Serum SARM1 Levels and Diabetic Peripheral Neuropathy in Type 2 Diabetes: Correlation with Clinical Neuropathy Scales and Nerve Conduction Studies and Impact of COVID-19 vaccination.

Patients with peripheral neuropathy with type 2 diabetes mellitus (T2DM) are more likely to have functional impairments. Recently, the gene for serum sterile alpha and toll/interleukin receptor motif-containing protein 1 (SARM1), which may contribute to the pathogenesis of Wallerian degeneration, was discovered in mice models of peripheral neuropathy. We set out to assess serum SARM1's activity as a potential biomarker for the early identification of diabetic peripheral neuropathy in T2DM patients while also examining the impact of the COVID-19 vaccine on SARM1 levels. We assessed the cross-sectional relationships between the SARM1 biomarker, clinical neuropathy scales, and nerve conduction parameters in 80 participants aged between 30 years and 60 years. The analysis was carried out after the patients were split into two groups since we discovered a significant increase in SARM1 levels following the second dose of the COVID-19 vaccination, where group A received one dose of the COVID-19 vaccine inoculation, and group B received two doses of the COVID-19 vaccine. SARM1 was correlated significantly (p < 0.05) with MNSIe and NSS in group A and showed a consistent positive correlation with the other neuropathy clinical scales in group A and group B without reaching statistical significance. Additionally, SARM1 was negatively correlated significantly (p < 0.05) with the median sensory amplitude in group A and showed a consistent negative correlation with the six other sensory and motor nerves' potential amplitude in group A and group B without reaching statistical significance. In conclusion, SARM1 showed a consistent correlation with clinical neuropathy scales and nerve conduction parameters after accounting for the influence of COVID-19 vaccination doses.

Values and diagnostic accuracy of sensory nerve action potentials in control participants and participants with diabetes with and without clinical diabetic neuropathy, based on neuropathy scale measurements.

BACKGROUND: The assessment of the normative values of sensory nerve action potentials (SNAP) and their diagnostic accuracies using validated neuropathy-assessment tools to classify participants into groups with and without neuropathy was not previously described in the literature. METHODS: The Utah Early Neuropathy Scale (UENS), Michigan neuropathy-screening instrument, and nerve conduction data were collected prospectively. We described and compared the values of the sural, superficial peroneal sensory (SPS), and superficial radial SNAP amplitude in different age groups for three groups. Group 1 (G1)-control participants (UENS <5), group 2 (G2)-participants with diabetes without clinical diabetic neuropathy (UENS <5), and group 3 (G3)-participants with clinical diabetic neuropathy (UENS ≥5). We also described the diagnostic accuracy of single-nerve amplitude and a combined sensory polyneuropathy index (CSPNI) that consists of four total points (one point for each of the following nerves if their amplitude was <25% lower limit of normal: right sural, left sural, right SPS, and left SPS potentials). RESULTS: We assessed 135 participants, including 41, 37, and 57 participants in G1, G2, and G3, respectively, with age median (interquartile ranges) of 51 (45-56), 47 (38-56), and 54 (51-61) years, respectively, whereas 19 (46.3%), 18 (48.7%), and 32 (56.14%) of them were males, respectively. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) scores were 68.4%, 92.3%, 86.7%, and 80% for the sural amplitude; 86%, 58.3%, 62%, and 84% for the SPS amplitude; 66.7%, 94.4%, 90.5%, and 78.2% for the CSPNI of 3; and 54.4%, 98.6%, 96.9%, and 73.2% for the CSPNI of 4, respectively. CONCLUSION: Sural nerve had a high specificity for neuropathy; however, the CSPNI had the highest specificity and PPV, whereas the SPS had the highest sensitivity and NPV.

Utility of Initial Arterial Blood Gas in Neuromuscular versus Non-Neuromuscular Acute Respiratory Failure in Intensive Care Unit Patients.

Background: The arterial blood gas (ABG) parameters of patients admitted to intensive care units (ICUs) with acute neuromuscular respiratory failure (NMRF) and non-NMRF have not been defined or compared in the literature. Methods: We retrospectively collected the initial ABG parameters (pH, PaCO2, PaO2, and HCO3) of patients admitted to ICUs with acute respiratory failure. We compared ABG parameter ranges and the prevalence of abnormalities in NMRF versus non-NMRF and its categories, including primary pulmonary disease (PPD) (chronic obstructive pulmonary disease, asthma, and bronchiectasis), pneumonia, and pulmonary edema. Results: We included 287 patients (NMRF, n = 69; non-NMRF, n = 218). The difference between NMRF and non-NMRF included the median (interquartile range (IQR)) of pH (7.39 (7.32−7.43), 7.33 (7.22−7.39), p < 0.001), PaO2 (86.9 (71.4−123), 79.6 (64.6−99.1) mmHg, p = 0.02), and HCO3 (24.85 (22.9−27.8), 23.4 (19.4−26.8) mmol/L, p = 0.006). We found differences in the median of PaCO2 in NMRF (41.5 mmHg) versus PPD (63.3 mmHg), PaO2 in NMRF (86.9 mmHg) versus pneumonia (74.3 mmHg), and HCO3 in NMRF (24.8 mmol/L) versus pulmonary edema (20.9 mmol/L) (all p < 0.01). NMRF compared to non-NMRF patients had a lower frequency of hypercarbia (24.6% versus 39.9%) and hypoxia (33.8% versus 50.5%) (all p < 0.05). NMRF compared to PPD patients had lower frequency of combined hypoxia and hypercarbia (13.2% versus 37.8%) but more frequently isolated high bicarbonate (33.8% versus 8.9%) (all p < 0.001). Conclusions: The ranges of ABG changes in NMRF patients differed from those of non-NMRF patients, with a greater reduction in PaO2 in non-NMRF than in NMRF patients. Combined hypoxemia and hypercarbia were most frequent in PPD patients, whereas isolated high bicarbonate was most frequent in NMRF patients.

Atypical Familial Amyotrophic Lateral Sclerosis Secondary to Superoxide Dismutase 1 Gene Mutation With Coexistent Axonal Polyneuropathy: A Challenging Diagnosis.

Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease, is a neurodegenerative disease that involves both the upper and lower motor neurons. Familial ALS, including superoxide dismutase 1 (SOD1) mutation, accounts for 5-10% of all cases of ALS. Typically, the symptoms of ALS are purely motor, though coexistent sensory symptoms have been reported in rare cases. In this report, we describe the case of a 47-year-old man who presented with progressive bilateral lower limb weakness and numbness for the last four years. A nerve conduction study (NCS) showed evidence of coexistent axonal sensorimotor polyneuropathy in addition to the typical findings of ALS in needle electromyography. Genetic testing confirmed the diagnosis of familial ALS secondary to the SOD1 genetic mutation. This report highlights that the presence of sensory symptoms should not exclude the possibility of ALS in an appropriate clinical setting.

Translation, validation, and diagnostic accuracy of the Arabic version of the Michigan neuropathy screening instrument.

ABSTRACT: The Michigan Neuropathy Screening Instrument (MNSI) is used to screen patients for diabetic neuropathy (DNP). We aimed to translate the MNSI questionnaire into Arabic (MNSIq-Ar) and to assess the validity and diagnostic performance of the MNSI Arabic version (MNSI-Ar).Cronbach alpha α and the interclass correlation coefficient were used to measure the reliability and reproducibility of the MNSIq-Ar. The instrument's validity was assessed by Spearman correlation with the Utah Early Neuropathy Scale (UENS), the Modified Toronto Neuropathy Score (mTCNS), diabetic neuropathy symptoms (DNS), and sural nerve amplitude (SNA). The construct validity of the MNSI-Ar was assessed by its ability to differentiate the severity of DNP (using the Kruskal-Wallis test). The diagnostic performance was assessed through the receiver operator curve area.We recruited 89 participants (mean [SD] age, 50.8 [12.3] years; 48% men). The MNSIq-Ar showed an α of 0.81 and intraclass correlation coefficient = 0.94, and the correlation coefficients with UENS, mTCNS, DNS, and sural nerve amplitude were 0.67, 0.83, 0.73, and -0.49, respectively (all P < .0001). The MNSI-Ar was able to differentiate the different severities of DNP. The receiver operator curve area was 0.93 with a high sensitivity of 95.9% and 100% for probable and confirmed DNP, respectively.MNSI-Ar is a reliable and valid tool to screen for diabetic neuropathy in the Arabic language with a good diagnostic performance and high sensitivity.

Adult-type dermatomyositis with secondary lymphoid follicles harbouring reactive B-cells component.

Dermatomyositis (DM) is an immune-mediated inflammatory disease characterized by pathognomic lesions in skin and skeletal muscle including lymphocytic infiltrates. It rarely presents with ectopic lymphoid structures, as other autoimmune and chronic inflammatory diseases. We describe a case of a 47-year-old male, who presented clinically with proximal muscle weakness, skin rash and elevated creatin kinase (CK) levels. The muscle biopsy revealed inflammatory myopathy, with perifascicular pathology, and scattered ectopic lymphoid follicles-like structures harboring reactive B-cells. Clonality analysis of B-cells using polymerase chain reaction ruled out malignant lymphoma. The patient responded favorably to steroid therapy, and his muscle weakness improved. In conclusion, the clinical and histopathologic features of DM can be atypical, and the presence of lymphoid follicles, although rare, is not inevitably linked to an unfavorable prognosis.

Clinical features and outcome of Guillain-Barre syndrome in Saudi Arabia: a multicenter, retrospective study.

BACKGROUND: Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy characterized by rapidly evolving weakness and areflexia, reaching nadir within 4 weeks. Data on the characteristic of GBS in Saudi Arabia are limited. This study aimed to describe the clinical, electrophysiological, and laboratory characteristics and outcome of a multicenter cohort of patients with GBS. METHODS: This is a retrospective multicenter nationwide study. Patients who had GBS, identified through Brighton Criteria, between January 2015 and December 2019 were included. Data collected included demographics, clinical features, cerebrospinal fluid profile, reported electrophysiological patterns, treatment, and outcome. Reported GBS subtypes were compared using chi-square, Fisher's exact, or Mann-Whitney U tests, as appropriate. RESULTS: A total of 156 patients with GBS were included (men, 61.5%), with a median age of 38 (interquartile range, 26.25-53.5) years. The most commonly reported antecedent illnesses were upper respiratory tract infection (39.1%) and diarrhea (27.8%). All but two patients (98.7%) had weakness, 64.1% had sensory symptoms, 43.1% had facial diplegia, 33.8% had oropharyngeal weakness, 12.4% had ophthalmoplegia, and 26.3% needed mechanical ventilation. Cytoalbuminological dissociation was observed in 69.1% of the patients. GBS-specific therapy was administered in 96.8% of the patients, of whom 88.1% had intravenous immunoglobulin, and 11.9% had plasmapheresis. Approximately half of the patients were able to walk independently within 9 months after discharge, and a third regained the ability to walk independently thereafter. Death of one patient was caused by septicemia. Acute inflammatory demyelinating polyradiculoneuropathy was the most commonly reported GBS subtype (37.7%), followed by acute motor axonal neuropathy (29.5%), and acute motor-sensory axonal neuropathy (19.2%). CONCLUSION: The clinical and laboratory characteristics and outcome of GBS in the Arab population of Saudi Arabia are similar to the international cohorts. The overall prognosis is favorable.

A novel DOK7 mutation causing congenital myasthenic syndrome with limb-girdle weakness: case series of three family members.

Congenital myasthenia syndrome (CMS) is a group of heterogeneous diseases affecting the neuromuscular endplate. CMS has a considerably different phenotypic presentations, with the onset time ranging from early infancy to late adulthood. Here, we report a case of a CMS due to a new DOK7 mutation in a 28-year-old man and two of his sisters, who have a pure limb-girdle weakness. DOK7 CMS has a varying presentation. Typically, the onset occurs in childhood with ptosis, bulbar symptoms, difficulty walking, weakness, and gait abnormality. This case sheds light on a novel DOK7 gene mutation with a unique presentation of CMS and provides insight into its unique phenotypic presentation.

Mills' syndrome: Reporting the disease course with a monthly intravenous immunoglobulin program.

Mills' syndrome is an extremely rare, slowly progressive, unilateral ascending or descending clinical syndrome of upper motor neuron-predominant hemiparesis. In this article, we describe a case of a middle-aged woman (initial presentation and three years follow-up) who presented with progressive ascending hemiparesis with clinically isolated upper motor neuron signs and normal sensory examination. The patient received monthly intravenous immunoglobulin (IVIG) for three years with no progression of her weakness. To our best knowledge, the response of Mills' syndrome to such an IVIG program has not been reported in the literature so far. We aim to document the clinical response to IVIG in such rare syndrome.

A timed Phalen's test predicts abnormal electrophysiology in carpal tunnel syndrome.

OBJECTIVE: Previous studies reported variable sensitivity and specificity of the Phalen test. We investigated whether a timed Phalen's test (TPT) could predict abnormal nerve conduction studies (NCS) results in carpal tunnel syndrome (CTS). METHODS: Patients with CTS were consecutively recruited. A neurologist confirmed the clinical diagnosis of CTS and recorded the TPT before NCS were performed. Another neurologist, blinded to the TPT, graded the severity of NCS. The TPT sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) were calculated. RESULTS: In total, 403 patients with 706 hands were recruited and diagnosed with CTS; 465 hands had positive TPT, and 611 hands showed abnormal NCS results. A positive TPT at ≤ 10 s had a specificity of 96.8% and a PPV of 96.6% in predicting abnormal NCS. The sensitivity and NPV of TPT were insignificant. DISCUSSION: A positive TPT at ≤ 10 s can be useful in predicting NCS abnormalities in patients with CTS.

Exacerbation Rate in Generalized Myasthenia Gravis and Its Predictors.

OBJECTIVE: The aim of the study was to estimate the exacerbation incidence rate (IR) in acetylcholine receptor antibody (AChR)-positive generalized myasthenia gravis (MG) and its predictors. METHODS: The primary outcome in this retrospective study was to estimate moderate-to-severe (M-S) exacerbations IR in the early course of generalized MG. The secondary outcome was to explore the predictors of MG exacerbations. RESULTS: Between 1999 and 2015, we identified 78 AChR-positive generalized MG patients and 37 M-S exacerbations over the first 6 years following the onset of generalized MG symptoms. The M-S exacerbation IR was 12.2 per 100 person years (95% confidence interval [CI] 8.8-16.8). Any exacerbation (including mild) IR was 24.4 per 100 person years (95% CI 19.4-30.7). After controlling for confounding factors, MG exacerbation IR predictors included gender, disease severity at onset, and prednisone dose reduction with risk ratio of 0.34 (male gender), 2.67, and 20.8, respectively (all p values <0.05). M-S exacerbation occurred in 25 cases (32.1%), while any exacerbation (mild or M-S) was detected in 45 cases (57.7%). CONCLUSION: More than half of newly diagnosed AChR + MG cases experience an exacerbation in the first 6 years. Gender, disease severity at onset and prednisone dose reduction are predictors that could inform clinical practice and future research.

The Assessment of Major Histocompatibility Complex (MHC) Class-I Expression in Different Neuromuscular Diseases.

BACKGROUND: Major histocompatibility complex (MHC) class-1 antigen is a glycoprotein expressed in all nucleated cells. The aim of this study was to assess MHC class-I expression in different neuromuscular diseases. METHODS: The authors reviewed the data of 54 patients with neuromuscular diseases. Anti MHC class-I antibody was performed on the frozen muscle tissues using immunohistochemistry. MHC class-I was scored based on its expression on muscle fibers (0: normal, 1: expression <5 fibers, 2: expression in 5-10 fibers, 3: expression in >10 fibers). The pattern was only assessed in cases with MHC class-I scored 3 as: (1: Sarcocapillary, 2: Sarcocapillary and necrotic fibers, 3: Perifascicular). The relationship between MHC class-I expression and neuromuscular diseases was statistically analyzed. RESULTS: The mean age of the patients was 39.1 ± 18.5 years. Around 50% of patients showed normal CK levels and 5% of the cases showed elevated CK levels. There was a significance difference in MHC class-I expression between cases with normal and elevated CK levels when MHC class-I score was 3 (p= 0.020). There was a significant difference in MHC class-I expression among different neuromuscular diseases (p<0.001). All cases with idiopathic inflammatory myopathies (IIMs) have expressed MHC class-I in more than 10 fibers. MHC class-I was expressed in 15 cases of non-IIMs. CONCLUSION: MHC class-I cannot be solely used as a biomarker to distinguish IIMs from non-IIMs. The presence of MHC class-I molecules in non-IIMs might be related to immunoproteasomes mechanism. Further studies, with different muscle proteins expression and genomic sequencing, must be conducted to understand the role of MHC Class-I in neuromuscular diseases.

Amyotrophic lateral sclerosis care in Saudi Arabia: A survey of providers' perceptions.

OBJECTIVE: Provision of care for patients with amyotrophic lateral sclerosis (ALS) is complex and requires the contribution of multiple healthcare professionals. Several international ALS care measures were developed to ensure optimal care for ALS patients. We looked at the rate of inconsistency in providing standard ALS care measures in Saudi Arabia (SA). METHODS: A 5-point response survey was distributed to practicing neurologists in SA. They were asked to grade their perceived consistency of accessibility for 19 items of ALS care measures at their center. The list of ALS care measures items was derived from international ALS guidelines. RESULTS: The response rate from neurologists was 47.3% (62/131), and the responses of 39 neurologists who follow ALS cases were included. Most of the selected ALS care measure items, 63.1% (12/19), were perceived by 50% or more of the ALS care providers to be not consistently accessible to their patients. The perception of ALS care providers of the inconsistent accessibility for ALS patients to ALS care measures was high for communication devices (92.3%), supportive equipment such as motorized wheelchairs (76.9%), end-of-life discussion (74.4%), and respiratory monitoring (66.7%). CONCLUSION: Our data show that ALS patients in SA do not have consistent access to the recommended ALS care measures.

Impact of neurological diseases on family planning: A single-center experience.

This cross-sectional study aimed to assess the impact of epilepsy, myasthenia gravis (MG), and multiple sclerosis (MS) on pregnancy and family planning decision-making in a cohort of Saudi women. Women with epilepsy, MG, and MS were recruited consecutively at the time of their follow-up visits at a neurology clinic. Data were collected using 3 standardized questionnaires, and presented using descriptive statistics. A logistic regression was performed to determine variables associated with decisions regarding abstaining from pregnancy and encouraging other women to conceive. A total of 272 (83 epilepsy, 69 MG, and 120 MS) women with a mean age of 29.9 ±â€Š8.0 years participated. The proportion of women who abstained from or postponed pregnancy was 41.2% and 31.4%, respectively. The concerns mentioned most often were disease worsening during pregnancy, peripartum and postpartum, side effects of medications on the unborn child, and inability to care for the child. Older age was independently associated with the decision to abstain from pregnancy (odds ratio [OR] 1.14, 95% confidence interval [CI] 1.04 - 1.25). Higher knowledge levels were independently associated with encouraging other women to have children (OR 1.3, 95% CI 1.11-1.53). Over 50% of women reported that they were not counseled on issues related to pregnancy and childbirth. In conclusion, we identified a major influence of epilepsy, MG, and MS on pregnancy and family planning. Comprehensive counseling programs are needed to help women with these neurological diseases make informed family-planning decisions.

Brucellosis causing subacute motor polyradiculopathy and the pathological correlation of pseudomyopathic electromyography: A case report.

INTRODUCTION: Brucellosis is a rare cause of polyradiculopathy. We aim to present a case of subacute motor polyradiculopathy (SAMPR), along with the electromyographic pseudomyopathic changes, and their histopathological correlation. CASE PRESENTATION: A 24-year-old man presented with gradually progressive bilateral lower limb weakness for three weeks that progressed to a loss of ambulation in seven weeks. He had no ocular, facial, or sphincteric weakness and no sensory symptoms. He showed normal cognitive, cranial nerve, and upper limb exams. His lower limb power was medical research council (MRC) grade 3 proximally, and 4 distally. His reflexes were grade 2+ in the upper limbs and grade 0 in the lower limbs. The nerve conduction studies were normal. Electromyography (EMG) showed active denervation with a short-duration motor unit potential (MUP) and early recruitment. MRI showed a diffuse enhancement of the lumbosacral nerve roots. Cerebrospinal fluid (CSF) showed a protein of 2.7 g/L and a white blood cells (WBC) count of 420 cells per microliter. Muscle biopsy revealed neurogenic changes with secondary degenerating and regenerating fibers, explaining the small and short MUPs in the EMG. CSF grew Brucella after fourteen days of incubation. Serum showed high antibody titers for the Brucella species "Melitensis" and "Abortus". The patient started to walk again, ten months after starting a course of antibiotics. CONCLUSION: Neurobrucellosis can present primarily as SAMPR, sparing the sensory system. SAMPR, with ongoing degenerating and regenerating muscle fibers, may explain the pseudomyopathic changes found in electromyographic studies.

Carpal Tunnel Decompression Surgery Outcome and Effect of Diabetes.

OBJECTIVE: The benefits of carpal tunnel decompressive surgery (CTDS) among diabetic patients with carpal tunnel syndrome (CTS) were previously investigated through comparing the outcome before and after CTDS, and in comparison to nondiabetic CTS. We sought to investigate if diabetes mitigates the benefits of CTDS compared to not receiving CTDS. METHODS: In this retrospective study, we compared the risk of reporting any unfavorable outcomes among CTS patients (diabetic and nondiabetic) who underwent CTDS versus no CTDS after controlling for diabetes. We also compared the risk of reporting any unfavorable outcomes (waking up at night, pain during the day or during daily activities, or hand weakness) among diabetic CTS patients who underwent CTDS versus no CTDS after controlling for severity. RESULTS: We included 207 patients; of these, 105 patients had CTDS and 102 did not. There were 60 diabetic and 147 nondiabetic patients. The risk of any unfavorable outcomes was reduced by CTDS from 83.3 to 66.6%, with an odds ratio (OR), after controlling for diabetes, of 0.39 (95% confidence interval [CI] 0.20-0.78). Among diabetic patients, there was no difference between the CTDS and non-CTDS groups in the risk of reporting any unfavorable outcomes; however, after adjustment for severity, the risk of hand weakness was less with CTDS, with an OR of 0.13 (95% CI 0.02-0.86). CONCLUSION: Diabetes did not mitigate the benefits of CTDS. CTDS may prevent hand weakness among diabetic CTS patients.

Symptom Recognition Is Impaired in Patients With Orthostatic Hypotension.

Failure to recognize symptoms of orthostatic hypotension (OH) may result in falls, syncope, and injuries. The relationship between orthostatic changes in blood pressure and symptom occurrence and severity is not known. The goal of the present study was to define the relationship between the occurrence and severity of the symptoms of orthostatic hypotension (OH) and (1) the upright systolic blood pressure (SBP) and (2) the fall in SBP after tilting in patients with OH. We prospectively studied 89 patients with OH. Reported BP values include the lowest BP in the first 3 minutes of tilt and the change in blood pressure during tilt. Subjects were queried about symptoms of orthostatic intolerance while supine and during the first 3 minutes of tilt testing using Question 1 of the Orthostatic Hypotension Questionnaire. Mean tilted SBP was 101.6±26.1 mm Hg and mean SBP fall 47.9±18.1 mm Hg. Mean symptom scores when upright were: light-headedness (2.3/10±2.7), dizziness (1.6/10±2.5), and impending blackout (0.8/10±1.9). The majority of patients were asymptomatic or mildly symptomatic and no discrete cutoff for symptoms was observed. The magnitude of the SBP fall (r=-0.07, P=NS) and the lowest upright SBP (r=0.08, P=NS) did not correlate with any reported symptom. These results suggest a poor relationship between the magnitude of the orthostatic BP fall, the upright orthostatic BP, and symptoms. Many patients are asymptomatic despite substantial SBP falls and low orthostatic blood pressures. These findings have implications for clinical care of patients with OH and clinical trials to treat patients with OH.

Acute Paralytic Post-Bariatric Surgery Axonal Polyneuropathy: Clinical Features and Outcome.

INTRODUCTION: The syndrome of post-bariatric surgery axonal polyneuropathy (PAP) may present with various sensory and motor symptoms including paralysis. We aim to describe the diagnostic features and outcome of treatment of the acute paralytic form of PAP (acute paralytic PAP [APPAP]) as it was not described in a separate cohort previously. METHODS: We retrospectively reviewed medical charts and described the clinical, electrodiagnostic features, treatment, and outcome for patients who presented to our clinical neurophysiology unit with disabling weakness within 24 months post-bariatric surgery. The main outcome measure was the percent of patients who are able to walk independently at the last visit and comparing the group who resumed walking independently at 6 months to the group who did not, in regards to the use of intravenous immunoglobulin (IVIg). RESULTS: Thirteen patients were included (10 women and 3 men) with a mean age of 29.8 years (SD 12.5). All presented with loss of ambulation resembling Guillain-Barre Syndrome. The median time of onset was 4 months (interquartile range [IQR] 3-6) post-surgery, and the median time to weakness nadir was 3 weeks (IQR 3-3.5) with an average weight loss of 38.6 kg (SD 17.09). All patients regained their ability to ambulate; however, the ability to walk independently was achieved in 66.7% of patients. The percent of patients who were able to ambulate independently at 6 months were 16% with IVIg versus 66.7% without IVIg. CONCLUSION: The syndrome of -APPAP develops in the first-year post-bariatric surgery. The majority of patients regain independent ambulation.

Ulnar Neuropathy at the Elbow Associated With Focal Demyelination in the Proximal Forearm and Intraoperative Imaging Correlation.

Ulnar nerve focal demyelination (FD) in the forearm [defined as conduction block (CB) and or temporal dispersion (TD)] has been described with immune-mediated neuropathy and with compression affecting the forearm segment of the nerve. The association of FD in the forearm with entrapment ulnar neuropathy at the elbow, as well as the intraoperative imaging of the abnormal ulnar nerve at the flexor carpi ulnaris muscle level (FCU), has not been reported before. We report a 33-years-old woman presented with only sensory symptoms of the right hand suggestive of right ulnar neuropathy for the last 10 years. On clinical examination, she had reduced pinprick sensation on the little and ring fingers with no motor deficit. Nerve-conduction study showed slowing of conduction velocity across the elbow on the right when recording at the abductor digiti minimi (ADM) and first dorsal interossei (FDI). There was 63% amplitude drop when stimulating below the elbow compared to distal stimulation at the wrist. Increment inching study localized the block at 5 cm distal to the medial epicondyle. During surgical transposition, the ulnar nerve was swollen, and edematous in the segment where the nerve enters the FCU muscle, which provides a physiological explanation for the electrophysiological findings. After the surgery, the patient reported complete resolution of the symptoms. This case demonstrate that ulnar nerve motor potential FD at the proximal forearm could be recorded and it is still compatible with ulnar-nerve entrapment at the elbow.

Translation and validation of the arabic version of the myasthenia gravis activities of daily living scale.

INTRODUCTION: We translated the myasthenia gravis (MG)-specific activities of daily living (MG-ADL) scale into Arabic (MG-ADL-A) and assessed its psychometric properties. METHODS: We assessed reliability using Cronbach's α, reproducibility using the intraclass correlation coefficient, and validity using Spearman's correlations with MG composite (MGC) score, MG-specific manual muscle test (MG-MMT), and MG quality-of-life revised Arabic version (MGQOL15R-A). Differences in MG-ADL-A scores among patients with different disease severity were evaluated by using the Kruskal-Wallis test. Sensitivity to change was examined by using the Wilcoxon signed-rank test. RESULTS: We recruited 87 patients. The mean MG-ADL-A score was 3.38 ± 3.38 (α = 0.77, ICC = 0.99). The correlation coefficients between the MG-ADL-A and MGQOL15R-A, MGC, and MG-MMT were 0.63, 0.74, and 0.61, respectively (P < 0.001). The MG-ADL-A discriminated between different severity groups and was responsive to clinical improvement at follow-up. DISCUSSION: The MG-ADL-A has rigorous psychometric properties and can be used with Arabic-speaking patients with MG. Muscle Nerve 59:583-583, 2019.