Myasthenia Gravis is a neuromuscular disorder characterized by fluctuating weakness that worsens with activity and improves with rest. It results from antibodies blocking or lessening the effects of acetylcholine at the neuromuscular junction. Symptoms often begin with weakness of the eye muscles or face. While treatments can help control symptoms, there is currently no cure. Management involves anticholinesterase medications, immunosuppressants, plasmapheresis, thymectomy, and ventilatory support during myasthenic crises.
Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature.
Guillain-Barré syndrome (GBS) can be described as a collection of clinical syndromes that manifests as an acute inflammatory polyradiculoneuropathy with resultant weakness and diminished reflexes.
Although the classic description of GBS is that of a demyelinating neuropathy with ascending weakness, many clinical variants have been well documented in the medical literature.
Now a days TBM is super most disease in Indian children.
Tuberculous meningitis (TBM) is difficult to diagnose, and a high index of suspicion is needed to make an early diagnosis.
references:
1-European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision.
2-Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants By Kelly Gwathmey, MD
3-Patient Journey in CIDP: Burden, Symptoms, and Diagnosis Jeffrey A. Allen, MD; Richard A. Lewis, MD
Disorders of the neuromuscular junction include Myasthenia gravis, Lambert-Eaton myasthenic syndrome, Botulism, Tetanus, Strychnine intoxication, Organophosphates poisoning and neuromyotonia. Pharmacology of the NMJ is also reviewed in brief.
This presentation briefly summarizes pathophysiology, clinical features, diagnosis and treatment of different types of tuberculosis of brain and spinal cord.
Myasthenia gravis (MG) is a long-term neuromuscular disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, drooping eyelids, trouble talking, and trouble walking.
TRANSVERSE MYELITIS
The term myelitis refers to inflammation of the spinal cord; transverse simply describes the position of the inflammation, that is, across the width of the spinal cord.
A pathogenically heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or sub acute development of motor weakness, sensory impairment, bowel or bladder dysfunction and autonomic dysfunction
Now a days TBM is super most disease in Indian children.
Tuberculous meningitis (TBM) is difficult to diagnose, and a high index of suspicion is needed to make an early diagnosis.
references:
1-European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint Task Force—Second revision.
2-Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Its Variants By Kelly Gwathmey, MD
3-Patient Journey in CIDP: Burden, Symptoms, and Diagnosis Jeffrey A. Allen, MD; Richard A. Lewis, MD
Disorders of the neuromuscular junction include Myasthenia gravis, Lambert-Eaton myasthenic syndrome, Botulism, Tetanus, Strychnine intoxication, Organophosphates poisoning and neuromyotonia. Pharmacology of the NMJ is also reviewed in brief.
This presentation briefly summarizes pathophysiology, clinical features, diagnosis and treatment of different types of tuberculosis of brain and spinal cord.
Myasthenia gravis (MG) is a long-term neuromuscular disease that leads to varying degrees of skeletal muscle weakness. The most commonly affected muscles are those of the eyes, face, and swallowing. It can result in double vision, drooping eyelids, trouble talking, and trouble walking.
TRANSVERSE MYELITIS
The term myelitis refers to inflammation of the spinal cord; transverse simply describes the position of the inflammation, that is, across the width of the spinal cord.
A pathogenically heterogeneous focal inflammatory disorder of the spinal cord characterized by acute or sub acute development of motor weakness, sensory impairment, bowel or bladder dysfunction and autonomic dysfunction
Myasthenia gravis (MG) is a neuromuscular disorder characterized by weakness and fatigability of skeletal muscles.
The underlying defect is a decrease in the number of available acetylcholine receptors (AChRs) at neuromuscular junctions due to an antibody-mediated autoimmune attack
Basavarajeeyam is an important text for ayurvedic physician belonging to andhra pradehs. It is a popular compendium in various parts of our country as well as in andhra pradesh. The content of the text was presented in sanskrit and telugu language (Bilingual). One of the most famous book in ayurvedic pharmaceutics and therapeutics. This book contains 25 chapters called as prakaranas. Many rasaoushadis were explained, pioneer of dhatu druti, nadi pareeksha, mutra pareeksha etc. Belongs to the period of 15-16 century. New diseases like upadamsha, phiranga rogas are explained.
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
263778731218 Abortion Clinic /Pills In Harare ,sisternakatoto
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Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
MANAGEMENT OF ATRIOVENTRICULAR CONDUCTION BLOCK.pdfJim Jacob Roy
Cardiac conduction defects can occur due to various causes.
Atrioventricular conduction blocks ( AV blocks ) are classified into 3 types.
This document describes the acute management of AV block.
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
2. Introduction
Most common primary disorder of
neuromuscular transmission
Usually due to acquired immunological
abnormality
Also due to genetic abnormalities at
neuromuscular junction.
3. History
In 1862, Willis described a disease with
fluctuating weakness that varied
throughout the day.
Erb described the classic signs of
Myasthenia gravis in 3 patients &
recognized that fluctuating weakness
differed from that seen in other
diseases.
In 1893, Goldflam provided a
comprehensive description of the
disease
In 1895, Jolly used the term “
Myasthenia Gravis Pseudoparalytica”
4. Epidemiology
Most common age of onset :
women : 2nd & 3rd decades
men : 5th & 6th decades
< 40 yrs , females are affected 2 to 3
times as often as males.
Later in life, incidence is higher in
males.
Of pts with thymomas- is a tumor,
majority are older ( 50 – 60 yrs ) &
males.
5. Clinical Presentation
c/o specific muscle weakness rather &
not of generalized fatigue.
2/3rd – ocular motor disturbances,
ptosis
or diplopia
1/6th – oropharyngeal muscle
weakness
difficulty in chewing, swallowing
or talking
10 % - limb weakness
6. Clinical Presentation
Severity of weakness fluctuates during
the day
least severe in the morning & worse
as the day progresses, especially after
prolonged use of affected muscles.
Patient usually gives h/o
Worsening of ocular symptoms while
reading, watching television, driving.
Worsening of jaw muscle weakness
on prolonged chewing – meat / chewy
candy.
7. Clinical Presentation
Also give h/o :
Frequent purchase of new eyeglasses
to correct blurred vision
Avoidance of foods that became
difficult to chew or swallow
Cessation of activities that require
prolonged use of specific muscles.
8. Clinical Presentation
Course of disease is variable but often
progressive.
Symptoms fluctuate over a short
period & then become more severe for
several years ( Active Stage )
Followed by a period in which
fluctuations in strength still occurred (
Inactive Stage )
After 15-20 yrs, weakness becomes
fixed & most severely involved
muscles become atrophic ( Burnt-out
9. Ocular manifestations :
Weakness of levator palpebrae &
extraocular muscles – initial
manifestation in ½ cases.
Ocular palsies, ptosis-dropping of one
or both upper eyelids, usually
accompanied by weakness of eye
closure – always myopathic & not
neuropathic in origin
Diplopia- due to asymmetric weakness
of muscles in both eyes.
10.
11. Ocular manifestations :
Sustained upgaze for 30 or more
seconds – induce / exaggerate ptosis &
uncover myasthenic motor weakness.
Lid-twitch sign : twitching of upper eyelid
appears a moment after the patient
moves the eyes from downward to
primary position
After sustained upward gaze, 1or more
twitches are observed with closure of
eyelids.
13. Ocular manifestations :
Combined weakness of extraocular
muscles, levators & orbicularis oculi
combined with
Normal pupillary response to light
Normal accomodation
is virtually diagnostic of myasthenia.
Bright light aggravates ocular signs
Cold ( application of ice pack ) improves
them.
14. Oropharyngeal manifestations
:
Voice may be nasal after prolonged
talking
Weakness of laryngeal muscles –
hoarseness
Frequent choking due to difficulty in
swallowing & chewing after eating for
a while.
Characteristic facial appearance
15. Rest smiling
At rest, b/l lid ptosis,
downward curve of
corners of mouth,
giving pt a sad
appearance
Smiling: myasthenic
snarl – resulting from
upward movement of
medial portion of
upper lip & horizontal
contraction of
corners of mouth
16. Oropharyngeal manifestations
:
Jaw weakness – shown by manually
opening the jaw against resistance,
which is not possible in normal people.
Patient holds
jaw closed with thumb under chin,
middle finger curled under nose/lower
lip
index finger extended up the cheek
producing studious appearance.
17. Neck flexors are weaker than neck
extensors
Bulbar weakness is prominent
Limb weakness is often proximal &
asymmetric
Tendon reflexes are unaffected
18. Clinical Presentation
I Ocular myasthenia ( 15-20% )
II A. Mild, generalized myasthenia with
slow progression; no crises;
drug responsive ( 30% )
II B. Moderately severe generalized
myasthenia; severe skeletal &
bulbar
involvement but no crises;
drug response –less
satisfactory(25%)
19. Clinical Presentation
III. Acute fulminant myasthenia;
rapid progression of severe
symptoms
with respiratory crises & poor drug
response; high incidence of
thymoma;
high mortality ( 15% )
IV. Late severe myasthenia;
symptoms
same as III; resulting from
steady progression over 2 years
20. Congenital Myasthenic
Syndromes
type Clinical features genetics treatment
SLOW
CHANNEL
MOST COMMON
WEAK
FOREARM
EXTENSORS
AUTOSOMAL
DOMINANT
QUINIDINE
LOW AFFINITY
FAST CHANNEL
PTOSIS AUTOSOMAL
RECESSIVE
3,4-DAP,
ANTI AChE
SEVERE AChR
DEFICIENCIES
VARIABLE
SEVERITY
TYPICAL MG
FEATURES
AUTOSOMAL
RECESSIVE
ANTI AChE
? 3,4-DAP
AChE
DEFICIENCY ABSENT
PUPILLARY
RESPONSE
- WORSE WITH
ANTI AChE
DRUGS
21. Pathophysiology
Decrease in number of available
AChR at postsynaptic muscle
membrane due to antibody mediated
autoimmune attack.
Postsynaptic folds are flattened or
simplified.
So, although ACh is released normally,
it produces small endplate potentials
that may fail to trigger muscle action
potential.
Failure of transmission at many NMJs
22.
23. Pathophysiology
Decreased efficiency of
neuromuscular transmission
combined with presynaptic rundown
results in activation of fewer & fewer
muscle fibres by successive nerve
impulses
& hence increasing weakness /
myasthenic fatigue
26. Anticholinesterase Test /
Edrophonium Chloride ( Tensilon )
Test
Positive in > 90 % of patients with MG
Initially 2mg Edrophonium IV given,
response monitored for 60 sec
- if definite improvement of muscular
weakness occurs, it is + & test is
terminated.
If no change, additional 8mg IV is
given in 2 parts ( 3mg & 5 mg ) , if
improvement is seen within 60 sec
after any dose, no further injections
are given.
27. Anticholinesterase Test /
Edrophonium Chloride ( Tensilon )
Test
10 mg of Edrophonium does not
weaken normal muscle & occurrence
of weakness indicates neuromuscular
transmission weakness.
IM Neostigmine can be used ( infants
& children )
False positive in neurologic disorders
like Amyotropic lateral sclerosis
28. Antibodies to AChR , MuSK :
Anti-AChR Radioimmunoassay :
85 % positive in generalized MG
50 % positive in ocular MG
Presence of Anti-AChR antibodies is
virtually diagnostic
But negative result does not rule out
MG
31. Single Fiber
Electromyography
Most sensitive clinical test of
neuromuscular transmission & shows
increased jitter in some muscles in
almost all pts with MG.
It is confirmatory but not specific
Pts with mild / purely ocular muscle
weakness may have increased jitter
only in facial muscles.
When jitter increases, EMG should be
done.
32. JITTER
Single fibre needle generally records from a single
muscle fibre, because the muscle fibre in a motor
unit are randomly destributed.
It is possible, to position the needle so as to record
from 2 or more muscle fibre of the same motor unit.
A pair of single muscle fibre potentials that
recorded trigering potential and slave potential.
The time interval between these two potentials
varies from one discharge to another is known as
jitter.
33. CT / MRI
For ocular MG : Do CT / MRI to
exclude
intracranial lesions
34. Disorders associated with
Myasthenia gravis
Disorders of thymus : thymoma,
hyperplasia
Other auto-immune disorders :
Hashimoto’s Thyroiditis
Graves’ Disease
Rheumatoid Arthritis
SLE
37. Disorders that interfere with
therapy
Tuberculosis
Diabetes
Peptic ulcer
GI bleeding
Renal disease
Hypertension
Asthma
Osteoporosis
Obesity
38. Investigations -
CT /MRI of Mediastinum
ANA –anti nuclear antibody, RA
Factor, Antithyroid antibodies
Thyroid function tests
Mantoux- for Tb
Chest X Ray
FBS, HbA1c
Pulmonary Function Tests
Bone densitometry
40. Based on natural history of disease in
each patient & predicted response to
specific form of treatment
Treatment goals are individualized
Successful treatment requires close
medical supervision & long term
followup
Return of any weakness after a period
of improvement – to be taken as
heralding further progression.
41. Cholinesterase Inhibitors
Pyridostigmine Bromide
initial dose 30 – 60 mg TID / QID
Dose to be tailored according to pts
need
Used as diagnostic test
Early symptomatic treatment
May be satisfactory chronic treatment
in some.
Neostigmine, Mestinon,
Ambenonium chloride are also used.
42. Thymectomy - to remove the
thymus
Recommended for most pts with MG
Maximal favourable response occurs
2 - 5yrs after surgery
Best response is seen in young
people operated early in the course of
disease
But improvement can occur even after
30 yrs of symptoms.
Improvement is also seen in
seronegative MG pts.
43. Corticosteroids
Produce rapid improvement in many
pts
Produce total remission / marked
improvement in > 75 % of patients
Used as secondary treatment in who
do not respond to thymectomy /
immunosuppressive therapy
Initial dose prednisone 15 – 25
mg/day increased until maximal
improvement is seen or upto 50 – 60
44. Immunosuppressants
Produces marked & sustained
improvement in many
Azathioprine – initially 50 mg OD,
which is increased in 50 mg/day
increments every 7 days to total of
150-200 mg/day
Cyclosporine – initially 5-6 mg/kg/day
Cyclophosphamide – IV 200 mg/day-
5days
150-200mg/day
45. Plasma exchange
Produces rapid improvement
Mainly used as adjunctive treatment
As treatment in those who have not
responded to other forms of treatment
46. Ocular myasthenia
Started on Cholinesterase inhibitors
If unsatisfactory – prednisone is added
Thymectomy in young
47. Generalized myasthenia
onset < 60 yrs
High dose daily prednisone / plasma
exchange preoperatively
Thymectomy in all
Weakness + after surgery / recurs / no
improvement 12 months after surgery
– high dose daily prednisone,
cyclosporine / azathioprine
48. Generalized myasthenia
onset > 60 yrs
Initially cholinesterase inhibitors
If response is unsatisfactory –
add azathioprine
If response is unsatisfactory –
add high dose prednisone or
substitute cyclosporine for
azathioprine
49. Thymoma
Thymectomy in all cases
Pretreated with high dose prednisone
with / without plasma exchange
Postoperative radiation is used if
tumour resection is incomplete /
tumour is spread beyond thymic
capsule
Small tumours – managed medically
50. Juvenile myasthenia gravis
Onset of immune mediated MG < 20
yrs is referred to as Juvenile MG
Female : male = 3 : 1
When myasthenic symptoms develop
in childhood – determine if pt has
acquired form or genetic form that
does not respond to immunotherapy
Cholinesterase inhibitors initially
Later thymectomy can be done.
51. Seronegative myasthenia
gravis
More likely male
Have milder disease
Ocular myasthenia, fewer thymomas,
less frequent thymic hyperplasia, more
frequent thymic atrophy
52. Myasthenic Crisis
An exacerbation of weakness
sufficient to endanger life , usually
consists of respiratory failure caused
by diaphragmatic & intercostal muscle
weakness.
Usually have precipitating event such
as infection (most common), surgery,
rapid tapering of immunosuppression
53. Cholinergic crisis
Respiratory failure from overdose of
cholinesterase inhibitors
It was more common before the
introduction of immunosuppressive
therapy
Possibility that deterioration could be
due to cholinergic crisis is best
excluded by temporarily stopping the
anticholinesterase drugs.
54. Management
Admit in intensive care unit
Discontinue all cholinesterase
inhibitors
Ventilate the patient
Cholinesterase inhibitors should be
resumed at low doses & slowly
increased as needed
Treat the intercurrent infection