Week 4 - Neuro Diseases / Disorders Flashcards
(243 cards)
1
Q
stroke
A
cerebral vascular accident (CVA), brain attack, sudden onset focal central nervous system deficit due to vascular causes
2
Q
transient symptoms with infarct
A
increased stroke risk in following weeks/months, symptom resolution but evidence of infarct on imaging
3
Q
transient ischemic attack (TIA)
A
transient neuological dysfunction due to focal ischemia in brain, spinal cord, or retina without acute infarction - no evidence on imaging
4
Q
stroke impact
A
leading cause of disability, often requires chronic care, Hispanic / American Indian / Alaska Native / African American more likely
5
Q
stroke etiology
A
atherosclerotic, occlusion of cerebral vessesl -> lack of O2 -> neuronal death (4 min after blood cessation)
6
Q
large vessel stroke - more likely over 45
A
direct thrombosis or embolism of cerebral arteries from cervical arteries / aorta / heart
7
Q
additional causes of large vessel stroke - more likely under 45
A
vasculitis, sickle cell crisis, preeclampsia, vertebral / carotid artery dissection, migraine vasospasm, sympathetic vasospasm (Rx, cocaine, amphetamines)
8
Q
thrombosis
A
blood clot
9
Q
embolism
A
intravascular mass from distant site
10
Q
hemorrhagic stroke
A
hemorrhage leaks into brain tissue
11
Q
ischemic stroke
A
slot stops blood supply to area of the brain
12
Q
large vessel ischemic stroke
A
anterior - common carotid -> middle or anterior cerebral arteries; posterior - vertebral artery -> basilar artery -> posterior cerebral artery
13
Q
small vessel ischemic stroke (lacunar)
A
on penetrating arteries off of cerebral arteries
14
Q
chronic hypertension and lacunar stroke
A
endothelial injury / tunica media smooth muscle degeneration -> plasma protein deposition -> collagenous fibers -> decreased vessel elasticity; endothelial injury -> platelet aggregation and activated clotting factor -> vessel occlusion
15
Q
intracerebral hemorrhagic stroke
A
13% of all strokes, most common hemorrhagic stroke, bleeding directly in brain tissue, risk - age / male / hypertension / alcohol / tobacco / diabetes
16
Q
subarachnoid hemorrhagic stroke
A
bleeding subarachnoid / subdural / epidural space
17
Q
hypertensive intracerebral hemorrhage (ICH)
A
deep structures of the brain / brainstem / cerebellum, less common on cortex
18
Q
cortex bleeding
A
associated with mass lesions, vascular malformation, amyloid angiopathy
19
Q
intracerebral hemorrhage in putamen
A
half of intracerebral bleeds from lenticulostriate branches of middle cerebral artery, symptoms - contralateral hemiparesis (weakness on one side of body), gaze paresis (weakness), aphasia (language disturbance), hemineglect (inability to pay attention to 1/2 visual field)
20
Q
intracerebral hemorrage in thalamus
A
contralateral hemianesthesia
21
Q
intracerebral hemorrage in cerebellum
A
vomitting, ataxia, nystagmus, facial paralysis, ipsilateral gaze palsy, decreased level of conciousness
22
Q
intracerebral hemorrage in pons
A
coma, quadripelgia, pinpoint pupils, autonomic instability
23
Q
intracerebral hemorrage imaging
A
noncontrast head CT shows large white area
24
Q
intracerebral hemorrage management
A
surgical removal of clots if supratentorial, stop anticoagulants, possible benefit if lower BP - no benefit steroids / mannitol / glycerol
25
subarachnoid hemorrhages (SAH)
intracraniral vessels between archoid and pia in the subarachnoid space with CSF, often traumatic shear force
26
non-traumatic subarachnoid hemorrhage
aneurysm rupture, 80% spont subarachnoid hemorrhages, 55, men, African Americans, 2-5% of all strokes
27
subarachnoid hemorrage symptoms
severe headache, thunderclap/worst ever, nausea, vomiting, nuchal rigidity, photophobia, altered consciousness, series of small headaches prior to rupture, CN VI or CN III palsy, increased cranial pressure, deficits based on location
28
anterior cerebral artery aneurysm example
bilateral leg weakness
29
middle cerebral artery aneurysm example
hemiparesis (weakness on 1/2 body) or aphasia (language problem)
30
subarachnoid hemorrhage imaging
noncontrast head CT (best within 6 hours when pt is still conscious) - shows lighter diffuse areas or blood pooled in ventricles, lumbar puncture showing blood (xanthochromia or RBC)
31
subarachnoid hemorrhage management
prevent rebleeding, prevent vasospasm, treat neuro complications, nimodipine - Ca channel blocker in CNS dilates small vessels to increase cerebral profusion
32
subarachnoid hemorrhage surgery
bleeding aneurysm, clipped or placement of endovascular coiling device (better outcomes)
33
subarachnoid hemorrhage risks
smoking, hypertension, alcohol, minorities, 1st degree family Hx - aneurysm monitoring, NO association with recurrent headaches or NSAID use
34
anterior circulation stroke
contralateral motor and sensory deficits
35
middle cerebral artery
supplies lateral frontal / parietal / temporal lobes
36
middle cerebral artery stroke
face and arms more affected, homonymous hemianopsia, ipsilateral gaze deviation
37
dominant cerebral hemisphere stroke
usually left side, aphasia (language problems)
38
nondominant cerebral hemisphere stroke
contralateral hemineglect
39
anterior cerebral artery stroke
legs affected, personality changes, rare
40
ophthalmic artery occlusion
amurosis fugax (blindness in one eye), like a shade being drawn
41
posterior circulation stroke (vertebrobasilar)
20% of ischemic strokes
42
vertebral artery stroke
inferior cerebellum and lateral medulla
43
cerebellar strokes (vertebral artery)
vertigo, blurred vision, vomiting, nystagmus, ataxia, postural instability
44
lateral medullary infarct (Wallenberg syndrome)
crossed symptoms, ipsilateral face from cranial nerve damage and contralateral body from ascending afferents that have already crossed over
45
basilar artery stroke
rostral brainstem, occipital lobes, CN palsies, gaze problems, hemianopsia, miosis, altered consciousness (damage to reticular activating system), possible loss of consciousness, 90% mortality
46
posterior cerebral artery stroke
contralateral homonymous hemianopsia, often with macular sparing, possible difficulty naming colors, if nondominant hemisphere - neglect of affected vision field, less obvious type of stroke
47
small vessel lacunar stroke
branches off middle cerebral artery to deep brain = lack of cortical stroke signs (no aphasia, neglects, visual field loss), 5 different stroke syndromes
48
pure motor lacunar stroke
most common lacunar stroke syndrome, infarction of posterior limb of internal capsule, hemiparesis (weakness on 1/2 body) of legs, arms, and face, disruption of descending corticospinal and corticobulbar tracts
49
pure sensory lacunar stroke
infarction of lateral thalamic nucleus, numbness of legs, arms, and face on contralateral side due to disruption of ascending spinothalamic and dorsal column medial lemniscus pathways
50
time and stroke management
critical to determine time last deficit free, pt with ischemic stroke and symptoms for less than 4.5 hrs are candidates for fibrinolytics
51
stroke and hospitalization
acute stroke yes, risk of complications, some transient ischemic attack patients may be outpatient
52
complications of stroke
hemodynamic or respiratory problems, worsening other conditions, psychosocial problems, aspiration, falling, intracranial hemorrhage, cardiac arrhythmia, myocardial infarct, stroke progression
53
stroke Hx
onset, head trauma?, relevant medical Hx and risk factors (atheroscloerosis, heart disease, meds, substances, migraines, seizures, infection, preganancy, trauma), candidate for fibrinolytics?
54
stroke PE
head/neck for signs of seizure/trauma (contusions or tongue laceration), neuro, heart, GI (occult blood if TPA candidate), stroke scale
55
stroke scale
level of consciousness observed / questions / commands, horizontal gaze, visual fields, facial palsy, motor arm, motor leg, limb ataxia, sensory, language, dysarthria, extinction / inattention (neglect)
56
hemorrhagic stroke imaging
noncontrast CT scan
57
acute / focal ischemic stroke imaging
hard to see early changes with noncontrast CT scan, MRI with diffusion weighted imaging good for acute / focal ischemic stroke
58
vertebrobasilar ischemic stroke imaging
magnetic resonance angiography (MRA) or computed tomography angiography (CTA), looks for vascular malformations / aneurysms that can be surgically corrected
59
stroke labs
looks for underlying causes and risk factors, CBC, BMP (basal metabolic panel), hepatic panel, lipid profile, INR (coagulation), EKG (arrhythmia - MI), ultrasound if anterior circulation stroke (came from common carotid with artery stenosis), echocardiogram (ventricular thrombi, valvuvar disease), younger patients (agitated saline echocardiogram - foramen ovale or septal defects)
60
stroke pts <45, screen for
substance abuse, pregnancy, thrombophilias, vasculitis, endocarditis, cervical artery dissection, migraines, hypertension, sickle cells disease, CNS infection, cerebral venous thrombosis, paradoxical embolism, premature atherosclerosis
61
stroke care
observed for neurologic decline, supportive care, maintain normal volume and blood glucose, PT or speech therapy, discharge planning
62
patent foramen ovale
10% of ischemic strokes, allows embolism from venous circulation into arterial circulation to the brain, tx antiplatelet therapy or anticoagulant or surgery
63
carotid stenosis
endarterectomy, greater benefit if greater stenosis, only for pts with >50% stenosis
64
stroke prevention
modifiable risk factors - lower hypertension (140/90), diabetics HA1c <35in waist in women), active 30 min day, antifibrinogen INR 2-3
65
stroke complications
depression, dementia, dysphagia, sexual dysfunction, incontinence, UTI, falls, blood clots, neurologic dysfunction
66
case - 61, diabetic, HTN, smile droops left, left arm very weak, left leg slightly weak, decreased left side face senation, gaze to the right, can not name things seen on the left - where is stroke???
right middle cerebral artery stroke
67
case - 54, African American, diabetic, HTN, atrial fibrillation - coumadin, high blood sugar, no abnormalities on head CT - next step???
lumbar puncture - looking for blood in CNS if there is a hemorrhagic stroke
68
case - 71, male, HTN management, irregular heart beat with left-side carotid bruit, 40% stenosis of carotid on ultrasound - next Tx???
coumadin blood thinner to reduce risk of stroke, greater benefit than aspirin
69
case - 68, male, episodic unilateral headaches, bumping into things on his right side, all CN and motor exam normal, right visual field deficit - where is stroke???
left posterior cerebral artery
70
case - 63, female, numbness in right arm, unable to speak, symptoms resolved - what test to ID problem???
carotid doppler ultrasound - transient ischemic attack was likely in the left middle cerebral artery coming from carotid stenosis
71
MS and MRI
white spots on brain from degradation of myelin creating water which creates H ions (that is what MRIs measure), axial brain with white dots, sagittal brain with white spots, sagittal spinal cord with white spots
72
MS and evoked potentials
apply stimulus in one place and measure action potential in another place, measures nerve conductance, MS shows weaker action potentials and longer latent period
73
CNS myelin
made by oligiodendroctyes processes wrapping axons
74
PNS myelin
schwann cells, entire cells wraps axons
75
myelin membrane differences from normal CNS cell membranes
myelin - 2 lipids : 1 protein (normal cell 1 lipid : 2 protein), more cholesterol, less phopsholipid (ethanolamine dominant instead of lecithin), 4x more galactolipid (cerebrosides, sulfatides), less phosphatidylinostitol that is used in cell signaling pathways
76
ceramide galactosyltransferase deficient mice (CgT)
form myelin with reduced thickness, cytoplasm retention, and disorganized myelin folding
77
ceramide synthase deficient mice (CerS)
form myelin with focal detachments of individual or groups of myelin lamellae
78
fatty acid 2-hydroxylase (Fa2 h) deficient mice
for adding OH group to fatty acid, make normal myelin, but develop myelin degeneration in old age
79
myelin lipid creation from fatty acids
glycerol backbone replaced with ethanolamine backbone and sugar and phosphate attached, also has ether bond (C-O-C) instead of ester bond (O=C)
80
CNS - proteins in myelin
myelin basic protein (MBP), proteolipid protein (PLP), combined = 60-80% of myelin proteins
81
PNS - proteins in myelin
myelin basic protein (MBP), protein 0 (P0), PMP-22 trace protein
82
myelin sheath structure
formed by apposition of external (intraperiodic line) and internal (major dense line) surfaces, cytoplasm squeezed out, MBP holds inner to inner together making dark ring, PLP / P0 with small carb span membrane and hold inner and outer together making light ring
83
external myelin membrane
cholesterol, galactocerbroside, s-galactocerbroside
84
internal myelin membrane
cholesterol, inostoldiphosphate, ethanolamine plasmalogen, P-serine
85
CNS demyelination diseases
myelin degrades, acquired, ex: MS, leukoencephalopathy, encephalomyelitis, central pontine myelolysis, anti-MAG disease
86
CNS dysmyelinating disease
molecular gene defect in myelin, inherited, ex: leukodystrophies, Krabbe disease
87
PNS acquired neuropathy
autoimmune, demyelinating, polyneuropathy, ex: Guillain-Barr Syndrome (acute)
88
PNS inherited neuropathy
dysmyelinating, ex: Charcot-Marie-Tooth disease, Krabbe disease,
89
Lorenzos Oil
movie about leukodystrophy accumulation of long chain fatty acids, Tx with oil with concentrated long chain fatty acids to suppress the synthesis of long chain fatty acids in the body
90
proteolipidprotein myelin disorders
4 transmembrane protein with two external side loops and one internal side loops, holds inner and outer surfaces together, mutations of protein cause disorders, ex: jimpy, rumpshaker, jimpy-msd - most mutations are on external loop
91
myelin basic protein disorders
large protein with 7 exons, if all exons expressed = large protein, if alternative RNA splicing = smaller protein, different sized MBP at different times in life and naturally mixed together, deletion / inversion of part of gene causes disorder
92
shiverer mutation in myelin basic protein
first animal study with effective gene therapy (gene rescue), transfected normal gene improves the amount of myelin
93
Charcot-Marie-Tooth Disease
PNS dysmyelination disease, high arches, claw foot, muscle atrophy in lower legs, make too much myelin, two copies of the proteolipidprotein gene (PMP22) on chromosome
94
experimental allergic encephalomyelitis
animal model for MS, injected with mutated myelin basic protein - animal model for autoimmune CNS demyelinating disorders
95
experimental allergic neuritis
animal model of Guillain-Barre Syndrome, model for autoimmune PNS demyelinating disorders
96
MS
autoimmune, lymphoctyes cross BBB with alpha4beta1 and VCAM1 adhesion molecules, lympocytes release cytokines that call in macrophages, macrophages destroy myelin
97
MS Tx - natalizumab (Tysabri)
molecular antibody against alpha4beta1 lymphocyte adhesion molecules to prevent them from crossing the BBB
98
demyelinating disorders
myeline degradation and sometimes loss of axons, ex: MS, acute disseminated encephalomyelitis (ADEM), acute necrotizing hemorrhagic encephalomyelitis, central pontine myelinosis
99
multiple sclerosis, MS
common 1/1000, more F, <50 onset, autoimmune CNS demyelination, relapsing and remitting course of neurologic deficits, white matter lesions, HLA-DR2 antigen presenting cell receptor most likely to get MS, linked to IL-2 and IL-7 receptors, depleted B cells = less likely to get MS, genetic and environmental, optic effects common
100
oligiocolonal bands
MS diagnostic, dark gamma area in gel, from various B cell clones making antibodies
101
MS plaques
lots, adjacent ventricles or just deep to gray matter, can be in spinal cord too, depressed and firm, active or inactive, white on MRI, dark red in fresh tissue, often follow blood vessels
102
active plaques
lipid stuffed macrophages, T cells cuffing vessels, axons present but not myelinated
103
inactive plaques
no myelin, decreased oligiodendrocytes, gliosis (astroctytes), decreased axons, empty
104
MS plaques microscopic
lighter (no myelin to stain), fewer cells, lymphoctye cuffed vessels, reactive astrocytosis
105
sharply demarcated active plaque around blood vessel
two forms - type I with Ig and complement, type II without Ig and complement
106
poorly demarcated active plaque not around blood vessels
two types - type III widespread oligodendrocyte apoptosis, type IV central oligodendrocyte apoptosis
107
shadow plaques
plaque border indistinct, thin myelin sheaths at edges, possible remyelination?, not an inactive plaque
108
type I and II, type III and VI plaques
found with eachother only, suggests more than one mechanism for MS
109
MS central symptoms
unilateral visual impairment with optic nerve involvement (often first presentation), cranial nerve signs, ataxia, nystagmus (brainstem involved)
110
MS spinal symptoms
motor / sensory impairment in trunk / limbs, spasticity, bladder control problems
111
acute disseminated encephalomyelitis
diffuse, monophasic, following viral infection, children, rapid onset, headache, lethargy, coma, fatal 10%, acute autoimmune reaction
112
acute necrotizing hemorrhagic encephalomyelitis
very severe and acute, young adults / children, follows upper resp infection, fatal or deficits in survivors, hyperacute variant of acute disseminated encephalomyelitis
113
central pontine myelinolysis
symmetric myelin loss in basis pontis and pontine tegmentum, caused by hyponatremia (low Na+) that is corrected too rapidly, rapid quadriplegia / quadraparesis, monophasic, alcoholics, organ transplants, malnutrition
114
human prion disease
first documented in 1957 in Fore tribe of Papua New Guinea were tribe practiced cannibalism of the dead, tribe quit eating dead but cases seen for decades due to long latent period
115
Kuru
Fore tribe prion disease, strange walk, slurred speech, facial ticks, uncontrolled fits of laughter, death in 6-12 months, more women and children, CBC normal, no cultures, brain autopsy with minimal inflammation, demonstrated human to human transmission with chimp experimentation, incubation of up to 40 years
116
prion (PrPsc)
survives boiling, freezing, formaldehyde, carboxylic acid, chloroform, desiccation, UV, nucleases - smaller than a virus - sensitive to proteases, misfolded PrPc protein with more beta sheets, converts (seeds) PrPc to PrPsc creating amyloid
117
amyloids
fibrous protein deposit, associated disease, binds congo red and thioflavin T dyes
118
human prion diseases
Creutzfeldt-Jakob disease, CJD variant, fatal familial insomnia, kuru
119
animal prion diseases
scrapie, mad cow, chronic wasting
120
Creutzfeldt-Jakob disease
60s, sporadic, few familial or transmitted, memory / behavior changes, rapid progressive dementia, startle myoclonus, fatal in ~7 months, spongiform brain change but no plaques, no Tx
121
variant Creutzfeldt-Jakob disease
young adults, behavior change / depression, slower progression than CJD, more cortical plaques, linked to mad cow disease (spongiform bovine encephalopathy), had eaten infected cow meat, can also be transmitted by blood transfusion, amyloid plaques and spongiform change, no Tx
122
familial fatal insomnia
sleep disturbances, ataxia, autonomic problems, coma, inherited mutation of PRNP gene, PrPc similar to CJD, no spongiform change, neuronal loss, death in 3 years, barbituate sleeping pills make worse, no Tx
123
Kuru
human prion disease, amyloid plaques and spongiform change, no Tx
124
familial CJD (fCJD)
autosomal dominant, no Tx
125
CJD labs
EEG - sharp wave complexes, MRI - intense signal in striatum / linear lesions and bilateral posterior thalamus, CSF - high proteins (also encephalitits, cerebral infarct), biochemistry - western blot of PrPsc, DNA sequencing - PRNP gene for fCJD, brain biopsy - spongy change and PrPsc by ELISA and immunochemistry
126
transmissible spongiform encephalopathies
cattle to humans causes vCJD, 10 year latent period
127
vCJD
20s, 13-14 months, behavior change, painful sensory, delayed neurologic, thalamus MRI sign, plagues, accumulated PrPres, in lymphoid tissue, increased glycoform ratia, genotype of methionine / methionine at codon 129 of PrPc gene
128
classic CJD
60s, 4-5 months, dementia, early neurological signs, sharp EEG waves, polymorphic genotype at codon 129 of PrPc gene
129
chronic wasting diseases
cervids - deer, elk, moose, middle America into Canada, prion found in urine / feces / saliva / ground water, experimental innoculation of squirrel monkey vs macaques suggests not transmissible to humans, careful butchering and deer tested by DNR
130
human amyloid formation diseases
alzheimer, parkinsons, type II diabetes, cataracts - not prion diseases; spongiform encephalitis - prion disease
131
chaperone proteins
chaperone proteins establish normal molecular structure and may be a good target to treat human prion diseases
132
Alzheimer compared to CJD
slower progression, no muscular effects, no spongiform change (but amyloid plaques like vCJD), tau protein marker (marker fof CJD too)
133
exotoxins
secreted from cell, high toxic (1ug fatal), neutralized with high-titer antibodies called antitoxin, heat liable, resistant to digestive enzymes, tx antibiotics / antitoxin / symptomatic
134
toxoid
inactive toxin used in vaccine, ex: tetinis shot
135
clostridium tetani
gram +, spore forming, rod, anaerobic
136
clostridium tetani
spores enter skin via trauma, germinate in anaerobic necrotic tissue, tetanospasmin exotoxin blocks release of glycine and GABA at inhibitory Renshaw cell interneuron causing spastic paralysis / trismus (lockjaw) / respiratory problems, Tx neutralize with human tetanus immune globulins, immunization booster, penicillin, muscle relaxants - can be transmitted by IV drug use
137
clostridium botulinum
gram +, spore forming, rod, anaerobic
138
clostridium botulinum
spores in water / soil, alkaline canned veggies, exotoxin released on death of cell, infants can get in honey, bilateral cranial nerve palsy with diplopia, dysphagia, muscle weakness, resp problems, floppy baby, flaccid paralysis, prevents release of ACh by blocking vesicle fusion SNARE proteins, Tx antitoxin, immunoglobin, penicillin, ventilator
139
clostridium botulinum (botox) uses
muscle spasms, chronic migraine, excessive sweating, crossed eyes, muscular dystrophy
140
corynebacterium diphtheriae
gram +, rod, nonmotile, no spores
141
corynebacterium diphtheriae
resp droplets to pharynx, possible meningitis, must be lysogenized by bacteriophage to release exotoxin that damages neurons and heart cells by interfering with protein synthesis, peripheral nerve palsies, Guillain Barre-like, palatal paralysis, cranial neuropathy, culture on potassium tellurite agar and Loeffler's coagulated blood serumn, Tx antitoxin, penicillin, DPT vaccine (exposure does not guarantee immunity)
142
corynebacterium diptheriae mechanism
shuts down translation in neurons and heart cells, B subunit binds cell membrane receptor and allow A subunit to enter cell, A subunit inhibits translation by binding elongation factor 2 (EF2)
143
Taenia solium - neurocysticerosis
parasitic worm, ingest larvae in uncooked pork, causes neurocysticerosis (swiss cheese brain)
144
Taenia solium - neurocysticerosis life cycle
pigs and human, scolex head allows attachment in intestine and eggs / proglottids in feces, cysterol in muscles / subcu / brain / eyes
145
heamatogenous spread to CNS
H. influ, N. meningitidis, Strep p, mycob tuberculosis, fungi, enteroviruses
146
other routes of spread to CNS
trauma, surgery, intraneural
147
intra-neural spread to CNS
rabies - along sensory nerves, herpes along trigeminal or sacral nerves, poliovirus, togavirus
148
infections that can be transferred from mother to child with CNS involvement
herpes (HS2), H. influ, chlamydia, strep B, CMV, N. gonorrhea (conjunctivitis), HIV, E. coli, toxoplasmosis
149
CNS disease that transfer from mother to fetus
group B strep, listeria monocytogenes, E. coli - causing acute bacterial sepsis or meningitis; N. gonorrhoeae - conjunctivitis; Chlamydia trachomatis - conjunctivitis; congenital syphillis - neurosyphillis
150
Treponema pallidum
neurosyphilis, subacute meningitis, Argyll - Robertson pupil (does not react to light, does react to convergence), meningovascular syphilis, tabes dorsalis, paresis
151
tabes dorsalis
untreated traponema pallidum, syphillis, damage to posterior column and dorsal roots, ataxia, loss of reflexes, loss of pain and temp sensation
152
general paresis
untreated treponema pallidum, syphillis, mental deterioration, psychiatric symptoms
153
congenital syphillus
untreated treponema pallidum, crosses placenta, high mortality, CN VIII deafness
154
infections that can cross placenta (TORCHES)
T - toxoplasmosis, O - other (HIV), R - rubella / chorioretinitis, C - cytomegalovirus, H - herpes simplex
155
herpes labialis - HSV1
keratoconjunctivitis - dendritic ulcers, temporal lobe encephalitis, via trigeminal ganglia, Tx acyclovir and topical trifluridine
156
herpes genitalis - HSV2
aseptic meningitis, myelitis, neonatal meningitis, cervical carcinogenesis, via sacral ganglia, Tx acyclovir
157
varicella zoster virus - chicken pox, shingles
meningoencephalitis, keratitis, via trigeminal nerve or dorsal root ganglia, Tx acyclovir and varicella vaccine
158
ebstein bar virus - infectious mononucleosis
meningoencephalotiti, via B lymphocytes, Tx self limiting
159
cycolomegalovirus
encephalitis, chorioretinitis, Alzheimer, via mononuclear cells, Tx ganciclovir
160
cerebral palsy
is a type of static encephalopathy, traumatic brain injury involving blood flow and O2, perinatal, may not show right away after birth, milder will take longer to show up, premature babies may have for no apparent reason, shows by 2 years
161
premature cerebral palsy
blood vessel aren't pruned yet, periventricular injury, frontal lobe fibers to legs affected (spastic biplegia), arms fine
162
term cerebral palsy
injury where cerebral arteries are after blood vessels have been pruned, arms will be more affected than legs
163
autism
improves for most, personality profile, primary - seen alone, syndrome - seen with other problems, spectrum disorder, other delays common
164
neurologic syndromes
if diagnosed are useful, give family genetic counseling and prognosis infromation
165
leukodystrophy
don't want to miss, white matter injury that is diffuse, progresses, gets worse, loss of motor ability, autosomal recessive, tx bone marrow transplant, looks like cerebral palsy but later onset, ex: child learns to walk and then loses ability to walk
166
congenital stroke
may not show until 6-12 months due to dominant extra pyramidal system that is bilateral
167
muscular dystrophy
normal until 2-4 years old, gradual decline
168
epilepsy
many causes, infantile spasms, don't want to miss, can be generalized, focal, secondarily generalized, EEG determines Tx, should have loss of consciousness
169
generalized epilepsy
spike in one area of EEG
170
focal epilepsy
spikes on many parts of EEG
171
benign epilepsy disorder
seizures on face, normal EEG, outgrow most of the time
172
non-epileptic seizures
look like seizures but are not, no loss of consciousness, many with psychological causes, ex: syncope (fainting), panic, hypertventilation, gastroesphageal reflux / Sandifer's syndrome, tic disorders (can control)
173
Sandifer's syndrome
gastroesophageal reflux, stiffness, turn blue, hold breath, not a seizure
174
impulse control disorders
ADHD, OCD, Tourette, tic - can be seen in same pt or in same family
175
static encephalopathy
single incident of injury to the brain, does not progress, ex: infection or cerebral palsy
176
central pontine myelinolysis
acute demyelination in basilar pons, interrupts corticobulbar and corticospinal tracts, preserves sensory input, rapid quadriplegia, too rapid correction of hyponatremia (should be 1 mEq/L/hr)
177
diabetic neuropathy / vit B12 deficiency
decreased ankle reflexes and leg sensation
178
ataxic gate
alcohol intoxication or cerebellar vermis atrophy
179
multiple sclerosis
demyelination in CNS, symptoms separated in time and space in white matter, episodes last longer than 24 hours, autoimmune attack of oligodendrocytes, periventricular plaques on MRI, optic neuritis in one eye common
180
bilateral lesion of pyramidal tract at pyramidal decussation
fine hand control deficits, loss of thumb / index finger opposition and digit extension leads to cupping of hand, reaching / locomotion / larger movement intact, shows in finer motor control because there are other intact systems contributing to gross motor control
181
upper motor neuron syndrome
lesion of primary motor cortex, all contralateral effects that include weakness in one side (paresis), increased extensor tone, increased stretch reflex, positive Babinski sign, fine motor deficits (hand/foot/mouth/tongue), corticobulbar signs of affected cranial nerve nuclei
182
corticobulbar signs of primary motor cortex lesion - lower quadrant facial muscles
contralateral side, lateral part of CN VII nuclei, loss of smiling, baring of teeth, and puffing of cheeks - can put eyerbrows up and wrinkle forehead because that is ipsilateral and contralateral CN VII innervation
183
corticobulbar signs of primary motor cortex - accessory nucleus lesion
weakness of ipsilateral trapezius and sternocleidomastoid (turns head toward opposite side of body)
184
corticobulbar signs of primary motor cortex - hypoglossal nucleus lesion
weakness of contralateral tongue, tongue protrudes toward weak side
185
stroke in primary motor cortex
paresis, recovered proximal joint function, no extension of digits, buttoning and using fork hard, clumsy fingers, didn't know next move, mental effort to do things
186
lesion of premotor cortex
can't execute complex motor plans that require visuomotor transformation (ex: reaching around a clear barrier to grasp food), can't steer arm correctly, can't learn new sensory-motor associations (ex: stopping at a stop sign when driving)
187
lesion of supplementary motor area
deficits in internally driven and sequenced movements, pt is stimulus bound and will react to a sensory stimulus no matter where they are (ex: urinal in public place), alien hand syndrome (sense that someone else is moving their hand), trouble with learned sequences leading to semi-purposeful movements outside pt control
188
vestibulocerebellum disorder
equilibrium/balance disturbed (fall toward side of lesion), nystagmus, loss of smooth pursuit (can't follow moving object smoothly, follow with saccadic eye movement), wide based gate to add stability --- drunk, cerebellum is sensitive to alcohol
189
lesion of fastigial nucleus
affects vestibulocerebellum, loss of equilibrium/balance, nystagmus, loss of smooth eye pursuit (replaced by saccadic), wide based gate
190
lesion of spinocerebellum
disorders ipsilateral to side of lesions, ataxic gate if vermis involved, hypotonia (not weak), action/target/intention tremor, limb dysmetria, timing off, movement decomposition into individual joint movements
191
lesions of emboliform and globose nuclei
disorders ipsilateral to side of lesions, ataxic gate if vermis involved, hypotonia (not weak), action/target/intention tremor, limb dysmetria, timing off, movement decomposition into individual joint movements
192
cerebrocerebellum lesions
interacts with contralateral side of cortex, effects ipsilateral side of body, ataxia of finest movements (hand shape, writing), possible cognitive effects
193
lesion of dentate nucleus
interacts with contralateral side of cortex, effects ipsilateral side of body, ataxia of finest movements (hand shape, writing), possible cognitive effects
194
hypotonia
= cerebellar problem, not weakness, ex: limb can flex completely without resistance but with arm held up hand will drop
195
hypertonia
not cerebellum, some other problem, likely lack of inhibtion to thalamus
196
case - left side paresis / paralysis in leg
likely a stroke in the right anterior cerebral artery, causes left sided paresis / paralysis and leg part of motor homunculus is in the interhemispheric fissure which is suppied by the anterior cerebral artery
197
upper motor neuron lesion
CNS, hyperreflexia, weakness, paralysis, hypertonia, spasticity, no abdominal reflex, positive Babinski, muscle fasciculations
198
lower motor neuron lesion
spinal/peripheral, hyporeflexia, muscle atrophy, hypotonia, weakness, paralysis, normal abdominal and plantar (negative Babinski) reflexes
199
lesion of subthalamic nucleus
commonly stroke, hyperkinetic dyskinesia, ballismus (wild exaggerated limb movement on contralateral side), due to loss of subthalamic excitation of globus pallidus internus which inhibits thalamus
200
Parkinson
loss of cells in substantia nigra compacta in dopamine loop leading to decreased dopamine levels, direct pathway no longer inhibits globus pallidus internus and indirect pathway stimulates globus pallidus internus to inhibit thalamus, decreased thalamic activity = decreased movement
201
Parkinson clinical findings
bradykinesia / akinesia, rigidity, resting tremor from over inhibited oscillating signal of thalamus, postural instability
202
causes of parkinson
possible oxidative stress, encephalitis lethargica (viral encephalitis epidemic after WWI - destroyed substantia nigra compacta), toxins (CO, Mn), head trauma (bowers)
203
Parkinson Tx
restore dopamine levels with L-DOPA, can produce hyperkinetic movements (shakes)
204
globus pallidus lesion
removes tonic globus pallidus internus inhibition of thalamus, flexion dystonia/posturing, dytonia/rigidity from increased motor activity
205
putamen lesion
motoric, hyperactive, stereotypic, part of movement repeated
206
caudate lesion
complex changes, cognitive disorders with hyperactivity, vulgarity, implusiveness, increased appetite, polydipsia, hypersexuality
207
huntington chorea (dance)
autosomal dominant, short arm on chromosome 4, CGA gain of function repeat, 25-40 yr onset, death of GABA/ENK spiny neurons in neostriatum, loss of cerebral cortex neurons, striatum dies, effects indirect pathway - allows GPe to inhibit STh from stimulating GPi which would inhibit thalamus, thalamus increases activity, involuntary chorea or athetosis, dementia, cognitive decline, akinesia
208
involuntary movements
indirect pathway is affected, GPi is not inhibiting thalamus
209
akinesia
direct pathway effected, GPi no longer inhibited allow for tonic activation of thalamus
210
case - 26, white male, involuntary movement of limbs, body, trunk, progressive, loss of cognitive function, father had disease
huntington
211
case - 30, white male, MVA, head injuries, comatose, increased extensor tone in arms / legs
decerebrate rigidity, emergency, must relieve pressure on brain
212
huntington pathogenesis
loss of basal ganglia neurons (especially spiny striatal neurons) leads to increased motor output, neuron loss in cortex leads to cognitive changes
213
gross features of huntington
small brain, atrophy of striatum (especially caudate), atrophy of frontal lobe, dilated lateral / 3rd ventricles due to fill space left by atrophy (hyrdocephalus ex vacuo)
214
microscopic features of huntington
neuronal loss and astrocytosis (gliosis), ubiquitin tagged Htt protein forms aggregates (should be eaten by proteasome), aggregates contain protein with 40+ glutamines that make thin, threadlike aggregates
215
lacunar infarct
involves penetrating branches of middle cerebral artery called lateral striate arteries, supply caudate, putamen, globus pallidus, and internal capsule, causes small cavitary lesions around arteries in lateral striate, common with long standing hypertension, common in basal ganglia and thalamus
216
lesion of primary motor cortex in interhemispheric fissure on both sides
paraplegia, paralysis of both legs
217
Urbach-Wiethe disease
bilateral calcification of amygdala, trouble recognizing fear in facial expression, trouble distinguishing between similar emotions, memory loss of emotional content (need amygdala to pair neutral stimulus and harmful stimulus)
218
post traumatic stress disorder
re-experience, avoidance, hyperarousal, prefrontal cortex activity decreased, amygdala activity increased, prefrontal cortex normally inhibits anygdala
219
schizophrenia
fragmentation of mood / motivation / movement, positive delusions / hallucination, negative social withdrawal / loss of executive function, Tx on effect positive symptoms, 1% pop, dopamine and glutamate hypotheses of causation
220
dopamine hypothesis - schizophrenia
increased DA receptor activity, ex: amphetamines cause schizophrenic-like symptoms by increasing DA release and blocking DA reuptake, target for typical and atypical anti-psychotics
221
glutamate hypothesis - schizophrenia
phencyclidine (PCP) causes schizophrenia like symptoms by blocking NMDA glutamate receptors, possible tx by increasing glutamate
222
depression
fatique, anhadonia, social withdrawal, 20% women, 13% men, ~17% pop, monoamine hypothesis - decreased norepinephrine / serotonin activity, tx monoamine oxidase inhibitors, tricyclics, specific serotonin reuptake inhibitors
223
Korsakoff's syndrome
no new memories, disoriented in space and time, confabulation in response to questions, chronic alcoholism with thiamine (vit B1) deficiency, affects mammillary bodies and mamo-thalamic tract
224
Kluver-Bucy syndrome
bilateral medial temporal lobe damage, same damage from herpes encephalitis, oral tendencies, loss of emotion (neutral affect) due to amygdala dysfunction, hypersexuality due to hypothalamus dysfunction, visual agnosia (loss of visual cortex input)
225
Alzheimer
progressive, loss of memory, mood disorder (anxiety/depression), motor dysfunction, loss of cognitive function, 50% of pop > 80, loss of cholinergic input from nucleus basalis to hippocampus, loss of neurons, neurofibrillary tangles (phosphorylated tau proteins) and beta-amyloid plaques, huge sulci / ventricles due to brain matter loss, tx donepezil (aricept)
226
chronic traumatic encephalopathy
neurodegenerative, repeated head trauma, loss of memory / executive function, depression / apathy, impulsiveness / aggressiveness, 30-50, APO e4 allele increases, contact sports, atrophy of frontal / temporal lobes, neurofibrillary tangles with phosphorylated tau proteins in hippocampus and amygdala
227
Weber syndrome
superior alternating hemiplegia, ipsilateral oculomotor nerve dysfunction on one side of face and motor dysfunction on contralateral side of body
228
medial pontine syndrome
middle alternating hemiplegia, ipsilateral abducens dysfunction (LR), motor and touch sensation dysfunction on contralateral side of body
229
lateral pontine syndrome
CN VII / VIII dysfunction, loss of pain and temp sensation on contralateral body, loss of trunk and limb muscle control on contralateral side of body, loss of face pain and temp sensation on ipsilateral side of body
230
medial medullary syndrome (inferior)
hypoglossal alternating hemiplegia, hypoglossal dysfunction on ipsilateral side (tongue deviates toward bad side), loss of motor on contralateral side of body, loss of touch sensation on contralateral side of body
231
Wallenberg syndrome (PICA syndrome)
lateral medullary syndrome (superior), CN IX and CN X dysfunction on ipsilateral face, loss of pain and temp on contralateral body, loss of trunk and limb coordination on contralateral body, loss of pain and temp sensation on ipsilateral face
232
Parkinson
60, unilateral resting tremor, small hand writing, worse when anxious, anosmia, monotone voice, masked face, slowed rapid hand movements, arm does not move when walking, hard to initiate walk, shuffle bent forward walk
233
Mn toxicity parkinsonism
all symptoms similar to parkinson but does not respond to tx as well - common with welders, more general symptoms, heavy metals do not go away
234
parkinson pathology report
decreased pigmentation in substantia nigra and locus ceruleus, decreased neurons in substantia nigra, Lewy bodies
235
deep brain stimulation
used on globus palludis internus in parkinson to reduce inhibition of thamalus
236
diabetes insipidus
trauma / pituitary stalk section/autoimmune/idiopathic, increased urination (polyuria), increased thirst (polydipsia), due to lack of vasopressin from posterior pituitary, tx desaminovasopressin only acts on kidneys - not smooth muscle
237
galactorrhea-amenorrhea
hyperprolactinemia and amenorrhea (lactation and no period), prolactin level high, lutenizing and follicle stimulating hormones low, pituitary microadenoma, tx surgery or dopamine to inhibit prolactin secretion
238
Prader-Willi syndrome
deletion of chrom 15, fetal hypotonia, mild mental retardation, hypogonadotropic hypogonadism (low LH, low FSH, low gonadal function), obesity due to hyperplegia from excessive ghrelin secretion
239
basilar stroke
rostral brainstem and occipital lobe affected, CN palsy - gaze problems, hemianopsoa, miosis, damage to reticular activating system causes altered / loss of consciousness; only type of stroke with loss of consciousness
240
loss of consiousness seizures
must be reported to the DMV or dept of public safety
241
MS
can be minicked in animals by injecting bacterial toxins
242
Mn poisoning
can cause hallucinations
243
destruction of substantia nigra compacta
decreases firing in ventral lateral thalamus
