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Myasthenia Gravis: MG-epidemiology

This document discusses myasthenia gravis (MG), an autoimmune neuromuscular disease caused by antibodies against acetylcholine receptors. It defines MG, describes its typical symptoms of progressive muscle weakness, and notes that it often begins in the eye muscles and soft palate. The document also summarizes MG's epidemiology, pathogenesis, diagnostic testing including repetitive nerve stimulation electromyography, treatment typically involving immunosuppression and thymectomy, and compares it to the related condition Lambert-Eaton myasthenic syndrome.

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Remigius Riyan
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62 views3 pages

Myasthenia Gravis: MG-epidemiology

This document discusses myasthenia gravis (MG), an autoimmune neuromuscular disease caused by antibodies against acetylcholine receptors. It defines MG, describes its typical symptoms of progressive muscle weakness, and notes that it often begins in the eye muscles and soft palate. The document also summarizes MG's epidemiology, pathogenesis, diagnostic testing including repetitive nerve stimulation electromyography, treatment typically involving immunosuppression and thymectomy, and compares it to the related condition Lambert-Eaton myasthenic syndrome.

Uploaded by

Remigius Riyan
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
Available Formats
Download as PDF, TXT or read online on Scribd
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24.11.

2016

Myasthenia gravis Definition: autoimmune disease with circulating antibodies


against Ach-R (Acetylcholine Receptor), or other antigens
of postsynaptic membrane (titin, MUsK-enzyme,...)

Clinical symptoms: progredient muscle weakness during


daily activities, with evening acces, repair after rest time
Onset: small muscles – eye (diplopia), pharynx
(dysphagia), soft palate (rhinolalia), general ...

Acquired autoimmune disease


MG- epidemiology
Ab+Ach-Receptor => functional
Incidence: 14,8 / mil. inhabitants block and destruction=> decrease
postsynaptic actional potential =>
• Prevalence: 191 /mil. inhab. /SR 1.1.2007/ insuficient muscle contraction =>
muscle weakness

Disease onset: mostly: 30. year (F), 60.-70. year (M)


Sex rate F:M= 1,7:1

No hereditary cases, familial increased


susceptibility for autoimmune disease (HLA)

MG- manifestation
MG- disease course
Clinical symptoms: progredient muscle weakness Subjective: abnormal muscle fatigue, weakness - paresis,
during daily activities, with evening acces, repair after recovery after resting
rest time
Objective:
Onset: small muscles – eye (diplopia), pharynx repetitive muscle activity provocates weakness- ptosis,
(dysphagia), soft palate (rhinolalia), general ... diplopia, rhinolalia, dysphagia, dysarthria, dysphonia
weak of jawing, mimic paresis
Thymus abnormalities - ¾ of MG patients neck dicrease
85% - hyperplasia short breathing
15% - thymoma (benign / malign) tendon reflexes- presented or slight decrease

1
24.11.2016

MGFA (Foundation of America)- Clinical


symptoms scaling MG- diagnosis
(Osserman´s classification) 1. History
2. EMG
I. Ocular form MG
3. Lab: serum antibodies anti-Ach-R (75% positivity)
II. Ocular + slight generalised MG (limbs /bulbar)
4. Clinical tests: Simpson´s test- vertical gaze
III: Moderate weakness of ocular + extraocular Seeman´s test- dysarthria
muscles (limbs, respiratory, bulbar muscles)
Gorelick´s test
IV: Severe weakness of ocular + extraocular muscles pharmacol. Tensilon test: iv.
V: Respiratory failure, suported ventilation amp. inhib. AchE
5. X-rays chest, mediastinal CT, MRI

Static and dynamic (repetitive) tests - Gorelick´s sign – bilateral asymetrical ptosis: patient is looking
up, finger elevation of eyelid opposite side, on contralateral side
- Demasking of latent MG or enhancing of present muscle wa can see total decrease
weakness
- (pathognomic for MG)
- Simpson´s test – slight ptosis – patient is looking upward 1
minute- more severe ptosis

Thymoma

2
24.11.2016

MG- EMG MG- therapy


• EMG - repetitive stimulation, low freq.stimulation - 3Hz Currently - no deaths, previously - 30% mortality
Abnormality: gradual decrement of response amplitude
1. Pharmacological: IS + symptomatic th
min.15%, max. in the 2.- 4. response, normalization of EMG
after Tensilone inj. 2. Surgical – thymectomy

Pharmacological therapy:
• Immunosupression: Prednison, Azathioprine,Cyclosporine A
• Plasma exchange/ or IVIG
• inhib.ChE (Pyridostigmine, Mestinon)

LEMS, Lambert- Eaton myasthenic syndrom


• is a rare autoimmune disorder
LEMS
• muscle weakness of the limb
High frequency repetitive
• Antibodies against presynaptic voltage-gated EMG (30 Hz) => gradual
calcium channels, and likely other nerve terminal
proteins increase of AP amplitude
• Prevalence: 3.4 cases/million
• Around 60% of LEMS -have an underlying
malignancy (small cell lung cancer) Therapy:
• paraneoplastic syndrome
steroids, Azathioprine,
• KP: fatigue, weakness of proximal mm., inferior plasma exchange
extremities, spared eye and bulbar mm.
• autonomic difficulties: dry mouth, low
lacrimation, orthostatic collapses, impotentia

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