Neuro Five Flashcards
(118 cards)
1
Q
MS signs
A
Motor and sensory from spinal cord
Ataxia and nystagmus
Frequency of relapses decrease during course but steady neurologic deterioration
2
Q
Central pontine myeloysis
A
Symmetric demyelination
Myelin loss no inflammation
3
Q
Parkinson histo
A
Loss of pigmentation substantia nigra
Lewy body
4
Q
ALS time
A
Fifty or older
SODone is on chromosome twenty one get gain of function alanine to valine
5
Q
Btwelve defiency
A
Anemia
Or neurologic which presents a few weeks as numbness, tingling, slight ataxia in LE
Rapid progression:spastic weakness of LE
Complete paraplegia
Replacement can improve unless complete paraplegia has developed
6
Q
Histo Btwelve defiency
A
Swelling of myelin layers make vacuoles
Axons of ascending and descending tracts degenerate
SuBACute combined degeneration of SPC spinal cord
Axons of both are degenerated
7
Q
Corneal stroma
A
No blood vessels or lymphatics
8
Q
Why get corneal vasculrization
A
Chronic corneal edema
Inflammation scarring
9
Q
Risk factor glaucoma
A
DM
10
Q
Cytotoxic edema can lead to
A
Hernation
11
Q
Vasogenic edema follows what
A
Ischemic injury
12
Q
Thrombosis is mainly due to
A
Atherosclerosis
Rupture , ulceration or erosion of plaque exposes blood to thrombogenic substance get clot
13
Q
Tia transient ischemic attack
A
One hour smal infarct One day function lost Neurologic emergency Fifteen perfect have a stroke causes persistent deficits within three months, Half within first forty eight hours
MOA embolus temporarily occluded then dissolves , get thrombosis formation or vasoaspasm
14
Q
Hypertension and brain
A
Lacune
Slit hemorrhages
Hypertensive encephalopathy
-deep brain
Vascular multi infarct dementia-dementia gait and pseudobulbar signs
Binswanger disease-large area of subcortical white matter with myelin and axon loss
Charcot Bouchard-microaneurysms associate with chronic HTN
15
Q
Bacterial meningitis complication
A
Seizure, encephalitis, hearing loss, blindness, paralysis
Fulminant with meningococcemia, rash
Adrenal hemorrhage->death
Waterhouse friderichsen syndrome
16
Q
Bacterial meningitis
A
Pressure over 10 Cloudy turbid Neutrophils over 100 Lymph over 5 ml Glucose less than 50 Protein 50-1000
17
Q
Virus meningitis
A
Clear colorless, no neutrophils, lymph mono 0-5
Glucose 50-80
Protein 15-45
18
Q
Neisseria meningitidis
Epidemiology
A
Colonized the oropharynx and rhinopharynx of asymptomatic carriers and spreads by direct contact with respiratoy secretions
Higher in crowded populations like dorms prisons
19
Q
N meningitidis clinical manifestations
A
Rapidly progressive septicemia with fever, hypotension DIC, petechial and purpuric lesions
Purpura fulimans:hemorrhagic skin lesions which progress to gangrene;occurs in distal portions of limbs
Hemorrhagic infarction of adrenal glands
20
Q
Chronic meningitis symptoms
A
Fever, headache, lethargy, confusion, nausea, vomiting, stiff neck
21
Q
CSF chronic meningitis
A
Elevated protein concentration, predominantly lymphocytic pleocytosis, sometimes a low glucose level
TB, neuroborreliosis, neurosyphilis
22
Q
Diagnose chronic meningitis
A
Diagnosis is made if symptoms and CSF persist or progress for a period of at least 4 weeks
23
Q
Treponema pallidus
A
Neurosyphilis
Meningovascular neurosyphilis
Paretic neurosyphilis
Tabes dorsalis
24
Q
Neurosyphilis
A
Tertiary stage: only about 10% of untreated PTs develop
25
Meningovascular neurosyphilis
Chronic meningitis involving base of the brain (variable convexities and spinal leptomeninges)
26
Paretic neurosyphilis
Insidious but progressive mental deficits associated with mood alterations (delusions of gradeur0 that terminate in severe dementia (general paresis of the insane ) perivascular iron deposits
Granular ependymomas:proliferation of subependymal glia under damaged ependymal linings associated with hydrocephalus
27
Tables dorsalis
Damage to the sensory nerves (loss of myelin and axons) in the dorsal roots; impaired joint position sense and resultant ataxia (locomotor ataxia);loss of pain sensation (Charcot joints) lightening pains;absence of DTR
28
Herpes
Chickenpox cutaneous
Latent phase:in sensory neurons of dorsal root or trigeminal ganglia
Reactivation(shingles) painful ) vesicular skin eruption limited to a single or limited dermatome
Persistent postherpetic neuralgia syndrome:perstent pain as well as painful sensation following nonpainful stimuli
29
Rabies
Incubation period 1-3 months depends on distance of wound to the brain
Infection ascends along peripheral nerves fromt he wound site
Nonspecific symptoms (malaise HA, fever) with local paresthesia around wound (diagnostic)
Extraordinary CNS excitability;violent motor responses progressing to convulsions
Flaccid paralysis , respiratoy center failure
Hydrophobia dopamine at the mouth due to contracture of pharyngeal muscles that produce an aversion to swallowing
30
CJD
90% prion seventh decade
Iatrogenic transmission:corneal transplant, brain implant or electrodes and HGH
Rapidly progressice dementia with startle myoclonus 7 months
So rapid , little if any gross evidence of brain atrophy (spongiform changes microscopic)
31
VCJD
UK young adults
Behavioral changes early in DZ
Slower progression
Exposure to bovine spongiform encephalopathy
Kuru plaque : extracellular deposits of aggregated ABN protein +Congo red and +PAS
Cerebellum of VCJD
32
FFI fatal familial insomnia
Sleep disturbances initially less than three year survival
Aspartate substitution at 129
Ataxia, autonomic disturbances, stupor and coma
33
Most cases of AD are
Sporadic@
5-10% familial
Pathological examination necessary for definitive diagnosis
34
Treat parkinson
L DOPA replaced with Carbidopa
35
Huntington
4p16.3
CAG normal 10-36 get adult onset with 40-50
Juvenile over 60
36
ALS early sign
Drop objects muscle strength and bulk dismissed
37
Fasciculations ALS
Involuntary muscle contraction
38
Respiratoy infections ALS
Respiratoy muscle involved
39
Progressive Muscl atrophy
LMN
40
Progressive bulbar palsy
Lower brainstem and cranial motor nuclei early and prognosis rapidly
2 year dead
41
ALS
2 year dead
42
Pilocytic astrocytoma
First two decades of life
Cerebral hemispheres or cerebellumNF1 predisposes, espicially optic tumors
Functional loss of neurofibromin in tumor
Well circumscribed often cystic with a ural nodule
Radiology discrete, contrast enhancement, lack or surrounding edema, cyst
43
Pilocytic astrocytoma
Biphasic pattern:loose glial with cystic changes and dense piloted. Tissue
Hair like cells with long bipolar processes
Rosenthal fibers
Eosinophilia granular bodies (EGBs)
Extension into subarachnoid space is common (not sign of aggressiveness) espicially with optic nerve lesions
GFAP positive
44
Glioblastoma (IV/IV)
Most common primary brain neoplasm
Primary vs secondary neoplasms
Primary-later in like (older patient, no precursor lesion (EGFR and PTEN)
Secondary-preceded by lower grade lesions (TP53), IDH1 (R132 H mutation has better prognosis) and IDH2 typically younger
Throughout brain
Contrast ring enhancing hypodense central necrosisi
Cells follow fiber tracts and extend well beyond imaging
45
Glioblastoma histology
Necrosis : serpentine pattern of necrosis in hypercellular areas
Pseudo-palisading of cells around necrosis
Vascular/endothelial proliferation
- VEGF produced by malignant astrocytes in response to hypoxia is responsible
- tufts of cells pile up and bulge into vascular lumen
- glomeruloid body: MKD proliferation produces a ball like structure
46
Oligodendroglioma
10-15% of all gliomas, primarily adults
Primary cerebral hemispheres
Calcification usually restricted to the cortex, curving around or gyriform distribution
Histology:perineuronal satellitosis, perivascular aggregation and subpial accumulation of tumor cells
Perinuclear halos fried eggs
Delicate anastomsing capillaries chicken wire
Most are well differentiate grade II, IV
IDH1 and IDH2 isocitrates dehydrogenase genes, most common mutation in oligodendrocytes (90%) favorable prognosis
1p19q loss favorable prognosis
Anaplastic oligo II/Iv-vascular hypertrophy and necrosis, often retains geometric vascularity often retains geometric vascularity
Increased grease with increased N;C, mitosis and cellularity
Often these features found in nodules located within a IIIV tumor
Poor prognosis similar to glioblastoma
47
Four molecular groups
Malignant embryonal tumor in children (20% of brain tumors in kids)
- 4 molecular GRPS
- WNT: older kids, monosomy CHR 6, B catenin, 5 YR survival 90%
- SHH: sonic hedgehog, infants-young adults, nodular desmoplastic morph, MYCN AMP, prognosis intermediate between WNT and GRPS 3 and4)
Group 3:MYC AMP 17(i17Q) infants, kids-classic or LG cell, worst prognosis
Group 4 17Q, NO MYC or MYCN, intermediate, 17Q poor prognosis, classic or LG cell morphology
48
Medulloblastoma
Malignant embryonal tumor in kids (20% of brain tumors in kids)0
Cerebellum, midline, occlude CSF flow-sheets of anaplastic cells, abundant mitosis, homer wright rosettes, drop metz:disseminate thru CSF to Cauda equina
- exquisitely radiosensitive: total excision pls XRT-?75% 5 year survival
- CNS supratentorial PNET (primitive neuroectodermal tumor)
49
Paraneoplastic syndromes
Subacute cerebellar degeneration
Limbic encephalitis
Eye movement DOs
Lambert Eaton myasthenic syndrome
50
Subacute cerebellar degeneration
PCA-1 antibody anti yo
Destruction of purkinje cells, gliosis and milked chronic inflammatory infiltrate
Women with ovarian , uterine, or breast carcinoma
51
Limbic encephalitis
ANNA-1 antibody:neuronal nuclei (CNS and PNS):small cell carcinoma of lung
NMDA receptor”hippocampal neurons:ovarian teratomas
VGKC-complex antibody:voltage gated K channel, periph neuropathy, SX in Limbic encephalitis often appear before any malignancy is suspected
52
Eye movement DO
Opsoclonus, neuroblastoma in kids
53
Lambert Eaton myasthenic syndrome
Voltage gated calcium channels
54
Treat paraneoplastic syndromes
Immunotherapy to remove circulating ABs remove tumor
55
NF1 associated lesion
Pheochromocytoma
56
Features NF2
Neurofibromatosis cafe au late optic nerve glioma
57
Von hipped lindau associated lesion
Pheochromocytoma , paraganglia
58
Other features of Von hipped lindau
Renal cell carcinoma
Hemangioblastomas
Pancreatic endocrine neoplasm
59
Cowden gene
PTEN AD 10q23
60
Cowden
Lipid phosphatse/benign follicular appendage tumors (trichilemmonas):internal adenocarcinoma (breast or endometrial)
61
Tuberous sclerosis
TSC1/hamartin
| TSC2/tuberin
62
What do TSC1 and TSC2 do
Work together in a complex that negatively regulates mTOR
In absence angiofibromas and mental retardation
63
NF1 gene
17q11 neurofibromin
64
NF2 gene
Merlin
65
What does Merlin do
Integrates cytoskeletal signaling
66
VHL
AD 3p25.3 tumor suppressor
Involved in regulating expression of erythropoietin ->polycythemia
Hemangioblastomas of the CNS (cerebellum and retina)
Cysts of pancreas
Renal cell carcinoma
Pheochromocytoma
67
NF1
```
Common AD
Neurofibromas
Optic nerve gliomas
Lisch nodules
Cafe au last
HYPERPIGMENTED cutaneous nodules
```
68
NF2
Less common
Bilateral schwannomas
Increased meningiomas and ependymomas
69
Rosenthal fibers
Pilocytic astrocytoma thick elongated eosinophilia irregular structures that occur with astrocytes processes
Ab crystalin and ubiquitin
70
What is pilocytic astrocytoma
Low grade non infiltrating neoplasm in kids
71
Alexander disease
Leukodystrophy from GFAP
| Abundant rosenthal fibers
72
Corpora amlacea
Common in old people from degeneration of astrocytes
CAG polymers with HSP and ubiquitin
Wherever there are atrocities end processes
PAS
Basophils
73
Alford bodies
Myoclonic epilepsy
74
Microglia
CR3 and 68
| Get long nuclei gamma is neurosyphilis granulomas
75
What is global cerebral ischemia
Generalized reduction of cerebral perfusion (cardiac arrest, shock, and severe hypotension)
76
Respirator brain
Brain dead left on ventilator laminar necrosis and gradual liquefication
77
Linear parasagittal infarction
Sickle cerebral convexity a few centimeters lateral to interhemispheric fissure
Between ACA MCA
Hypoxic/hypoglycemic stress
After hypotensive events
78
Bone marrow embolism after trauma
Widespread white matter hemorrhages characteristic of embolization of non emarrow after trauma
79
What may cause luminal narrowing
Infectious vasculitis
Polyarteritis nodosa
Primary angiitis
80
What is contraindicated in hemorrhagic infarcts
Thrombolytic therapy
81
In hemorrhagic and non hemorrhagic infarct, __ and __ mater are unaffected and do not contribute to the healing process
Pia arachnoid
82
Where are Charcot Bouchard most common
Small vessels within the basal ganglia , saccular berry aneurysms occur in larger intracranial vessels in the subarachnoid space
83
CAA histology
No fibrosis
Only the leptomeningeal and cerebral cortical arterioles
84
Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy
AS NOTCH3 misfolds and in vascular smooth muscle
Recurrent strokes and dementia
35 changes and infarcts 40
Loss of smooth muscle cells
Infarct or hemorrhage
Hematoma
85
Pathogenesis of saccular aneurysm
Absence of smooth muscle or intimal elastic lamina at birth
Smooth muscle defects are congenital and aneurysms are subsequently acquired
86
Rupture of berry and chronis adhesice arachnoiditis
After rupture a non communicating hydrocephalus results from organization of the subarachnoid hemorrhage occluding foramina of luschka and magendie
87
AVM
Cerebral hemispheres young adult
Vessels in subarachnoid or brain
Tangled vessels that shunt-bypass capillary bet
Can be respected
Males
Seizures, intracranial hemorrhage, subarachnoid hemorrhage, MCA common sot
CHF due to shunt effects
88
How do infectious agents spread
Hematogenous
89
Adolescent meningitis
N meningitidis
90
Old people meningitis
Strep p and listeria monocytogenes
91
Immune suppressed meningitis
Klebsiella
92
Acute aseptic meningitis
Enterovirus no bacteria but have meningitis
Self limited asymptomatic
93
Bacterial meningitis
EXUDATE within leptomeninges pia and arachnoid
| Neutrophils
94
Bacterial meningitis adhesive arachnoiditis
Capsular polysaccharide of the microbe can make gelatinous exudate that promotes arachnoid fibrosis from SCARRING
95
Abscesses
Focus necrosis of brain tissue with inflammation that is usually caused by bacteria
96
What do abscess look like
Ring enhancement
Bacterial organisms granulation tissue with fibrosis is typical healing inflammatory response reaction to a cerebral abscess LUNG INFECTION
97
Most common bacteria in brain abscesses of non immunosuppressed patients
Strep and staph
98
Clinical presentation Brian abscesses
Progressive focal neuro deficit
99
Negri bodies
Found in pyramidal neurons of the hippocampus and purkinje cells of cerebellum
Rabies
100
PML
Demyelination is its principle pathological effect
Immune compromised
JC polymoavirus
Glassy amphophilic viral inclusions
101
Subacute sclerosing panencephalitis
Kids or young adults months or years after an initial infection by measles virus
102
Toxoplasmosis
AIDS CNS abscesses
Protozoa
103
Prions protective
Heterozygosity at 129 protective
104
CJD
Rapidly progressive dementia
105
Gerstmann straussler scheinker syndrome
Progressive cerebellar ataxia
106
Presentation CJD
Subtle changes in memory and behavior followers by rapid dementia and startle myoclonus
107
VCJD
129 MET MET
Heterozygous protective
108
CJD morphology
Congo red PAS positive
Kuru plaques
Status spongiosus cyst like
109
FFI
Sleep disturbances initially
PRNP gene
3 years
Get ataxia, autonomic disturbances, stupor, and coma
NO spongiform pathology
-neuronal loss and reactive gliosis int he nation rventral and dorsomedial nucleus of the thalamus
110
FFI vs CJD
Asparagins at 178 and methionine at 129–FFI
Asparagine at 178 and valine at 129 CJD
111
MELAS
Lactic acidosis
Stroke like reversible deficits do not correspond with discrete vascular areas
No cytochrome c oxidase
MTTL1
112
MERRF
Maternal
| Myoclonus myopathy ragged red fibers in muscles and ataxia
113
Hypoglycemia
Initially injury to large pyramidal neurons
114
Hyperglycemia
Severe dehydration that leads to confusion, stupor and coma
| Rehydration must be slow of get edema
115
Methanol
Kidd retinal ganglion cells becomes formic acid and formaldehyde
Formate
116
CO
CA1 purkinje cells
Inhibits cytochrome C in os phos
Lays II and sommers secto
117
Ethanol
Atrophy and loss of granule cells in anterior vermis of the cerebellum loss of purkinje cells and proliferation of adjacent astrocytes
118
Radiation
Coagulation necrosis, sclerosis ,
