This document provides an overview of approaching a patient with peripheral neuropathy. It discusses:
- The global burden of peripheral neuropathy, with diabetes being a leading cause.
- The basic anatomy of peripheral nerves and pathogenic mechanisms of nerve damage, including segmental degeneration, Wallerian degeneration, and axonal degeneration.
- Key questions to ask patients regarding the systems involved, duration and nature of symptoms, evidence of upper motor involvement, temporal evolution, potential hereditary causes, and other medical conditions.
- Types of neuropathy classified by pattern of involvement, clinical course, and other factors to help differentiate diagnoses.
Dr Neerav Goyal discusses the various aspects of acute liver failure that includes the criteria, pre transplant issues, critical care management, overall survival.
Sudden temporary change in PHYSICAL movement, SENSATION, BEHAVIOUR because of abnormal discharged of electrical impulses from nerve cells.
CLASSIFICATION
PARTIAL SEIZURE / FOCAL SEIZURE
>> Aimed to determine:
Type of seizure
Frequency
Severity
Aura
LOC
Dyspnea
Fixed and dilated pupil
Incontinence
Factors that precipitate them.
Developmental history taking (events of pregnancy and childbirth)
Questioned about illnesses or head injury
Management Of Chronic Hepatitis B
by Dr S Khan
Courtesy Of Javed iqbal Farooqi
http://www.drkhanblogs.com/2015/05/management-of-chronic-hepatitis-b.html
A Clinical Case wherein patient presented with signs & symptoms of Fanconi Syndrome, on further evaluating the history; it was found that he matched the clinical criteria for Lowe Syndrome
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
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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
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
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
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
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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.
Report Back from SGO 2024: What’s the Latest in Cervical Cancer?
Approach to a patient with peripheral neuropathy
1. APPROACH TO A PATIENT
WITH PERIPHERAL
NEUROPATHY
BY:
Dr. Tikal Kansara
R2 Medicine D Unit
2. GLOBAL BURDEN
• Overall prevalence – 2 to 4 %
– But increases to 8 % in patients over 55 years of
age (Does not include traumatic neuropathies)
• In developed world, Diabetes Mellitus is the
most common cause (Dutch study of 362,693
patients – mononeuropathy (4.3/1000/year), carpel
tunnel syndrome (2.3/1000/year), diabetic neuropathy
(0.72/1000/year), post herpetic neuralgia
(0.42/1000/year)
• In developing countries, leprosy is the most
common cause
• In India, with rise in incidence of diabetes,
diabetic neuropathy is also likely to increase
3. • Over 100 causes of neuropathy
• Among several sudies, around 20 to 50 %
patients remain undiagnosed
5. Components of a Peripheral Nerve
• Peripheral Nerves including the cranial nerves
(except the Ist and IInd)
• Spinal Nerve Roots
• Dorsal Root Ganglia
• Peripheral Nerve trunk with its terminal
branches
• Peripheral Autonomic Nervous System
8. Components of a Peripheral Nerve
Inflammation of interstitium
Necrotising panarteritis
Endoneural
amyloid
deposition
Vasculitis,
ischemic
infarctions
9.
10. Pathogenic Mechanism of Peripheral
Nerve Damage
• Several changes are identified but are not
disease specific:
– Segmental Degeneration
– Wallerian Degeneration
– Axonal Degeneration
• Myelin sheath is the most suseptible to
damage. It can break down as a parimary
process afecting Schwann Cells, myelin itself,
or secondarily to the diseases affecting axon.
11. • Focal degeneration of myelin
sheath with sparing of the
axon is called segmental
Degeneration.
• The characteristic change is
the disappearance of sheath
over segments of variable
length, bounded on each end
by a preserved segment of
myelin.
Segmental Degeneration
(Demyelinating)
12. Wallerian Degeneration
• It is the reaction of both
axons and myelin distal
to the site of disruption
of an axon.
• Described as “dying-
forward” phenomenon
13. Axonal Degeneration
• A “dying-back” phenomenon which
typically occurs in a generalised
metabolically determined
polyneuropathy
• Axon affected progressively from distal-
most site to the proximal, with
dissolution of myelin parallel with axon.
Certain toxic and metabolic processes
affect axons uniformly along their
length or impair anterograde axonal
trasnport to the periphery; functional
impairment is proportioanl to the size
and length of axon blocked.
14. • Destruction of proximal spinal root – gradual
destruction of distal motor nerve and its myelin sheath.
(Wallerian degeneration)
• Neuronal motor cell body undergoes characteristic
morphological change but does not die.
• Destruction of dorsal spinal root produces secondary
Wallerian Degneration of posterior columns of spinal
cord, but not of the peripheral sensory nerve.
16. Topography
• Mononeuropathy
–Single peripheral nerve involvemment
• Trauma, compression or entrapement
– Carpel Tunnel Syndrome, Ulnar nerve entrapement,
peroneal nerve entrapement
• Related to pregnancy, thyroid disease or
occupation
• Or due to hereditary disorder liable to pressure
palsy
17. • Mononeuropathy multiplex
– Multiple, separate, noncontiguous peripheral
nerves either simultaneously or sequentially
• Leprosy, Vasculitis (PAN, Churg-Strauss Syndrome, RA,
Sjogren’s Syndrome) and requires urgent diagnosis
• Superficial peroneal or sural nerve palsy often helpful
(Vasculitis)
• Polyneuropathy
– A general degeneration of nerves that spread
typically towards the center of body
18. • Ganglionopathy / Neuronopathy
– Sensory neuronopathies (SN) are specific group
diseases characherised by primary degeneration of
dorsal root ganglia and their projections
– Multifocal sensory symptoms are often associated
with ataxia
• Localised
– Herpes Zoster
• Immune
– Sjogren’s syndrome
– Autoimmune Heptitis
– Celiac Disease
• Idiopathic
– Pan-sensory
– CANVAS (Cerebellar Ataxia, Neuronopathy, Vestibular Areflexia
Syndrome)
• Hereditary
19.
20. Vital Questions to ask for
• What systems are involved?
• What is the duration of weakness?
• What is the nature of sensory involvement?
• Is there evidence of Upper Motor Involvement?
• What is the temporal evolution?
• Is there evidence of hereditary neuropathy?
• Does the patient have any other medical
conditions?
22. Sensory Symptoms
Loss of function
“- symptoms”
Disordered function
“+ symptoms”
Sensory
“Large Fiber”
↓ Vibration
↓ Proprioception
Hyporeflexia
Sensory ataxia
Paresthesias
Sensory
“Small Fiber”
↓ Pain
↓ Temperature
Dysesthesias
Allodynia
23. Motor Symptoms
Loss of function
“- symptoms”
Disturbed function
“+ symptoms”
Motor nerves
Large fibre
Wasting
Hypotonia
Weakness
Hyporeflexia
Orthopedic deformity
Fasciculation
Cramps
•Initially distal motor weakness is common – difficulty in turning
keys of lock, unfasten buttons and opening lids of bottles and jars
•Proximal Motor Weakness – Difficulty in keeping and bringing
down bags in upper parts of closets, hanging shirts, climbing stairs
24. Autonomic Symptoms
Loss of function
“- symptoms”
Disturbed function
“+ symptoms”
Autonomic
nerves
↓ Sweating
Hypotension
Urinary retention
Impotence
Vascular color changes
GI Complaints
↑ Sweating
Hypertension
25. What is the distribution of weakness?
• Distal, proximal or both
• Focal and asymmetric or symmetric
• Symmetric distal and proximal weakness –
Immune demyelinating polyneuropathy (Acute &
Chronic)
• Asymmetric acute or subacute sensory and motor
symptoms and signs – radiculopathies,
compressive mononeuropathies, mononeuritis
multiplex must be coinsidered
26. Is there evidence of upper motor
neuron involvement?
• If symmetric distal sensory symptoms and
signs (distal sensory neuropathy), but
additional symmetric upper motor neuron
involvement – Combined Degeneration
– Vitamin B12 Deficinecy
– Copper Deficiency
– HIV Infection
– Severe Hepatic Disease
27. What is the Temporal Evolution?
• Acute (days to 4 weeks)
– Vasculitis – hyperacute mononeuropathies 24 – 72 hours
– AIDP
• Subacute (4 to 8 weeks)
– CIDP
• Chronic (>8 weeks)
– Diabetes
– Hereditary
• Monophasic, progressive or relapsing
– Monophasic – GBS
– Progressive – Diabetes, CIDP
– Relapsing – Recurrent GBS
28. Is there evidence of Hereditary
Neuropathy?
• Very slowly progressive weakness over many
years – with minimal sensory symptoms but
significant sensory signs – Hereditary
Neuropathy
• Eg. CMT Disease – arch and toe abnormalities
(High or flat arched, hammer toes, etc.)
• Evidence from remote history like funny feet,
unevenly worn shoes, childhood onset
clumsiness especially in sports and tasks that
require fine coordination
29. Does the patient have any other
medical condition?
• Associated medical conditions
– Diabetes
– Systemic Lupus Erythemstosus
– Anaemia
• Preceding or concurrent infections
– Diarrhoea in GBS
• Surgeries
– Gastric bypass and nutritional neuropathies
• Medications
– Toxic Neuropathy
• Alcohol
• Dietary Habits
• Dental Fixatures (zinc leading to copper deficinecy)
30. Past History
• Concurrent illness, particularly organ failure,
endocrine disorders, and connective tissue
disease
• Diabetes, and latent diabetes, should always
be sought.
31. Family History
• Detailed family history is often crucial, and if
negative
– Examination of relatives; atleast tapping the ankle
jerk
– Recording a lower limb motor conduction velocity
• Worthwhile to ask for family history of
porphyria, if present
48. Differencial Diagnosis of Peripheral
Neuropathy / Pitfalls in diagnosis @ nerve
level
• In patients with neuropathic symptoms, spinal
cord is the most common differential
• Spinal Canal Stenosis – intermittent
claudiacation symptoms, but in advanced
stages, may present with persistent symptoms
simulating peripheral neuropathy
• In elderly, co existance of cervical spondylitic
meylopathy with late onset predominantly
sensory axonal neuropathy
49. • In the same way, spondylotic radiculopathies
may co-occur with upper limb entrapement
neuropathies
• As stated before, coexistance with CNS disease
is common, Vitamin B12 deficiency,
neuroacanthocytosis, spinocerebellar
syndromes
• Lacunar stroke may rarely present with
sensory loss in the region of median or ulnar
nerve distribution
53. Neuropathies with less common
patterns of involvement
Neuropathies with cranial nerve involvement
– Diabetes mellitus
– Guillian-Barre Syndrome
– HIV/AIDS
– Lyme disease
– Sarcoidosis
– Neoplastic invasion of skull base or meninges
– Diptheria
– Friedrich’s ataxia
58. Laboratory Tests
• First line screening tests
– Blood counts, ESR
– Blood sugar
– Liver and renal function tests
– Serum Vitamin B12 levels
– Paraprotein levels
– Thyroid funtion tests
– Vasculitis Profile
59. NERVE CONDUCTION STUDIES (NCS)
• NCS is a medical diagnostic test commonly
used to evaluate the function, especially the
ability of electrical conduction, of the motor
and sensory nerves
• Three important conclusions are
– Confirm that the disease process is due to
neuropathy
– Find out subclinical neuropathies
– Prognosticate the condition
60.
61. Evoked Potentials
•Electrical signals passes
recording and reference
electrodes and is shown on
oscilloscope
•Time to onset and amplitude
of the electrical signal is
measured
65. AXONAL
DEGENERATION
SEGMENTAL
SEGMENTATION
Motor Nerve Conduction Studies
CMAP amplitude Decreased Normal
Distal Latency Normal Prolonged
Conduction Velocity Normal Slow
F wave Normal or absent Prolonged or absent
H reflex Normal or absent Prolonged or absent
Sensory Nerve Conduction Studies
SNAP amplitude Decreased Normal or decreased
Distal latency Normal Prolonged
Conduction velocity Normal Slow
66. • If Hx, examination, EDx and the above
mentioned Ix does not reveal a diagnosis,then
– Revise family history or examine the relatives
• Nerve Biopsy – Vasculitis restricted to nerves
or neuropathy of recent origin, amyloid, CIDP
• Anti-Hu antibodies
• CSF
– AIDP, CIDP and other chronic immune-medicated
axonal neuropathies
– Significant pleocytosis shoud raise the suspicion of
Borrelia, Sarcoidosis, HIV
67. • Genetic Testing
– E.g. PMP22 duplication test and others in CMT
– Due to high mutation rate, genetic disorder can
also be considered without a family history
• Anti-ganglioside antibodies elevated in
patients with multifocal motor neuropathy
with conduction block (MMN-CB) in 50%
patients
• Anti-GQ1b IgG – Miller-Fisher Syndrome
• Motor Axonal Variant of GBS – Anti-GM1 and
anti-GDI antibodies
68. • Nerve Biopsy
– Places where electron microscope, teased fibre
technique and immunohistochemistry are
available
– Indications: Suspicion of vasculitis , amyloid
deposition, leprosy, sarcoidosis or immune
neuropathies (eg CIDP)
– Location: superficial sensory nerves are generally
used. Superficial peroneal nerve in legs &
superficial radial nerve or branch of ulnar nerve
• Combined nerve and muscle biopsy may
sometimes be required
75. Diabetic Distal Symmetric Sensory and
Sensorimotor Polyneuropathy (DSPN)
• Begins in toes, gradually progress over time
upto legs and into arms and legs
• When severe, sensory loss also occurs on
trunk, initially over midline anteriorly and
then laterally
• Tingling, burning, deep aching pains occur
76. Diabetic Autonomic Neuropathy
• Abnormal sweating, dysfunctional
thermoregulation, dry eyes and mouth, pupillary
abnormailties, cardiac arrythmias, postural
hypotension, GI abnormalities (gastroparesis,
post prandial bloating, chronic diarrhoea or
constipation) and genitourinary disturbances
(impotence, retrograde ejaculation, incontience)
• Tests for autonomic dysfunction are abnormal –
sympathetic skin responses and quantitative
sudomotor axon reflex testing
77. Diabetic Radiculoplexus Neuropathy
(Diabetic Amyotrophy or Bruns-
Garland Syndrome)
• Severe pain in lower back, hip and thigh in one leg
• Atrophy and weakness of proximal and distal
muscles in the affected leg becomes appatent
within a few days to weeks
• Severe weight loss
• Recovery is slow and partial with residual
weakness, sensory loss, and pain
• ESR, CSF protein are elevated
• Presentation maybe like the typical lumbosacral
radiculoplexus neuropathy, thoracic radiculopathy
or even an uncommon cervical
polyradiculoneuropathy
78. Diabetic Mononeuropathies
• Median neuropathy at wrist
• Ulnar neuropathy at elbow
• Peroneal neuropathy at fibular head
• Cranial Neuropathies
– Seventh cranial nerve
– Third cranial nerve palsy (typically pupil sparing)
79. HIV
• HIV- related distal symmetric polyneuropathy
• HIV – related inflammatory demyelinating
polyneuropathy
• HIV – related progressive radiculopathy
• HIV – related multiple mononeuropathies
• HIV – related neuronpathy / ganglionopathy
80. HIV – Related Distal Symmetric
Polyneuropathy (DSP)
• DSP most common neuropathy in HIV-AIDS
• Characterised by numbness and painful
paresthesias in distal extremities
• Immune mediated – cytokines from
surrounding inflammation
• Antiretroviral drugs – Dideoxycytidine,
stavudine causes painful sensory neuropathy
81. HIV – Related Inflammatory
Demyelinating Polyneuropathy
• AIDP and CIDP occurs in HIV
• AIDP during the time of sero-conversion, while
CIDP can occur at any stage in the disease
• Elevated protein levels, lymphocytic
pleocytosis is evident on CSF; which helps to
distinguish it from idiopathic AIDP/CIDP
82. HIV – related progressive
polyradiculopathy
• Acute progressive lumbosacral
polyradiculopathy occurs secondary to CMV
infection
• Severe radicular pain, numbness and
weakness in legs – usually asymmetric
83. HIV – related multiple
mononeuropathies
• Weakness, numbness, paresthesias, and pain
occur in distribution of affected nerves
• Axonal degeneration with necrotizing
vasculitis or perivascular inflammation occurs
84. HIV – related sensory neuronopathy /
ganglionopathy
• Dorsal root ganglianitis occurs and
neuronopathy is the presenting feature
• Sensory ataxia
85. Nutritional Neuropathies
• Cobalamin (Vitamin B12) deficiency
– Numb hands typically appear before lower
extremity parasthesias
– Large fibre sensory loss affecting proprioception &
vibration with sparing of small fibre modalities,
sensory ataxia
– Diffuse hyporeflexia and absent Achilles reflex
– Sometimes – optic atrophy with behavioral
changes may be visible
86. Thiamine Deficiency
• Consequence of chronic alcohol abuse,
recurrent vomitting, total parentral nutrition
and bariatric surgery
• Mild sensory loss to burning dysesthesias in
toes and feet and aching and cramping in
lower legs followed by distal sensory loss
87. Copper Deficiency
• Myeloneuropathy
• Lower limb paresthesias, weakness, spasticity
and gait difficulties
• Most commonly large fibres sensory function
is affected, but sometimes; small fibres are
affected too
88. Leprosy (Hansen’s Disease)
• Most common cause of peripheral neuropathy
in Southeast Asia, Africa and South America
• Superficial cutaneous nerves of the ears and
distal limbs are commonly affected
• Mononeuropathies, multiple
mononeuropathy occurs
• Slowly progressive distal symmetric
sensorimotor polyneuropathy
89. • Sensory NCVs usually absent in lower limbs
and reduced in amplitude in arms
• Motor NCS reduced amplitude in affected
nerves and also shows delayed latent
conduction velocities
90. Acute Inflammatory Demyelinating
Polyneuropathies
• Clinical Features
– Areflexic motor paralysis with or without sensory
disturbances
– Ascending paralysis with dysesthesias in the extremities
– Facial diparesis in 50 % of patients
– Lower cranial nerves also be involved
– Pain in neck, shoulder, back or over spine in 50 % patients
– Autonomic dysfunction (fluctuation in BP, arrythmias)
• Bladder dysfunction not a prominent feature, but if
present suggests some alternative diagnosis (spinal
cord)
91. Subtypes of GBS
SUBTYPE FEATURES ELECTRODIAGNOSIS
Acute inflammatory
demyelinating
polyneuropathy (AIDP)
Adults more than
children; anti-GM1
antibodies
Demyelinating
Acute motor axonal
neuropathy (AMAN)
Children and yourng
adults, maybe seasonal;
anti-GD1a antibodies
Axonal
Acute motor sensory
sxonal neuropathy
(AMSAN)
Mostly adults, recovery
slow and often
incomplete
Axonal
Miller Fisher Syndrome
(MFS)
Adults and children;
ophthalmoplegia, ataxia
and areflexia; anti-GQ1B
antibodies
Axonal or demyelinating
92. Antecedent Events
• 70% GBS occurs 1 – 3 weeks after acute infectious
process, usually respiratory or gastrointestinal
• 20 – 30 % cases in western world after C. jejuni
infection
• Others include – HHV, CMV, EBV, M. peumoniae
• Older type of rabies vaccine, prepared in nervous
system, is implicated as a trigger for GBS in
developing countries.
• GBS also occurs in lymphona. HIV-seopositive
patients, SLE
94. Laboratory Features
• CSF – elevated protein levels without accompanying
pleocytosis
• CSF normal if symptoms <= 48 hours
• In AIDP, the earliest features are prolonged F-wave
latencies, prolonged distal latencies, and reduced
amplitude of compound muscle action potentials (CMAP)
• Late findings, slowing of conduction velocity, conduction
blocks, temporal dispersion may be appreciated
• In primary axonal pathology, Edx shows reduced
amplitude of CMAPs without conduction slowing or
prolongation of distal latencies
95.
96.
97. Diagnosis
Brighton Criteria for GBS
• Level 1 of diagnostic certainity
– Motor (B/l and flaccid)
– Reflexes
– Antecedent illness
– Edx
– CSF
– Absence of any alternative diagnosis
• Level 2 of diagnostic certainty
– Above except as in
– CSF / Edx
• Level 3 of diagnostic certainty
– Motor
– Reflexes
– Antecedent illness
– Alternative diagnosis
99. Chronic Inflammatory Demyelinating
Polyneuropathy
• Onset gradual over a few months or longer
• Acute onset form of CIDP when GBS deteriorates > 9
weeks after onset or relapse three times
• Weakness of limbs is symmetric but can be asymmetric
in multifocal aquired demyelinating sensory and motor
(MADSAM) neuropathy variant
• Some have chronic progressive course, while others
have replasing and remitting course
• Some have only motor findings, while some have pure
sensory ataxia
• Tremors in 10 % patients
100. Pathogenesis
• Biopsy reveals – inflammation and onion-bulb
appearance (from recurernt remyelination and
demyelinatin)
• CIDP responds to glucocorticoids
• Some patients have antibodies against P0, myelin
P2, PMP22 or neurofuscin
• 25% patients have monoclonal gammopathy and
antibodies against myelin-associated glycoprotein
(MAG)
101. Diagnosis
• Clinical, CSF and Edx
• CSF
– Albuminocytological dissociation
• Edx
– Variable degrees of conduction slowing, prolonged distal latencies,
distal and temporal dispersion of CMAPs, and conduction block
– Presence of conduction block is hallmark of aquired demyelinating
process
– Axonal loss, secondary to demyelination, is present in 50% patients
• Serum protein electrophoresis and immunofixation –
Monoclonal gammopathy
• CIDP – exclude vasculitis, collagen vascular disease (especially
SLE), chronic hepatitis, HIV, amyloidosis, and diabetes
102. Treatment
• Treatment initialted when progression is rapid or
walking is compromised
• High dose IVIg, PE and glucocorticoids all more
effective than placebo
• Patients who fail this therapy, should be given a
trial of
– Azathioprine, methotrexate, cyclosporine, and
cyclophosphamide
– Anti-CD20 (Rituximab) is also promsing
• Fails to respond to treatment, think of POEMS
(monoclonal gammopathy)
103. Charcot-Marie-Tooth Disease
• Most common type of hereditary neuropathy
• Classified on the basis of nerve conduction
velocities, predominant pathology and
inheritance pattern
– Type I – Demyelinating
• Nerve Conduction Velocity < 38 m/s
– Type II – Axonal
• Nerve Conduction Velocity > 38 m/s
• CMT 1, 2 & 3 – Autosomal Dominant; CMT 4 –
Autosomal Recessive
104.
105. • CMT Type 1
– (anterior compartment) – inverted champagne
bottle legs
– First to third decade of life with distal weakness
(e.g. footdrop); but many remain asymmtomatic
– Reduced sensations to all modalities on
examination
Three copies of
peripheral myelin
protein – 22 (PMP-
22)
106. • CMT Type 2
– Usually becomes symptomatic in second decade
of life;
– Clinically indistinguishable from CMT1; but NCVs
show a slightly higher velocity
• CMTDI – dominant Intermediate, NCVs faster
than in CMT1, but slower than CMT2
• CMT3
– Hereditary demyelinating sensorimotor
polyneuropathy in infancy or childhood
• NCVs usually in 5 – 10 m/s
107. • CMT4
– Severe, childhood-onset sensorimotor
polyneuropathy inherited as autosomal recessive
pattern
– EDx & histology shows demyelinating and axonal
features
108. • CMT1X
– X-linked dominant disorder; with clinical features
similar to CMT1 and CMT2, but more severe in
men than in women
– 10 – 15 % of CMT overall
– First two decades of life with atrophy and
weakness of distal arms and legs, pes cavus, and
hammertoes
– NCVs in between 15 – 35 m/s; (among which
majority in 25 – 35 m/s – intermediate slowing)
– Mutation in connexin 32
109. Hereditary Neuralgic Amotrophy
(HMA)
• Autosomal dominant; mutation in Septin 9 (SEPT 9)
• Recurrent attacks of pain, weakness and sensory loss in
the distribution of brachial plexus, which begins in
childhood; with recovery in weeks or months
(sensorimotor)
• Confused with brachial plexitis
• Occurs in postpartum state, stress or following surgery
• Clincal:
– Slighly dysmorphic features including hypotelorism,
epicantal folds, cleft palate, syndactyly, micrognathia, and
facial asymmetry occurs
• NCV – Axonal neuropathy
110. • Clinical patterns
– Classic (severe pain followed by paresis and
symptom-free interval in between)
– Chronic (interictal persistance of pain and paresis)
111. • Treatment:
– NSAIDS in combination with opoids and/or TCAs
like amitryptylline or BZDs
– Supportive physical therapy including
transcutaneous electrical neurostimulation (TENS)
112. Hereditary Neuropathy with Liability to
Pressure Palsies (HNPP)
• Second or third decade of life; due to a single
copy of PMP-22; autosomal dominant
• Painless numbness and weakness in the
distribution of a single peripheral nerve,
although multiple mononeuropathies can
occur; which takes weeks or months to resolve
• Precipitated by trivial compression of nerves –
wearing a backpack, leaning on elbows,
crossed legs
113. Hereditary Sensory and Autonomic
Neuropathy (HSAN)
• Sensory and Autonomic dysfunction predominated
over muscle weakness
• HSAN 1 in adults – second to forth decade of life,
autosomal dominant
• Degeneration of small myelinated and unmyelinated
nerve fibres
– Severe loss of pain and temperature sensations, gross foot
and hand deformities, bone loss, recurrent osteomyelitis,
amputed digits – self mutilating behaviour
– Sometimes, bladder dysfunction, reduced sweating in feet
can occur
114.
115. Fabry’s disease
• Angiokeratoderma corporis
diffusum; X-linked dominant
disorder.
• Angiokeratomas – umbilicus,
scrotum, inguinal region
andperineum
• Burning pain in hands and feet
occur, but disease presents with
other system complications like
HTN, Renal disease, cardiac disease
and stroke, dialted cardiomyopathy
• Mutation in a-galactosidase gene,
acumulation of ceramide trihexoside
in nerves and blood vessels
116. Refsum’s Disease
• Infancy to early childhood with tetrad of:
– Peripheral Neuropathy
– Retinitis Pigmentosa
– Cerebellar ataxia
– Elevated CSF protein levels
• Progressive distal sensory loss and weakness,
leading to foot drop in their 20s
117.
118. Tangier Disease
• Autosomal recessive disorder with reduced
amount of HDL cholestrol
– Asymmetric multiple mononeuropathies
– Slowly progressive symmetric polyneuropathy
predominantly in legs
– Pseudo-syringomyelia pattern with dissociated
sensory loss
119. Primary or AL Amyloidosis
• Multiple myeloma, Waldenstrom macroglobulinemia,
lymphoma or plasmacytomas
• 30 % patients with neuropathy – painful dysesthesias
and burning sensation of feet. Sometimes
mononeuritis multiplex can occur
• Slowly progressive eventually with large fibre sensory
loss
• Autonomic dysfunction with postural hypotension,
syncope, bowel and bladder disturbances,
constipation, impotence and impaired sweating
120. • Monoclonal protein may be composed of IgG,
IgA, IgM, or only free light chain. Lambda is more
common than kappa light chain
• CSF protein is often increased (with normal cell
count) mimicking neuropathy
• Nerve Biopsy – axonal degeneration and amyloid
deposition
• Chemotherapy reduces the concentration of
monoclonal proteins, and autologous stem cell
transplantation may prolong survival, but
improvement of neuropathy is controversial
121. Hypothyroidism
• Commonly associated with proximal
myopathy, but some patients typically develop
Carpel Tunnel Syndrome
• Generalised neuropathy characterised by
painful paresthesias and numbness in legs and
hands can occur
• Rx – Correction of hypothyroidism
122. Sjorgen’s Syndrome
• Complex of sicca complex of xerophthalmia,
xerostomia, dryness of mucus membranes,
can be complicated by neuropathy
• Length-dependent axonal sensorimotor
neuropathy of small fibre or cranial
neuronopathy, particularly of trigeminal nerve
• Progressive tingling and numbness of trunk
and limbs, or face (with sometimes face >
limbs)
123. • Patients with sensory ganglionopathies
develop progressive numbness and tingling of
the limbs, trunk, and face in a non-length-
dependent manner; so that face or arms are
affected more than legs
• ANAs, SS-A/Ro and SS-B/La antibodies in the
serum
• NCVs & nerve biopsy – axonal degeneration
• Vasculitis is suspected, immunosuppressive
agents can be beneficial
124. Rheumatoid Arthritis
• Seen in 50% of patients
• Vasculitis
• Mononeuropathy multiplex, generalised
pattern or combination of both
• Also be due to drugs used to treat RA (tumor
necrosis blockers, leflunomide)
• Responsive to immunomodulating therapies
125. Systemic Lupus Erythmatosus (SLE)
• 2 to 27% patients develop neuropathy
• Slowly progressive sensory loss beginning in
feet
• Burning pain and paresthesias with normal
reflex (small fibers)
• NCS – pure small-fiber neuropathy
• Immunosuppressive therapy is beneficial in
SLE with vasculitis
126. Systemic Sclerosis (Scleroderma)
• Distal symmetric, mainly sensory, polyneuropathy
complicated 5 – 67% of scleroderma cases
• Cranial mononeuropathies, commonly trigeminal,
produces numbness and dysesthesias in the face
• Muliple mononuropathies also occur
• EDx & Biopsy – axonal sensory >> motor
polyneuropathy
127. Uremic Neuropathy
• 60 % of patients with renal failure develops
polyneuropathy – length dependent
numbness, tingling, allodynia and mild distal
weakness
• Rapidly progressive weakness and sensory
loss, very similar to GBS, can occur which
improves by increasing the intensity of dialysis
or with transplantation
• Mononeuropathies (eg CTS can occur)
128. Sarcoidosis
• Cranial nerve – VII – affected bilaterally
• Radiculopathy or polyradiculopathy
• If generalised root involvement is present, then it
mimics GBS
• Multiple mononeuropathies or a generalised,
slowly progressive, sensory greater than motor
polyneuropathy can occur
• EDx – Axonal Neuropathy
129. Celiac Disease (Gluten-induced
enteropathy or nontropical sprue)
• Ataxia and peripheral neuropathy occurs in 10%
of patients with celiac disease
• Generalised sensorimotor polyneuropathy, pure
motor neuropathy, multiple mononeuropathies,
autonomic neuropathy, small-fiber neuropathy,
and neuromyotonia
• Nerve biopsy – loss of large myelinated fiber
– (Secondary to malabsorption of Vitamin B12 and E)
130. Inflammatory Bowel Disease
• UC & Crohn may be complicated by GBS, CIDP,
generalised axonal sensory or sensorimotor
polyneuropathy, small fibre neuropathy or
mononeuropathy
131. Lyme Disease
• Neurological complications occurs in second
and third stages of infection
• Facial neuropathy is most common and is
bilateral
• Other nerves are symmetrically affected
• It is a primary axonopathy
• Rx - Antibiotics
132. Diphtheritic Neuropathy
• 20 – 70 % patients develop peripheral
neuropathy by a toxin released by bacteria
• In third to forth week of illness, decreased
sensation in throat, dysphagia, dysarthria,
hoarseness and blurred vision due to impaired
accomodation occurs
• More generalised neuropathy occurs 2 to 3
months later, characterised by numbness,
paresthesias, weakness of arms and legs and
respiratory failure
133. • CSF protein can be elevated with or without
pleocytosis
• EDx – diffuse axonal sensorimotor
polyneuropathy
• Antitoxin and antibiotics should be given
within 48 hours of symptom onset; however, it
does not alter the natural history of associated
peripheral neuropathy
134. Herpes Varicella-Zoster Virus
• 2/3rd adults there is dermal zoster with pain
and paresthesias in dermatomal region
followed within a week or two by vesicular
rash in same distribution
• Weakness in muscles innervated by roots
corresponding to dermatological distribution
of skin lesions occurs in 5 – 30% patients
• About 25 % patients have continued pain
(post herpetic neuralgia – PHN)
135. Cytomegalovirus
• Acute lumbosacral polyradiculopathy and
multiple mononeuropathies in patients with
HIV and other immune deficiency disorders
136. Epstein-Barr Virus
• Associated with GBS, cranial neuropathies,
mononeuropathy multiplex, brachial
plexopathy, lumbosacral radiculoplexopathy,
and sensory neuronopathies
137. Chronic Liver Disease
• Generalised senorimotor neuropathy –
numbness, tingling, and minor weakness in
the distal aspects of lower limbs
• EDx – sensory >> motor axonal neuropathy
138. Critical Illness Polneuropathy
• Acute generalised weakness leading to admission in ICU
are GBS and myasthenia gravis
• However, weakness developing in critically ill patients
while in ICU is caused by critical illness poluneuropathy
(CIP) or critical illness myopathy (CIM)
• Complication of sepsis or multiple organ failure
– Leads to inability to wean patient off the ventilator
• Exact mechanism is not known; but circulating toxins and
metabolic abnormalities are associated with sepsis and
multiorgan failure impair axonal transport or
mitochondrial function, leading to axonal degeneration
139. Neuropathies Associated With
Malignancy
• Mechanism of neuropathy
– Direct effect of cancer by invasion or compression
of the nerves
– Remote or paraneoplastic effect
– Toxic effect of treatment
– Consequence of immune compromise by
immunosuppressive medications
• Most common is lung cancer.
140. Paraneoplastic Sensory
Neuronopathy/ Ganglionopathy
• Paraneoplastic encephalomyelitis/sensory
neuronopathy (PEM/SN) – Small – Cell
Carcinoma
• Polyclonal antineuronal antibodies (IgG) –
anti-Hu antigen
• Numbness or parasthesias in distal
extremities, sensory ataxia, motor
neuronopathy (pseudoathetosis, inability to
walk, stand or even sit without support)
141. • Neurological symptoms precede on an
average of 6 months before identification of
SCLC
• Even though, most of subacute sensory
neuropathies are idiopathic, more than half
the cases have underlying lung cancer
– So in selected cases, it is necessaryto screen for
paraneoplastic antibody, or even PET Scan for
early diagnosis of the tumor
142. Other malignancies
• Lymphoma
• Multiple myeloma
• As a complication of bone marrow
transplantation
• Associated with monoclonal gammopathy of
uncertain significance
• Even as a complication of chemotherapy
143. Toxic Neuropathies
• Chloroquine and Hydroxychloroquine
– Neuromyopathy
• Pyridoxime (Vitamin B6) Toxicity
– Severe sensory neuropathy with dysesthesias
• Lead
– Most common presentation is encephalopathy
– But primary motor neuropathy can also occur
• Weakness – usually beginning in arms, involving wrist
and finger extensors.
144. • Thallium
– Burning parasthesias of the feet, abdominal pain and
vomitting
– First week – Pigmentation of hairs, acne-like rash in
malar area of face, hyperreflexia
– Second & third week – autonomic instability with
labile heart rate and BO
– Third to forth week – hyporeflexia and alopeciA
• Arsenic
– Mimics GBS
– Abrupt onset abdominal pain, nanusea, vomitting,
pain and diarrhoea follwed several days later by
burning sensation in feets and hands
145. Cryptogenic (Idiopathic) Sensory and
Sensorimotor Polyneuropathy
• SCPN – diagnosis of exclusion
• 6ht or 7th decade of life
• Distal numbness, tingling, often burning pain
that begins in feet and eventually involves the
fingers and hands
• Both small and large fibre loss on neurological
exam and EDx
148. Multifocal Motor Neuropathy
• Uncommon neuropathy presents as slowly
progressive motor weakness and atrophy
developing over years in distribution of selected
nerve trunks
• Sensory fibres are spared; >75 % males
• < 50% patients presents with high titres of
polyclonal IgM antibody to ganglioside GM1
• Demyelinating and mild inflammatory changes at
the sites of conduction block
• Treatment – IVIg; sometimes rituximab or
cyclophosphamide
150. Multiple Myeloma
• Polyneuropathy occurs in 5% of the patients of
MM who exhibit either lytic or diffuse
osteroporotic bone lesions
• Sensorimotor, mild and slowly progressive and
do not reverse with supression of myeloma
151. Specific Characteristics of
osterosclerotic MM
• Demyelinating in nature and resembles CIDP
• Respond to radiation therapy or removal of
primary lesion
• Assocciated with lambda light chain. Lytic lesions
are associated with kappa light chain
• Refractory to typical treatment of CIDP
• Associated with other systemic findings:
– Organomegaly
– Endocrinopathy
– Skin Changes
• These features with polyneuropathy and Monoclonal
Gammopathy forms POEMS Syndrome
152. Monoclonal Gammopathy of
Undetermined Significance
• Immunoglobulin types IgG, IgA and IgM
• Sensorimotor neuropathy
• Generally males, >50 years
• Do not respond to immunotherapies desgined
to reduce monoclonal proteins
• Indistinguishable from CIDP
• Demyelination and remyelination occurs
153. Vasculitic Neuropathy
• Common in PAN (Polyarteritis Nodosa);
ooccuring in half the cases clinically and in
100% cases on post mortem examination
• Multifocal (asymmetric) motor-sensory
neuropathy (mononeuropathy multiplex) due
to ischemic lesions of nerve trunks and roots
154. • Suspected when mononeuropathy multiplex
occurs in conjugation with constitutional
symptoms (fever, anorexia, weight loss, malaise)
• Diagnosis by combined nerve and muscle biopsy
• Connective Tissue Disorders
– Rheumatoid Arthritis
– Cryoglobulinemia
– Wegener’s
– SLE
– Systemic Sclerosis
155. Metachromatic Leukodystrophy (MLD)
• MLD is a rare autosomal recessive lysosomal
storage disease that causes progressive
demyelination of CNS and PNS
• Disease diagnosed in childhood
• Result of consangious marriage
• Arylsulfatase A deficiency
• Children in 1 to 2 years of age presents with
progresive weakness, hypotonia and diminished
reflexes
156.
157. Krabbe disease
• Globoid celll leukodystrophy which is a fatal
degenerative disorder that affects the myelin
sheath of nervous system
158. Adrenoleukodystrophy
• Disorder of peroxisomal fatty acid beta oxidation
which results in accumulation of very long chain
fatty acids in tissues throughout the body
• Affects myelin in CNS, adrenal cortex and Leydig
cells of testis
• A variant of ALD; adrenimyeloneuropathy has age
of onset 21 – 37 and presents with progressive
neuropathy, paraparsis and cerebral involvement
159. Friedreich’s Ataxia
• Autosomal recessive ataxia resulting from mutation in
gene locus 9 (leading to GAA triplet repeats; reduced
frataxin)
• Causes progressive damage to the nervous system.
• Clinical Features:
– Muscle weakness in arms and legs
– Loss of coordination
– Vision and hearing defects
– Slurred speech
– Diabetes & heart disorders
– High-arched palate
160. • Pathology
– Sclerosis and degeneratino of dorsal root
ganglion, spinocerebellar tracks, lateral
corticospinal tracks and posterior column
– Loss of myelin in large myelinated fibres
161. Treatment of Neuropathy in general
• Treat the underlying cause
– Eg. Plasmapheresis, IVIg in AIDP
– Steroids in CIDP
• Symptomatic
– For Sensory Neuropathy
• TCAs, Gabapentin, pregabalin
• Carbamazepine, Phenytion, Vanlafaxine
• Mexiletine
– For Motor Neuropathy
• Rehabilitation and Physiotherapy