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.

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.

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.

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.

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.

Satisfactory Response With Achieving Maintenance Low-Dose Prednisone in Generalized Myasthenia Gravis.

OBJECTIVES: To estimate the satisfactory response rate (SR%) with achieving maintenance, low-dose prednisone in acetylcholine receptor antibody-positive generalized myasthenia gravis. METHODS: In this retrospective study, we estimate the SR% as defined by (remission/minimal manifestations status for at least 6 months using 7.5 mg or less of prednisone daily, for maintenance treatment at 2, 4, and 6 years after symptoms onset) for patients who were not taking steroid-sparing immunosuppressant (SSI) as a primary outcome and for patients taking an SSI as a secondary outcome. RESULTS: Forty-five patients were not taking an SSI at 2 years, 34 patients at 4 years, and 17 patients at 6 years; SR% was 44.4%, 64.7%, and 58.8%, respectively. Thirty-six patients were taking an SSI at 2 years, 22 patients at 4 years, and 15 patients at 6 years; the SR% was 50.0%, 45.4%, and 66.7%, respectively. CONCLUSIONS: Nearly half of the generalized myasthenia gravis patients who were not taking an SSI achieved an SR.

Translation and validation of the arabic version of the revised 15-item myasthenia gravis quality-of-life questionnaire.

INTRODUCTION: We sought to translate, culturally adapt, and assess the Arabic version of the 15-item myasthenia gravis quality-of-life revised scale (MGQOL15R). METHODS: We assessed reliability with Cronbach α; reproducibility with intraclass correlation coefficient (ICC); validity with Spearman correlations for myasthenia gravis (MG)-specific activities of daily living (MG-ADL), MG composite (MGC) score, and MG manual muscle test (MG-MMT) and with MGQOL15R in patients with different disease severity through the Kruskal-Wallis test; and sensitivity to change with Wilcoxon signed-rank test. RESULTS: In 65 enrolled patients, the mean MGQOL15R score was 10.84 ± 8.11 (α = 0.94, ICC = 0.95). The correlation coefficients between MGQOL15R and MGC, MG-ADL, and MG-MMT scores were 0.75, 0.75, and 0.74, respectively (P < 0.001). MGQOL15R scores were significantly higher (worse) in patients with more severe disease at baseline and significantly lower (better) in improved patients at follow-up. DISCUSSION: The Arabic version of MGQOL15R is valid, reliable, stable, and sensitive to changes. Muscle Nerve 57: 581-585, 2018.

Less is More in Diabetic Neuropathy Diagnosis: Comparison of Quantitative Sudomotor Axon Reflex and Skin Biopsy.

OBJECTIVES: To compare the frequency of abnormalities in epidermal nerve fiber density (ENFD) and quantitative sudomotor axon reflex (QSART) in patients with diabetic distal symmetric polyneuropathy (DSPN). METHODS: Nerve conduction studies, ENFD, and QSART data were obtained pre- and postexercise, in patients enrolled in a prospective diabetic neuropathy study. McNemar's test was applied to compare the yield of ENFD and QSART. RESULTS: Eighteen patients (58 ± 4 years) were enrolled, with 36 data collection points. In diabetic DSPN and diabetic large fiber DSPN (DSPN-L), abnormal ENFD (77% and 100% respectively) is more frequent than abnormal QSART (39% and 35%, respectively) (P value = 0.001 in diabetic DSPN and P value = 0.0002 in diabetic DSPN-L), whereas in diabetic small fiber DSPN (DSPN-S), both tests have similar yields (47%). CONCLUSIONS: ENFD has a high diagnostic yield in diabetic DSPN and DSPN-L. Including QSART data adds little to the sensitivity of EFND in DSPN-S.

Stroke recurrence rates among patients with symptomatic intracranial vertebrobasilar stenoses: systematic review and meta-analysis.

BACKGROUND: A recent randomized trial of patients with primarily anterior circulation intracranial artery stenosis showed that intensive medical therapy was superior to intracranial stenting in preventing recurrent stroke. The rate of stroke recurrence or death in symptomatic intracranial vertebrobasilar stenosis with medical therapy alone may be especially high, and rates compared with endovascular therapy need further study. METHODS: We conducted a systematic review and meta-analysis of studies reporting the rates of stroke recurrence or death (the primary outcome) in symptomatic intracranial vertebrobasilar stenosis with medical or endovascular treatment over a minimum follow-up period of 6 months. We included all studies in any language indexed in MEDLINE or EMBASE, supplemented by bibliography searches and by contacting the authors. The secondary endpoints were stroke recurrence, and basilar artery and vertebral artery stroke recurrence rates. RESULTS: 23 studies (592 medical treatment patients and 480 endovascular treatment patients) were included. The risk of combined stroke recurrence or death was 14.8 per 100 person-years (95% CI 9.5 to 20.1) in the medical group compared with 8.9 per 100 person-years (95% CI 6.9 to 11.0) in the endovascular group. The incidence rate ratio was 1.3 (95% CI 1.0 to 1.7). The stroke recurrence rate was 9.6 per 100 person-years (95% CI 5.1 to 14.1) in the medical group compared with 7.2 per 100 person-years (95% CI 5.5 to 9.0) in the endovascular group. CONCLUSIONS: Our results showed that the risk of stroke recurrence or death or the risk of stroke recurrence alone was comparable between the medical and endovascular therapy groups. A small preventive effect of endovascular therapy may exist, particularly if the 30 day postprocedural risk is reduced.

Etiologic classification of TIA and minor stroke by A-S-C-O and causative classification system as compared to TOAST reduces the proportion of patients categorized as cause undetermined.

BACKGROUND: The assortment of patients based on the underlying pathophysiology is central to preventing recurrent stroke after a transient ischemic attack and minor stroke (TIA-MS). The causative classification of stroke (CCS) and the A-S-C-O (A for atherosclerosis, S for small vessel disease, C for Cardiac source, O for other cause) classification schemes have recently been developed. These systems have not been specifically applied to the TIA-MS population. We hypothesized that both CCS and A-S-C-O would increase the proportion of patients with a definitive etiologic mechanism for TIA-MS as compared with TOAST. METHODS: Patients were analyzed from the CATCH study. A single-stroke physician assigned all patients to an etiologic subtype using published algorithms for TOAST, CCS and ASCO. We compared the proportions in the various categories for each classification scheme and then the association with stroke progression or recurrence was assessed. RESULTS: TOAST, CCS and A-S-C-O classification schemes were applied in 469 TIA-MS patients. When compared to TOAST both CCS (58.0 vs. 65.3%; p < 0.0001) and ASCO grade 1 or 2 (37.5 vs. 65.3%; p < 0.0001) assigned fewer patients as cause undetermined. CCS had increased assignment of cardioembolism (+3.8%, p = 0.0001) as compared with TOAST. ASCO grade 1 or 2 had increased assignment of cardioembolism (+8.5%, p < 0.0001), large artery atherosclerosis (+14.9%, p < 0.0001) and small artery occlusion (+4.3%, p < 0.0001) as compared with TOAST. Compared with CCS, using ASCO resulted in a 20.5% absolute reduction in patients assigned to the 'cause undetermined' category (p < 0.0001). Patients who had multiple high-risk etiologies either by CCS or ASCO classification or an ASCO undetermined classification had a higher chance of having a recurrent event. CONCLUSION: Both CCS and ASCO schemes reduce the proportion of TIA and minor stroke patients classified as 'cause undetermined.' ASCO resulted in the fewest patients classified as cause undetermined. Stroke recurrence after TIA-MS is highest in patients with multiple high-risk etiologies or cryptogenic stroke classified by ASCO.