Dementia and Behavioral Neurology Flashcards
What is used in PET scans to image beta-amyloid plaques in the brain in AD research?
Pittsburgh Compound B
What is memantine (Namenda)?
NMDA receptor blocker used in AD
Where is ACh produced?
Nucleus Basalis of Meynert
Alzheimer’s disease is uncommon in patients less than 60 years old. In such patients, where the disease often runs in the family, what protein is likely to be mutated?
Amyloid precursor
Which protein increases AD risk and lowers mean age of onset?
e4 allele of apolipoprotein E
Which genetic disease is thought to be a neurodevelopmental model for AD?
Down’s Syndrome
How can delirium be best distinguished from Alzheimer’s dementia?
Dementia lacks fluctuations in level of arousal
Types of medications that can cause dementia symptoms:
anticholinergics, benzos, opiates
What are Lewy Bodies composed of?
alpha-synuclein; seen namely in Parkinson’s disease
A highly suggestive sign of diffuse lewy body dementia (other than motor/gait abnormalities):
fluctuating cognition with variations in attention and alertness
LBD patients are hypersensitive to which medications?
Antipsychotics
Charles-Bonnet Syndrome
Patients are mentally healthy, often with significant visual loss. Hallucinations of small animals and people. Understand that they are not real and are typically not bothered by them.
An unusual feature of some patients with frontotemporal degeneration may be:
The development of new artistic abilities
3 patterns of behavior in frontotemporal degeneration (Pick’s disease):
Disinhibition; Apathy; Language Disturbance.
Occurs at younger age (< 65) and has more rapid progression, memory is often relatively unaffected.
What is Ropinirole (Requip)?
DA agonist used in PD. Can increase impulsivity (i.e. gambling, hypersexual behavior).
Which vitamin abnormality is responsible for Wernicke-Korsakoff syndrome?
thiamine/B1 deficiency
What is Ganser’s syndrome?
Prison psychosis or “the syndrome of approximate answers.”
Typically presents in a “step-wise” fashion and patients have focal neurological deficits.
Vascular dementia
After VP shunt there is risk for what? Why?
SDH; due to the reduction of intracranial pressure that pulls the brain away from the meninges and tears veins.
Mneumonic for NPH
Wobbly, weird, and wet. Magnetic gait, dementia, and urinary urgency/frequency.
Transient Global Amnesia
Complete and reversible anterograde and retrograde memory loss lasting up to 24 hours. Thought to be migraine phenomemnon in context of emotional event or sexual activity.
Creutzfelt-Jacob Disease
Prion disease characterized by rapid onset change in mental status AND myoclonus provoked by a startle.
Kluver-Bucy Syndrome
Caused by damage to bilateral amygdalae. Hypersexuality, diminished fear response/docile, overeat/PICA, difficulty recognizing familiar faces/objects.
Where is the lesion in Wernicke’s/Receptive aphasia?
Left superior temporal gyrus
Where is the lesion in Broca’s/Expressive aphasia?
Left inferior frontal gyrus
Where is the lesion in patients with conduction aphasia?
Arcuate fasciculus
Abnormal Prosody lesion is where?
Right inferior frontal gyrus, giving monotonicity to speech.
Where is the lesion in pure word deafness?
Bilateral damage to posterior superior temporal lobes (auditory cortex), cannot comprehend the meaning of speech.
Where is the lesion in akinetic mutism (can speak under extreme duress)?
bilateral cingulate gyrus
What supplies Broca’s and Wernicke’s areas?
The MCA
What is dysarthria?
A speech problem caused by trouble with the muscles of articulation.
What is dysphonia?
A problem of vocal cords and patients sound hoarse or breathy when they speak.
What is anomic aphasia?
Trouble naming objects
What is Gerstmann’s Syndrome?
Lesion to the dominant angular gyrus resulting in agraphia, acalculia, finger agnosia, left/right confusion.
What is apraxia?
A disorder of skilled movement not caused by weakness, akinesia, deafferentation, abnormal tone/posture, movement disorders, intellectual deterioration, uncooperativeness.
What is prosopagnosia and where is the lesion?
Inability to recognize familiar faces; Fusiform gyrus (FFA)
Alexia without agraphia
Left occipital lobe, involving the splenium of the corpus callosum. Visual information reaches only the right occipital lobe, but cannot be accessed by the language areas of the brain. Therefore, patients cannot read. Since the language areas are otherwise fine, people can write.
What is Anton’s syndrome?
Denial of blindness due to damage in bilateral occipital lobes.
What is Bálint’s syndrome and how is it characterized?
Due to damage to both posterior parietal lobes:
- Optic ataxia: incoordination of hand and eye movement.
- Oculomotor apraxia: the inability to voluntarily guide eye movements.
- Simultanagnosia: the inability to perceive more than one object at a time in one’s visual field.
What is posterior reversible encephalopathy syndrome?
Acute onset of confusion and cortical blindness, usually due to sudden, drastic increases in BP.
What is Kleine-Levin syndrome?
A rare sleep disorder characterized by persistent episodic hypersomnia and cognitive or mood changes. Many patients also experience hyperphagia, hypersexuality and other symptoms.
What is pseudobulbar palsy?
Seen in diseases such as MS, ALS, AD, laughing/tears for no reason, no explanation/emotion. Treated with dextromethorphan/quinidine.
Corticobasal degeneration results in what?
Alien hand syndrome. Classiaclly described as a consequence of epilepsy surgery and CC severing.