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PERIPHERAL NERVE DISEASE (dying-back neuropathy,distal axonopathy).In demyelinating

OVERVIEW inflammatory neuropathies, distal pattern is not found. The
Also called Peripheral neuropathy. Peripheral neuropathy and weakness affect proximal limb and facial nerve before or at the
polyneuropathy are terms that describe syndromes resulting from same time.
diffuse lesions of peripheral nerves, usually manifested by weakness, 2. Tendon Reflexes
sensory loss, and autonomic dysfunction. In small fiber tendon reflex is usually retained, but in neuropathies
that affect the largest diameter,heavily myelinated fibers, the tendon
ETIOLOGY reflexes are diminished early and out of proportion to weakness.
Vitamin Deficiencies (Nutrition) B6, B12, B1 Immune Mediated 3. Sensory Loss
Vascular Damage (decrease oxygen and nutrient to nerve Guillain-Barre andInvariants
most types of neuropathy, all sensory modalities (touch-
leading to neuropathy) IgM antibody associated pain and temperature, vibratory and joint position senses)
pressure,
Infection are impairedor eventually lost, although one may be affected out of
Monoclonal gammopathy
Lyme disease the others, or superficial sensation may be impaired more than deep
Autonomic neuropathy
HIV-1 Vasculitis sensation. Vibratory sense is often affected more than position and
Hepatitis C Sacroid tactile senses
Herpes zoster Celiac disease A pattern that is the converse of the large fiber sensory may be
Cytomegalovirus Rheumatologicalmanifest
diseasesin some diseasesnamely, a loss of pain and temperature
Paraneoplastic sensation
Sjogren syndrome with either sparing or lesser impairment of touchpressure,
Lung cancer Lupus vibratory, and position senses(e.g: Sjogren disease,
Waldenstrom syndrome Wegener scleroderma).
granulomatosis
Myeloma 4. Paresthesias,
Rheumatoid arthritis Pain, and Dysesthesias
Metabolic/Toxic Hereditary Parasthesia: morbid or perverted sensation; an abnormal
CMT1-1 sensation, as burning, prickling, formication, Pins and needles,
Diabetes
CMT-2 stabbing, tingling, prickling, electrical, and Novocain-like feeling.
Renal failure
Thyroid disease MitochondrialThey tend to be especially marked in the hands and feet.
Heavy metal toxicity (Arsenic, Doxorubicin) Other Dysthesia: distortion of any sense, especially of the sense of
touch.(mechanism is still unknown)
5. Sensory Ataxia and Tremor
SYMPTOMATOLOGY OF PERIPHERAL NERVE DISEASE Proprioceptive deafferentation with retention of a reasonable
degree of motor function may give rise to ataxia of gait and of
1. Impairment of Motor Function limb movement. Severe ataxias of this type occur with sensory
Happen because of either segmental demyelination, axonal ganglionopathy. Characteristic of the sensory (tabetic) ataxic gait
interruption, or destruction of motor neurons. The degree of are brusque, flinging, slapping movements of the legs. Loss of
weakness is proportional to the number of axons or motor neurons proprioception may also give rise to small wavering, fluctuating
affected, although pain and kinesthetic loss may add to the movements of the outstretched fingers called pseudoathetotic, or
functional impairment. dancing fingers. An action tremor of fast-frequency type may
The nutritional, metabolic, and toxic neuropathies commonly have also appear during certain phases of a polyneuropathy
distal, axonal pattern. The pathologic changes in such cases begin 6. Deformity and Trophic
in the far distal parts of the largest and longest nerves and Changes In a number of chronic polyneuropathies, the feet,
advance along the affected fibers toward their nerve cell bodies hands, and spine become deformed. For example is pes cavus.
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Caused by: atrophic paralysis of the intrinsic foot muscles while
the bones are forming, that allows the long extensors of the toes
to dorsiflex the proximal phalanges and the long flexors to shorten
the foot, heighten the arch, and flex the distal phalanges.
7. Autonomic Dysfunction
Anhidrosis and orthostatic hypotension,two of the most frequent
manifestations of autonomic dysfunction. They occur most frequently
in amyloidosis and certain other hereditary small-fiber
polyneuropathies, especially diabetic, and several congenital types.
Other manifestations of autonomic paralysis are small or medium-
sized unreactive pupils, lack of sweat, tears, and saliva, sexual
impotence; weak bowel and bladder sphincters with urinary retention
or overflow incontinence; and weakness and dilatation of the
esophagus and colon
8. Fasciculations, Cramps, and Spasms
Chronic root compression can lead to fasciculations or painful
spasms in the innervated muscles. Other closely related phenomena
are spasms or involuntary movements of the toes and feet. Other
possible mechanisms for cramps and spasms are ephaptic cross-
transmission, segmental hyperactivity from deafferentation, and
neuronal sprouting during reinnervation.
CLINICAL PATTERNS OF NEUROPATHY
1. Polyneuropathy
In polyneuropathy, a generalized process affecting the
peripheral nerves, weakness is symmetrical and bilateral, reflexes are
lost in affected parts, but particularly at the ankles, sensory
complaints and loss of sensation are most pronounced distally, and in
the feet before the hands in most cases.
2. Radiculopathy or polyradiculopathy
Polyradiculopathy, a disease of multiple spinal roots, differs
from polyneuropathy in that the neurologic signs are asymmetrical
and with an erratic distribution that may, for example, be proximal in
one limb and distal in another.ln radiculopathy, most often the result
of root compression by disease of the spinal column, is identified by
the presence of pain, sensory, motor, and reflex change solely in the
distribution of a spinal nerve root.
3. Neuronopathymotor or sensory
In sensory neuronopathy, the ganglion cells rather than the
peripheral sensory nerves are predominantly affected. This gives rise
to symptoms and signs of sensory loss in both a proximal and distal
distribution.
Motor neuronopathy is essentially the opposite condition, a
disorder of the anterior horn cells causing weakness, fasciculations,
and atrophy in a widespread distribution
4. Mononeuropathy
Mononeuropathy is the most circumscribed form of peripheral
nerve disease. It is reflected by weakness and sensory loss in the
territory of a single peripheral nerve. Ex: mononeuropathy in L5 cause
weakness in inversion of the foot. Inversion of the foot, innervated by
the tibial nerve, is affected, only with L5 root lesions.
5. Multiple mononeuropathies (mononeuropathy, or mononeuritis
multiplex)
Cumulation of multiple mononeuropathies, termed
mononeuritis multiplex.
6. Plexopathy (involvement of multiple nerves in a plexus)
Usually brachial or lumbosacral, create the most confusing
patterns of motor and sensory involvement; only one limb is affected
but the motor, sensory, and reflex loss do not conform to a pattern of
several adjacent nerve roots or nerves.
DIAGNOSTIC TOOLS
1. Determination of Topogaphy, Characteristic and Time Onset
For example: In the analysis of a polyneuropathy it is of further
value to determine whether the process is predominantly motor with
less sensory involvement (motor-sensory), or the converse
(sensorimotor), or purely sensory, motor, or autonomic.
The time course of the disease is also extremely informative. An
acute onset (rapid evolution usually days) is nearly always diagnostic
of an inflammatory, immunologic, toxic, or vascular etiology.
Subacute (weeks) is usually toxic or nutritional
Chronic (months or year) is usually a hereditary or, rarely, a
metabolic disease. Most of the toxic, nutritional,
2. Neurologic Examination
3. Needle examination of muscles [electromyogram (EMG)]
clarify the type of abnormality in each of the main categories of
neuropathic disease and greatly reduce the number of possible
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diagnoses. In some EMG abnormalities are so characteristic as to (3) nerve and muscle biopsy
virtually define a type of neuropathy (4) measurement of immunoglobulins and antineural antibodies
4. Laboratory procedures that relate to immune-mediated neuropathies;
(1) biochemical tests to identify metabolic, nutritional, or toxic (5) genetic testing for several of the inherited neuropathies
states; (6) nerve biopsy.
(2) CSF examination (increase in protein and in cells that indicate
radicular or meningeal involvement);