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61 Cards in this Set
- Front
- Back
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**What is the definition of dementia?
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-acquired decline in INTELLECTUAL FXN: impairment of memory, language, calculation, spatial oreintation, decision making, judgment and abstract reasoning
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***What is the MCC of dementia?
2nd MCC? |
1. AD
2. cerebrovascular disease generating multiple cerebral infarcts |
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*What is the definition of AD?
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-progressive dementia of unknown etiology involving diffuse atrophy of CEREBRAL CORTEX and amyloid depositions in brain GRAY substance and vessel walls
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**What is the macroscopic appearance of AD?
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-Diffuse atrophy of cerebral cortex - narrowing of gyri and widening of sulci in the frontal, parietal and temporal regions leading to compensatory ventricular enlargment (hydrocephalus ex vacuo)
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What are Neuritic Plaques (Senile Plaques)?
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-focal collections of dilated neuritic processes surrounding an amyloid core
-microglial cells and reactive astrocytosis --> cytokines --> produce damage |
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What are Neurofibrillary Tangles?
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-condensates of neuro-tubules forming a tangled mass of fibrils that displace or encircle the nucleus of a neuron
-contain phospho tau proteins and composed of paired helical filaments |
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What is Amyloid Angiopathy?
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-amyloid in walls of SUBARACHNOID and INTRA-CORTICAL ARTERIES of cortex (but NOT in vessels of WHITE)
-contains Beta peptide |
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What are Granulo - Vacuolar Degenerations?
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-intra neuronal cytoplasmic vacuoles containing argyrophilic granules
-also eosinophilic inclusions in proximal dendrites called HIRANO bodies |
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***Plaques and Tangles are found extensively in the?
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-cerebral cortex
-also in hippocampus, amygdala, and basal nuclei |
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What chromosomes tend to play a part in AD?
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-21: APP gene
-14: Prensilin 1 Gene -1: Prensilin 2 gene -19: Apo E gene (if mutated excess APO E4--> binds to TAU and then produces NF tangles --> AD) |
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What are some of the clinical features of AD?
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-begins w/ impairment of learning and recent memory
-progressive decline from a former level of cognitive fxn, memory loss, disorientation, poor judgment, aphasia, apraxia, alexia |
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What is often the cause of death in pts w/ AD?
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-sepsis from bronchoPNA or UTIs w/ multiresistant bacteria
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What is Pick Disease (Lobar Atrophy)
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-a rare, progressive dementia w/ early onset of behavioral changes, alterations in personality (frontal) and language disturbances (temporal)
-most sporadic, fam liked to TAU mut |
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What is the morphology of Pick disease
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-severe, sym atrophy of frontal and temporal lobes w/ sparing of the posterior 2/3 of superior temporal gyrus
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***What helps to separate Pick from AD?
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-in pick there is the sparing of the posterior 2/3 of superior temporal gyrus as well as rare involvement of parietal or occipital lobes
-there is bilateral atrophy of the caudate nucleus and putamen |
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Vascular Dementia
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-originated by vascular injuries generating various clinical syndromes
-small areas of infarction: granular atrophy and diffuse white matter injury (Binswanger disease, CADASIL) -Strategic infarcts (hippocamp, dorsal thalmus, or frontal cortex |
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***What is the definition of Parkinsons Disease (PD)?
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-ds characterized by tremor and rigidity d/t degeneration of the dopaminergic neurons in the substantia nigra and striatum
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Other the idiopathic which is MC, what are other etiologies of PD?
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-viral encephalitis
-major tranquilizers -heavy metal intox -other toxic agents - MPTP -repetitive trauma (boxers) |
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What are Lewy Bodies?
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-Cytoplasmic inclusions in nerve cells that are spherical, eosinophilic, and peripheral clear halos
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***Pathogenesis of PD?
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-because there is degeneration of the substantia nigra and thus degeneration of dopaminergic neurons there is no inhibition of the ACh excitatory neurons in the basal ganglia thus leading to excess GABA
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What is the clinical presentation of someone w/ PD?
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-disturbance in motor fxn producing fixed espressionless face, slowness of movement (bradykinesia) abnormalities in gait, rigidity, characteristic "pill-rolling" tremor, stooped posture
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What is the definition of Huntington Disease?
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-inherited AD ds characterized clinically by progressive movement disorders and dementia and histologically by degeneration of striatal neurons in the caudate nucleus
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What is the pathogenesis of HD?
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-loss of medium spiny striatal neurons brings dysreg of the BG circuitry that modulates motor output --> loss of striatal inhib output --> INC inhib input to subthalmic nucleus --> prevents it from reg effects on motor activity and leads to Choreo-athetosis
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What is the gross morphology in HD?
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-small brain (sometimes entire cortex)
-atrophy of caudate nucleus and putamen -may be atrophy of globus pallidus -dilated lat and 3rd cerbral ventricles |
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What is the microscopy of HD?
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-severe loss of striatal neurons in the caudate nucleus (putamen is less involved)
-fibrillar gliosis |
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What are the clinical features of HD?
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-Motor symptoms = chorea (involuntary, hyperkinetic, jerky movements, sometimes dystonic)
-Parkinsonsims: bradykinesia and rigidity develop later -Cog impairment - even later |
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What are Spinocerebellar Degenerations?
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-group of genetically distinct ds characterized by signs and sx referable to cerebellum (progressive ataxia), brainstem, SC, and periph nerves
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What is the pathogenesis of Spinocerebellar Degenerations?
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-neuronal loss from the affected areas
-2ndary degen of WHITE matter tracts |
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Friedrich Ataxia
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-auto recessive progressive illness, starts in the 1st decade of life w/ gait ataxia, followed by hand clumsiness and dysarthria
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Morphology of Friedrich Ataxia
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-loss of axons and gliosis in:
1. Post columns of SC (Clarke column) 2. Brainstem, Cerebellum, and Betz cells of the motor cortex 3. large primary sensory neurons of the DRG -also heart is enlarged w/ multifocal destruction of myocardial fibers |
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Clinical Aspects of Friedrich Ataxia
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-DTRs absent, Extensor Plantar reflex is present (Babinski)
-impaired jt position and vib senses -loss of pain and temp -kyphoscolios and wheelchair w/in 5 yrs -Death from pulmonary infx or cardiac disease |
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What is Ataxia-Telangiectasia disorder?
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-auto - recessive, begins early child
-11q22-23 locus that encodes a ATM protein -Carrier status 1% = INC risk of breast ca -cells in pts have INC sensitivity to X-ray induced chromo abnmLities |
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What is the morphology of Ataxia-Telangiectasia disorder?
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-Neuronal degen (Purkinje) and gliosis
-telangiectasias in conjunctiva and skin -hypoplastic lymph nodes and thymus -hypoplastic gonads |
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What are Capsule cells or Amphicytes?
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-cells that present w/ bizzarre enlargement of cell nucleus 2-5 x nL size in DRG, and periph nerves, endothelial cells and pituicytes
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What is affected in Ataxia-Telangiectasia disorder?
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-LMNs in ANTERIOR HORNS of SC and certain CN motor nuclei (5, 7, 9, 12) NOT EYES
-UMNs (Betz cells) in motor cortex - |
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Clinical presentations of Ataxia-Telangiectasia disorder?
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-muscular atrophy, weakness, and fasiculations (loss of LMNs)
-paresis, hyper-reflexia, spasticity, and Babinski + |
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What is Amyotrophic Lateral Sclerosis?
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-neuronal muscle atrophy and hyper-reflexia d/t loss of LMNs in the ANTERIOR HORNS of the SC and UMNs that project in CORTICO-SPINAL TRACTS
-50s, M, AD inheritance |
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What is the morphology of Amyotrophic Lateral Sclerosis?
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-atrophic SC, thin anterior roots
-sever cases -> atrophic PRECENTRAL GYRUS -neurogenic atrophy of Sk Muscles -Degen of myelin in Cortico Spinal |
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What are Bunina bodies?
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-in ALS they are intra cytoplasmic inclusion w/ a PAS - positive content remnant of autophagic vacuoles
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What is Spinal muscular atrophy (SMA) aka Werdnig - Hoffman ds?
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-wasting of Sk muscle caused by progressive degeneration of anterior horns of the SC in KIDS (diff from ALS)
-more severe in legs than in arms |
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What is the DSMIII R Definition of Dementia?
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-memory loss w/ other cognitive problems
-most exclusive cortex: occipital -Frontal and temporal areas common -worst effects = medial temporal lobe* |
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*What is the #1 risk factor for AD?
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-AGE
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What are the top 3 types of dementia?
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-Alzheimer = 70% (50% pure)
-Lewy Body (parkinson like) dementia is 25% -Vascular dementia is 25% |
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What is Ribot's law?
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-what we gain last in our memory pathway is the first to be lost w/ any ictus
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What is seen in early AD?
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-MC age >60
-memory impairment -behaviors inappropriate to society |
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What is seen in the middle course of AD?
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-aphasia language dysfxn
-perceptual difficulties = Agnosia -Motor dysfxn = Ataxia |
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What is seen in the late course of AD?
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-gait changes
-seizure -death by secondary cause |
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***What are unlikely signs/sx of AD?
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-sudden onset
-early seizures/gait disturbances -Focal findings (hemipareisis) |
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What would you see for imaging vascular dementia?
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-thalamic lacunes and tons of cortical infrarcts
-Binswanger disease is bad? (why noteservice is this here?) |
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AD vs Vascular Dementia
1. Onset 2. Neuro Exam 3. Gait 4. Executive fxn 5. Neuroimaging |
1. Gradual vs Sudden
2. nL vs Neuro deficits 3. nL vs disturbed early 4. Impaired later vs marked and early 5. nL or hippocamp vs infarct or white matter lesions |
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AD vs Dementia - Lewy Body (DLB)
1. Parkinsonism 2. Psych sx 3. Visual hallucinations 4. Neuroleptics response |
1. Less common vs More common
2. Less likely vs more likely and early 3. Less in early vs Common early 4. Behavioural response vs extrapyramidal side effects |
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What are some of the characteristics of DLB (Dementia - Lewy Body)
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-frequently see small people described as kids (visual hallucination)
-daytime sleep disorder -at night see Parkinson like REM sleep disorder w/ dream thrashing early w/ paralysis -sensitive to neuroleptics -cognitive impairment |
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What is the treatment for AD?
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-Best is combo of Cholinesterase inhib and Namenda (glutamate excitatory transmitter blocker)
1. blocks ACh breakdown (Aracept, Galantamine) 2. Not a complete blocker, but slows down excitatory stimulation that would nL cause death |
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What are characteristics of a Physiological Tremor?
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-action tremor but can involve the head
-dec by ETOH, B-blocker -Inc by Epi, adrenergic stimulants |
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What are characteristics of a Cerebellar Tremor?
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-intention tremor, ataxia, "Rubral" = coarse, slow tremor present at rest, intention and at posture
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***What are characteristics of a Parkinson Tremor?
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-resting tremor that disappears w/ action
-Intermittent, inc w/ stress, and dec by L-Dopa -rhythmic "pill rolling" of thumb and index finger -postural w/ hands outstretched |
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***What are characteristics of a Essential Tremor?
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-asymmetrical action induced
-head, arms, neck, jaw, voice -Inc by anxiety, B-adrenergics, Xanthines -Dec by ETOH, Propanol, Primodone -Disabling-> impairs fine motor skills |
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***What are Tx for Essential Tremor?
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1. Propanol
2. EtOH 3. Deep Brain Stimulation (DBS) of Thalmus = Tx of choice 4. Gabapentin, Pregabalin, Benzodiazepines |
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Primary Torsion Dystonia (Dystonia musculorum deformans)
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-most serious for childhood/adolescent onset
-focal or regional -Spine twisted b/c of tonic mm spasms in paravertebral mm ***important to R/O Wilson's |
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What are Tx for Primary Torsion Dystonia (Dystonia musculorum deformans)?
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-DBS of globus pallidus
-anti-cholinergic -anti-dopaminergic -Botulunum toxin |
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What are inherited disease w/ Chorea (dance like excessive movement)?
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-Huntington's
-Benign Hereditary Chorea -Neuroacanthocytosis |
