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AbstractMyasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG), affecting 15–20% of patients with MG at least once. The rapid and accurate diagnosis of MG is mandatory but challenging because patients with MC require intubation and mechanical ventilation or noninvasive ventilation (NIV). We describe a case of an 81-year-old woman diagnosed with MG related dysphagia. Simultaneous fiberoptic endoscopic examination of swallowing (FEES) and ice-pack test (FEES-ice pack test) revealed a significant decrease in pharyngeal residue, suggesting MG. The day following the test, she experienced respiratory failure. FEES-ice pack test results and respiratory distress suggested MC. NIV followed by mechanical ventilation was started quickly, leading to improved respiratory condition. We show that the FEES-ice pack test led to prompt treatment for MC. Although further investigations are required for sensitivity, specificity, and positive criteria, the FEES-ice pack test could be a safe and easy method to diagnose MG.

Myasthenic crisis (MC) is a life-threatening complication of myasthenia gravis (MG), affecting 15–20% of patients with MG at least once. The rapid and accurate diagnosis of MG is mandatory but challenging because patients with MC require intubation and mechanical ventilation or noninvasive ventilation (NIV). We describe a case of an 81-year-old woman diagnosed with MG related dysphagia. Simultaneous fiberoptic endoscopic examination of swallowing (FEES) and ice-pack test (FEES-ice pack test) revealed a significant decrease in pharyngeal residue, suggesting MG. The day following the test, she experienced respiratory failure. FEES-ice pack test results and respiratory distress suggested MC. NIV followed by mechanical ventilation was started quickly, leading to improved respiratory condition. We show that the FEES-ice pack test led to prompt treatment for MC. Although further investigations are required for sensitivity, specificity, and positive criteria, the FEES-ice pack test could be a safe and easy method to diagnose MG. [ABSTRACT FROM AUTHOR]

A rare clinical presentation of ocular myasthenia gravis (OMG) with thyroid disorders, has been reported and documented in the past. There is similarity in the presentation of patients with OMG and thyroid opthalmopathy as a complication of thyrotoxicosis. The tendency of myasthenia gravis to get overlooked as separate entity despite having thyrotoxic presentation con-comittantly necessitates knowledge, thorough examination, and an experienced approach, to aid in its diagnosis. We have discussed two cases in a thyrotoxic state, with symptoms of ocular myasthenia gravis and clinical approach in our setting. [ABSTRACT FROM AUTHOR]

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.

A 32-year-old man presented with complete third nerve palsy. Brain magnetic resonance imaging revealed a possible sporadic oculomotor nerve schwannoma in the left cavernous sinus. Interestingly, the patient’s ptosis was fluctuating and eased immediately after application of ice over his eyelid. The ice pack test (IPT) is a simple and fast method that can help diagnose myasthenia gravis (MG) in patients with ptosis. Additional diagnostic investigations (antibody assays and repetitive stimulation) excluded associated MG in the patient. Tumor treatment with Gamma Knife radiosurgery was planned. This case highlights the finding that IPT can also be positive in neurogenic (non-myasthenic) ptosis, but its usefulness in other disorders associated with muscle weakness and fatigability remains questionable.

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. [ABSTRACT FROM AUTHOR]

A 32-year-old man presented with complete third nerve palsy. Brain magnetic resonance imaging revealed a possible sporadic oculomotor nerve schwannoma in the left cavernous sinus. Interestingly, the patient's ptosis was fluctuating and eased immediately after application of ice over his eyelid. The ice pack test (IPT) is a simple and fast method that can help diagnose myasthenia gravis (MG) in patients with ptosis. Additional diagnostic investigations (antibody assays and repetitive stimulation) excluded associated MG in the patient. Tumor treatment with Gamma Knife radiosurgery was planned. This case highlights the finding that IPT can also be positive in neurogenic (non-myasthenic) ptosis, but its usefulness in other disorders associated with muscle weakness and fatigability remains questionable. [ABSTRACT FROM AUTHOR]

Documented cases of ipsilateral ptosis caused by midbrain infarction remain rare. Herein, we present a patient with isolated ipsilateral ptosis that was initially considered to be a consequence of myasthenia gravis but was subsequently attributed to ventral midbrain infarction. We also discuss the possible underlying mechanisms; ipsilateral ptosis in our patient was attributed to selective damage of the levator palpebral muscle branch of the oculomotor nerve. The patient was started on aspirin (200 mg once daily) and atorvastatin (40 mg once daily). Improvement in ptosis occurred from day 5 of admission, and the patient was subsequently discharged. Ptosis disappeared 1 month after onset. This report describes an extremely rare case of ventral midbrain infarction presenting with isolated ipsilateral ptosis. Careful examination, including magnetic resonance imaging, is essential in such patients, especially in those with multiple cerebrovascular risk factors., Competing Interests: Declaration of conflicting interestsThe authors declare that there is no conflict of interest.

Introduction: Myasthenia Gravis (MG) with ocular involvement has an elusive diagnosis. Ptosis and Diplopia are the most common and early presenting features of MG. Acetylcholine receptor (AChR) antibody titre, Nerve conduction studies and chest imaging are important diagnostic tests for MG. A simple test like an ice pack test can help in differentiating MG from other causes of neurological ptosis and other symptoms. Aim: To study the systemic presentation, the profile of ocular involvement, clinical and laboratory tests and treatment modalities used in patients diagnosed with MG in a tertiary hospital. Materials and Methods: This was a hospital based five year retrospective study of 14 cases of MG, diagnosed and treated between January 2012 and December 2016. Analysis of presenting signs and symptoms of generalised and ocular MG was done. Profile of diagnostic clinical, laboratory, and radiological tests performed for each patient and their results were noted and analysed. Various treatment modalities used to manage the cases were noted. Permission from the Institutional Ethics committee was taken prior to starting the study. SPSS software version 23 was used for data analysis. Results: Mean age of presentation was found to be 40.2 years with a predilection for male gender; 81.71% of patients, who presented with Generalised MG, 14.2% had ocular involvement. Ptosis was found to be the most common ocular symptom in combination with diplopia (SD-0.554). Lateral rectus was most commonly involved muscle. Ice pack test, nerve conduction studies were specific in diagnosis of MG. Antibody titres were positive in 66.6% patients. About 30% patients underwent HRCT chest out of which 60% showed some Thymus involvement. Various forms of treatment were used including oral Pyridostigmine, oral steroids and oral immunosuppressants. Conclusion: The pattern of involvement in MG in this study was slightly different. The mean age was lower, there was preponderance of male gender and lateral rectus muscle was most commonly involved muscle. A simple ice pack test is cheap and reliable test to diagnose MG with ocular involvement. We suggest HRCT to rule out thymic abnormalities.

Introduction: Myasthenia Gravis (MG) with ocular involvement has an elusive diagnosis. Ptosis and Diplopia are the most common and early presenting features of MG. Acetylcholine receptor (AChR) antibody titre, Nerve conduction studies and chest imaging are important diagnostic tests for MG. A simple test like an ice pack test can help in differentiating MG from other causes of neurological ptosis and other symptoms. Aim: To study the systemic presentation, the profile of ocular involvement, clinical and laboratory tests and treatment modalities used in patients diagnosed with MG in a tertiary hospital. Materials and Methods: This was a hospital based five year retrospective study of 14 cases of MG, diagnosed and treated between January 2012 and December 2016. Analysis of presenting signs and symptoms of generalised and ocular MG was done. Profile of diagnostic clinical, laboratory, and radiological tests performed for each patient and their results were noted and analysed. Various treatment modalities used to manage the cases were noted. Permission from the Institutional Ethics committee was taken prior to starting the study. SPSS software version 23 was used for data analysis. Results: Mean age of presentation was found to be 40.2 years with a predilection for male gender; 81.71% of patients, who presented with Generalised MG, 14.2% had ocular involvement. Ptosis was found to be the most common ocular symptom in combination with diplopia (SD-0.554). Lateral rectus was most commonly involved muscle. Ice pack test, nerve conduction studies were specific in diagnosis of MG. Antibody titres were positive in 66.6% patients. About 30% patients underwent HRCT chest out of which 60% showed some Thymus involvement. Various forms of treatment were used including oral Pyridostigmine, oral steroids and oral immunosuppressants. Conclusion: The pattern of involvement in MG in this study was slightly different. The mean age was lower, there was preponderance of male gender and lateral rectus muscle was most commonly involved muscle. A simple ice pack test is cheap and reliable test to diagnose MG with ocular involvement. We suggest HRCT to rule out thymic abnormalities. [ABSTRACT FROM AUTHOR]

Background: Diagnosis of myasthenia gravis is difficult, especially when the disease is restricted to ocular muscles. It is a well-known observation that myasthenic ptosis improves with cold and based on this, the ice pack test has been used as a tool in its differential diagnosis. The aim of the study is to investigate the diagnostic value of ice pack test as a preliminary test in the differential diagnosis of myasthenia and to find out the percentage of positivity and negativity of the ice pack test.Materials and Methods: The study included patients seeking treatment for ptosis in a tertiary care center in Kerala. All patients were subjected to the ice pack test. Evaluation of patients was made by recording the marginal reflex distance (MRD). MRD was recorded before and after the application of ice pack, and an improvement of more than 2 mm was considered positive. Patients were divided into two groups: (1) those who had ptosis and features suggestive of myasthenia; and, (2) those who had ptosis but no features of myasthenia. Patients of both groups were subjected to relevant investigations and the diagnosis of myasthenia was established.Results: Ice cold test was positive in 48 (96%) patients and negative in two patients of the myasthenia group. In the control group, 6 (12%) patients had a false positive ice cold test and in the remaining 44 patients (88%), ice cold test was negative.Conclusion: Ice pack test has a high specificity and sensitivity in the differential diagnosis of myasthenic ptosis. [ABSTRACT FROM AUTHOR]

ABSTRACT Introduction: Several studies have reported high diagnostic sensitivity and specificity for the ice test in myasthenia gravis. All of the studies employed a case-control design, in which the diagnosis was already known at the time of the test for both patients and controls, leading to case selection bias. This suggests that the available literature substantially overestimates the diagnostic utility of these tests. Methods: A retrospective cohort study without selection bias was performed to examine the sensitivity and specificity of the ice test. The sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of the ice test were determined by means of a 2 × 2 table. Results: The ice test has a sensitivity of 0.92 (95% CI 0.62-1.00), specificity of 0.79 (95% CI 0.56-1.00), PPV of 0.73 (95% CI 0.48-0.90), and NPV of 0.94 (95% CI 0.70-1.00). Conclusions: Due to its high negative predictive value the ice test is still a reliable and useful bed-side test. Muscle Nerve 48: 902-904, 2013 [ABSTRACT FROM AUTHOR]

Myasthenia gravis is the most common disorder of neuromuscular transmission. It is the "great imitator," masquerading as any internuclear or supranuclear dysfunction of ocular motility. Ophthalmologists and orthoptists are frequently the first medical practitioners to identify the disease because of the high incidence of ptosis and strabismus as presenting signs. Accurate diagnosis is imperative so that appropriate medical management may be initiated. [ABSTRACT FROM AUTHOR]

In the evaluation of ptosis, the ice pack test is a simple, quick, bedside method to ascertain whether it is of myasthenic origin, which requires no specialised medication or equipment and is free of reported adverse effects. Pooling of published studies suggests very high sensitivity, specificity and likelihood ratios, but standardisation and operationalisation of the test are important. False-negative tests may occasionally occur. [ABSTRACT FROM AUTHOR]