Factores asociados a respuesta favorable de tratamiento corticoidal en pacientes con polirradiculoneuropatía desmielinizante inflamatoria crónica / Variables associated with a good response to steroids in chronic inflammatory demyelinating polyradiculoneuropathy

Background: The treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is based on corticosteroids, immunoglobulin and plasmapheresis. In our Health System, corticosteroids are commonly used as first line therapy for economic reasons and accessibility. However, the factors associated with a good response are not well known. Aim: To assess the association of demographic, clinical and laboratory variables with a favorable response to corticosteroid therapy in patients with CIDP. Material and Methods: Observational, descriptive, longitudinal and retrospective study of 33 patients with a diagnosis of typical, definitive or probable CIDP, treated with corticosteroids for at least six months. Results: Twenty-three patients had a good clinical response to corticosteroid treatment and 10 were non-responders. The variables significantly associated with a good response to steroids were a disease lasting less than 1 year prior to the start of treatment, the absence of axonal damage in electromyography a relapsing-recurrent course and a favorable response within two months of treatment. Conclusions: Most of these patients with CIDP had good response to corticosteroid therapy.

Inmunoglobulina subcutánea: Tratamiento en polineuropatía desmielinizante inflamatoria crónica / Subcutaneous immunoglobulin. Treatment in chronic inflammatory demyelinating polyradiculo-neuropathy

La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad adquirida que puede afectar a raíces, plexos y nervios periféricos. A pesar de su baja incidencia, su diagnóstico cobra especial relevancia dado que actualmente existen tratamientos efectivos para la misma. La gammaglobulina humana endovenosa (IVIgG) es, junto con los esteroides y la plasmaféresis, uno de los tratamientos de primera elección. La vía de administración subcutánea se ha propuesto como una alternativa novedosa frente a la administración endovenosa con una eficacia similar. Presentamos tres casos de CIDP definitiva, clasificados según los criterios de la European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) en los cuales se utilizó tratamiento crónico con inmunoglobulina subcutánea (IgSC). Todos ellos habían recibido tratamiento previo con IVIgG. Se obtuvo mejoría de la fuerza evaluada por Overall Neuropathy Limitations Scale (ONLS) y los tres pacientes manifestaron una mejor adaptación a sus actividades de la vida diaria.

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disease that may affect nerve roots and peripheral nerves. Despite its low incidence, diagnosis is particularly important because there are different effective treatments. Human immunoglobulin is one of the mainstays of the treatment. Although there are few studies up to date, subcutaneous immunoglobulin (IgSC) has been proposed as an alternative to intravenous administration with similar efficacy. We present three cases with definite CIDP, classified according to the European Federation of Neurological Societies / Peripheral Nerve, Society (EFNS /PNS) criteria in which was used SCIgG as a treatment after success with the intravenous route. The Overall Neuropathy Limitations Scale (ONLS) was used to estimate the changes in the muscular strength before and after treatment.

study of chronic inflammatory demyelinating polyneuropathy [CIDP] in diabetics: clinical features, laboratory findings and response to treatment

The association of chronic inflammatory demyelinating neuropathy [CIDP] in diabetics is a recently recognized form of neuropathy. It is important to recognize CIDP occurring in diabetics because, unlike diabetic polyneuropathy, it is treatable. These patients can respond to immune therapies similar to patients with CIDP without diabetes. To study the clinical, electrophysiological, and laboratory features and response to immune modulating treatments in diabetic patients with CIDP. This was a retrospective cross sectional study from January 2009 till December 2012 carried out at Mayo Hospital and National Hospital, Lahore. The inclusion criteria included proven cases of diabetes mellitus with subacute motor weakness fulfilling the research criteria for diagnosis of CIDP. All patients underwent electrophysiological [EP] studies and cerebrospinal fluid analysis [CSF] especially for proteins. Diagnosed CIDP patients were treated with oral prednisolone 1mg/kg body weight along with azathioprine 50-150mg/day. The steroids were gradually tapered after achieving normal muscle strength or a static phase of one month without further improvement in muscle strength. The maintenance dose of prednisolone was continued to complete two years therapy. A course of IVIg [400mg/kg body weight daily for five days] or plasmapharesis [five sessions on alternate days] were used in patients with severe motor weakness to expedite the initial recovery phase. Follow was done at monthly interval for one year and bimonthly for subsequent years. The Hughes functional grading scale was used to assess the outcome. Treatment was considered effective when the patient's condition improved by 1 or more grade on the Hughes scale. There were 10 patients with 6[60%] males and 4[40%] females and M: F ratio of 1.5:1. The mean age of patients was 63.7 + 7.83 years. Mean duration of diabetes mellitus was 11.3 +3.77 years. All patients had Type 2 diabetes mellitus with six patients on Insulin and 4 on oral hypoglycemic agents. Mean duration of motor weakness before treatment was 5.30 + 1.16 months. Mean power as assessed by medical research council [MRC] grading in upper limbs was 3/5 [range 1-5] and lower limbs 1/5[range 0-2]. Seven [70%] patients had mixed demyelinating and axonal picture on EMG, and 3[30%] patients showed predominantly demyelinating type of neuropathy. CSF protein was high in all patients with mean CSF protein of 208.4 + 93.07 mg/dl. Mean duration of follow up after treatment was 25.10 + 15.82 months. Attempt was done to stop immunotherapy after 2 years but relapse occurred in 3 [30%] patients which again responded to steroids. Outcome was assessed by Hughes functional grading scale. Mean Hugh's functional severity grade before treatment was 4.10 + 0.316 and after treatment was 1.30 + 1.16. We conclude that CIDP in diabetics is potentially reversible type of neuropathy and needs careful evaluation for its recognition. The classical demyelinating pattern on EMG may be lacking because of coexistent axonal neuropathy but clinical history of subacute onset predominantly proximal motor weakness [LMN type] and high CSF protein are most sensitive markers to predict response to corticosteroids in these patients. This is more common in males and elderly long standing diabetics. The response to corticosteroids and other immunosuppressive therapies is excellent. The recognition of this entity is important as appropriate management can reverse the disability in these patients

Vitiligo y terapia con inmunoglobulina intravenosa: a propósito de un paciente con síndrome de Sjögren / Remission of vitiligo during treatment with intravenous immunoglobulin: Report of one case

Vitiligo is associated with other autoimmune diseases. We report a 52-year-old male with a Sjögren syndrome that was treated with monthly pulses of intravenous immunoglobulin for a chronicinflammatory demyelinating polyradiculoneuropathy. The neurological disorder responded adequately to the treatment and the patient also noted a marked remission of his vitiligo with almost compete re-pigmentation of the scalp and face and partial repigmentation of other areas.

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.

Interferon beta-1a in chronic inflammatory demyelinating polyneuropathy: case report

La polineuropatía crónica inflamatoria desmielinizante (PCID) es una neuropatía inmuno-mediada, que presenta un curso clínico primariamente progresivo o en forma de recaídas. Las manifestaciones sensoriales pueden ser su unica forma de expresión clínica. El tratamiento con interferon beta 1a (IFN beta 1a) ha sido ensayado en varias oportunidades, con diferentes respuestas terapéuticas, en pacientes refractarios a las terapias inmunomoduladoras convencionales. Nosotros comunicamos un paciente con una forma ataxica recurrente de PCID, que no respondió al tratamiento con inmunoglobulina endovenosa. Posteriormente fue tratado con IFN beta 1 a por tres años. Durante el período de seguimiento no mostró nuevas recaídas y su cuadro neurológico se estabilizó.