PERIPHERAL NEUROPATHIES

Overview
- Peripheral nerve disease or neuropathy is categorized by distribution into focal disease
(mononeuropathy or multiple mononeuropathies) or widespread disease (polyneuropathy) and by
underlying pathology into axonal (sparing the myelin sheath), demyelinating (sparing the axon) or
mixed types
- Peripheral neuropathies may affect motor, sensory or autonomic nerves or a combination of these
- Peripheral neuropathies are differentiated from CNS dysfunction by history and examination
findings

Features PNS origin CNS origin

Onset Subacute or insidious Sudden (stroke) or gradual
(space-occupying lesion
Motor symptoms/distribution Yes Yes
Focal, unilateral or generalized Focal or unilateral
Sensory symptoms/ distribution Possible Possible
Focal, unilateral or generalized Usually focal or unilateral
Cramps and fasciculation Possible No
Atrophy Yes (sometimes prominent) No
Fasciculation Possible No
Stretch reflexes Normal or diminished Hyperreflexia, pathologic
Sensory abnormalities In nerve territory, dermatome, or In an entire limb
stocking-glove distribution
EMG Positive results Negative results

Classification, findings and diagnosis

- Peripheral neuropathy sensory
symptoms can be classified as
negative (numbness), positive
(tingling, burning, and
dysesthesia) or both together
- The distribution of sensory
symptoms reflect the peripheral
nerve territory involved
- Motor symptoms can follow or
coexist with sensory
abnormalities
- Dysfunction of autonomic nerves
results in:
o Orthostatic hypotension
o Cardiac arrhythmias
o Changes in sweating
patterns
- Diabetes mellitus is the most
common cause of peripheral
neuropathy but the differential
diagnosis includes:
o Vasculitis
o Inflammatory diseases
o Infections
o Connective tissue
diseases
o Infiltrative diseases
o Paraneoplastic
syndromes
- Physical findings include: weakness, sensory loss, hyperesthesia, decreased or absent deep tendon
reflexe
- Small-fiber neuropathy, affecting pain and temperature transmitting fibers, can be particularly
challenging to diagnose because sensory and autonomic symptoms occur in the absence of
clinical weakness
 - BURNING dyesthesia of the feet or hands is characteristic feature of small-fiber neuropathy and
occurs in patients with poorly controlled DM
- Appropiate laboratory tests:
o Serum protein electrophoresis
o Blood count
o Erythrocyte sedimentation rate determination
o Measurement of fasting plasma glucose
o Hemoglobin glycosylated
o Vitamin B12
o LCR analysis in patients with rapidly progressive neuropathy  Guillan Barré
o EMG  nerve conduction studies and needle electrode examination (helps confirm and
quantify peripheral neuropathy)
o Needle electrode examination can detect active denervation of muscles only after at least 3
weeks have elapsed since nerve injury
- Small fiber neuropathies are best evaluated by quantitative sensory testing for pressure and
thermal function, a quantative sudomotor axon reflex test for sweating and determination of
intraepidermal nerve fiber density on skin biopsy
- Sural nerve biopsy can be useful in suspected vasculitis or amyloidosis
- Despite extensive testing, no cause is found in up to one third of patients with peripheral neuropathy

Bell Palsy
- Paralysis of the facial nerve resulting in weakness of ipsilateral facial muscles
- The peripheral nerve compression can be differentiated from central causes by evaluating forehead
and periorbital muscles
- With a lesion in the central nervous system, lower facial muscles are primarily affected and the
forehead and orbicularis oculi muscles are relatively spared (lower half of face)
- Peripheral nerve compression, as seen in Bells palsy causes more widespread facial paralysis
including the upper and lower face, inability to close both eyes tightly or raise both eyebrows
o Patients may also report hyperacusis, dry mouth, and impaired taste
o Abrupt onset of symptoms building over 1 to 2 days is typical
- Patching the eye and lubricating the cornea are essential to prevent abrasions
- 70% of patients recover, although some patients are left with weaknesses and evidence of aberrant
reinnervation causing synkinesis (such as voluntary smiling causing involuntary eyelid closure)
- use of prednisone in the first 72 hours has been shown in clinical trials to hasten the time of
recovery and increase the percentage of patients with complete recovery

Neuropathies of Diabetes Mellitus

- DM can cause almost any kind of neuropathy
- Predisposes to multifactorial nerve injury due to nerve
compression, ischemia, inflammation, and metabolic
changes
- Distal sensorimotor peripheral neuropathy is the most
common and presents with numbness, tingling, and
burning pain in a stocking-glove distribution
- Single mononeuropathies (such as median, ulnar and
isolated cranial neuropathy) are common complications of
diabetes
- In patients with lONG-standing diabetes, autonomic
dysfunction becomes increasingly prevalent
- Small fiber neuropathy occurs in patients with mild to
severe diabetes and even in patients with only impaired
glucose tolerance
- Patients typically report burning, lacinating extremity pain
without weakness
TRIGEMINAL NEURALGIA
- Older adults  after 40 years (90%)
- Recurrent episodes of brief electrical shooting pain limited to the face or forehead
- Most frequently diagnosed form of facial pain (4/100.000)
- This usually affects second or third division of the trigeminal nerve: (V1 is ophthalmic)
o Second (maxillary)  cheek
o Third (mandibular)  jaw, chin
- *** patients who have herpes zoster reactivation affecting the trigeminal nerve, commonly begins in
the ophthalmic branch and leads to clinical symptoms in the forehead, cornea and nose (V1)
- Mostly unilateral
- The pain only lasts 1 second – 2 minutes but it can recur
- Triggering factors: talking, chewing, touching specific trigger points (ex. Shaving) , eating, applying
cosmetics, brushing the teeth
- Sometimes exposure to breeze, bright light, or loud noise also can be provocative
- The pain can recur daily for weeks to months and then spontaneously remit for months or years or it
may display a more chronic progressive course
- The likely cause is vascular compression of the trigeminal root entry zone at the level of the pons
which leads to focal demyelination and aberrant neural discharge
- In younger people the cause is sclerosis multiple
- MRI WITH CONTRAST SHOULD ALWAYS BE OBTAINED EVEN THOUGH LESS TAN 10% OF
PATIENTS WILL HAVE A CAUSATIVE INTRACRANIAL LESION
- TREATMENT:
o CARBAMAZEPINE: should be initiated at low dose (100 mg twice daily) then gradually
increase the dose
o Add second line agent when carbamazepine isn’t enough  baclofen, gabapentin,
clonazepam, lamotriginic
o Patients should receive an adequate trial of at least 3 drugs before surgical treatment is
considered
- Other types of treatment when pharmacological treatment is unsuccessful include: nerve fiber
destruction, ballon compression, thermal lesioning, microvascular decompression

HERPES