Search

SUMMARY OBJECTIVE: The role of interleukins, such as IL-17 and IL-34, in the pathogenesis of autoimmune diseases has been established in the literature. In the current study, we aimed to identify the concentrations of IL-17 (IL-17A, IL-17F) and IL-34 in the cerebrospinal fluid (CSF) of patients with chronic inflammatory demyelinating polyneuropathy (CIDP) and acute inflammatory demyelinating neuropathy (AIDN). METHODS: We included in this study 8 patients with CIDP (none of them receiving immunomodulatory or immunosuppressant therapy), 7 patients with Guillain-Barre syndrome (GBS, AIDN), and 7 control subjects. The CIDP and AIDN diagnoses were made by clinical evaluation and electrophysiological investigations according to international criteria. CSF samples were obtained appropriately, and the levels of IL-17A, IL-17F, and IL-34 were measured by ELISA kits. RESULTS: The concentrations of IL-17A, IL-17F, and IL-34 were higher in those with CIDP and AIDN compared to the controls (p=0.005, p=0.01, and p=0.001, respectively). While IL-34 levels were significantly higher in AIDN patients than in CIDP patients (p=0.04), there were no significant differences between the AIDN and CIDP groups with regard to the levels of IL-17A and IL-17F (p=0.4 and p=0.2, respectively) CONCLUSION: Our results indicate that IL-17A, IL-17F, and IL-34 levels may have a role in CIDP and AIDN. Furthermore, the difference in the IL-34 levels of patients with AIDN and CIDP may indicate an important difference between the pathogenesis of these two sets of the disease. RESUMO OBJETIVO: O papel das interleucinas, como IL-17 e IL-34, na patogênese da doença auto-imune foi estabelecido na literatura. No presente estudo, objetivamos identificar as concentrações de IL-17 (IL-17A, IL-17F) e IL-34 no líquido cefalorraquidiano (LCR) de pacientes com polineuropatia desmielinizante inflamatória crônica (CIDP) e neuropatia desmielinizante inflamatória aguda (AIDN). MÉTODOS: incluímos neste estudo 8 pacientes com CIDP (nenhum deles recebendo terapia imunomoduladora ou imunossupressora), 7 pacientes com síndrome de Guillain-Barre (GBS, AIDN) e 7 indivíduos controle. Os diagnósticos CIDP e AIDN foram feitos por avaliação clínica e investigações eletrofisiológicas de acordo com critérios internacionais. As amostras de LCR foram obtidas adequadamente e os níveis de IL-17A, IL-17F e IL-34 foram medidos através de kits ELISA. RESULTADOS: As concentrações de IL-17A, IL-17F e IL-34 foram maiores naqueles com CIDP e AIDN em comparação aos controles (p = 0,005, p = 0,01 ep = 0,001, respectivamente). Enquanto os níveis de IL-34 foram significativamente mais altos nos pacientes com AIDN do que nos pacientes com CIDP (p = 0,04), não houve diferenças significativas entre os grupos com AIDN e CIDP em relação aos níveis de IL-17A e IL-17F (p = 0,4 ep = 0,2, respectivamente) CONCLUSÃO: Nossos resultados indicam que os níveis de IL-17A, IL-17F e IL-34 podem ter um papel no CIDP e no AIDN. Além disso, a diferença nos níveis de IL-34 de pacientes com AIDN e CIDP pode indicar uma diferença importante entre a patogênese desses dois conjuntos de doenças.

Guillian-Barre Syndrome (GBS) typically presents as symmetrical ascending flaccid muscle weakness with areflexia, and with or without sensory symptoms. However, some patients may present atypically, and accordingly, different variants of GBS have been reported in the literature. Pharyngeal-cervical-brachial variant is one of the rare variants and is characterized by muscle weakness extending from the oropharyngeal and neck area to the proximal upper extremities. Many physicians and neurologists are unfamiliar about pharyngeal-cervical-brachial variant, which is often misdiagnosed as brainstem stroke, myasthenia gravis or botulism. Herein, we report a case of pharyngeal-cervical-brachial variant of GBS. To the best of our knowledge, this is the first reported case of pharyngeal-cervical-brachial variant of GBS in children from Nepal. A 14-year-old Asian male presented with weakness of bilateral upper limb, dysphagia, and nasal intonation of voice. A diagnosis of pharyngeal-cervical-brachial variant of GBS was made after excluding all other possible differentials and based on cerebrospinal fluid analysis and nerve conduction study. The patient improved following conservative management. Pharyngeal-cervical-brachial variant of GBS should always be considered in any patient presenting with symmetrical upper limb weakness and bulbar palsy. This is to ensure early diagnosis, treatment, and follow-up of the potential complications.

A retrospective study of 42 cases of acute flaccid paralysis (AFP) in children aged between 7 months and 15 years, registered at the Municipal Clinical Hospital №1 throughout a 7 year period (2007—2014), was performed to investigate the features of pediatric Guillian-Barre Syndrome (GBS). GBS has shown to be the most common cause of AFP in children, with prevalence of 74% of all 31 cases. Clinical manifestations, functional status, laboratory and electrodiagnostic data were evaluated in group of 31 children in order to highlight particular features of childhood GBS in Russia. The highest frequency of GBS was observed in children aged between 1 to 3 with the median 6 [3; 11] years. Boys with GBS outnumbered girls by a 2,1:1 ratio. No seasonal dependence has been observed, with children equally suffering from this disease without a seasonal pattern throughout the year. According to the electrophysiological and clinical data, 24 children were diagnosed with acute inflammatory demyelinating polyradiculoneuropathy (AIDP) (77%), 5 with acute motor axonal neuropathy (AMAN) (16%) and 2 with аcute motor-sensory axonal neuropathy (AMSAN) in a total of cases (7%). Several exclusive features of GBS in children for Russia were discovered. The most common initial symptom was limb pain, with the impartial sensory disturbance found only in 13% of the patients observed, 10% of which were paresthesias and the remaining 3% belonging to hypostesias. Children reached the nadir state rapidly, the median time from onset to nadir was 9.5 [6,25; 12,5] days. Cranial nerve dysfunction at nadir was observed in a greater percentage of patients (51%) compared to that of 23% cases at the onset, with the facial palsy increasing from 10 to 32% and the bulbar palsy from 12 to 19%. The patients were given intravenous immunoglobulin in various doses: from 0.2 to 1.75 mg/kg per course (0.5 [0.5; 0.8] g/kg) and/or plasmapheresis with a median volume of 93 [81; 100] ml/kg per course. The treatment has shown to be effective for the majority of patients, but three children was resistant to the intravenous immunoglobulin. An important feature of pediatric GBS is a nonthreatening prognosis at the point of discharge, with the length of hospitalization numbering in with a median of 28 [20,5; 38] days.