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

Generalizations
• 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

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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

Weakness
• 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

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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

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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

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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)

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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

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