UKDI PREPARATION I:

Neurogenic Pain Syndromes

Peripheral Nerve Disorders
Dept. Neurology
College of Medicine
Atma Jaya University
 Patient presents with complaint of a headache

Detailed history*
1. Characteristics of the headache
2. Assess functional impairment
3. Past medical history
4. Family history of migraines
5. Current and previous medications for headache (Rx and over-
the-counter)
6. Social history
7. Review of systems – to rule out systemic illness

Focused physical examination

Focused neurological examination

 Consider primary
headache • Red flag features:
1. Subacute and/or progressive
headache over months 
No SOL/Tumor
2. New or different headache 
Causes
SOL/Vascular
3. “Worst headache ever” 
for Vascular;SAH
concern 4. Any headache of maximum severity
at onset  Vascular
? 5. Onset after the age of 50 years old
 Tumor
6. Symptoms of systemic illness
Yes (infection, encephalopathy)
7. Seizures (focal damage
Consider secondary Tumor/vascular)
headache 8. Any neurological signs
 Diagnosis of primary headache
An inadequate history is the probable cause of most
misdiagnosis of headache type.

Classification of primary headache

1. Migraine
2. Tension-type headache
3. Cluster headache and other Trigeminal autonomic
cephalalgias
4. Other primary headaches
Practitioners should consider using headache diaries and
D appropriate assessment questionnaires to support the
diagnosis and management of headache
 MIGRAINE
 Second most common cause of primary headache.
 It has a global lifetime prevalence of 10% in men and 22% in
women.
 a benign and recurring syndrome of headache associated with
other symptoms of neurologic dysfunction in varying admixtures.

TYPES OF MIGRAINE

1. Migraine with aura

2. Migraine without aura
3. Retinal migraine
4. Childhood periodic syndromes that are commonly precursors of migraine, eg
cyclical vomiting, abdominal migraine
5. Complicated migraine, eg status migrainosus, chronic migraine
6. Probable migraine
 CHARACTARISTICS OF MIGRAINE
 Episodic moderate to severe headache that causes
disability
 Unilateral
 Pulsating
 Builds up over minutes to hours
 Moderate to severe in intensity
 Associated with nausea and/or vomiting and/or
sensitivity to light and/or sensitivity to sound
 Aggravated by routine physical activity
 Typical aura (in 15 – 33% of patients with migraine)
 Exacerbation by physical activity
 Sensitivity to light between attacks
 Positive family history of migraine.
 Simplified Diagnostic Criteria for Migraine

Repeated attacks of headache lasting 4–72 h in patients

with a normal physical examination, no other reasonable
cause for the headache, and:
At least 2 of the following : Plus at least 1 of the
following :

Unilateral pain Nausea/vomiting

Throbbing pain Photophobia and

phonophobia
Aggravation by movement

Moderate or severe intensity

 Patients who present with a pattern of recurrent episodes of
severe disabling headache associated with nausea and
C sensitivity to light, and who have a normal neurological
examination, should be considered to have migraine.

INVESTIGATIONS OF MIGRAINE
Neuroimaging is not indicated in patients with a clear history
D of migraine, without red flag features for potential secondary
headache, and a normal neurological examination.
• Dietary-foods, alcohol,hunger, caffeine withdrawal
• Sleep Related-deprivation, excess, irregular
• Hormonal-menstrual,O.C.,menopause, HRT
• Physiological-fatigue, travel,exercise,smoking
• Emotional -anxiety, stress, relaxation post stress, excitement
• Physical- neck/back injury, head trauma, hypertension
• Flickering lights
• Sunlight/ Bright lights
• Heat
• High altitude
• Loud noise
• TV/ VDU screens
• Strong smells
• Meterological changes
• Barometric Changes
• unilateral scotoma
• Hemianopia
• Teichopsia-flashes of
light
• Fortification spectra
 TREATMENT OF PATIENTS WITH MIGRAINE
ACUTE TREATMENT

B Paracetamol 1,000 mg for mild for moderate migraine.

Aspirin 900 mg or ibuprofen 400 mg for all severities.

A
A combination of Aspirin and metoclopramide can be used for the
B treatment of patients with acute migraine attacks.

Oral and rectal anti-emetics can be used to reduce symptoms of nausea

D and vomiting and to promote gastric emptying.

A Oral triptans are recommended for all severities of migraine

Triptans should be taken at, or soon after, the onset of the headache
D phase of a migraine attack.
 Almotriptan 12.5 mg, eletriptan 40-80 mg or rizatriptan 10 mg, are the
A preferred oral triptans for acute migraine.

A combination of sumatriptan 50-100 mg and naproxen sodium 500 mg

C may be helpful in acute migraine particularly in prolonged attacks which
are associated with recurrence.

Opioid analgesics should not be routinely used for the treatment of

D patients with acute migraine due to the potential for development of
medication overuse headache.

CONSIDERATIONS
Practitioners should recognize that a patient’s standard therapy may not
give a consistent response.

When initiating acute treatment for migraine the risks of medication

overuse headache should be discussed with the patient.
 MIGRAINE AND ORAL CONTRACEPTION
Women with migraine with aura should not use a combined oral
B contraceptive pill as they have a relative risk of 8.72 for developing stroke.

Patients with migraine without aura who are over the age of 35 should
D not use a combined oral contraceptive pill as they have an increased risk
of ischemic stroke.

MIGRAINE AND PREGNANCY

Paracetamol 1,000 mg is the treatment of choice in pregnancy for all
patients with migraine and tension-type headache when the pain is
sufficient to require analgesia.

Aspirin is contraindicated during the third trimester of pregnancy. Long

term exposure or exposure to high doses of ibuprofen in late pregnancy is
associated with an increase d risk of fetal complications.
 TREATMENT OF PATIENTS WITH MIGRAINE (CONTD.)
PROPHYLAXIS
Trials demonstrate that prophylaxis provides reduction in severity
and frequency of headaches by 50%.

Betablockers
Propranolol 80-240 mg per day is recommended as first line therapy for
A prophylaxis in patients with migraine.

Antiepileptics
topiramate 50-200 mg per day for episodic migraine and chronic migraine.
A
Sodium valproate 800-1,500 mg per day for episodic migraine.
gabapentin 1,200-2,400 mg per day to reduce headache frequency for
C episodic migraine and chronic migraine.
Antidepressants
Amitriptyline 25-150 mg per day recommended for patients requiring
prophylaxis of migraine.

B Venlafaxine 75-150 mg per day is an effective alternative to tricyclic

antidepressants for prophylaxis of migraine.

NON-PHARMACOLOGICAL MANAGEMENT
Stress management should be considered as part of a
combined therapies program to help patients reduce the
frequency and severity of migraine headaches.
B Acupuncture should be considered for preventive
management in patients with migraine.
 TENSION-TYPE HEADACHE
 Tension type headache is the most common primary headache
disorder
 It has a global lifetime prevalence of 42% in men and 49% in
women
 The pain is generally not as severe as in migraine

CHARACTERSITICS
 bilateral
 pressing or tightening in quality
 mild to moderate in intensity
 no nausea
 not aggravated by physical activity
 may be pericranial tenderness, sensitivity to light or noise
 A diagnosis of tension type headache should be considered in a patient
C presenting with bilateral headache that is non-disabling where there is a
normal neurological examination.

TREATMENT OF PATIENTS WITH TENSION-TYPE HEADACHE

ACUTE TREATMENT
Aspirin and paracetamol are recommended for acute treatment in
A patients with tension-type headache.

PROPHYLAXIS

Tricyclic antidepressants, particularly amitriptyline, 25-150mg per day,

A are recommended as the agents of choice where prophylactic treatment
is being considered in a patient with chronic tension-type headache
 Headache type
Cluster headache Migraine

Duration 15 mins – 3 hrs 4 – 72 hours

Onset Rapid Gradual

Frequency 1 every other day-8/day <1/year-1/day

( median 1-2/month)

Restlessness during an 100% 0%

attack
Ipsilateral Prominent Occasional
autonomic
features
When a patient presents with frequent, brief, unilateral headaches with
D autonomic features a trigeminal autonomic cephalalgia should be
considered, and the patient should be referred for specialist assessment.
• Stereotypical episodic headache
• Frequent attack of short lasting, severe unilateral
head pain
• Autonomic symptoms
– Conjuctival injection
– Nasal congestion
– Rhinorrhea
– Partial horner’s syndrome
• Risk: smokers, alcohol usage.
TREATMENT OF PATIENTS WITH CLUSTER HEADACHE
ACUTE TREATMENT
Subcutaneous injection of 6 mg sumatriptan is recommended
as the first choice treatment for the relief of acute attacks of
cluster headache.
A
Nasal sumatriptan or zolmitriptan is recommended for treatment of
acute attacks of cluster headache in patients who cannot tolerate
subcutaneous sumatriptan.

PROPHYLAXIS
Verapamil, 240-960 mg is recommended for the prophylaxis
B of cluster headache.
• Sudden onset of excruciating intense stabbing
pain (during waking hours)
• Brief attacks (<2 minutes each)
• Almost always precipitated by trigger stimulus or
activity (e.g. chewing, speaking, swallowing,
touching the face, cold air, tooth brushing,
shaving)
• Located in the distribution of one or two
branches of trigeminal nerve, usually V2 and/or
V3
• May persist for weeks to months or
spontaneously remit for weeks to months
• Two forms
– Idiopathic: microvascular compression of
trigeminal nerve root
– Symptomatic: cerebellopontine angle tumors,
multiple sclerosis, vascular malformation
• 90%of patients are over the age of 40
• Diagnosis in younger patients should prompt
an evaluation for secondary causes
• 10% of patients harbour an intracranial lesion
therefore an MRI is recommended
• Treatment
– Spontaneous remissions
are common
– After 8 weeks of
successful therapy
successful therapy, slow
drug taper is
recommended
BELL’S PALSY
• Symptoms
• Pain / paresthesia
• Weakness
• Signs
• Reflex abnormalities
• Provocative Testing
• Spurling’s
• Abduction relief (Bakody’s)
• Upper Limb Tension test
• Neck Distraction test
 SYMPTOMS

Pain / Paresthesia
- Neck , Shoulder ,
Scapula , Arm ,
Hand

- Consider nerve
root distribution
 SYMPTOMS

Weakness
- Deltoid, Biceps,
Triceps, Wrist (ext/
flex), Interossei

- Consider nerve
root distribution

T1
Reflex Abnormality
- Biceps, Triceps,
Brachioradialis
 Provacative Tests

Spurling’s
Abduction Relief
(Bakody’s)
Upper Limb Tension Axial loading of slightly extended
Neck Distraction and rotated neck causing radiating
pain down upper extremity
Sensitivity 30-50%
Specificity 74-90%
 Provacative Tests

Spurling’s
Abduction Relief
(Bakody’s)
Upper Limb Tension Patient places hand of affected
side on head– relief of pain from
Neck Distraction reduced stretching pressure on
nerve root.
Sensitivity 30-42%
Specificity 90-100%
 Provacative Tests

Spurling’s
Abduction Relief
(Bakody’s)
Upper Limb Tension Patient abducts , extends shoulder with
elbow extended, hand supinated and –
Neck Distraction increased pain with contralateral neck
side bending; relief with ipsalateral side
bending
Sensitivity 97%
Specificity 22 %
 Provacative Tests

Spurling’s
Abduction Relief
(Bakody’s)
Upper Limb Tension Examiner with one hand on chin and
one on occiput lifts head and applies
Neck Distraction
10-15kg axial traction – pain relief
with distracting force
Sensitivity 44%
Specificity 90 %
Naffziger Test
 Valsava Maneuver
• The patient is seated and instructed to take a
deep breath and hold it while attempting to
exhale for 2-3 seconds. A positive response
occurs with reproduction of symptoms.
• The pushing increases intrathecal or
intraspinal pressure revealing presence of a
space occupying mass such as and extruded
intervertebral disc, or narrowing due to
osteophytes.
Lumbosacral
Sprain/Strain
 Mechanical stresses on spine
• Twisting
• Flexion
• Extension
• Rotation
• Lifting
• Repetitive work
• Static postures such as sitting at a
desk
Patterns of some back disorders
 Lumbosacral Sprain/Strain
Spraiin: Lig. Injuries
• sudden violent
contraction
• sudden torsion
• severe direct blows
• forceful straightening
from a crouched position.
• posterior ligaments are
more prone to injury.
Strain: tears of the muscle –
tendon unit
• violent muscular
contraction during an
excessively forceful
muscular stretch
• The most susceptible
muscles are those that span
several joints.
Lumbosacral Dermatomes
NEUROLOGIC LEVEL

L4 L4

L5

S1

MOTOR REFLEX SENSATION

TIBIALIS PATELLAR
ANTERIOR TENDON
(FOOT INVERSION)

S1 L5 L4
NEUROLOGIC LEVEL

L5 L4

L5

S1

MOTOR REFLEX SENSATION

EXTENSOR
DIGITORUM
LONGUS

NONE S1 L5 L4
NEUROLOGIC LEVEL

S1 L4

L5

S1

MOTOR REFLEX SENSATION

PERONEUS ACHILLES
LONGUS and TENDON
BREVIS

S1 L5 L4
• Symptoms
• Pain / paresthesia
• Weakness
• Signs
• Reflex abnormalities
• Abnormal gait (foot drop)
• Provocative Testing
• Straight leg raise
• Crossed straight leg raise
• Reverse straight leg raise
 SYMPTOMS

Pain / Paresthesia
- Buttock , Thigh
Leg , Foot

- Consider nerve
root dermatomal
distribution (but
overlap is common)
 SYMPTOMS

Weakness
- Buttock , Thigh
Leg , Foot

- Consider nerve
root dermatomal
distribution (but
overlap is common)
Reflex Abnormality
- Patellar (L4)
Achilles (S1)

Gait Abnormality
- Foot drop (L5)
 Straight Leg Raise
Crossed Straight Leg
Raise
Examiner raises straight leg (30 to 60
Reverse Straight Leg
degrees) eliciting radicular pain on same
Raise
side (Lasegue Sign). Then lowers leg
until pain goes away, the foot is then
dorsiflexed causing return of pain
Sensitivity 91%
Specificity 26 %
 Straight Leg Raise
Crossed Straight Leg
Raise
Reverse Straight Leg Examiner raises straight leg (30 to 60
Raise degrees) eliciting radicular pain on
opposite side.
Sensitivity 25%
Specificity 90-97%
 Straight Leg Raise
Crossed Straight Leg
Raise
Reverse Straight Leg Patient is prone, examiner raises
Raise straight leg (30 to 60 degrees) –pain
radiating to anterior thigh indicative of
L3-L4 root irritation
Sensitivity ?
Specificity ?
 • Neuroimaging
• MRI
• CT/MRI myelography

• EMG/NCS

• Laboratory
Additional Diagnostic
• CSF
Tests if MRI
• Metabolic
Neuroimaging are
• Serology/culture
Non-Diagnostic
 • Pain at night/lying down
Tumor Cauda
Malignancy • Fever Equina
• Saddle anesthesia
• Bowel/Bladder dysfunction
Cervical
Spinal • Rapidly progressing Infection -
Stenosis Abscess
neurologic deficit
• Bilaterally symptoms
Vascular • Significant Gait problems
Inflammatory
Infarction /
AVM
• Personal Hx of Cancer Demyelinating
disease