Welcome to our new feature: NEUROLOGY FLASHCARDS! Understanding is paramount, but unfortunately, one aspect of academic success does rely on simple memorization and repetition. In our new Neurology flashcards feature, we have taken the most commonly seen rote memory topics and “buzzwords” from neurology board and RITE* exams and made easy to use flashcards. These work on desktop, tablet, and mobile devices. This is a new feature, so please contact us if you see any issues! FLASHCARDS will be available for all chapters soon!

"Eye of the Tiger" Sign
Diffuse hypointensity of the bilateral globus pallidus with a small region of hyperintensity.





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Unilateral resting tremor, cogwheel rigidity, bradykinesia, and hypomimia
Parkinson's Disease
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Alpha Synucleinopathies
Parkinson's Disease, Multiple System Atrophy, and Lewy Body Dementia
This question is always asked on Board and RITE* exams. Every. Year.
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Tauopathies
Progressive Supranuclear Palsy (PSP), Corticobasal Degeneration (CBD), & Frontotemporal Dementia w/ Parkinsonism Linked to Chr. 17 (FTDP-17)
This question is always asked on Board and RITE* exams. Every. Year.
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"Hummingbird Sign"
Atrophy of the midbrain tegmentum, corpus callosum, and anterior cingulate gyrus.
Progressive Supranuclear Palsy (PSP)
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PD Med Side Effect: Livedo Reticularis
Amantadine
Partial dopamine agonist and partial NMDA receptor antagonist.
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Tolcapone and Entacapone
Mechanism of Action
Side Effects
Mechanism of Action: COMT Inhibitors: reduces methylation of levodopa and dopamine, increasing their half-life.

Side Effects: Diarrhea, discoloration of urine.
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FDA-approved Drug for Parkinson's Disease-related Psychosis
Pimavanserin
This is a Serotonin 5-HT2A receptor inverse agonist and antagonist.
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Medication Side Effect: Hypertensive Crisis with Tyramine Foods
Rasagiline and Selegiline
These are monoamine oxidase (MAO-B) inhibitors. If taken with tyramine-heavy foods like aged cheese or cured meat, can cause severe tachycardia and hypertensive crisis.
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Essential Tremor Treatment
(with Level A Evidence)
Propranolol or primidone
Primidone metabolizes into a barbiturate, so should be cautious in elderly populations. Atenolol and others have Level B evidence. Deep brain stimulation can be used in refractory cases.
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Locations for Deep Brain Stimulation (DBS)
Ventral intermediate nucleus of the thalamus: Improves tremor.

Globus pallidus interna or subthalamic nucleus: Improves tremor, bradykinesia, and rigidity.
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Parkinsonism, early falls, vertical gaze palsy, and impaired smooth oculomotor pursuit
Progressive Supranuclear Palsy (PSP)
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GTP cyclohydrolase I (GCH1) Mutation
Dopa-responsive Dystonia
Autosomal dominant, chromosome 14. Presents in childhood as progressive dystonia without any significant comorbidities.
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MAPT Gene Mutation
Chromosome 17
Frontotemporal Dementia with Parkinsonism Linked to Chromosome 17 (FTDP-17)
MAPT encodes Tau, and this disease is a tauopathy. Of note, MAPT gene mutation has also been associated with PSP, but less robustly.
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Treatment of Acute Dystonia Reaction
Benztropine (anticholinergic)
Or, diphenhydramine (antihistamine).
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Causes of Drug-induced Tremor
Lithium, valproic acid, amiodarone, SSRI
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Tourette's Syndrome Diagnostic Criteria
One vocal and one motor tic for >1 year before the age of 18. If present for <1 year, it is called "transient tic disorder."
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ADHD medication that will not worsen tics
Atomoxetine
ADHD and tic disorders are often seen together. If the patient needs ADHD medication, methylphenidate should be avoided as it will make tics worse. Atomoxetine can be used without worsening tics.
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Treatment of Episodic Ataxia
Acetazolamide
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Mutation in Episodic Ataxia
Type 1:

Type 2:
Type 1: KCNA1 (episodes last minutes)

Type 2: CACNA1a (episodes last hours to days). Can also cause familial hemiplegic migraine and spinocerebellar ataxia type 6.
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Friedrich Ataxia Mutation
GAA Trinucleotide Repeats
>66 repeats, on the FXN (frataxin) gene. Autosomal recessive.
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Ataxia Telangiectasia (ATM Gene Mutation)
Key Symptoms (4)
Progressive cerebellar ataxia, telangiectasias, and recurrent sinopulmonary infections. ATM coordinates DNA repair, so patients with this have a higher risk of various cancers
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Mutation in Fragile X Tremor Ataxia Syndrome (FXTAS)
CGG Trinucleotide
Between 50 and 200 repeats, in the FMR gene.
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Mutation in Dentatorubral-pallidoluysian Atrophy
CAG Trinucleotide Expansion in ATN1 Gene
Causes late-onset (age 30 on average) ataxia, myoclonic epilepsy, and dementia, most commonly in Japanese.
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Paroxysmal Kinesigenic Dyskinesia Treatment
Carbamazepine or Phenytoin
Has brief, <1 minute attacks of dystonia and/or choreoathetosis triggered by sudden movements or startle.
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Stiff Person Syndrome
Presents at age 40-50 with increased tone of axial muscles, especially paraspinal. Can be associated with anti-GAD65.
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"Face of the Giant Panda Sign"
MRI FLAIR with hyperintensities of the caudate, putamen and midbrain, with sparing of the red nuclei.
Wilson's Disease
a.k.a. hepatolenticular degeneration.
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Mutation in Wilson's Disease
ATP7B
Chromosome 13. Autosomal recessive. Causes copper accumulation in liver, brain, kidneys, and cornea because it can not attach to ceruloplasmin to transport properly. Without bound copper, ceruloplasmin degrades quickly, so ceruloplasmin levels are low.
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Treatment of Wilson's Disease
Penicillamine
Also, low copper diet, tetrathiomolybdate and zinc can decrease copper absorption. Liver transplant may be needed.
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Manganese Toxicity MRI
Bilateral Globus Pallidus Hyperintensities
Has progressive parkinsonism, ataxia, dysarthria, and bulbar dysfunction.

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Triad of Cognitive Decline, Symmetric Parkinsonism, and Visual Hallucinations
Lewy Body Dementia (LBD)
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Parkinsonism, Cerebellar Dysfunction, Pyramidal Tract Signs, Autonomic Dysfunction
Multiple Systems Atrophy
Autonomic symptoms typically beginning with bladder and erectile dysfunction, with prominent orthostasis on examination.
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Botulinum Mechanism of Action
Cleaves synaptosomal/SNARE proteins (SNAP/VAMP) which prevents exocytosis and in turn prevents the release of acetylcholine at neuromuscular junctions.
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Tetrabenazine Mechanism of Action
VMAT2 Inhibitor
Reversible inhibition of vesicular monoamine transporter 2 (VMAT2). This leads to the decreased uptake of monoamines (dopamine, serotonin, norepinephrine, histamine) into synaptic vesicles, as well as depletion of monoamine storage from nerve terminals. This is a medication for Huntington's Disease.
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"Hot Cross Bun" Sign
Loss of pontocerebellar fibers, creating appearance of a cross in the pons.
Multiple Systems Atrophy (MSA)
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Torsin A gene (DYT1) Mutation, Chromosome 9
General Dystonia (Early Onset Primary Dystonia)
Action-induced dystonia in early childhood.
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Balloon Neurons and Early Limb Apraxia
Corticobasal Degeneration (CBD)
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Caudate Atrophy on MRI
Huntington's Disease
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LRRK2 Mutation
Parkinson's Disease (PD)
LRRK2 mutations lead to 10% of familial and 5% of sporadic PD cases. It is seen more often in North African Arabs.
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Direct Dopamine Agonists (DDA) Side Effect
Impulse-control disorder, hallucinations, nausea, orthostatic hypotension
Bromocriptine, pramipexole, ropinirole, rotigotine, and apomorphine.
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Huntington's Disease Mutation
CAG Trinucleotide Repeat, Chromosome 4
Repeat count >40.
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REM sleep behavior disorder, constipation, and anosmia
Early signs of Parkinson's Disease
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Dopamine Transporter (DAT) Scan
Parkinson's Disease (PD)
DAT imaging can be used to assist in the diagnosis of PD when clinical diagnosis is unclear, but PD diagnosis is still purely clinical.
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Kayser-Fleischer Rings
Seen on slit lamp exam.
Wilson's Disease
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Ataxia and high α-fetoprotein
Ataxia Telangiectasia
Also seen with Ataxia with Oculomotor Apraxia Type 2 (AOA2)
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Medication for Cardiomyopathy if Friedreich's Ataxia
Idebenone
This is an analogue to Co-Q10.
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Developmental delay and seizures as child, dystonia and parkinsonism as adult. "Halo" on MRI in Substantia Nigra
Beta-propeller Protein-associated Neurodegeneration (BPAN)
This is a type of neurodegeneration with brain iron accumulation (NBIA) disorder. These disorders cause progressive movement problems. BPAN may have a "halo" of hyperintensity in the substantia nigra. Caused by WDR45 mutation.
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