH
- diabetické neuropatie * MeSH
- kohortové studie MeSH
- lidé středního věku MeSH
- lidé MeSH
- neuralgie * diagnóza epidemiologie MeSH
- průřezové studie MeSH
- rizikové faktory MeSH
- úzkost epidemiologie MeSH
- Check Tag
- lidé středního věku MeSH
- lidé MeSH
- mužské pohlaví MeSH
- ženské pohlaví MeSH
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- časopisecké články MeSH
- multicentrická studie MeSH
- pozorovací studie MeSH
- práce podpořená grantem MeSH
Diabetes mellitus spôsobuje celé spektrum periférnych neuropatických komplikácií vrátane akútnych a chronických foriem a postihuje všetky úrovne periférneho nervu, počnúc nervovým koreňom a končiac distálnym axónom. Na diabetickú neuropatiu (DN) sa v súčasnosti pozeráme ako na heterogénne ochorenie s odlišnými patogenetickými mechanizmy u rozdielnych klinických syndrómov. Najčastejšiou formou DN je distálna symetrická polyneuropatia (DSPN), ktorá často prebieha zákerne a bezpríznakovo a jej najzávažnejšou komplikáciou je diabetická noha. Okrem už známych rizikových faktorov ako sú trvanie diabetu či dlhodobá úroveň metabolickej kontroly sa ako dôležité ukazujú kardiovaskulárne faktory (hypertenzia, hyperlipidémia, obezita), vek, fajčenie a prekonanie opakovaných epizód ketoacidózy. V prevencii rozvoja neuropatie majú rozhodujúce miesto kontrola glykémie, úprava životného štýlu a cvičenie. Z patogenetických liečiv má svoje pevné miesto kyselina alfalipoová. Terapia neuropatickej bolesti je potrebná najmä z hľadiska zlepšenia celkovej kvality života diabetikov. Veľký význam má včasná diagnostika diabetickej neuropatie, na včasné podchytenie pacientov s možnosťou terapeutickej intervencie.
Diabetes causes a broad sprectrum of neuropathic complications, including acute and chronic forms affecting each level of theperipheral nerve, from the root to the distal axon. Diabetic neuropathy (DN) is currently considered a heterogeneous disease withdifferent pathogenetic mechanisms in different clinical syndromes. The most common form is distal symmetric polyneuropathy(DSPN), which often goes insidious and unnoticed and its most serious complication is diabetic foot. In addition to knownrisk factors such as diabetes duration or long-term metabolic control, new factors such as cardiovascular factors (hypertension,hyperlipidemia, obesity), age, smoking, and recurrent episodes of ketoacidosis have recently been identified. In preventing thedevelopment of neuropathy, metabolic control, lifestyle modification and exercise are very important. Among the pathogeneticdrugs, alphalipoic acid has a dominant position. Neuropathic pain treatment is particularly needed to improve the overall patient´s quality of life. Early diagnosis of diabetic neuropathy is crucial for early medical intervention.
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