Myasthenia Gravis and GBS

Dr. Germie Anne Navalta

Sept 24, 2020, 2pm

Take off case: RN 64/F from Iloilo, prev admitted 2015 with weakness at the end of the day, with difficulty in ambulation, drooping eyelids,
difficulty swallowing, dysarthria. Admitted at ICU. IVIG and Prednisone given. RNS done showed decreased.
PMHx- Hypertensive
2013- Myasthenia Gravis- Feb 2013, pyridostigmine 60mg
Non smoker, occasional alcoholic beverage- red wine
FHx- HTN, CVD- father
ROS: Weight gain, muscle cramps
Physical Exam- unremarkable
Neuro Exam- Normal motor and sensory

Assessment: MG, HTN Controlled

Plan: Referred to neuro
Maintained hypertensives.

Discussion:
MG is the rag doll illness because it causes muscle weakness. Fluctuating weakness and fatigability of voluntary muscles (ocular, bulbar, neck,
respitatory)
No gender predilection, but affects women under 40 and men over 60.

Myasthenia Gravis, predominantly diagnosed in females.

Mechanism:
Autoantibody against acetylcholine receptors

Mediastinal masses (thymoma) related to MG

Most commonly, the miost common affected musles are the ocular muscles which have a few number of acetylcholine receptors

Symptoms: Weakness of eye muscle, difficulty in swallowing

Classification

Class 1
-occular muscle weakness
Class 2
-Mild weakness
Class 2a- axial muscles, limbs
Class 2b- oropharyngeal and respiratory
Class 3
-moderate weakness
3a, 3b
Class 4- severe weakness
4a, 4b
Class V- defined by intubation with or without mechanical vantilation except when used during routine postoperative management
Use of a feeding tube without intubation

Diagnosis: By history and PELabs- Presence of anti ACHR or Anti MUSK

EMG NCV- 2-3Hz nerve stimulation with decrement

-Very clear positive response to immunosuppressive treatment and or plasma exchange or IVIG
-Unequivocally positive Tensilon or neostigmine test

A decrement of 10hz in repetitive stimulation tests may be diagnostic

Treatment
Anti cholinesterase drug
Chronic immunotherapies
Rapid immunotherapies
Thymectomy

Anticholinesterase
Pyridostigmine- 1st line drug in patients with MG (up to 60 mg five times a day)
Steroids, pulse methylprednisone
Immunosuppressives, azathiprine, mycophenolate, cyclosporine, cyclophosphamide
Plasma exchange- there are studies that highly recommend
IVIG

Edrophonium test- not drecommended

Myasthenic crisis- myridiasis, diarrhea, tachycardia, normal secretions

Cholinergic crisis, hypotension, abdominal pain, symptoms improve by giving anticholinergics

Management ( Thymectomy)- may be done. Work up in all suspected patients withmyasthenia gravis should include thymoma work up ,
documented via imaging

GUILLAIN BARRE SYNDROME

This is a rare disorder, usually affecting peripheral nervous system. The exact cause is usually unknown. However GBS has been related to C.
Jejuni, and has ascending pattern of muscle weakness
GI Infection— Diarrhea
Respiratory— Influenza, EBV, Zika
Hepatitis
Mycoplasma infections
COVID-19 infection

Symptoms:
First two weeks after symptom onset—> Greatest stage of weakness
Difficulty with eye muscles and vision
Pharyngeal weakness and symptoms
Neuropathic sensations

Types of GBS
AIDP- most common is muscle weakness

MFS- associated with unsteady gait. Common in asia. Ocular muscle weakness
AMAN, AMSAN
-China, Japan, Mexico

Lumbar puncture, EMG, nerve conduction studies

Tx- plasmapheresis IVIG, pain meds, rehabilitation

Worsens progressively for about two weeks, with a plateau within 4 weeks

Recovery begins usually lasting six to 12 months

Baseline vital capacity is important for respiratory evaluations, since we are expecting respiratory failure