Neurology Flashcards
1
Q
成人中CNS的肿瘤按常见程度排序？良、恶性？ Leading CNS/PNS tumors in children? spinal cord?
A
》50%是转移（lung, breast, kidney, skin melanoma)，多个病灶 In adults: 如果是CNS原发,多为solitary tumor. 按常见程度排序 1. GBM (glioblastoma multiforme): 高度恶性，locate within the hemisphere. soft and poorly defined, necrosis + hemorrhage 2. memingiomas: 良性，locate on the brain surface (attached to dura) 3. acoustic neuroma (Schwannoma): arise from CN VIII, locate at cerebellopontine angle In children: 1. astrocytoma; 2. medulloblastoma; 3. ependymoma 室管膜瘤 In spinal cord: 室管膜瘤
2
Q
CNS/PNS origin: neuroectoderm: give rise to ? Neural crest cells? microglia起源？
A
neuroectoderm: give rise to 1） CNS neurons, 2) ependymal cells; 3) astrocytes, 4) oligodendrocyte Neural crest cells - 1) PNS neurons, 2) Schwann cells microglia起源: mesoderm (like Macrophage)
3
Q
neural tube defects: 孕第几周？ AFP change? confirmative Dx? 哪几种形式？
A
孕第几周： 4th week AFP ↑ in amniotic fluid and maternal serum confirmative Dx: ↑ AchE in amniotic fluid 哪几种形式: 1. spina bifida occulta 隐性脊柱裂 no herniation, dura intact ; lower vertebral levels; often tuft hair 2. meningocele 脑(脊)膜突出: meninges (but not spinal cord) herniate through spinal canal [AFP normal!!!!} 3. meningomyelocele 脊髓脊膜突出 both meninges and spinal cord herniate through spinal canal
4
Q
anencephaly: Cx? associated with ? what can ↓ risk?
A
Cx: AFP ↑; polyhydramnios (no swallowing center in the fetus brain) associated with type I DM what can ↓ risk: maternal folate intake
5
Q
holoprosencephaly： 定义？发生孕期？signaling pathway affected? moderate form Cx? severe form Cx?
A
前脑无裂畸形 定义: failure of left and right hemisphere to separate 发生孕期: 5 - 6th wks signaling pathway affected: Shh moderate form Cx: cleft lip/palate severe form Cx: cyclopia 独眼[畸形]
6
Q
Arnoid-Chiari malformation： type I vs II? 各自的association?
A
congenital downward displacement of the cerebellar tonsils through the foramen magnum, sometimes causing non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid (CSF) outflow. Type I; mild, may be asymptomatic in infants, onset in adults as headache and ataxia (UW考的病例是22岁出现头疼+步态不稳 association: syringomyelia Type II: more severe, significant herniation of cerebellar tonsils and vermis through foremen magnum association: aquaductal stenosis, hydrocephalus Cx: infact lumbosacral myelomeningocele + paralysis below the defect MRI demonstration of type II may include: beaking of the tectal plate (顶盖）， aqueductal stenosis, kinking and transforaminal herniation of the medulla into the vertebral canal, and herniation and unrolling of the cerebellar vermis into the vertebral canal
7
Q
Dandy-Walker: 定义？ association?
A
agenesis （发育不全）of cerebellar vermis + cystic enlargement of 4th ventricle (fills the enlarged posterior fossa) associated with hydrocephalus and spina bifida
8
Q
syringomyelia: 起病时间？association? 好发脊髓节段？MRI? 横切面上最常累及部位？ 什么感觉丧失？
A
congenital, 起病时间: usually asymptomatic in childhood, manifests with headaches + cerebellar symptoms (起病和Chiari I非常类似，2个病也有association) 好发脊髓节段： C8- T1 MRI： fluid -filled cavity in spinal cord 横切面上最常累及部位：ventral while commissure and ventral horns 什么感觉丧失： bilateral loss of PAIN + Temprature sensation in Upper extremes (fine touch is spared - “dissociated anesthesia”) 常考的情况是病人不能感觉到burn, 被烫伤
9
Q
Nissl substance -can’t detect which cell type in CNS?
A
stain RER (cell bodies and dendrites, not in axon) can’t detect microglia
10
Q
以下neurons的形态？有无髓鞘？ 1、 olfactory N 2. auditory N 3. cutaneous N? 4. motor N?
A
1、 olfactory N: bipolar, nonmyelinated 2. auditory N: bipolar, myelinated 3. cutaneous N (in DRG): pseudounipolar myelinated 4. motor N: multipolar myelinated
11
Q
which structure ↑ conduction velocity? have high cenc. of which ion channel?
A
Nodes of Ranvier (on oligodendrites) high cenc. of Na+ ion channel
12
Q
Oligodendrocytes: in CNS? myelinate how many axons? exist where? origin? in PNS?myelinate how many axons? origin? morphology in H&E staining? injured in which diseases?
A
in CNS: oligodendrocytes myelin ate how many axons?: > 30 exist where: predominately in white matters origin: neuroectoderm in PNS:Swchann cells myelin ate how many axons?: 1 origin: neural crest!!! morphology in H&E staining: “fried egg” injured in which diseases: MS, PML (progressive multifocal leukoencephalopathy 进行性多病灶脑白质病), leukodystrophies 脑白质营养不良
13
Q
MS: neurological deficits associated with MS: Patho? 好发在什么节段？ Cx? MRI? CSF finding?
A
neurological deficits associated with MS: 1) Marcus Gunn pupil (relative afferent pupil) - swinging flash test ; 2) MLF sym (medial longitudinal fasciculus sym, 内侧纵束综合症）：inter-nuclear opthalmoplegia, 对侧外展神经核与同侧动眼N内直肌核失去fiber 联系， cause medial rectus palsy on attempted lateral conjugate gaze and nystagmus in the abducting eye;[impaired eye adduction during lateral gaze] convergence remains intact 3. Optic neuritis: visual disturbance, painful eye movement 4. cerebellar and motor syn: bowel and bladder dysfunction MS的病理基础是：immune-related demyelinating disease: myelin formed by oligodendrocytes undergoes an inflammatory reaction that impairs impulse transmission in axons in the CNS 常常是exacerbation and remission交替， 2 or more sensory or motor systems are affected in separate attacks. The only cranial or spinal nerve affected is the optic nerve （because all of the myelin sheathes of its axons are formed oligodendrocytes) MRI: scattered, periventricular, plaque-like lesions CSF finding: ↑ IgG (“oligoclonal band” on PAGE) Case: 34-yr female, diplopia; clumsiness of left hand and right foot; clumsiness of right hand before but resolved spontenously
14
Q
Dx: ascending paralysis several wks after febrile illness? association? affect what cells? microscope? CSF?
A
Guillain-Barre syn, after respiratory or GI infection; autoimmune ascending paralysis can affect respiratory muscles, and CN VII (Bell’s palsy) strong association with C. jejune affect what cells: Schwann cells demyelinate microscope: Peripheral N demyelination, endoneural inflammatory infiltrate (lymphocytes, macrophages) CSF: ↑ protein + normal or even low cell count [albumino-cytological dissociation]
15
Q
Bilateral acoustic neuroma associate with?
A
neurofibromatosis type 2
16
Q
4 sensory corpuscles各自传导什么感觉？ 1. free ending 2. Meissner 3. Pacinian 4. Merkel discs
A
- free ending: Pain, T 2. Meissner: light touch, position sense 3. Pacinian: pressure, vibration 4. Merkel discs: pressure, deep touch, position sense
17
Q
Which layer of peripheral N needs to be rejoined in microsurgery for limb attachment? What’s its function?
A
Perineurium (Permearbility barrier)
18
Q
神经递质在哪些情况上升？下降？ 在哪里合成？ NE, DA, 5-HT, Ach, GABA
A
NE, 蓝斑 DA： Ventral tegmentum 被盖， and SNc 5-HT,: Raphe nucleus Ach: basal nucleus of Meynert GABA: nucleus accumbens 伏核
19
Q
BBB3大成分？ 哪些部分没BBB?
A
BBB3大成分: 1. tight junction of endothelial cells 2. basement mem 3. astrocyte foot processes 哪些部分没BBB: 1. area postrema 【下丘脑】最后区 - vomit afte chemo 2. OVLT （Organum vasculosum laminae terminalis 【下丘脑】终板血管器） - osmotic sensing 3. neurosecretory products to enter circulation (eg. ADH release) 4. hypothalamic inputs/outputs permeate the BBB
20
Q
hypothalamus 10 nuclei: function总结? neurohypophysis makes 2 hormones, transport them into posterior pituitary: what are these 2 H, and they are made by which nuclei? ADH made by? oxytocin made by? 调节食欲和肥胖的核团？ 调节体温的核团？ 调节昼夜节律的核团？
A
hypothalamus wears TAN HATs T: thirst (ADH - supraoptic nucleus) A: adenohypophysis control anterior pituitary N: neurohypophysis releases hormones into posterior pituitary H: hunger (lateral nucleus); destruction ⇒ anorexia 厌食症； inhibited by leptin A: automonic regulation T: temperatura (anterior nucleus = cooling [A/C]; posterior nucleus: heating) S: sexual urges neurohypophysis makes 2 hormones: 1) ADH made by: supraoptic nucleus 2) oxytocin made by: paraventricular nucleus 调节食欲和肥胖的核团: 1) lateral nucleus: cause hunger; destruction ⇒ anorexia 厌食症； inhibited by leptin [If you zap lateral nucleus, you shrink laterally] 2) Ventromedial nucleus: satiety; destruction ⇒ hyperphagia [临床联系：craniopharyngioma颅咽管瘤中导致肥胖】 stimulated by leptin [If you zap ventramedial nucleus, you grow ventrally and medially] 调节体温的核团: 1) anterior nucleus = cooling [A/C]; Parasympathetic 2) posterior nucleus: heating; sympathetic 调节昼夜节律的核团: suprachiasmatic nucleus (SCN) - circadian rhythm
21
Q
sleep: controlled by ? driven by? involved neurotransmitters? phases? 每个阶段特点？EEG waveforms?
A
sleep: controlled by circadian; driven by: SCN (suprachiasmatic nucleus of hypothalamus) involved neurotransmitters: NE (made at SCN), ⇒ pineal gland to release melatonin phases? 每个阶段特点？EEG waveforms? At night, BATS Drink Blood 1. Awake, eyes open: beta (highest frequency, low amplitude) 2. Awake, eyes closed: Alpha 3. Non-REM, stage 1 (5%): Theta - light sleep 4. Non-REM, stage 2 (45%): sleep spindles + J complexes - deeper sleep [bruxism, 磨牙] 5. Non-REM, stage 3 (25%): Delta- lowest frequency, high amplitude; deepest sleep [sleep walking, night terrors, bedwetting] 6. REM (25%): beta; ↑ O2 use, dream, penile/clitoral tumescence 肿胀,memory processing
22
Q
Which thalamic nucleus controls sensation of pain + Temperature? what else does it mediate? input? output?
A
VPL also for pressure, touch, vibration, proprioception input: spinothalamic and dorsal columns, medial lemniscus output: primary somatosensory cortex
23
Q
Which thalamic nucleus controls sensation of face + taste? input? output?
A
VPM input: trigeminal + gustatory pathway output: primary somatosensory cortex “makeup goes to Face”
24
Q
Which thalamic nucleus controls sensation of vision? input? output?
A
LGN (lateral geniculate body) 外侧膝状体 input: CN II output: Calcarine sulcus 距状沟 “Lateral = Light”
25
Which thalamic nucleus controls sensation of hearing? input? output?
MGN (medial geniculate body) 内侧膝状体 input: superior olive + inferior colliculus of tectum 下丘(四迭体) output: auditory cortex of temporal lobe "Medial = Music"
26
limbic system: components? functions?
the famous 5Fs components: "FAC HiM" 1. F: fornix 2. A: amygdala 3. C: cingulate 4. Hi: hippocampus 5. M: mammillary bodies functions: Feeding, Fleeing, Fighting, Feeling, Sex
27
Cerebellum: input? output? 各自神经通路 Lateral lesions表现？ Medial lesions表现？
input: 1. 从大脑自上而下的input: contralateral cortex via middle cerebellar peduncle 2. 从脊柱自下而上的input: ipsilateral proprioception from spinal cord via inferior cerebellar peduncle (input nerves = climbing and mossy fibers) output: 1. sends info to contralateral cortex (modulate movement) output nerves: Purkinje cells → deep nuclei of cerebellum → contralateral cortex via superior cerebellar peduncle 2. deep nuclei (lateral → medial): Dentate, Emboliform 栓状核， Globose 球状核， Fastigial 顶核 “Don't Eat Greasy Food" Lateral lesions：fall toward injured (ipsilateral) side Medial lesions: truncal ataxia 躯干共济失调， nystagmus, head tilting, wide-based (cerebellar) gait, [note: generally, midline lesions lead to bilateral motor deficits affecting axial and proximal limb musculature]
28
Basal ganglia定义？功能？神经input, output? dopamine D1, D2 pathways分别是stimulatory or inhibitory?
striatum = putamen (motor) + caudate (cognitive) lentiform = putamen + globus pallidus 功能：voluntary movements, postural adjustments input: 1. motor cortex （stimulatory), 2. SNc (substantia nigra pars compacta, 黑质致密部分） (both stimulatory -D1; and inhibitory -D2) Output： 1. direct pathway: cortex → GABA stimulates striatum → dis-inhibit thalamus via GPi/SNr ⇒ ↑ motion 2. indirect pathway: cortex → D2 dis-inhibit STN via GPe + STN stimulates GPi/SNr → inhibit thalamus ⇒ ↓ motion D1 Receptor = DIRect pathway（stimulatory) D2: indirect pathway (inhibitory)
29
PD: patho changes? Cx?
patho: 1) Lewy bodies: intracellular eosinophilic inclusions of alpha-synuclein 2) loss of DA neurons Cx: PD TRAPS your body (Tremor at rest, cogwheel Rigidity, Akinesia/bradykinesia, Postural instability, Shuffling gait)
30
hemiballismus: Cx? lesion?
Cx: sudden, wild flailing of 1 arm +/- ipsilateral leg lesion: contralateral subthalamic nucleus (e.h. lacunar stroke
31
chorea: Cx? lesion?
Cx: sudden, jerky movements lesion: basal ganglia (HD)
32
athetosis: Cx? lesion?
手足徐动症 Cx: slow, writhing movements, especially the fingers lesion: basal ganglia (HD)
33
essential tremor: Cx? predisposition by? Rx?
Cx: action tremor, exacerbated by holding posture/limb position genetic predisposition; pts often self-mediated with EtOH (↓ tremor amplitude) Rx: b-blockers, primidone 扑痫酮
34
intention tremor: Cx? lesion?
Cx: slow, zigzag motion when pointing/extending toward a target lesion: cerebellar dysfuction
35
bilateral amygdala lesions (ie, bilateral ablation of anterior temporal lobes) - cause? association?
Kluver-Bucy syn hyperphagia, hypersexuality, disinhibited behavior, placidity/docility, psychic blindness (visual agnosia) association with HSV-1
36
Wernicke-Korsakoff Sym: Cx? brain lesion? Dx中要注意什么？
Wernicke problems come in a CAN of beer: associated with thiamine (VB1) deficiency and excessive EtOH use; Confusion (retro + anterograde amnesia), Confabulation, Ataxia, Nystagmus brain lesion: bilateral mammalliary bodies Dx中VB1-deficient pts一定要先补VB1, 再给glucose! Because giving glucose without B1 can precipitate this disease
37
which lesion (cerebellar hemisphere vs. vermis) affects lateral limbs? truncal?
cerebellar hemisphere: located laterally - affect lateral limbs Cx: intension tremor, limb ataxia, fall toward side of lesion, ipsilateral deficits cerebellar vermis: located centrally - affect central body Cx: truncal ataxia, dysarthria构音障碍
38
CPM (central pontine myelinolysis): cause? Cx? can lead to? MRI? affected tracts?
cause: a variant of osmotic demyelination syn; 2º to overly rapid correction of hyponatremia, or alcoholism Cx: acute paralysis, dysarthria 构音障碍, dysphagia, diplopia, loss of consciousness can lead to Lock-in syn MRI: massive axonal demyelination in pontine white matter affect corticospinal and corticobulbar tracts
39
correcting serum Na+ too fast will cause: from low to high? from high to low?
correcting serum Na+ too fast will cause: from low to high, your pons will die - CPM （central pontine myelinolysis) from high to low, your brain will blow - cerebral edema/herniation
40
aphasia和dysarthria的区别？ Broca aphasia? affected area? Wernicke aphasia? affected area?
aphasia: higher-order inability to speak dysarthria: motor deficit to speak Broca aphasia (Broca Broken Boca; boca: "mouth" in Spanish) nonconfluent aphasia + intact comprehension affected area: inferior frontal gyrus of frontal lobe Wernicke aphasia: "wordy but makes no sense; what?" fluent aphasia + impaired comprehension and repetition affected area: superior temporal gyrus of temporal lobe
41
major regulator of cerebral perfusion? why giving therapeutic hyperventilation when cerebral edema?
PCO2 (在PCO2 = 0 ~ 90 mmHg区间都是线性关系；\> 90 mmHg后platue) PO2对脑血流的调节仅仅在氧分压极低（\< 50 mmHg； normal PO2 = 100 mmHg)时起作用，线性增加cerebral blow flow why giving therapeutic hyperventilation when cerebral edema: ↓ PCO2 - ↓ intracranial pressure
42
stroke: MCA lesion location? syms?
1. motor cortex - contralateral paralysis of upper limb + face 2. sensory cortex - contralateral loss of sensation - upper and lower limbs + face 3. Temporal lobe (Wernike area - wordy, fluent aphasia) 4. Frontal lobe (Broca area - influent aphasia0 Aphasia if dominant (usually left) hemisphere; Hemineglect if non dominant (usually right) hemisphere
43
stroke: ACA lesion location? syms?
1. motor cortex - contralateral paralysis of lower limb 2. sensory cortex - contralateral loss of sensation - lower limb
44
stroke: lenticulo-striate artery lesion location? syms?
MCA分支 lesion location: striatum， internal capsule syms: contralateral hemiparesis 轻偏瘫/hemiplegia 偏瘫 common location of lacunar infarcts (\< 15 mm, 在偏瘫发生时常常CT看不出，病灶太小；几周后见lake-like cavity, 多在internal capsule; 可以仅仅出现一个肢体（如右手）的运动障碍等。）secondary to uncontrolled HTN, DM, smoking
45
Dx for stroke: which A affected? contralateral hemiparesis, contralateral proprioception ipsilateral hypoglossal dysfunction (a deviation of the tongue to the side of the infarct on attempted protrusions)
Medial medullary syn affected: ASA + vertebral A
46
Dx for stroke: which A affected? dysphagia, hoarseness, ↓ gag reflex ipsilateral Hornor syn
lateral medullary (Wallenberg) syn affected: PICA 小脑下后动脉 “Don't PICK A (PICA) horse (hoarseness) that can't eat (dysphagia)"
47
Dx for stroke: which A affected? paralysis of face, ↓ pain and T sensation ↓ lacrimation, salvation, ↓ of taste from anterior 2/3 of tongue, ↓ ipsilateral hearing ipsilateral Hornor syn
AICA 小脑下前动脉 Lateral pontine syn
48
stroke: PCA lesion location? syms?
lesion location: occipital cortex, visual cortex syms: contralateral hemianopia + muscular sparing
49
stroke: basilar A lesion location? syms?
lesion location: pons, etc syms: locked-in syn
50
stroke: ACom and PCom A? lesion location? syms?
aneurysms
51
Berry aneurysm: MC location: MC complication? Cx? If compress optic chiasm, Cx? Association? risk factors?
MC location: junction of ACA/ACom MC complication: rupture leading to subarachnoid hemorrhage ("worst headache of life, 剧烈头疼）； or hemorrhagic stroke If compress optic chiasm, Cx: bitemporal hemianopia Association: ADPKD, Ehlers-Danlos, Mafan risk factors: black, age, HTN, smoking
52
Charcot-Bouchard microaneurysm: affected location? association?
affect small vessels (e.g., in basal ganglia, thalamus) - cause intraperenchymal hemorrhage associated with chronic HTN
53
Dx: pain, initial sensation of numbness and tingling, followed in wks to mo by allodynia (ordinarily painless stimuli cause pain), and dysanethesia
Central post-stroke pain syn happens in 10% stroke pts neuropathic pain due to thalamic lesions
54
diff: epidural vs. subdural hematoma: cause? affected vessels? expansion speed? shape on CT? can or can't cross suture line, falx, tentorium?
page 485 on FA2014
55
Dx: Worst headache of my life Cx? spinal tap see? risk after 2-3 d? treatment?
subarachnoid hemorrhage
56
ischemic stroke: ? min causes irreversible damage? most vulnerable areas? early imaging? late imaging? how to exclude hemorrhage? - why is this important? time course-events: 12 -48 hr, 24-72 hrs, 3-5 d, 1-2 wks, \> 2 wks
ischemic stroke: 5 min causes irreversible damage most vulnerable areas: hippocampus, neocortex, cerebellum, watershed areas early imaging: bright on diffusion-weighed MRI in 3- 30 min late imaging: dark abnormality on non contrast CT in 12-24 hrs how to exclude hemorrhage: absence of bright areas on non contrast CT 准确排除出血 - why is this important： tPA禁忌症 time course-events: 12 -48 hr: red neurons 24-72 hrs: necrosis + neutrophils 3-5 d: macrophages 1-2 wks: reacitve gliosis + vascular prolifeation \> 2 wks: glial scars
57
ischemic stroke leads to ? type of necrosis? 分型? 各自位置？病因？
ischemic stroke leads to liquiefactive necrosis 3 types: 1. thrombotic - usually over Atherosclerotic lesions, clot forming at the site of infarction, usually MCA 2. embolic: embolus from other parts; can affect multiple vascular territories; often cardioembolic 3. hypoxic: hypoperfusion or hypoxemia, common during cardiovascular surgeries, affect watershed areas
58
lateral ventricle drains into 3rd ventricle via? 3rd ventricle drains into 4th ventricle via? 4th ventricle drains ? via?
lateral ventricle drains into 3rd ventricle via: foreman of Monro 门罗孔,室间孔 3rd ventricle drains into 4th ventricle via: cerebral aqueduct of Sylvius 4th ventricle drains into Subarachnoid space via: lateral： foramen of Luschka 路西卡孔,第四脑室外侧孔 medial: foramen of Magendie 马让迪孔,第四脑室正中孔
59
脑积水分型？各自imaging特点和Cx? 颅内压是否升高？
1. communicating (non-obstructive): cause: 吸收障碍，↓ CSF absorption by arachnoid granulations （例如arachnoid scarring post-meningitis)， 颅内压升高, papilledema, herniation 2. non-communicating (obstructive): cause: structural blockage in circulation within the ventricular system (例如stenosis of the aqueduct of Sylvius)， 颅内压升高, 3. normal pressure hydrocephalus: no increase in subarachnoid space volume, expansion of ventricles, Cx: triad of urinary incontinence, ataxia, cognitive dysfunction - "Wet, Wobble, and Wacky" 4. hydrocephalus ex vacuo 脑外积水: 其实不是CSF变化，而是脑萎缩导致imaging看起来像CSF上升 （atrophy: AD, advanced HIV, Pick disease - Frontotemporal Dementia, is a rare neurodegenerative disease). 颅内压正常，triad not seen
60
How many spinal nerves? 每个节段各有几条？ 从corresponding vertebra exit 的特点？
31 in total: 8 cervical 12 thoracic 5 lumbar 5 sacral 1 coccygeal 尾骨的 C1-C7: exit above the corresponding vertebra C8- below: exit below the corresponding vertebra
61
lumbar puncture位置？
L3/L4 or L4/L5 because spinal cord extends to L1-L2 vertebrae Subarachnoid space extends to lower border of S2
62
dorsal column: 上行还是下行？传导什么？ 肢体的organization? N走形？交叉？一级、次级、3级神经元：
ascending conduct PPTV (pressure, proprioception, touch, vibration) 一级神经元: DRG 进入spinal cord之后，经ipsilaterl dorsal column 上升，一直到medulla交叉换元 二级神经元在对侧medial lemniscus里上行, thalamus VPL进行第二次synapse 3级神经元: sensory cortex
63
spinothalamic tract: 上行还是下行？传导什么？ 肢体的organization? N走形？交叉？一级、次级、3级神经元：
ascending conduct PT (pain, Temperate) 肢体的organization: legs (lumbosacral) are lateral in Lateral spinothalamic tract 一级神经元: DRG 进入spinal cord之后，直奔ipsilaterl grey matter，交叉到对侧换元 对侧上行到 thalamus VPL进行第二次synapse 3级神经元: sensory cortex
64
UMN/ LMN signs?
Lower MN= everything lowered (less muscle tone, muscle mass, reflexes, down going toes) upper MN= everything up (muscle tone, reflexes, up going toes) LMN signs: flaccid paralysis UMN signs: Babinski +, spastic paralysis +, clasp knife spasticity +
65
仅仅损伤LMN的2个典型病？ lesion location?Cx?
Poliomyelitis, spinal muscular atrophy (Werdnig-Hoffman disease) LMN lesions in anterior horns; Cx: flaccid paralysis
66
ALS: lesion location? affect UMN or LMN? 可能的一种病因？ macroscope / microscope see? Cx? Rx及其机制?
ALS: Lou Gehrig disease lesion location: anterior horns (LMN) and lateral corticospinal tract (传导UMN来的信号) affect UMN or LMN： both (与poliomyelitis, Werdnig-Hoffman syn的仅仅LMN相鉴别） macroscope: thin anterior roots (loss of neurons in anterior horns), mild atrophy of pre central gyrus, degeneration of corticospinal tract Cx （记住霍金）：combined UMN and LMN signs, NO sensory, cognitive or oculomotor deficits 可能的一种病因： can be caused by defect in SOD1 (superoxide dismutase 1) Rx: Riluzole， which can decrease presynaptic glutamate release
67
Dx: HIV+ male, difficulty in walking; small, irregular pupils that do not react to light but constrict with accommodation （这种瞳孔叫什么？） DTR (deep tendon reflexes) absent, Romberg sign +? 病原体？神经系统损伤部位？ 在哪种病人中进程特别迅速？
Dx: Tabes dorsalis 脊髓痨,运动性共济失调 (梅毒3期） 病原体：梅毒苍白螺旋体（Treponema palladium of Sprirochete) 神经系统损伤部位: dorsal column of spinal cord - imparied sensation and proprioception, and progressive sensory ataxia (inability of feeling the legs) small, irregular pupils that do not react to light but constrict with accommodation - "Argyll Robertson pupils" DTR (deep tendon reflexes) absent, Romberg sign + (can't maintain the balance when eyes close) proceed rapid in HIV+ pts
68
VB12 or VE deficiency cause what neurodamage? Cx?
subacute combined degeneration - demyelination of dorsal columns, lateral spinocerebellar tracts, and lateral corticospinal tracts (累及运动及感觉） Cx: ataxic gait, parethesia, impaired position and vibration sense (dorsal column)
69
Poliomyelitis: pathogen? 什么属？DNA和被摸特性？ 传播方式？检测？ lesion location? Cx? Vaccine?
? 什么属？DNA和被摸特性？ -poliovirus, one of "PERCH" picornavirus (微小病毒属: PERCH: polio, echo, rhino, coxaki, HAV) no envelope, ss+ linear, icosahedral capsid 传播方式: fecal-oral; replicate in oropharynx and small intestine, then spread via bloodstream to CNS 检测: CSF: WBCs increase, protein slight increase, NO GLUCOSE change!! recover virus from throat and stool lesion location: anterior horn of spinal cord (LMN) Cx: 1) LMN signs (weakness, hypotonia, flaccid paralysis, muscle atrophy) 2) infection signs: fever, malaise, headache, nausea Vaccine: Salk/Sabin vaccine
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spinal muscular atrophy: 又名？ 遗传特征？ lesion location? Cx?
Werdnig-Hoffmann disease congenital, AR lesion at anterior horns (LMN) "floppy baby"; infantile type has median age of death at 7 mo
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Friedrich ataxia： 遗传特征？ lesion location? Cx?
AR, trinucleotide repeats, mutation of frataxin gene （iron binding protein) on chr# 9, which is essential for Mito function Cx: degeneration of multiple spinal cord tracts → muscle weakness and loss of DTRs, vibration sense, proprioception. has the same spinal cord pathology as symptoms as subacute combined degeneration (caused by VitB12 deficiency in pernicious anemia)- Staggering gait, falling, nystagmus, dysarthria, pea cavus (弓形足） hypertrophic cardiomyopathy （cause of death). Present in childhood with kyphoscoliosis 特征病变： kyphoscoliosis, foot abnormalities (high plantar arch, 跖） "Friedreich is Fratastic (frataxin): he is always staggering and falling, but has a big heart"
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Brown-Sequard syn: Cx 如果发生在T1以上，有什么表现？
脊髓半断综合征 5大要点： 1. 运动： 1）at the level of lesion: LMN signs (flaccid paralysis) 2) below the level of lesion: UMN signs (corticospinal tract damage) 2. 感觉： 3) at the level of lesion: 同侧感觉完全丧失 4）below the level of lesion: 同侧感觉丧失: proprioception, joint and position sensation, vibration, 2-point discrimination 5）below the level of lesion: 对侧感觉丧失: pain and temperature sensation (spinothalamic tract) 如果发生在T1以上，Horner syn (damage to oculosymphathetic pathway) 主要损伤3个神经通路： 1。 传导crude touch, pain, temperature 的lateral spinothalamic tract上行通路：因为在脊髓里交叉，病灶对侧 1-2个segments以下的感觉丧失，同侧无损； Interruption of lateral spinothalamic tracts: Contralateral loss of pain and temperature sensation. This usually occurs 2-3 segments below the level of the lesion. 2。 传导fine touch, sense of vibration, form recognition, proprioception的dorsal (posteriors) column - medial lemniscus pathway 上行通路受损：因为在medulla才交叉，所以在脊髓水平的截断伤导致病灶同侧fine touch的丧失，对侧无损； Interruption of posterior white column:: Ipsilateral loss of tactile discrimination, vibratory, and position sensation below the level of the lesion 3。下行的运动通路lateral corticospinal tract损伤：因为在脊髓水平交叉，脊髓截断伤导致病灶同侧的运动功能丧失： psilateral spastic paralysis below the level of the lesion Babinski sign ipsilateral to lesion
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Horner syn: 发生节段？Cx? Association?
Sympathectomy of face: (above T1) Ptosis, anhidrosis, flushing, miosis "PAM is horny" Associated with lesion of spinal cord above T1: Pancoast tumor, Brown-Sequard syn (Cord hemisection), late-stage syringomyelina
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以下反射由什么神经控制： biceps triceps patella [pә'telә] 膝盖 Achilles testicle move anal wink reflex
biceps: C5,6 triceps: C7, 8 patella [pә'telә] 膝盖: L4 Achilles: S1, testicle move: L1, 2 anal wink reflex: S3,4
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Primitive reflexes: 正常消失时间？成人中出现意味着？ Moro Rooting sucking palmar plantar Galant
Primitive reflexes: 正常消失时间 \< 1 yr; 成人中出现意味着front lobe lesions (loss of inhibition) Moro: 抓握 Rooting：nipple seeking sucking: strike the roof of mouth ⇒ suck palmar: finger curl plantar: 脚趾背曲 （成人中为Babinski) Galant : 加兰特反射（下腹部深层反射）
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brain stem ventral view: which CNs lie medially?
III, VI, XII 3\*2 = 6 (\*2) = 12
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midbrain最显著的几个结构？临床最常见的疾病？
pineal body (melatonin) superior colliculi - conjugate vertical gaze center inferior colliculi - auditory center 临床最常见的疾病: Parinaud syn (= dorsal midbrain syn, 松果体瘤引起的压迫症状）
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Which CN is the only one without thalamic relay to cortex?
CN 1 (olfactory)
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Which CN controls pupillary constriction? innervate which muscle? controlled by what nucleus?
CN III; sphincter pupillae - Edinger-Westpal nucleus (Muscarinic R)
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Which CNs control eyelid opening/closing? innervate which muscles?
eyelid opening: CN III (oculomotor) - levator palpebrae closing: CN VII (Facial) - orbicularis oculi
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Which CN controls stapedius muscle? what is special about its course?
CN VII (facial) - it courses through the parotid gland (salivation), but does not innervate it
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function of CN XI?
Accessory - head moving; shoulder shrugging （SCM， trapezius 斜方肌）
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CN nuclei 分布？
in tegmentum (被盖）in brain stem: midbrain: CN III, IV, pons: CN V, VI, VII, VIII medulla: XI, X, XII spinal cord: XI (注意：副神经核团在脊柱里！！！） 感觉神经核团在外侧，运动神经核团在内侧： Lateral nuclei = sensory (aLar plate) Medial nuclei = Motor
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reflex afferent/efferent: corneal?
afferent: V1 opthalmic (nasociliary 鼻睫状的） efferent: VII (temporal branch; orbicularis oculi)
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reflex afferent/efferent: lacrimation?
afferent: V1 (loss of reflex does nor preclude emotional tears) efferent: VII 和corneal reflex一样
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reflex afferent/efferent: jaw jerk
afferent: V3 (sensory: muscle spindle from masseter) efferent: V3 (motor: masseter)
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reflex afferent/efferent: pupillary
afferent: II efferent: III
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reflex afferent/efferent: gag
afferent: IX 舌咽 efferent: X 迷走
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vagal nuclei包括哪几个核团？各自的功能？哪些神经传入？
1. Nucleus Solitarius 孤束核 功能： visceral sensory (taste, baroreceptors, gut distention) VII 面，IX, 舌咽，X 迷走 2. Nucleus aMbioguus 疑核 功能： Motor innvervation of pharynx, larynx, upper esphogus (swallowing, palate elevation) IX, 舌咽，X 迷走, XI 副 3. dorsal motor nucleus: 功能： send parasympathetic fibers to heart, lungs and upper GI X 迷走
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define "cavernous sinus": direction of blood flow? contains which CNs and vessels? If damaged (by mass, fistula, thrombosis）, Cx?
cavernous sinus: a collection of venous sinuses on both sides of the pituitary. direction of blood flow: eye and superficial cortex → cavernous sinus → internal jugular vein contains which CNs and vessels： CN III, IV, V1, V2, VI, + postganglionic sympathetic fibers en route to the orbit If damaged (by mass, fistula, thrombosis): "cavernous sinus syn) - ophthalmoplegia, ↓ corneal and maxillary sensation [but normal visual acuity 视觉分辨率】 CN VI (外展）commonly affected
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CN V motor lesion causes ?
jaw deviates toward side of lesion (no opposite force from the opposite pterygoid muscle)
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CN X motor lesion causes ?
Uvula ('yu:vila, 悬雍垂）deviates away from the side of lesion, weak side collapse and uvula points away.
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CN XII motor lesion (LMN) causes ?
Tongue deviates TOWARD side of lesion (lick your wounds) due to weakened tongue muscles on the affected side
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2 tuning fork tests? normal vs. abnormal? lesion location?
1. Weber's test: 音叉放头顶， normal: hear equally on both sides pt with unilateral conduction deafness: localized to affected side pt with unilateral nerve deafness: localized to affected side 2. Rinne test: compare air vs. bone conduction (place the vibrating fork on the mastoid process until it is no longer heard, then held in front of the ear) 正常情况空气跑得比骨头快
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facial lesions：UMN vs. LMN表现？ Facial nerve palsy: Cx? Association? Rx?
facial lesions： UMN: lesions in motor cortex, or connection between cortex and facial nucleus - contralateral paralysis of lower face [forehead spared - bilateral innervation] LMN: ipsilateral paralysis of [upper + lower] face Facial nerve palsy: 若是原发性，即为Bell palsy Cx: 外周同侧面神经瘫，drooping smile, can't close eye on lesion side Association: Lyme disease, HSV (herpes simplex), HZV (zoster), sarcoidosis, tumors, DM Rx: corticosteroids
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muscles to open/close jaw? innervation?
muscles to open jaw: 一条肌肉 （lateral pterygoid 翼外肌）- Lateral Lower jaws muscles to close jaw： 3条肌肉 （medial pterygoid 翼内肌， ma'sseter 咬肌，teMporalis 颞肌） "M's Munch" innervation: all by CN V3
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Define the refractive eye conditions/累及部位？ 1. hyperopia 2. myopia 3. a'stigmatism 4. presby'opia
1. hyperopia 远视; eye too short for refractive power of cornea and lens → light focused behind retina 2. myopia: 近视; eye too long for refractive power of cornea and lens → light focused in front of retina 3. a'stigmatism 散光； abnormal curvature of cornea → different refractive power at different axes 4. presby'opia 老花； ↓ in focusing ability during accommodation due to sclerosis and ↓ elasticity
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Uveitis: lesion? Cx? Association?
炎症位置在anterior uvea and iris, with hypopyon [hai'poupion], 眼前房积脓 （sterile pus) conjuctival redness Association: often systemic inflammatory disorders (sarcoid, RA, juvenile idiopathic arthritis, TB, HLA-B27-associated conditions)
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retinitis: Cx? cause? association?
retinitis: Cx: retina edema and necrosis leading to scar cause: often viral (CMV, HSV, HZV) association: immunusuppression
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acute, painless monocular vision loss + retina cloudy with attenuated vessels and "cherry-red" spots at the fovea 中央凹： Dx?
central retinal artery occlusion
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Cause for retinal v. occlusion? Cx?
blockage of central or branch retinal v, due to compression from nearby arterial atherosclerosis Cx: retinal hemorrhage and edema in affected area
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Diabetic retinopathy分型？Cx? Rx?
2 types: 1. non-proliferative: damaged capillaries leak blood → lipid and fluid seep into retina → hemorrhages and macular edema Rx: blood sugar control; macular laser 2. proliferative: chronic hypoxia → new blood vessel formation → traction on retina Rx: peripheral photocoagulation 光凝固术, anti-VEGF injeciton
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aqueous humor pathway: 由什么细胞生成？location? flow pathway?
由什么细胞生成：ciliary epithelium location: 房水位于lens与cornea之间 flow pathway：在后房生成，通过lens和sphincer之间进入前房，进入trabecular meshwork, → canal of Schlemm
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Glaucoma: 分型， 各自的association? Primary, secondary cause? Cx?
1. open angle: painless, common in US association: ↑ age, African American, family history; Primary cause: unknown secondary cause: blocked trabecular meshwork from WBCs （uveitis), RBCs (hemorrhage), retinal detachment 2. closed/narrow angle: chronic - painless, acute - 剧痛, ↑ IOP, sudden vision loss, halos around lights, rock-hard eye, frontal headache, [Do NOT give Epinephrine - 有散瞳作用 mydriatic effect!!!!!!] Primary cause: 房水流动通道上出现阻塞, → fluid builds up behind iris, impede flow thru trabecular meshwork secondary cause: hypoxia (i,e.: DM, vein occlusion) → vasoproliferation in iris → contracts angle
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6条动眼肌的神经控制？ N damage眼睛的表现？ test for each extra ocular muscles?
"LR6SO4R3" Lateral rectum m 外直肌： CN VI 外展N Superior oblique m 上斜肌：CN IV 滑车N R3: the rests are controlled by CN III 动眼N CN III damage: eye looks down and out; ptosis 上睑下垂， pupillary dilation, loss of accommodation CN IV damage: eye moves upward, especially with contralateral gaze; head tilt to the lesion side - pts have problems going downstair, may have to tilt the head in the opposite direction for compensation CN VI damage: eye medially direct, can't abduct (外展N受损） test for each extra ocular muscles：IOU (test IO: look up)
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Dx: eye looks down and out; ptosis 上睑下垂， pupillary dilation, loss of accommodation
CN III damage
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Dx: eye moves upward, especially with contralateral gaze; head tilt to the lesion side - pts have problems going downstair, may have to tilt the head in the opposite direction for compensation
CN IV damage
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pupillary control: 交感vs. 副交感，1级、2级, 3级neuron location? 神经走行？muscles involved?
1. miosis (constriction - parasym) 1st neuron: Edinger-Westphal nucleus to ciliary ganglion via CN III 2nd neuron: short ciliary n to pupillary sphincter muscles 2. mydriasis (散瞳，交感） 1st neuron:hypothalamus to ciliospinal center of Budge (C8-T2) 2nd neuron: exit at T1 to superior cervical ganglion (travel along cervical sympathetic chain near lung apex and subclavian vessels) 3rd neurons: plexus along internal carotid, thru cavernous sinus; enter orbit as long ciliary n to pupillary dilator muscles
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pupillary light reflex: 整个回路？
light in either retina → send the signal via CN II (视神经） → pretectal nuclei in midbrain (中脑顶盖前核） → activate bilateral Edinger-Westphal nuclei → CN III 动眼神经
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Define: Marcus Gunn pupil
afferent pupillary defect: ↓ bilateral pupillary constriction when light is shone in affected eye relative to unaffected eye (swinging flashlight test" due to optic nerve damage or several retinal injury
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Dx: 什么肌肉、神经受损？Cause? 1. ptosis + "down and out gaze" 2. ↓ or absent pupillary light reflex ("blown pupil", often + "down and out gaze"
CN III has both central (motor) and peripheral (parasympathetic) components 1. ptosis + "down and out gaze": - CN III motor output to ocular muscles (LR6SO4R3) affected, 主要的病因是DM: ↓ diffusion of oxygen and nutrients to the interior fibers 2. ↓ or absent pupillary light reflex ("blown pupil", often + "down and out gaze" - affect the parasympathetic output: fibers on the periphery are affected by compression (posterior communicating artery aneurysm， uncial herniation)
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retinal detachment: happen between which layers? result? cause? sign? Rx?
retinal detachment: happen between neurosensory layer (rods and cones) and the outermost pigmented epithelium (normally shields excess light, support retina) result: degeneration of photoreceptors → vision loss cause: often 2° to retinal breaks, DM, inflammatory effusions; breaks common in pts with high myopia (高度近视), often preceded by posterior vitreous detachment (玻璃体后部脱落） sign: ”curtain drawn down" Rx: surgical emergency
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age-related macular degeneration Cx? 分型？cause? Rx?
Cx: distortion (metamorphopsia 视物变形） and loss of central vision (scotoma 盲点） 分2型： 1. dry (nonexudative, \> 80%) - deposition of yellowish extracellular material in and beneath Bruch membrane and retinal pigment epithelium, gradual ↓ vision Rx: prevention with multivitamin + antioxidant supplements 2. wet (exudative, 10 - 15%）- rapid loss of vision due to bleeding 2° to choroidal neovascularization Rx: anti-VGEF injection or laser
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define "IFO" seen in which diseases? Cx? direction terms (e.g: right IFO) refer to?
IFO = internuclear ophthalmoplegia 核间性眼肌麻痹, indicate lesion in MLF (medial longitudinal fasciculus) MLF: pair of tracts - allow crosstalk between CN III (控制LR 外直肌）and CN VI (外展神经，控制除了LR, SO之外的4条肌肉） highly myelinated - 需要极快的速度crosstalk to coordinate the eye movements, 因此病变见于脱髓鞘的疾病如MS中 （MLF in MS） Cx: convergence normal, but when lateral gaze: 正常侧眼睛出现nystagmus (CN VI overfires, trying hard to stimulate contralateral CN III) direction terms (e.g: right IFO) refer to which eye is paralyzed (right INO: right eye is paralyzed)
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AD: -3 genes for early onset, and chr association: - late onset： gene and cur? which gene (on chr ?) is protective?
-3 genes for early onset, and chr association: APP (chr 2) Presenilin-1 (chr 14) presenilin-2 (chr 1) - late onset：Apo E4 (chr 19) which gene (on chr ?) is protective: Apo E2 (chr 19)
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Pick disease: define Cx spare which region? diff? Patho?
Pick disease: frontotemporal dementia Cx: dementia, aphasia (frontal lobe Broca区受损症状），parkinsonian aspects, personality change spare which region: parietal lobe and posterior 2/3 of superior temporal gyrus diff: AD (which is generalized brain atrophy) Patho: pick body (tau protein aggregates), frontotemporal atrophy
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lewy body dementia: define Cx Patho?
LBD: umbrella term for two related diagnoses: 1) Parkinson’s disease dementia and 2) dementia with Lewy bodies. Cx: initially dementia and visual hallucinations, followed by parkinsonian features path: a-synuclein defect
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Creutzfeldt-Jakob disease: define Cx Cause?
define: spongiform cortex Cx: rapid progressive (wks to months) dementia + myoclonus ("startle myoclonus") Cause: Prions: PrPc → PrPsc sheet: beta-pleated sheet, resistant to protease
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which vit deficits will cause dementia?
VB1, B3, B12
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acute inflammatory demyelinating polyradiculopathy: 急性炎症性脱髓鞘多神经跟病： Cause Cx prognosis? Lab? association? Rx?
Cause: MC variant of Guillain-Barre syn; autoimmune destroy of Schwann cells → inflammation and demyelination of peripheral n, and motor fibers Cx: symmetric ascending muscle weakness; start in lower extremities, facial paralysis in 50% pts prognosis: good, almost all pts survive; the majority recover completely after wks to mo Lab: 1) ↑ CSF protein, but cell count normal (albuminocytologic dissociation) - 和格林巴利一样 2） ↑ protein ⟹ papilledema association: infections (Campylobacter jejuni, CMV) - caused autoimmune attack Rx: 1) respiratory support (critical until recovery) 2) plasmapheresis, IVIG
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progressive multifocal leukoencephalopathy: Cause association? prognosis? ↑ risk with a specific drug?
Cause: CNS demyelination due to destruction of oligodendrocytes association: with JCV (John Cunningham virus), seen in 2-4% AID (reactivation of latent virus) prognosis: rapid progressive, usually fatal Rx: ↑ risk with natalizumab (drug for MS and Crohn disease, McAb for a4-integrin)
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acute disseminated encephalomyelitis (postinfectious encephalomyelitis) 急性播散性脑脊髓炎, 传染病后脑炎
multiple perivencular inflammation and demyelination after infection (commonly measles or VZV) or certain vaccination (rabies, smallpox)
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metachromatic leukodystrophy （MLD): 异染性脑白质营养不良 MC? findings?
AR; lysosomal storage disease, MC due to arylsulfatase A deficiency (芳香基硫酸酯酶A缺乏）→ buildup of sulfatides 硫脂类 → impaired production of myelin sheath Cx: CNS + PNS demyelination, ataxia, dementia
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Charcot-Marie-Tooth disease: also known as? Cause? Genetic inheritance pattern? association?
also known as hereditary motor and sensory neuropathy (HMSN) Cause: a group of progressive hereditary disorders, related to defective proteins involved in the structure and function of peripheral n or the myelin sheath Genetic inheritance pattern: usually AD association: scoliosis and foot deformities (high or flat arches)
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Krabbe disease: Cause? Genetic inheritance pattern? Cx?
Genetic inheritance pattern: AR; lysosomal storage disease Cause: deficiency in galactocerebrosidase 半乳糖(基)脑苷脂酶 buildup of galactocerebroside + pyschosine → destroy myelin sheath Cx: PNS neuropathy, developmental delay, optic atrophy, globoid cells
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adrenoleukodystrophy: Genetic inheritance pattern Cause? Cx?
脑白质肾上腺萎缩症 X-linked, usually affect males Cause: disrupt metabolism of very-long-chain FA → excessive buildup in nervous system, adrenal glands, and testes Cx: progressive, can lead to long-term coma /death and adrenal gland crisis
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Cluster headache: localization? duration? Cx? Rx? diff from trigeminal neuralgia?
localization: unilateral duration: 15 min - 3hr, repetitive [diff from trigeminal neuralgia: which \< 1 min] Cx: repetitive, brief headachesl excruciating periorbital pain, + lacrimnation and rhinorrhea may induce Horner syn Rx: 1) inhaled oxygen ; 2. sumatriptan 舒马曲坦
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Tension headache: localization? duration? Cx? Rx?
localization: bilateral duration: \> 30 min [typical 3-6 hr], constant Cx: steady pain [diff from migraine: has no photophobia, or phono phobia, no aura] Rx: 1) analgesic, NSAIDs ; acetaminophen; amitriptyline [阿米替林(抗抑郁药)]for chronic pain
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Migraine headache: localization? duration? Cx? Rx?
localization: unilateral duration: 4-72 hr, Cx: pulsating pain with nausea, photophobia, phono phobia, may have aura Rx: 1) abortive therapies: triptans, NSAIDs, 2) prophylactic: propranolol, topiramate, CCB, amitriptyline [阿米替林(抗抑郁药) "POUND": Pulsative, One-day duration, Unilateral, Nausea, Disabling
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Vertigo: types? causes? tests?
1. Peripheral: more common. Causes: inner ear etiology (such as Meniere disease) positioning test: delayed horizontal nystagmus 2. Central vertigo: Cause: brain stem or cerebellar lesion (e.g., stroke affecting vestibular nuclei or posterior fossa tumor) Cx: directional change of nystagmus, skew deviation, diplopia, dysmetria positioning test: immediate nystagmus in any direction, may change direction + focal neurological findings
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Sturge-weber syn: 遗传特征？mutation? affect what? Cx?
congenital, non-inhertied (somatic) mutation, developmental anomaly of neural crest derivation (meso/ectoderm) due to activating mutation of GNAQ gene [Guanine nucleotide-binding protein G(q) subunit alpha] affect capillary vessels → non-neoplastic birthmark in CN V1/V2 distribution "STURGE-weber": Sporadic, port-wine Stain Tram track Ca2+ in opposite gyro Unilateral Retardation Glaucoma (early-onset), GNAQ Epilepsy
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Tuberous sclerosis: 遗传特征？ Cx? ↑ incidence of which diseases?
结节性(脑)硬化 遗传特征: AD HAMARTOMAS: Hamartomas in CNS and skin Angiofibromas Mitral regurgitation Ash-leaf spots Rhabdomyoma Tuberous sclerosis autosomal dOminant Mental retardation renal Angiomylipoma Seizures, Shagreen patches ↑ incidence of subependymal astrocytomas and ungual fibromas
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Neurofibromatosis: type I gene mutation? chr#? Patho? skin tumors are derived from ? Cx?
von Recklinghausen disease 雷克林霍曾(氏)病,神经纤维瘤病 gene mutation: NF1 tumor suppressor (neurofibromin: a negative regulator of Ras) chr#: 17 Cx: 1. Cafe-au-lait spots (skin tumors are derived from neural crest cells) 2. Lisch nodules (pigmented iris hemartomas) 3. neurofibromas in skin 4. optic gliomas 5. pheochromocytomas
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von Hippel-Lindau disease (VHL): 遗传特征？ gene mutation? chr#? Patho? Cx?
血管母细胞瘤 遗传特征： AD VHL抑癌基因突变：chr 3P constitutive expression of HIF and activation of angiogenic growth factors (e.g.., Epo, VEGF) 累及小脑、脊髓、肾脏以及视网膜。其若干病变包括肾脏血管瘤、肾细胞癌以及嗜铬细胞瘤等 1, cavernous hemangiomas in skin, mucosa, organs; 2. bilateral renal cell carcinoma 3. hemangioblastoma in retina, brain stem, cerebellum (hyperchromatic nuclei) 4. pheochromocytomas
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#1 primary brain tumor in adults? median survival? Macroscope? microscope? Staining marker?
Glioblastoma multiforme: median survival: 1 yr found in hemisphere, pleomorphic tumor -border central areas of necrosis and hemorraghe Can cross corpus callosum - "bufferfly glioma" microscope: pseudopalisading 假栅栏形成 marker: GFAP
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pilocytic astrocytoma: 好发人群？部位？良、恶性？ marker? macroscopic finding? microscopic finding?
毛细胞性星形细胞瘤 好发人群-children 部位: posterior fossa of cerebellum 后颅凹, may be supratentoial 良、恶性: benign, good prognosis marker: GFAP + macroscopic finding: cystic + solid, well circumscribed microscopic finding: 1) Rosenthal fiber 罗森塔尔纤维, - eosinophilic 2) corkscrew fibers 螺旋状纤维
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medulloblastoma: 好发人群？良、恶性？ 部位？ 胚胎起源？ macroscopic finding? microscopic finding?
children cerebellum - compress the 4th ventricle - hydrocephalus ectoderm
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craniopharyngioma: 好发人群？良、恶性？ 部位？ 胚胎起源？ 鉴别诊断？ macroscopic finding? microscopic finding?
好发人- children， MC childhood supratentorial tumor 良、恶性 - benign 部位: near pituitary (diff: both can cause bitemporal hemianopia) 胚胎起源- Rathke pouch 拉特克囊,神经颊囊
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Rx: glaucoma mechanism for each drug? side effects? use which drug for emergency?
1. a-agonists: ↓ aqueous humor synthesis 1) Epi: [note: mydriasis, 不能用于closed-angle glaucoma] 2) Brimonidine (a2 specific) 2. beta-blockers: ↓ aqueous humor synthesis Timolol: 噻吗洛尔, 青眼露 betaxolol, 倍他洛尔 carteolol 3. Diuretics: Acetazolamide 乙酰唑胺 (inhibit carbonic anhydrase) 4. cholinomimetics: 拟胆碱药; ↑ outflow of aqueous humor via contraction of ciliary muscle and opening of trabecular meshwork 1) direct: pilocarpine, carbachol [卡巴胆碱】 - 2) indirect: physostigmine, echothiophate 二乙氧膦酰硫胆硷 Use pilocarpine in emergencies - open meshwork into canal of Schlemm 5. Prostaglandin: ↑ outflow of aqueous humor latanoprost: 拉坦前列素 side effect: darkens color of iris (browning)
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Opioid analgesics: include which drugs? opioid receptors? - mechanisms? toxicity? treated with?
M3D2, Let's FC! Morphine, Methadone 美散痛 [maintenance programs for heroin addicts] Meperidine [me'peridi:n] 杜冷丁 dextromethorphan: 右甲吗喃 [cough suppression] diphenoxylate 苯乙哌啶 [diarrhea] Loperamide [lou'peramid] 易蒙停 [diarrhea] Fentanyl 芬太尼 Codeine opioid receptors: MDK (mu = morphine, delta = enkephalin 脑啡肽, kappa = dynorphin) mechanism: modulate synaptic transmission - open K+ channels, close Ca2+ channels - ↓ synaptic transmission, inhibit release of Ach, 5-HT, glutamate, substance P toxicity: addiction, respiratory depression, constipation, miosis treated with: naloxone or naltrexone [opoid receptor antagonist]
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Ethosuximide: what kind of drug? clinical use？ mechanism? side effects? notes?
乙琥胺 epilepsy drugs (for Generalized/absence) mechanism: block thalamic T-type Ca2+ channels side effects: "EFGHIJ" - Ethosuximide causes Fatigue, GI distress, Headache, Itching, Stevens-Johnson syn [(SJS) is a milder form of toxic epidermal necrolysis (TEN)] notes: Sucks to have Silent (absence) Seizures
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Benzodiazepines有2种用于epilepsy: which 2? for what kind of epilepsy? clinical use？ mechanism? side effects? notes?
diazepam, 地西泮; 安定 lorazepam 劳拉西泮 for what kind of epilepsy: status (DOC: lorazepam) mechanism: ↑ GABAa action side effects: sedation, tolerance, dependence, respiratory depression notes: also for for eclampsia seizure (子痫; DOC: MgSO4)
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Phenytoin: when used for epilepsy, for what kind?？ mechanism? kinetics? side effects? notes?
when used for epilepsy, for what kind： all partial (simple, complex) and tonic-clonic [1st line], status epilepticus [1st line + DOC for prophylaxis] mechanism: ↑ Na+ channel inactivation; kinetics: zero order side effects: ↑ P450, Stevens-Johnson syn [(SJS) is a milder form of toxic epidermal necrolysis (TEN)], SLE-like syn, teratogenesis, notes: Fosphenytoin for parenteral use
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Diff: Seizure vs. epilepsy types of seizures? what type is medical emergency?
Seizures: synchronized, high-frequency neuronal firing epilepsy: recurrent seizures (febrile seizures are not epilepsy) types of seizures: 1. Partial (focal) seizures: affect only 1 area of the brain 1) simple partial: consciousness intact 2) complex partial: consciousness impaired 2. Generalized seizures: diffuse 1） absence: blank stare 2) myoclonic: quick, repetitive jerks 3) tonic-clonic (grand mal) - alternating stiffening and movement 4) tonic - stiffening 5) atonic: "drop" seizures - fall to floor, commonly mistaken for fainting medical emergency: Status epilepticus - continuous seizures \> 30 min
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Carbamazepine: 叉癫宁, 镇痉宁 when used for epilepsy, for what kind?？ mechanism? kinetics? side effects? 除了用于癫痫，是first line for ?
跟phenytoin的机制、副作用极为类似 when used for epilepsy, for what kind： all partial (simple, complex) and tonic-clonic [1st line] mechanism: ↑ Na+ channel inactivation; side effects: agranulocytosis, aplastic anemia, liver toxicity ↑ P450, Stevens-Johnson syn [(SJS) is a milder form of toxic epidermal necrolysis (TEN)], SIADH, teratogenesis, 除了用于癫痫，是first line for trigeminal neuralgia
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Valproic acid: 丙戊酸 when used for epilepsy, for what kind?？ mechanism? kinetics? side effects? 除了用于癫痫，also used for ?
when used for epilepsy, for what kind： all partial (simple, complex), tonic-clonic [1st line] absence myoclonic mechanism: ↑ Na+ channel inactivation; ↑ [GABA] side effects: teratogenesis (contradicted in pregnancy), rare but fetal hepatotoxicity 除了用于癫痫，also for bipolar disorder
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gabapentin
加巴喷丁(抗焦虑药)
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1st line anti-seizure drug in neonates? mechanism?
phenobarbital ↑ GABAa action
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lamotrigine
拉莫三嗪
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Stevens-Johnson Syn
prodrome of malaise + fever, followed by rapid onset of erythematous/purpuric macules - progress to epidermal necrosis and sloughing
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Barbiturates: 代表药物？ Mechanisms? contradiction? Use? toxicity and Rx?
代表药物: 1. Phenobartial, 2. pentobarbital, 3. thiopental, 硫喷妥钠 4. Secobarbital: 速可巴比妥 Mechanisms: ↑ GABAa action by ↑ duration of CL- channel opening (barbiDUARATes ↑ duration: 与BZDs区别，which ↑ frequency of CL- channel opening) contradiction: porphyria Use: 1. sedative for anxiety 2. seizures 3. insomnia 4. induction of anesthesia (thiopental) toxicity and Rx: 1. respiratory and cardiovascular depression - can be fetal 2. CNS depression (can be exacerbated by EtOH) 3. dependence 4. drug interactions - ↑ P450 overdose treated by supportive (assist respiration and maintain BP)
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Benzodiazepines 苯并二氧杂卓 代表药物？ Mechanisms? 与哪2大类物质类似？各自区别？ Use? toxicity and overdose Rx?
代表药物: "zepam": diazepam, lorazepam, temazepam, oxazepam "zolam": triazolem, midazolam, alprazolam chlordiazepoxide 利眠宁 Mechanism: ↑ GABAa action by ↑ frequency of CL- channel opening, ↓ REM sleep (barbiDUARATes ↑ duration: 与BZDs区别，which ↑ frequency of CL- channel opening) Benzons, barbs and EtOH all bind the GABAa R [a ligand-gate CL- channel] toxicity and Rx: 1) Biggest issue is [dependence]. 2) additive CNS depression with EtOH 3) less risk of respiratory and cardiovascular depression and coma than with barbiturates overdose Rx: Flumazenil 氟马泽尼
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Non-benzodiazepine hypnotics: 代表药物？ Mechanisms? Use? toxicity and overdose Rx? 与BZD比有什么好处？
代表药物: ZZZ (Zolpidem -Ambien, zaleplon扎来普隆, esZopiclone) [All ZZZs put you to sleep] Mechanisms: act via the BZ1 subtype of GABA R; overdose Rx: Flumazenil 氟马泽尼 Use: insomnia 与BZD比有什么好处： ↓ dependence risk than BZDs
154
吸入性麻醉药里MAC的概念？ blood solubility, lipid solubility和麻醉药的影响？
MAC: Minimal Alveolar Concentration (of inhaled anesthetic) required to prevent 50% of subjects from moving in response to noxious stimulus ↓ blood solubility = rapid induction and recovery ↑ lipid solubility = ↑ potency = 1/MAC
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inhaled anesthetics: 代表药物？ mechanism? effects? toxicity, most severe side effect and Rx?
inhaled anesthetics: 代表药物: halothane 氟烷，isoflurane 异氟烷，enflurane, sevoflurane, methoxyflurane, Nitrous oxide mechanism: unknown effects: 1) myocardial depression 2) respiratory depression 3) nausea/emesis 呕吐 4) ↑ cerebral BF (↓ cerebral metabolic demand) toxicity and Rx?: 1) halothane 氟烷: hepatotoxicity 2) methoxyflurane 甲氧氟烷: nephrotoxicity 3) enflurane 安氟醚: proconvulsant 4) NO: expansion of trapped gas in body cavity most severe side effect: malignant hypertherima Rx: dantrolene 硝苯呋海因[肌松药]
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IV anesthetics: 哪几种？代表药物？分别用于什么麻醉？toxicity and Rx?
“B.B. King on OPIOIDS PROPOses FOOLishl" 1. Barbiturates: thiopental 硫喷妥 - high potency, high lipid solubility, rapid enter into brain, rapid clearance (re-distribute into tissue and fat) - ↓ cerebral BF - used for induction, short surgical procedures 2. BZDs: midazolam 咪达唑仑 - MC drug for endoscopy, used adjectively with gaseous anesthetics and narcotics - may cause severe p.o. respiratory depression - ↓ BP - anterograde amnesia overdose Rx: flumazenil 氟马泽尼 3. Ketamine (arylcyclohexylamines 芳基环己胺) - block NMDA R - cardiovascular stimulants - disorientation, hallucination, bad dreams - ↑ cerebral BF 4. Opioids: Morphine, fentynyl - used with other CNS depressants during general anesthesia 5. Propofol 异丙酚 - potentiates GABAa - used for sedation in ICU, rapid induction and short procedures (less p.o. nausea than thiopental)
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Ketamine: 什么药？机制？副作用？对脑血流的作用？
Ketamine (arylcyclohexylamines 芳基环己胺) - block NMDA R - cardiovascular stimulants - disorientation, hallucination, bad dreams - ↑ cerebral BF
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local anesthetics: 主要分类？ 机制？
主要分类： 1. esters: procaine, cocaine, tetracaine 2. amides: lIdocaIne, mepIvacaIne, bupIvacaIne, (amIdes have 2 Is in name) 机制: inhibit Na+ channel
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neuromuscular blockers: 用途？ 主要分类？ 机制？ 如何reverse? complications?
用途: muscle paralysis in surgery or mechanical ventilation - selective for motor (vs. autonomic) nicotinic R 主要分类: 1. depolarizing: succinylcholine 机制: strong Ach R agonist, - produce sustained depolarization and prevent muscle contraction 如何reverse blockade: - Phase I (prolonged deploarization) - NO ANTITODE ; 反之而且还会potentiated by cholinesterase inhibitors - Phase II: antitode - cholineasterase inhibitors complications: - hypercalcemia - hyperkalemia - malignant hyperthermia 2. non-depolariazing: compete with Ach for N type R (curaine, acurium, ronium) tubocurarine 筒箭毒硷 Atracurium 阿曲库铵,卡肌宁 Mivacurium 米伐克龙[肌松药] pancuronium 双哌雄双酯 vecuronium, rocuromnium reserve the blockage: Cholineasterase inhibitors 1) Neostigmine [must + Atropine to prevent Muscarinic effects such as bradycardia!!!!] 2) edrophonium 腾喜龙 [氯化腾喜龙试验诊断重症肌无力]
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Dantrolene: 什么药？ 机制？ use?
硝苯呋海因[肌松药] 机制: prevent Ca2+ release from SR of skeletal muscles use: 1) treat malignant hyperthermia 2) treat neuroleptic malignant syndrome [抗精神病药的恶性综合征:一种罕见的有时是致死的反应,特点为高烧、强直及昏迷]
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PD drugs: 代表药物？ 机制？ 口诀？
"BALSA" Bromocriptine 溴隐亭 Amantadine 金刚烷胺 L-dopa Selegiline 司立吉林(食欲抑制药) Antimuscrinics
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Bromocriptine： 什么药？ 机制？ use?
溴隐亭 （ergot) dopamine agonists - used in PD
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Amantadine: 什么药？ 机制？ use? toxicity
金刚烷胺: ↑ dopamine (may ↑ dopamine release) use: 1) PD 2) antiviral (for Influenza A, rubella) toxicity: ataxia
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selegiline 什么药？ 机制？ use? toxicity
司来吉兰 : PD drug selective MAO-B: [非常有趣的一个机制：preferentially metabolizes dopamine over NE and 5-HT, thereby ↑ the availability of dopamine] - 把DA的竞争者都搞死之后，DA就脱颖而出了 toxicity： may enhance adverse effects of L-dopa
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Benztropine: 什么药？ 机制？ use? toxicity
PD drug- "Park your Benz" antimuscurinic drug: curb excess cholinergic activity - improve tremor and rigidity, but has little effect on bradykinesia
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AD drugs: 机制？ toxicity
MD is GReatT! 1. Memantine: 机制: NMDA R antagonist - prevent excitotoxicity mediated by Ca2+ toxicity: dizziness, confusion, hallucinations 2. AchE inhibitors: 1) Donepezil 多奈哌齐 2) galantamine 加兰他敏(抗胆碱酯酶药) 3) rivastigmine 利伐斯的明 4) tacrine 他克林 toxicity: dizziness, nausea, insomnia
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HD drugs: neurotransmitter changes in HD? Rx机制？
neurotransmitter changes in HD: ↓ GABA, ↓Ach, ↑ Dopamine (记住HD和PD在多巴胺上的变化相反） Rx： 1. inhibitors against monoamine transporter (VMAT) - limit vesicle packaging and release 1） Tetrabenazine 丁苯那嗪 2) reserpine [ri'sә:pin] 利血平 2. Haloperidol 氟派啶醇: DA receptor antagonist
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Sumatriptan: 什么药？ 机制？ use? toxicity
舒马曲坦
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Malignant hyperthemia: Cx? Cause? Rx?
Cx: after inhalation anesthetics and/or succinylcholine (e.g., after surgery): fever + muscle rigidity, BP ↑, HR ↑ 4大特征：tachycardia, HTN, hyperkalemia, myoglobinema Cause: AD trait for susceptibility. abnormal ryanodine receptors - ↑ Ca2+ in cytoplasm - excessive ATP consumption via ATP-dependent Ca2+ retake, this process generates heat + muscle damage (rhabdomyolysis) Rx: dantrolene
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一生中反复感染的病人一般病因？如果出现脑膜炎，最常见病菌？
MAC (membrane attach complex: C5b-C9) deficiency 如果出现脑膜炎，最常见病菌: Neisseria
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viral vs. bacterial meningitis: 1. CSF WBC? 2. Glucose & protein?
1. CSF WBC: viral: WBC \< 500 cells/ul, lymphocytic predominance bac.: WBC \> 1000 cells/ul, neutrophilic predominance 2. Glucose: viral: normal or ↓ bac: much lower, ↓↓ 3. protein? viral: ↑, but \< 150 mg/dL bac: ↑
