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Clumsy Hand
Neurophysiological Evaluation
DMW Dharmakeerthi
• Brief case discussion bit uncommon case
• Common clinical problem
• Spectrum of clinical neurophysiology
• 45 years lady, bank officer, weakness, clumsiness
and paresthesia of right hand for 3 - 4 weeks.
• Insidious onset over a month
• No neck or hand pain
• Inconsistent numb sensation along the medial
aspect of forearm and hand
• No nocturnal symptoms or diurnal variation
• Not increased with a particular manure
• PMHx- nill
• Referral: ? R / Ulnar neuropathy
• Any thoughts ?
• Ulnar neuropathy
• CTS
• Cx Radiculopathy
• MNM (combined median and ulnar)
• Lower Brachial Plexus
• Cervical syrinx
• Is it a generalized process
Examination
• Wasting of thenar muscle
• Mild dorsal guttering
• Weak thumb abduction, adduction, flexion
• Weak finger abduction, adduction
• Phalen’s and Tinel’s negative
• DD?
• CTS
• Ulnar neuropathy
• Cx Radiculopathy
• MNM (combined median and ulnar)
• Lower Brachial Plexus
• Cervical syrinx
• Is it a generalized process
• Anything else in examination ???
• Biceps
• Triceps
• Supinator
• Deltoid
• KJ
• AJ
• Jaw jerk
• Plantar equivocal
• DD?
• CTS
• Cx myelo-Radiculopathy
• Ulnar neuropathy
• MNM (combined median and ulnar)
• Lower Brachial Plexus
• Cervical syrinx (should have prominent sensory)
• Is it a generalized process
What is the generalized pathology ?
• Further questioning ?
• Leg cramps
• Worms creeping in arms and legs
• More throat clearing than earlier
• Frequent food stuck in throat
• No bladder or bowel problems
Could it be a ??
• Cx myelo Radiculopathy (brisk Jaw jerk)
• Syrinx(no prominent sensory symptoms)
• AHCD
Nerve conductions
Results
• Normal median and ulnar sensory responses
• Reduced ulnar CMAP, but no slowing at the
wrist or elbow
• Reduced median CMAP, but no slowing at the
wrist or forearm
• Normal sensory in LL
• Reduced CMAPs with normal CV in LL
• Normal sensory system with abnormal motor
system which shows generalized reduction of
motor units.
Electromyography (EMG)
EMG results
FDIO B/L
APB
EIP
FPL
EDC
TR
Biceps
Deltoid
Tongue
Sternomastoid
Rectus abdominis
VM
TA
Diagnosis
• Fulfills Avaji lower motor criteria for a
diagnosis of probable ALS type AHCD.
AHCD/MND/Lou Gehrig's disease
• Prevalence 2/100000
• Many types – spectrum
• ALS – Amyotrophic Lateral Sclerosis
• PLS – Primary Lateral Sclerosis
• PBP – Progressive Bulbar Palsy
• SMA – Spinal Muscular Atrophy
• HSP – Hereditary Spastic Para paresis
• Kennedy
• PPS – Post Polio Syndrome
• Commonest – ALS (5-10 % familial)
• Survival – 50% < 3 years 20% >5-10< years
• Conservative Mx
• No drugs to halt the disease
• Riluzole (Max 3months)
Carpal Tunnel Syndrome
• Carpal tunnel syndrome, the most common
focal peripheral focal neuropathy, results from
compression of the median nerve at the wrist.
Carpal Tunnel Syndrome
Clinical Features
• Numbness
• Tingling
• Pain
• Symptoms are usually worse at night and can
awaken patients from sleep.
• To relieve the symptoms, patients often “flick”
their wrist as if shaking down a thermometer
(flick sign).
Atrophy
Physical examination
• Phalen’s maneuver
• Tinel’s sign
• weak thumb abduction.
• two-point discrimination
Phalen’s maneuver
Tinel’s sign
Diagnostic
• History
• Physical examination
• Nerve Conduction Study
The Canterbury NCS Severity Scale for CTS
• Normal (grade 0)
• Very mild (grade 1), CTS demonstrable only with most sensitive
tests
• Mild (grade 2), sensory nerve conduction velocity slow on
finger/wrist measurement, normal terminal motor latency
• Moderate (grade 3), sensory potential preserved with motor
slowing, distal motor latency to abductor pollicis brevis (APB)
>4.5- < 5.5 ms
• Severe (grade 4), sensory potentials absent but motor response
preserved, distal motor latency to APB >5.5 < 6. 5 ms
• Very severe (grade 5), terminal latency to APB > 6.5 ms
• Extremely severe (grade 6), sensory and motor potentials
effectively un recordable (surface motor potential from APB < 0.2
mV amplitude)
Differential Diagnostics
• Tendonitis
• Tenosynovitis
• Diabetic and other neuropathies
• Kienbock's disease
• Compression of the Median nerve at the
elbow
• If acute onset, could be a more serious ??GBS
Treatment
• CONSERVATIVE TREATMENTS
– General measures
– WRIST SPLINTS
– ORAL MEDICATIONS
– LOCAL INJECTION
– ULTRASOUND THERAPY
– Predicting the Outcome of Conservative
Treatment
• SURGERY
GENERAL MEASURES
• Avoid repetitive wrist and hand motions that
may exacerbate symptoms or make symptom
relief difficult to achieve.
• Not use vibratory tools
• Ergonomic measures to relieve symptoms
depending on the motion that needs to be
minimized
WRIST SPLINTS
• Probably most
effective when it is
applied within
three months of the
onset of symptoms
• Optimal splinting
regimen ?
WRIST SPLINTS
ORAL MEDICATIONS
• Diuretics
• Nonsteroidal anti-inflammatory drugs
(NSAIDs)
• pyridoxine (vitamin B6)
• Orally administered corticosteroids
– Prednisolone
– 20 mg per day for two weeks
– followed by 10 mg per day for two weeks
LOCAL INJECTION
• A mixture of 10 to 20 mg of lidocaine
(Xylocaine) without epinephrine and 20 to 40
mg of methylprednisolone acetate (Depo-
Medrol) or similar corticosteroid preparation
is injected with a 25-gauge needle at the distal
wrist crease (or 1 cm proximal to it).
LOCAL INJECTION
LOCAL INJECTION
• Splinting is generally recommended after local
corticosteroid injection.
• If the first injection is successful, a repeat
injection can be considered after a few
months
• Surgery should be considered if a patient
needs more than two injections
ULTRASOUND THERAPY
•May be beneficial in the
long term management
•More studies are needed
to confirm it’s usefulness
SURGERY
• Should be considered in patients with
symptoms that do not respond to
conservative measures and in patients with
significant nerve entrapment (Grade 3-6) as
evidenced by nerve conduction studies.
SURGERY
Complications of surgery
• Injury to the palmar cutaneous or recurrent motor
branch of the median nerve
• Hypertrophic scarring
• Tendon adhesion
• Postoperative infection
• Hematoma
• Arterial injury
• Stiffness
• RSDS
SURGERY FAQ
• How long does it take ?
• One or both hands ?
• When can I go to work?
• Under LA/GA?
• Can it get worse ?? Of course it can
PREGNANCY
• Alterations in fluid balance may
predispose some pregnant women to
develop carpal tunnel syndrome.
• Symptoms are typically bilateral and
first noted during the third trimester.
• Conservative measures are
appropriate, because symptoms
resolve after delivery in most women
with pregnancy-related carpal tunnel
syndrome.
Conclusion
• Neurophysiology is an extension of
neurological examination
• Always follow basic rules of medicine
• History and psycho-physical examination
• No machine can replace your thinking process,
clinical skills and common sense
• Questioning is highly appreciated
NCS
NCS
• Motor Nerve Conduction
• Sensory Nerve Conduction
• Mixed Nerve conduction
• Late Responses
• Generalized neuropathies (Acquired / Heredit)
• Sub Classification
• Decide treatment and prognosis
• Radiculopathies, plexopathies and focal
Neuropathies (Traumatic / immune/MNM)
• Assessment of progression
Motor NCS
Sensory NCS
Late responses
• F Wave
• H reflex
• Blink Reflex
F Wave
H Reflex
Cranial nerves by NCS
Small Fiber Analysis
• SSR
• Cutaneous Silent Period
• Thermal Threshold Testing
• Micro Neurography
Needle EMG
• Diagnosis of lower motor pathologies (MND)
• Sub classification of generalized pathologies
• Localization of focal pathologies (traumatic)
• Pattern recognition (CIDP / Diabetic neuropa)
• Assessment of recovery (Brachial plexopathy)
• Myopathies
• Neuromyotonias (Dystrophic/ Non dystrophic)
Sphincter EMG
• Sacral plexus analysis
• Internal urethral sphincter dysfunction
• Time consuming and invasive
• Peri-urethral aproach
• If really necessary
NMJ
NMJ / SFEMG
SFEMG
MG
Movement disorder analysis
EMG / MDA
• Myoclonus (cortical/sub cortical)
• Tremor analysis
• Functional disorders
Cranial Nerves by EP
• ERG
• VEP
• BAEP
BAEP
SSEP
Magnetic Brain Stimulation
CMCT / SSEP
TMS
EEG / ECoG
• Epilepsy (important for syndromic diagnosis)
• Encephalopathy (Less specific)
• Encephalitis
• Structural abnormalities (Not specific)
• Ambulatory monitoring
• Video telemetry
• Brain mapping
• Localization during epilepsy surgeries
IOM / ECoG
EEG Brain Mapping
IOM / Spinal surgeries
IOM
• Help and guide the surgeon
• Retrospective analysis
• Research
Therapeutics
• Deep brain stimulation
• EMG guided Botox
• Advanced epilepsy management (vagal
stimulation)
• Sphincter management (sacral nerve
stimulation)
Thank You for your patience

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Hand Neurophysiological Evaluation Reveals ALS

  • 2. • Brief case discussion bit uncommon case • Common clinical problem • Spectrum of clinical neurophysiology
  • 3. • 45 years lady, bank officer, weakness, clumsiness and paresthesia of right hand for 3 - 4 weeks. • Insidious onset over a month • No neck or hand pain • Inconsistent numb sensation along the medial aspect of forearm and hand • No nocturnal symptoms or diurnal variation • Not increased with a particular manure • PMHx- nill • Referral: ? R / Ulnar neuropathy
  • 4. • Any thoughts ? • Ulnar neuropathy • CTS • Cx Radiculopathy • MNM (combined median and ulnar) • Lower Brachial Plexus • Cervical syrinx • Is it a generalized process
  • 5. Examination • Wasting of thenar muscle • Mild dorsal guttering • Weak thumb abduction, adduction, flexion • Weak finger abduction, adduction • Phalen’s and Tinel’s negative
  • 6.
  • 7. • DD? • CTS • Ulnar neuropathy • Cx Radiculopathy • MNM (combined median and ulnar) • Lower Brachial Plexus • Cervical syrinx • Is it a generalized process
  • 8. • Anything else in examination ???
  • 9.
  • 10. • Biceps • Triceps • Supinator • Deltoid • KJ • AJ • Jaw jerk • Plantar equivocal
  • 11. • DD? • CTS • Cx myelo-Radiculopathy • Ulnar neuropathy • MNM (combined median and ulnar) • Lower Brachial Plexus • Cervical syrinx (should have prominent sensory) • Is it a generalized process
  • 12. What is the generalized pathology ? • Further questioning ? • Leg cramps • Worms creeping in arms and legs • More throat clearing than earlier • Frequent food stuck in throat • No bladder or bowel problems
  • 13. Could it be a ?? • Cx myelo Radiculopathy (brisk Jaw jerk) • Syrinx(no prominent sensory symptoms) • AHCD
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  • 17. Results • Normal median and ulnar sensory responses • Reduced ulnar CMAP, but no slowing at the wrist or elbow • Reduced median CMAP, but no slowing at the wrist or forearm • Normal sensory in LL • Reduced CMAPs with normal CV in LL
  • 18. • Normal sensory system with abnormal motor system which shows generalized reduction of motor units.
  • 19.
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  • 25. Diagnosis • Fulfills Avaji lower motor criteria for a diagnosis of probable ALS type AHCD.
  • 26. AHCD/MND/Lou Gehrig's disease • Prevalence 2/100000 • Many types – spectrum • ALS – Amyotrophic Lateral Sclerosis • PLS – Primary Lateral Sclerosis • PBP – Progressive Bulbar Palsy • SMA – Spinal Muscular Atrophy • HSP – Hereditary Spastic Para paresis • Kennedy • PPS – Post Polio Syndrome
  • 27. • Commonest – ALS (5-10 % familial) • Survival – 50% < 3 years 20% >5-10< years • Conservative Mx • No drugs to halt the disease • Riluzole (Max 3months)
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  • 32. Carpal Tunnel Syndrome • Carpal tunnel syndrome, the most common focal peripheral focal neuropathy, results from compression of the median nerve at the wrist.
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  • 35. Clinical Features • Numbness • Tingling • Pain • Symptoms are usually worse at night and can awaken patients from sleep. • To relieve the symptoms, patients often “flick” their wrist as if shaking down a thermometer (flick sign).
  • 36.
  • 38. Physical examination • Phalen’s maneuver • Tinel’s sign • weak thumb abduction. • two-point discrimination
  • 41. Diagnostic • History • Physical examination • Nerve Conduction Study
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  • 44. The Canterbury NCS Severity Scale for CTS • Normal (grade 0) • Very mild (grade 1), CTS demonstrable only with most sensitive tests • Mild (grade 2), sensory nerve conduction velocity slow on finger/wrist measurement, normal terminal motor latency • Moderate (grade 3), sensory potential preserved with motor slowing, distal motor latency to abductor pollicis brevis (APB) >4.5- < 5.5 ms • Severe (grade 4), sensory potentials absent but motor response preserved, distal motor latency to APB >5.5 < 6. 5 ms • Very severe (grade 5), terminal latency to APB > 6.5 ms • Extremely severe (grade 6), sensory and motor potentials effectively un recordable (surface motor potential from APB < 0.2 mV amplitude)
  • 45. Differential Diagnostics • Tendonitis • Tenosynovitis • Diabetic and other neuropathies • Kienbock's disease • Compression of the Median nerve at the elbow • If acute onset, could be a more serious ??GBS
  • 46. Treatment • CONSERVATIVE TREATMENTS – General measures – WRIST SPLINTS – ORAL MEDICATIONS – LOCAL INJECTION – ULTRASOUND THERAPY – Predicting the Outcome of Conservative Treatment • SURGERY
  • 47. GENERAL MEASURES • Avoid repetitive wrist and hand motions that may exacerbate symptoms or make symptom relief difficult to achieve. • Not use vibratory tools • Ergonomic measures to relieve symptoms depending on the motion that needs to be minimized
  • 48.
  • 49. WRIST SPLINTS • Probably most effective when it is applied within three months of the onset of symptoms • Optimal splinting regimen ?
  • 51. ORAL MEDICATIONS • Diuretics • Nonsteroidal anti-inflammatory drugs (NSAIDs) • pyridoxine (vitamin B6) • Orally administered corticosteroids – Prednisolone – 20 mg per day for two weeks – followed by 10 mg per day for two weeks
  • 52. LOCAL INJECTION • A mixture of 10 to 20 mg of lidocaine (Xylocaine) without epinephrine and 20 to 40 mg of methylprednisolone acetate (Depo- Medrol) or similar corticosteroid preparation is injected with a 25-gauge needle at the distal wrist crease (or 1 cm proximal to it).
  • 54. LOCAL INJECTION • Splinting is generally recommended after local corticosteroid injection. • If the first injection is successful, a repeat injection can be considered after a few months • Surgery should be considered if a patient needs more than two injections
  • 55. ULTRASOUND THERAPY •May be beneficial in the long term management •More studies are needed to confirm it’s usefulness
  • 56. SURGERY • Should be considered in patients with symptoms that do not respond to conservative measures and in patients with significant nerve entrapment (Grade 3-6) as evidenced by nerve conduction studies.
  • 57. SURGERY Complications of surgery • Injury to the palmar cutaneous or recurrent motor branch of the median nerve • Hypertrophic scarring • Tendon adhesion • Postoperative infection • Hematoma • Arterial injury • Stiffness • RSDS
  • 58. SURGERY FAQ • How long does it take ? • One or both hands ? • When can I go to work? • Under LA/GA? • Can it get worse ?? Of course it can
  • 59.
  • 60. PREGNANCY • Alterations in fluid balance may predispose some pregnant women to develop carpal tunnel syndrome. • Symptoms are typically bilateral and first noted during the third trimester. • Conservative measures are appropriate, because symptoms resolve after delivery in most women with pregnancy-related carpal tunnel syndrome.
  • 61. Conclusion • Neurophysiology is an extension of neurological examination • Always follow basic rules of medicine • History and psycho-physical examination • No machine can replace your thinking process, clinical skills and common sense • Questioning is highly appreciated
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  • 64. NCS
  • 65. NCS • Motor Nerve Conduction • Sensory Nerve Conduction • Mixed Nerve conduction • Late Responses
  • 66. • Generalized neuropathies (Acquired / Heredit) • Sub Classification • Decide treatment and prognosis • Radiculopathies, plexopathies and focal Neuropathies (Traumatic / immune/MNM) • Assessment of progression
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  • 71. Late responses • F Wave • H reflex • Blink Reflex
  • 75. Small Fiber Analysis • SSR • Cutaneous Silent Period • Thermal Threshold Testing • Micro Neurography
  • 77. • Diagnosis of lower motor pathologies (MND) • Sub classification of generalized pathologies • Localization of focal pathologies (traumatic) • Pattern recognition (CIDP / Diabetic neuropa) • Assessment of recovery (Brachial plexopathy) • Myopathies • Neuromyotonias (Dystrophic/ Non dystrophic)
  • 78. Sphincter EMG • Sacral plexus analysis • Internal urethral sphincter dysfunction • Time consuming and invasive • Peri-urethral aproach • If really necessary
  • 79. NMJ
  • 81. SFEMG
  • 82. MG
  • 84. • Myoclonus (cortical/sub cortical) • Tremor analysis • Functional disorders
  • 85. Cranial Nerves by EP • ERG • VEP • BAEP
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  • 87.
  • 88. BAEP
  • 89. SSEP
  • 91.
  • 92. TMS
  • 94. • Epilepsy (important for syndromic diagnosis) • Encephalopathy (Less specific) • Encephalitis • Structural abnormalities (Not specific) • Ambulatory monitoring • Video telemetry • Brain mapping
  • 95. • Localization during epilepsy surgeries
  • 98. IOM / Spinal surgeries
  • 99. IOM
  • 100. • Help and guide the surgeon • Retrospective analysis • Research
  • 101. Therapeutics • Deep brain stimulation • EMG guided Botox • Advanced epilepsy management (vagal stimulation) • Sphincter management (sacral nerve stimulation)
  • 102. Thank You for your patience