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PNS Disorders
Dr BALAJI B S
ASSOCIATE CONSULTANT NEUROLOGY
01-09-2020 2
01-09-2020 3
01-09-2020 4
What is the Peripheral Nervous System?
• CNS is confined to brain and spinal cord
• PNS includes (in anatomical order)
– Anterior horn cell (located within spinal cord)
– Spinal nerve roots (radicles)
– Plexi (brachial and lumbosacral)
– Named peripheral nerves (e.g. median, peroneal)
– Tiny nerve endings (sensory fibers and tiny branches of lower motor
axons at the neuromuscular junction)
– Neuromuscular junction
5
Neuromuscular junction and muscle
Ulnar nerve
Brachial
plexus
C8 spinal nerve root
Anterior horn
cells (LMN)
The PNS
(Emphasizing Motor Structures)
Symptoms of PNS Disease
• Single focal lesions: weakness/numbness/ pain in
one limb, often defined to one part of the limb
• Multiple or diffuse lesions: weakness/
numbness/pain in more than one limb, usually
bilateral and distal
7
Signs of PNS Disease
• Lower motor neuron signs (atrophy, fasciculations)
• Hyporeflexia or areflexia
• Patch of sensory loss, or stocking-glove sensory
loss
• Not UMN signs (spasticity, hyperreflexia, upgoing
toe) or “brain” signs (impaired consciousness,
cognition, or language)
8
Reflexes… repeated
• Hyperreflexia and spasticity occur with upper
motor neuron lesions (CNS)
• Hyporeflexia, fasciculations, atrophy with
lower motor neuron lesions (PNS)
9
Workup
• Serologies, especially for treatable causes
• EMG helps localize and characterize lesions of
PNS
• Imaging for some focal lesions, or to exclude CNS
mimics (such as cord lesion or stroke)
• CSF analysis in demyelinating neuropathies, or
polyradiculopathy
• Nerve biopsy
10
Anterior Horn Cell
11
Amyotrophic lateral sclerosis
• Anterior horn cells (lower motor neurons) and
upper motor neurons degenerate
• Mix of UMN and LMN signs/symptoms
• Weakness, spasticity, multifocal muscle atrophy
• No sensory loss from ALS!
• Loss of speech, swallow, respiration Death in
2-5 years
12
Manifestations
• Initial: spastic, weak muscles with increased
DTRs; muscle flaccidity, paresis, paralysis,
atrophy; clients note muscle weakness and
fasciculations; muscles weaken, atrophy; client
complains of progressive fatigue; usually involves
hands, shoulders, upper arms, and then legs
• Atrophy of tongue and facial muscles result in
dysphagia and dysarthria; emotional lability and
loss of control occur
• 50% of clients die within 2 – 5 years of diagnosis,
often from respiratory failure or aspiration
pneumonia
Diagnostic Test
• Testing rules out other conditions that
may mimic early ALS such as
hyperthyroidism, compression of spinal
cord, infections, neoplasms
• EMG to differentiate neuropathy from
myopathy
• Muscle biopsy shows atrophy and loss of
muscle fiber
• Serum creatine kinase if elevated (non-
specific)
• Pulmonary function tests: to determine
Therapeutic Interventions
 Muscle Relaxants : Central
 Riluzole
 PT/ OT/ ST
 Pain Control
 Tube Feedings
 Prevention of Infection
Neuromuscular junction and muscle
Ulnar nerve
Brachial
plexus
C8 spinal nerve root
Radiculopathy
Spinal Nerve Root Disorders
• Most common: monoradiculopathy (cervical or lumbosacral)
• Radiating pain, +/- weakness, +/- sensory loss. Reduced reflex
for that root level
• Commonest causes: disk herniation, minor trauma,
degenerative change
• Usually self-limited
• Image if severe, worsening, or concern for cancer, infection
17
18
G u illa in -B a r r é S y n d r o m e
(G B S )
Acute autoimmune
inflammatory demyelinating
disorder of peripheral
nervous system
characterized by acute
onset of ascending motor
paralysis
M a n if e s t a t io n s
• Most clients have symmetric weakness beginning in
lower extremities
• Ascends body to include upper extremities, torso, and
cranial nerves
• Sensory involvement causes severe pain, paresthesia and
numbness
• Paralysis of intercostals and diaphragmatic muscle
• Autonomic nervous system involvement: blood pressure
fluctuations, cardiac dysrhythmias, paralytic ileus,
urinary retention
• Weakness usually plateaus or starts to improve in the
fourth week with slow return of muscle strength
Diagnostic Tests
• diagnosis made thorough history
and clinical examination; there is
no specific test
• CSF analysis: increased protein
• EMG: decrease nerve conduction
• Pulmonary function test reflect
degree of respiratory involvement
Medical management
• IVIG OR
• Plasmapheresis
• Enteral feeding
• Tracheostomy
Neuromuscular junction and muscle
Ulnar nerve
Brachial
plexus
C8 spinal nerve root
Plexopathy
Plexopathy
• PNS syndrome in one limb not explained by a single spinal
root, or by a single “named” peripheral nerve
• Causes: trauma or stretch, compression by tumor or
hematoma, radiation, diabetic
• EMG confirms plexopathy
• Image if compressive lesion suspected
24
Brachial Plexus
behind clavicle, in upper thorax
25
Lumbosacral
Plexus: Pelvic,
Retroperitoneal
26
Neuromuscular junction and muscle
Ulnar nerve
Brachial
plexus
C8 spinal nerve root
Mononeuropathy
Mononeuropathy
• Weakness, numbness, pain, paresthesias
confined to the distribution of
– UE: median nerve, radial n., ulnar n.
– LE: femoral n., sciatic n., peroneal n.
• Most common causes: entrapment, trauma,
prolonged limb immobility (e.g., surgery)
28
Important Mononeuropathies
• Median mononeuropathy at the wrist (carpal
tunnel syndrome)
• Ulnar mononeuropathy at the elbow (cubital
tunnel syndrome)
• Radial mononeuropathy (“Saturday night
palsy”) wrist and finger drop
• Peroneal mononeuropathy (e.g., from leg
crossing) one cause of footdrop
29
Median n.
Ulnar n.
Peroneal n.
Named peripheral nerves have well-defined
sensory territories (and muscle targets)
30
31
“Peripheral Neuropathy”
• Distal symmetric polyneuropathy
• Affects longest sensory/ motor/ autonomic
nerves
• Nerves are “dying back”
• Length dependent (“stocking glove”)
• Symmetric loss of pain/ temp / vibration/
proprioception; distal reflex loss
• Usually chronic. Many possible causes!
32
Peripheral polyneuropathy symptoms
• Initially, feet numb with paresthesia/ pain
• Symptoms ascend:  legs fingertips
• Distal weakness (feet, or fingers/grip), atrophy,
• Severe sensory loss can cause “steppage gait”,
“sensory ataxia”, imbalance, falls
• Feet prone to injuries, ulcers, deformation
(e.g., “Charcot foot”)
• Autonomic: orthostasis, bladder and erectile
dysfunction
33
Causes of peripheral polyneuropathy
• Usually toxic or metabolic
• #1 cause: diabetes & impaired glucose tolerance
• B12 defeciency
• Hematologic (e.g., multiple myeloma) or other immunoglobulin disorders (check
SPEP)
• Drugs: Li, chemotherapy
• Alcoholic neuropathy
• Liver or kidney disease
• HIV and neurosyphilis
• Inflammatory causes: connective tissue disease
34
Workup for peripheral neuropathy?
• For typical distal symmetric sensory > motor
neuropathy: glucose / Hba1c, B12, SPEP
• Need EMG and more if rapid or severe,
prominent weakness, asymmetry, young
patient
Manifestations
Seen in the muscles that are affected:
• Ptosis (drooping of eyelids), diplopia
(double vision)
• Weakness in mouth muscles resulting in
dysarthria and dysplagia
• Weak voice, smile appears as snarl
• Head juts forward
• Muscles are weak but DTRs are normal
• Weakness and fatigue exacerbated by
stress, fever, overexertion, exposure to
Diagnostic Tests
• Physical examination and history
• Tensilon Test: edrophonium chloride (Tensilon)
administered and client with myasthenia will
show significant improvement lasting 5 minutes
• SFEMG: senstive
• Antiacetylcholine receptor antibody serum
levels: increased in 80% MG clients; used to
follow course of treatment
• Serum assay of circulating acetylcholine
receptor antibodies: if increased, is diagnostic
of MG
Therapeutic Interventions
 Thymectomy- < production of actecycholine antibodies
 Cholinergic Agents- pyridostigmine,
 Steroids- predisone
 Plasmapheresis- remove antibodies from the blood
Trigeminal Neuralgia
 Pathophysiology
 Irritation of the trigeminal nerve (5th cranial nerve), affects sensory portion of
nerve
 Etiology
 Irritation or Chronic Compression
 Dx: H&P, CT, MRI
Trigeminal Innervation
Signs & Symptoms
 Intense Pain on One Side of Face
 Forehead, Cheek, Nose, Jaw
 Triggered by Touch, Talking, Other Stimulation
Therapeutic Interventions
 Anticonvulsants
 Nerve Blocks
 Surgery to Block Pain Signals
Bell’s Palsy
 Pathophysiology/Etiology
 Inflammation and Edema of Facial Nerve
 Loss of Motor Control
 Etiology Unknown
 Dx: H&P, EMG, rule out stroke
Signs & Symptoms
 One Sided Facial
 Pain
 Weakness
 Speech Difficulty
 Drooling
 Tearing of Eye
 Inability to Blink
Therapeutic Interventions
 Prednisone
 Antiviral Medication
 Facial physio, facial nerve stimulation
 Eye drops
Thank you

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PNS disorders Nursing.pptx

  • 1. PNS Disorders Dr BALAJI B S ASSOCIATE CONSULTANT NEUROLOGY
  • 5. What is the Peripheral Nervous System? • CNS is confined to brain and spinal cord • PNS includes (in anatomical order) – Anterior horn cell (located within spinal cord) – Spinal nerve roots (radicles) – Plexi (brachial and lumbosacral) – Named peripheral nerves (e.g. median, peroneal) – Tiny nerve endings (sensory fibers and tiny branches of lower motor axons at the neuromuscular junction) – Neuromuscular junction 5
  • 6. Neuromuscular junction and muscle Ulnar nerve Brachial plexus C8 spinal nerve root Anterior horn cells (LMN) The PNS (Emphasizing Motor Structures)
  • 7. Symptoms of PNS Disease • Single focal lesions: weakness/numbness/ pain in one limb, often defined to one part of the limb • Multiple or diffuse lesions: weakness/ numbness/pain in more than one limb, usually bilateral and distal 7
  • 8. Signs of PNS Disease • Lower motor neuron signs (atrophy, fasciculations) • Hyporeflexia or areflexia • Patch of sensory loss, or stocking-glove sensory loss • Not UMN signs (spasticity, hyperreflexia, upgoing toe) or “brain” signs (impaired consciousness, cognition, or language) 8
  • 9. Reflexes… repeated • Hyperreflexia and spasticity occur with upper motor neuron lesions (CNS) • Hyporeflexia, fasciculations, atrophy with lower motor neuron lesions (PNS) 9
  • 10. Workup • Serologies, especially for treatable causes • EMG helps localize and characterize lesions of PNS • Imaging for some focal lesions, or to exclude CNS mimics (such as cord lesion or stroke) • CSF analysis in demyelinating neuropathies, or polyradiculopathy • Nerve biopsy 10
  • 12. Amyotrophic lateral sclerosis • Anterior horn cells (lower motor neurons) and upper motor neurons degenerate • Mix of UMN and LMN signs/symptoms • Weakness, spasticity, multifocal muscle atrophy • No sensory loss from ALS! • Loss of speech, swallow, respiration Death in 2-5 years 12
  • 13. Manifestations • Initial: spastic, weak muscles with increased DTRs; muscle flaccidity, paresis, paralysis, atrophy; clients note muscle weakness and fasciculations; muscles weaken, atrophy; client complains of progressive fatigue; usually involves hands, shoulders, upper arms, and then legs • Atrophy of tongue and facial muscles result in dysphagia and dysarthria; emotional lability and loss of control occur • 50% of clients die within 2 – 5 years of diagnosis, often from respiratory failure or aspiration pneumonia
  • 14. Diagnostic Test • Testing rules out other conditions that may mimic early ALS such as hyperthyroidism, compression of spinal cord, infections, neoplasms • EMG to differentiate neuropathy from myopathy • Muscle biopsy shows atrophy and loss of muscle fiber • Serum creatine kinase if elevated (non- specific) • Pulmonary function tests: to determine
  • 15. Therapeutic Interventions  Muscle Relaxants : Central  Riluzole  PT/ OT/ ST  Pain Control  Tube Feedings  Prevention of Infection
  • 16. Neuromuscular junction and muscle Ulnar nerve Brachial plexus C8 spinal nerve root Radiculopathy
  • 17. Spinal Nerve Root Disorders • Most common: monoradiculopathy (cervical or lumbosacral) • Radiating pain, +/- weakness, +/- sensory loss. Reduced reflex for that root level • Commonest causes: disk herniation, minor trauma, degenerative change • Usually self-limited • Image if severe, worsening, or concern for cancer, infection 17
  • 18. 18
  • 19. G u illa in -B a r r é S y n d r o m e (G B S ) Acute autoimmune inflammatory demyelinating disorder of peripheral nervous system characterized by acute onset of ascending motor paralysis
  • 20. M a n if e s t a t io n s • Most clients have symmetric weakness beginning in lower extremities • Ascends body to include upper extremities, torso, and cranial nerves • Sensory involvement causes severe pain, paresthesia and numbness • Paralysis of intercostals and diaphragmatic muscle • Autonomic nervous system involvement: blood pressure fluctuations, cardiac dysrhythmias, paralytic ileus, urinary retention • Weakness usually plateaus or starts to improve in the fourth week with slow return of muscle strength
  • 21. Diagnostic Tests • diagnosis made thorough history and clinical examination; there is no specific test • CSF analysis: increased protein • EMG: decrease nerve conduction • Pulmonary function test reflect degree of respiratory involvement
  • 22. Medical management • IVIG OR • Plasmapheresis • Enteral feeding • Tracheostomy
  • 23. Neuromuscular junction and muscle Ulnar nerve Brachial plexus C8 spinal nerve root Plexopathy
  • 24. Plexopathy • PNS syndrome in one limb not explained by a single spinal root, or by a single “named” peripheral nerve • Causes: trauma or stretch, compression by tumor or hematoma, radiation, diabetic • EMG confirms plexopathy • Image if compressive lesion suspected 24
  • 25. Brachial Plexus behind clavicle, in upper thorax 25
  • 27. Neuromuscular junction and muscle Ulnar nerve Brachial plexus C8 spinal nerve root Mononeuropathy
  • 28. Mononeuropathy • Weakness, numbness, pain, paresthesias confined to the distribution of – UE: median nerve, radial n., ulnar n. – LE: femoral n., sciatic n., peroneal n. • Most common causes: entrapment, trauma, prolonged limb immobility (e.g., surgery) 28
  • 29. Important Mononeuropathies • Median mononeuropathy at the wrist (carpal tunnel syndrome) • Ulnar mononeuropathy at the elbow (cubital tunnel syndrome) • Radial mononeuropathy (“Saturday night palsy”) wrist and finger drop • Peroneal mononeuropathy (e.g., from leg crossing) one cause of footdrop 29
  • 30. Median n. Ulnar n. Peroneal n. Named peripheral nerves have well-defined sensory territories (and muscle targets) 30
  • 31. 31
  • 32. “Peripheral Neuropathy” • Distal symmetric polyneuropathy • Affects longest sensory/ motor/ autonomic nerves • Nerves are “dying back” • Length dependent (“stocking glove”) • Symmetric loss of pain/ temp / vibration/ proprioception; distal reflex loss • Usually chronic. Many possible causes! 32
  • 33. Peripheral polyneuropathy symptoms • Initially, feet numb with paresthesia/ pain • Symptoms ascend:  legs fingertips • Distal weakness (feet, or fingers/grip), atrophy, • Severe sensory loss can cause “steppage gait”, “sensory ataxia”, imbalance, falls • Feet prone to injuries, ulcers, deformation (e.g., “Charcot foot”) • Autonomic: orthostasis, bladder and erectile dysfunction 33
  • 34. Causes of peripheral polyneuropathy • Usually toxic or metabolic • #1 cause: diabetes & impaired glucose tolerance • B12 defeciency • Hematologic (e.g., multiple myeloma) or other immunoglobulin disorders (check SPEP) • Drugs: Li, chemotherapy • Alcoholic neuropathy • Liver or kidney disease • HIV and neurosyphilis • Inflammatory causes: connective tissue disease 34
  • 35. Workup for peripheral neuropathy? • For typical distal symmetric sensory > motor neuropathy: glucose / Hba1c, B12, SPEP • Need EMG and more if rapid or severe, prominent weakness, asymmetry, young patient
  • 36.
  • 37. Manifestations Seen in the muscles that are affected: • Ptosis (drooping of eyelids), diplopia (double vision) • Weakness in mouth muscles resulting in dysarthria and dysplagia • Weak voice, smile appears as snarl • Head juts forward • Muscles are weak but DTRs are normal • Weakness and fatigue exacerbated by stress, fever, overexertion, exposure to
  • 38. Diagnostic Tests • Physical examination and history • Tensilon Test: edrophonium chloride (Tensilon) administered and client with myasthenia will show significant improvement lasting 5 minutes • SFEMG: senstive • Antiacetylcholine receptor antibody serum levels: increased in 80% MG clients; used to follow course of treatment • Serum assay of circulating acetylcholine receptor antibodies: if increased, is diagnostic of MG
  • 39. Therapeutic Interventions  Thymectomy- < production of actecycholine antibodies  Cholinergic Agents- pyridostigmine,  Steroids- predisone  Plasmapheresis- remove antibodies from the blood
  • 40. Trigeminal Neuralgia  Pathophysiology  Irritation of the trigeminal nerve (5th cranial nerve), affects sensory portion of nerve  Etiology  Irritation or Chronic Compression  Dx: H&P, CT, MRI
  • 42. Signs & Symptoms  Intense Pain on One Side of Face  Forehead, Cheek, Nose, Jaw  Triggered by Touch, Talking, Other Stimulation
  • 43. Therapeutic Interventions  Anticonvulsants  Nerve Blocks  Surgery to Block Pain Signals
  • 44. Bell’s Palsy  Pathophysiology/Etiology  Inflammation and Edema of Facial Nerve  Loss of Motor Control  Etiology Unknown  Dx: H&P, EMG, rule out stroke
  • 45. Signs & Symptoms  One Sided Facial  Pain  Weakness  Speech Difficulty  Drooling  Tearing of Eye  Inability to Blink
  • 46. Therapeutic Interventions  Prednisone  Antiviral Medication  Facial physio, facial nerve stimulation  Eye drops