Severe Generalized Weakness, Paraplegia following administration of Oxaliplatin in a patient with refractory T-cell non-Hodgkin lymphoma: A case report

Introduction Oxaliplatin is a third-generation platinum compound that used extensively for the treatment of various types of cancer especially gastrointestinal neoplasms. The main dose-limiting toxicities of oxaliplatin are hematological toxicity and peripheral sensory neuropathy. Case report A 42-year-old man with refractory peripheral T-cell lymphoma (PTCL) was admitted to receive GEMOX chemotherapy regimen (gemcitabine, oxaliplatin). Three days after receiving his third cycle of chemotherapy regimen, he was re-admitted to the emergency department with complaint of severe generalized weakness, and paraplegia in the lower extremities. According to clinical and para-clinical findings, chronic sensorimotor polyneuropathy with ongoing axonal loss was confirmed. Management & Outcome Intravenous dexamethasone 8 mg three times daily was started at the time of admission for the patient. Muscle weakness and sensory impairment improved dramatically within 10 days and the patient was able to walk with assistance. Discussion Several cases of neuropathy following oxaliplatin and only one case with gemcitabine-based chemotherapy regimen have been previously reported. However, motor symptoms are rare unless in the setting of acute neuropathy due to oxaliplatin. The most striking finding of our study was the incidence of a chronic sensorimotor axonaldemyelinating polyneuropathy in a patient who were subjected to oxaliplatin therapy. In conclusion, we report a case of severe generalized weakness and paraplegia following administration of Oxaliplatin.

Guillain-Barré Syndrome with Rapid Onset and Autonomic Dysfunction Following First Dose of Pfizer-BioNTech COVID-19 Vaccine: A Case Report

Guillain-Barre syndrome (GBS) is an immune-mediated, often post-infectious illness manifesting as an acute, characteristically monophasic, polyradiculoneuropathy. We present a case of GBS with autonomic involvement following an mRNA-based vaccine against SARS-COV2 (Pfizer/BioNTech mRNA-BNT162b2). A 58-year-old woman presented with fatigue, distal extremity paresthesias, and severe back pain within 3 days after receiving her first vaccine dose. She developed worsening back pain and paresthesias in distal extremities which prompted her initial presentation to the hospital. By the third week post-vaccine, she developed increasing gait unsteadiness, progression of paresthesias, and new autonomic symptoms including presyncopal episodes and constipation. Neurological exam showed bilateral distal predominant lower extremity weakness, decreased sensation in a length-dependent pattern, and areflexia. EMG/NCS showed a diffuse sensorimotor polyneuropathy with mixed demyelinating and axonal features consistent with GBS. She was treated with 2 g/kg of IVIG over 3 days and also received prednisone 60 mg daily for 3 days for severe back pain, with improvement of symptoms. This possible association with mRNA-based vaccination expands the potential triggers for an autoimmune-based attack on the peripheral nervous system.

Management of a patient with myofascial pain syndrome (piriformis syndrome): clinical follow-up

The article considers a clinical case of treatment of one of the variants of myofascial pain syndrome – piriformis syndrome without signs of sciatic nerve neuropathy. The peculiarity of the case is the comorbidity of the opioid syndrome with diabetic sensorimotor polyneuropathy and osteoporosis, which required the appointment of complex therapy. The diagnosis was confirmed by additional research methods: spondylography, MRI of the lumbosacral spine, ultrasound of the piriformis muscle, electroneuromyography. A patient management tactic was chosen based on federal clinical guidelines for the treatment of patients with nonspecific back pain. Treatment included non-medicinal and medicinal methods. In order to relieve pain, dexketoprofen was prescribed 2 ml intramuscularly per 2 ml of 0.5% lidocaine solution – 1 time а day No. 2 – under navigation by ultrasound. Subsequently, the transfer was made to oral administration of 25 mg 3 times а day for 3 days. A step-by-step scheme of prescribing dexketoprofen: its introduction into the piriformis muscle with subsequent transfer to oral administration allowed to significantly reduce the severity of pain after 5 days of treatment. The complex effect on the spasmodic piriformis muscle with the help of a tableted muscle relaxant in combination with postisometric relaxation made it possible to quickly stop the pain syndrome and prevent its chronization. The administration of the preparation of thioctic acid pursued two goals: to improve the metabolism of the spasmodic muscle and restore the energy metabolism of peripheral nerves. As a result of the use of complex, pathogenetically based therapy, a positive effect was achieved.

CHARACTERISTICS OF PERIPHERAL NERVOUS SYSTEM IN PATIENTS WITH PSORIASIS

Psoriasis is a chronic non-infectious inflammatory dermatosis characterized by excessive proliferation of epithelial cells, impaired differentiation, often involving the musculoskeletal system. Materials and Methods. The authors examined 60 patients with psoriasis, 39 men and 21 women, aged from 21 to 69 (the average subject age was 46.3±13.94). All patients underwent a standard dermatological and neurological examination. Motor function was assessed according to knee jerk, elbow jerk and Achilles jerk. Pain sensitivity was established after pricking the thumb dorsum with a special blunt-end needle. Nonparametric methods were used for statistical analysis. The Mann-Whitney U-test was used to check the differences between independent groups of patients. The distribution of characteristic was assessed using a probability calculator. Differences were statistically significant at 95 % probability (p<0.05). Results. Mild psoriasis was found in 8 patients (13.3 %), moderate-to-severe psoriasis – in 14 patients (23 %) (PASI=11–30), severe psoriasis – in 38 patients (63.7 %) (PASI>30). Type I psoriasis was detected in 32 patients (53.2 %), type II – in 28 patients (46.8 %). Dysfunctions of the peripheral nervous system were found in 30 patients (50 %). In 12 subjects (20 %) symmetrical sensory and motor disturbances were observed in the distal parts of all four limbs. In 9 patients (15 %) symmetrical decrease in knee and Achilles jerks was observed. Symmetrical decrease in tactile and temperature sensitivity in the distal parts of the lower extremities was noted in 9 patients with psoriasis (15 %). Conclusion. Distal symmetric sensorimotor polyneuropathy is common in patients with psoriasis. Severe psoriasis is accompanied by distal symmetric sensorimotor polyneuropathy. Key words: psoriasis, comorbidity, demyelination, polyneuropathy. Псориаз – это хронический неинфекционный воспалительный дерматоз, характеризующийся избыточной пролиферацией эпителиоцитов, нарушением их дифференциации, нередко вовлекающий в процесс опорно-двигательный аппарат. Материалы и методы. Обследовано 60 больных псориазом, из них 39 мужчин и 21 женщина в возрасте от 21 до 69 лет (средний возраст обследуемых 46,3±13,94 года). Всем больным проводился стандартный дерматологический и неврологический осмотр. Двигательная функция исследовалась с помощью оценки коленного, локтевого и ахиллова рефлексов. Болевая чувствительность устанавливалась после укола тыльной поверхности большого пальца специальной иглой с притупленным концом. Для статистического анализа использовались непараметрические методы. Для проверки отличий между независимыми группами больных применялся Mann – Whitney U-test. Распределение признака оценивалось с помощью вероятностного калькулятора. Статистические значимыми считались отличия при уровне вероятности более 95 % (p<0,05). Результаты. Легкая степень псориаза установлена у 8 (13,3 %) больных, умеренно тяжелый псориаз – у 14 (23 %) пациентов (индекс PASI 11–30 баллов), тяжелая форма псориаза – у 38 (63,7 %) больных (индекс PASI более 30 баллов). Псориаз I типа определялся у 32 (53,2 %) больных, II типа – у 28 (46,8 %). У 30 (50 %) больных обнаружены нарушения функции периферической нервной системы. У 12 (20 %) чел. наблюдались симметричные сенсорные и двигательные нарушения в дистальных отделах всех четырех конечностей. У 9 (15 %) чел. отмечалось симметричное снижение коленного и ахиллова рефлексов. Девять (15 %) больных псориазом имели симметричное снижение тактильной и температурной чувствительности в дистальных отделах нижних конечностей. Выводы. У больных псориазом часто встречается дистальная симметричная сенсорно-моторная полинейропатия. Тяжелое течение псориаза сопровождается дистальной симметричной сенсорно-моторной полинейропатией. Ключевые слова: псориаз, коморбидность, демиелинизация, полинейропатия.

Skin Advanced Glycation End Products among Subjects with Type 2 Diabetes Mellitus with or without Distal Sensorimotor Polyneuropathy

Aim of the Study. To examine the correlation between skin AGEs and parameters of distal sensorimotor polyneuropathy (DSPN) in type 2 diabetes mellitus (T2DM). Materials and Methods. We included 132 subjects (88 men) with a mean age of 64.57 years and median T2DM duration of 14.5 years. Skin AGEs were measured with AGE reader mu connect (Diagnoptics) on the dominant arm. The device enables single and automated triplicate measurements: both of these were performed. DSPN was diagnosed through the neuropathy disability score (NDS). Small nerve fibre function was assessed by temperature and pinprick sensation on the foot. Bilateral measurement of the vibration perception threshold (VPT) on the hallux was carried out by using a neurothesiometer (Horwell Scientific Laboratory Supplies). Results. Single and triplicate AGE measurements were positively correlated with each other (Pearson’s correlation coefficient r = 0.991 , 95 % CI = 0.987 -0.994, p < 0.001 ). AGEs were higher among subjects with vs. those without DSPN ( p < 0.001 ). Furthermore, they were higher among subjects with reduced vs. normal temperature sensation ( p < 0.001 ), among subjects with reduced vs. normal pinprick sensation ( p = 0.002 ), among those with abnormal vs. normal monofilament examination ( p < 0.001 ), and among those with abnormal vs. normal VPT ( p < 0.001 ). AGEs were correlated with NDS, VPT, and monofilament score. Conclusions. In T2DM, skin AGEs are increased in the presence of DSPN. This holds true both for large and for small nerve function impairment. Moreover, AGEs are correlated with DSPN severity.

Peripheral polyneuropathy from electrodiagnostic tests: a 10-year etiology and neurophysiology overview

ABSTRACT Background: Polyneuropathies are characterized by a symmetrical impairment of the peripheral nervous system, resulting in sensory, motor and/or autonomic deficits. Due to the heterogeneity of causes, an etiological diagnosis for polyneuropathy is challenging. Objective: The aim of this study was to determine the main causes of polyneuropathy confirmed by electrodiagnostic (EDX) tests in a tertiary service and its neurophysiological aspects. Methods: This observational cross-sectional study from a neuromuscular disorders center included individuals whose electrodiagnostic tests performed between 2008 and 2017 confirmed a diagnosis of polyneuropathy. Through analysis of medical records, polyneuropathies were classified according to etiology and neurophysiological aspect. Results: Of the 380 included patients, 59.5% were male, with a median age of 43 years. The main etiologies were: inflammatory (23.7%), hereditary (18.9%), idiopathic (13.7%), multifactorial (11.1%), and diabetes (10.8%). The main electrophysiological patterns were axonal sensorimotor polyneuropathy (36.1%) and “demyelinating and axonal” sensorimotor polyneuropathy (27.9%). Axonal patterns showed greater etiological heterogeneity, with a predominance of idiopathic and multifactorial polyneuropathy, while demyelinating and “demyelinating and axonal” polyneuropathies had a significantly fewer etiologies, with a predominance of hereditary and inflammatory polyneuropathies. Conclusion: The main causes of polyneuropathy confirmed by EDX test in this study were those that presented a severe, atypical and/or rapidly progressing pattern. Other causes were hereditary and those that defy clinical reasoning, such as multiple risk factors; some polyneuropathies did not have a specific etiology. EDX tests are useful for etiological diagnosis of rare polyneuropathies, because neurophysiological patterns are correlated with specific etiologies.

Sensory-Motor Polyneuropathy and Digital Ischemia: A Rare Presentation of Granulomatosis with Polyangiitis

Granulomatosis with polyangiitis (GPA) typically presents with upper or lower respiratory tract symptoms and/or with renal involvement. Although it can affect the peripheral nervous system frequently, with mononeuritis multiplex being the most common pattern, the occurrence of peripheral sensory-motor polyneuropathy as a presenting manifestation is distinctly rare. Prevalence of digital gangrene is also extremely rare in GPA. We describe a 46-year-old woman presenting with severe peripheral sensorimotor polyneuropathy affecting bilateral lower limbs preceded by a purpuric skin rash and multiple painful ulcers confined to the lower limbs. She had evidence of digital ischemia affecting multiple toes and dry gangrene of the left 4th toe. Diagnosis of GPA was made based on skin biopsy, positive ANCA serology, and clinical criteria. She made a good recovery following aggressive immunosuppressive treatment with methylprednisolone and cyclophosphamide and was maintained on prednisolone and azathioprine. This case highlights the importance of suspecting GPA in a patient presenting with sensorimotor polyneuropathy and/or digital ischemia even in the absence of more classic presenting features and underlies the necessity of accurate differential diagnosis in evaluating a case of peripheral neuropathy.

Stiff-person syndrome with sensorimotor polyneuropathy – case report

Stiff-person syndrome (SPS) is a rare disorder with an estimated prevalence in the general population of 1–2 cases/1,000,000. It is 2–3 times more common in females, with symptom onset at the age of 20–50 years in most cases. Although stiff-person syndrome is associated with antibodies against glutamic acid decarboxylase and amphiphysin, their presence is not necessary for the diagnosis. The treatment should be multidirectional and include immunomodulation, symptomatic treatment as well as monitoring and treatment of overlapping autoimmune, and surgery. We present a case report of a patient diagnosed with stiff-person syndrome overlapping with axonal and demyelinating sensorimotor polyneuropathy. The diagnostic workup indicated diabetes-related polyneuropathy. About 30% of patients diagnosed with stiff-person syndrome also have diabetes. Polyneuropathy alone is rarely reported to overlap with the disorder. In our opinion, polyneuropathy may have a beneficial effect on the clinical presentation of stiff-person syndrome.

Involvement of Cutaneous Sensory Corpuscles in Non-Painful and Painful Diabetic Neuropathy

Distal diabetic sensorimotor polyneuropathy (DDSP) is the most prevalent form of diabetic neuropathy, and some of the patients develop gradual pain. Specialized sensory structures present in the skin encode different modalities of somatosensitivity such as temperature, touch, and pain. The cutaneous sensory structures responsible for the qualities of mechanosensitivity (fine touch, vibration) are collectively known as cutaneous mechanoreceptors (Meissner corpuscles, Pacinian corpuscles, and Merkel cell–axonal complexes), which results are altered during diabetes. Here, we used immunohistochemistry to analyze the density, localization within the dermis, arrangement of corpuscular components (axons and Schwann-like cells), and expression of putative mechanoproteins (PIEZO2, ASIC2, and TRPV4) in cutaneous mechanoreceptors of subjects suffering clinically diagnosed non-painful and painful distal diabetic sensorimotor polyneuropathy. The number of Meissner corpuscles, Pacinian corpuscles, and Merkel cells was found to be severely decreased in the non-painful presentation of the disease, and almost disappeared in the painful presentation. Furthermore, there was a marked reduction in the expression of axonal and Schwann-like cell markers (with are characteristics of corpuscular denervation) as well as of all investigated mechanoproteins in the non-painful distal diabetic sensorimotor polyneuropathy, and these were absent in the painful form. Taken together, these alterations might explain, at least partly, the impairment of mechanosensitivity system associated with distal diabetic sensorimotor polyneuropathy. Furthermore, our results support that an increasing severity of DDSP may increase the risk of developing painful neuropathic symptoms. However, why the absence of cutaneous mechanoreceptors is associated with pain remains to be elucidated.