Ice Pack Test Research Articles

Accuracy of Repetitive Ocular Vestibular-Evoked Myogenic Potentials to Diagnose Myasthenia Gravis in Patients With Ptosis or Diplopia.

We developed repetitive ocular vestibular-evoked myogenic potentials (roVEMP) as an electrophysiologic test that allows us to elicit the characteristic decrement of extraocular muscles in patients with ocular myasthenia gravis (OMG). Case-control studies demonstrated that roVEMP reliably differentiates patients with OMG from healthy controls. We now aimed to evaluate the diagnostic accuracy of roVEMP for OMG diagnosis in patients with ptosis and/or diplopia. In this blinded prospective diagnostic accuracy trial, we compared roVEMP in 89 consecutive patients presenting with ptosis and/or diplopia suspicious of OMG with a multimodal diagnostic approach, including clinical examination, antibodies, edrophonium testing, repetitive nerve stimulation of accessory and facial nerves, and single-fiber EMG (SFEMG). We calculated the roVEMP decrement as the ratio between the mean of the first 2 responses compared with the mean of the sixth-ninth responses in the train and used cutoff of >9% (unilateral decrement) in a 30 Hz stimulation paradigm. Following a complete diagnostic work-up, 39 patients (44%) were diagnosed with ocular MG, while 50 patients (56%) had various other neuro-ophthalmologic conditions, but not MG (non-MG). roVEMP yielded 88.2% sensitivity, 30.2% specificity, 50% positive predictive value (PPV), and 76.5% negative predictive value (NPV). For comparison, SFEMG resulted in 75% sensitivity, 56% specificity, 55.1% PPV, and 75.7% NPV. All other diagnostic tests (except for the ice pack test) also yielded significantly higher positive results in patients with MG compared with non-MG. The study revealed a high sensitivity of 88.2% for roVEMP in OMG, but specificity and PPV were too low to allow for the OMG diagnosis as a single test. Thus, differentiating ocular MG from other neuro-ophthalmologic conditions remains challenging, and the highest diagnostic accuracy is still obtained by a multimodal approach. In this study, roVEMP can complement the diagnostic armamentarium for the diagnosis of MG. This study provides Class I evidence that in patients with diplopia and ptosis, roVEMP alone does not accurately distinguish MG from non-MG disorders. ClinicalTrials.gov: NCT03049956.

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report

BackgroundUraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy.Case presentationA 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain.ConclusionsUraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.

Progressive Bilateral Ptosis in Adduction Deficit: True Internuclear Ophthalmoplegia (INO) or Pseudo-INO?

Abstract
 Introduction : Bilateral INO is a rare disease caused by a lesion in the medial longitudinal fasciculus (MLF) in the pons, resulting in adduction deficit. Progression to ptosis is rare and may confuse with pseudo-INO as in ocular myasthenia gravis (OMG). This case aims to report progressive ptosis in bilateral INO that mimics pseudo-INO.
 Case Illustration : A 36-year-old woman complained sudden binocular diplopia 4 days before admission. Bilateral adduction deficits (-3 and -2 of right and left eye, respectively) with nystagmus were seen. There was no ptosis. The patient was diagnosed with bilateral INO. One month later, the patient returned with bilateral ptosis. The marginal reflex distance 1 (MRD1) were 0 on both eyes. Post fatigue and ice pack test showed 2mm difference of MRD1. The single fiber electromyography (EMG) result came out negative with jitter <10%. Brain MRI revealed lesions in the posterior pons and periaqueductal on the level of midbrain.
 Discussion : The MLF controls 6th and contralateral 3rd cranial nerve nucleus for horizontal gaze. Ptosis in INO can occurred due to central caudal subnucleus involvement in midbrain that innervates levator palpebra. The result of fatigue and ice pack test may be misleading, as those are not pathognomonic for OMG. The single-fiber EMG are valuable modalities to exclude pseudo-INO in this case.
 Conclusion : Progressive bilateral ptosis may occur in bilateral INO. Fatigue and ice pack test may give false- positive results leading to confusion with pseudo-INO, rather than true INO. Supporting examinations are needed to confirm the diagnosis.

Prevalences of Other Non-Thyroid Autoimmune Diseases and Factor Associated with Their Presence in Ocular Myasthenia Gravis.

To report the prevalences of other non-thyroid autoimmune diseases and identify factors associated with their presence in ocular myasthenia gravis (OMG) subjects. A total of 208 subjects with OMG diagnosis were included. Demographic data, clinical characteristics, the ice-pack test, the acetylcholine receptor (AChR) antibody test, electrophysiology tests (single-fiber electromyography and repetitive nerve stimulation), the presence of thymoma, generalized myasthenia gravis conversion, and the presence of other non-thyroid autoimmune diseases (defined as the presence of at least one other non-thyroid autoimmune disease) were retrospectively reviewed. Factors associated with the presence of other non-thyroid autoimmune diseases were analyzed by univariate and multivariate logistic regression. Of the total 208 subjects, 21 (10.10%) exhibited the presence of other non-thyroid autoimmune diseases (19 subjects (9.14%) and 2 subjects (0.96%) had one and two other non-thyroid autoimmune diseases, respectively), and systemic lupus erythematosus (SLE) was diagnosed in 9 subjects, followed by Sjogren's syndrome (7 subjects), rheumatoid arthritis (6 subjects), and ankylosing spondylitis (1 subject). Therefore, the prevalences of SLE, Sjogren's syndrome, rheumatoid arthritis, and ankylosing spondylitis in OMG subjects were estimated to be 4.33% (95% confidence interval (CI): 2.29-8.02%), 3.37% (95% CI: 1.64-6.79%), 2.88% (95% CI: 1.33-6.14%), and 0.48% (95% CI: 0.08-2.67%), respectively. Positivity of the AChR antibody was the only significant factor associated with the presence of other non-thyroid autoimmune diseases (odds ratio 4.10, 95% CI: 1.11-15.21, p = 0.035). The presence of other non-thyroid autoimmune diseases was found in approximately 10% of OMG patients, with SLE displaying the highest prevalence. We recommend screening and monitoring for other non-thyroid autoimmune diseases in OMG patients, particularly those with positivity of the AChR antibody.

Levator Resection in Neurogenic Ptosis Oculi Sinistra et causa Third Nerve Paresis + Type II Diabetes: A Case Report

Introduction : Neurogenic ptosis is abnormal position of the upper eyelid caused by dysfunction or damage to the oculomotor or sympathetic nerves supplying the eyelids. Third nerve palsy (35.7%) is the most common cause among others. We report a case of neurogenic ptosis managed surgically by levator resection.
 Case Illustration : A 67-year-old male complained of drooping left upper eyelid, getting worse in 7 months. He had history of falling off motorcycle 6 months ago. Clinical histories of hypertension and diabetes mellitus were found. Right and left eyelids measurements in millimeter showed horizontal palpebral fissure (HPF) 28/26, vertical palpebral fissure (VPF) 6/3, margin reflex distance 1 (MRD1) 2/-2, margin reflex distance 2 (MRD2) 6/5, margin limbal distance (MLD) 8/4, and levator function (LF) 8/3. Bell’s phenomenon was negative in both eyes. Ice pack test was negative. There were ocular movement restriction in his left eye. Patient underwent a maximum levator resection and had better levator function after 3 weeks follow up.
 Discussion : Neurogenic ptosis caused by third nerve injury is associated with microvascular ischemia such as diabetes mellitus and hypertension. Operative management is carried out after 3 to 6 months observation. Evaluation of levator function allows the clinician to correctly classify the degree and type of ptosis. Maximal levator resection is the treatment of choice for ptosis with poor levator function.
 Conclusion : Although various surgical techniques have been developed, ptosis correction should be performed according to the indications and clinical condition of the patient.

GOOD OUTCOMES OF OCULAR MYASTHENIA GRAVIS IN A YOUNG ADULT MALE: A CASE REPORT

Abstract
 Introduction : Myasthenia gravis (MG) is characterized by fluctuating weakening and fatigability of skeletal muscles. Ocular MG (OMG) occurs when the symptoms of MG are limited to the ocular muscles.
 Case Illustration : A 21-year-old male presents with a two-month history of drooping both eyelids. The drooping eyelids improved in the morning or after a nap, and got worse in the evening. Ocular examination revealed limitation towards all gazes with bilateral ptosis. Visual acuity, anterior segment, visual fields, slit lamp, and fundus examinations were unremarkable. The ice pack and rest test resulted in a significant improvement after two minutes of ice pack application and 30-minute rest, raising suspicion for the diagnosis of MG. Acetylcholine receptor antibody (AChR) test was positive for AChR binding antibody. Treatment for OMG is initiated with an oral acetylcholinesterase inhibitor, pyridostigmine, and corticosteroid. The ptosis and ophthalmoplegia improved after one week follow- up and recovered completely after eight weeks of follow-up.
 Discussion : The hallmarks of MG are fluctuating fatigable muscle weakness that worsen with activity and improve on rest. Neurological exam and clinical neurological history are essential to establish the diagnosis. Bedsite tests, electrophysiologic test, and the antibody test all support the clinical diagnosis of MG.
 Conclusion : OMG progresses to its generalized form 90% of the time during the first two years of the onset of ocular symptoms. Early detection and prompt treatment are the key which may help to prevent or minimize the occurrence of myasthenia crisis.

Myasthenia gravis patients exhibiting an eyelid myotonia-like phenomenon

We report here a retrospective case series of 3 MG patients suffering from difficulty opening eyes that appeared together with a diagnosis of MG. All are male patients with late-onset MG who are seropositive for anti-acetylcholine receptor antibodies. The phenomenon was characterized by difficulty opening the eyes after forced closure or reflex eye closure, improving with the ice pack test and with repeated forced eye closure but worsening with pyridostigmine treatment. We provide a detailed clinical, serological, imaging and electrophysiological examination of these patients. Electromyography evaluation did not show spontaneous muscle activity or myotonia at rest in the orbital part of the orbicularis oculi muscle. However, there was sustained muscle activity lasting several seconds in the pre-tarsal and pre-septal parts of this muscle. Videos of those reported symptoms were produced and provided. We discuss the possible neurological pathophysiology of this disorder and suggest to name this rare ocular disorder “myotonia-like disorder of the pre-tarsal and pre-septal parts of the orbicularis oculi”. This study expands our knowledge of this rare clinical feature of MG and highlights the need for increased awareness of it and further investigation of this ocular manifestation.

Diagnostic challenges in myasthenia gravis: a clinical approach

The development of antibody tests and neurophysiological techniques have aided in confirming the diagnosis of myasthenia gravis (MG) over the years. However, there still remains an unmet diagnostic need in the subgroup of MG patients with weakness restricted to ocular muscles (OMG) as routine diagnostic tests are less sensitive in this group: around 50% of these patients have no positive antibody test and around 71% have no significant decrement with repetitive stimulation EMG. Moreover, virtually all disorders that can cause a pupil-sparing ptosis or diplopia have been reported to be confused with OMG. Among the most mentioned mimicks for OMG are (concomitant) Graves ophtalmopathy, cranial nerve palsies, ocular tendinomuscular deficits (such as levator dehiscence), myopathy, demyelinating disease and stroke. Diagnostic delay and confusion of OMG with mimicking disorders might lead to a worse prognosis due to a possible increased risk of generalization of disease and the need of emergency treatments. A careful clinical follow-up of patients with suspected OMG by systematically assessing changes in ocular weakness patterns between visits can aid in confirming the diagnosis. In addition, the ice pack test can be a diagnostic aid in cases of both evident ptosis as ophtalmoparesis. In the foreseeable future, cell-based assays (CBA) might aid in the diagnostic confirmation of OMG. There is a need of studies that investigates the yield of new and not-routinely used diagnostic tests in suspected OMG with negative antibody and inconclusive EMG and SF-EMG, such as the repetitive ocular vestibular evoked myogenic potentials (RoVEMP) test and CBA. Lastly, the effect of early immunosuppressive treatment should be further investigated in OMG.

Triple M Syndrome with Triple Seronegative Myasthenia Gravis presenting as a Non-ST Elevation Myocardial Infarction (P1-8.002)

<h3>Objective:</h3> N/A <h3>Background:</h3> Immune checkpoint inhibitors hinder different receptors in the immune system that maintain immunologic self-tolerance. Despite their efficacy, multiple reports of immune related adverse events exist. 1–3% of these cases are neurological. There is no clear consensus on how to treat this patient population. <h3>Design/Methods:</h3> We present a case of myocarditis, myositis and triple seronegative myasthenia gravis overlap syndrome secondary to PD-1 inhibitor. <h3>Results:</h3> 75-year-old male with a history of lung cancer and severe coronary artery disease who presented for chest pain and dyspnea. He had recently been started on gabapentin for chemotherapy related neuropathy. Due to recurrent lung cancer he was started on a new PD-1 inhibitor. On admission, he had elevated troponins. Emergency cardiac catheterization was negative for new coronary artery disease. Echocardiogram showed septal and LV wall hypokinesis, patient was admitted as NSTEMI. On physical exam, he had neck muscle weakness with drooping head sign, right eye ptosis, and proximal limb muscle weakness with fatigability of all extremities. Ice pack test done at the bedside showed improvement in right eye ptosis and neck drop. Anti-Ach Binding, Block and Modulating, Anti-Musk and Anti-LRP4 antibodies were negative. Due to suspicion of triple M syndrome he was treated IV Methylprednisolone and Pyridostigmine which stabilized his weakness. His dyspnea continued to worsen despite steroid therapy and he was started on IVIG. After 5 doses he significantly improved and was discharged to an acute rehabilitation facility. <h3>Conclusions:</h3> ICIs associated myocarditis can occur in 1% of patients. Up to 10% of these patients can have overlapping myasthenia gravis and myositis. 10% of patients with this syndrome can have seronegative myasthenia gravis. There are no guidelines adressing the correct treatment approach for this syndrome. In our case, gabapentin possibly precipitated the myasthenic symptoms and treatment with Corticosteroids and IVIG resolved the patient’s symptomatology. <b>Disclosure:</b> Dr. Carranza-Renteria has nothing to disclose. Dr. Mattner has nothing to disclose. Ms. Sial has nothing to disclose. Dr. Babici has nothing to disclose. Dr. Dragomir has nothing to disclose. Dr. Rodriguez-Hernandez has nothing to disclose. Dr. Hammond has nothing to disclose.

The Icepack Test in the Diagnosis of Myasthenia Gravis with Ocular Features: A Systematic Review of Diagnostic Accuracy, Technique, and Economic Utility

ABSTRACT Background The diagnosis of myasthenia gravis (MG) may be challenging and require multiple specialised testing modalities. Accessing these investigations can involve significant waiting time and costs. The bedside icepack test (IPT) has been proposed to assist with the diagnosis of MG with ocular features, and may prove an economically viable; however, there have been there is heterogeneity in the literature evaluating the IPT. Objectives A systematic review was performed examining the accuracy, described techniques, and economic implications of the IPT for the diagnosis of MG with ocular features. Method The databases EMBASE, PubMed, and the Cochrane Library were searched from inception to July 2022. The systematic review adhered to PRISMA guidelines. Eligibility determination was undertaken with a standardised form using appropriate inclusion criteria. The Cochrane risk of bias assessment tool for diagnostic test accuracy was employed to evaluate studies that presented the diagnostic performance of the IPT. The Johanna Briggs Institute Critical Appraisal Checklist for Economic Evaluations was used for the assessment of studies presenting economic evaluations of the IPT. Results 20 articles met the specified criteria and included a total of 1264 participants. The IPT had a sensitivity ranging from 38.5% to 100%. Specificity was found to be > 95% in six studies. Excluding two outlier results of 25% and 31.3%, the lowest specificity recorded was 62.5%. The most commonly described method of evaluating the IPT involved applying ice to both eyelids and using a >2 mm change as a threshold for a positive test (evaluated with a ruler). There were no adverse effects described with the IPT. There were no studies that formally examined the economic implications of the IPT. Conclusions The IPT is a well-tolerated and readily available diagnostic tool that has an important role in the evaluation of possible MG with ocular features in specific contexts. Despite limited economic evaluation of this test, it is likely the use of the IPT may result in significant financial and time savings.

Incidence and causes of overdiagnosis of myasthenia gravis.

We have encountered non-myasthenic patients being given a diagnosis of myasthenia gravis (MG). This study aims to investigate the frequency of, and factors contributing to, overdiagnosis of MG. This is a retrospective analysis of patients referred to our tertiary neuromuscular center for evaluation due to a previously suspected/confirmed MG diagnosis during a 6-year span. A total of 531 patients sought a second opinion regarding their MG diagnosis, and 77 (14.5%) were found to have non-myasthenic conditions. A total of 11 patients tested positive for acetylcholine receptor (AChR) antibodies. Repeated AChR antibodies became negative in five patients while in four patients, AChR binding antibody titers were persistently low. In seven patients, striational antibody was the only positive antibody identified. In 25 patients, a prior electrodiagnostic (EDX) study was deemed positive, including 14 patients with abnormal repetitive nerve stimulation (RNS) and 12 with abnormal single fiber electromyography (SFEMG). Technical issues were noted on prior RNS studies in 8 patients, and repeat RNS was negative in 10 patients. In eight patients with previously abnormal SFEMG, results showed minimal or equivocal abnormalities. In two patients, a repeat SFEMG was normal. Further analysis revealed atypical clinical presentation, deceptively positive ice pack test, clinically insignificant antibody result and misleading EDX finding as main contributors to MG overdiagnosis. Overdiagnosis of MG is not uncommon, and occurs more frequently in seronegative patients. To make an accurate diagnosis of MG, there is a need to recognize atypical presentations, and avoid overreliance on minor or non-specific serological and electrodiagnostic findings.

Pattern of Ocular Myasthenia Gravis in a Tertiary Eye Care Hospital, Bangladesh

Purpose: The purpose of this study was to evaluate pattern of ocular myasthenia gravis patients in a tertiary eye care hospital in Bangladesh.&#x0D; Methodology: This observational study was conducted in National Institute of Ophthalmology and Hospital (NIOH), Dhaka from January 2019 to December 2019. A total of 33 cases were purposively selected among ocular myasthenia gravis patients attending in the Department of neuro ophthalmology of NIOH irrespective of age, gender, residence. Study variables wereage, gender, clinical features, presence of thymoma, clinical, pharmacological and serological test findings. A proforma was prepared to record the data on particulars of the patients.&#x0D; Results: Among the cases, 45.5% were male and 54.5% were female where the mean age was 38.7 years. All patients presented with ptosis (100.0%) and most of them had fatigability(87.9%), cogan twice sign (81.8%) andorbicularis weakness (81.8%) while 63.6% had diplopia. Diabetes mellitus was observed in 18.2%patients and 15.1% had thyroid associations. Most of them had positive ice test(87.9%), 51.5% had positive serological test and 6.1% had positive neostigmine test. Thymoma was present in 6.1% cases.&#x0D; Conclusion: Ptosis was the most common clinical presentation for ocular myasthenia. Clinical diagnosis was confirmed by ice pack test and neostigmine test. Ocular myasthenia gravis patients may be have other systemic diseases.&#x0D; J. Natl. Inst. Ophthalmol. 2022;5(1):16-20

Lambert-Eaton Myasthenic Syndrome Unmasked by Administration of Aggravating Medications

We present a case of an elderly female patient presenting with symptoms strongly suggestive of Myasthenia Gravis (MG), but later serological workup was shown to be more consistent with Lambert Eaton Myasthenic Syndrome (LEMS). On presentation, the patient had both profound ptosis and generalized weakness of the upper and lower extremities that were suspected clinically to be due to MG. The case was further complicated by the administration of several medications, which were believed to have unmasked her disease. Diagnostic imaging to assess for thymoma and lung cancer was negative. Screening with the ice-pack test was positive; however, serology for MG was negative. This case illustrates the importance of keeping the differential of LEMS in view in every suspected MG case, even if there is a low suspicion of LEMS initially. In addition, it demonstrates the possibility that the "ice-pack test" can also be positive in LEMS, and MG-unmasking medications may also unmask LEMS, an association that has not been described in our literature review.

Myasthenia Gravis Presenting after Administration of the mRNA-1273 Vaccine.

The mRNA-1273 SARS-CoV-2 vaccine received emergency use authorization in December 2021. We present a case of myasthenia gravis (MG) which became clinically apparent following vaccination against SARS-CoV-2. A 30-year-old man developed acute onset diplopia, 2 days after receiving his first mRNA-1273 vaccination against SARS-CoV-2. He reported blurred vision with horizontally displaced images, which worsened with increased eye strain. Diplopia resolved when one eye was covered. He also had fatigable arm weakness, but denied dysphagia, dysarthria, dysphonia or dyspnoea. On examination, he had left-sided ptosis and esotropia at rest which worsened with sustained upward gaze and prolonged focus. He also had fatigable weakness of neck flexion and extension (4+/5), and generalized, fatigable weakness (4/5). His single-breath count was 38. Cranial nerves, sensory examination and deep tendon reflexes were normal. A 2-min ice-pack test and neostigmine test temporarily improved his diplopia and ptosis. The acetylcholine receptor (AChR) antibody was borderline high and muscle-specific tyrosine kinase (MuSK) antibody was negative. Chest CT and brain MRI with contrast were unremarkable. The patient was diagnosed with MG and oral pyridostigmine and prednisone therapy were initiated.We present a case of newly diagnosed MG after administration of mRNA-1273 vaccination against SARS-CoV-2. Although there has been long-standing discussion regarding the potential for vaccines to exacerbate autoimmune conditions, data remain sparse and consensus has not been reached. Consequently, this case is important to make providers aware of potential side effects of a novel vaccine, and may also help guide the selection of vaccination candidates and monitoring parameters.LEARNING POINTSWe present a case of newly diagnosed myasthenia gravis after administration of the mRNA-1273 SARS-CoV-2 vaccine.mRNA-1273 vaccination against SARS-CoV-2 may exacerbate subclinical cases of myasthenia gravis.Recognition of new vaccine side effects may guide the selection of vulnerable patients.

What is in the Neuromuscular Junction Literature?

This update covers a number of treatment topics starting with Fc receptor inhibitors and the Federal Drug Administration approval of efgartigimod. Some uncertainties regarding the use of corticosteroids are addressed, namely the risk of exacerbation with initiation of treatment and how to taper. The presence and potential importance of antibody overshoot following plasmapheresis is noted and the evolving increase in usefulness of acetylcholine receptor antibodies in diagnosing ocular myasthenia. Several recent series and case reports regarding coronavirus 2019 and myasthenia gravis are reviewed. The topics of myasthenia gravis and pregnancy, and another look at thymectomy in MG are provided. Finally, a couple of case reports on Lambert-Eaton myasthenic syndrome concentrate on the ice pack test and an autoantibody association with paraneoplastic cerebellar degeneration and Lambert-Eaton myasthenic syndrome in the same patient.

Diagnostic accuracy and clinical utility of bed side tests versus laboratory tests in the diagnosis of ocular myasthenia.

Purpose:To assess the diagnostic accuracy of forced eyelid closure test (FECT), ice pack test (IPT), repetitive nerve stimulation test (RNS), and acetylcholine receptor (AchR) antibody test in patients with suspected ocular myasthenia. To assess the clinical utility of AchR antibody test in predicting disease progression.Methods:Retrospective cohort study of patients diagnosed with ocular myasthenia at a South-Indian neuro-ophthalmology tertiary eye clinic. Baseline characteristics; ocular myasthenia symptoms; results of FECT, IPT, RNS, and AchR antibody test; and progression time to generalized myasthenia (GM) over 36 months from the time of diagnosis were recorded and analyzed using receiver operator curve analysis, multiple logistic regression, and Kaplan–Meier survival analysis.Results:FECT had a sensitivity of 96.7% (95% CI: 88.5–99.6) and a specificity of 75% (95% CI: 34.9–96.8). Combination of FECT and IPT, using the positivity of at least one test, increased the sensitivity to 98.3% (95% CI: 91–100), reducing the specificity to 50% (95% CI: 15.7–84.3), whereas using the positivity of both tests, we obtained a sensitivity of 71.7% (95% CI: 58.6–82.5) and a specificity of 100% (95% CI: 63.1–100). In the subset of patients with double negative RNS and AchR antibodies, the positive predictive value of combined FECT and IPT (double positive) was 100%. Patients who developed GM were more likely to have a positive AchR antibody test result (P = 0.001)Conclusion:Combined FECT and IPT (double positive) has high diagnostic accuracy even among patients with normal RNS and negative AchR antibodies. Despite low sensitivity, AchR-antibody test has a significant predictive value in disease progression.

Ice Pack Test in Myasthenic Ptosis as an Important Tool for Clinical Practice.

De Moura Campos, Marcelo Henrique; Magno Gonçalves, Marcus Vinicius MD, PhD Author Information

Juvenile Generalized Myasthenia Gravis: Presented as Unilateral Blepharoptosis and Successfully Managed with Pulse Intravenous Methylprednisolone

Myasthenia Gravis is a rare autoimmune disorder of childhood and this is rarer in South Asia. We present a pre-pubertal 7 year old female child of seropositive Generalized Juvenile Myasthenia Gravis. She presented with unilateral blepharoptosis and later generalized symptoms appeared. Ice-pack test, Neostigmine challenge test and acetylcholine receptor antibody test were positive. Serum muscle specific tyrosine kinase antibody test was normal. She did not have thymic abnormalities. She did not respond to high dose (26 mg/kg/day) of Pyridostigmine and oral Prednisolone (2 mg/kg/day), but was successfully treated with a combination of pulse intravenous Methylprednisolone (30 mg/kg once a month for 6 months) and daily doses of oral Prednisolone (2 mg/kg/day) along with Pyridostigmine without significant side effects. This combination can be considered a potential inexpensive treatment for Juvenile Myasthenia Gravis in a resource limited area where other immunosuppressive treatments such as intravenous immunoglobulin is expensive and unaffordable.

Myasthenic or cataplectic facies? Ice pack test response in paediatric type 1 narcolepsy

Myasthenic or cataplectic facies? Ice pack test response in paediatric type 1 narcolepsy

Editors' Note: Comparison of Ice Pack Test and Single-Fiber EMG Diagnostic Accuracy in Patients Referred for Myasthenic Ptosis

In “Comparison of Ice Pack Test and Single-Fiber EMG Diagnostic Accuracy in Patients Referred for Myasthenic Ptosis,” Giannoccaro et al. compared the diagnostic accuracy of the ice pack test (IPT) with single-fiber EMG (SF-EMG) on the orbicularis oculi muscle in 155 patients, with ptosis being evaluated for ocular myasthenia defined as a positive response to the edrophonium test, presence of acetylcholine-receptor antibodies, a decrement of >10% of the third to fifth compound muscle action potential after repetitive nerve stimulation, or unequivocal response to oral steroids or acetylcholinesterase inhibitors for at least 3 months. They found that the IPT and SF-EMG had similar diagnostic accuracy in this patient population. Silvestri noted interest that (1) the IPT, a simple and cheap bedside assessment, is comparable with a complicated test like the SF-EMG; (2) the results of the IPT and SF-EMG were discordant for 10% of subjects (mostly in the setting of mild or isolated ptosis); and (3) the utility of the IPT was not evaluated in patients with isolated diplopia. Giannoccaro and Liguori responded that the discordance in results generally occurred in the setting of a negative IPT, which may be related to the lack of repetition of the test. They also cited previously published data that the IPT is useful in patients with isolated diplopia, particularly because SF-EMG may be negative in these patients. In “Comparison of Ice Pack Test and Single-Fiber EMG Diagnostic Accuracy in Patients Referred for Myasthenic Ptosis,” Giannoccaro et al. compared the diagnostic accuracy of the ice pack test (IPT) with single-fiber EMG (SF-EMG) on the orbicularis oculi muscle in 155 patients, with ptosis being evaluated for ocular myasthenia defined as a positive response to the edrophonium test, presence of acetylcholine-receptor antibodies, a decrement of >10% of the third to fifth compound muscle action potential after repetitive nerve stimulation, or unequivocal response to oral steroids or acetylcholinesterase inhibitors for at least 3 months. They found that the IPT and SF-EMG had similar diagnostic accuracy in this patient population. Silvestri noted interest that (1) the IPT, a simple and cheap bedside assessment, is comparable with a complicated test like the SF-EMG; (2) the results of the IPT and SF-EMG were discordant for 10% of subjects (mostly in the setting of mild or isolated ptosis); and (3) the utility of the IPT was not evaluated in patients with isolated diplopia. Giannoccaro and Liguori responded that the discordance in results generally occurred in the setting of a negative IPT, which may be related to the lack of repetition of the test. They also cited previously published data that the IPT is useful in patients with isolated diplopia, particularly because SF-EMG may be negative in these patients.