This document provides guidelines for the comprehensive management of patients with amyotrophic lateral sclerosis (ALS). It discusses presenting the diagnosis, specific pharmacotherapies including riluzole and edaravone, symptomatic treatments, multi-disciplinary care, physical and speech rehabilitation, nutritional management, respiratory care, and end-of-life care. Key recommendations include using riluzole to prolong survival, considering non-invasive ventilation to prolong survival and slow respiratory decline, and providing home or hospice care for terminally ill patients to maintain dignity.
Physiotherapy in MND
Dr. Quazi Ibtesaam Huma (MPT)
Dr. Suvarna Ganvir (Phd, Prof & HOD)
Dept. of Neurophysiotherapy
DVVPF’s College of Physiotherapy
Content
Introduction
Types of MND
Clinical Features of MND
Diagnostic Procedure
Management: 1) Pharmaceutical
2) Physiotherapy
Motor Neuron Disease
Motor Neuron Disease are a group of neurodegenerative disorders that affects the nerves in the spine and brain to progressively lose its function.
Motor neuron diseases (MND) include a heterogeneous spectrum of inherited and sporadic (no family history) clinical disorders of the upper motor neurons (UMNs), lower motor neurons (LMNs), or a combination of both.
Types of MND
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized by progressive degeneration of motor neurons in the spinal cord, brain stem, and motor cortex, leading to progressive muscle atrophy and weakness.
Clinical Features
UPPER MOTOR NEURON
Loss of Dexterity
Muscle Weakness
Spasticity
Hyperreflexia
Pathological reflexes
LOWER MOTOR NEURON
Muscle Weakness
Muscle Atrophy
Hypotonicity
Hyporeflexia
Fasciculation
Muscle Cramp
Impairment related to LMN
Other clinical features
Diagnostic Criteria
Diagnostic Procedure
EMG-
It include signs of active denervation, such as fibrillation potentials and positive sharp waves;
Signs of chronic denervation, such as large motor unit potentials (increased duration, increased proportion of polyphasic potentials, increased amplitude)
Unstable motor unit potential
Nerve Conduction Velocity Studies,
Muscle And Nerve Biopsies,
Neuroimaging Studies - MRI
Management- Multidisciplinary Approach
Physical Therapy Examination
Cognition
Pain
Psychosocial Function
Joint integrity, ROM and Muscle strength.
Motor Function: Gross motor and Fine motor
Muscle tone and reflexes
Cranial nerve integrity
Sensations
Gait
Respiratory Function
Physiotherapy goals in MND treatment.
Pain reduction
Prevention for contractures
Maintenance of joint mobility
Regular review of posture
Positioning to relieve discomfort
House Modification and ergonomic advice.
Management of Sialorrhea and Pseudobulbar Affect
Management for Dysphagia
PEG procedure.
A PEG may be recommended as the disease progresses.
A PEG is a type of gastrostomy tube inserted via endoscopic surgery that creates a permanent opening into the stomach for the introduction of food.
Studies have found that PEG insertion may prolong survival. Patients with PEG were found to live 1 to 4 months longer than those individuals who refused it.
Management of Dysphagia
A palatal lift prosthesis may be prescribed for individuals with good articulation but who have a breathy voice quality or decreased loudness because of excessive air loss through the nose.
The device, a dental appliance designed to attach to the existing teeth and to elevate the soft palate, is custom-made by a prosthodontist.
Physiotherapy in MND
Dr. Quazi Ibtesaam Huma (MPT)
Dr. Suvarna Ganvir (Phd, Prof & HOD)
Dept. of Neurophysiotherapy
DVVPF’s College of Physiotherapy
Content
Introduction
Types of MND
Clinical Features of MND
Diagnostic Procedure
Management: 1) Pharmaceutical
2) Physiotherapy
Motor Neuron Disease
Motor Neuron Disease are a group of neurodegenerative disorders that affects the nerves in the spine and brain to progressively lose its function.
Motor neuron diseases (MND) include a heterogeneous spectrum of inherited and sporadic (no family history) clinical disorders of the upper motor neurons (UMNs), lower motor neurons (LMNs), or a combination of both.
Types of MND
Amyotrophic Lateral Sclerosis
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized by progressive degeneration of motor neurons in the spinal cord, brain stem, and motor cortex, leading to progressive muscle atrophy and weakness.
Clinical Features
UPPER MOTOR NEURON
Loss of Dexterity
Muscle Weakness
Spasticity
Hyperreflexia
Pathological reflexes
LOWER MOTOR NEURON
Muscle Weakness
Muscle Atrophy
Hypotonicity
Hyporeflexia
Fasciculation
Muscle Cramp
Impairment related to LMN
Other clinical features
Diagnostic Criteria
Diagnostic Procedure
EMG-
It include signs of active denervation, such as fibrillation potentials and positive sharp waves;
Signs of chronic denervation, such as large motor unit potentials (increased duration, increased proportion of polyphasic potentials, increased amplitude)
Unstable motor unit potential
Nerve Conduction Velocity Studies,
Muscle And Nerve Biopsies,
Neuroimaging Studies - MRI
Management- Multidisciplinary Approach
Physical Therapy Examination
Cognition
Pain
Psychosocial Function
Joint integrity, ROM and Muscle strength.
Motor Function: Gross motor and Fine motor
Muscle tone and reflexes
Cranial nerve integrity
Sensations
Gait
Respiratory Function
Physiotherapy goals in MND treatment.
Pain reduction
Prevention for contractures
Maintenance of joint mobility
Regular review of posture
Positioning to relieve discomfort
House Modification and ergonomic advice.
Management of Sialorrhea and Pseudobulbar Affect
Management for Dysphagia
PEG procedure.
A PEG may be recommended as the disease progresses.
A PEG is a type of gastrostomy tube inserted via endoscopic surgery that creates a permanent opening into the stomach for the introduction of food.
Studies have found that PEG insertion may prolong survival. Patients with PEG were found to live 1 to 4 months longer than those individuals who refused it.
Management of Dysphagia
A palatal lift prosthesis may be prescribed for individuals with good articulation but who have a breathy voice quality or decreased loudness because of excessive air loss through the nose.
The device, a dental appliance designed to attach to the existing teeth and to elevate the soft palate, is custom-made by a prosthodontist.
-Detailed Introduction, Patho-physiology, Evaluation & Physiotherapy Management of Parkinsonism.
-Clinical classification is discussed.
-Various measures of evaluation and physical therapy is discussed in this.
-Detailed Introduction, Patho-physiology, Evaluation & Physiotherapy Management of Parkinsonism.
-Clinical classification is discussed.
-Various measures of evaluation and physical therapy is discussed in this.
DEFINITION
Pulmonary rehabilitation is a restorative and preventive process for patients with chronic respiratory disease. It is defined as a “multi-disciplinary program of care for patients with chronic respiratory impairment that is individually tailored and designed to optimize physical and social performance and autonomy.”
Consequences of Respiratory Disease
Peripheral Muscle dysfunction
Respiratory muscle dysfunction
Nutritional abnormalities
Cardiac impairment
Skeletal disease
Sensory defects
Psychosocial dysfunction
ASSESSMENT
At the start of the pulmonary rehabilitation program, your medical history will be obtained and your fitness level will be assessed, usually by doing a walking test. From this assessment, an exercise program will be set for you at your fitness level.
Another assessment will be completed at the end of the program.
Chart Review
Patient examination
medical history
Family history
Social history
Signs & symptoms
Patient Interview (1)Use of tobacco, alcohol, and nonprescription drugs
• Usual activity level, including employment, recreation, and home
• Regularity of exercise, including availability of equipment at home)
2)The nutritional evaluation should include the following:
• Weight• Height• Calculation of BMI• Documentation of recent weight change
The latest guidelines on the management of a COPD patient ( Stable COPD, patient with an exacerbation of COPD), latest modalities of treatment of a COPD patient
Geriatrics is a sub-specialty of medicine that focuses on health care of the elderly. It aims to promote health and to prevent and treat diseases and disabilities in older adults.
Similar to Management of motor neuron disease (20)
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
- 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
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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.
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
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Title: Sense of Taste
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 structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
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.
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.
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.
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
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,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
1. D R . S A C H I N A D U K I A
Management of MND as per
guidelines
2. INTRODUCTION
Neuro-degenerative disorder which affects the motor neurons in the motor
cortex, spinal cord and brainstem
Development of clinical weakness till about 30% AHC are lost
Rostral to caudal progression is faster than otherwise.
There can be transitory improvement, plateau or sudden worsening.
3. INTRODUCTION
mean 5 year survival rate is around 22%
mean 10 year survival rate is around 10%
Most robust poor prognostic factors are
Older age at onset
Bulbar onset
Short interval between onset and clinical diagnosis
Rapid progression rates
Low BMI
FTD-ALS presentation
Rapid decline in respiratory function
4. COMPREHENSIVE MANAGEMENT OF
PATIENT WITH ALS
Presentation of the diagnosis
Specific pharmacotherapy
Symptomatic treatment
Multi-disciplinary team approach
Physical rehabilitation
Speech and communication management
Nutritional care
Respiratory care
Home and hospice care
5. Breaking the news
No RCTs available to conclude ideal method.
Following methods can be considered:
Compassionate yet informative manner
Allow adequate time
Patient should not be alone
Arrange second appointment for queries and after thoughts
Provide information about latest research, clinical trials
Advanced directives and issues about terminal care
6. SPECIFIC PHARMACOTHERAPY
Riluzole: antiglutamate agent
Approved by U.S FDA in 1996
A Cochrane meta-analysis : 100mg/d results in 9% increase in probability of
survival for 1 year and prolongs median survival by 2-3 months when taken for
18 months(Miller et al, 2012)
7. riluzole 50 mg BID to prolong survival for those with definite or probable ALS
less than 5 years duration, with forced vital capacity (FVC) 60%, and without
tracheostomy (Level A).
modest beneficial effect in slowing disease progression (prolonged survival of
2–3 months) based on 4 Class I trials.
should be offered to slow disease progression in patients with ALS (Level A).
8. Edaravone was approved in May 2015 for the treatment of ALS by FDA
IV Edaravone 60 mg over 60 minutes
Treatment is started with daily infusion for 14 days, followed by 14 days off
treatment.
Subsequent daily edaravone 60 mg infusions on 10 days within a 14 day
period, followed by 14 days off treatment.
9. SYMPTOMATIC MANAGEMENT
What are the most effective treatments for sialorrhea?
Agents like
Hyoscyamine sulphate
Diphenhydramine
Scopolamine patch
Glycopyrrolate
Atropine
TCA
In medically refractory sialorrhea,
Botox should be considered (Level B)
low-dose radiation therapy to salivary glands may be considered (Class I, Level
C).
10. For pseudobulbar affect
combination of Dexmethorphan / Quinidine sulphate is probably effective (1
Class I study)
Pending FDA approval (Level B).
11. To reduce fatigue?
Agents:
Pyridostigmine
Antidepressants
Methylphenidate
Amantadine
Modafinil
There are no controlled studies of pharmacologic agents relieving fatigue in
ALS.
Riluzole possibly causes fatigue in some patients (2 Class III studies).
12. Other measures
Energy conservation
Work modification
BiPAP if sleep study is abnormal.
13. To reduce cramps?
Agents
Quinine sulphate
Baclofen
Vitamin E
Clonazapam
Other therapies
Massage
Physical therapy
14. Studies of gabapentin, vitamin E, and riluzole for treating cramps were all
negative (Class III).
There are safety concerns about quinine.
There are insufficient data to support or refute any specific intervention for the
treatment of cramps in ALS (Level U).
16. Evidence is insufficient to recommend exercise or medication for treating
spasticity in ALS (Class III).
There are insufficient data to support or refute exercise or medication for
treating spasticity in ALS (Level U).
17. To reduce depression?
Agents:
TCAs
SSRIs
Venlafaxine
Mirtazipine
Bupropion
Other therapies
Counselling
Support group meetings
No controlled trials available
18. To reduce insomnia?
Agents
Zolpidem
Lorazepam
Opioids
TCAs
Other therapies
Air mattresses
NPPV
No clinical trials available
19. COGNITIVE AND BEHAVIOURAL
IMPAIRMENT
Specific ALS phenotypes include
Pure motor degeneration (ALS)
ALS with cognitive impairment (ALSci)
ALS with behavioral impairment (ALSbi)
ALS with a dementia meeting the Neary criteria for frontotemporal dementia
(FTD) (ALS-FTD).
20. A significant proportion of patients with ALS demonstrate cognitive
impairment and some have dementia (2 Class II, multiple Class III studies).
Neither behavioral impairment in ALS nor the natural progression of cognitive
or behavioral impairments has been adequately studied.
Screening for cognitive and behavioral impairment should be considered in
patients with ALS (Level B).
21. SPEECH AND COMMUNICATION MANAGEMENT
Speech pathologists should assess speech and communication immediately
after establishing diagnosis.
Non verbal techniques like
gestures,
body language alphabet and picture boards,
voice synthesizer can be used.
22. PHYSICAL REHABILITATION
Objective is to improve the ability to carry out activities of daily living as far as
possible without causing physical or emotional strain.
Exercises to enhance strength, endurance and range of motion, occupational
therapy.
Adaptive and assistive devices like walkers, wheelchairs, splints and collars.
Modification of home environment.
23. NUTRITION MANAGEMENT
In ALS, factors that restrict adequate nutrition develop insidiously and
progressively worsen.
functional consequences are choking, aspiration, weight loss, and dehydration.
Strategies to maintain oral nutritional intake consist of altering food
consistency and using nutritional supplements.
(PEG) or radiologically inserted device [RIG]) - an alternative route for
delivering nutrition
PEG does not eliminate oral feeding but offers a convenient method for
administering medication and fluid and stabilizing weight.
24.
25. What is the efficacy of vitamin and nutritional supplements on prolonging
survival or quality of life?
Creatine should not be given as treatment for ALS because it is not effective in
slowing disease progression (Level A).
High-dose vitamin E should not be considered as treatment for ALS (Level B),
while the equivocal evidence regarding low-dose vitamin E permits no
recommendation (Level U).
26. RESPIRATORY MANAGEMENT
The diagnosis and management of respiratory insufficiency is critical because
most deaths from ALS are due to respiratory failure.
27.
28. What are the optimal pulmonary tests to detect respiratory insufficiency?
Nocturnal oximetry may be considered to detect hypoventilation (regardless of the
FVC) (Level C).
Supine FVC and MIP may be considered useful in routine respiratory monitoring,
in addition to the erect FVC (Level C).
Sniff nasal pressure may be considered to detect hypercapnia and nocturnal
hypoxemia (Level C)
29. Does NIV improve respiratory function or increase survival?
NIV is probably effective in prolonging survival (1 Class I, 3 Class III studies)
and in slowing the rate of FVC decline (1 Class I, 1 Class III study).
NIV should be considered to treat respiratory insufficiency in ALS, both to
lengthen survival and to slow the rate of FVC decline (Level B).
30. What is the efficacy of targeted respiratory interventions for clearing secretions
Expiratory respiratory muscle weakness can lead to ineffective cough, retained
upper airway secretions, and pulmonary infection. PCEFs greater than 160
L/min are needed to clear secretions and clinicians recommend assistive
devices when the PCEF falls below 270 L/min (Class III).
Mechanical insufflation/exsufflation (MIE) increased the PCEF by 17% in
healthy controls, 26% in bulbar patients, and 28% in nonbulbar patients (Class
III).
Manually assisted cough increased flow by 11% in bulbar and 13% in nonbulbar
patients.
31. HOME CARE AND HOSPICE CARE FOR TERMINALLY ILL
When the patient is terminally ill, home or hospice care is required.
(Mitsumoto et al, 2005)
Close collaboration between patients, care givers, nurses and the ALS team
maintain independence and dignity of the patient in the terminal stages.
32. References
Miller RG, Jackson CE, Kasarskis EJ, et al. Practice parameter update: the care of
the patient with amyotrophic lateral sclerosis: drug, nutritional, and respiratory
therapies (an evidence-based review): report of the Quality Standards
Subcommittee of the American Academy of Neurology. Neurology 2009; 73:1218.
FDA approves drug to treat ALS. https://www.fda.gov/NewsEvents
/Newsroom/PressAnnouncements/ucm557102.htm
Hardiman O, van den Berg LH. Edaravone: a new treatment for ALS on the
horizon? Lancet Neurol 2017; 16:490.