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Flashcards in MS/Pituitary Deck (45):
1

MS

-chronic, inflammatory disease of CNS, demyelination disease (myelin, neurons, axons are destroyed)
-when myelin is lost in multiple areas, plaques occur
-results in periodic loss of neurological function (relapse) and often progressive disability

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Uhthoff's phenomena

-MS symptoms worsen in the heat

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Relapse

-new neurologic disability that lasts greater than 24 hrs
-may be recurrence of old symptom or a new one
-onset usually subacute

4

Clinical definition of MS

-disease that is separated by time & space

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MS tests

-spinal fluid (inflammatory profile): lymphocytes, IgG, oligoclonal bands (NEVER neutrophils in classic MS)
-MRI: plaques are hyperintense (can be enhanced with gadolinium)-inflammation & breakdown of BBB to let it enter
-Visual Evoked Response: P100, measures how quickly the cortex detects light input from retina (normal is 100msec)

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Classical Diagnostic Criteria for MS

-clinical evidence for lesions that reflect white matter dysfunction disseminated in time & space in age 18-50
-objective abnormalities on neurological exam
-at least 2 clear cut episodes of functionally sig. symptoms each lasting over 24 hrs separated by at least 1 month
-slow progressive deterioration of the same disseminated pattern evolving over at least 6 months

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MS Plaque

-damage to white matter which contains myelin
-many present in regions adjacent to cortical gray matter
-located in deep white matter and periventricular areas
-Juxtacortical
-demyelination (luxo fast blue stain stains normal myelin blue)
-inflammatory )Perivascular "cuff", T & B cells, macrophages & microglia
-damage of nerve cells & axons

8

Pathogenesis of MS

-cause uncertain
-immune-mediated inflammatory disease of central nervous system
-may develop in genetically susceptible individuals who are exposed to undefined environmental "triggers"
-leukocytes penetrate BBB, secrete inflammatory cytokines
-T-cells, B-cells, & macs orchestrate autoimmune attack against myelin antigens

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MS Epidemiology

-350,000 in US affected (1 million worldwide)
-diagnosed 20-40
-2/3 are women (RRMS)
-equal for PPMS
-incidence inc. with distance from equator
-8,500 to 10,000 new cases per year

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Clinical Types of MS

1. Relapsing
2. Progressive

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Relapsing MS

-exacerbations followed by complete or incomplete recovery
-slow, inconsistent accumulation of disability occurs in the majority of patients
~85% of patients

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Progressive MS

-steady progression of disability with few or no exacerbations
-develop spastic paraparesis over a period of years
-corticospinal dysfunction (spasticity, weakness), sensory disturbance & urinary symptoms

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Benign MS

-few relapses, never progress
-rare

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Secondary Progressive MS

-# of plaques increases within the CNS, patient worsen when relapses stop

15

Primary Progressive MS

-patients worsen without relapses
-usually lose ability to ambulate due to spastic paraparesis & other symptoms referable to CNS systems that affect gait (balance, sensory input)

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Natural History of ALS

-1 relapse per year (fewer over time)
-25% of patients never lose ability to perform daily activities
-15% become severely disabled within short time
-median time to reach moderate disability is 15 yrs, severe is 46 yrs
-mortality from MS as primary cause is low

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How is MS diagnosed?

-Clinical signs & symptoms
-MRI
-Spinal tap
-Evoked Potentials

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Symptoms of MS

-weakness
-numbness
-fatigue
-vision problems
-slurred speech
-poor coordination
-short-term memory loss
-depression
-bladder & bowel dysfunction
-partial or complete paralysis (severe)

19

Optic Neuritis

demyelinating syndrome
-unilateral, retrobulbar, pain
-no retinal exudates, disc hemorrhage infrequent, some recovery

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Myelitis

demyelinating syndrome
-partial sensory > motor (assymetrical)
-band-like pressure
-Lhermitte's sign
-Bowel & bladder sx are common
-Acute dystonias

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Brainstem demyelinating syndrome

-ocular motor (INO, nystagmus)
-trigeminal neuralgia, hemifacial spasm, Bell's palsy
-vestibulopathy & other cranial neuropathies

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Cerebellum demyelinating syndrome

-acute ataxia, tremor, eye movement problems

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Afferent Pupillary defects

-consistent with a demyelination & axon damage on side of lesion

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Clinically Isolated Syndrome

-a clinical episode suggestive of a MS patients first relapse

-optic nerves, brainstem, spinal cord (partial transverse myelitis)
-lasts at least 24 hrs
-typically ages 20-45
-no evidence of infection, fever or encephalopathy

25

Lesion

-plaque

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Attack

-relapse

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Treatments for MS

-Drugs for MS related symptoms
(steroids, baclofen, tizanidine, amantadine, modafinal, antidepressnats)
-Treat underlying cause: reduce risk of relapse, new plaque formation & neurological progression
(INF-beta, glatiramer acetate, mitoxantrone, natalizumab, teriflunomide, fingolimod)

28

Transverse Myelitis

-acute neurologic condition that reflects focal inflammation of spinal cord
-acute or subacutely developing motor, sensory & sphincter disturbance (usually asymmetrical)
-spinal segmental level of sensory disturbance with well defined upper limit
-no evidence of spinal cord compression
-absence of other neuro disease
-may be first sign of MS (30%)
-treat: IV steroids

29

Neuromyelitis Optica (NMO)
-

-severe thoracic pain, bouts of optic neuritis, acute paraparesis with urinary retention
-demyelination of grey & white matter
-infiltration of macrophages
-thickened, hyalinized blood vessels
-mech: ab mediated demyelination & axonal injury & necrosis

30

Internuclear Ophthalmoplegia (INO)

-damage to medial longitudinal fasciculus
-lesion is on the side of the adduction deficit

31

ADH

-kidney water retention

32

Oxytocin

-uterine contractions
-milk let down

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Prolactin

-milk production

34

FSH & LH

-estrogen, progesterone, testosterone production

35

ACTH

-corticosteroids

36

THS

-thyroid T3 & T4

37

GH

long bone growth

38

Adenamatous Growths of Pituitary

-benign neoplasma that present as endocrine hyperactivity disorders dependent upon what cell type & what hormone is over secreted
-size determines therapeutic approach
1. Microademomas (10mm) surgery
80% of pit tumors

39

Craniopharyngioma

-rare tumor derived from remnants of Rathke's pouch along a line from the nasopharynx to diencephalon
-located intrasellar or suprasellar
-1/2 present clinically during childhood or adolescence other after 2nd/3rd decade
-present with growth retardation in kids (interfere with GH), adults (visual defects) compress optic chiasm/nerve or diabetes insipidus

40

Meningioma

-benign tumors derived from meningeal membranes
-arise anywhere within calvaria and those that occur near sella cause visual impairment & hormonal deficiencies `

41

Pituicytoma

-rare, low-grade gliomas derived from the pituicytes of the posterior pituitary
-present as mass lesion with signs/symptoms similar to meningioma
-non-secreting tumors

42

Somatotroph Adenomas

-secrete GH (acromegaly)

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Corticotroph adenomas

-secrete ACTH (cushing disease)

44

Thyrothroh adenomas

-may secrete or not (TSH)
present as pit mass or hyperthyroidism

45

Gonadotroph adenomas

-usually nonsecreting but may cause infertility