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Lewis-Sumner syndrome is the most common atypical form of chronic inflammatory demyelinating polyneuropathy (CIDP). In most patients, the disease is slowly progressive, which slows down the correct diagnosis. Timely diagnosis in some cases is also complicated by an abnormal primary lesion of the lower limb nerves in patients with Lewis-Sumner syndrome, for whom the typical clinical picture is upper flaccid distal paraparesis. The objective of the study is to determine the frequency of Lewis-Sumner syndrome (LSS), with the lower limb nerve onset; to characterize clinical and paraclinical characteristics of patients with the syndrome. Materials and Methods. The authors analyzed clinical data, results of stimulation electroneuromyography and ultrasound examination of peripheral nerves of 36 LSS patients. Results. The authors observed a high percentage (44 %) of LSS patients with lower limb nerve onset. However, changes in the neurophysiological and sonographic examination of the lower limb nerves, specific for CIDP, were not revealed. Changes typical of dysimmune neuropathy were verified only in the study of clinically intact long upper limb nerves. Conclusion. In asymmetric neuropathy of the lower limbs of idiopathic genesis, one should remember about LSS and, even despite the presence of symptoms only in the lower limbs, examine the peripheral nerves of the upper limbs. Key words: chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, atypical form. Синдром Льюиса – Самнера является наиболее частой атипичной формой хронической воспалительной демиелинизирующей полинейропатии (ХВДП). У большинства больных заболевание носит медленно прогрессирующий характер, что является одним из факторов увеличения сроков постановки верного диагноза. Своевременную диагностику в ряде случаев затрудняет и нехарактерное первичное поражение нервов ног у пациентов с синдромом Льюиса – Самнера, для которых типичной клинической картиной является верхний вялый дистальный парапарез. Цель исследования: определить частоту встречаемости синдрома Льюиса – Самнера (СЛС), дебютирующего с поражения нервов ног; охарактеризовать клинические и параклинические особенности больных с данным синдромом. Материалы и методы. Проанализированы клинические данные, результаты стимуляционной электронейромиографии и ультразвукового исследования периферических нервов 36 пациентов с СЛС. Результаты. Установлен высокий процент (44 %) встречаемости пациентов с СЛС с дебютом с поражения нервов ног. Однако специфические, характерные для ХВДП, изменения при нейрофизиологическом и сонографическом исследовании нервов ног не выявлены. Изменения, типичные для дизиммунной нейропатии, верифицированы только при исследовании клинически интактных длинных нервов рук. Выводы. В случаях асимметричной нейропатии нижних конечностей идиопатического генеза следует иметь настороженность в отношении СЛС и, даже несмотря на наличие симптоматики только в нижних конечностях, обследовать периферические нервы рук. Ключевые слова: хроническая воспалительная демиелинизирующая полинейропатия, синдром Льюиса – Самнера, атипичная форма.
Lewis-Sumner syndrome is the most common atypical form of chronic inflammatory demyelinating polyneuropathy (CIDP). In most patients, the disease is slowly progressive, which slows down the correct diagnosis. Timely diagnosis in some cases is also complicated by an abnormal primary lesion of the lower limb nerves in patients with Lewis-Sumner syndrome, for whom the typical clinical picture is upper flaccid distal paraparesis. The objective of the study is to determine the frequency of Lewis-Sumner syndrome (LSS), with the lower limb nerve onset; to characterize clinical and paraclinical characteristics of patients with the syndrome. Materials and Methods. The authors analyzed clinical data, results of stimulation electroneuromyography and ultrasound examination of peripheral nerves of 36 LSS patients. Results. The authors observed a high percentage (44 %) of LSS patients with lower limb nerve onset. However, changes in the neurophysiological and sonographic examination of the lower limb nerves, specific for CIDP, were not revealed. Changes typical of dysimmune neuropathy were verified only in the study of clinically intact long upper limb nerves. Conclusion. In asymmetric neuropathy of the lower limbs of idiopathic genesis, one should remember about LSS and, even despite the presence of symptoms only in the lower limbs, examine the peripheral nerves of the upper limbs. Key words: chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, atypical form. Синдром Льюиса – Самнера является наиболее частой атипичной формой хронической воспалительной демиелинизирующей полинейропатии (ХВДП). У большинства больных заболевание носит медленно прогрессирующий характер, что является одним из факторов увеличения сроков постановки верного диагноза. Своевременную диагностику в ряде случаев затрудняет и нехарактерное первичное поражение нервов ног у пациентов с синдромом Льюиса – Самнера, для которых типичной клинической картиной является верхний вялый дистальный парапарез. Цель исследования: определить частоту встречаемости синдрома Льюиса – Самнера (СЛС), дебютирующего с поражения нервов ног; охарактеризовать клинические и параклинические особенности больных с данным синдромом. Материалы и методы. Проанализированы клинические данные, результаты стимуляционной электронейромиографии и ультразвукового исследования периферических нервов 36 пациентов с СЛС. Результаты. Установлен высокий процент (44 %) встречаемости пациентов с СЛС с дебютом с поражения нервов ног. Однако специфические, характерные для ХВДП, изменения при нейрофизиологическом и сонографическом исследовании нервов ног не выявлены. Изменения, типичные для дизиммунной нейропатии, верифицированы только при исследовании клинически интактных длинных нервов рук. Выводы. В случаях асимметричной нейропатии нижних конечностей идиопатического генеза следует иметь настороженность в отношении СЛС и, даже несмотря на наличие симптоматики только в нижних конечностях, обследовать периферические нервы рук. Ключевые слова: хроническая воспалительная демиелинизирующая полинейропатия, синдром Льюиса – Самнера, атипичная форма.
Introduction. Despite the improving diagnostic criteria for the chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), its verification is still an issue. Objective: to study the rate and the causes of CIDP misdiagnosis. Materials and methods. We prospectively and retrospectively analyzed the clinical and paraclinical data of 223 patients admitted to the Research Center of Neurology from 2018 to 2022 with a CIDP referral. Results. We revised the CIDP diagnosis in 150/223 patients (67%; median age 55.5 [43; 63] years; 75 males and 75 females; 3-year follow-up history [1.75; 5.25].) Once the definitive diagnosis was clarified, we divided the patients into the following groups: polyneuropathy of other etiology (n = 94; 63%), other neuromuscular disorders (n = 39; 27%), CNS disorders (n = 10; 7%), no structural NS disease (n = 7; 5%). Patients did not meet the 2021 EAN/PNS diagnostic criteria at the history-taking stage in 65% of cases, at the neurological examination stage in 39% of cases, and at the electroneuromyography stage in 92% of cases. Conclusions. The rate of CIDP misdiagnosis in Russia is 67%, and most often this refers to patients with polyneuropathy of other etiologies. The main cause for the CIDP misdiagnosis was inaccurate electroneuromyography. We should bear in mind that CIDP is a rare disorder with an extensive differential diagnosis, so it should be verified according to the current 2021 EAN/PNS diagnostic criteria.
Introduction. Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by long-term progressive or relapsing course, neurological deficit, and disability of varied severity. The course of CIDP after specific therapy and, if necessary, long-term maintenance treatment are to be studied. Objective: To evaluate CIDP clinical and history characteristics over the long-term follow-up ( 5 years), to compare long-term CIDP course in a number of clinical variants and onset types, and to determine clinical predictors of unfavorable CIDP course. Materials and methods. The study included 45 patients diagnosed with CIDP based on EAN/PNS 2021 criteria lasting for 5 or more years. Retrospective collection and analysis of medical records and clinical history were performed. Internationally accepted scales were used to assess neurological deficit (NIS, MRCss), disability (INCAT), and disease activity status (CDAS). The criteria of unfavorable course were developed to evaluate factors affecting CIDP course. Results. Among the patients with CIDP history of 5 years, each third (34%) had no neurological deficit and remained in long-term clinical remission (CDAS 1). The vast majority (90%) responded to first-line therapy in early disease, while only 53% of patients required maintenance treatment in 5 or more years of the onset. With the developed criteria (poor response to glucocorticosteroids (GCS), need for maintenance therapy, and CDAS 3–5), unfavourable CIDP course was detected in 24 (53.3%) participants. Its probability increased in later onset (47 [30; 50] years), the chronic type of onset, and delayed specific therapy. The most significant predictors included low total NIS score at onset (60 points) and multifocal CIDP. Conclusions. The course of typical CIDP is relatively favorable if timely diagnosed, and pathogenetic treatment initiated. Patients with acute and subacute onset demonstrate the best long-term status. The predictors of unfavourable disease course include mild neurological deficit at onset (NIS total score 60 points) and multifocal CIDP.
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