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INTRODUCTION/AIMS: Laboratory and clinical data suggest a link between neurologically mediated inflammation and psoriasis, but the risk and features of peripheral neuropathy in psoriasis or psoriatic arthritis remain unknown. The aim of this exploratory study was to evaluate the risk and to describe the features of peripheral neuropathy in patients with psoriasis and psoriatic arthritis. METHODS: One hundred patients with psoriasis and/or psoriatic arthritis and 100 control subjects were consecutively enrolled. Diagnostic confirmation included electrophysiological examination, skin biopsy, and nerve ultrasound for confirmed polyneuropathy. RESULTS: Nine patients were diagnosed with confirmed polyneuropathy, while none of the control subjects had the condition (relative risk [RR] = 19.00, 95% confidence interval [CI] = 1.12-322.11). Specific relative risks for polyneuropathy were 22.09 (95% CI = 1.17-416.43) in psoriasis patients and 18.75 (95% CI = 1.07-327.62) in psoriatic arthritis patients. The observed polyneuropathy in all nine patients was length-dependent, symmetrical, and predominantly sensory, with minimal or no disability. Comorbidities and exposure to therapies known to increase the risk of polyneuropathy were more frequent in psoriasis and/or psoriatic arthritis patients compared to controls (42% vs. 4%, p = .0001). Analyzing data after excluding possible contributory causes, the risk of polyneuropathy in patients with psoriasis and/or psoriatic arthritis was not significant. DISCUSSION: Psoriasis and psoriatic arthritis appear to be associated with an increased risk of polyneuropathy. This increased risk seems to be linked to the higher prevalence of contributing factors for polyneuropathy, rather than a direct increase in neuropathy risk specifically related to psoriasis and psoriatic arthritis.
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Artritis Psoriásica , Enfermedades del Sistema Nervioso Periférico , Psoriasis , Humanos , Femenino , Masculino , Artritis Psoriásica/complicaciones , Artritis Psoriásica/epidemiología , Persona de Mediana Edad , Psoriasis/complicaciones , Psoriasis/epidemiología , Enfermedades del Sistema Nervioso Periférico/epidemiología , Enfermedades del Sistema Nervioso Periférico/etiología , Adulto , Estudios Prospectivos , Anciano , Estudios de Cohortes , Factores de RiesgoRESUMEN
BACKGROUND AND PURPOSE: A diagnostic score was developed to discriminate anti-myelin-associated-glycoprotein (MAG) neuropathy from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and applied it to patients with atypical anti-MAG neuropathy. METHODS: The clinical and electrophysiological features of patients with a diagnosis of typical anti-MAG neuropathy were compared to those of patients with a diagnosis of CIDP. The association of each feature with the diagnosis was assessed in the two groups. Features showing a significant association with the diagnosis were included in a multivariable logistic regression model and adjusted odds ratios were estimated for each feature. A score ranging from 1 to 3 was applied to each feature based on the magnitude of the estimated odds ratios. The score was then applied to patients with a clinical diagnosis of CIDP who also had high anti-MAG antibody titers (CIDP-MAG). RESULTS: Thirty-one anti-MAG neuropathy patients, 45 typical CIDP patients and 16 CIDP-MAG patients were included. Scores in anti-MAG antibody patients ranged from 1 to 5 and in CIDP patients from -7 to -1. Using the score, 4/16 CIDP-MAG patients were diagnosed to have anti-MAG neuropathy and 12/16 patients to have CIDP. Response to intravenous immunoglobulin in the CIDP-MAG patients classified as CIDP was similar to that of definite CIDP patients and higher than that of anti-MAG neuropathy patients. CONCLUSIONS: Our score allowed an accurate discrimination to be made, amongst patients with anti-MAG antibodies, of those affected by CIDP and the patients with anti-MAG neuropathy. This score may help proper treatment to be chosen for patients with anti-MAG antibodies with a CIDP-like presentation.
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Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante , Polirradiculoneuropatía , Humanos , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/terapia , Inmunoglobulina M , Inmunoglobulinas Intravenosas/uso terapéutico , Autoanticuerpos , Glicoproteína Asociada a Mielina , Polirradiculoneuropatía/tratamiento farmacológicoRESUMEN
BACKGROUND AND AIMS: To develop recommendations for vaccination for coronavirus-19 (COVID-19) in patients with inflammatory neuropathies. METHODS: Key questions were formulated in order to perform a literature review on the safety and efficacy of vaccines in patients with inflammatory neuropathies. Based on the best evidence and expert opinion, a list of recommendations was formulated to inform decision on vaccination for COVID-19 in patients with inflammatory neuropathies and increase adherence to vaccination programmes. RESULTS: Recommendations addressing safety and efficacy of vaccination in patients with inflammatory neuropathies were formulated. No data are currently available on the safety and efficacy of COVID-19 vaccines in patients with inflammatory neuropathies or other immune-mediated conditions. There is only sparse data on the safety of previous available vaccines in patients with inflammatory neuropathies, but studies on other autoimmune disorders indicate that these are safe and mostly efficacious. Patients with inflammatory neuropathies might be at increased risk for severe illness from COVID-19. INTERPRETATION: Patients with inflammatory neuropathies should be encouraged to adhere to the vaccination campaign for COVID-19. These recommendations provide guidance on the management of vaccinations for COVID-19 in patients with inflammatory neuropathies. More research is needed regarding the safety and efficacy of vaccination in patients with inflammatory neuropathies and other immune conditions.
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Vacunas contra la COVID-19/uso terapéutico , Síndrome de Guillain-Barré , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante , Guías de Práctica Clínica como Asunto/normas , Sociedades Médicas/normas , Vacunación/normas , Vacunas contra la COVID-19/efectos adversos , Humanos , ItaliaRESUMEN
BACKGROUND: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a rare and disabling immunomediated radiculoneuropathy. Its worldwide epidemiology is heterogeneous and, in adults, CIDP prevalence varies from 0.6 to 9 cases per 100,000 population. Juvenile CIDP (jCIDP) is even rarer, with age-specific prevalence rates varying from 0.23 to 1.26 owing to different diagnostic criteria (American Academy of Neurology [AAN] and European Federation of Neurological Societies/Peripheral Nerve Society [EFNS/PNS]), different age grouping or, genuine differences. OBJECTIVES: We assessed jCIDP incidence and prevalence in Sardinia, an area at very-high risk for autoimmune diseases, using comparable methods. DESIGN: The study area was the northern Sardinia, insular Italy, with 491,571 inhabitants and a pediatric population (0-18 years) of 79,086 individuals. RESULTS: On prevalence day (December 31, 2019) the total crude, age-specific prevalence rate were 6.32 per 100,000 according with AAN criteria, 7.58 per 100,000 population with European Neuromuscular Center (ENMC) criteria, and 8.85 per 100,000 population with both 2006 and 2010 EFNS/PNS criteria. Crude mean incidence rate were 0.42 per 100,000 per year with AAN criteria, 0.50 per 100,000 per year with ENMC criteria, and 0.59 per 100,000 per year using 2006 and 2010 EFNS/PNS criteria. Of the eight patients, six had typical CIDP, one had multifocal-acquired demyelinating sensory and motor neuropathy (MADSAM), and one chronic immune sensory polyradiculopathy (CISP). Patient's disability was generally mild. Clinical course was progressive, monophasic, or relapsing. CONCLUSION: jCIDP prevalence and incidence rates in Sardinia were criteria-dependent, the lowest obtained when using AAN criteria, the highest using the EFNS/PNS. Nonetheless, even with the exclusion of the "possible" category, by using comparable methodology, prevalence rates in Sardinia are considerably higher than the range reported in all previous jCIDP studies.
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Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/epidemiología , Guías de Práctica Clínica como Asunto , Adolescente , Niño , Preescolar , Femenino , Humanos , Incidencia , Lactante , Italia , Islas del Mediterráneo/epidemiología , PrevalenciaRESUMEN
INTRODUCTION: Monitoring grip strength at home may detect improvement between intravenous immunoglobulin (IVIg) treatments in patients with chronic inflammatory neuropathies (CINs). METHODS: Fifteen patients recorded grip strength each day, from one IVIg treatment until the next. We analyzed grip strength changes comparing thresholds of 8 kPa and 14 kPa. "Random" fluctuations of grip strength were distinguished from treatment response by smoothing the data. RESULTS: "Random" fluctuations of at least 8 kPa occurred in 27% of patients. Smoothed daily grip strength increased by at least 8 kPa above baseline in 11 (73%) patients. Grip strength increased by at least 8 kPa for 3 consecutive days in 9 (60%) patients, and 5-day block mean increased by at least 8 kPa in 10 (67%) patients. DISCUSSION: Home monitoring of grip strength confirmed treatment response in most patients with CINs on IVIg. To detect improvement in an individual patient, we suggest a threshold of at least 8 kPa on 3 consecutive days or on 5-day block mean.
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Fuerza de la Mano/fisiología , Servicios de Atención de Salud a Domicilio/tendencias , Inmunoglobulinas Intravenosas/administración & dosificación , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/tratamiento farmacológico , Adulto , Anciano , Femenino , Servicios de Atención de Salud a Domicilio/normas , Humanos , Masculino , Persona de Mediana Edad , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/fisiopatología , Autoinforme/normasRESUMEN
PURPOSE OF REVIEW: To review the recent advances in the management and treatment of chronic inflammatory demyelinating polyradiculopathy (CIDP). RECENT FINDINGS: Recent studies confirm the efficacy/safety of long-term intravenous immunoglobulin (IVIg) and short-term subcutaneous immunoglobulin (SCIg) therapy in CIDP. New outcome measures have been recently proposed and further studies evaluated the properties of those already in use. The presence of antibodies against proteins at the node of Ranvier was associated with specific clinical features and treatment response. Fingolimod adds to the list of immunosuppressive agents that failed to be effective in a controlled trial. SUMMARY: Several studies evaluating the best strategy to provide maintenance IVIg treatment in CIDP are in progress. SCIg were shown to be an alternative to IVIg for maintenance treatment while their efficacy as initial therapy should be further addressed. New outcome measures have been shown to be effective in detecting treatment response in clinical trials, but their use in clinical practice remains uncertain. Similarly unsettled is the role of nerve imaging techniques as biomarker in CIDP. The discovery of antibodies against proteins at the node of Ranvier has rekindled a keen interest in the pathogenesis of CIDP and the potential therapeutic role of new agents.
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Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/terapia , Clorhidrato de Fingolimod/uso terapéutico , Humanos , Inmunización Pasiva , Inmunoglobulinas Intravenosas/uso terapéutico , Inmunosupresores/uso terapéutico , Insuficiencia del Tratamiento , Resultado del TratamientoRESUMEN
Ulnar tunnel syndrome indicates ulnar neuropathy at different sites within the wrist. Several classifications of ulnar tunnel syndrome are present in literature, based upon typical nerve anatomy. However, anatomical variations are not uncommon and can complicate assessment. The etiology is also complex, due to the numerous potential causes of entrapment. Clinical examination, neurophysiological testing, and imaging are all used to support the diagnosis. At present, many therapeutic approaches are available, ranging from observation to surgical management. Although ulnar neuropathy at the wrist has undergone extensive prior study, unresolved questions on diagnosis and treatment remain. In the current paper, we review relevant literature and present the current knowledge on ulnar tunnel syndrome.
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Nervio Cubital/fisiopatología , Neuropatías Cubitales/patología , Muñeca/fisiopatología , Electrofisiología , Humanos , Neuroimagen , Nervio Cubital/diagnóstico por imagen , Neuropatías Cubitales/diagnóstico por imagen , Neuropatías Cubitales/etiología , Neuropatías Cubitales/terapia , Muñeca/diagnóstico por imagen , Muñeca/inervaciónRESUMEN
A paradoxical effect of antiepileptic drugs was defined as an increased seizure frequency or severity occurring shortly after introducing a drug considered effective for that kind of epilepsy. In addition, this effect should occur at nontoxic drug serum levels. So far, pathophysiological mechanisms underlying this phenomenon have not been clarified. Recent evidence suggests that the variability of drug effects may depend on precise intrinsic properties of dynamic networks involving the drug and its binding site. Although several reports of paradoxical seizure exacerbation have been reported for levetiracetam (LEV), a possible association with focal cortical dysplasia has never been described nor investigated. In this report, we document a paradoxical effect induced by LEV monotherapy in a patient with type II focal cortical dysplasia at LEV serum levels within the therapeutic range. A hint of pathophysiological hypothesis underlying this potential relationship will be also suggested.