MYOPATHIES A SPECIAL AND SEPERATE ENTITY WITH SPECIFIC FEATURES IN EACH DISORDER MAKING US EASY FOR DIAGNOSIS,CONFIRMATION BY MUSCLE BIOPSY.THE SEMINAR WAS PRSENTED ON 06/07/2011...AT 09.00AM
HAVE A LOOK ..AND COMMENT..WITHOUT BIAS..
MYOPATHIES A SPECIAL AND SEPERATE ENTITY WITH SPECIFIC FEATURES IN EACH DISORDER MAKING US EASY FOR DIAGNOSIS,CONFIRMATION BY MUSCLE BIOPSY.THE SEMINAR WAS PRSENTED ON 06/07/2011...AT 09.00AM
HAVE A LOOK ..AND COMMENT..WITHOUT BIAS..
This ppt describes various movement disorders found commonly in elderly persons. It also describes hyper and hypokinetic disorder categorization with cause and pathophysiology of movement disorders.
This presentation is about different diseases which presents or are associated with myotonia. Referrences were taken from Bashir Katirji Neuromuscular textbook, continuum, and seminar of neurology journal.
This ppt describes various movement disorders found commonly in elderly persons. It also describes hyper and hypokinetic disorder categorization with cause and pathophysiology of movement disorders.
This presentation is about different diseases which presents or are associated with myotonia. Referrences were taken from Bashir Katirji Neuromuscular textbook, continuum, and seminar of neurology journal.
OSCE REVISION IN OBSTETRICS AND GYNECOLOGY 2015,NEARLY COVERING COURSE CURRICULUM .Prepared by Dr Manal Behery.Professor of OB&Gyne .Faculty of medicine,Zagazig University
This ppt nots specially for physiotherapy students this is for study purpose if you need this kind of short and brief study material keep following my website.. Education adda
Neurofibroma - A neurofibroma is a type of nerve tumor that forms soft bumps on or under the skin.
It occurs in approximately 1 in 4,000 births
It is a genetic disorder that can affect the brain, spinal cord, nerves and skin
Central Nervous System, Epilepsy, Parkinson, Alzheimer, Stroke and Migraine.Dr. Kiran Dhamak
Central Nervous System is one of the unit in Pharmacotherapeutics Subject which is for Second Year Diploma in Pharmacy. The unit covers diseases like Epilepsy, Parkinson, Alzheimer, Stroke and Migraine. The presentation includes the point as per diploma in pharmacy students may understand very easily. The syllabus is framed by Pharmacy Council of India which is implemented by MSBTE ER 2020-2021
Similar to Neuropathies & myopathies - an overview (20)
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.
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.
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Acute scrotum is a general term referring to an emergency condition affecting the contents or the wall of the scrotum.
There are a number of conditions that present acutely, predominantly with pain and/or swelling
A careful and detailed history and examination, and in some cases, investigations allow differentiation between these diagnoses. A prompt diagnosis is essential as the patient may require urgent surgical intervention
Testicular torsion refers to twisting of the spermatic cord, causing ischaemia of the testicle.
Testicular torsion results from inadequate fixation of the testis to the tunica vaginalis producing ischemia from reduced arterial inflow and venous outflow obstruction.
The prevalence of testicular torsion in adult patients hospitalized with acute scrotal pain is approximately 25 to 50 percent
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.
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Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
New Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
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- GENE THERAPY
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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
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
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3. Definition
Damage to nerves which may be caused
either by diseases or trauma to the nerve or
as a component of systemic illness
11/19/2011 Jipmer physiologist 3
4. • The neuropathy is a symptom of another
disorder
• In most common forms of polyneuropathy, the
nerve fibers most distant from the brain and the
spinal cord malfunction first.
• Pain and other symptoms often appear
symmetrically
11/19/2011 Jipmer physiologist 4
5. • The peripheral nerves include:
Cranial nerves
(with the exception of the second)
Spinal nerve roots
Dorsal root ganglia
Peripheral nerve trunks and their terminal
branches
Peripheral autonomic nervous system
11/19/2011 Jipmer physiologist 5
6. Symptoms in neuropathy
A wide array of symptoms
can occur when nerves are
damaged
o Paresthesia
o Sensitivity to touch,
Positive
Pins and needles
Tingling
Burning
Negative
Numbness
Deadness
As if wearing shocks and
walk
11/19/2011 Jipmer physiologist 6
7. In chronic course symptoms worse, muscle
wasting, paralysis, or gland dysfunction
11/19/2011 Jipmer physiologist 7
8. Neuropathy - Signs
• Distal sensory loss
• Distal weakness and
atrophy
• Decreased or absent
reflexes
– Ankle jerks lost first
11/19/2011 Jipmer physiologist 8
12. Pathophysiological classification
• Motor , sensory, or autonomic
• Mononeuropathy , polyneuropathy or
mononueritis multiplex
• Focal, multifocal or symmetric
• Proximal or distal
• Axonal, demyelinating or both
• Acute, sub acute or chronic
11/19/2011 Jipmer physiologist 12
13. • Some neuropathies may affect all three
types of nerves, others primarily affect one
or two types.
• Predominately motor neuropathy
• Predominately sensory neuropathy
• Sensory-motor neuropathy
• Autonomic neuropathy
• Impaired function and symptoms depend
on the type of nerves that are damaged.
11/19/2011 Jipmer physiologist 13
14. • Mononeuropathy involve damage to only one
nerve
• When multiple nerves supplying one limb are
affected-called polyneuropathy.
• Two or more isolated nerves in separate areas
of the body are affected-called mononeuritis
multiplex
11/19/2011 Jipmer physiologist 14
15. o Focal neuropathies include common
compressive neuropathies such as carpal
tunnel syndrome, ulnar neuropathy ,peroneal
neuropathy
o Multifocal neuropathy suggests a
mononeuritis multiplex that may be
caused, for example, by vasculitis or diabetes
11/19/2011 Jipmer physiologist 15
16. Axonal degeneration
• Primary destruction of the axon with secondary
degeneration of its myelin sheath
• Generalized abnormality in the neuron cell body-
neuronopathy
• Abnormality in the axon - axonapathy
11/19/2011 Jipmer physiologist 16
17. Segmental demyelination
Dysfucntion of Schwann cell or damage to the myelin sheath
Denuded axon provide signal for remyelination
Precursor cells within endoneurium replace injured cells
Cells proliferate and engulf axon.= remyelination in time
11/19/2011 Jipmer physiologist 17
18. Neurophysiological classification
• Uniform demyelinating sensorimotor poly neuropathy
• Segmental demyelinating, motor more than sensory
neuropathy
• Axonal , motor more than sensory polyneuropathy
• Axonal sensory polyneuropathy
• Axonal mixed sensorimotor polyneuropathy
• Mixed axonal and demyelinating sensorimotor
polyneuropathy
11/19/2011 Jipmer physiologist 18
20. HMSN I (Charcot- Marie- Tooth I)
• HSMN I – AD is the most common hereditary
neuropathy.
• CMT-I A chromosome 17p11 , CMT-IB
chromosome 1q22 , CMT-IC
16p13, chromosome , CMT-IX chromosome
Xq13.1
• Slowly progressive distal weakness
• Foot deformity, areflexia , distal sensory loss
• Upper limb ataxia, tremor, peripheral n
hypertrophy
11/19/2011 Jipmer physiologist 20
21. Neurophysiological features
• Conduction velocity less than 25% of lower limit
• Median motor forearm conduction< 38 m/s
• Uniform NCV changes in adjacent nerves
• Absence of conduction block and temporal
dispersion
• F response
• Needle EMG shows minimal fibrillations in distal
muscles.
11/19/2011 Jipmer physiologist 21
22. Uniform demyelinating sensorimotor
poly neuropathy-
Electrophysiological studies ( NCS ) show
o Uniform slowing of NCV
o Similar NCV slowing in adjacent nerves
o Absence of conduction block and temporal
dispersion
o Prolongation of F response commensurate with
NCV slowing
11/19/2011 Jipmer physiologist 22
23. Segmental demyelinating motor more
than sensory neuropathy
o Acute inflammatory demyelinating poly radiculo
neuropathy AIDP
o Chronic inflammatory demyelinating poly
radiculo neuropathy CIDP
o Multifocal motor neuropathy
o Paraproteinemia
o HIV neuropathy
o Lyme disease
o Diphtheria
o Penicillamine
11/19/2011 Jipmer physiologist 23
24. AIDP
o The prototype
o Distal paresthesia with symmetric weakness
o Distal areflexia
o Variants are pure motor, pure
sensory, autonomic, relapsing, and Miller fisher types
o Cranial nerves esp facial n and bulbar may be involved
o Respiratory muscles are severely involved in about 25
11/19/2011 Jipmer physiologist 24
26. Pathophysiology of GBS
• Pathological findings include inflammatory and
demyelinating changes.
• Monocytes and macrophages appear to attack myelin
sheaths.
• Myelinated fibers show segmental demyelination during
the first few days. Segmental remyelination occurs
subsequently.
• The lesions have a perivenular distribution
11/19/2011 Jipmer physiologist 26
27. Chronic inflammatory demyelinating
polyneuropathy
• Chronic progressive or relapsing neuropathy, motor >
sensory.
• Electrophysiology: slow conduction velocity & conduction
block
• Pathology: segmental demyelination and
remyelination, onion bulbs, fibrosis and little or no
lymphocytic infiltration of tissue.
11/19/2011 Jipmer physiologist 27
28. Segmental demyelinating motor more
than sensory neuropathy
Nerve conduction studies
o Slowing of motor and sensory conduction
velocity
o Prolongation of terminal latency
o Conduction block
o Dispersion and prolonged or absent F waves
11/19/2011 Jipmer physiologist 28
29. Axonal, motor more than sensory
neuropathy
• Axonal type of GBS
• Acute intermittent porphyria
• HSMN type II , V
• Toxic neuropathies such as lead, dapsone
• Paraneoplastic syndrome
• Metabolic – hypoglycemia
• Critical care neuropathy
11/19/2011 Jipmer physiologist 29
30. • Distal symmetric weakness and wasting
with minimal sensory loss
11/19/2011 Jipmer physiologist 30
31. Axonal,motor more than sensory
neuropathy
• Nerve conduction studies
• Reduced CMAP amplitude
• NCV is normal
• SNAP amplitudes are also decreased
• Fibrillations appear in distal muscles
11/19/2011 Jipmer physiologist 31
33. Diabetic neuropathy
• Onset of neuropathy depends upon the duration
of illness
• 50% diabetics have peripheral neuropathy of
which 80% have had the illness for >15 years
• Distal symmetric sensory or
sensorimotor, autonomic, focal or multifocal
asymmetric
• Symmetric neuropathy involves distal sensory
, motor nerves .
• Decreased sensation, loss of pain sensation –
ulcer
11/19/2011 Jipmer physiologist 33
34. Diabetic neuropathy
• Predominant pathology is axonal neuropathy.
• In chronic cases segmental demyelination also
seen
Pathophysiology –
• Loss of small myelinated fibers and unmyelinated
fibers. But large fibers can also be affected.
• Endoneurial arterioles show
thickening, hyalination, intense PAS positivity in
the walls and extrensive reduplication basement
membrane
11/19/2011 Jipmer physiologist 34
35. Sensory axonal polyneuropathy
• Nerve conduction studies
• Diminished or absent SNAP amplitude in
the setting of normal motor nerve
conduction velocity
11/19/2011 Jipmer physiologist 35
37. • Paresthesia and dyesthesia of feet and
distal legs
• Wasting is marked
• Loss of ankle reflex
• Pathophysiology – evidence of
degeneration of distal portion of axons
11/19/2011 Jipmer physiologist 37
38. Axonal type of mixed sensorimotor
neuropathy
Nerve conduction studies
• Reduced or absent SNAP
• CMAP amplitude decreases and motor
conduction velocity also decrease in later
stage
EMG
• Fibrillations and positive sharp waves are
prominent in distal muscles.
• Temporal dispersion on proximal stimulation
is not found as in demyelinating neuropathies
11/19/2011 Jipmer physiologist 38
39. Mixed axonal loss and demyelinating
neuropathy
• Diabetes
• Uremia
• Paraproteinemia
11/19/2011 Jipmer physiologist 39
40. • Paresthesia, dyesthesia or numbness
• Reduced vibration and two point
discrimination
• Pathophysiology – segmental
demyelination and remyelination along
with axonal degeneration
11/19/2011 Jipmer physiologist 40
41. Mixed axonal loss and
demyelinating neuropathy
• Nerve conduction studies
• Reduced or unrecordable CMAP, SNAP or
both
• Moderate to severe slowing of NCV with
temporal dispersion of CMAP
11/19/2011 Jipmer physiologist 41
42. Clinical examination
• Thorough history and physical examination is
needed.
• Cranial nerve examination
• Motor , sensory, autonomic nervous system
examination
• Fundus examination
• Lymphadenopathy , hepatomegaly or
splenomegaly, and skin lesions
11/19/2011 Jipmer physiologist 42
44. Electrophysiologic studies
• EMG and nerve conduction studies (NCS) are often the
most useful initial laboratory studies in the evaluation of
a patient with peripheral neuropathy
• Confirm the presence of a neuropathy
• Provide information as to the type of fibers involved
(motor, sensory, or both), the pathophysiology (axonal
loss versus demyelination) and a symmetric versus
asymmetric or multifocal pattern of involvement.
11/19/2011 Jipmer physiologist 44
45. Electrophysiologic studies
• The limitations of EMG/NCS.
– There is no reliable means of studying proximal
sensory nerves.
– NCS results can be normal in patients with small-fiber
neuropathies
– Lower extremity sensory responses can be absent in
normal elderly patients.
• EMG/NCS are not substitutes for a good clinical
examination.
11/19/2011 Jipmer physiologist 45
46. Treatment
• The goal of treatment is to manage the
underlying condition causing the neuropathy and
repair damage, as well as provide symptom
relief.
11/19/2011 Jipmer physiologist 46
47. Treatment
• Medical management
– Analgesics .
– antiepileptic drugs, including
gabapentin, phenytoin, and carbamazepine
– some classes of antidepressants, including tricyclics
such as amitriptyline.
– Mexiletine
– local anesthetics such as lidocaine or topical patches
containing lidocaine
– Codeine/oxycodone
11/19/2011 Jipmer physiologist 47
48. Treatment
• Mechanical aids can help reduce pain and lessen the
impact of physical disability.
– Hand or foot braces can compensate for muscle weakness or
alleviate nerve compression.
– Orthopedic shoes can improve gait disturbances and help
prevent foot injuries in people with a loss of pain sensation.
• If breathing becomes severely impaired, mechanical
ventilation can provide essential life support.
11/19/2011 Jipmer physiologist 48
49. Treatment
• Surgical intervention often can provide
immediate relief from mononeuropathies caused
by compression or entrapment injuries.
– Repair of a slipped disk can reduce pressure on
nerves where they emerge from the spinal cord; the
removal of benign or malignant tumors can also
alleviate damaging pressure on nerves.
– Nerve entrapment often can be corrected by the
surgical release of ligaments or tendons.
11/19/2011 Jipmer physiologist 49
51. Definition
Neuromuscular disorders in which the
primary symptom is muscle weakness due to
dysfunction of muscle fiber
11/19/2011 Jipmer physiologist 51
52. Introduction
• Worldwide incidence of all inheritable
myopathies is about 14%
• Overall incidence of muscular dystrophy is about
63 per 1 million.
• Worldwide incidence of inflammatory
myopathies is about 5–10 per 100,000 people.
More common in women
• Corticosteroid myopathy is the most common
endocrine myopathy and endocrine disorders
are more common in women
• Incidence of metabolic myopathies – increasing
11/19/2011 Jipmer physiologist 52
53. Myopathy: symptoms
• Muscle pain and fatigue; exercise
intolerance
• Proximal and symmetric weakness
– Waddling gait; difficulty of rising from
sitting, climbing stairs; Gower’s sign
– Hyperextension of the knee
– Increased lordosis of the lumbar
spine, scoliosis
– Contractures, tight Achilles tendons
• Myopathic face
• Muscle atrophy; pseudohypertrophy
• Myotonia
• Tendon reflexes are normal or
depressed
11/19/2011 Jipmer physiologist 53
54. Clinical examination
• Thorough clinical
examination!
• Observation – look for muscle
atrophy, deformities
• Strength testing
• Functional testing
– Stand up from a chair
– Walk
– Step up on a low stool
• REFLEXES and SENSATION
11/19/2011 Jipmer physiologist 54
57. Diagnostic histological features
of myopathies
• Absence of neurogenic abnormalities
• Necrotic muscle fibers
• Basophilic (regenerating) myofibers
• Fibrosis of the endomysium
• Special pathological features (inflammatory
cells, ragged red fibers etc.)
11/19/2011 Jipmer physiologist 57
58. Muscle dystrophies
• Hereditary myopathies, characterized by
progressive weakness and muscle atrophy
• Genetic defect of proteins constituting the
sarcolemma-associated cytoskeleton
system
11/19/2011 Jipmer physiologist 58
59. Duchenne muscular dystrophy
• First described in 1881- dystrophin gene
discovered in the early 1980's
11/19/2011 Jipmer physiologist 59
60. Duchenne muscular dystrophy
• X-chromosome linked, recessive inheritance
• 1 in 3500 live births,
11/19/2011 Jipmer physiologist 60
61. Clinical features
• Onset of weakness before age 5
• Progressive
weakness, proximal>distal, and muscle
wasting
• Gower’s sign
• Hypertrophy of calves,
• psuedohypertrophy of deltoid, gluteal
• Skeletal deformities
• Cardiomyopathy
• wheel chair dependence by the age of
12, respiratory infection at 16-18 years.
Fatal in the third decade
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62. Electrophysiology
• EMG changes – rate of muscle fiber destruction
and extent of regeneration.
• Fiber loss-Low amplitude short duration MUPs,
• Fiber degeneration- polyphasic MUPs
• Necrosis - fibrillations with low amplitude and
short duration
• Nerve conduction studies – generally normal
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63. • Elevated CPK levels to 20 to 100 folds
• Variation in size and shape of muscle fibers and
small groups of necrotic and regenerating fibers-
muscle biopsy.
• Absence of dystrophin gene in biopsied muscles
or genetic defect analysis in WBCs
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64. Management
• No specific treatment
• Physiotherapy
• Aerobic exercise
• Low intensity anabolic steroids
• Prednisone supplements
• Orthoses (orthopaedic appliances used for
support)
• Orthopaedic surgery
• Critical care
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65. Beckers muscular dystrophy
• Allelic defect in DMD gene.
• 10 times less frequent than DMD
• Better prognosis. Patient lives upto 40-50 years.
• Mental retardation and heart failure can occur
• Muscle biopsy – variable muscle fiber size with
aberrant large fibers. Endomysial fibrosis and
fatty infiltration
• Patchy staining of DMD gene
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66. Gene Clinical feature Pathophysiology
Fascioscapulohumeral MD - AD FSHMD1B ge Progressive muscular
weakness and atrophy
Dystrophic myopathy with
inflammatory infiltrates
involving the face,
scapular, proximal arm
and peroneal muscles
myopathic face,
Oculopharyngeal MD - AD PABP2 Ptosis and extra ocular Dystrophic myopathy incl
muscle weakness rimmed vacuoles
Emery – Dreifuss MD – X - linked EMD, LMNA Triad of early Mild myopathic changes.
contracture, humero- Absent emerin by
peroneal weakness and immunohistochemistry
cardiac conduction
defects
Congenital –MD AR Laminin Neonatal hypotonia , Variable fiber size and
alpha 2 muscle weakness extensive endomysial
fibrosis
Congenital –MD – Fukuyama type AR Fukutin Neonatal hypotonia and Variable fiber size and
MR extensive endomysial
fibrosis. CNS
malformation
Congenital –MD Protein o Neonatal hypotonia and Variable fiber size and
Walker – warnburg type mannosyl MR, ocular malformation extensive endomysial
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transferase 66
fibrosis. CNS, ocular
malformation
67. Gene Clinical feature Pathophysiology
Limb-girdle dystrophies
Sarcoglycanopathies AR
α, β, γ, δ
sarcoglyc
ge
Starts between 2 and 20
years
Normal dystrophin
immunostaining,
Α, β, γ, δ sarcoglycans ans Clinically indistinguishable abnormal
from duchenne-dystrophy immunostaining with
No cardiac involvement sarcoglycans
, Genetic defect
analysis
Myotonic dystrophy AD CTG repeat •Myotonia: hyperexcitability Muscle biopsy showing
expansion in a of muscle membrane mild myopathic changes
gene on chr. 19 inability of quick muscle and grouping of atrophic
relaxation fast fibres
•Progressive muscular
weakness and wasting,
most prominent in cranial
and distal muscles
•Cataracts, frontal balding,
testicular atrophy
•Cardiac abnormalities,
mental retardation
Myotonia congenita AD, AR Mucle cl gene Myotonia (hyperexcitability
Autosomal of the muscle membrane):
dominant form: muscle stiffness and
Thomsen, abnormal muscle
autosomal relaxation, warm-up
recessive form: phenomenon
11/19/2011 Becker Hypertrophied muscles
Jipmer physiologist 67
68. Inflammatory myopathies
PATHOPHYSIOLOGY
• Polymyositis and inclusion body myositis (IBM)
have autoaggressive CD8 lymphocytes that
appear to attack myofibers and suggest an
autoimmune role.
• However,a major question exists about the
etiology of IBM.
• Dermatomyositis is thought to be caused by auto
antibodies, possibly targeting an antigen of the
endothelium. Fiber injury may be caused by
ischemia.
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69. Dermatomyositis Polymyositis Incusion body myositis
Sub acute progressive Sub acute progressive Slowly progressive
weakness weakness weakness,
proximal>distal proximal>distal proximal and distal.
Children and adults, women adults, women adults, mostly men
Characteristic rash and
periorbital heliotrope.
Electromyogram myopathic potentials, myopathic potentials,
myopathic spontaneous spontaneous
potentials, spontaneous activity
Elevated serum creatine Elevated serum creatine Mildly elevated serum
kinase activity. kinase activity creatine kinase or normal.
inflammatory myopathy inflammatory myopathy : inflammatory myopathy
affecting chiefly the endomysium affecting
chiefly the perimysium with
11/19/2011 Jipmer physiologist chiefly the endomysium, but
69
70. Polymyositis Inclusion body myositis Dermatomyositis
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71. Congenital myopathies
• Group of muscle disorders
• Early onset
• Slowly progressive
• Hereditary
• Generalised or proximal weakness and wasting
• Hypotonia
• Contractures
• Normally or mildly elevated CPK
• Normal or myopathic EMG
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72. Congenital Central core disease Nemaline Myotubular
myopathies myopathy myopathy
Inheritence AD AD , AR XL, AD, AR
Gene RYR- 1 gene AD –NEM1 -TMP3 XL – MTM1
AR- NEM2 - NEB AD – DNM2
AR- NEM3 - ACTA AR – BIN1
AR- NEM4 – TMP2
AR- NEM5 -
TNNT1
AR- NEM7 - CFL2
Clinical features Early onset hypotonia Chilhood weakness Severe congenital
and weakness . .variable hypotonia. Floppy
Floppy infant . presentation. infant .poor
Associated skeletal Floppy infant prognosis
deformities
Pathophysiology Cytoplasmic cores Aggregates of Abundance of
are distinct from subsarcolemmal centrally located
surrounding spindle shaped rods nuclei involving the
11/19/2011 Sarcoplasm. Jipmer physiologist majority of muscles
72
79. Treatment
o There is no single treatment for myopathy.
o Treatment of the symptoms to specific cause-
targeting treatments.
o Drug therapy
o Physical therapy
o Bracing for support,
o Surgery
o Massage
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80. Care !….the best rehabilitation method
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