Transcript Cranial Neuropathies

Cranial Neuropathies
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Unilateral, electric-like pain in upper, middle,
and/or lower face
Focal demyelination of Trigeminal Nerve
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Mostly seen in elderly patients
Usually a central cause in younger patients (MS)
May be due to intracranial lesion—nerve irritation
Usually diagnosed clinically
Treat with anticonvulsants, muscle relaxants, and
analgesics
Janetta Procedure: relieves pressure on the
trigeminal nerve root
Trigeminal Neuralgia
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Unilateral facial weakness, hyperacusis, ear pain
Inflammation, compression, demyelination, or
infarct of CN VII
 Bell’s Palsy; Lyme disease; sarcoidosis; Melkersson
Syndrome—swelling of lips, eyelids, and face
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History and exam are most important
Most patients recover w/o therapy; recovery time
may be reduced with steroid and antiviral therapy
Monitor patient for corneal irritation
Facial Nerve Palsy (CNVII)
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Unilateral pain in and around throat, jaw, ear,
larynx, or tongue
Usually due to vascular compression or structural
abnormality
Diagnosis made clinically with MRI to look for
certain cancers
90% of patients find relief in local anesthetic applied
to tonsils and pharynx
Anticonvulsants, muscle relaxants, and analgesics
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Refractory cases may require intracranial sectioning
at jugular foramen
Glossopharyngeal Neuralgia
CN IX/X
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Possibly a variant of cluster headaches
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Diagnostic studies:
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Unilateral stabbing, boring, or throbbing pain
localized to temporal, frontal, ocular, aural, and
maxillary region
CT or MIR
Angiography
Test for glaucoma
Treat with Indomethacin 25-50mg up to 3 times
a day
Consider preventative therapy used in
migraine or cluster headaches
Chronic Paroxysmal
Hemicrania
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Unilateral stabbing, throbbing, or dull pain arising
from the region of the cervical carotid artery (jaw,
face, ear, head)
Tenderness, pulsations, and soft tissue swelling
along carotid artery are common
May represent variant of migraines
Must evaluate to exclude other causes of head and
neck pain
Diagnostic tests: ultrasounds, MRI/CT of neck, MRI
or brain, lab studies
Treatment
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Acute: NSAIDs, analgesics
Chronic: Indomethacin25-50mg x3; preventative
migraine medications
Carotodynia
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Acute neck and head pain associated with
stroke-like neurological symptoms
Usually 45 and older
Weakness of arterial wall which results in
separation of internal elastic lamina from the
medial layer
May be caused by trivial trauma
Consider differential diagnosis in evaluation
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MRI of neck, MR or CT angiography of carotid
arteries, LP to look for subarachnoid hemorrhage
Treat with anticoagulation therapy; dissection
will resolve on its own
Carotid Artery Dissection
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Acute outbreak of vesicles preceded by various
sensory disturbances; mostly unilateral in specific
nerve
After initial breakout, pain can persist for weeks to
months: hyperesthesia, allodynia, sharp, stabbing
pain
Zoster infection causes inflammatory necrosis of
DRG that extends to meninges and entry zone of
involved segments
Treatment
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Acute: oral steroids, antivirals, analgesics, low dose
amitriptyline or nortriptyline
Topical agents, oral medications (tricyclic
antidepressants; anticonvulsants), analgesics,
neural blockade
Post Herpetic Neuralgia
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Peripheral neuropathy: dysfunction of PNS
Neuronopathy: dysfunction of nerve cell body
Polyneuropathy: dysfunction of multiple
nerves
Mononeuropathy: dysfunction of a single
nerve, usually due to entrapment
Mononeuritis multiplex: abnormality or
multiple nerves independently involving
different limbs simultaneously
Radiculopathy: abnormality at the nerve root
Polyradiculopathy: abnormalities involving
multiple nerve root levels
Polyradiculoneuropathy: abnormalities
involving multiple nerve roots and nerve
trunks
Definitions
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Axonal: pathological process affects underlying
axon
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Normal conduction velocity
Reduced compound muscle Aps
Demyelinating: pathological process that
damages o rdestroys the myelin sheath
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Delayed conduction
Classification
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Most common cause of peripheral neuropathy
in western world
PROBABLY due to metabolic and vascular
abnormalities
Classifications
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Focal and multifocal
Polyneuropathies
Onset of symptoms may precede diagnosis by 1
to 2 years
Treatment: controlling sugars and then treat
neuropathic pain
Diabetic Neuropathy
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Extensive de- and remyelination or peripheral
nerves causing thickening and palpable
enlargement
Autosomal dominant
Onset usually in first decade; weakness and
wasting begins in lower limbs and progresses
upwards; reduced sensation; slowed motor
nerve conduction velocities; sensorineural
hearing loss; pes cavus
Diagnosis made clinically: history, exam, nerve
conduction studies, genetic testing
Charcot-Marie-Tooth Disease
Type I
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Autosomal dominant
Onset of symptoms in second decade
Slower progression that type I
Usually normal nerve conduction studies
Charcot-Marie-Tooth Disease
Type 2
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Symptomatic within first year of life:
hypotonia, muscular atrophy, weakness, ataxia,
sensory disturbances, facial weakness and thick
lips
Autosomal recessive
Slow nerve conduction velocities; mostly distal
sensory abnormalities, kyphoscoliosis and pes
cavus
Dejerine-Sottas Disease
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Symptomatic between ages of 4 and 7
Demyelinating peripheral neuropathy
Ichthyosis, sensorineural deafness,
cardiomyopathy, retinitis pigmentosa
Nerve damage as result of excess phytanic acid:
block in alpha-oxidation of phytanic to
prostanoic acid
Refsum Disease
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Type V
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Looks like Type I but with pyramidal tract signs
and normal nerve conduction
Type VI
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Looks like HMSN Type I but with optic atrophy
and deafness
Other HSMNs
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Dominant transmission
Recurrent peripheral nerve palsies
Tomaculus Neuropathy
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Acute autoimmune demyelinating polyneuropathy
Medical/neurological emergency
Begins with distal parethesias followed by
ascending muscle weakness—can lead to respiratory
failure
Serious complications may arise due to autonomic
dysfunctions
Preceded by viral or bacterial infection
(Campylobacter jejuni), stress, or can be idiopathic
Diagnosis
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Treatment
 Supportive Care
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LP: elevated protein, no cells
Nerve conduction studies will be abnormal early in
disease
IVIg or plasma exchange
NO ROLE FOR STEROIDS
Disease plateaus around 3 weeks; 80% recover
within 6 months; 15% have residual deficits
Guillain-Barre Syndrome
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Autoimmune disorder characterized by slowly
progressive weakness and sensory loss
Peaks btwn 40 and 60 years of age
Symmetric and involves proximal and distal muscles
Begins in the lower extremities
Doesn’t usually have CN neuropathies associate
with Guillain- Barre
Diagnosis
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Treatment
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CIPD
LP: elevated proteins, no cells
Nerve biopsy shows “onion-bulb formation”
Corticosteroid
Plasma exchange or IVIg
Immunosuppressant drugs for long term
management
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Early Stage:
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Intermediate Stage:
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Multiple mononueropathy
Late Stage:
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ADIP and CIDP
Facial nerve palsy
Distal symmetrical polyneuropathy
Lumbosacral polyradiculopathy
Autonomic neuropathy
Any Stage:
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Mononeuropathies
Herpes zoster radiculitis
HIV Neuropathies
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AKA Parsonage-Turner Syndrome
Usually upper trunk of brachial plexus
May be idiopathic or following illness,
immunization, or surgery
Usually begins as neck, shoulder, or upper arm pain
that is deep or aching
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Can evolve over hours to days
Pt will hold arm close to body and flexed at the
elbow
After several days, sensory loss and weakness begin
to appear—muscle atrophy may occur
Pain usually subsides after 3-6 weeks
Sensory loos and weakness improve over months,
but deficits may persist
Treatment is supportive
Brachial Plexitis
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C spine is most vulnerable to injury because of
its level of mobility and “exposure”
Spinal canal is capacious and so pt who survive
are usually left neurologically intact
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Below C3, canal is smaller, so injury here is more
likely to cause cord injury
C5 and C6 are most commonly injured due to
location at apex of lordotic curve
Cervical Spine
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Hallmark of cord injury is sensory level: most
caudal level of spinal cord with NORMAL
sensory fn
Motor Level: lowest key muscle tested that has
3/5 strength
Neurologic Level: combination of sensory and
motor level; determined clinically
Boney level: vertebra at which the damaged
bone is causing injury to spinal cord
Classification of Spinal
Cord Injuries
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Complete: no demonstrable motor or sensory
fn can be elicited on exam below level of injury
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Incomplete: ANY motor or sensory fn remains
intact
Classification of Spinal
Cord Injuries
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Complete quadriplegia
Incomplete quadriplegia
Complete paraplegia
Incomplete paraplegia
Lesion above T1– quadriplegia
Lesion below T1--paraplegia
Classification of Spinal
Cord Injuries
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Quadriplegia and dissociated sensory loss
Seen most commonly in fracture dislocation
and burst fractures
Most likely cause is infarction to area of spinal
cord supplied by Anterior Spinal Artery
Anterior Spinal Cord
Syndrome
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Ipsilateral motor and position and vibratory
sensation loss with contralateral loss of pain
and temperature one of two levels below cord
injury
Usually caused by “jumped facets”
Some recovery usually seen over time
Brown-Sequard Syndrome
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Disproportionately greater loss of motor power
in upper extremities than in lower extremities
Usually seen after forward fall causing
hyperextension of neck
Central Grey matter motor neurons and medial
most descending motor fibers of CST are most
affected
Better prognosis for return of fn than other
syndromes
Central Cord
Syndrome
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Severe flexion/distraction injury
Usually fatal
Common cause of death in Shaken Baby
Syndrome
Atlanto-Occipital Dislocation
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~5% of all cervical fx
Most common is Jefferson fracture: burst of
ring due to axial loading
Rarely associated with neurological deficits but
fx is considered unstable
Atlas Fractures (C1)
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Type I: tip of the process only
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Type II: fx through base of dens; most common;
unstable
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Suspect central cord syndrome
Type III: fx at base of dens that extends
obliquely into C2 body
Odontoid Fx
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Posterior Element:
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Hangman’s Fx: involves both pedicles of C2;
caused by hyper-extension; unstable
Fx through one or both facets or lamina or
spinous process
Considered unstable unless proven otherwise
Axis Fx (C2)
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C5, C6, C7 are most common vertebrae to fx
Most common fx patterns:
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Incidence of neurological injury increases with
facet dislocation
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Vertebral body fx
Unilateral or bilateral subluxed (“jumped”)
facets
Posterior element fx
Bilateral dislocation: 84% complete cord injury
Fx-dislocation of C6/C7: spinal canal
compromise—cord compression; suspect
anterior spinal cord syndrome or complete
cord lesion
Burst Fx: usually due to vertical compression
on top of head
Sub-axial Spine Fx (C3-C7)
THE INITIAL GOAL IN
THE TREATMENT OF A
CERVICAL SPINE
INJURY IS TO PREVENT
FURTHER
NEUROLOGIC LOSS!
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Immobilization; semi-rigid collar; log roll
If pt is conscious, cooperative, and able to
concentrate:
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AND no neck pain or tenderness on palpation
AND no pain or tenderness with voluntary
movement
AND no neurological deficits,
No further evaluation needed.
If any of the above symptoms are seen, keep pt
in collar and get x-ray of ENTIRE spine
Management
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Reduce subluxation and restore spinal
alignment
Decompress neural elements
Restore stability
Manage ABC’s
Definitive Treatment