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26 Cards in this Set
- Front
- Back
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List some signs and symptoms of multiple sclerosis.
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-initial presentation is weakness, numbness, tingling, limb unsteadiness, spastic hemiparesis, retrobulbar neuritis, diplopia, disequilibrium, sphincter disturbances- may go away after a few days or week
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List the four primary categories of multiple sclerosis.
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—Relapsing-remitting form (85% of cases) - progression does not occur between attacks
—Secondary progressive form (80% of cases after 25 years) - characterized by a gradually progressive course after an initial relapsing-remitting pattern —Primary progressive form (10% of cases) - in which there is gradual progression of disability from clinical onset —Progressive-relapsing form (rare) - with acute relapses being superimposed on a primary progressive course. |
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Describe the relapsing-remitting form of MS
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—Relapsing-remitting form (85% of cases) - progression does not occur between attacks
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Describe the secondary progressive form of MS.
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—(80% of cases after 25 years) - characterized by a gradually progressive course after an initial relapsing-remitting pattern
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Describe the primary progressive form of MS.
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—Primary progressive form (10% of cases) - in which there is gradual progression of disability from clinical onset
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Describe the progressive relapsing form of MS.
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- with acute relapses being superimposed on a primary progressive course.
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What is required before you can make a dianosis of MS.
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The diagnosis of multiple sclerosis requires evidence that at least two different regions of the central white matter have been affected at different times
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Describe some of the lab values you can expect in a MS patient.
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-mild lymphocytosis
-slightly increased protein in CSF (esp. soon after an acute relapse) -Elevated IgG in CSF and discrete bands of IgG (oligoclonal bands) are present in many patients (not specific to MS, though) |
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List 6 factors that predispose a pt to developing MS.
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a.)Greatest incidence in young adults (20-40 y/o)
b.)Women are 2x as likely to be affected than men c.)Genetic d.)The closer to the equator you live, the less chance you have of getting it e.)More common in persons of western European lineage f.)Optic neuritis |
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List various imaging techniques and possible findings used in the diagnosis of MS.
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MRI of brain or cervical cord to detect multiple lesion
-T1 weighted imageshypointense “black holes”=probably represent areas of permanent axonal - Gd enhanced T1-weighted images may highlight areas of inflammation with breakdown of the BBB -T2 weighted images provide info about number of lesions -CT scans are not as helpful as MRI -Used to rule out other diseases (such as Arnold-Chiari malformation)? |
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List 3 classes of drugs used in the treatment of MS.
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Interferons, corticosteroids (ONLY AFTER A RELAPSE- NOT FOR LONG TERM USE), immunosupressants
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Name 2 immunomodulator class drugs used in the treatment of MS.
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—Interferon Beta-1a (Avonex®) given intramuscularly once weekly
—Interferon Beta-1a (Rebif®) given subcutaneoulsy 3 times/week —Interferon Beta-1b (Betaseron®) given subcutaneously every other day —Glatiramer acetate (Copaxone®) given subcutaneously daily (a mixture of random polymers simulating the amino acid composition of myelin basic protein; blocks myelin T-cells by acting as a decoy) —Natalizumab (Tysabri®) – IV infusion once a month; for patients who have had an inadequate response to, or unable to tolerate other disease modifying medications |
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Name an immunosuppressant drug used in the treatment of MS.
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—Immunosuppressants
—Mitoxantrone (Novantrone®) ** chemotherapy**– suppresses T-cells, B-cells, and macrophages; IV infusion once every three months; tx of worsening relapsing-remitting forms |
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Name some side effects of interferons.
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The most common side effects of interferons are a flu-like syndrome and (in the case of interferon -1b) injection site reactions
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ADEM (acute disseminated encephalomyelitis) is characterized by ____________.
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-Characterized by a widespread demyelination that predominantly involves the white matter of the brain and spinal cord.
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Frequent neurological signs and symptoms of ADEM:
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-Unilateral or bilateral pyramidal signs (60-95%) – weakness, hyperreflexia, Babinski
-Acute hemiplegia (76%) -Ataxia (18-65%) -Cranial nerve palsies (22-45%) -Visual loss due to optic neuritis (7-23%) -Seizures (13-35%) -Spinal cord involvement (24%) -Impairment of speech (slow, slurred, aphasia) (5-21%) -Hemiparesthesia (2-3%) |
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Describe treatment for ADEM.
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No standard therapy for ADEM
-Most widely reported treatment is high dose steroids (solumedrol or dexamethasone) -IVIg (which has been used alone or with corticosteroids) anti-inflammatory treatment -Plasmapheresis is used in small number of cases when steroid treatment has failed -No reports of interferon-β or glatiramer acetate used in acute stages of ADEM -Some improvement with cyclophosphamide use in adult ADEM who responded poorly to steroids 50-80% full recovery |
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Define ADEM.
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Acute Disseminated Encephalomyelitis is an acute immune-mediated inflammatory disorder of the CNS; widespread demyelination involving the white matter of the brain and spinal cord
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Describe the population most affected by ADEM.
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Most common in pediatric patients (5 to 8 y/o) and slight male predominance
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Describe possible causes of ADEM.
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Most patient report an antecedent viral infection or vaccine during the prior few weeks (linked to specific vaccines such as Semple rabies vaccine, hepatitis B, pertussis, diphtheria, MMR, influenza, polio)
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Typical clinical course of ADEM.
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Usually a monophasic, rapid, progressive, self limiting course that develops over hours to 4-5 days; however recurrent forms making it difficult to distinguish from multiple sclerosis; seasonal more in winter and spring months
Approximately 70-80% of patients report a clinically evident antecedent infection or vaccination during the prior few weeks (2-21 days) Rapid onset encephalopathy associated with a combination of multifocal neurologic deficits Prodomal phase of fever, malaise, HA, nausea, and vomiting before meningeal signs and drowsiness Fever and HA occur more frequently in children, while sensory deficits predominate in adults Clinical course is rapidly progressive and usually develops over hours to maximum deficits within mean of 4-5 days |
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Describe the common presentation of transverse myelitis.
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rapidly evolving (several hours to days) symmetrical or asymmetrical, paraparesis or paraplegia, ascending paresthesia, loss of deep sensibility in feet, a sensory level on trunk, bilateral Babinski sign; pain in the head neck or back ; a bandlike tightness around the chest or abdomen, weakness, numbness, tingling of the feet and legs and difficulty voiding develops over hours; MRI shows focal demyelination
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Define acute transverse myelitis.
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An acutely evolving inflammatory-demyelinative lesion of the spinal cord, which proves in many but not all instances to be an expression of MS.
The myelitic lesion is analogous to that of optic neuritis. |
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Define Neuromyelitis optica (Devic’s Disease) and describe its common presentation.
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Simultaneous or successive and usually severe involvement of optic nerves and spinal cord.
Its principal features are the acute to subacute onset of blindness in one or both eyes, preceded or followed within days or weeks by a severe transverse myelitis. The spinal cord lesions in cases of neuromyelitis optica are often necrotizing rather than purely demyelinative in type, leading eventually to cavitation; the clinical effects are more likely to be permanent than those of demyelination. |
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Describe the common presentation of Acute inflammatory demyelinating polyradiculoneuropathy (ADP) Specifically Guillain-Barre syndrome
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acute polyneuropathy that can follow minor infective illness (Campylobacter jejuni); weakness that is symmetric usually begins in the legs and moves upward, sensory complaints and no deep tendon reflexes; tachycardia, labile blood pressure, sweating, impaired pulmonary function, sphincter disturbances, paralytic ileus
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List the essentials of diagnosis for MS.
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-Episodic neurologic symptoms
-Pt is usually under 55 yrs at onset -Single pathologic lesion cannot explain clinical findings -Multiple foci best visualized by MRI |
