This document compares hypokalemic periodic paralysis (Hypo PP) and hyperkalemic periodic paralysis (Hyper PP). Hypo PP is caused by mutations in the CACNA1S or SCN4A genes and is characterized by acute onset flaccid paralysis, low serum potassium levels, and triggers including high carbohydrate intake and exercise. Hyper PP is caused by mutations in the SCN4A gene and presents with weakness of proximal muscles, normal or high potassium levels, and triggers including rest after exercise and potassium intake. Both involve defective sodium or calcium channels and can be distinguished based on clinical features, laboratory findings, and genetic testing.
Dr Abdullah Ansari
PG-2 (Medicine)
AMU ALIGARH
A general approach to periodic paralysis....
(including hypokalemic periodic paralysis and thyrotoxic periodic paralysis, and other “Channelopathies” or “Membranopathies)
Pathophysiology
Epidemiology
Primary or familial periodic paralysis
Secondary periodic paralysis
Conventional classification of periodic paralysis
Classification of primary periodic paralysis based on ion-channel abnormalities
Clinical approach to a case of periodic paralysis
History of muscle weakness
Age of onset
Family history
Timing
Intensity
History of administration of certain drugs
Clinical examination
Differential Diagnosis
Laboratory investigations
Serum K+
CPK and serum myoglobin
ECG
EMG
Nerve conduction studies
Provocative Testing
Muscle biopsy
Treatment
Prognosis
Dr Abdullah Ansari
PG-2 (Medicine)
AMU ALIGARH
A general approach to periodic paralysis....
(including hypokalemic periodic paralysis and thyrotoxic periodic paralysis, and other “Channelopathies” or “Membranopathies)
Pathophysiology
Epidemiology
Primary or familial periodic paralysis
Secondary periodic paralysis
Conventional classification of periodic paralysis
Classification of primary periodic paralysis based on ion-channel abnormalities
Clinical approach to a case of periodic paralysis
History of muscle weakness
Age of onset
Family history
Timing
Intensity
History of administration of certain drugs
Clinical examination
Differential Diagnosis
Laboratory investigations
Serum K+
CPK and serum myoglobin
ECG
EMG
Nerve conduction studies
Provocative Testing
Muscle biopsy
Treatment
Prognosis
Approach to a case of Fever with altered sensoriumRoy Shilanjan
A brief description about the possible d/d of fever with alteration of sensorium and how to approach the diagnosis through systematic yet focused history taking , physical examination and lab and radiological investigations.
Approach to a case of Fever with altered sensoriumRoy Shilanjan
A brief description about the possible d/d of fever with alteration of sensorium and how to approach the diagnosis through systematic yet focused history taking , physical examination and lab and radiological investigations.
metabolic acidosis develops because of defects in the ability of the renal tubules to perform the normal functions required to maintain acid-base balance.
Epileptogenesis is the process by which a brain network that was previously normal is functionally altered toward increased seizure susceptibility, thus having an enhanced probability to generate spontaneous recurrent seizures (SRSs). The process of epileptogenesis occurs in 3 phases: the occurrence of a precipitating injury; a 'latent' period of epileptogenesis and chronic, established epilepsy. Structural and molecular changes associated with epileptogenesis include selective neuronal loss,axonal and dendritic reorganisation, neurogenesis, altered expression of neurotransmitters, and changes at glial architecture. Antiepileptogenesis can be complete or partial. Complete prevention aborts the development of epilepsy while partial prevention can delay the development of epilepsy or reduce its severity. Targeting signaling pathways that alter the expression of genes involved in epileptogenesis may provide novel therapeutic approaches for preventing epileptogenesis. The mTOR and REST pathways are exciting new potential targets for intervention in the epileptogenic process.
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
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
- 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
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
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.
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
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
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
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.
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.
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.
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!
7. Hyperkalemic Periodic Paralysis
• term hyperkalemic is misleading since patients
are often normokalemic during attacks.
• Onset first decade
• M : F 1:1
• Attacks are brief and mild, usually lasting 30
minutes to 4 hours.
• Weakness affects proximal muscles, sparing
bulbar muscles.
• Attacks are precipitated by rest following exercise
and fasting.
8. • In a variant of this disorder, the predominant
symptom is myotonia without weakness
(potassium-aggravated myotonia).
• The symptoms are aggravated by cold, and
myotonia makes the muscles stiff and painful.
• Clinically apparent myotonia is seen less than
20% of patients, but electrical myotonia may
be found in 50-75%.
9. Pathophysiology
In hyperKPP, Na+ channels fail to inactivate and
prolonged openings and depolarization result.
Increased extracellular K+ levels worsen the
inactivation of Na+ channels
10. INVESTIGATIONS
• Potassium may be slightly elevated but may also
be normal during an attack.
• NCV reduced motor amplitudes and In between
attacks of weakness, the conduction studies are
normal.
• EMG may be silent in very weak muscles.
• often demonstrate myotonic discharges during
and between attacks.
• Muscle biopsy shows vacuoles that are smaller,
less numerous, and more peripheral compared to
the hypokalemic form or tubular aggregates.
11. TREATMENT
• For patients with
frequent attacks,
acetazolamide (125–
1000 mg/d) is helpful.
• mexiletine is helpful in
patients with significant
myotonia.
12. HYPOKALEMIC HYPERKALEMIC
PREVELANCE 1:100,000 1:200,000
AGE OF ONSET FIRST AND SECOND
DECADE OF LIFE
FIRST DECADE
SYMPTOMS
DURING ATTACKS
ACUTE ONSELT
FLACCID PARALYSIS
PROXIMAL >>>
DISTAL
WEAKNESS OF
PROXIMAL
MUSCLE,SPARING
BULBAR MUSCLE
SYMPTOMS
BETWEEN ATTACKS
ASYMPTOMATIC ASYMPTOMATIC
FREQUENCY Daily to yearly May be 2–3/d
DURATION 2–12 h From 1–2 h to >1 d
13. HYPOKALEMIC HYPERKALEMIC
Effect of muscle
cooling
No change Increased myotonia
TRIGGERS HIGH
CARBOHYDRATE,
HIGH SALT,
DRUGS-BETA
AGONISTS, INSULIN
REST FOLLOWING
PROLONGED
EXERCISE
REST AFTER
EXERCISE
STRESS
FATIGUE
FOOD HIGH IN
POTASSIUM
ALCOHOL
INFECTION
POSTASSIUM
SUPPLEMENTATION
TREATMENT PROVOCATIVE TEST
14. SERUM
POTASSIUM
CONCENTRATION
LOW HIGH, NORMAL
ECG HYPOKALEMIC
CHANGES
HYPERKALEMIC CHANGES
CHANGES
MUSCLE BIOPSY SINGLE OR MULTIPLE
CENTRALLY PLACED
VACUOLES
SMALLER, LESS
NUMEROUS
PERIPHERALLY PLACED
VACUOLES
NERVE CONDUCTION TEST REDUCED
AMPLITUDE OF
ACTION POTENTIAL
REDUCED AMPLITUDE OF
ACTION POTENTIAL
ELECTROMYGRAPHY ELECTRICALLY SILENT ELECTRICALLY SILENT
MYOTONIC DISCHARGE
BETWEEN ATTACKS
GENETIC STUDY CALCL1A3, SCN4A SCN4A
18. OUTLINE
• DIFFERENTIAL
DIAGNOSIS OF AIDP
• PRESENTATON OF AIDP
• CAUSES OF PERIODIC
PARALYSIS
• PRESENTATION OF
HYPOKALEMIC PP
• COMPARISON
BETWEEN AIDP Vs
Hypo PP
21. AIDP
• The most common acute neuromuscular disease seen in the intensive care
unit is GBS
Epidemiology
• incidence 2 /100,000/year.
• Sex
• M/F 1.1-1.7:1
• Age
• 2 months to 95 years
• Average 15-35 years
• Childhood GBS average age is 4-8 years
22. Antecedent events (causes)
• 60-70% cases –post infectious .
– 1-3 weeks after an acute infectious process respiratory or
GIT.
– 20-30% cases –campylobacter jejuni
– Other agents –HHV (EBV,CMV)
– Mycoplasma pneumoniae
• Recent immunisation –swine influenza vaccine,older
rabies vaccine (nervous system)
• Can be seen in patients with lymphoma,HIV
positive,SLE.
23. Presentation
• FEVER AND CONSTITUTIONAL SYMPTOMS ARE ABSENT AT THE ONSET
AND IF PRESENT ,CAST DOUBT ON DIAGNOSIS.
• Progressive weakness usually begins in the feet. at
presentation, 60% have weakness in all 4 limbs. usually
symmetric.
• GBS with a descending pattern of weakness seen in 14%
cases.
• Paresthesias often precede the onset of weakness by 1 or
more days. Often gait ataxia with distal limb
paresthesias. Pain, temp relatively spared.
• At presentation half have some facial weakness
• Ophthalmoparesis see in 10-20% of patients. (around
10%.Dyck and Thomas ). Abducens palsy most common;
usually bilateral.
24. Oropharyngeal weakness present in almost 50% of
cases
>1/3 require mechanical ventilation
Areflexia: 70% at presentation and eventually in all
58 to 76% of patients have sensory NCS abnormalities
(Oh et al, NEUROLOGY 2001)
Autonomic dysfunction two thirds of patients. most
common is sinus tachycardia. Retention of urine 1/3
cases, GI dysmotility 15% (Semin Neurol 2008)
plateaus 50% in 2 weeks, over 90% by 4 weeks
Improvement usually begins 1-4 weeks after the plateau.
25. IMMUNOPATHOGENESIS
• An autoimmune basis.
• Both cellular and humoral immunity involved.
• T cells activation –IL2,IL2 receptor,IL-6,TNF alpha,IFN gamma.
• All GBS results from immune responses to non self
anitgnes(infectious agents,vaccines) that misdirect to host
nerve tissue through a resemblance of epitope(molecular
mimicry).
• Neural targets are gangliosides.
• Anti ganglioside ab –GM1 (20-50% cases of C.jejuni)
• Anti GQ1b ab - >90% MFS
26.
27. GM1 on nerve, nodes of ranvier
Anti GB1 ab as part of
molecular mimicry
Complement mediated injury at
Paranodal axon – glial junction
Disrupts the cluster of sodium
channels
Conduction block
Flaccid paralysis
28. Asbury Criteria for diagnosis
REQUIRED :
• 1.progressive weakness of 2 or more limbs due to neuropathy.
• 2.areflexia
• 3.disease course < 4 weeks
• 4 exclusion of other causes (vasculitis,PAN,SLE,churg strauss
syndrome,toxins,lead,botulism,diptheria,porphyria.,cauda equinal
syndrome)
SUPPORTIVE:
• 1.relatively symmetric weakness
• 2.mild sensory involvement
• 3.facial nerve or other cranial nerve involvement
• 4.absence of fever
• 5.typical CSF profile(aceelualr,increase in protein level)
• 6.electrophysiologic evidence of demyelination
29. Variants
• MFS: most common variant, ophthalmoplegia, areflexia, and ataxia usually in
adults, also common in children. Most have GQ1b Ab
• Regional variants : eg pharyngeal-cervical-brachial weakness are rare (acute
progression of oropharyngeal, neck, and shoulder weakness. facial palsy,
blepharoptosis, no sensory disturbance, preserved DTR in the legs, elevated CSF
protein and denervation and decreased conduction velocity on EMG, reported in
1986 by Ropper )
• Pure pandysautonomia usually no weakness, many have areflexia
• AMAN China, developing countries. weakness only. little or no demyelination or
inflammation, more prevalent in kids
• AMSAN
30. Work up
• ESR and SE are normal ,Sometimes Liver enzymes
are elevated
Albuminocytologic dissociation on CSF
• protein > 55mg/dl
• cells <10 mononuclear leukocytes/ml
• 2/3 in 1st week, 82% have it by 2 weeks after
symptom onset.
• no association with clinical severity.
• Some have oligoclonal bands or Myelin basic protein
31. • Early on, NCS often normal.
• 90% are abnormal within 3 weeks of onset.
• 3 of the 4 NCS criteria = clear primary demyelinating neuropathy
(Cornblath)
• Reduced conduction velocity
• Conduction block or abnormal dispersion
• Prolonged distal latencies
• Prolonged F-waves
• Autonomic tests
• PFT: FVC < 20 mL/kg ICU; FVC< 15mL/kg or NIF<-25 Intubation
• Nerve biopsy if prolonged clinical course.
32. Treatment
• Initiate as soon as possible.
• Each day counts 2 weeks after the first motor symptoms –
immunotherapy is no longer effective.
• IVIg IVIg-first choice,easy to administer Five daily infusions
2g/kg body weight
• Plasmapheresis - 40-50ml/kg four times a week
• Combination is not effective
• Treatment reduces need for ventilation by half.,increases full
recovery at an year.
• Glucocorticoids are not effective in GBS.
• Conservative management in mild cases.
33. Prognosis
• total recovery in adults around 75%
• during the early stage of GBS, increasing severity in neurologic
disability scores ,cranial nerve involvement, urinary
incontinence, respiratory signs, and the need for ventilator
support are associated with poor prognoses
• Low CMAP amplitudes (< 20% of normal) bad prognostic
indicator.
• 10% may have a relapse in 1-6 weeks after completing
immunomodulatory therapy
• 15% end up with significant neurological residuals
• Mortality 2-6 %
36. HYPOKALEMIC PERIODIC PARALYSIS
MUTATED GENE CALCL1A3 SCN4A
CHROMOSOME 1q31 17q
DEFECTIVE
CHANNEL
CALCIUM SODIUM
MODE OF
INHERITENCE
AUTOSOMAL DOMINANT
TYPE 1 TYPE 2
37. HYPOKALEMIC PERIODIC PARALYSIS
PREVELANCE 1:100,000, AD
AGE OF ONSET FIRST AND SECOND DECADE OF
LIFE
M:F 3 or 4:1
SYMPTOMS DURING ATTACKS Occur anytime of the day; more
common in morning
Absence of myotonia
Proximal > distal weakness; legs
> arms
Sparing of facial, ventilatory
and sphincter muscles
Lasts several hours to more
than a day
38. HYPOKALEMIC PERIODIC PARALYSIS
SYMPTOMS BETWEEN ATTACKS REGAIN FULL STRENGTH
BETWEEN ATTACKS
TRIGGERS HIGH CARBOHYDRATE,HIGH
SALT,
DRUGS- BETA AGONISTS,
INSULIN
REST FOLLOWING PROLONGED
EXERCISE
FEVER /LACK OF SLEEP/STRESS
ETOH consumption
39. SERUM POTASSIUM
CONCENTRATION
LOW
ECG U waves, flattening of T
waves
MUSCLE BIOPSY SINGLE OR MULTIPLE
CENTRALLY PLACED
VACUOLES
NERVE CONDUCTION TEST REDUCED AMPLITUDE OF
ACTION POTENTIAL
ELECTROMYGRAPHY ELECTRICALLY SILENT
40. TREATMENT ORAL KCL
SUPPLEMENTATION
KCL VIA INFUSION
DONOT GIVE IN DEXTROSE
PROPHYLAXIS ACETAZOLAMIDE
(125-1000 Mg)
PROGNOSIS USUALLY GOOD
RARE DEVELOPMENT OF
PROXIMAL MYOPATHY
*Never forget to measure the thyroid hormones.
42. • Frequency Of Attacks : highly variable
• Frequency decreases after age 30; may become
attack free in 40s and 50s
• Permanent fixed weakness or slowly progressive
weakness more common with HypoKPP1
42
PROGNOSIS
43. SUMMARY
• 1.acute rapidly evolving areflexic ascending motor paralysis
with or without sensory disturbances.
• 2.fever is absent at the onset of weakness
• 3.bladder involvement in severe cases –transient
• 4.campylobacter jejuni 20-30 % cases
• 5.autoimmune basis ,molecular mimicry
• 6.anti GM1 ab (MC),anti GD1a,anti GQ1b (MFS)
• 7.autonomic involvement is common
• 8.facial nerve is the one most commonly affected,optic nerve.
• 9.30% require ventilatory support.
44. • 10.course is usually < 4 weeks
• 11 .typical CSF profile shows high protein,no pleocytosis
• 12.electrographically conduction block present
• 13.treatment as soon as possible
• 14.IVIg,plasmapheresis –both are equally good
• 15.glucocorticoids are not effective in GBS
• 16.think of miller fischer variant in presence of
ophthalmoplegia,ataxia,areflexia.
45. HYPOKALEMIC AIDP
PREVELANCE 1:100,000 AD M>>>F 2:100,000 S M>F
AGE OF ONSET FIRST AND SECOND
DECADE OF LIFE
ANY AGE SROUP[MC 2-
4]
SYMPTOMS ACUTE ONSELT FLACCID
PARALYSIS
PROXIMAL >>> DISTAL
WITHOUT SENSORY
COMPLAINTS BUT BODY
ACHE PRESENT
ASCENDING WEAKNESS
OF PROXIMAL AND
DISTALMUSCLES WITH
PARASTHESIAS AND
PAIN
CRANIAL
NERVE/BULBAR/RESPIR
ATORY MUSCLE
INVOVLMENT
RARELY SEEN
BLADDER/BOWEL- NOT
SEEN
AUTONOMIC
DYSFUNTION - ABSENT
COMMON++++
URINARY RETENTION +
CONSTIPATION +
COMMONLY+++
TRIGGERS HIGH CARBOHYDRATE,
HIGH SALT,FEVER
DRUGS-BETA
AGONISTS, INSULIN
REST FOLLOWING
PROLONGED EXERCISE
H/O OF FEBRILE ILLNESS
WITH GIT/RS
INVOVLMENT PRIOR TO
WEAKNESS IN 66%.H/O
VACINATIONS+
46. HYPOKALEMIC AIDP
COURSE Episodic
Lasts several hours to
more than a day
AttacksFrequency :
highly variable
Frequency decreases
after age 30; may
become attack free in
40s and 50s
Permanent fixed
weakness or slowly
progressive weakness
more common with
HypoKPP1
Progressive initially
plateaus 50% in 2
weeks, over 90% by 4
weeks Improvement
usually begins 1-4
weeks after the plateau
3 attacks or >8 weeks -
CIDP
47. SERUM
POTASSIUM
CONCENTRATION
LOW NORMAL
ECG U waves, flattening of
T waves
TACHYCARDIA 2nd or 3rd
degree conduction
block, QRS
prolongation and T
wave abnormality
MUSCLE BIOPSY SINGLE OR MULTIPLE
CENTRALLY PLACED
VACUOLES
NI/NORMAL
NERVE CONDUCTION
TEST/EMG
REDUCED
AMPLITUDE OF
ACTION
POTENTIAL/EMG
ELECTRICALLY SILENT
Primary
demyelinating
neuropathy
CSF EXAM NI/NORMAL Albuminocytologic
dissociation on CSF