Peripheral Neuropathies Classification and Management

20-12-2017 Jipmer physiologist 1
By : Dr. Radhika Mantry
Guide : Dr Himanshu Dua

Damage to nerves which may be caused either
by diseases or trauma to the nerve or as a
component of systemic illness

 In most common forms of polyneuropathy, the
nerve fibers most distant from the brain and
the spinal cord malfunction first.
 Pain and other symptoms often appear
symmetrically

 The peripheral nerves include:
 Cranial nerves
(with the exception of the second)
 Spinal nerve roots
 Dorsal root ganglia
 Peripheral nerve trunks and their terminal branches
 Peripheral autonomic nervous system

In chronic course symptoms worse, muscle
wasting, paralysis, or gland dysfunction

 Distal sensory loss
 Distal weakness and
atrophy
 Decreased or absent
reflexes
 Ankle jerks lost first

Various classifications

Hereditary Neuropathies
(A) Hereditary motor and sensory neuropathy
1. Hereditary motor and sensory
neuropathy – type I
2. HMSN – Type II
3. Dejerine – Sottas Neuropathy HMSN-
type III
4. HSMN – type IV
5. HSMN – type V
B – Distal Hereditary motor neurono pathies
(dHMN)
C – Hereditary Sensory and Autonomic
Neropathy (1 to 5)
Inflammatory neuropathies
 Immune mediated
o Guillain-Barré syndrome(AIDP)
o Chronic inflammatory demyelinating
polyradiculoneuropathy
 Infectious
 Leprosy
 Diphtheria
 Varicella – zoster
Acquired metabolic and toxic
neuropathies
 Peripheral neuropathy in adult
onset Diabetes
 Metabolic and nutritional
peripheral neuropathies
 Neuropathies associated with
malignancy
 Toxic neuropathies
Traumatic neuropathies

Axonal
Vincristine
Nitrous oxide
Colchicine
Isoniazid
Hydralazine
Metronidazole
Pyridoxine
Lithium
Alfa interferon
Dapsone
Phenytoin
Disulfiram
Chloroquine
Ethambutol
Amitriptyline
o Demyelinating
Amiodarone
Chloroquine
Suramin
Gold
o Neuronopathy
Thalidomide
Cisplatin
Pyridoxine

• Motor , sensory, or autonomic
 Mononeuropathy , polyneuropathy or
mononueritis multiplex
 Focal, multifocal or symmetric
 Proximal or distal
 Axonal, demyelinating or both
 Acute, sub acute or chronic

 Some neuropathies may affect all three types
of nerves, others primarily affect one or two
types.
 Predominately motor neuropathy
 Predominately sensory neuropathy
 Sensory-motor neuropathy
 Autonomic neuropathy
 Impaired function and symptoms depend on
the type of nerves that are damaged.

 Mononeuropathy involve damage to only one
nerve
 When multiple nerves supplying one limb are
affected-called polyneuropathy.
 Two or more isolated nerves in separate areas
of the body are affected-called mononeuritis
multiplex

o Focal neuropathies include common compressive
neuropathies such as carpal tunnel syndrome, ulnar
neuropathy ,peroneal neuropathy
o Multifocal neuropathy suggests a mononeuritis
multiplex that may be caused, for example, by
vasculitis or diabetes

 Primary destruction of the axon with
secondary degeneration of its myelin sheath
 Generalized abnormality in the neuron cell
body- neuronopathy
 Abnormality in the axon - axonapathy

15
Dysfucntion of Schwann cell or damage to the myelin sheath
Denuded axon provide signal for remyelination
Precursor cells within endoneurium replace injured cells
Cells proliferate and engulf axon.= remyelination in time

o The prototype
o Distal paresthesia with symmetric weakness
o Distal areflexia
o Variants are pure motor, pure sensory, autonomic,
relapsing, and Miller fisher types
o Cranial nerves esp facial n and bulbar may be involved
o Respiratory muscles are severely involved in about 25

 Pathological findings include inflammatory and
demyelinating changes.
 Monocytes and macrophages appear to attack myelin
sheaths.
 Myelinated fibers show segmental demyelination
during the first few days. Segmental remyelination
occurs subsequently.
 The lesions have a perivenular distribution

 Chronic progressive or relapsing neuropathy, motor >
sensory.
 Electrophysiology: slow conduction velocity &
conduction block
 Pathology: segmental demyelination and
remyelination, onion bulbs, fibrosis and little or no
lymphocytic infiltration of tissue.

 Onset of neuropathy depends upon the
duration of illness
 50% diabetics have peripheral neuropathy of
which 80% have had the illness for >15 years
 Distal symmetric sensory or sensorimotor,
autonomic, focal or multifocal asymmetric
 Symmetric neuropathy involves distal sensory ,
motor nerves .
 Decreased sensation, loss of pain sensation –
ulcer

1. Hereditary motor sensory neuropathy [ I – VII ]
(Charcot Marie Tooth Disease)
21
CMT 1 CMT 2 CMT3 – Dejerine
Sottas syndrome
1 Demyelenating Axonal Demyelenating
(Hypomyelenating)
2 1st – 2nd decade Later Neonatal
3 AD AD/AR/X linked AD
4 Severe Muscular
Atrophy
Milder Hypotonia ,breathing
and feeding difficulty
,pupilary abnormality,
arythrogryposis
5 Sensory Involvement Decreased Present
6 Onion Bulb Absent Present
7 NCV Decreased Less Decreased Severely Decreased

1. 1st decade
2. Lower > Upper
3. Distal > Proximal
4. Stork Legs
5. Hammer toes
6. Cumberland Ankle instability tool
7. Foot Drop
8. Claw Hand
9. Rhomberg Sign – Prop & Vibration
10. DTR Lost

1. NCV < 38 m/s
2. EMG - Cycle of Denervation and Renervation
3. CSF Protiens May be Elevated
4. Diagnostic Test – Surnal Nerve Biopsy
5. Onion Bulb Formation – Interstitial
hypertrophic neuropathy

1. Stabilisation of Ankle Joint
2. Stiff Boot that extend to mid calf
3. Light weight plastic splint custom made to
extend beneath the foot & around the back of
the ankle
4. External short leg braces
5. Surgical fusion of ankle

1. AR
2. Phytanic acid −−−− Pristanic Acid
3. Infantile Refsum Disease - Zellweger
Syndrome Secondary to Primary Peroxismal
Disorder
• Ataxia
• Anorexia
• Icthyosis
• SNHL
• Retinitis Pigmentosa
• Steatorrhea
Phytanic acid oxidaseSpinach ,nuts coffee
• Skeletal Malformations
• Cardiac Defects
• Conduction defects
• Cardiomyopathy

1. Friedreichs Ataxia
2. Giant Axonal Neuropathy
3. Infantile Neuropathy
4. Metachromatic Leukodystrophy
5. Adrenoleukodystrophy
6. Krabbes Disease
The structure of central and peripheral myelin is essentially the same. Myelin is
composed of 70% lipids and 30% protein. There are some important differences
in myelin proteins between CNS and PNS. These differences explain why an
allergic reaction against PNS myelin does not cause central demyelination and
vice versa; and why inherited metabolic disorders of myelin proteins that affect
peripheral nerves do not damage central myelin. On the other hand, lipids are
similar between PNS and CNS myelin. For this reason, metabolic disorders of
myelin lipids, such as metachromatic leukodystrophy, affect both, the central
white matter and peripheral nerves.

1. AR
2. Chromosome – 9
3. Frataxin Protein
4. GAA repeats
5. Cf –
 Progressive Ataxia
 Dysarthria
 Position vibration loss
 Pes cavus
 DTR
 Intellect preserved
 Cardiomyopathy
6. Treatment – No Cure (Symptomatic)

1. AR
2. Chromosome 16 – Gigaxonin
3. Progressive Axonal Neuropathy
4. Abnormal Cytoplasmic Microfilament – Shwann Cells,Fibroblast
,Endothelium
5. Cf -
 Progressive Gait Disturbance
 Kinky Hairs
 Long curly eyelashes
 High Forehead
 Leucoencephalopathy and Cognitive impairment
 Declining Motor function
 CNS Involvement
 Optic Atrophy
 MR
 Spasticity
6. Diagnosis – Peripheral Nerve shows Axonal loss and increased Neuro
filaments

1. PLA2G6 gene Mutation
2. Cytosolic Phospholipids – membrane homeostasis
3. Cf –
 Loss of mental plus motor milestones
 Seizures
 Progressive Motor Sensory Neuropathy
 Hypotonia
 Decreased DTRs
 Neuroregression
 Urinary retention
 Hypothalamic Failure –DI ,Hypothyroidism
 ANS Affected
Diagnosis –
Central ,peripheral ,Autonomic neurons contain Spheroids Of Various sizes
Sural Nerve Biopsy – PAS Positive ,Ballooning of axons ,Spheroids Present

 Mutations:
•arylsulfatase A gene (ARSA) on
chromosome 22q13
•AR
 Late infantile (18-24 months)
 Gait disturbances
 Memory deficits
 Seizures (may be present)
 Loss of motor developmental
milestones
 Decreased attention span
 Speech disturbances
 Decline in school performance
 Investigations:
 Spinal fluid – moderately elevated
protein at 1.5 – 3.0 g/L Urine
 Deficiency in arylsulfatase A
activity (or in leukocytes)
 Metachromatic granules
 Cholecystogram/ultrasound –
decreased gall bladder function
 Evoked potentials – abnormalities in
ABR, VEP, SSEP
 Nerve conduction velocities
decreased
 MRI – symmetric diffuse signal
abnormalities

 Decreased oligodendrocytes in areas of demyelination
 Globoid cells – periodic acid-Schiff (PAS) staining cells in CNS white
matter
 Genetics:
 Galactocerebroside ß-galactosidase (GALC gene, chromosome 14)
 AR
 Pure neurologic condition
 Onset at 3-8 months of age
 Irritability, intermittent fevers, heightened startle reflex, feeding
problems
 Develop seizures, opisthotonus
 Deafness and blindness by 9 months
37

 Predominant pathology is axonal neuropathy.
 In chronic cases segmental demyelination also seen
Pathophysiology –
 Loss of small myelinated fibers and unmyelinated
fibers. But large fibers can also be affected.
 Endoneurial arterioles show thickening,
hyalination, intense PAS positivity in the walls and
extrensive reduplication basement membrane

 Nerve conduction studies
 Diminished or absent SNAP amplitude in the
setting of normal motor nerve conduction
velocity

 Paresthesia and dyesthesia of feet and distal
legs
 Wasting is marked
 Loss of ankle reflex
 Pathophysiology – evidence of degeneration of
distal portion of axons

Nerve conduction studies
 Reduced or absent SNAP
 CMAP amplitude decreases and motor
conduction velocity also decrease in later stage
EMG
 Fibrillations and positive sharp waves are
prominent in distal muscles.
 Temporal dispersion on proximal stimulation
is not found as in demyelinating neuropathies

 Diabetes
 Uremia
 Paraproteinemia

 Paresthesia, dyesthesia or numbness
 Reduced vibration and two point
discrimination
 Pathophysiology – segmental demyelination
and remyelination along with axonal
degeneration

 Nerve conduction studies
 Reduced or unrecordable CMAP, SNAP or
both
 Moderate to severe slowing of NCV with
temporal dispersion of CMAP

 Thorough history and physical examination is
needed.
 Cranial nerve examination
 Motor , sensory, autonomic nervous system
examination
 Fundus examination
 Lymphadenopathy , hepatomegaly or
splenomegaly, and skin lesions

 CBC, electrolytes, ESR
 Fasting serum glucose, glycosylated hemoglobin,
blood urea nitrogen, creatinine,
 Liver , kidney,, thyroid function studies
 Inflammatory markers,
 Total protein level
 Vit D, B12,
 Cytology
 CSF
 Urinalysis
 Nerve biopsy

 EMG and nerve conduction studies (NCS) are often the
most useful initial laboratory studies in the evaluation
of a patient with peripheral neuropathy
 Confirm the presence of a neuropathy
 Provide information as to the type of fibers involved
(motor, sensory, or both), the pathophysiology (axonal
loss versus demyelination) and a symmetric versus
asymmetric or multifocal pattern of involvement.

 The limitations of EMG/NCS.
 There is no reliable means of studying proximal
sensory nerves.
 NCS results can be normal in patients with small-
fiber neuropathies
 Lower extremity sensory responses can be absent in
normal elderly patients.
 EMG/NCS are not substitutes for a good
clinical examination.

 The goal of treatment is to manage the
underlying condition causing the neuropathy
and repair damage, as well as provide
symptom relief.

 Medical management
 Analgesics .
 antiepileptic drugs, including gabapentin,
phenytoin, and carbamazepine
 some classes of antidepressants, including tricyclics
such as amitriptyline.
 Mexiletine
 local anesthetics such as lidocaine or topical patches
containing lidocaine
 Codeine/oxycodone

 Mechanical aids can help reduce pain and lessen the
impact of physical disability.
 Hand or foot braces can compensate for muscle weakness or
alleviate nerve compression.
 Orthopedic shoes can improve gait disturbances and help
prevent foot injuries in people with a loss of pain sensation.
 If breathing becomes severely impaired, mechanical
ventilation can provide essential life support.

 Surgical intervention often can provide
immediate relief from mononeuropathies
caused by compression or entrapment injuries.
 Repair of a slipped disk can reduce pressure on nerves
where they emerge from the spinal cord; the removal
of benign or malignant tumors can also alleviate
damaging pressure on nerves.
 Nerve entrapment often can be corrected by the
surgical release of ligaments or tendons.

Peripheral Neuropathies Classification and Management

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