MS/Pituitary Flashcards
1
Q
MS
A
- 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
2
Q
Uhthoff’s phenomena
A
-MS symptoms worsen in the heat
3
Q
Relapse
A
- new neurologic disability that lasts greater than 24 hrs
- may be recurrence of old symptom or a new one
- onset usually subacute
4
Q
Clinical definition of MS
A
-disease that is separated by time & space
5
Q
MS tests
A
- 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)
6
Q
Classical Diagnostic Criteria for MS
A
- 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
7
Q
MS Plaque
A
- 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
Q
Pathogenesis of MS
A
- 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
9
Q
MS Epidemiology
A
- 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
10
Q
Clinical Types of MS
A
- Relapsing
2. Progressive
11
Q
Relapsing MS
A
-exacerbations followed by complete or incomplete recovery
-slow, inconsistent accumulation of disability occurs in the majority of patients
~85% of patients
12
Q
Progressive MS
A
- 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
13
Q
Benign MS
A
- few relapses, never progress
- rare
14
Q
Secondary Progressive MS
A
-# of plaques increases within the CNS, patient worsen when relapses stop
15
Q
Primary Progressive MS
A
- 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)
16
Q
Natural History of ALS
A
- 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
17
Q
How is MS diagnosed?
A
- Clinical signs & symptoms
- MRI
- Spinal tap
- Evoked Potentials
18
Q
Symptoms of MS
A
- weakness
- numbness
- fatigue
- vision problems
- slurred speech
- poor coordination
- short-term memory loss
- depression
- bladder & bowel dysfunction
- partial or complete paralysis (severe)
19
Q
Optic Neuritis
A
demyelinating syndrome
- unilateral, retrobulbar, pain
- no retinal exudates, disc hemorrhage infrequent, some recovery
20
Q
Myelitis
A
demyelinating syndrome
- partial sensory > motor (assymetrical)
- band-like pressure
- Lhermitte’s sign
- Bowel & bladder sx are common
- Acute dystonias
21
Q
Brainstem demyelinating syndrome
A
- ocular motor (INO, nystagmus)
- trigeminal neuralgia, hemifacial spasm, Bell’s palsy
- vestibulopathy & other cranial neuropathies
22
Q
Cerebellum demyelinating syndrome
A
-acute ataxia, tremor, eye movement problems
23
Q
Afferent Pupillary defects
A
-consistent with a demyelination & axon damage on side of lesion
24
Q
Clinically Isolated Syndrome
A
-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
26
Attack
-relapse
27
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
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Neuromyelitis Optica (NMO)
-
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- 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
33
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)
43
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
