University of Khartoum

Myasthenia Gravis presentation with reference to Sudan

Myasthenia Gravis presentation with reference to Sudan

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Title: Myasthenia Gravis presentation with reference to Sudan
Author: Ahmed, Ammar EM; Musa, Afraa MM
Abstract: Myasthenia gravis (MG) is an acquired autoimmune disorder characterized clinically by weakness of skeletal muscles and fatigability on exertion. Thomas Willis reported the first clinical description in 1672. Myasthenia gravis presents at any age. Female incidence peaks in the third decade of life, whereas male incidence peaks in the sixth or seventh decade. Myasthenia gravis is characterized by fluctuating weakness increased by exertion. Weakness increases during the day and improves with rest. Extraocular muscle (EOM) weakness or ptosis is present initially in 50% of patients and occurs during the course of illness in 90%. Bulbar muscle weakness is also common, along with weakness of head extension and flexion. Weakness may involve limb musculature with proximal weakness greater than distal muscle weakness. Isolated limb muscle weakness as the presenting symptom is rare and occurs in fewer than 10% of patients. Patients progress from mild to more severe disease over weeks to months. Weakness tends to spread from the ocular to facial to bulbar muscles and then to truncal and limb muscles. On the other hand, symptoms may remain limited to the EOM and eyelid muscles for years. Rarely, patients with severe, generalized weakness may not have associated ocular muscle weakness. The disease remains ocular in only 16% of patients. About 87% of patients generalize within 13 months after onset. In patients with generalized disease, the interval from onset to maximal weakness is less than 36 months in 83% of patients. Intercurrent illness or medication can exacerbate weakness, quickly precipitating a myasthenic crisis and rapid respiratory compromise. Spontaneous remissions are rare. Long and complete remissions are even less common. Most remissions with treatment occur during the first 3 years of disease. The Medical Scientific Advisory Board (MSAB) of the Myasthenia Gravis Foundation of America (MGFA) formed a Task Force in May 1997 to address the need for universally accepted classifications, grading systems, and methods of analysis for patients undergoing therapy and for use in therapeutic research trials. Thus, MGFA Clinical Classification was created. On examination: weakness can be present in a variety of different muscles and is usually proximal and symmetric. Sensory examination and deep tendon reflexes are normal. For investigations: [data from worldwide and Sudan will be compared and reviewed]. Methods of investigations include: immunology, electrophysiology [Repetitive nerve stimulation & single fibre electromyography], chest radiograph & CT scan. Some rare presentations of myasthenia in Sudanese patients will be described with treatment follow up.
URI: http://khartoumspace.uofk.edu/handle/123456789/19626
Date: 2015


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