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Neuropathy Management in

Primary Care
Winnugroho Wiratman

Department of Neurology, Faculty of Medicine, Universitas Indonesia


Dr. Cipto Mangunkusumo Hospital | Universitas Indonesia Hospital
Neuropathy in primary care (SKDI
2019)
• Neuropati (3A) • Myasthenia Gravis (3B)
• Carpal Tunnel Syndrome (3A) • Amyotrophic Lateral Sclerosis (1)
• Tarsal Tunnel Syndrome (3A) • Myositis (1)
• Peroneal palsy (3A)
• Guillain Barre Syndrome (3B)
Diagnosis Approach for
Neuropathy
Peripheral Nerve Anatomy

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Neuroanatomical-functional approach
Case Lesion?
• Central nervous system (CNS)
• Young ♀ isolated weakness of • Peripheral nervous system,
the right abductor pollicis brevis • C8 or T1 anterior horn cells
• C8 or T1 radix
• Brachial plexus
• Lower trunk
• Medical cord
• Median nerve
• Neuromuscular junction
• Muscle

London ZN. Continuum. 2020


Neuroanatomical-functional approach
Case Lesion?
• Central nervous system (CNS)
• Young ♀ isolated weakness of • Peripheral nervous system,
the right abductor pollicis brevis • C8 or T1 anterior horn cells
• C8 or T1 radix
• Brachial plexus
• Lower trunk
• Numbness over palmar of right • Medical cord
index finger • Median nerve
• Neuromuscular junction
• Muscle

London ZN. Continuum. 2020


Neuroanatomical-functional approach
Case Lesion?
• Central nervous system (CNS)
• Young ♀ isolated weakness of • Peripheral nervous system,
the right abductor pollicis brevis • C8 or T1 anterior horn cells
• C8 or T1 radix
• Brachial plexus
• Lower trunk
• Numbness over palmar of right • Medical cord
index finger • Median nerve
• Neuromuscular junction
• Muscle

London ZN. Continuum. 2020


Neuroanatomical-functional approach
Case Lesion?
• Central nervous system (CNS)
• Young ♀ isolated weakness of • Peripheral nervous system,
the right abductor pollicis brevis • C8 or T1 anterior horn cells
• C8 or T1 radix
• Brachial plexus
• Lower trunk
• Numbness over palmar of right • Medical cord
index finger • Median nerve
• Neuromuscular junction
• Muscle

London ZN. Continuum. 2020


Neuroanatomical-functional approach
Case Lesion?
• Central nervous system (CNS)
• Young ♀ isolated weakness of • Peripheral nervous system,
the right abductor pollicis brevis • C8 or T1 anterior horn cells
• C8 or T1 radix
Median nerve • Brachial plexus
• Lower trunk
• Numbness over palmar of right • Medical cord
index finger • Median nerve
• Neuromuscular junction
• Muscle

London ZN. Continuum. 2020


Peripheral nervous system localization

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Neuroanatomical-functional approach on
Peripheral nervous system localization

London ZN. Continuum. 2020


Pattern approach on
Peripheral nervous system localization

• If it is multifocal or generalized:
o Does it involve sensory loss
or weakness, or both?
o Is it primarily proximal or
distal, or both?
o Is it symmetric or
asymmetric?
• Is it acute or subacute in onset?

London ZN. Continuum. 2020


A rational approach to a patient with
sensory or motor symptoms
• Does it localize to the CNS or to the peripheral nervous system?
• Can it localize to a single named peripheral nervous system
structure?
• If it is multifocal or generalized:
o Does it involve sensory loss or weakness, or both?
o Is it primarily proximal or distal, or both?
o Is it symmetric or asymmetric?
• Is it acute or subacute in onset?

London ZN. Continuum. 2020


Does it localize to the CNS or to the PNS?
CNS Peripheral Nervous System
Brain/ brainstem Spinal Cord
Brisk reflexes in affected limb Hyporeflexia in affected limb
Increased tone in an affected
limb
Axial or limb pain (rare, Axial or limb pain (+++)
except in thalamic lesion)
Weakness and diminished Decreased pinprick sensation Weakness and diminished
pain sensation in the same on one side, weakness and pain sensation in the same
limb diminished proprioception on limb
the other side
Sensory or motor symptoms Sensory loss in both legs with Sensory loss that involves the
limited to two limbs on the a sensory level on the trunk hands and feet but not the
same side of the body trunk
Weakness of extensors in the
upper extremity
Weakness of flexors in the
lower extremity

London ZN. Continuum. 2020


Can it localize to a single named peripheral
nervous system structure?

Radiculopathy Plexopathy Mononeuropathy


Myotomal and dermatomal Distribution of the weakness is
approach most helpful in localizing
Sensory symptoms often precede Sensory and motor symptoms May pure motor symptoms or
motor symptoms mixed. Sensory symptoms may
precede motor symptoms
Most common etiology: Most common etiology: injury, CTS, TTS, Saturday night palsy,
intervertebral disk herniation and breast cancer Peroneal palsy, cubital canal
spondylosis syndrome, etc
Prone to trauma and infiltration Prone to entrapment

London ZN. Continuum. 2020


If it is multifocal or generalized:

London ZN. Continuum. 2020


Carpal Tunnel Syndrome
Median nerve anatomy
Proximal median neuropathy:
• Anterior interosseus syndrome
• Pronator syndrome
• Entrapment at Struthers ligament

Distal median neuropathy:


• Carpal Tunnel Syndrome

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Clinical symptoms of median
neuropathy

CTS AINS PS Struthers

• Median nerve • Median nerve • Median nerve • Median nerve


paresthesia paresthesia paresthesia paresthesia
• Pain/paresthesias • Unable to make • Pain radiate • Pain in the volar
associated with “OK” sign aggravated by forearm
grasping. • Pronation repeated exacerbated by
• Nocturnal weakness. pronation/ supination and
paresthesias supination. extension
• Flick (+), • Tinel’s sign
• Phalen’s (+)

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Median nerve anatomy
C6-C7
• Proximal muscles
• Digiti 1, 2, 3 sensory

C8-T1
• Proximal muscles
• Latela 4th digiti sensory

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Median nerve anatomy

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


CARPAL TUNNEL SYNDROME

Risk Factors Sign & Symptoms Treatment

• Age • Nocturnal • Conservative


• Women paresthesias • Minimal invasive
• Obese • Pain/paresthesias • Surgery
• Occupation/ work associated with
grasping.
• Digiti 1, 2, 3, lateral 4
paresthesia
• Flick (+)
• Phalen’s (+)

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


CARPAL TUNNEL SYNDROME

Risk Factors Sign & Symptoms Treatment

• Age • Nocturnal • Conservative


• Women paresthesias • Minimal invasive
• Obese • Pain/paresthesias • Surgery
• Occupation/ work associated with
grasping.
• Digiti 1, 2, 3, lateral 4
paresthesia
• Flick (+) NCS & EMG
• Phalen’s (+) by neurologist

Preston DC, Shapiro BE. Electromyography and Neuromuscular Disorders. 2013


Conservative treatment

Hand splinting Occupational/ exercise


• Nocturnal • Minimise activities that
• Maintain at neutral angle exacerbate symptoms
• 35-80% efficiency 2-3 weeks • Tendon/ nerve gliding exercise
• Inexpensive

Saint-Lary et al. Front. Med. 2015


Minimal invasive treatment

Steroid Injection
• Relief symptoms (temporary)
• 70% of cases 1 month after
injection
• Triamcinolone acetate : Lidocaine
• Diabetes not good indication
1% à 3:1
• Familiar/ training needed
• 25G, 1.5” needle
• Proximal to wrist crease between
palmaris longus and flexor carpi
ulnaris tendon, 30º – 45º
Saint-Lary et al. Front. Med. 2015
Treatment algorithm

Saint-Lary et al. Front. Med. 2015


Guillain Barre Syndrome
• Inflammatory disease of the PNS.
• The most common cause of acute flaccid paralysis.
• Typically, weakness and sensory signs in the legs that progress to the
arms & cranial muscles.
• Clinical presentation is heterogeneous & several variant.
• Monophasic disease, progression can be rapid, and most reach their
maximum disability within 2 weeks.
• 20% develop respiratory failure à mechanical ventilation.
• Autonomic involvement (rare) contributes mortality.

Leonhard SE, et al. Nature Review Neurology. 2019


If it is multifocal or generalized:

London ZN. Continuum. 2020


Diagnosis

Leonhard SE, et al. Nature Review Neurology. 2019


Diagnosis
Preceding events
- Infection within 6 weeks
- C. jejuni
- M. pneumoniae
- Zika virus, etc
- Vaccination

Leonhard SE, et al. Nature Review Neurology. 2019


Diagnosis

Features required for diagnosis


• Progressive bilateral weakness of arms and legs (initially only legs may be involved)
• Absent or decreased tendon reflexes in affected limbs (at some point in clinical
course)

Leonhard SE, et al. Nature Review Neurology. 2019


Diagnosis
Features that strongly support diagnosis
• Progressive phase lasts from days to 4 weeks (usually <2 weeks).
• Relative symmetry of symptoms and signs.
• Relatively mild sensory symptoms and signs (absent in pure motor variant).
• Cranial nerve involvement, especially bilateral facial palsy.
• Autonomic dysfunction (but not as first symptoms).
• Muscular or radicular back or limb pain.
• Increased protein level in cerebrospinal fluid (CSF); normal protein levels
do not rule out the diagnosis.
• Electrodiagnostic features of motor or sensorimotor neuropathy (normal
electrophysiology in the early stages does not rule out the diagnosis).

Leonhard SE, et al. Nature Review Neurology. 2019


Diagnosis
Features that cast doubt on diagnosis
• Increased numbers of mononuclear or • Sharp sensory level indicating spinal cord
polymorphonuclear cells in CSF injury
(>50×106/l) • Hyper-reflexia or clonus
• Marked, persistent asymmetry of • Extensor plantar responses
weakness
• Bladder or bowel dysfunction at onset or • Abdominal pain
persistent during disease course • Slow progression with limited weakness
• Severe respiratory dysfunction with without respiratory involvement
limited limb weakness at onset • Continued progression for >4 weeks after
• Sensory signs with limited weakness at start of symptoms
onset • Alteration of consciousness (except in
• Fever at onset Bickerstaff brainstem encephalitis
• Nadir <24hb

Leonhard SE, et al. Nature Review Neurology. 2019


Acute care

Leonhard SE, et al. Nature Review Neurology. 2019


Diabetic Neuropathy
• Most common complications of diabetes
• Earlier onset DM2 vs DM1
• Controlled diabetes up to 42% in 10 years
• Uncontrolled diabetes up to 68% in 4 years
• Diabetic neuropathies encompass a broad range of conditions

Gibbons CH. Continuum. 2020


If it is multifocal or generalized:

London ZN. Continuum. 2020


Length-dependent Acute diabetic Mononeuropathies Autonomic neuropathies CIDP ↑↑
axonal polyneuropathy neuropathies

Gibbons CH. Continuum. 2020


Distal Symmetric Polyneuropathy
(DSPN) of DM
• Accounting for roughly 50% to 75% of cases of diabetic neuropathy,
• Involves small unmyelinated axons (neuropathic pain), and gradually
progresses to involvement of large myelinated axons (motor
complication)
• DM1 : 20% after 20 years
• DM2 : >15% at the time at diagnosis, 50% after 10 years
• Risk factors: glucose control, tobacco, weight, lipid levels

Gibbons CH. Continuum. 2020


Clinical Features DSPN
• The “dying back” neuropathy
• Some patients present with pain in the distribution of
the neuropathy (approximately 25% to 35%), whereas
other patients are asymptomatic
• Examination of muscle strength, deep tendon
reflexes, and sensation (vibration, proprioception,
thermal, pain, and light touch sensation)
• Pinprick loss > 1 sites indicates highrisk of foot
ulceration

Gibbons CH. Continuum. 2020


DSPN of DM Management
• Education
• Glycemic control
• Modifying risk factors: hyperlipidemia, hypertension, obesity, tobacco
use.
• Exercise & dietary recommendation. Alpha-lipoic.
• Gabapentin started with 100mg up to 3000mg, etc, for painful DSPN
• nerve conduction studies (KHS) and EMG

Gibbons CH. Continuum. 2020


Treatment Induced Neuropathy of
Diabetes
• (insulin neuritis)/ (acute painful neuropathy)
• develops suddenly following rapid improvement in glycemic control in
the setting of chronic hyperglycemia
• related to diabetic neuropathic cachexia
• severity is tied to the magnitude and rate of the change in
hemoglobin A1c
• predominantly involves small fiber sensory and autonomic nerve
fibers

Gibbons CH. Continuum. 2020


Clinical Features of Treatment Induced
Neuropathy of Diabetes
• acute onset (2-6 weeks after glucose control) of neuropathic pain in a
length-dependent distribution
• autonomic symptoms (orthostatic intolerance or hypotension,
hyperhidrosis or anhidrosis, early satiety, and erectile dysfunction)

Gibbons CH. Continuum. 2020


Treatment Induced Neuropathy of
Diabetes Management
• avoiding dramatic improvements in hemoglobin A1c
• limiting HbA1c change to 1% reduction per month
• focus on managing symptomatic pain, gabapentin, etc

Gibbons CH. Continuum. 2020


Case
• A 47-year-old woman
• An infection in the bottom of her right great toe, with
osteomyelitis
• Had not noticed the injury
• DM 2 for 8 years
• HbA1c 8% to 9% range.
• Acute asymmetric neuropathy? (the ulcer occurred only in
the right foot, and she had never reported neuropathy
symptoms).
Case
• No symptoms of pain in either foot
• Had not noticed anything wrong with her feet until she
found the injury on her toe.
• Had not noticed the sensation of the cold tile bathroom floor
on her (both) feet in many years.
Case
• Extensor hallucis longus strength 4/5 bilaterally.
• Reduced patellar and absent ankle reflexes bilaterally.
• Absent vibration, pinprick, and temperature sensation at the
great toes, but increase in sensation to the midshin of both
legs.
• Reduced proprioception in the toes and light touch in the
(both) feet.
• Loss of hair and venous stasis changes in the (both) lower
limbs.
Which neuropathy this patient had?
1. Acute asymmetric neuropathy?
0%
2. Distal symmetric polyneuropathy?
0%
3. Mononeuropathy?
0%
4. Autonomic neuropathy?
0%
Further discussion
winnugroho@ui.ac.id

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