basal Ganglia Flashcards

1
Q

بسم الله الرحمن الرحيم
________________
Neurodegnrative diseas mean ?

A

Disorders charcterized by dgenrration of group on neurons related by function not location
May be assoicated with protein accumulation
__________________
Classified by:
Clinical presnetation
Accumulated proten
Pathological process
genetic abnormatilits

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2
Q

Neurodeneratic diesaes affecting basal ganglia results in ……. diorders as ?

A

Motor disorders as?
Parkinson’s disease
Hunitinton chorea

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3
Q

Parkinson disease descibe

A

The most common motor neurodegenrative disease
Casuing Hypokinetic motor movements
due to loss of dopaminergic neruons in substantis nigra
Pathology: Loss of doapmin in neurons and accumulation of intranuclear A-synuclein

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4
Q

Parkinson’s disease causes ?

A

Idiopathic 85 %
____________-
2VTE
viral encepalitis
vascular - trauma
Environmental toxin
Drug induced
(antipsychotics - anti emetics )
___________sporadic both autosomal dominant and recessive exist
___________defect in a synculein casues automsomal dominat PD

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5
Q

Clinical Features of Parkinsonism :

A

3S 2D Progressive movement disorder along 10 years casuing slowing of movements severe!
Tremors static + Shuffling gait with small steps
_____________
demetia
death
_______________
Parkisnosim syndrome due to loss of dopaminergic neurons is SN due to drug or toxin

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6
Q

Grross SN In Parkinson

A

pale due to degentation of neurons
loss of pigment
Repleced by gliosis

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7
Q

Pathognomoinc for Parkinsoism

A

Lewy Bodies
Intracytolasmic Eosinophilic Inclusions formed of dense core of a-synuclein surrounded by a halo
may be single or multiple

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8
Q

Huntingtion iseas HD describe

A

Progressive neurodenertaive disease 30-40ys casuing death along 15 years from onset
Hyperkinetic Movements diorders caused
___________________Choreic Movements
Cognitive and Psyichatric disorders
_________________

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9
Q

Pathogenesis of HD ?

A

CAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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9
Q

Pathogenesis of HD ?

A

CAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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9
Q

Pathogenesis of HD ?

A

CAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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9
Q

Pathogenesis of HD ?

A

GCAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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9
Q

Pathogenesis of HD ?

A

CAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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10
Q

Pathogenesis of HD ?

A

CAG trinuclotide repeat expansion > 35 times
________
abnormal huntigtin protein accumulation in cytoplam + nuclei + axons of neurons casuing it to degnerate
__________
chromosme 4p

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11
Q

gross of HD

A

Shrunken brain due to atrophy of Caudate + putamen so dilated ventricles
Atrophy of frontal parietal and entire cortex

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12
Q

Microscopic of HD ?

A

lOSS OF GABA =SEVER GLIOSIS
_________________
intranuclear cytoplasmuc inculsions of Aggrgate of Ubiquittainated Hintign proten

13
Q

الحمدلله رب العالمين

A
14
Q

Cognitive symptoms of Huntington’s is …..while behavioural is …..

A

Severe dementia
Suicide

15
Q

Compare pathogensis of Huntington & Parkinson

A

H, CAG trinucleotide repeat expansion of gene encoding huntigtin protein
P, point mutation & duplication of gene encoding alpha-synuclein, a protein involved in synaptic transmission, this cause AD PD, there is also AR form

16
Q

Describe PD grossly

A

Pallor of the substantia nigra

17
Q

Describe microscopic picture of PD

A

Loss of pigmented catecholaminergic neurons associated with gliosis. There are Lewy bodies in remaining neurons which are intracytoplasmic eosinophilic inclusions which are round to elongated have dense core surrounded by pale halo. They contain alpha-synuclein