Dementia
A patient loses the ability to make new memories. Where is the brain lesion?
Hippocampus (on both sides, leads to anterograde amnesia)
Huntington's Disease falls under the category of ____________ disorders.
Trinucleotide Repeat
What are the 2 most common causes of dementia in the elderly?
Alzheimer and Vascular Dementia
What is the former name of Fronto-temporal Dementia?
Pick Dementia
Where is the lesion located in Parkinson Disease?
Substantia Nigra (Pars compacta)
What condition is characterised by periodic sharp waves on EEG and increased 14-3-3 proteins in the CSF?
Creutzfeldt-Jakob Disease
A phenomenon in which the signs and symptoms of some genetic conditions tend to become more severe and/or appear at an earlier age as the disorder is passed from one generation to the next.
Anticipation
Why are patients with Down syndrome at higher risk of developing Alzheimer Disease?
Because the altered protein in familial forms, APP, is located on chromosome 21
What forms the inclusions in frontotemporal Dementia?
Hyper-phosphorylated Tau (round pick bodies) or Ubiquitinated TDP-43
What are the three most common drugs associated with Parkinsonism?
Categories
Antipsychotic Drugs
Anti-emetics that block dopamine
Valproic Acid
What form of dementia results from multiple arterial infarcts and/ or chronic ischemia?
Vascular Dementia
What are the gross examination findings of Huntington's disease?
Hint: Seen on CT and MRI in advanced patients
What gross changes are seen in the brain of a patient with Alzheimers Disease?
Widespread Cortical Atrophy, especially in the hippocampus with narrowing of the gyri and widening of the sulci
What are the two variants of Fronto-temporal Dementia that commonly present?
Behavioral Variant- Early changes in personality and behavior
Aphasia variant- primary progressive Aphasia/ May be associated with movement disorder
A 65-year-old woman is brought to her primary care physician by her husband because she has new-onset hallucinations. Her husband notes that, for the past year, she has thrashed around in bed, and he has taken to sleeping in a different room. She often reports seeing small children in her apartment. She has become forgetful about appointments and no longer pays bills on time. Her blood pressure is 115/80 mm Hg supine and 90/60 mm Hg standing. Physical examination reveals an alert, oriented patient with a resting tremor, mild extremity rigidity, postural instability, and bradykinesia. Mini-Mental State Examination reveals deficits in long-term recall.
A. Amyloid plaques in neuropil
B. Birefringent Amyloidosis
C. Dark Pigmentation in the Substantia Nigra
D. Eosinophilic cytoplasmic inclusions
E. Neurofibrillary Tangles in neurons
E. Eosinophilic Cytoplasmic Inclusions
These aggregates are mutated of alpha- synuclein proteins.
Amyloid plaques in neuropil and neurofibrillary tangles in neurons are characteristic of Alzheimer disease.
Dark pigmentation in the Substantia Nigra is a normal finding.
Where is the lesion in Wernicke- Korsakoff syndrome?
What type of amnesia is it associated with?
Mammillary Bodies
Anterograde Amnesia
Sudden, Jerky purposeless movement or slow writhing movements are characteristic of lesions in which part of the brain?
2x points: What are these movements called?
Basal Ganglia
Chorea and Athetosis
Aggregation of which two proteins leads to Alzheimer Disease?
B-Amyloid (Form plaques) and Tau (Forms Neurofibrillary tangles) are the two proteins
Pick disease, a type of front-otemporal dementia, can cause________ on brain imaging due to decreased brain tissue.
ex vacuo ventriculomegaly (enlarged ventricles)
A 69-year-old woman comes to the office because of a 6-month history of a right-hand tremor and slowed movement. She has to hold her hand to prevent it from shaking at rest, but the tremor resolves with intentional movement. Her family members have commented on her slower movements while walking or performing activities.
Which of the following neurotransmitters is most likely dysregulated in this patient?
A. GABA
B. Dopamine
C. Epinephrine
D. Glutamate
E. Norepinephrine
F. Glycine
B. Dopamine
Patient has Parkinson's.
Deficiency in GABA is associated with anxiety and seizure disorders
Glycine is inhibitory and abnormally high levels in the brain during seizures
Glutamate is excitatory and is increased in Alzheimer
Epinephrine is in medulla oblongata, modulating visceral activities
Norepinephrine is synthesised in locus ceruleus. It is increased in anxiety and decreased in depression.
Be specific
Cerebellar Vermis
Damage to the Vermis causes Truncal ataxia, dysarthria
In Huntington Disease, what mechanism is primarily responsible for neuronal cell death?
Excessive N-Methyl-d-aspartate (NMDA) receptor binding causing glutamate excitotoxicity
A patient with which of the following genetic mutations is most likely to develop late-onset Alzheimer disease?
A. Mutation of Presenilin 1
B. Mutation of Presenilin 2
C. Mutation of the E2 allele of APOE
D. Mutation of the E4 Allele of APOE
E. Trisomy 21
D. Mutation of the E4 Allele of APOE
Familial mutation of Presenilin 1/2, Trisomy 21 are all associated with early onset.
E2 allele of APOE are at decreased risk of developing Alzheimer Disease
What is the cure for FTLD?
If there is no cure, what medications are used to control the symptoms?
Broad group not specific meds.
SSRI's (Selective serotonin re-uptake inhibitors)
Antipsychotics
What is the difference between Lewy Body Dementia and Dementia secondary to Parkinson's Disease?
If cognitive and motor symptoms onset are <1 year apart, it is called Lewy Body.... Otherwise it is Dementia secondary to Parkinson Disease