28 Questions
What is the typical progression of Lewy body dementia?
Progresses over 10 to 15 years, eventually producing severe motor slowing or near immobility
What is the relationship between Lewy body dementia and Parkinson's disease?
Lewy body dementia occurs when the cortex is involved, in addition to the basal ganglia as in Parkinson's disease
What is the primary cause of death in patients with Lewy body dementia?
Aspiration pneumonia or trauma from falls caused by postural instability
How does the effectiveness of L-DOPA treatment change over the course of Lewy body dementia?
L-DOPA is initially effective, but becomes less effective with disease progression
What is the difference between fine and coarse tremors in Parkinson's disease?
Fine tremors are seen in hyperthyroidism or hypoglycemia, while coarse tremors are seen in Parkinson's disease
How does the slowed movement (bradykinesia) affect a Parkinson's patient's gait?
Patients take shorter steps and have difficulty initiating movement
What characterizes neurodegenerative diseases involving the cortex?
Memory loss, behavioral changes, and language difficulties
Which disease is characterized by difficulty in swallowing and respiratory problems due to muscle weakness?
Amyotrophic Lateral Sclerosis
What is the main cause of Parkinson's Disease?
Loss of dopaminergic neurons from the substantia nigra
Which disease presents as a hypokinetic movement disorder?
Parkinson's Disease
What neurological symptom is associated with diseases affecting the cerebellum?
Ataxia
Which diseases involve cognitive abnormalities like memory loss and behavior changes?
Frontotemporal Dementia and Pick Disease
What is the relationship between the number of CAG repeats and the age of onset of the disease?
More CAG repeats lead to an earlier age of onset.
What is the consequence of further expansions of CAG repeats during spermatogenesis?
Early onset disease in the next generation.
Which brain region shows striking atrophy in Huntington's disease?
Caudate nucleus and putamen
What is the main type of neurons that are especially sensitive and disappear early in Huntington's disease affected regions?
Spiny neurons releasing GABA, enkephalin, dynorphin, and substance P
What is a significant characteristic of the intranuclear inclusions seen in Huntington's disease?
They contain aggregates of ubiquitinated huntingtin protein.
How do larger numbers of CAG repeats influence disease onset in Huntington's?
Lead to earlier onset due to proteolysis and aggregation of the mutant protein.
What is the second most common neurodegenerative disorder after Alzheimer's disease?
Parkinsonism
What is the primary characteristic of the resting tremor in Parkinsonism?
It is a coarse tremor that disappears with movement
What is the primary cause of Parkinsonism?
Damage to dopaminergic neurons projecting from the substantia nigra to the striatum
What is the key diagnostic feature of Parkinsonism?
Lewy bodies containing $\alpha$-synuclein
What is the underlying mechanism that leads to neuronal loss in Parkinsonism?
All of the above
How does Parkinsonism differ from Parkinson's disease?
Parkinsonism is a clinical syndrome, while Parkinson's disease is a specific neurodegenerative disorder
Based on the information provided, which condition is the patient most likely suffering from?
Lewy body dementia
What is the protein accumulated in this patient's condition?
Alpha-synuclein
Which of the following is NOT a characteristic feature of Lewy body dementia?
Intranuclear inclusions
In which of the following conditions are intranuclear inclusions observed?
Huntington's disease
Explore the causes and effects of neurodegenerative disorders in the context of neuronal loss and cognitive abnormalities such as memory, behavior, and language issues. Understand how different diseases affecting the cortex can lead to atrophy and specific signs and symptoms.
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