1. The first goal is to determine the site of the lesion by examining the distribution and pattern of weakness. Common patterns include proximal limb-girdle weakness, distal weakness, proximal arm/distal leg weakness, and distal arm/proximal leg weakness.
2. The second goal is to determine the cause by taking a thorough history including symptoms, temporal evolution, family history, and associated systemic features. Precipitating factors can provide clues to specific disorders.
3. The third goal is to determine if a specific treatment exists and if not, optimally manage symptoms to maximize function and quality of life. A pattern-recognition approach using historical and examination findings helps narrow the differential diagnosis.
This presentation will give a brief idea on proximal myopathy, causes, clinical presentation, history and physical examination, investigations to diagnose the disease easily.
It will be more helpful to medical students.
This presentation will give a brief idea on proximal myopathy, causes, clinical presentation, history and physical examination, investigations to diagnose the disease easily.
It will be more helpful to medical students.
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.
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
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
Knee anatomy and clinical tests 2024.pdfvimalpl1234
This includes all relevant anatomy and clinical tests compiled from standard textbooks, Campbell,netter etc..It is comprehensive and best suited for orthopaedicians and orthopaedic residents.
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ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
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
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?bkling
Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
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.
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!
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
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
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Ve...kevinkariuki227
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
TEST BANK for Operations Management, 14th Edition by William J. Stevenson, Verified Chapters 1 - 19, Complete Newest Version.pdf
Pulmonary Thromboembolism - etilogy, types, medical- Surgical and nursing man...VarunMahajani
Disruption of blood supply to lung alveoli due to blockage of one or more pulmonary blood vessels is called as Pulmonary thromboembolism. In this presentation we will discuss its causes, types and its management in depth.
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
2. 1. The first goal in approaching a patient with a
suspected muscle disease is to determine the
correct site of the lesion.
1. The second goal is to determine the cause of the
myopathy.
1. The third goal is to determine whether a specific
treatment is available and if not, to optimally
manage the patient’s symptoms to maximize his or
her functional abilities and enhance quality of life.
5. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
6. Which negative and/or positive symptoms and
signs does the patient demonstrate?
• Negative
– Weakness
– Fatigue
– Exercise intolerance
– Muscle atrophy
• Positive
– Myalgia
– Cramps
– Contractures
– myotonia
– myoglobinuria
7. 1. Weakness:
a) Proximal lower extremities:
• difficulty climbing stairs, arising from a low chair or toilet,
or getting up from a squatted position.
b) Proximal upper extremities:
• trouble lifting objects over their head and brushing their
hair.
c) Distal upper extremities:
• difficulty opening jars, inability to turn a key in the ignition.
d) Distal lower extremities:
• tripping due to footdrop.
e) Cranial muscle weakness,
• dysarthria, dysphagia, or ptosis.
8. 2. Fatigue
• less useful negative symptom
• Nonspecific
• abnormal fatigability after exercise can result
from certain metabolic and mitochondrial
myopathies, and it is important to define the
duration and intensity of exercise that provokes
the fatigue.
9. 3. Myalgia, like fatigue, is another nonspecific
symptom
• episodic (metabolic myopathies)
• constant (inflammatory muscle disorders)
• more likely to be due to orthopedic or
rheumatological disorders
10. 4. Muscle cramp
– They are typically benign, occurring frequently in
normal individuals, and are seldom a feature of a
primary myopathy
– Cramps can occur with:
• dehydration,
• hyponatremia,
• azotemia,
• myxedema
• disorders of the nerve or motor neuron (especially
amyotrophic lateral sclerosis).
11. 5. Muscle contractures
– uncommon
– They are typically provoked by exercise in patients with
glycolytic enzyme defects.
– Contractures differ from cramps in that they usually last
longer and are electrically silent with needle EMG.
12. 6. Myotonia is the phenomenon of impaired
relaxation of muscle after forceful voluntary
contraction and most commonly involves the
hands and eyelids.
– Myotonia is due to repetitive depolarization of the muscle
membrane.
– Patients may complain of muscle stiffness or tightness
resulting in difficulty releasing their handgrip after a
handshake, unscrewing a bottle top, or opening their
eyelids if they forcefully shut their eyes.
– Myotonia classically improves with repeated exercise.
13. 6. paramyotonia congenita: ‘‘paradoxical
myotonia’’ in that symptoms are typically
worsened by exercise or repeated muscle
contractions.
• Exposure to cold results in worsening of both
myotonia and paramyotonia.
15. 8. Myoglobinuria
– uncommon manifestation of muscle disease
– caused by the excessive release of myoglobin from
muscle during periods of rapid muscle destruction
(rhabdomyolysis).
– Severe myoglobinuria can result in renal failure due to
acute tubular necrosis.
– If patients complain of exercise-induced weakness and
myalgias, they should be asked if their urine has ever
turned cola-colored or red during or after these episodes.
– Recurrent myoglobinuria is usually due to an underlying
metabolic myopathy
– isolated episodes, particularly occurring after
unaccustomed strenuous exercise, are frequently
idiopathic.
16.
17. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
18. What is the temporal evolution?
• onset,
• duration, and
• evolution of the patient’s symptoms
22. Evolution and duration
• episodic periods of weakness with normal
strength interictally
– (periodic paralysis, metabolic myopathies due to
certain glycolytic pathway disorders).
• constant weakness
23. Constant weakness
• acute or subacute progression
– inflammatory myopathies (dermatomyositis and
polymyositis);
• chronic slow progression over years
– most muscular dystrophies
– IBM
• nonprogressive weakness with little change
over decades
– congenital myopathies
24. Monophasic or relapsing
• polymyositis can occasionally have an acute
monophasic course with complete resolution
of strength within weeks or months.
• Patients with periodic paralysis or metabolic
myopathies can have recurrent attacks of
weakness over many years,
• a patient with acute rhabdomyolysis due to
cocaine may have a single episode.
25. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
26. Precipitating factors
• illegal drug or prescription medication use that might produce
a myopathy.
• weakness, pain, and/or myoglobinuria provoked by exercise
– a glycolytic pathway defect.
• Episodes of weakness with a fever
– carnitine palmityl transferase deficiency.
• Periodic paralysis is characteristically provoked by exercise or
ingestion of a carbohydrate meal followed by a period of rest.
• Patients with paramyotonia congenita frequently report that
cold exposure may precipitate their symptoms of muscle
stiffness.
27. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
30. Hepatomegaly
• may be seen in myopathies associated with
deficiencies in acid maltase, debranching
enzyme,
• infectious disease.
• mitochondrial disorder.
31. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
32. Family history
• A thorough family history is clearly of great
importance in making a correct diagnosis.
• Questions regarding family members’ use of
canes or wheelchairs, skeletal deformities, or
functional limitations are usually more
informative than questions such as, ‘‘Does any
member of your family have a muscle
disease?’’
34. History
Which negative and/or positive
symptoms and signs does the
patient demonstrate?
What is the distribution of
weakness?
Family history What is the temporal
evolution?
Are there precipitating
factors that trigger episodic
weakness or myotonia?
Are associated systemic
symptoms or signs present?
35. What is the distribution of weakness?
• it is important to know which muscles to test
and how to grade their power
36. – Atrophy
• selective atrophy of the
quadriceps and forearm
flexor muscles is highly
suggestive of inclusion
body myositis.
• Distal myopathies may have
profound atrophy of the
anterior or posterior lower
extremity compartments.
• (LGMD 2G in 50% of pt)
– Hypertrophy
• LGMD2C–2F, LGMD2I,
(LGMD 2G in 50% of pt)
• myotonia congenita
• amyloidosis,
• sarcoidosis, and
• hypothyroid myopath
– Pseudohypertrophy
• Duchenne and Becker
dystrophy
– Fasciculation- MND or
neuropathy
– Scapular wining
• FSHD.
• LGMD1B (laminopathy),
LGMD2A (calpainopathy),
LGMD2C–2F
(sarcoglycanopathies).
Inspection
40. Pattern 1: Proximal Limb-Girdle Weakness
• The most common pattern of muscle weakness in myopathies
• symmetrical weakness affecting predominantly the proximal
muscles of the legs and arms
• The distal muscles are usually involved, but to a much lesser
extent.
• Neck extensor and flexor muscles are also frequently affected.
• This pattern of weakness is seen in most hereditary and
acquired myopathies and therefore is the least specific in ar-
riving at a particular diagnosis
41. Pattern 2: Distal Weakness
• This pattern of weakness predominantly
involves the distal muscles of the upper or
lower extremities (anterior or posterior
compartment muscle groups)
• The involvement is usually, although not
invariably, symmetrical.
• more commonly a feature of neuropathies
42. Myopathies Characterized by Predominantly Distal
Weakness
• Distal Myopathies
– Late adult-onset distalmyopathy type 1
(Welander)
– Late adult-onset distal myopathy type 2
(Markesbery/Udd)
– Early adult-onset distal myopathy type 1
(Nonaka)
– Early adult-onset distal myopathy type 2
(Miyoshi)
– Early adult-onset distal myopathy type 3
(Laing)
– Desmin myopathy
– Childhood-onset distal myopathy"
• Myotonic Dystrophy
• FSHD*"
• Scapuloperoneal Myopathy*"
• OPMD
• Emery-Dreifuss Humeroperoneal
Dystrophy*"
• Inflammatory Myopathies
• Inclusion Body Myositis
• Metabolic Myopathies
– Debrancher deficiency
– Acid-maltase deficiency*"
• Congenital Myopathies
– Nemaline myopathy*
– Central core myopathy*
– Centronuclear myopathy
*" Scapuloperoneal pattern can occur
43. Pattern 3: Proximal Arm/Distal Leg Weakness
• This pattern of weakness affects the periscapular muscles of
the proximal arm and the anterior compartment muscles of
the distal lower extremity (scapuloperoneal distribution)
• The scapular muscle weakness is usually characterized by
scapular winging.
• Weakness can be very asymmetrical.
44. Myopathies with proximal arm/distal leg
involvement (scapuloperoneal)
• Scapuloperoneal dystrophy,
• Emery-Dreifuss dystrophy,
• LGMD1B, LGMD2A, LGMD2C–2F,
• Congenital myopathies,
– Nemaline myopathy
– Central core myopathy
• acid maltase deficiency.
• When this pattern is associated with facial
weakness->(FSHD)
45. Pattern 4: Distal Arm/ProximalLeg Weakness
• This pattern is associated with distal arm weakness involving
the distal forearm muscles (wrist and finger flexors) and
proximal leg weakness involving the knee extensors
(quadriceps).
• The facial muscles are usually spared.
• Involvement of other muscles is extremely variable.
• The weakness is often asymmetrical between the two sides,
which is uncommon in most myopathies.
• This pattern is essentially pathognomonic for inclusion body
myositis (IBM).
• This pattern may also represent an uncommon presentation
of myotonic dystropy; however, unlike IBM, muscle weakness
is usually symmetrical
46. Pattern 5: Ptosis With or Without
Ophthalmoplegia
• usually, although not always, occurs without
symptoms of diplopia.
• Facial weakness is not uncommon,
• extremity weakness is extremely variable,
depending on the diagnosis.
47. • The combination of ptosis, ophthalmoplegia (without
diplopia), and dysphagia should suggest the
diagnosis of oculopharyngeal dystrophy, especially if
the onset is in middle age or later.
• Ptosis and ophthalmoplegia without prominent
pharyngeal involvement is a hallmark of many of the
mitochondrial myopathies.
• Ptosis and facial weakness without ophthalmoplegia
is a common feature of myotonic dystrophy.
53. Creatine Kinase
• The CK is elevated in the majority of myopathies but may be
normal in slowly progressive myopathies.
• Duchenne dystrophy, the CK level is invariably at least 10
times (and often up to 100 times) normal.
• CK may also be markedly elevated:
– LGMD1C (caveolinopathy),
– LGMD2A (calpainopathy), and
– LGMD2B (dysferlinopathy),
• The CK level may not be elevated in
– corticosteroid administration,
– collagen diseases,
– alcoholism
– hyperthyroidism
– profound muscle wasting
54. Differential Diagnosis of Creatine Kinase
Elevation
• Myopathies
– Muscular dystrophies
– Congenital myopathies
– Metabolic myopathies
– Inflammatory myopathies
– Drug/toxin-induced
– Carrier state (dystrophinopathies)
• Channelopathies
• Motor Neuron Diseases
– ALS
– SMA
– Postpolio syndrome
• Neuropathies
– GBS
– CIDP
• Viral Illness
• Medications
• Hypothyroidism/ Hypoparathyroidism
• SurgeryTrauma (electromyography
studies, intramuscular or
subcutaneous injections)
• Strenuous Exercise
• Increased Muscle Mass
• Race
• Sex
• ‘‘Idiopathic HyperCKemia’’
55. Electrophysiological Studies
• Confirm localization
• can be a guide as to which muscle to
biopsy.
• r/o neuropathy, NMG disease, MND.
• NCS are typically normal in patients
with myopathy.
• Needle EMG examination: motor
units
– Brief duration,
– small-amplitude
– Early recruitment
56. Muscle biopsy
• Selection of the appropriate muscle to biopsy is critical.
• Muscles that are severely weak (MRC grade 3 or less) should
not be biopsied, since the results are likely to show only
evidence of end stage muscle disease
• muscles that have recently been studied by needle EMG
should be avoided because of the possibility of artifacts
created by needle insertion.
57. • Biopsies should generally be taken from muscles that
demonstrate MRC grade 4 strength.
• The muscle of choice:
– biceps.
– vastus lateralis.
• The gastrocnemius should be avoided, since its
tendon insertion extends throughout the muscle and
inadvertent sampling of a myotendinous junction
may cause difficulty with interpretation.
58. • Typical myopathic abnormalities include:
– central nuclei,
– both small and large hypertrophic round fibers,
– split fibers, and
– degenerating and regenerating fibers.
– Chronic myopathies frequently show evidence of
increased connective tissue and fat.
59. Inflammatory myopathies
• characterized by:
– the presence of mononuclear inflammatory cells
in the endomysial and perimysial connective
tissue between fibers and occasionally around
blood vessels ( perivascular)
60. Dermatomyositis
• …….In addition,
• perifascicular atrophy,
characterized by atrophy of
fibers located on the periphery
of a muscle fascicle, is a
common finding.
• Inflammatory cell invasion of
nonnecrotic fibers is not
prominent.
• deposition of the C5b-9 or
membrane attack complex on
or around small blood vessels
61. polymyositis
• variability in fiber size,
• scattered necrotic and
regenerating fibers,
• endomysial inflammation
with invasion of non-
necrotic muscle fibers
62. IBM
• endomysial inflammation with invasion of
non-necrotic muscle fibers
• small groups of atrophic fibers,
• eosinophilic cytoplasmic inclusions, and
muscle fibers with one or more rimmed
vacuoles lined with granular material
• Amyloid deposition is evident on Congo red
staining.
• TDP-43 positive
• Ubiquitin positive
• Electron microscopy demonstrates 15-nm to
21-nm cytoplasmic and intranuclear
tubulofilaments
63.
64. Mitochondrial disorder
• The typical muscle
histopathology is “ragged red’’
fibers
• subsarcolemmal accumulation
of abnormal mitochondria
stains red.
• best seen with modified–
Gomori trichrome stain
65. • Type 1 fibers (slow-twitch, fatigue-resistant,
oxidative metabolism) stain lightly at alkaline
and darkly at acidic pH levels.
• Type 2 fibers (fast- twitch, fatigue-prone,
glycolytic metabolism) stain darkly at alkaline
and lightly at acidic pH levels.
• Normally, a random distribution of the two
fiber types occurs, and generally twice as
many type 2 as type 1 fibers are identified.
66.
67. • W
• Increased variability in muscle fiber size
• degeneration, regeneration, isolated ‘‘opaque’’
hypertrophic fibers, and significant replacement of
muscle by fat and connective tissue.
• Central nuclei are present in 2% to 4% of the fibers.
• Type 1 fiber predominance is seen in most patients.
• Inflammatory cell infiltrates in the perimysium,
endomysium, and perivascular spaces and consist
mostly of mononuclear cells, especially
macrophages.
• endomysial and perimysial fibrosis with disease
progression
68.
69.
70. Nemaline myopathy
• Type I fiber predominance and atrophy
• Marked disproportion in fiber size, with
uniformly small type I fibers and normal
to large type II fibers
• Sarcoplasmic rods (nemaline bodies)
– Short, granular-appearing
– Subsarcolemmal and perinuclear
localization in type I fibers (especially
large rods)
– Composed of α-actinin, actin, and
other Z diskproteins, and appear to
arise from, are attached to,and
thicken the Z disk (small rods)
• Intranuclear rods
71. • Myotubular (centronuclear)
Myopathy
– Is called “centronuclear” because of
predominance of small type I fibers
with central nucleib.
– Type I fiber predominance (often
small, atrophied)
– Central pallor noted on ATPase
stainingd.
– Radial distribution of sarcoplasmic
strands apparent on NADH
reactione.
– Interstitial connective tissue:
normal or mildly increased
72. • Central Core Disease
• Type I fiber predominance
• Central cores
– Single, well-circumscribed regions
– Selectively involve type I fibers
– Deficient in mitochondria and sarcoplasmic reticulum as well as oxidative
enzymes and phosphorylase activity (detected best on sections with oxidative
enzyme immunohistochemistry ,as poorly stained central demarcated areas)
– Extend entire length of muscle fiber
• Increased endomysial connective tissue.
• Often, increased number of internal nuclei
73.
74. Molecular Genetic Studies
• Disorders With Commercially Available Molecular Genetic
Studies Performed With Peripheral Blood Samples
– Duchenne and Becker muscular dystrophies ”
– FSHD
– MD (types 1 and 2) "
– OPMD
– LGMD1B, 2A, 2C–2F, and 2I "
– Congenital muscular dystrophy (FKRP, FCMD, MEB, and POMT1
mutations) "
– Nonaka myopathy/inclusion body myopathy type 2 "
– Nemaline myopathy (ACTA1 mutations) "
– Myotubular myopathy (MTM1 mutations) "
– MERRF
– MELAS
75.
76. Case 1
• A 51-year-old man without significant past
medical
• Slowly progressive muscle weakness for the
past 7 years.
• His symptoms initially began with difficulty
walking down stairs because his left knee
would ‘‘give out.’’
• He currently has difficulty arising from a chair
and grasping objects with his right hand.
77.
78. • Current examination reveals intact cranial nerves,
sensation, and muscle stretch reflexes.
• Motor examination in the right upper extremity
shows MRC grade 5 shoulder abduction, grade 5
elbow flexion/extension, grade 4 wrist flexion,
grade 5 wrist extension, and grade 3 finger
flexion.
• Strength in the left upper extremity is normal
except for grade 4+ finger flexion.
• In the left lower extremity, the patient exhibits
grade 4+ hip flexion, grade 3+ knee extension,
and grade 4+ ankle dorsiflexion.
• In the right lower extremity, strength is normal
except for grade 4+ knee extension.
79. • The chronic onset, asymmetrical distribution
of weakness, and selective involvement of
wrist/finger flexion and knee extension
• CK= 500
• EMG= myopathy
• Muscle biopsy
82. FSHD
• normal deltoids
• biceps, triceps commonly weak, forearm
muscles are less involved resulting in a popeye-
like appearance
• wrist flexors stronger than wrist extensors
• hip flexors and quadriceps are weaker, plantar
flexors are preserved, ankle dorsiflexion is
weak and foot drop may be the presenting
symptom
• Scapular winging may be very asymmetric
(misdiagnosed as long thoracic nerve of Bell
palsy)
Identifying the age that symptoms began can provide crucial information leading to the correct diagnosis. For example, symptoms of Duchenne mus- cular dystrophy usually are identified byage 3, whereas most facioscapulohum- eral and limb-girdle muscular dystro- phies (LGMDs) begin in adolescence or later. Of the inflammatory myopathies, dermatomyositis occurs in children andadults; polymyositis occurs rarely in children but at any decade in the adult years; and inclusion body myositis oc- curs most commonly in the elderly.
Focal muscle enlargement can also be due to a neo- plastic or inflammatory process, ectopic ossification, tendon rupture, or partial denervation.
Race = ck often is above the normal range in some African American individuals and in patients with enlarged muscles.
Needle EMG can also provide a clue as to which muscles have had recent or ongoing muscle in- jury and can be a guide as to which muscle to biopsy. It is important to realize, however, that the EMG can be normal in a patient with myopathy, and the results of electrodiagnostic studies need to be evaluated in the context of the patient’s history, neurological ex- amination, and other laboratory studies.
A, C, D, Muscle biopsy of Duchenne muscular dystrophy and (B) normal control. By light microscopy, the biopsy demonstrates marked variability in fiber size,degenerating and regenerating fibers, ‘‘opaque’’ hypertrophic fibers, and replacement of muscle by fat and connective tissue (most findings shown in A and C). Note the lack of these findings in a normal muscle biopsy (B). In C, arrows show small regenerating muscle fibers and a necrotic fiber invaded by mononuclear cells (open arrow). In D, arrows indicatethe dark-staining ‘‘opaque’’ or hypercontracted large fibers. A, B, D, Hematoxylin and eosin; C, modified trichrome.
Immunostaining of frozen sections of skeletal muscle biopsies. D through N show antidystrophin antibodies using indirect immunofluorescence. A, B, and C display hematoxylin andeosin–stained sections corresponding to D, E, and F respectively. There is complete absence of immunofluorescence at the sarcolemma in a muscle section from a patient with Duchenne muscular dystrophy (DMD) (C and F), compared with the homogeneous staining of the plasma membrane in normal muscle (A and D) and in muscle biopsies from patients with Emery-Dreifuss muscular dystrophy (G), Fukuyama type of congenital muscular dystrophy (H), limb-girdle muscular dystrophy (I), facioscapulohumeral muscular dystrophy (J), myotonic dystrophy (K), and Kugelberg-Welander type of spinal muscular atrophy (L). In a frozen section of muscle from a patient with BMD (B), partial staining of the sarcolemma is observed (E). Note the mosaic pattern of immunostaining in muscle biopsies from a symptomatic (M) and an asymptomatic (N) DMD carrier.
Myofibrillar myopathy. A and B, Trichrome-stained sections show aggregates of dense granular and filamentous amor- phous material and vacuolar change. Immunohistochemistry performed in sections corresponding to that in B demonstrates accumulation of desmin (C) and αB-crystallin (D).
Muscle involved: serratus anterior or long thoracic nerve
Weakness of trapezius causes winging of scapula when the arm is abducted.
Also seen in LGMD
The genetic abnormality is a deletion in a 3.3-kb repeating sequence termed D4Z4.
Chromosome 4