Eficacia de la movilización de células madre en pacientes con Polineuropatía Desmielinizante Inflamatoria Crónica refractaria / Efficacy of mobilization of stem cells in patients with Refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Introducción: La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad desmielinizante e inflamatoria de mediación autoinmune. El tratamiento convencional es basado en la inmunomodulación e inmunosupresión. El uso de células madre es una terapia novedosa en los trastornos autoinmune, siendo incluida como terapia. Objetivo: Determinar la eficacia de la movilización de células madre mediante la aplicación del factor estimulador de colonias granulocíticas (F-ECG) en pacientes con CIDP que han recibido otras líneas de tratamiento. Material y Métodos: Se realizó un estudio aleatorizado, doble ciego sobre una cohorte de 45 pacientes con CIDP, donde se administró el (F-ECG) en 25 pacientes y 20 continuaron con el tratamiento habitual, tratados anteriormente con otras variantes terapéuticas por más de tres años, sin respuesta satisfactoria. Resultados: Predominio de los hombres para 64,4 por ciento, la Diabetes Mellitus tuvo mayor asociación y la medicación más usada fueron los esteroides. Los síntomas y signos clínicos mejoraron significativamente tras el tratamiento. Los valores de la puntuación del TCSS al mes y 3 meses después del tratamiento disminuyeron significativamente; pero este decremento no se mantuvo al final del estudio. La velocidad de conducción y el potencial de acción de los nervios sensoriales y motores mejoraron considerablemente después del tratamiento. Conclusiones: La efectividad de la aplicación del (F-ECG) para la mejoría de los síntomas clínicos y resultados de estudios neurofisiológicos evolutivamente son mayores que otras variantes terapéuticas en los primeros meses, con buena seguridad y tolerabilidad, por lo que se puede incluir en la terapéutica convencional para la CIDP(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune demyelinating disease. Conventional treatment is based on immunomodulation and immunosuppression. The use of stem cells is a novel therapy in autoimmune disorders, so it is included as therapy. Objective: To determine the efficacy of mobilization of stem cells by applying granulocyte colony-stimulating factor (G-CSF) in patients with CIDP who have followed other lines of treatment. Material and Methods: A randomized, double-blind study was carried out on a cohort of 45 patients with CIDP. G-CSF was administered to 25 patients and 20 of them continued with the usual treatment. These patients were previously treated with other therapeutic variants for more than three years without satisfactory response. Results: There was a prevalence of men (64.4 percent), Diabetes Mellitus had a greater association, and the most used medications were steroids. Clinical symptoms and signs improved significantly after treatment. TCSS scores significantly decreased at one and three months after treatment, but this decrease was not maintained at the end of the study. The conduction velocity and action potential of sensory and motor nerves improved considerably after treatment. Conclusions: The effectiveness of the use of G-CSF shows an improvement of clinical symptoms. The results of neurophysiological studies have a better course than other therapeutic variants during the first months, with good safety and tolerability, so it can be included in the conventional therapy for the CIDP(AU)

Polineuropatía desmielinizante inflamatoria crónica como forma de presentación de lupus eritematoso sistémico / Chronic inflammatory demyelinating polyneuropathy as a form of presentation of systemic lupus erythematosus

Se describe el caso de una mujer de 35 años que presenta polineuropatía desmielinizante inflamatoria crónica como compromiso neurológico en su diagnóstico inicial de lupus eritematoso sistémico (LES). Si bien el compromiso neurológico es de una prevalencia variable en lupus, la asociación que se describe no es frecuente y tiene importantes connotaciones en el tratamiento.

We described a 35 years old female, who developed Chronic inflammatory demyelinating polyneuropathy as neurologic commitment during the early diagnosis in Systemic Lupus Erithematosus (SLE). While the neuropsychiatric commitment has a variable prevalence in SLE, the association that we describe is infrequent and it has important concerns during its treatment.

Polineuropatía desmielinizante inflamatoria crónica como forma de presentación de lupus eritematoso sistémico / Chronic inflammatory demyelinating polyneuropathy such as presentation of systemic lupus erythematosus

Se describe el caso de una mujer de 35 años que presenta polineuropatía desmielinizante inflamatoria crónica como compromiso neurológico en su diagnóstico inicial de lupus eritematoso sistémico (LES). Si bien el compromiso neurológico es de una prevalencia variable en lupus, la asociación que se describe no es frecuente y tiene importantes connotaciones en el tratamiento.

We described a 35 years old female, who developed Chronic inflammatory demyelinating polyneuropathy as neurologic commitment during the early diagnosis in Systemic Lupus Erithematosus (SLE). While the neuropsychiatric commitment has a variable prevalence in SLE, the association that we describe is infrequent and it has important concerns during its treatment.

Tonic pupils: an unusual autonomic involvement in chronic inflammatory demyelinating polyneuropathy (CIDP).

BACKGROUND: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neuropathy which affects mainly large myelinated axons and has a typically mild autonomic dysfunction mainly from postganglionic nerve fiber involvement. CASE REPORT: We report here an acute onset CIDP initially diagnosed as Guillain-Barré syndrome (GBS), unresponsive to treatment with intravenous immunoglobulin (IVIg), which later responded to plasmapheresis and corticoids. The patient had a markedly distal demyelination, prominent cranial nerve involvement and, interestingly, bilateral fixed dilated pupils. Despite complete clinical recovery, this neurological sign remained. CONCLUSIONS: Tonic pupils have previously been described in different neurologic conditions, including GBS, but not yet in acute onset CIDP or in variants with predominantly distal demyelination. It differs from the classical Adie's pupil because it lacks the light-near dissociation. This case report expands the range of possible autonomic signs in acute onset CIDP, which could help physicians establish optimal treatment strategies earlier on.

[Chronic inflammatory demyelinating polyneuropathy in a diabetic patient with syphilis]. / Polineuropatía desmielinizante inflamatoria crónica en paciente diabética con sífilis.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder characterized by motor symptoms such as weakness in both proximal and distal muscles with globally diminished or absent reflexes. Insulin neuritis is referred as an acute pain in the extremities, due to the damage of peripheral nerves affecting mainly small fibers, in diabetic patients treated with insulin who achieved rapid glycemic control. Pain is unusual in classic CIDP. We report the case of a 54-year-old female patient with type II diabetes mellitus, and a recent onset of insulin therapy, who presented at the emergency room with a 2-month history of weakness and hyperalgesia of extremities. Physical examination showed marked pain and proximal and distal allodynia in the 4 limbs, with reduced muscle strength of the proximal muscles and patellar and achillear areflexia. Electrophysiological study showed sensory and motor polyneuropathy with a demyelinating predominance. Treatment with recombinant human immunoglobin was started, and the patient presented a total remission of the condition. Complementary studies confirmed weak serum positivity of GM1, GD1a, GD1b and anti-asialo GM1. Prior to hospital discharge, results of positive serum VDRL and FTA-Abs were received. VDRL in cerebrospinal fluid was negative, so neurosyphilis was ruled out, and treatment with benzathine penicillin was indicated.

Polineuropatía desmielinizante inflamatoria crónica en paciente diabética con sífilis / Chronic inflammatory demyelinating polyneuropathy in a diabetic patient with syphilis

La polineuropatía desmielinizante inflamatoria crónica (PDIC) se presenta generalmente con síntomas motores, debilidad tanto en los músculos proximales como en los distales con reflejos globalmente disminuidos o ausentes. La neuritis insulínica es un trastorno caracterizado por dolor agudo de las extremidades, y daño de los nervios periféricos con afectación predominante de las fibras pequeñas, en los pacientes diabéticos sometidos a un rápido control glucémico. El dolor es raro en la PDIC clásica. Describimos el caso de una mujer de 54 años con diabetes mellitus (DB) tipo II, en tratamiento reciente con insulina, que consultó por un cuadro de debilidad e hiperalgesia de los cuatro miembros de dos meses de evolución. Al examen físico presentaba dolor de intensidad 10/10 y alodinia en los cuatro miembros, a niveles proximal y distal, con fuerza muscular reducida de los músculos proximales y arreflexia patelar y aquilea bilateral. Se realizó un estudio electrofisiológico, el cual mostró una polineuropatía sensitiva y motora desmielinizante. Se indicó tratamiento con inmunoglobina humana recombinante, con total remisión del cuadro. Estudios realizados posteriormente demostraron positividad débil de los anticuerpos GM1, GD1a, GD1b y anti-asialo GM1. Previo al alta hospitalaria se recibieron los resultados de VDRL sérica positiva, y FTA-Abs. VDRL en líquido cefalorraquídeo fue negativa por lo que se descartó neurosífilis, indicándose tratamiento con penicilina benzatínica.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a disorder characterized by motor symptoms such as weakness in both proximal and distal muscles with globally diminished or absent reflexes. Insulin neuritis is referred as an acute pain in the extremities, due to the damage of peripheral nerves affecting mainly small fibers, in diabetic patients treated with insulin who achieved rapid glycemic control. Pain is unusual in classic CIDP. We report the case of a 54-year-old female patient with type II diabetes mellitus, and a recent onset of insulin therapy, who presented at the emergency room with a 2-month history of weakness and hyperalgesia of extremities. Physical examination showed marked pain and proximal and distal allodynia in the 4 limbs, with reduced muscle strength of the proximal muscles and patellar and achillear areflexia. Electrophysiological study showed sensory and motor polyneuropathy with a demyelinating predominance. Treatment with recombinant human immunoglobin was started, and the patient presented a total remission of the condition. Complementary studies confirmed weak serum positivity of GM1, GD1a, GD1b and anti-asialo GM1. Prior to hospital discharge, results of positive serum VDRL and FTA-Abs were received. VDRL in cerebrospinal fluid was negative, so neurosyphilis was ruled out, and treatment with benzathine penicillin was indicated.

Differences between acute-onset chronic inflammatory demyelinating polyneuropathy and acute inflammatory demyelinating polyneuropathy in adult patients.

Acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are conditions presenting overlapping clinical features during early stages (first 4 weeks), although the latter may progress after 8 weeks. The aim of this study was to identify predictive factors contributing to their differential diagnosis. Clinical records of adult patients with AIDP or A-CIDP diagnosed at our institution between January 2006 and July 2017 were retrospectively reviewed. Demographic characteristics, clinical manifestations, cerebrospinal-fluid (CSF) findings, treatment and clinical evolution were analyzed. Nerve conduction studies were performed in all patients with at least 12 months follow-up. A total of 91 patients were included (AIDP, n = 77; A-CIDP, n = 14). The median age was 55.5 years in patients with A-CIDP vs 43 years in AIDP (P = .07). The history of diabetes mellitus was more frequent in A-CIDP (29% vs 8%, P = .04). No significant differences between groups were observed with respect to: human immunodeficiency virus (HIV) status, presence of auto-immune disorder or oncologic disease. Cranial, motor and autonomic nerve involvement rates were similar in both groups. Patients in the A-CIDP group showed higher frequency of proprioceptive disturbances (83% vs 28%; P < .001), sensory ataxia (46% vs 16%; P = .01), and the use of combined immunotherapy with corticoids (29% vs 3%; P = .005). There were no significant differences in CSF findings, intensive care unit (ICU) admission, or mortality rates. During the first 8 weeks both entities are practically indistinguishable. Alterations in proprioception could suggest A-CIDP. Searching for markers that allow early differentiation could favor the onset of corticotherapy without delay.

Miopatía inflamatoria idiopática asociada a polineuropatía desmielinizante inflamatoria crónica. Reporte de un caso / Idiopatic inflammatory myopathies (IIM) associated with chronic inflammatory demyelinating polyneuropathy (CIDP): a case report

Introducción: las Miopatías Inflamatorias Idiopáticas (MII) y la Polineuropatía Desmielinizante Inflamatoria Crónica (PDIC) son desórdenes autoinmunes. Sólo se ha comunicado un caso infantil con estas dos entidades asociadas. Presentamos un adulto que desarrolló una PDIC asociando aumento de creatinfosfoquinasa (CK) y cambios inflamatorios en la biopsia muscular. Caso clínico: Paciente masculino, 52 años, que presenta debilidad proximal y distal de cuatro miembros, arreflexia y trastornos sensitivos distales. Estudios lectrofisiológicos: latencias distales prolongadas con velocidades de conducción disminuidas, sin denervación. CK: 1516 UI/l. Biopsia muscular: infiltrado inflamatorio perimisial, y perivascular focal. Conclusión: Según la revisión bibliográfica realizada a la fecha sería la primera comunicación de PDIC y MII asociadas en adultos.

Introduction: idiopathic Inflammatory Myopathies (IIM) and ChronicInflammatory Demyelinating Polyneuropathy (CIDP) are autoimmunedisorders. Only one child case with the association of these two entitieshas been reported. We report an adult patient with CIDP whopresented creatine kinase (CK) elevation and inflammatory changeson muscle biopsy. Case report: 52 year old man who presentedproximal and distal weakness with areflexia and mild distal sensorydisturbances. Electrophysiological studies showed prolonged distallatencies and decreased conduction velocities without significantdenervation. CK was 1516 IU/l. A Muscle biopsy showed focal perivascularand perimysial inflammatory infiltrates. Conclusion: Accordingto the literature review to date this would be the first communicationof CIDP and IIM associated in an adult.

Polineuropatia desmielinizante inflamatória crônica pós-tratamento com interferon peguilado alfa 2b em um paciente co-infectado HIV/HCV: relato de caso / Chronic inflammatory demyelinating polyneuropathy after treatment with pegylated interferon alpha 2b in a patient with HIV/HCV coinfection: case report

A polineuropatia desmielinizante inflamatória cônica possui forte associação com a infecção pelo HIV e HCV. Uma rara associação entre PDIC e o tratamento da hepatite C com interferon peguilado alfa foi descrita recentemente. Nós descrevemos o primeiro caso de polineuropatia desmielinizante inflamatória crônica em um paciente branco, sexo masculino infectado por HIV e HCV associado a interferon peguilado alfa 2b. O paciente recuperou-se completamente após o uso de imunoglobulina hiperimune endovenosa. Infectologistas e hapatologistas devem estar atentos à esta rara e grave associação, que exige imediata descontinuação da droga e tratamento precoce.

Chronic inflammatory demyelinating polyneuropathy has a strong association with HIV and HCV infection. A rare association between chronic inflammatory demyelinating polyneuropathy and hepatitis C treatment with pegylated interferon alpha was described recently. We described the first case of chronic inflammatory demyelinating polyneuropathy associated with pegylated interferon alpha 2b in a white man infected with HIV and HCV. The patient recovered completely with the use of intravenous hyperimmune immunoglobulin. Infectologists and hepatologists should be alert regarding this rare and serious association, which requires immediately drug discontinuation and early treatment.