Factors Affecting Generalization of Ocular Myasthenia Gravis in Palembang

Introduction. Ocular myasthenia gravis (OMG) is an autoimmune disease which is characterized by weakness of extraocular muscles, levator palpebrae and orbicularis oculi, resulting in ptosis and binocular diplopia. Nearly all patients present with eyelid and extra ocular muscles involvement. Approximately 30% to 80% of patients with OMG experience a conversion to generalized myasthenia gravis (GMG) within 2 years. There are not only have ptosis and diplopia but also limb weakness,bulbar symptoms, or even respiratory failure. This study was aimed to observe the clinical features of OMG to GMG and risk factors and median time to conversion of OMG to GMG of myasthenia gravis patients in Mohammad Hoesin General Hospital Palembang. Method. This study is a cohort retrospective study and the data were collected from the medical records of 91 patients who were registered as myasthenia gravis patients during September 2018 to March 2020. Sosiodemographic and clinical characteristics, including onset of OMG to GMG, history of smoking, presence of thymic abnormalities, and medications received were reviewed retrospectively. Result. A total of 91 OMG patients were observed in this study with 32 (35,2%) patients converted from ocular myastenia gravis to general myastenia gravis. Median conversion time to GMG was 34 months. Risk factor for convertion cases of OMG to MGG was receiving immunosupressive agents (Risk: 14.7, 95% CI 4.83, 44.7), thymus hyperplasia (Risk: 3.36, CI 95% 0.33, 33.6), Female (Risk: 2.41, 95% CI 0.94, 6.17), Smoking (Risk: 1.56, 95% CI 0.31, 7.81). Conclusion. Ptosis was the definitive sign for OMG in this study, with all patients had ptosis, thus it needs the colaboration from neuroophthalmologist and neurologist to diagnose and manage this case. Most of converted case was female and those who receive an immunosupressive agent therapy.

Predicting clinical outcomes using morphometric changes in adults with complex Chiari malformation undergoing occipitocervical fusion with or without ventral decompression: patient series

BACKGROUND The authors assessed the connection between clinical outcomes and morphometrics in patients with complex Chiari malformation (CM) who have undergone posterior fossa decompression (PFD) and subsequent occipitocervical fusion (OCF) with or without ventral decompression (VD). OBSERVATIONS The authors retrospectively reviewed 33 patients with CM aged over 21 years who underwent PFD and OCF with or without endoscopic endonasal odontoidectomy at the authors’ institution (21 OCF only and 12 OCF + VD). Clivoaxial angle (CXA), pB-C2 (perpendicular line to the line between the basion and C2), atlantodental interval (ADI), basion-dens interval (BDI), basion-axial interval (BAI), and C1 canal diameter were measured on preoperative and approximately 3-month postoperative computed tomography or magnetic resonance imaging scans. Common symptoms included headache, paresthesia, and bulbar symptoms. Clinical improvement after surgery was observed in 78.8% of patients. CXA, ADI, and BDI all significantly increased after surgery, whereas pB-C2 and BAI significantly decreased. OCF + VD had a significantly more acute CXA and longer pB-C2 preoperatively than OCF only. Patients who clinically improved postoperatively showed the same significant morphometric changes, but those who did not improve showed no significant morphometric changes. LESSONS Patients showing improvement had greater corrections in skull base morphometrics than those who did not. Although there are various mutually nonexclusive reasons why certain patients do not improve after surgery, smaller degrees of morphometric correction could play a role.

NCMP-19. RADIATION-INDUCED CRANIAL NEUROPATHY PRESENTING AS PROGRESSIVE BULBAR SYNDROME

Neuropathy is a well-known complication of radiation, especially when administered in proximity to the brachial plexus (BC), with symptoms usually appearing years after radiation. Pain is a less prominent feature when compared to neoplastic infiltration, with numbness and weakness being a more common and disabling complaint. Radiation-induced neuropathies tend to reach clinical stabilization after several months with por response to treatment. This is a case report of a 59 year-old Malaysian man who was diagnosed with nasopharyngeal carcinoma in 2002 treated with radiation to the nasopharynx in China. He has no evidence of disease to this date. In 2016 he started to develop progressive bulbar symptoms, such as hoarseness, hypophonia, and dysphagia. Symptoms worsened over the subsequent 18 months after presentation with clinical stabilization thereafter. Following the initial bulbar symptoms, he also developed bilateral hearing loss, atrophy of bilateral sternocleidomastoid muscles, significant tongue atrophy with fasciculations and tongue deviation. His clinical picture was consistent with bulbar amyotrophic lateral sclerosis. MRI brain and total spine did not show any intraparenchymal or nerve disease. An electromyography test was performed, which showed myokymia of the tongue and trapezius consistent with radiation-induced cranial neuropathy (CN). He received steroids without any clinical improvement, followed by hyperbaric oxygen with minimal improvement of his dysphagia, hoarseness and hypophonia. On last follow up his symptoms continued to be stable. Radiation to nasopharynx can cause radiation-induced CN with similar electromyographic findings to radiation-induced brachial plexopathy, such as myokymia. Symptoms can appear more than 10 years from radiotherapy, with progression over several months followed by clinical stabilization. Treatment with steroids, bevacizumab or hyperbaric oxygen only provide minimal benefit on this population.

Neurological manifestations of polyarteritis nodosa: a tour of the neuroaxis by case series

Background Heterogenous central nervous system (CNS) neurologic manifestations of polyarteritis nodosa (PAN) are underrecognized. We review three cases of patients with PAN that illustrate a range of nervous system pathology, including the classical mononeuritis multiplex as well as uncommon brain and spinal cord vascular manifestations. Case presentation Case 1 presented with mononeuritis multiplex and characteristic skin findings. Case 2 presented with thunderclap headache and myelopathy due to spinal artery aneurysm rupture. Both patients experienced disease remission upon treatment. Case 3 presented with headache and bulbar symptoms due to partially thrombosed intracranial aneurysms, followed by systemic manifestations related to visceral aneurysms. She demonstrated clinical improvement with treatment, was lost to follow-up, then clinically deteriorated and entered hospice care. Conclusions Although the peripheral manifestations of PAN are well-known, PAN association with CNS neurovascular disease is relatively underappreciated. Clinician awareness of the spectrum of neurologic disease is required to reduce diagnostic delay and promote prompt diagnosis and treatment with immunosuppressants.

Factors Affecting Generalization of Ocular Myasthenia Gravis in Palembang

Introduction. Ocular myasthenia gravis (OMG) is an autoimmune disease which is characterized by weakness of extraocular muscles, levator palpebrae and orbicularis oculi, resulting in ptosis and binocular diplopia. Nearly all patients present with eyelid and extra ocular muscles involvement. Approximately 30% to 80% of patients with OMG experience a conversion to generalized myasthenia gravis (GMG) within 2 years. There are not only have ptosis and diplopia but also limb weakness,bulbar symptoms, or even respiratory failure. Objective. To observe the clinical features of OMG to GMG and risk factors and median time to conversion of OMG to GMG of myasthenia gravis patients in Mohammad Hoesin General Hospital Palembang. Method. This study is a cohort retrospective study and the data were collected from the medical records of 91 patients who were registered as myasthenia gravis patients during September 2018 to March 2020. Sosiodemographic and clinical characteristics, including onset of OMG to GMG, history of smoking, presence of thymic abnormalities, and medications received were reviewed retrospectively. Result. A total of 91 OMG patients were observed in this study with 32 (35,2%) patients converted from ocular myastenia gravis to general myastenia gravis. Median conversion time to GMG was 34 months. Risk factor for convertion cases of OMG to MGG was receiving immunosupressive agents(Risk: 14.7, 95% CI 4.83, 44.7), thymus hyperplasia (Risk: 3.36, CI 95% 0.33, 33.6), Female (Risk: 2.41, 95% CI 0.94, 6.17), Smoking (Risk: 1.56, 95% CI 0.31, 7.81). Conclusion. Ptosis was the definitive sign for OMG in this study, with all patients had ptosis, thus it needs the colaboration from neuroophthalmologist and neurologist to diagnose and manage this case. Most of converted case was female and those who receive an immunosupressive agent therapy.

Late-onset double-seronegative myasthenia gravis syndrome and myasthenic crisis due to nivolumab use for Hodgkin’s lymphoma

Introduction Insofar, use of programmed cell death-1 (PD-1) immune checkpoint inhibitors in oncology has been linked with several immune-mediated neurologic effects. However, grade 3 to 4 adverse events such as myasthenic crisis have been vanishingly rare. Case presentation: We present herein a unique patient with Hodgkin lymphoma who developed late-onset double-seronegative myasthenia gravis syndrome followed by myasthenic crisis after 16 weeks of therapy with nivolumab. One day prior to this event, she developed ptosis, diplopia, bulbar symptoms of dysphagia, dysarthria, orthopnea as well as extremity weakness. She required intubation, mechanical ventilation, plasmapheresis and steroid therapy. Management and outcome: She gradually achieved a near-complete resolution of neurologic symptoms over the next several weeks. On a follow-up visit eight weeks later, she only has some residual diplopia. Restaging scans showed a continued decrease in size of the mediastinal mass, without abnormal uptake. She remains on prednisone 10 mg orally daily. Discussion Prompt recognition of this rare phenomenon, immediate discontinuation of checkpoint inhibitor therapy and subsequent management with immunosuppressive therapy are necessary steps in order to minimize the considerable rates of morbidity and mortality.

Long Term Follow-Up on Pediatric Cases With Congenital Myasthenic Syndromes—A Retrospective Single Centre Cohort Study

Introduction: Congenital myasthenic syndromes (CMS) refer to a heterogenic group of neuromuscular transmission disorders. CMS-subtypes are diverse regarding exercise intolerance and muscular weakness, varying from mild symptoms to life-limiting forms with neonatal onset. Long-term follow-up studies on disease progression and treatment-response in pediatric patients are rare.Patients and Methods: We analyzed retrospective clinical and medication data in a cohort of 32 CMS-patients including the application of a standardized, not yet validated test (CMS-ST) to examine muscular strength and endurance in 21 patients at the last follow-up. Findings obtained in our cohort were compared with long-term follow-up studies of (adult) CMS-cohorts from the literature by considering the underlying molecular mechanisms. Outcomes of CMS-ST were compared to results of normal clinical assessment.Results: Thirty-two pediatric patients with defects in eight different CMS-genes were followed by a median time of 12.8 years. Fifty-nine percentage of patients manifested with first symptoms as neonates, 35% as infants. While 53% of patients presented a reduced walking distance, 34% were wheelchair-bound. Even under adequate therapy with pyridostigmine (PS) and 3,4-diaminopyridine, CHAT-mutations led to the progression of muscular weakness partly in combination with persistent respiratory and bulbar symptoms. RAPSN, CHRND, and CHRNB1 patients with neonatal manifestation, early respiratory problems, and bulbar symptoms showed a good and maintained treatment response. CHAT and CHRNE patients required higher PS dosages, whereas RAPSN patients needed a lower mean dosage at the last follow-up. The benefits of short-term medication and long-term progression of symptoms were highly dependent on the specific genetic defect. CMS-ST was carried out in 17/21 patients, determined affected muscle groups including bulbar and ocular symptoms, some of which were not reported by the patients.Conclusions: Our findings and comparison with the literature- suggest a better treatment-response and less severe progression of symptoms present in patients suffering from mutations in CMS-genes directly associated with receptor deficiency, while patients with defects leading to synaptopathy and presynaptic defects tend to have worse outcomes. Assessment of affected muscular groups and clinical symptoms by CMS-ST may be a useful tool for optimal therapeutic management of the patients, especially for future clinical studies.