What Part Of The Brain Does Myasthenia Gravis Affect – An 85-year-old man presented to the hospital as an emergency, having difficulty swallowing and speaking. In the emergency department, he was assessed as having acute dysphagia and dysarthria consistent with an acute stroke. It subsequently became apparent that although the symptoms were indeed of relatively acute onset, there was a clear patient description of fatigue and diurnal variation, leading to a clinical working diagnosis of myasthenia gravis. The patient had a turbulent clinical course, and the interpretation of the investigation results proved not to be straightforward in the acute setting. Myasthenia gravis is an uncommon disorder, but it is more common in the elderly. This case provides key learning points, particularly emphasizing the value of prompt and accurate clinical assessment and the importance of adherence to the formulation of the clinical diagnosis.

An 85-year-old man presented to the hospital as an emergency, having difficulty swallowing and speaking. In the emergency department, he was assessed as having acute dysphagia and dysarthria consistent with an acute stroke. He first developed symptoms 3 days earlier. No other neurological deficit was recorded. The general examination was otherwise unremarkable. He had no other significant medical history. A chest x-ray showed only a small pleural effusion on the right side. He was transferred to the stroke unit and underwent a brain magnetic resonance (MRI) scan urgently (Fig 1). MRI (including a negative diffusion imaging study) showed diffuse high signal white matter changes consistent with cerebral small vessel disease, with no evidence of acute infarction in the anterior or posterior circulation territories.

What Part Of The Brain Does Myasthenia Gravis Affect

What Part Of The Brain Does Myasthenia Gravis Affect

Axial T2-weighted magnetic resonance imaging showing multiple periventricular foci and high-signal deep white matter with early confluence.

A Case Study About Patients With Myasthenia Gravis Improved With Thc A

The next day (4 days after symptom onset), the patient was seen at the stroke unit by a consultant neurologist (HE). Although the dysphagia was indeed of relatively acute onset, the patient clearly described fatigue and diurnal variation. He described dysphagia of only a few days duration, which had clearly worsened in the 24 to 48 hours prior to admission. He noted that difficulty eating was more evident towards the end of the day and that he had particular difficulty during a meal while eating a meat pie. He had found that swallowing was easier again when he had breakfast the next morning. Similarly, he commented that his family had noticed in recent days that his speech was becoming increasingly slurred the more he spoke. Neurological examination revealed no additional features: there was no fatiguing weakness of extraocular movements and no limb fatigue. The initial working neurological diagnosis was bulbar myasthenia gravis (MG). Antibodies to acetylcholine receptors were requested.

Against a diagnosis of stroke, which is a clinical syndrome characterized by sudden-onset focal neurologic deficit(s), is the fact that symptoms of dysphagia and dysarthria showed fatigue and diurnal variation, clinical features that are considered hallmarks of MG. On clinical grounds, this was the most likely diagnosis.

Bulbar involvement can occur early in Guillain-Barré syndrome (GBS) – especially the pharyngeal-cervical-brachial variant, so this had to be considered as a differential diagnosis as well. An apparently acute presentation of bulbar or pseudobulbar insufficiency may also be seen when clinical manifestations of motor neuron disease are delayed, but there were no other features on examination to support this diagnosis.

Structural lesions of the brainstem (such as syringobulbia) have already been ruled out by the MRI scan of the brain. Lambert-Eaton myasthenic syndrome (LEMS), despite its name, is not usually characterized by fatigue and does not often produce dysarthria. Therefore, LEMS was not considered to be a likely diagnosis, and further investigation results also suggested that this was not a case of LEMS.

Pseudoabducens Palsy Mimicking Myasthenia Gravis

The patient was kept sedated orally pending electromyography (EMG). A 10-French nasogastric (NG) tube was passed at 56 cm, with aspirated pH 3.0. NG feeding was started. Overnight, it got shorter and shorter. He had a sinus tachycardia rate of 124 bpm, bilateral coarse breath sounds, and a decrease in forced vital capacity (FVC) to 1.04 L from 2.16 L 3 hours earlier. A 12-lead electrocardiogram showed ST-segment depression in leads V3 through V5. Troponin T was elevated to 42 ng/l (normal range 0–14 ng/l), although this decreased to 33 ng/l over the next 8 hours, the elevation probably reflecting tachycardia rather than myocardial infarction.

The patient maintained oxygen saturations at 96% on air and FVC stabilized, but persistent tachycardia and tachypnea (respiratory rate 24/minute) prompted CT pulmonary angiography (CTPA) to rule out pulmonary embolism. Unfortunately, he quickly developed a combination of decreased oxygen saturations (down to 88% of room air) and increased oxygen dependence, drowsiness, and the development of rapid atrial fibrillation. This prompted admission to the intensive care unit, where he required ventilatory support. It has become too bad to tolerate a CTPA. Chest radiographs suggested right basal consolidation consistent with suspected aspiration of NG feed. At this time, given the presence of several comorbidities, a poor prognosis was communicated to the patient’s family. He was treated with iv gentamicin, meropenem, verapamil and amiodarone and later underwent tracheostomy.

Subsequent neurologic examination was difficult given the patient’s condition and intensive care unit setting, but he was found to have become areflexic with bilateral facial weakness. Given the nature of his impairment and the additional possibility of autonomic instability (tachycardia), it was important to rule out GBS. EMG and nerve conduction studies (5 days after symptom onset) were also very challenging. Repetitive nerve stimulation did not show a consistent decrease, so the findings were insufficient to diagnose MG. There was no evidence to support GBS. Active neuropathic changes involving the L4, L5, S1, and S2 innervated muscles on the right side have been suggested to be the result of a proximal cause such as acute lumbosacral radiculopathy. The patient underwent a lumbar puncture, which revealed an increase in cerebrospinal fluid (CSF) protein (1.24 g/l), but otherwise normal constituents. On clinical grounds, prominent fatigue favored MG, but the finding of CSF cytoalbuminological dissociation clearly raised the possibility of GBS.

What Part Of The Brain Does Myasthenia Gravis Affect

The patient received treatment with intravenous immunoglobulin (total dose 140 g for 5 days) and showed gradual improvement. Acetylcholine receptor antibodies were found to be strongly positive (59.5 nmol/l). Antiglycolipid antibodies were negative. A repeat neurophysiological examination (17 days after symptom onset) revealed peripheral sensory studies to be within acceptable limits of normal in the upper and lower limbs. Repetitive nerve stimulation showed a significant decrease (10 to 17%) when stimulating the right median nerve and right facial nerve, supporting generalized MG. Again, supportive evidence was provided for lumbosacral radiculopathy. An MRI of the lumbosacral spine showed diffuse degenerative changes but only mild canal stenosis at the L4/L5 level.

Ocular Myasthenia Gravis

Currently, 20 months after initial presentation, the patient remains under outpatient follow-up and continues to have a favorable clinical course with mycophenolate mofetil and pyridostigmine.

The prevalence of MG has recently been estimated at 131–145 per million population, with incidence rates of 6.7 per million in those younger than 50 years and 34 per million in those over 50 years of age.1 Diagnosis of MG is based on clinical and paraclinical findings, especially symptoms, the effect of acetylcholinesterase inhibitors, EMG, and the presence of autoantibodies or thymoma. Diagnostic and clinical classification has been discussed with expertise very recently.2

Although MG is an uncommon disorder, it is increasingly common in an aging population. In the acute care setting, a variety of acute-onset neurologic symptoms are not infrequently initially attributed to stroke. Therefore, it is crucial to be aware of the diversity of conditions that can cause apparently acute or subacute neurological dysfunction. Anecdotally, as a neurologist attending a stroke unit serving a population of approximately 380,000, one of the authors (HE) encountered new-onset MG presenting to the stroke unit approximately once a year.

There were a number of complexities in this case, not least the difficulty posed by the rapid deterioration of the patient in establishing the diagnosis as quickly as possible. In terms of confirming the clinical suspicion of MG, EMG is difficult to undertake and interpret in the context of an acutely ill patient; Acetylcholine receptor antibody testing is generally subject to delay resulting from sample lotting by clinical laboratories; and the utility and safety of a tensilon test is questionable in an elderly patient who has a degree of hemodynamic instability. Elevated CSF protein was a confounding factor, but was probably attributable to a combination of lumbosacral spine disease3 and cerebral small vessel disease leading to loss of blood-brain barrier integrity.4 An alternative possibility, the co-occurrence of MG and GBS ( which has been described5), was considered to be an extremely unlikely explanation for the elevated CSF protein. GBS was not supported by the neurophysiological findings in our patient, whereas a significant decrease, sufficient to meet the diagnostic criteria for MG, was observed on repetitive nerve stimulation.

Myasthenia Gravis: Lebih Dari 208 Vektor Stok & Seni Vektor Yang Dapat Dibeli Lisensinya Tanpa Royalti

This case reaffirms the value of prompt, accurate, neurological assessment and adherence to the formulation of the clinical diagnosis. This is particularly important in the acute setting where the interpretation of investigational results is not straightforward. Like celiac disease (CD), non-celiac gluten sensitivity (NCGS) has also been associated with several autoimmunities.

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