LEWIS-SUMNER SYNDROME: ANALYSIS OF ATYPICAL ONSET WITH PRIMARY ASYMMETRIC LESIONS OF LOWER LIMB NERVES

2021 ◽  
pp. 79-88
Author(s):  
A.S. Rizvanova ◽  
E.A. Mel'nik ◽  
D.A. Grishina ◽  
N.A. Suponeva
Keyword(s):  
Lower Limb ◽  
Peripheral Nerves ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Correct Diagnosis ◽  
Demyelinating Polyneuropathy ◽  
Lower Limbs ◽  
Sonographic Examination ◽  
Atypical Form ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Syndrome Analysis

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.

scholarly journals The Challenge of Distinguishing POEMS from Chronic Inflammatory Demyelinating Polyneuropathy—Importance of Early Recognition and Diagnosis of POEMS

US Neurology ◽  
2018 ◽  
Vol 14 (2) ◽  
pp. 94
Author(s):  
Frances Chow ◽  
Leila Darki ◽  
Said R Beydoun ◽  
◽  
◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Early Recognition ◽  
Correct Diagnosis ◽  
Demyelinating Polyneuropathy ◽  
Laboratory Findings ◽  
High Clinical Suspicion ◽  
Underlying Malignancy ◽  
M Proteins ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Proper Diagnosis

POEMS is a rare syndrome characterized by the unique constellation of polyneuropathy, organomegaly, endocrinopathy, M-proteins, and skin changes. Correct diagnosis is often delayed in early stages of the syndrome when patients exhibit only isolated polyneuropathy due to the clinical and electrodiagnostic similarities with chronic inflammatory demyelinating polyneuropathy. We describe a case in which early suspicion for POEMS uncovered underlying malignancy, and we review the clinical, electrophysiological, pathological, and laboratory findings characteristic of POEMS. The importance of high clinical suspicion is key in the proper diagnosis and management of this complex syndrome.

scholarly journals Chronic inflammatory demyelinating polyneuropathy: a rare cause of falls

2019 ◽  
Vol 12 (12) ◽  
pp. e231676
Author(s):  
Toni C Saad ◽  
William Owen Pickrell ◽  
Gareth Payne ◽  
Khalid Hamandi
Keyword(s):  
Lower Limb ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Definitive Treatment ◽  
Good Recovery ◽  
Limb Weakness ◽  
Lower Limb Weakness ◽  
Symmetrical Pattern ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Compressive Myelopathy

This case of chronic inflammatory demyelinating polyneuropathy (CIDP) shows that a patient’s condition can evolve from the point of admission, gradually manifesting its underlying cause. Our patient’s initial presentation of backpain and lower limb weakness prompted investigations which ruled out compressive myelopathy and neuropathy. As upper limb weakness developed later, along with a more proximal and symmetrical pattern of lower limb weakness, the clinical picture suggested polyneuropathy. The diagnosis of CIDP became apparent only after numerous negative tests and nerve conduction studies which identified demyelination. Diagnosing CIDP enabled the commencement of definitive treatment which led to a good recovery.

scholarly journals Nerve Ultrasound Comparison Between Transthyretin Familial Amyloid Polyneuropathy and Chronic Inflammatory Demyelinating Polyneuropathy

2021 ◽  
Vol 12 ◽  
Author(s):  
Kang Du ◽  
Ke Xu ◽  
Si Cheng ◽  
He Lv ◽  
Wei Zhang ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Compound Muscle Action Potential ◽  
Familial Amyloid Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Lower Limbs ◽  
Nerve Ultrasound ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Amyloid Polyneuropathy ◽  
The Mean ◽  
Transthyretin Familial Amyloid Polyneuropathy

Backgrounds: Transthyretin familial amyloid polyneuropathy (TTR-FAP) is frequently misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP) because of similar phenotypes in the two diseases. This study was intended to identify the role of nerve ultrasonography in evaluating TTR-FAP and CIDP.Methods: Eighteen patients with TTR-FAP, 13 patients with CIDP, and 14 healthy controls (HC) were enrolled in this study. Consecutive ultrasonography scanning was performed in six pairs of nerves of bilateral limbs with 30 sites. The cross-sectional areas (CSAs) and CSA variability data of different groups were calculated and compared.Results: Both TTR-FAP and CIDP showed larger CSAs at most sites of both upper and lower limbs than in HC groups. CIDP patients had larger CSAs than TTR-FAP patients at 8/15 of these sites, especially at U1-3, Sci2 sites (p < 0.01). However, the CSAs at above sites were not a credible index to differentiate TTR-FAP from CIDP with a low area under the curve (<0.8). The CSA variability of median nerves was significantly higher in CIDP than in TTR-FAP and HC groups, with high sensitivity (0.692) and specificity (0.833) to differentiate CIDP from TTR-FAP. The CSA variability of ulnar nerves was not significantly different between the three groups. For the TTR-FAP group, mean CSAs at each site were not correlated with different Coutinho stages, modified polyneuropathy disability, course of sensory motor peripheral neuropathy, Neuropathy Impairment Score, or Norfolk Quality of life-diabetic neuropathy score. The mean compound muscle action potential of ulnar nerves was negatively correlated with the mean CSAs of ulnar nerves.Interpretation: TTR-FAP patients had milder nerve enlargement with less variability in CSAs of median nerves than those with CIDP, suggesting that nerve ultrasound can be a potential useful auxiliary tool to help differentiate the two neuropathies.

scholarly journals Clinical case: criteria for differential diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy

2015 ◽  
pp. 48-52
Author(s):  
N.O. Kravchuk
Keyword(s):  
Peripheral Nerves ◽  
Progressive Disease ◽  
Age Of Onset ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Age Group ◽  
Nerve Roots ◽  
Acute Inflammatory Demyelinating Polyneuropathy ◽  
Sensory Disorders ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disease that is characterized by inflammation of nerve roots and peripheral nerves, nerve destruction of the myelin sheath, the appearance of slowly-progressive symmetric symptoms, motor and sensory disorders. CIDPsometimes considered a chronic form of acute inflammatory demyelinating polyneuropathy (АIDP) - Guillain Barré syndrome (GBS). In contrast to GBS, most patients do not mark HDPNP previous viral or infectious disease. CIDP is subacute-progressive disease that clinically takes 3-4 weeks, then usually comes plateau phase, which changes the phase of gradual regression of symptoms. CIDP can affect any age group. CIDP registered twice as often in men. The average age of onset of the disease is 50 years. CIDP prevalence is estimated at about 5-7 cases per 100 thousand people.    

scholarly journals Chronic Inflammatory Demyelinating Polyneuropathy after ChAdOx1 nCoV-19 Vaccination

Vaccines ◽  
2021 ◽  
Vol 9 (12) ◽  
pp. 1502
Author(s):  
Caterina Francesca Bagella ◽  
Davide G. Corda ◽  
Pietro Zara ◽  
Antonio Emanuele Elia ◽  
Elisa Ruiu ◽  
...  
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Term Treatment ◽  
Demyelinating Polyneuropathy ◽  
Lower Limbs ◽  
Guillain Barre Syndrome ◽  
Close Monitoring ◽  
Long Term Treatment ◽  
Guillain Barré Syndrome ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Guillain Barré

Recently several patients, who developed Guillain–Barré syndrome characterized by prominent bifacial weakness after ChAdOx1 nCoV-19 vaccination, were described from different centers. We recently observed a patient who developed a similar syndrome, later in the follow up he showed worsening of the neuropathy two months after the initial presentation. Repeat EMG showed reduced nerve sensory and motor conduction velocities of both upper and lower limbs, and a diagnosis of chronic inflammatory demyelinating polyneuropathy (typical CIDP) was made according to established criteria. Our report expands on the possible outcomes in patients who develop Guillain–Barrè syndrome after COVID-19 vaccinations and suggest that close monitoring after the acute phase is needed in these patients to exclude a chronic evolution of the disease, which has important implications for long-term treatment.

042 Chronic inflammatory demyelinating polyneuropathy presenting as focal amyotrophy

2018 ◽  
Vol 89 (6) ◽  
pp. A17.3-A18
Author(s):  
Shadi El-Wahsh ◽  
Dennis Cordato ◽  
Con Yiannikas
Keyword(s):  
Lower Limb ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Oral Prednisone ◽  
Sural Nerve Biopsy ◽  
Demyelinating Polyneuropathy ◽  
Pet Scan ◽  
Whole Body ◽  
High Dose ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Chronic Denervation

IntroductionWe aimed to report a rare case of chronic inflammatory demyelinating polyneuropathy (CIDP) presenting as focal lower limb amyotrophy.CaseA 65 year old man presented with a 12 month history of painless, left anterior thigh wasting. Examination revealed marked left quadriceps and mild left hamstring and tibial wasting (Image 1). He had MRC grade 4/5 left hip flexion and 3/5 knee extension weakness, with normal power elsewhere. His left knee jerk was absent. Remaining reflexes were retained. There was reduced left L5 pinprick and light touch sensation. His neurological exam was otherwise normal. Blood tests, including creatine kinase, blood glucose, and autoimmune panel, were normal. Anti-neuronal, anti-ganglioside and anti-MAG antibodies were negative. Trace IgM lambda para-proteinaemia was detected. Cerebrospinal fluid was unremarkable [protein 0.37 g/L (n<0.45), glucose 4.1 mmol/L (2.5–4.5), leukocytes 0×106/L]. Nerve conduction studies showed normal lower limb motor velocities with reduced left peroneal motor amplitudes and mildly prolonged sural sensory latencies bilaterally. Electromyography showed chronic denervation of bilateral L3-S1 innervated muscles without fibrillation potentials. Posterior tibial somatosensory evoked potential studies demonstrated absent lumbar responses and prolonged cortical latencies. Muscle biopsy confirmed chronic denervation without evidence of myopathy or vasculitis. The patient declined sural nerve biopsy. MRI revealed bilateral multifocal lumbosacral plexopathy with increased T2 signal and thickening of multiple nerves, including femoral, lateral femoral cutaneous and L5 nerve roots (Image 2). CT chest, abdomen and pelvis, and whole body PET scan were normal. High-dose oral prednisone and induction intravenous immunoglobulin (IVIG) therapy followed by 4-weekly maintenance IVIG and low-dose prednisolone were commenced for a presumed diagnosis of CIDP.ConclusionIn this patient, the presence of multifocal nerve thickening on MRI was consistent with an inflammatory neuropathy. The normal PET scan and CSF studies made other diagnoses, such as neuro-lymphoma, less likely. CIDP should be considered in atypical presentations such as focal amyotrophy.

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