A case report of Tangier disease presents with acute sensorimotor polyneuropathy and its treatment approach.

Polyneuropathy is a frequently encountered clinical presentation where peripheral nerves are affected due to the same cause and physiopathological processes. We report a case of acute sensorimotor polyneuropathy in a patient with Tangier disease (TD) who was treated with miglustat which is a glycosphingolipid synthesis inhibitor. TD is a very rare genetic disorder caused by mutations in the ATP-binding cassette transporter A1 (ABCA1) gene which encodes the cholesterol efflux regulatory protein. It leads to accumulation of cholesterol esters within various tissues and affects lipid metabolism by deficiency of high-density lipoprotein (HDL) in the blood. Due to the accumulation of cholesterol esters in Schwann cells, it could provoke polyneuropathy in TD. Our case presented to our clinic with quadriparesis and after treated with miglustat therapy his weakness regressed.

Determining the utility of minimum F-wave latency alterations in the electrodiagnosis of ulnar neuropathy at the elbow.

BACKGROUND: Ulnar neuropathy at the elbow (UNE) is the second most common entrapment neuropathy. There is little information about the application of F-wave studies for evaluation of UNE. OBJECTIVE: The aim of this study was to evaluate the diagnostic value of minimum F-wave (F-min) latency alterations by comparing this with nerve conduction analyses in UNE-suspected patients. METHODS: Ninety-four UNE-suspected patients were admitted to this study. Sensory and motor nerve conduction and F-wave analyses on the median and ulnar nerves were performed on both upper extremities. RESULTS: A total of 188 upper extremities of 94 patients were examined. Their mean age was 41.4±12.9 years, and 69 patients were female (73.4%). The mean ulnar-nerve across-elbow motor conduction velocity (MCV) in the affected arms was significantly slower than the velocity in healthy arms. The mean ulnar-nerve F-min latencies were significantly longer in the affected arms. Fifty-one patients were electrophysiologically diagnosed as presenting UNE (54.2%). Significantly slower mean ulnar-nerve across-elbow MCV, longer mean ulnar-nerve F-min latency and longer distal onset latency were detected in UNE-positive arms. Lastly, patients who were symptomatic but had normal nerve conduction were evaluated separately. Only the mean ulnar F-min latency was significantly longer in this group, compared with the healthy arms. CONCLUSION: Our study confirmed the utility of F-min latency measurements in the electrodiagnosis of UNE. F-wave latency differences can help in making an early diagnosis to provide better treatment options.

The prognostic value of demyelinating electrophysiologic findings and cerebrospinal fluid protein levels in acute inflammatory demyelinating polyneuropathy.

BACKGROUND: Guillain-Barre syndrome is an acute immune-mediated polyneuropathy characterized by rapidly evolving symptoms and disability. Cerebrospinal fluid analysis and electrophysiological studies are crucial in the diagnosis of this syndrome. OBJECTIVE: To evaluate the prognostic value of the type and number of demyelinating findings and cerebrospinal fluid protein levels in patients with acute inflammatory demyelinating polyneuropathy. METHODS: We retrospectively analyzed electrophysiological data and cerebrospinal fluid of 67 consecutive patients with acute inflammatory demyelinating polyneuropathy from Istanbul, Turkey (2011-2019) studied ≤ 24 hours post-onset. RESULTS: The patients who met a higher number of demyelinating criteria had increased disability scores in the first day and first month, and higher cerebrospinal fluid protein levels were correlated with worse prognosis both on the first day and the first month. However, the disability scores did not correlate with any single specific criterion, and no significant correlation was found between the number of satisfied criteria and cerebrospinal fluid protein levels. CONCLUSIONS: The number of demyelinating criteria that are met and high cerebrospinal fluid protein levels at the disease onset may be valuable prognostic markers. More systematic studies conducted with serial nerve conduction studies are required to highlight the roles of the suggested criteria in clinical practice.

The prognostic value of demyelinating electrophysiologic findings and cerebrospinal fluid protein levels in acute inflammatory demyelinating polyneuropathy / O valor prognóstico dos achados eletrofisiológicos desmielinizantes e os níveis de proteína do líquido cefalorraquidiano na polineuropatia desmielinizante inflamatória aguda

ABSTRACT Background: Guillain-Barre syndrome is an acute immune-mediated polyneuropathy characterized by rapidly evolving symptoms and disability. Cerebrospinal fluid analysis and electrophysiological studies are crucial in the diagnosis of this syndrome. Objective: To evaluate the prognostic value of the type and number of demyelinating findings and cerebrospinal fluid protein levels in patients with acute inflammatory demyelinating polyneuropathy. Methods: We retrospectively analyzed electrophysiological data and cerebrospinal fluid of 67 consecutive patients with acute inflammatory demyelinating polyneuropathy from Istanbul, Turkey (2011-2019) studied ≤ 24 hours post-onset. Results: The patients who met a higher number of demyelinating criteria had increased disability scores in the first day and first month, and higher cerebrospinal fluid protein levels were correlated with worse prognosis both on the first day and the first month. However, the disability scores did not correlate with any single specific criterion, and no significant correlation was found between the number of satisfied criteria and cerebrospinal fluid protein levels. Conclusions: The number of demyelinating criteria that are met and high cerebrospinal fluid protein levels at the disease onset may be valuable prognostic markers. More systematic studies conducted with serial nerve conduction studies are required to highlight the roles of the suggested criteria in clinical practice.

RESUMO Introdução: A síndrome de Guillain-Barré é uma polineuropatia imunomediada aguda caracterizada por sintomas e incapacidade em rápida evolução. A análise do líquido cefalorraquidiano e os estudos eletrofisiológicos são cruciais no diagnóstico dessa síndrome. Objetivo: Avaliar o valor prognóstico do tipo e número de achados desmielinizantes e dos níveis de proteínas do líquido cefalorraquidiano em pacientes com polineuropatia desmielinizante inflamatória aguda. Métodos: Analisamos retrospectivamente dados eletrofisiológicos e líquido cefalorraquidiano de 67 pacientes consecutivos com polineuropatia desmielinizante inflamatória aguda de Istambul, Turquia (2011-2019), estudados ≤24 horas após o início. Resultados: Os pacientes que atenderam a um número maior de critérios desmielinizantes apresentaram escores de incapacidade aumentados no primeiro dia e no primeiro mês, e níveis mais altos de proteína do líquido cefalorraquidiano foram correlacionados com pior prognóstico no primeiro dia e no primeiro mês. No entanto, os escores de incapacidade não se correlacionaram com nenhum critério específico e não foi encontrada correlação significativa entre o número de critérios satisfeitos e os níveis de proteína do líquido cefalorraquidiano. Conclusões: O número de critérios desmielinizantes atendidos e altos níveis de proteína no líquido cefalorraquidiano no início da doença podem ser marcadores prognósticos valiosos. Estudos mais sistemáticos conduzidos com estudos de condução nervosa em série são necessários para destacar os papéis dos critérios sugeridos na prática clínica.

Prolonged hypotension after carotid artery stenting: incidence, predictors and consequences.

BACKGROUND: Hemodynamic changes frequently occur after carotid artery stenting (CAS), and in some patients these changes, particularly hypotension, may be prolonged. There are discrepant results for predicting patients at high risk for these prolonged hemodynamic changes and identifying the effect on clinical outcome. In this study, we aimed to determine the frequency, predictors and consequences associated with prolonged hypotension (PH) after CAS in our center. METHODS: We retrospectively analyzed the demographics, risk factors, nature of carotid disease, degree of stenosis of both internal carotid arteries, stent diameter and site of dilatation during stenting in 137 CAS procedures. After CAS, duration of hospital stay, complications during hospital stay and major vascular events or death in a 3-month period were evaluated. PH was defined as a systolic blood pressure <90 mmHg lasting more than 1 h despite adequate treatment after CAS. RESULTS: PH occured in 23 (16.8%) patients. The presence of contralateral stenosis ≥70% and absence of diabetes mellitus were significantly associated with PH. Duration of hospital stay was significantly longer in patients with PH. No patients with PH had a periprocedural complication or major vascular events in the follow-up period. CONCLUSION: PH was more prevalent in patients with contralateral high-degree carotid stenosis and patients without diabetes mellitus after CAS. PH did not cause any post-procedural complications or major vascular events at follow-up, but it resulted longer hospital stays. Further studies are needed to better define the pathophysiologic mechanisms underlying these hemodynamic alterations.

Risk Factors, Etiological Classification, Topographical Location, and Outcome in Medullary Infarctions.

An understanding of the etiological mechanisms is important for therapeutic decisions and prognostic evaluation of patients with ischemic stroke. The object of this study was to evaluate the risk factors, etiological subtypes, and topography of lesion in patients with medullary infarctions (MIs). Besides, we also investigated early neurological deterioration, new vascular events, and functional outcome of all patients at 3-month follow-up. We analyzed our database consisting of patients who were diagnosed with acute MI and who were admitted within 24 hours of onset. Etiological classification of stroke was made on the basis of the Trial of Org 1972 in Acute Stroke Treatment criteria. All of the infarctions were grouped into anteromedial, anterolateral, lateral, and posterior arterial territories and also categorized into those involving the upper, middle, or lower medulla oblongata. Early neurological deterioration, major vascular events within the first 3 months of follow-up and modified Rankin Score at 3 months were reviewed. A total of 65 patients with medullary infarctions were reviewed. Involved arterial territories differed according to the etiological classification. Large artery atherosclerosis was the most common etiological subtype; however, small vessel disease was the most common subtype in medial MIs. The lesions involving the anteromedial territory were common in the upper medullary region, whereas the lesions involving the posterior and lateral territories were common in the lower medulla oblangata. Recurrent stroke was seen in the posterior and lateral territories; however, early progression and poor functional outcome were mostly seen in lesions involving the anteromedial territories.

The Relationship of Serum S100B Levels with Infarction Size and Clinical Outcome in Acute Ischemic Stroke Patients.

INTRODUCTION: S100B protein, which helps nerve development and differentiation, is produced by astrocytes and can be detected in peripheral circulation after brain damage. In this study, we aimed to investigate the relationship between the serum S100B protein level and the infarction volume and clinical outcome and also the early prognostic role of serum S100B protein in patients with ischemic stroke. METHOD: Fifty patients admitted in the first 24-hour period of acute ischemic stroke were evaluated prospectively, and the findings were compared to those of the controls (n=26). S100B levels of the patients and neurological findings on days 1, 3, and 5 and their functional outcomes on the discharge day and at the first month were recorded by the same examiner. RESULTS: S100B levels were not affected by sex, age, or concomitant systemic diseases. The maximum levels of S100B were recorded on the 3rd day, and there was a correlation between infarct size and S100B levels. No correlation between the severity of stroke and S100B level was found. There was a poor correlation between the functional outcomes of the patients at the 1st month and S100B levels and on the 3rd day. CONCLUSION: The detection of high S100B levels in peripheral circulation after acute ischemic stroke and the correlations of S100B levels with infarct size (good) and disability (poor) imply that S100B protein may be used as a peripheral marker in acute ischemic stroke patients.