CNS Flashcards

Question

Neurocutaneous Syndome (Sturge-Weber)

Answer 1

* ***Cong vascular malformation*** affecting head, face, brain * **_Clinical presentation:_** * @ birth w/port-wine nevus (reddish-brown/pink dicoloration of face) * Follows distrubution of CN V (trigeminal)-^Sturge-weber * **_{^{Motor & sensory}}**

Answer 2

* **_"Cortical Tubers"_** * Autosomal dominant - **^{w/mental retardation & seizures}** * **Starts early in life** * Pts can present with ***Cardiac rhabdomyomas*** * **_{^{Fatty foci in myocardium}}** * Facial angiofibromas "Butterfly pattern" * Multisystemic=Non-malignant tumors * **_{^{Brain, kidneys, heart, eyes, lungs, & Skin}}**

Answer 3

* **_Cause:_** Fragile portion of brain damaged **_{^{(2nd tri abortions)}}** * Common in pre-term infants (before 35 weeks) * **_{^{Associated w/hyaline membrane & necrotizing renal issues}}** * Germinal matrix _highly cellular & highly vascularized_ * **_{^{Source of neurons & glial cells migrate out to other regions of brain}}** * Most active during ***8-28 weeks gestation*** * **_Morphology: _**Intraventricular hemorrhage (IVH) * **_{^{Origin subpendymal region ("germinal matrix")}}** * Baby presents w/seizure = _obstructive hydrocephalus_ * ^{_{Increased cranial circumferance}}

Answer 4

* **_Disorder of movement-Causes:_** * ***Grey matter necrosis***-Hypoxic/ischemic injury **_{^{(Red neuron)}}** * **_{^{Makes holes/cystic cavity in brain}}** * **_Symptoms _{^{(motor problems-paralysis)}}:_** * Spastic diplengia **_{^{(affects symmetrical parts of body)}}** * Spastic hemiplegia **_{^{(affects one side of body)}}** * Presents in infancy to childhood * ***Non-progressive*** due to developing brain plasticity

Answer 5

* Inflammation of ***Pia/Arachnoid*** * **_Acute bacterial/pyogenic:_** * _{^{Low glucose}} * _{^{Pressure increase}} * _{^{Increase in neutorphils}} * **_{^{+ Culture}}** * **_Acute viral:_** * _{^{Glucose Normal}} * _{^{Slight increase in pressure}} * _{^{Slight increase in lymphocytes}} * _{^{- Culture}}

Answer 6

* **_Neonates_**: ***Ecoli***, Group B strep **_{^{(Birthing process)}}** * **_Babies:_** H. influ **_{^{(non-vacinnated)}}** & Strep pneumo * **_Epidemics amongst adults:_** Neisseria men * **_{^{Pathogens enter through Nose}}** * **_{^{Associated w/"Waterhouse Fredrichson syndrome"-Bilateral adrenal insuff/DIC}}** * **Septicemia can present w/_purpuric rash does NOT lose colo_r when pressed** * **_Infection following surgery_**: Staph aureus **__{^{(IV drug user)}}_** * **_Morphology:_** Opaque meninges **_^(exudates)** * **_Histo:_** Neutrophils in subarachnoid space * Acute hydrocephalus _{^{(high pressure)}}-_{^{Headache & Papilledema (optic swell)}}

Answer 7

* **_General properties:_** * Perivascular inflammatory infiltrates (lymphocytes) * _{^{Stain pink/blue in histo}} * Microglial nodules **__{^{(microglial cells surrounding necrotizing tissue)}}_** * Inclusion bodies **__{^{(viral replication in bacterium or host cell)}}_** * **_Progressive multifocal leukocephalopathy:_** * JC virus will stain brown-***Polyomavirus*** **__{^{similar to BK-SV40}}_** * ***Glial cells/white matter*** progressive inflammation * Similar to ***MS*** BUT more rapid * **_Herpes simplex_**-Attacks ***Temporal Lobe***

Answer 8

* _Toxoplasma-"***Cat Stratch Disease***"_ * Retinits __{^{(cause blindness)}}_ in new born * **_MRI:_** Calcification in brain * Lead to ***Brain abscess*** * **_Fungal meningitis:_** * **Cryptococcus**-_{^{present in HIV or Lymphandenopathy pts (cancer of lymph)}} * Use ***indian ink stain*** * _Found in **Virchow-Robin spaces:**_ * Perivascular, fluid filled canals surround perforating arteries/veins in parenchyma **_{^{(inside brain)}}** * **Regulate CNS movement & Drainage**

Answer 9

* "Prion Disease" * **_Transmission:_** * Blood/Sputum * Fatal familial insomnia **__{^{(mutation PrPsc gene)}}_** * Kuru (humans) * Eating infected food or ***cannibalism*** * **_Clinical:_** _^{Changes in memory & behavior w/rapid dementia}_ * ***Startle myoclonus*** **_{^{(brief muscle spasm)}}** * Average ***survival 7 months*** from onset * PrPc _{^{(Alpha-helices)}} & ***PrPsc*** _{^{(beta sheets)}} **_{^{differ in 3D confirmation}}** * **_{^{PrPsc goes back to PrPc to make more PrPsc}}** * **_Histo:_** Amyloid rich ***kuru plaques*** _^{_{(intra-vacoule)}}_

Answer 10

* Sporadic chronic relapsing & remitting diease * **_Genetics-_**HLA DR2 * **_Morphology-_**Plaque in CNS white matter **_{^{(Brain/spinal cord)}}** * **Plaques = _Astrogliosis_** * **_Autoimmune_**-caused by CD4 Tcell mediated injury to myelin sheath **_{^{(oligodentrocytes)}}** * **_CSF-_**High lympocytes w/Oligoclonal IgG bands * **_Clnical:_** * Unilateral visual impairment __{^{(optic neuritis)}}_ few days * ***Brainstem***-**_{^{CN signs, Ataxia, Nystagmus, Slurred speeach}}** * ***Spinal cord-*****_{^{Motor/sensory impairment (spasticity)}}** * Difficulty w/voluntary control of bladder

Answer 11

* Acute ascending polyneuritis (PNS) * Segmental myelin loss of PNS-***Schwann cells*** * Spontaneous or due to ***Viral/Mycoplasma*** infection * **_Symptoms:_** * Rapid ascending motor weakness ***over weeks*** * Lead to respiratory failure-death * **_{^{Begins in feet & hands migrates toward trunk}}**

Answer 12

* Changes seen in ***hippocampus (CA1 region)*** * **_{^{Imaging will show shrinkage w/Hydrocephalus ex vacuo}}** * **_Symptoms _{^{(cortex/gray matter degeneration)}}:_** * Impaired memory, loss motor skills, incontenence, & immobility/mute ***(late)*** * **_Genetics-_**Chrom 19 Tau protein **^(ApoE)** & **21 _{^{(increase in APP)}}** * **_Generation of Amyliod-B_** * ****_{^{2 sequential extracelluar cleavages of APP by BACE/A-secretase}} * _{^{Alpha-secretase prevents generation of Amyloid-B (cannot be brokendown)}} * **_Histo:_** ***Neuritic Plaque stain*** * Tau protein (Brown)-**_{^{proteins that stabalize microtubules}}** * Beta-Amyloid-_{^{E4 upregulated}} (Red Dot) * Neufibrillary tangles **_{^{(Hyperphospho Tau)-}}** * **Bielschowsky silver stain**

Answer 13

* Fronto***TEMPORAL*** lobar degeneration * Early behavior & language symptoms-Dementia * Spares parietal/occipital lobes * _{^{Rare neurodegenerative disease}} * Progresses to ***destruction of nerve cells*** * **_Histo:_** Round Tau protein aggregates

Answer 14

* Paralysis agitans * Autosomal dominant-_{^{mutations in alpha-synuclein gene}} * Autosomal recessive-_{^{mutations in ubiquitin-protein ligase (parkin gene)}} * Loss of doperinergic **_{^{(catecolaminergic)}}**neurons in ***pars-compacta of substantia nigra*** * **_Symptoms (damage basal ganglia)-_** * Shuffling gait * Cogwheel-rigidity of limbs * Pill-rolling tremor @ rest **_{^{(absent w/movement)}}** * **_Histo:_** ***Lewy body*** in abnormal neuron **__{^{(Alpha sy)}}_** * ***Pigment melanine***

Answer 15

* **_Chorea-_**Unopposed muscle contrations * **_Trinucleotide repeat disorders_**-caused by length of repeated section of gene exceeding normal range * **_HTT gene_**-p Chromosome 4/13 * 3 DNA bases-***CAG repeated several times* _^(Glutamine)** * Early HT affects ***Extrapyramidal motor*** * Late ***atrophy of caudate nuclei*** w/enlargement of frontal horns of lat vet **_{^{(hydrocephalus ex-vacuo)}}** * _{^{Degeneration of GABA neurons (caudate nucleus)}} * **_Psychiatric Symptoms-_** * _{^{Irraitability, Apathy, Aniety, Depressed mood (suicide)}}

Answer 16

* **_Motor neuron disease_** **^{(upper & lower of pyramidal system)}** * **_{^{Overall nerve atrophy}}** * _Rapidly progressive weakness w/***muscle atrophy***-_ * ***Gliosis in motor cortex***, pallor of lateral cortico w/***neuronal loss in ant horns*** (motor) * Fasiculations _{^{(muscle twitching)}} * Muscle spasticity _{^{(impaired muscle coordination)}} * Diffculty speaking _^(dysathria) * Diffculty swallowing _^(Dysphagia) * Difficulty breathing _^(dyspnea)

CNS Flashcards

(40 cards)