Clinical Significance of Demyelinating Lesions of the CNS

Elaine S. Edmonds, MD. PhD University of New Mexico Department of Neurology MS Center

Underlying Factors of MS: Inflammatory Cascade
Inflammation Demyelination

Axonal loss

Demyelinating Lesions

Normal spinal cord

Spinal cord of MS patient

Demyelinating Lesions

• MS symptoms are highly variable
– Early in the disease symptoms resolve 80% of the time – Further episodes recur on average 0.5-1.2/year 0.5– Most patients present as relapsing-remitting but after several relapsingyears become slowly progressive

• The most common sites of initial lesions are optic nerve, cervical spinal cord and brainstem • Symptoms of new episodes last >24 hours • Most (80-90%) of lesions produce no obvious symptoms (80• Pseudoexacerbation- recurrent symptoms due to Pseudoexacerbationincreased body temperature (2° to exercise or infection) (2°
Normal brain Brain of MS patient


Initial Symptoms of Multiple Sclerosis
• • • • • • • Weakness in one or more limbs Optic Neuritis Paresthesias, sensory symptoms Diplopia (double vision) Vertigo Urinary symptoms Ataxia 35% 20% 20% 10% 5% 5% 3%

Eventual Symptoms of Multiple Sclerosis
• Weakness (spasticity) • Sensory symptoms • Visual symptoms Decreased vision or diplopia • Balance problems (ataxia) • Bladder symptoms • Lhermitte’s sign Lhermitte’ • Fatigue • Cognitive problems 80% 73% 50% 72% 62% 30% 48% 27%

Monosymptomatic > Polysymptomatic

• Upper motor neuron weakness with spasticity
– Lesion in pyramidal or extrapyramidal pathways from the cortex to the lower motor neuron in the spinal cord – Pattern can be paraparesis or paraplegia (mc), monoparesis or monoplegia, hemiparesis or monoplegia, hemiplegia

Associated Signs and Symptoms
• Signs
– Babinski’s sign (extensor plantar reflex) Babinski’ – Hyperreflexia – Increased tone

• Symptoms
– – – – – Stiffness Pain Weakness, worse if tired or overheated Extensor or flexor spasms Clonus

• Rarely, lower motor neuron weakness due to anterior horn involvement, associated with atrophy and decreased or absent reflexes

Location of Spinal Cord Lesions Producing Motor Symptoms

Corticospinal and Corticobulbar Tracts


Sensory Symptoms
• Numbness, tingling, paresthesias and dysesthesias are common • Usually occur with other symptoms • Can affect bowel, bladder or sexual function • Typically decreased rather than abolished sensation • Pain (decrease or increase), temperature, tactile, vibration and position sense can be affected • Can affect motor function=pseudoweakness function=pseudoweakness • Useless hand- due to loss of position sense hand• Lesion in posterior columns of spinal cord

Posterior Column- Medial ColumnLemniscal Pathway

Vibration, position sense, tactile sense

Spinothalamic Pathway

Spinal Cord Tracts

Ipsilateral loss Ipsilateral effect Contralateral loss

Pain and temperature

Lhermitte’s Sign
• Electric sensation passing from the neck down the back and legs (arms) on flexion of the neck, “like a waterfall”. waterfall” • Produced by stretching the posterior columns in the cervical spinal cord • Occurs with cord compression, radiation myelopathy and B12 deficiency or demyelination

Ataxia- Cerebellar Signs
• Rarely occurs as only symptom (if in isolation, cerebellar degeneration is more likely) • Action tremor (UE>LE) rather than resting tremor is present in MS patients, very disabling
– Plaques in the cerebellum or its connections in the brainstem

• Gait ataxia can be due to lesions in multiple sites: spinal cord, brainstem and/or cerebellum


Cerebellar Connections

Lesions in pons and cerebellum

Cerebellar Input

Cerebellar Output

Normal Brain

Brain of MS patient

MS Lesion Producing Action Tremor

Optic Neuritis
• Caused by plaque in optic nerve(s) nerve(s) • Accompanied by eye pain esp. 2° eye movement 2° • Decrease in VA (or blindness) increasing over 1-2 1weeks, generally improving in 8 weeks
– Recovery is usual although not always complete

• Usually (2/3) no optic disc abnormality initially
– Marcus-Gunn pupil=relative afferent pupillary defect Marcus-

• Subsequently optic disc pallor is present • Abnormal visual evoked potentials (not specific) • Associated with multiple sclerosis
– Risk of developing multiple sclerosis subsequently 40-50% 40Normal Brain Brain of MS patient

Optic Neuritis

Internuclear Ophthalmoplegia
• Bilateral INO is pathognomonic for MS
– Can rarely be caused by stroke – Due to a lesion in the medial longitudinal fasciculus
• Connection between the third and sixth nerve nuclei • Necessary for conjugate eye movements to the opposite side

• Includes lag or failure to adduct the eye and nystagmus of the abducting eye on the same side as the movement with preservation of convergence


Internuclear Ophthalmoplegia
• Bilateral INO is pathognmonic for MS
– Can rarely be caused by stroke – Due to a lesion in the medial longitudinal fasciculus
• Connection between the third and sixth nerve nuclei • Necessary for conjugate eye movements to the opposite side

Medial Longitudinal Fasciculus

• Includes failure to adduct the eye opposite to the movement and nystagmus of the abducting eye on the same side as the movement • Can be asymptomatic or cause positional diplopia or oscillopsia

INO Video

Transverse Myelitis
• Can be associated with various autoimmune disorders inc. multiple sclerosis • Ascending acute or subacute, sensory loss (level) subacute, and assymetric paraparesis + bladder symptoms • Partial or complete recovery usually in 8-24 8weeks • Increased risk (88%) of multiple sclerosis if CSF or brain MRI are abnormal, more likely to be a partial transverse myelitis (assymetric symptoms) (assymetric

INO Demo.wmv

INO Demo.wmv

Symptoms of Transverse Myelitis
Loss of proprioception, vibration, position sense, tactile (B- loss of bladder control and sensory level)

• Thank you for your attention

Weakness and spasticity (B- paraparesis, loss of bladder control) Loss of pain and temperature (B- sensory level)

Other symptoms: band-like constriction around trunk or extremities, back or radicular pain