polineuropathies

Definitions
Injury of a single peripheral nerve is
called mononeuropathy
Injury of multiple nerves in a symmetric
fashion is called polineuropathy
Injury of noncontiguous nerves from
different plexuses in an asymmetric
fashion is called multiple mononeuropathy
 Peripheral nerves are mixt nerves with motor, sensory
and autonomic fibers.
Clinical picture of a polineuropathy:
Variable motor involvement usually distal starting in the
legs
Hypotonia and atrophy of involved muscles.
Abolished tendon reflexes starting from distal to proximal
bilateral.
Sensory deficit or pain and paresthesias usually distal
progressing to proximal
Proprioceptive troubles (ataxia)
Autonomic disturbance (redness, trophic changes of the
skin, edema).
 Diagnosis
Clinical pattern and symptom evolution
(acute, subacute, chronic)
Paraclinic tests:
Blood tests: metabolic, toxic, autoimmune
Electrophysiology
Imaging studies
Biopsy of muscles and nerves
Genetic tests
 Electrophysiology
Nerve conduction studies for motor and
sensory conduction velocities
Based on stimulation in 2 sites along the
nerve and recording of an action potential
(AP) from the muscle with surface electrodes.
Picture suggesting demielination:
Decreased CV with long distal latencies +/_
conduction bloc (drop in APs amplitude between
2 sites of stimulation with more than 30%)
 Picture suggesting axonal involvement
Low APs amplitudes with a mild decrease of CV,
No conduction bloc

As a rule the APs amplitude is a marker for axonal

loss
And CV is a marker for demielination

Generally speaking most of the polineuropathies are

characterized by a mixt pathology with some
dominance of axonal or demielinating component.
 Left peroneal CB

CIDP patient 50% drop in amplitude between ankle and

colus peroneus stimulation and low CV 24,6 m/s (normal
over 35m/s at 40 y of age)
Needle electrode recordings (detection
studies)
Detection studies are based on muscular activity recordings
with a needle electrode inserted into the muscle we intend
to study.
Usually we can record during the resting state and gradual to
maximal muscle contraction.
During rest, normally we obtain silence = isoelectric line.
As the muscular contraction develops we can see MUPs
(motor unit potentials) triphasic potetials meaning that a
certain motor unit has been recruited into contraction.
The stronger the muscular contraction is, the more MUs are
recruited and the recording is more rich in MUPs until the
maximal contraction status is established being characterized
by a so called interferential recording (succession of MUPs
continuously without any pause).
 Pathologic changes for needle
examination
Neuropathic disorder:
During rest fasciculations, positive waves, complex
repetitive discharges (denervated MU may have
spontaneous, aberrant contractions during the period
of active injuries). When the denervation is
completed (chronic period) this spontaneous activity
disappear.
Fibrilation potentials that could be recorded
during rest are nonspecific being present in either
neuropathic or muscular primary disorders (simply
express the individual muscle fiber spontaneous
contraction as a consequence of membrane
instability, effect of either active denervation or
progressive muscle fibers direct injury ex.
Inflammatory miopathy)
 Pathologic changes
Gradual to maximum contraction
Neuropathic disorders:
Loss of MU and reinervation that occurs as time passes
determine MU reorganization and as a consequence we
can find higher amplitude and longer duration for the
MUPs with no interference pattern to maximal
contraction but only poor recruitment of MU.

Myopathic disorders:
Isolated muscular fibers injury distributed randomly
and muscle fiber splitting resulting in satellites that
could be reinnervated has a consequence of lower
amplitude longer duration MUPs with rapid
interference pattern even at lower amounts of effort.
 Imagistic studies
CT scan, MRI can visualize:
Spinal channel: stenosis, tumors, disc
herniation
Root injury compression, inflammation
Plexus involvement tumoral compression or
infiltration, traumatic avulsion
Nerve trunk compression, tumors,
hypertrophic changes (dysmyelinating
neuropathies)
Muscle atrophy
 Genetic studies
Confirms diagnosis for a genetic disorder
Evaluates outcome
Allows evaluation of genetic risk for
relatives and pregnancy planning
 Biopsy
Nerve:
Usually the sural nerve is preferred (harmless and
informative)
Can detect the primary pattern of involvement
demielynating or axonal
Can detect inflammatory cells, vascular changes, amiloid
infiltration, nevroma, tomaculas (in HNPP)

Muscle:
In neuropathies we can see the atrophy of muscle fibers
that respects MU borders resulting in the so called group
amiotrophy that helps confirmation (sometimes difficult
to make) of differential diagnosis between a primary nerve
or muscle involvement
 Etiological classification of
polineuropathies
I. Acute, predominant motor ( but with
sensory, and autonomic signs as well),
symmetric, distal and proximal involvement:
Guillain Barre syndrome (acute poliradiculoneuropathy)
Viral (Epstein Barr, hepatitic, HIV)
Lyme disease
Porphiria acuta intermitenta
Toxic (TOCP, thallium salts)
Uremic poliradiculoneuropathy
Diphtheritic polineuropathy
Critical illness polineuropathy (critical care patients,
high doses of corticosteroids and respiratory support
for long periods of time usually is a myopathy
 II. Subacute sensorimotor
polineuropathies
A. symmetrical
Deficiency state: alcoholism, pellagra, vit. B12
deficiency, chronic gastrointestinal disease
Poisoning: heavy metals and solvents
Drug toxicity: isoniazid, nitrofurantoin, vincristin,
vinblastin, cloramphenicol, phenitoin
Uremia
Subacute inflammatory polineuropathy
B. asymmetrical (mononeuropathy multiplex)

Diabetes
PAR, vasculitides
Mixed cryoglobulinemia
Sjogren sicca syndrome
Sarcoidosis
Ischemic from peripheral vascular disease
Lyme
C. meningeal based nerve root disease: neoplastic,
granulomas, sarcoid, osteoarthritic spondylitis
 Sydrome of chronic sensorimotor
polineuropathy
A. Acquired forms, less chronic:
Paraneoplastic
CIDP
Paraproteinemias
Uremia
Diabetes
Connective tissue diorders
Amyloidosis
Hypothyroidism
Benign sensory form of the elderly
 B. Inherited, more chronic: - genetic
disorders
Peroneal muscular atrophy (Charcot-Marie-Tooth)
Hypertrophic polyneuropathy of Dejerine-Sotas
Refsum disease
Adrenoleucodistrophy
Amyloid
Fabry
Abetalipoproteinemia
Congenital insensitivity to pain
Dominant mutilating sensory neuropathy in adults
 Guillain Barre Syndrome (acute
inflammatory polyradiculoneuropahy )
Acute ,autoimmune disease, nonseasonal,
nonepidemic
From early childhood to elderly (incidence 3/100 000,
mortality 2-12%)
Most common cause of acute flaccid paralysis after
poliomielitis infection control in developed countries
Mechanism:
segmental demyelination determined by autoantibodies
against myelin components and cell mediated
immunologic reaction directed at peripheral nerve
A viral infection, that occurs several weeks before the
onset, triggers the immune response (respiratory,
digestive Campilobacter Jejuni, vaccination, lymfoma,
exposure to thrombolytic agents.
 Clinical syndrome
Acute ascending weakness that could
evolve to total paralysis develops in hours
or days
Motor deficit affects distal as well as proximal
muscles,
Can cause respiratory failure
Can affect cranial and cervical muscles
resulting in head drop, troubles of deglutition,
facial paralysis, oftalmoplegia
Absent tendon reflexes (areflexia) early
and consistent findings.
 Numbness and paresthesia are frequently
early symptoms
Ataxia is present as a result of
proprioceptive involvement
Autonomic signs (cardiac arrhythmias , BP
oscilations) are common end persist for a
week or two being, sometimes, life
threatening.
 Variants of typical pattern:
Facial diplegia
Ophtalmoplegia, ataxia and areflexia Miller
Fisher variant with a specific antineural antibody
anti GQ1B
Pharingo-cervico-brachial
Purely ataxic
 Diagnosis
During the first week after onset -
paraclinical tests might not be positive so
diagnosis is based on clinical grounds
After 7-10 days
LP CSF analysis increase in protein content
with normal elements = albumino-citologic
dissociation
Some rare cases: protein content could be
normal or CSF analysis might show pleocytosis
(suspect HIV infection)
 Electrophysiology
Nerve conduction studies arguments for
demyelination
Prolonged distal motor latencies (after 3-5 days)
Slowing of MCV (after 7 days)
Conduction block
+/- sensory involvement

Needle electrode studies after at least 10 days

Arguments for active denervation prognostic signs for
incomplete motor recovery due to axonal loss secondary to
severe or persistent segmental demyelination.
Sometimes primary axonal involvement is possible (AMAN
antibodies anti Campilobacter Jejuni and preceded by
diarrhea)
 Complementary tests
Serology is rarely recommended - only
the diagnosis is of doubt
MRI may be helpful to show root
inflammation
Biopsy rarely needed shows multifocal
demyelination and variable degree of
axonal degeneration, scattered cellular
infiltrate throughout nerves and roots.
 Management
During the initial phase (2-3 weeks) the risk of
death is related to respiratory failure and
autonomic involvement with cardiovascular
instability
As a consequence careful follow-up of vital
capacity and cardiovascular function
monitoring are mandator as well as early
treatment of decompensation with respiratory
support.
Careful nursing key role in prevention of long
term complications due to immobilization
Early rehabilitation procedures (mobilization,
positioning, etc)
 Therapeutic interventions
IV IG 2g/kg/5 days total dose
OR
Plasma exchange or plasmapheresis

similar as efficacy
better tolerability for IV IG
 Outcome
~3% mortality even in the most competent
centers.
~50% good recovery without significant
residual deficit
~40% various degree of motor residual
deficit or sensory impairments ( pain,
dysesthesias)