PERIPHERAL NEUROPATHIES Flashcards
What is peripheral neuropathy?
A broad term that refers to any disorder of the peripheral nervous system
Types of peripheral neuropathies?
Polyneuropathy
Radiculopathy
Plexopathy
Mononeuropathy
Mononeuritis multiplex
What is polyneuropathy?
Generalised damage to multiple peripheral nerves
Distal nerves are usually affected most prominently
Often symmetrical e.g. glove and stocking
What is radiculopathy?
Symptoms related to the involvement of a specific spinal nerve root
What is a mononeuropathy?
Damage to a single peripheral nerve
What is mononeuritis multiplex?
Simultaneous involvement of at least 2 separate nerves
Causes of peripheral neuropathy?
Diabetes
Alcoholism
Vitamin B12 deficiency
Guillain-Barré syndrome
Porphyria
Lead poisoning
Charcot-Marie-tooth syndrome
Chronic inflammatory demyelinating polyneuropathy
Diphtheria
Amyloidosis
Uraemia
Leprosy
What are the 3 major mechanisms of peripheral neuropathy?
Axonal degeneration - damage to nerve axons. Usually causes symmetrical polyneuropathy with weakness
Wallerian degeneration - damage to nerve axon due to lesion or physical compression. Commonly seen in mononeuropathies with the portion of the nerve dista to compression affected e.g. carpal tunnel syndrome
Demyelination - degeneration of myelin sheath
Most common cause of polyneuropathy?
Diabetes mellitus - causes “glove and stocking” distribution
Causes of acute polyneuropathies?
Guillain-Barré syndrome
Vasculitis
Toxins
Critical illness
What typically causes chronic polyneuropathies?
Diabetes mellitus
CKS - caused by uraemia. Very common
Herediatary neuropathies
Alcohol excess
HIV/AIDs
Neoplasia - particuarly myeloma - caused by infiltration or paraneoplastic
Lupus
Iatrogenic - isoniazid and phenytoin
What classically causes demyelinating polyneuropathies?
Guillain barre syndrome (a post-infective demyelinating peripheral neuropathy)
Chronic inflammatory demyelinating polyneuropathy
Charcot-Marie tooth disease
What classically causes axonal degeneration polyneuropathies?
Diabetic neuropathy
Alcoholic neuropathy
Toxic neuropathies e.g. lead
Examples of motor polyneuropathies i..e damage to motor nerves
Chronic demyelinating inflammatory polyneuropathy
Guillain-Barré syndrome
Charcot-Marie-tooth syndrome
Examples of sensory polyneuropathies i..e damage to sensory nerves
Diabetes mellitus
Alcohol excess
CKD
Paraneoplastic syndromes
How does diabetes polyneuropathy typically present?
Distal, symmetrical, polyneuropathy with predominant sensory loss
As it progresses, motor function may also be lost
Pathophysiology of nerve damage in diabetes?
Perisstent hyperglycaemia leads to oxidative stress which causes nerve damage
Damage to vasa nervorum also leads to nerve damage
What is the most common hereditary peripheral neuropathy?
Charcot-Marie-tooth disease
What is Charcot-Marie-tooth disease?
A collection of peripheral neuropathies due to an inherited mutation that results in predominantly motor loss
No cure
Onset 5-15
Features of Charcot-Marie-tooth disease?
Predominantly motor peripheral neuropathy!!
Muscle weakness in feet, ankles and legs
There may be a history of frequently falling or sprained ankles
Foot drop + Pes cavus+ Hammer toes + high stepping gait
Peripheral sensory loss
Distal muscle atrophy “inverted champagne bottle legs”
Hyporeflexia
Hypotonia
Fatigue
What is Guillain-Barré syndrome?
An immune-mediated demyelination of the PNS often triggered by an infection
Causes progressive, ascending neuropathy
Rare
How does Guillain-Barré syndrome typically present?
Often 2-4 weeks post-infection
Initial symptoms: back/leg pain in the initial stages
Others:
Rapidly progressive, symmetrical, ascending limb weakness
Paraesthesia in lower limbs and hands - mild
Issues with balance and coordination
Reflexes reduced or absent
Can affect chest causing respiratory issues
Can be cranial nerve involvement - diplopia, facial nerve palsy, oropharyngeal weakness
Autonomic involvement can occur - urinary retention and diarrhoea
What most commonly triggers Guillain-Barré syndrome?
Campylobacter jejuni
Investigtaions for Guillain-Barré syndrome?
Nerve conduction studies - will show decreased motor nerve conduction velocity due to demyelination, prolonged distal motor latency, increased F wave latency
Lumbar puncture - will show rise in protein with normal WBC
Why can Guillain-Barré syndrome be life threatening?
It can cause autonomic instability and hypoventilation due to respiratory muscle weakness
What is chronic inflammatory demyelinating polyneuropathy?
An acquired immune-mediated cause of polyneuropathy - demyelination of PNS
Presentation of chronic inflammatory demyelinating polyneuropathy?
Symmetrical sensorimotor polyneuropathy (motor symptoms predominate) in proximal and distal muscle groups
Can be slow progressive, acute or cause radiculopathies
How does vitamin B12 deficiency induced polyneuropathy present?
Distal, symmetrical sensory polyneuropathy
What can happen in severe cases of vitamin B12 deficiency?
Subacute combined degeneration of the spinal cord
What is Subacute combined degeneration of the spinal cord?
Impairment of the dorsal columns, lateral corticospinal tracts and spinocerebellar tracts due to vitamin B12 deficiency
Features of Subacute combined degeneration of the spinal cord?
dorsal column involvement:
distal tingling/burning/sensory loss is symmetrical and tends to affect the legs more than the arms
impaired proprioception and vibration sense
lateral corticospinal tract involvement:
muscle weakness, hyperreflexia, and spasticity
upper motor neuron signs typically develop in the legs first
brisk knee reflexes
absent ankle jerks
extensor plantars
spinocerebellar tract involvement:
sensory ataxia → gait abnormalities
positive Romberg’s sign
Sensory features of polyneuropathy?
Burning sensation
Paraesthesia
Sensory loss (touch, pain, temperature)
Ataxia
Loss of light touch
Loss of vibration
Loss of proprioception
Motor features of polyneuropathy?
Weakness
Reduced/absent reflexes
Hypotonia
Fasciculations
Muscle atrophy
Muscle cramping
Deformity (e.g. pes cavus, clawing)
What is used to confirm peripheral neuropathy?
Electrodiagnostic tests (electromyography and nerve conduction studies)
Management of polyneuropathy?
Treat underlying cause e.g. avoid alcohol, vitamin B12 replacement, BG control in DM
Neuropathic pain medications
Other Tx but specialist
What can cause an acute, rapidly progressive polyneuropathy with predominant weakness?
Guillain-Barré syndrome
Most common type of radiculopathy?
Lumbosacral radiculopathy L1-S3
Types of radiculopathy?
Cervical C1-C8
Thoracic T1-T12
Lumbosacral L1-S3
What is a dermatome and a myotome?
Myotome: a collection of muscles innervated by a single spinal nerve root
Dermatome: an area of skin supplied by a single spinal nerve root
Myotome for shoulder elevation?
C4