Mcphs Mmd Ii Multiple Sclerosis And Als

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Idiopathic inflammatory autoimmune disorder of the CNS
Pathologically characterized by demyelination and axonal degeneration
Onset may be gradual or abrupt

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Multiple Sclerosis

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Accounts for approximately 80-85% of MS cases
Characterized by acute exacerbations of worsening neurologic function followed by remission interval; no apparent worsening or progression of the disease

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Relapsing Remitting MS (RRMS)

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Evolution of RRMS with worsening of neurologic dysfunction without recovery between periods

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Secondary Progressive (SPMS)

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Characterized by steady worsening of neurologic function from disease onset without distinct exacerbations or periods of remission
Usually associated with greater spinal cord involvement

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Primary Progressive MS (PPMS)

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Primary Progressive but with occasional exacerbations

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Progressive Relapsing MS (PRMS)

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What are the symptoms of MS?

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Visual Impairment
Painful monocular vision loss (Optic Neuritis)
Diplopia

Lhermitte’s Sign
Electrical sensation down the spine & into the limbs with neck flexion
Numbness, Paresthesias, Pain

Dizziness or vertigo

Fatigue: Physical exhaustion or mental/cognitive slowing

Heat Sensitivity

Urinary Bladder Dysfunction,

Constipation

Weakness

Behavioral/Cognitive Manifestations: Social Disinhibition, Dementia or Depression

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What are the signs of MS?

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Impaired visual acuity or red color perception with optic disc pallor

Afferent pupillary defect,
Disconjugate eye movements

Nystagmus

Trigeminal Neuralgia or bilateral facial weakness

Positive Babinski’s Sign

Spasticity

Hyperreflexia

Decreased strength

Decreased perception of pain, vibration or position

Action Tremor

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Diagnosis is based on two demyelinating lesions that occur in different parts of the CNS at least three months apart with no other apparent cause

Impulses are delayed due to demyelination

MRI is sensitive in detecting plaques

Plaques usually appear round or oval with finger-shaped projections (Dawson’s fingers) in the white matter of the brain, brainstem, optic nerves and spinal cord

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Multiple Sclerosis Diagnoses

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More than nine lesions
Lesion enhancement with contrast medium (gadolinium)
Location of lesions: periventricular, corpus callosum, juxtacortical
Involvement of brainstem, cerebellum or corpus callosum

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MS lesions seen in the Brain

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One or two vertical segments in length
Incomplete cross-sectional involvement
Less likely to enhance with contrast medium
No cord swelling
Better seen with short tau inversion recovery (STIR) MRI sequences

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Imaging of the spinal cord in MS

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What is involved with the CSF analysis of a patient with MS?

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CSF IgG concentration elevated in comparison to other CSF proteins in the majority of MS patients
Oligoclonal IgG bands are identified on CSF gel electrophoresis

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What is Evoked Potential Testing in MS?

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Used to detect clinically silent lesions of the visual, auditory and somatosensory pathways
Visual evoked potentials are the most sensitive

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Naturally occurring cytokines with immunomodulating and antiviral activities

Recommended as first line treatment for relapsing-remitting MS & for patients who can’t tolerate Glatiramer (Copaxone)
IM Interferon beta-1a (Avonex)
SC Interferon beta-1a (Rebif)
SC Interferon beta-1b (Betaseron, Extavia)

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Beta Interferons

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Mix of amino acids that mimics myelin protein
Recommended as first line treatment for relapsing-remitting MS & for patients who can’t tolerate beta interferons

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Glatiramer (Copaxone)

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Chemotherapeutic agent
Recommended for use in patients with worsening forms of MS or in patients that have failed immunomodulatory drug therapy as well as secondary progressive MS
Black Box Warning: Toxicity leading to Cardiomyopathy

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Mitoxantrone (Novantrone)

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MS Therapy

Monoclonal antibody
Block Box Warning: Potential for OIs
Generally recommended for patients who have inadequate response to other treatments
Associated with increase risk of Progressive Multifocal Leukoencephalopathy (PML)

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Natalizumab (Tysabri)

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Mainstay of treatment for symptomatic relapses of MS (acute exacerbations)
Shorten the duration of an acute relapse and speeds recovery
IV therapy with methylprednisolone for three to five days followed by PO taper of Prednisone

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Adrenal Corticosteroids

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This is characterized by progressive degeneration of both upper and lower motor neurons
Upper motor neuron degeneration results in weakness, hyperreflexia, positive Babinski Sign and clonus
Lower motor neuron degeneration results in weakness, muscle atrophy and fasciculations
Bulbar muscles may be affected

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ALS

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Lower motor neuron disease only
Clinical course usually indistinguishable from classic ALS

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Progressive Spinal Muscular Atrophy

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Upper motor neuron disease only
Survival typically longer than classic ALS

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Primary Lateral Sclerosis

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Disease limited to the bulbar muscles
Most patients evolve to classic ALS

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Primary Bulbar Palsy

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Pathology is marked by loss of motor neurons with astrocytic gliosis
Fibrosis astrocytes cause the affected anterior and lateral spinal cord columns to become hard
Peripheral nerves show secondary degeneration of myelin and axons

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Pathologic Features of ALS

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The classic presentation of this is progressive asymmetric muscle weakness and atrophy with fasciculations and hyperreflexia
Presentations may include tripping, stumbling, loss of manual dexterity (difficulty picking up small objects or buttoning clothes)
Estimated that 1/3 of motor neurons are destroyed before atrophy becomes apparent
Bulbar symptoms present initially in approximately 25% of cases
Presentations may include slurred speech, hoarseness, decreased volume of speech
Drooling may occur

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ALS

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The following show what kind of dysfunction in ALS?

Weakness
Spasticity
Hyperreflexia
Positive Babinski Sign
Clonus

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Upper Motor Neuron Dysfunction

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The following show what kind of dysfunction in ALS?

Weakness
Asymmetric Limb Weakness
Foot Drop
Muscle Atrophy
Fasciculations
Muscle Cramps
Breathing Difficulties

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Lower Motor Neuron Dysfunction

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In ALS this may present as difficulty swallowing, chewing, coughing, dysarthria, hyperactive gag reflex

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Bulbar involvement

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Each of the following must be present:
Lower motor neuron signs in two or more limbs
Upper motor neuron signs in at least one region (bulbar, cervical or lumbrosacral)
Progression of the disease by increasing symptomatic impairment per patient history

All of the following must be absent:
Sensory signs
Neurogenic sphincter abnormalities
Clinically evident CNS disease or peripheral nervous system disease

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El Escorial Diagnostic Criteria

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This is traditionally used to exclude other diagnoses in ALS?

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MRI

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This should be performed whenever bulbar disease and UMN signs are present

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Brain MRI

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These can be used to evaluate LMN signs in ALS?

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Cervical and lumbrosacral MRIs

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This drug has been shown to extend life expectancy by 2-3 months in ALS patients.

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Riluzole 50mg twice a day has been shown to extend life expectancy by 2-3 months

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This may be required for an ALS patient as the disease progresses?

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Mechanical Ventilation

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These two drugs can be used to treat spasticity in ALS?

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Baclofen, Diazepam

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This drug may help relieve fasciculations in ALS?

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Lorazepam

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These two drugs may help relieve drooling associated with ALS?

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Atropine, Scopolamine