Neurology Jeopardy

Neuroimmunology

100

A ______ is defined as an episode of new or worsened neurologic sxs, lasting more than 24 hours, not due to fever or infection.

Relapse. AKA attack, bout, or exacerbation

200

INO: disorder of _____

Medial longitudinal fasciculus

Ex: with a R INO, on left gaze- cannot ADDuct R eye. Have L eye abduction nystagmus

300

Your 34 yo AA female pt with relapsing neurologic symptoms has CSF testing. Which of the following findings would prompt you to consider diagnoses other than MS?
a. Elevated kappa chains

b. WBC count of 22

c. Neutrophilic pleocytosis

d. Elevated IgG synthesis rate

e. Normal CSF immunologic parameters

c. Neutrophilic pleocytosis. Unusual in MS, can be seen in NMO

Elevated kappa chains indicate Ab formation in CNS- can be seen in NS

WBC can be elevated (no more than 50 cells) and are usually lymphocytic predominant

IgG synthesis rate elevation is measure of increased intrathecal elaboration of Ab and can be seen in MS

Some pts have normal CSF- does not r/o MS

400

This phenomenon, is when transient symptoms of demyelination are elicited by heat or exercise

Uthoff's phenomenon

Manifests as blurry vision with exercise d/t impaired conduction in an optic nerve that has had demyelinating injury

500

 

Tumefactive MS

Subform of RRMS with large, mass like lesions

Incomplete peripheral enhancement- "open ring sign"

Tx: IV steroids but may need PLEX or IVIG

600

This MS medication inhibits the mitochondrial enzyme, dihydroorotate dehydrogenase. Associated with n/v/d, elev LFTs, and alopecia. Pregnancy Class X

Teriflunomide (Aubagio)

700

Which of the following best describes relationship of ADEM with MS?

a. It appears to be a separate neuro-immunologic disorder

b. It usually transitions into MS

c. It never transitions into MS

d. It is the pediatric equivalent of MS

e. It is the same as MS

a. It appears to be a separate neuro-immunologic disorder

However, some pts diagnosed with ADEM later develop relapsing pattern consistent with MS. This may partially be due to tendency in pediatric pop. to diagnose ADEM rather than a first episode of MS

800

Orally active modulator of sphingosine-1 phosphate (S1P) receptors on thymocytes and lymphocytes. Induces uncoupling and internalization of the receptor. Cells become unresponsive to signaling. Reduces relapse in RRMS.

A/E: macular edema and first dose bradycardia- requires 6 hrs observation after 1st administration

Others: elev LFTs, bronchitis. Asso. of risk of severe VZV infxns. Pregnancy Category C

Fingolimod (Gilenya) 

900

Seen in MS, visual phenomenon where pts have trouble following moving objects visually. Lateral motion in VF is perceived as having depth component

Pulfrich's phenomenon

1000

____ hand of ____: when, because of sensory deafferentation, the hand feels useless with otherwise normal motor function?

Useless hand of Oppenheim

Cranial nerves

100

Taste anterior two- thirds of tongue

Facial nerve

200

Corneal reflex: afferent and efferent

CN V, CN VII (trigeminal, facial)

300

Closure of eye when smiling (reverse may also occur). When some axons from motor neurons to labial muscles involved in smiling may regenerate and misdirect to the orbicularis oculi

Synkinesis

400

Name for type of afferent pupillary defect: no response to direct light, but response to consensual light in contralateral eye present 

Marcus Gunn pupil

500

V3, mandibular nerve aka 3rd division of trigeminal nerve passes through which skull foramina?

Standing Room Only (V1, V2, V3): Superior orbital fissure, foramen Rotundum, foramen Ovale

600

Taste posterior one- third of tongue

Glossopharyngeal nerve

700

Nuclei for taste sensation

Rostral nucleus solitarius

Solitary nucleus, also called nucleus of the solitary tract (SN or NTS) is a series of purely sensory nuclei forming a vertical column of grey matter embedded in the dorsolateral medulla. Through the center of the SN runs the solitary tract, a white bundle of nerve fibers, including fibers from the facial, glossopharyngeal and vagus nerves. The SN projects to, among other regions, the reticular formation, parasympathetic preganglionic neurons, hypothalamus and thalamus, forming circuits that contribute to autonomic regulation. 

800

Nuclei for parasympathetic output to chest, thorax, and GI tract

Dorsal motor nucleus of vagus

900

Parasympathetic source to head and neck, which nucleus?

Superior salivatory nucleus

1000

Caloric testing. Explain.

Vascular

100

Syndrome. Right hemiparesis, RHH, and aphasia

Left MCA syndrome

200

Structure (mostly) affected in pure sensory lacunar syndrome. Presents as c/l hemisensory loss

Thalamus

300

Structure affected in pure motor lacunar syndrome

Posterior limb of internal capsule. Presents as c/l motor deficits

400

This artery forms the vascular supply to both medial thalami

Artery of Percheron

500

Limited upward gaze, convergence retraction nystagmus, light- near dissociation, lid retraction, and skew deviation of eyes

Parinaud's syndrome

Lesion affecting quadrigeminal plate (constituted by superior and inferior colliculi). AKA tectum- dorsal portion of midbrain. Located posteriorly to cerebral aqueduct

600

Deep branch from ACA that supplies anterior limb of internal capsule, inferior part of head of caudate nucleus, and anterior part of globus pallidus

Recurrent artery of Heubner

700

According to this trial, in patients with 70-99% symptomatic carotid stenosis, two- year ipsilateral stroke rate was 26% with medical tx versus 9% with CEA

NASCET trial

Symptomatic stenosis of 70-99% should be revascularized. Symptomatic stenosis of 50-69% may also benefit with CEA, with greater impact in men than women, in those with previous stroke/TIAs, and with hemispheric versus retinal symptoms

800

Patients undergoing carotid angioplasty and stenting (CAS) have lower rates of MI compared to those with carotid endarterectomy (CEA). True or False?

True 

CREST- higher rate of peri-procedural stroke in CAS and higher rate of peri- procedural MI in CEA group. Differential outcome based on age was noted

CAS favors pts younger than 70, CEA favors pts older than 70

900

Ipsilateral third nerve palsy and contralateral ataxia, weakness and tremor

Claude's syndrome

Lesion in the dorsal portion of mesencephalic tegmentum

1000

This study demonstrated that compared to warfarin, high-dose dabigatran (110mg BID) reduces stroke  (and systemic embolism) risk without increasing the risk of major bleeding among patients with nv atrial fibrillation.

RE-LY study

Headache

100

Acetazolamide inhibits this enzyme

Carbonic anhydrase

200

Name some headache red flags

Systemic symptoms (fever, chills, wt loss)

Hx of prior cancer. Immunodeficiency

FND

Thunderclap HA

New onset HA after age 50

Precipitation by exertion, strain, or position changes

Increasing HA frequency and severity

New onset seizures

300

New name for basilar migraine

Migraine with brainstem aura. Reflects the fact that migraine is now viewed as primarily neurologic disorder rather than vascular disorder. 

(Outdated vascular theory is basis for ongoing contraindication to triptan and DHE in migraine with BS aura and hemiplegic migraine- deemed to be safe)

400

DDx for bilateral trigeminal neuralgia or trigeminal neuralgia in a young pt

MS, sarcoidosis, Lyme disease

500

Is there an association b/w women who have migraine with aura and increased stroke risk?

YES

In women <45yo, who have migraine WITH aura, there is two-fold increased risk of stroke (migraine w/o aura does not have same increased risk)

Risk increases to six- fold with OCP use and nine- fold with smoking+OCP

600

Botox MOA

Onabotulinumtoxin A: cleaves the SNAP 25 protein (which is a SNARE)

Endocytosis of toxin into cell membrane of motor nerve and into its cytoplasm. Cleaving SNAP 25 prevents ACh containing vesicles from fusing with membrane and prevents ACh release into NMJ. Muscle contraction blocked. 

700

Indomethacin- responsive headaches (x2)

Hemicrania continua, paroxysmal hemicrania

800

Name (at least 2) type of HAs that are classified as TACs (trigeminal autonomic cephalalgias)

Cluster HA

Paroxysmal hemicrania

Hemicrania continua

SUNCT

SUNA

900

CADASIL gene mutation

NOTCH 3 (missense mutation, chromosome 19)

Presents with migraine with aura, stroke/TIAs, progressive dementia, and other ND findings. Autosomal Dominant. 

1000

Diagnosis of ____ requires a distinct and clearly remembered onset of a continuous and unremitting headache, and it must be present for >3 months

New daily persistent headache (NDPH)

HA associated with NDPH often resembles migraine in its features, with days when there is light or sound sensitivity, nausea, and throbbing pain. Or it may resemble a tension-type milder headache without any of those features. 

Lack of evidence for any specific treatment for NDPH

Neuromuscular

100

Presynaptic P/Q type voltage gate calcium channel antibodies are seen in this syndrome

Lambert- Eaton myasthenic syndrome

200

This protein is absent in Duchenne muscular dystrophy

Inheritance: X- linked recessive

Dystrophin

300

This medication inhibits glutamate release, which slows disease progression (prolongs survival by approx 3 months) in patient with ALS

Riluzole

400

Antibody associated with Miller Fisher syndrome

GQ1b

500

_____ is an inflammatory demyelinating disorder of childhood in which there is monophasic immunologic reaction to a viral illness. Present with encephalopathy, see confluent white matter changes on MRI and inflammatory markers in CSF. SC may be affected with features of TM

Acute disseminated encephalomyelitis (ADEM)

Tx: high dose IV steroids

600

Antibody associated with multifocal motor neuropathy with conduction block

GM1

700

Histologic finding in Inclusion Body Myositis

Rimmed vacuoles

800

Mutation in this gene causes central core myopathy. Associated with malignant hyperthermia

RYR1

900

Condition where there is hypertrophy of extramedullary adipose tissue in the epidural space, and is associated with chronic use of steroids

Epidural lipomatosis

1000

This enzyme is deficient in McArdle's disease

Myophosphorylase deficiency

Psychiatry

100

Immature defense mechanism involving the misattribution of one's unacceptable feelings or impulses to another person who does not have them

Projection

200

Pathologic impulses to set fire

Pyromania

300

This medication is an agonist at GABAb receptors. Used often as muscle relaxer

Baclofen

400

Duration of symptoms distinguishing acute stress reaction from PTSD

<1 month: ASR

>/1month: PTSD

500

Antipsychotic notorious for causing agranulocytosis

Clozapine

600

Duration of symptoms distinguishing schizophrenia, schizophreniform disorder, and brief psychotic disorder

Brief psychotic disorder <1month

Schizophreniform disorder >1 to <6 months

Schizophrenia >/6 months

700

Chronic and intense preoccupation with a perceived defect of appearance or over- concern with minor physical abnormalities. Oftens eek unnecessary and repeated surgical procedures to correct their perceived deformity.

Body dysmorphic disorder

800

Clozapine, Olanzapine, Ziprasizone, and Aripiprazole: which 2 cause weight gain?

Clozapine and Olazapine

900

Name some whole brain and CSF volume changes seen in patients with schizophrenia

Hint: brain gets bigger or smaller?

CSF volume increased or decreased?

Reduced brain volumes and higher CSF volumes

Evidence of ventricular enlargement (3rd and lateral) with sulcal widening. Atrophy of frontal and temporal lobes seen, as well as thalamus and hippocampus. PET studies have shown hypo-metabolism in DL prefrontal cortex during activation tasks

1000

Case: 19 yo M arrested after denied seating at a restaurant and proceeds to yell and threaten staff. Has longstanding hx of detentions at school for angry outbursts. Has gotten into many fights and previously been arrested for physical aggression against classmate. After each occurrence, he expresses regret though he often attempts to justify his actions. When not aggravated, he is usually pleasant. Most likely diagnosis?

1. Anti-social PD 2. Bipolar disorder 3. Intermittent explosive disorder 4. Oppositional defiant disorder 5. Borderline personality disorder

Intermittent explosive disorder- impulse control disorder characterized by verbal/physical aggression out of proportion to instigating event. May express remorse. Tx: psychotherapy, as well as pharmacotherapy with mood stabilizers or SSRIs. 

History

100

Wrote the book "The Man Who Mistook His Wife for a Hat"; describes a man with visual agnosia

Oliver Sacks

200

Austrian neurologist and the founder of psychoanalysis, a clinical method for evaluating and treating pathologies in the psyche through dialogue between a patient and a psychoanalyst. 

Sigmund Freud

300

French Neurologist. Has many associated eponyms including a pathological reflex where the great toe extends in presence of an injury to the pyramidal tract. Suffered from PD in his last years.

Joseph Babinski

400

Father of modern Neurology

Named and was the first to describe multiple sclerosis

Jean- Martin Charcot

500

French physician, anatomist and anthropologist. Best known for his research on ___ area, a region of the frontal lobe that is named after him. This area is involved with language. His work revealed that the brains of patients with aphasia contained lesions in a particular part of the cortex, in the left frontal region. This was the first anatomical proof of localization of brain function.

Paul Broca

600

Canadian neurologist whose notable contributions include first detailed descriptions of lacunar strokes, identification of TIAs as stroke precursors, identification of link between carotid atherosclerosis and stroke, and the description of a variant form of Guillain–Barré syndrome which bears his name.

C. Miller Fisher

700

Named after him, ___ sign is dorsiflexion of the great toe elicited by irritation downward of the medial side of the tibia. It is one of a number of Babinski-like responses.The sign's presence indicates a damage to the pyramidal tract.

Hermann Oppenheim

800

Spanish neuroscientist, pathologist, and histologist specializing in neuroanatomy and the central nervous system. He and Camillo Golgi received the Nobel Prize in Physiology. He was the first person of Spanish origin to win a scientific Nobel Prize. His original investigations of the microscopic structure of the brain made him a pioneer of modern neuroscience.

Santiago Ramon y Cajal

900

German neuro-psychiatrist. He is best known as the inventor of electroencephalography (EEG) in 1924. Discoverer of the alpha wave, which have been eponymously referred to as the "___ wave"

Hans Berger

1000

Father of Central Texas Neurology. Hobbies include Jazzercise and fancy statue collection.

Systemic diseases/ pregnancy

100

Syndrome. Dry eyes, dry mouth, painful dysesthesias with sensory loss

Sjogren syndrome; sensory neuronopathy

200

Headache, oral, and genital ulcers in patient of Eastern Mediterranean descent

Behcet disease

300

Recurrent sinusitis, renal disease, multiple cranial neuropathies, see granulomas

Wegener granulomatosis

Check c-ANCA

400

Fever, abdominal pain, headache, mononeuritis multiplex in patient with Hep B

Polyarteritis nodosa; check p-ANCA

500

Facial palsy involving both upper and lower face, hilar adenopathy

Sarcoidosis

600

Pentad of TTP

Fever

Anemia (microangiopathic hemolytic)

Thrombocytopenia

Renal dysfunction

Neurologic s/s

FATRN

700

Disorder of childhood characterized by acute onset of fever, conjunctivits, mucositis, polymorphous rash, lymphadenopathy, increased risk of coronary artery aneurysms, occurrence of aseptic meningitis

Kawasaki Disease

800

Antibodies against ____ are detected in serum of patients with IgM MGUS

Anti- MAG (myelin associated glycoprotein)

Primarily demyelinating

High risk of developing symptomatic multiple myeloma (BM inv, anemia, renal failure, lytic lesions, hypercalcemia)

900

Syndrome. Tongue fissuring and angioedema along with neurologic complications of demyelinating and/or axonal neuropathies, myopathies, cranial neuropathies (particularly CN VII palsy)

Melkersson- Rosenthal syndrome

Can be seen in patients with IBD such as Crohn's or UC. Risk of both venous and arterial thrombosis

1000

Syndrome. Type of hereditary thrombophilia. Most likely to cause thrombosis involving CNS. VST is most common neurologic manifestation. Ischemic infarcts occur 2/2 paradoxical emboli through intracardiac R->L shunt. Other neuro manifestations include acute onset chorea (can be pronounced during pregnancy), headaches, and seizures

Anti-phospholipid antibody syndrome (APLS)

Movement disorders

100

Direct pathway: excitatory or inhibitory to cortex?

Excitatory- increases thalamic excitation of cortex

200

Phenomenon that dystonias can be diminished by sensory tricks like touch in affected body part

Geste-Antagoniste

300

Name 3 synucleinopathies

PD

LBD

MSA

400

PD Med: COMT inhibitor

Entacapone

500

______ "buzz word" for finding on MRI showing hyperintensity surrounded by hypointensity in basal ganglia. Seen in pantothenate-kinase-associated neurodegeneration (PKAN)

Eye of the tiger

600

Name 3 structures that make up Guillain- Mollaret triangle

Dentate nucleus of cerebellum

Inferior olive

Red nucleus

700

Specify syndrome with episodes of ataxia and facial twitching. Gene inv: KCN1A. Triggers: exercise, startle. Tx: AED

Episodic ataxia Type I

In Type II, there is ataxia with nystagmus and dysarthria. Tx: DIamox

800

Antibodies in stiff person syndrome

Autoimmune

Paraneoplastic

anti- GAD

anti- amphiphysin

900

When using DBS to treat PD, the subthalamic nucleus (STN) is a better target to use than the globus pallidus interna (GPi) in patients with __________.

a. Axial symptoms prevalent

b. Gait issues

c. Higher medication requirements

d. Pronounced dyskinesia

GPi is favorable for those with more axial symptoms, gait issues, depression, and word fluency problems. Many also favor GPi when dyskinesias are especially problematic. In turn, the STN is often favored in those with higher medication requirements, and in those with greater tremor.

1000

High arched feet, scoliosis, neuropathy, ataxia, cardiomyopathy. Diagnosis?

Friedrich's ataxia

Trinucleotide repeat GAA expansion in Frataxin gene on chromosome 9. AR.

P.S. medication that improves outcome of CM in Friedrich's ataxia: Idebenone (coq10 analogue)