Neuropathies
NSS Block- Year 3
2021-2122
Dr R Zaidan
PNS
• Motor neurons & theirs axons
• Sensory Neurons & theirs dendrites/axons
• Autonomic neurons: intra/extra
(ganglionar)
– Sympathetic
– Parasympathetic
Objectives
• Definitions,
• Peripheral NS, anatomy, histology, structues,
physiology
• Pathophysiology
• NP types: based on…
• Clinical expression
• Dg
• Investigations
• Major NP aetiologies & syndromes
? Neuropathies
• Dysfunction of peripheral nerves, due to an
aggression of the nerves fibers, leading to
symptoms and signs that are variable
depending on the cause, onset, severity and
duration of the aggression
Peripheral nerves
• Spinal nerves
– Somatic
– Visceral
• Cranial nerves
– Root
– The Cr. Nerve
Peripheral nerves
Roots (anterior & posterior)
↓
Spinal nerve
↓
Plexus
↓
nerves
The peripheral Nerves
Nerve fibers
Nerves Fibers
• Myelinated
(myelin sheet)
• Weakly
myelinated
• Unmyelinated
Classification of Neuropathies
based on involved nerves/roots
• Mono-neuropathy
• Neuritis multiplex
• Polyneuropathy
• Polyradiculo-neuropathy
The Neuropathies
• Mono-radiculopathy
• Multi-radiculopathy
• Plexo-pathy
THE ONSET
Of
The
Neuropathy
Onset-based Classification of NP
• “Sudden”
– Traumatic, dissection,
compression…
– Vascular (ischemia,
hemorrhage…)
• Acute
– Infection: diphteria
– Inflammatory (GBS)
– Toxics (Lead, Hg…), Drugs..
– Vascular (vasculitis)
Traumatic NP
Ischemic NP
GBS
(Pathophysiology)
Onset-based Classification of NP
• Sub-acute
– Inflammatory: autoimmune: (CIDP,
Paraneoplastic…)
– Metabolic, vitamins deficiency (B1, B12…)
– Toxics: alcohol, drugs (chemotherpies…)
– Vascular (?vasculopathies, ?vasculitis)
– Entrapments
• CTS
• TTS
• Ulnar
• Peroneal
Auto-Immune NP
Onset-based Classification of NP
• Chronic
– Hereditary
– Entrapment
– Toxic (heavy metals: led…)
– Metabolic: DM, CRF, hepatic failure…
Which fiber’s structure?
Is
?Affected
Structure-based Classification of NP
• Demyelinating
– Inflammatory (GBS, CIDP…)
– Hereditary…
• Axonal
– Toxic
– hereditary
– metabolic…
• Mixt:
– demyelinating + axonal
Inherited, metabolic…
Which fiber’s type?
Is primarily
Involved
Fibers-based Classification of NP
• Sensory (DM, paraneo….)
• Motor (MMNP…)
• Mixt: the most common (DM….)
• Autonomic (DM….)
• Any axonal damage may be
complicated with myelin
damage
• & Any myelin damage may
be complicated with axonal
damage
Mainly if:
• Prolonged exposure to the
agression
• Or severe and massive
lesion
• Acute damage: distal & proximal
• Chronic damage: distal >>> proximal
The end of the fibers is the most difficult part
of the axon to maintain in function & “repair”
Clinical Expression of NP: The
Symptoms
• Pain
• Burning
• Paresthesia: numbness, tinglings…
• Hyperesthesia
• Dysesthesia
• Electrical like discharge
• Weakness
• Neurovegetative symptoms: coldness, sweating…
Clinical Expression of NP: signs
• DTR: abolished / absent
• Sensory deficit: hypoesthesia / anesthesia
• Motor deficit: weakness mild🡪severe
• Autnomic
Length dependent damage…
Clinical Expression of NP: signs
• Sphincters dysfunction
– GBS
– DM autonomic NP…
• Autonomic:
– hypotension,
– Diarrhea, constipation, or both…
– Charcot joint (arthropathy)
• Sexual:
– decreased libido
– impotentia
Neuropathies: Diagnosis
• Clinical
– Hx: subjective findings
– Examination: subjective & objective…
• Investigations
– ?To confirm the diagnosis:
• Neurophysiology: NCS/EMG/SSR
• Pathology: nerve biopsy
– To discover the cause…
• Hematology, biochemistry…
Cranial neuropathies: “Isolated”
• Optic NP
• Bell’s palsy & other facial n. palsies
– “Idiopathic”
– 2dary to specific diseases
• Oculomotor
– (3d)palsy: partial, total…
– (6th)
”Cranial neuropathies: “Isolated
• Trigeminal NP: neuralgia, 2dary Vth NP…
• Facial N NP
• 8th n NP: deafness +/- vestibular syndrome…
• Others:
– hypoglossal NP,
– Glossopharyngeal NP
Cranial neuropathies: “multiple”
Multiple Cr NP
• Involved nerves:
– Local
– diffuse lesions
• Causes:
– Auto-immune
– Basal meningitis:
• Infectious: TB, bacterial…
• neoplastic…
Mono-neuropathies
• Entrapment syndromes:
– CTS
– Ulnar: wrist, elbow
– C. Peroneal
– TTS
– Meralgia paresthetica
• Other mono-neuropathies
– Traumatic
– Ischemic
– Compressive…
– Metabolic: meralgia in DM….
Neuritis Multiplex
• Multiple and asymmetrical nerves
involvement 🡪 asymmetrical, “patchy like”
symptoms and signs..
• Causes:
– DM
– Other metabolic: porphyria, CRF…
– Vasculitis & other aut-immune syndromes
– Paraneo…
Peripheral NP
• Symmetrical distal
– The most common
– Distal and slowly progressing sensory motor
symptoms & signs
– Chronic setting: mostly
– Severity depends of the cause
– DM & : distal& symmetrical: the most common cause
– Hereditary NP: deformity….
Ziegler 2008
Proximal NP
• LL NP:
– Proximal sensory and signs in the LL
– Amyotrophy is common in this form
– DM is the most common cause
Polyradiculopathies
1. Acute settings
• GBS
– Proximal and distal nerve acute demyelinating foci
– Most common
– 🡪 autoimmune process
– Axonal forms: more severe/devastating
• GBS like polyradiculopathies:
– Malignabcy
– Toxic (chemotherapies…)
Polyradiculopathies
2. Sub-acute/chronic
– Vitamin B12 deficierncy: subacute combined
sclerosis
– CIDP: Monophasic
Slowly progressive
Relapsing/remitting
DM NP
• Most Common
• All types:
– Mono NP: entrapment like…
– Neuritis multiplex
– Distal symmetrical
– Autonomic (small fibers)
– Amyotrophic form (proximal)
– Polyradiculopathy like syndrome…
– Cranial N, NP: Bell’s…, IIId,
Hereditary NP
• Wide ranges of presentations
• Usually symmetrical
• Chronic/insidious onset
• May be with disability…
• Friedreich ataxia
• CMT NP