Seizures
AEDs
Name that diagnosis
Headaches
Potpourri
100
A 7 year-old boy with no past medical history has an event of right face twitching and arm jerking lasting 45 seconds at approximately 6AM. What is the diagnosis?
What is Benign Epilepsy with Centrotemporal Spikes
100
What is fetal hydantoin syndrome?
What is digit and nail hypoplasia, microcephaly, unusal facies, and developmental delay due to in-utero exposure to dilantin
100
A 10 year-old youngster presents to the emergency department due to difficulty voiding. Neurologic examination reveals him to be alert and oriented, fluent, good strength of facial muscles and upper extremities, but lower extremity weakness (proximal and distal). Reflexes at patella and ankle present. Diminished sensation below the chest bilaterally.
What is Guillain-Barré acute inflammatory demyelinating polyneuropathy
100
A 12-year-old girl presents with her third headache in the last 2 months. She describes the pain as pounding, sharp, and severe. The pain is bifrontal and has been present for 1 hour. Past history is notable for motion sickness at age 4 years. Physical examination results are normal, but the girl draws a picture of dots when asked if she sees anything prior to pain. What are the two features in children with migraines that are different than adults?
What is duration and location
100
The most common complication of bacterial (Pneumococcal) meningitis
What is hearing loss
200
13 month old boy, who presented with a history of the following spells. Over 5 minutes, there were clusters of sudden forward flexion of the head and neck; along with flaring of arms outward, and then inward. The eyes would roll up briefly with each spell. These clusters occurred about three times per day, usually shortly after awakening. He babbled without words, and could not stand. Name 3 physical exam findings other than a thorough neurological exam you should check for.
What is head circumference, skin exam, limb length
200
You are seeing Johnny, a 17 yo boy with GTCs since age 12 as a second opinion in Sankar's clinic. He continues to have GTCs 3-4 times a month. The only AED he has been on is Dilantin. You order a dilantin level and it comes back 16 mcg/mL. What is your next step?
What is obtain routine EEG, start Keppra, then wean Dilantin slowly. Order DEXA scan. Key: The therapeutic range for phenytoin is 10 to 20 mcg/mL.
200
You are evaluating a 7 mo girl who parents are concerned about abnormal eye movements. They described them as jerky horizontal movements of her left eye. Then several days later, she started nodding her head intermittently. She is otherwise doing well and developmentally on track. She has a normal neurologic exam.
What is spasmus nutans. Triad: nystagmus, titubation, torticollis. Typically seen in infants 4-8 months. Usually resolves by age 3.
200
19 year old male presents with complaint of headaches several times per week, always in the afternoon or at the end of the day. The pain starts at the base of the neck and slowly moves up until it is at his forehead. The movement from neck to forehead takes about a hour. The patient has tried eating more regularly and drinking more water and decreasing or increasing caffeine intake, but none of these is effective. When he has a headache, the only thing that helps is to lie down and close his eyes. The headaches seem to be worse with increased time at the computer. On exam, muscles of the neck, especially the sub-occipital muscle group, are very tight and tender. There are multiple trigger points in the sub-occipital muscles and in the sternocleidomastoid on the right. Pressure on the sub-occipital trigger points reproduces the headache. Range of motion is decreased in neck flexion and right rotation. Other orthopedic testing for nerve, muscle and joint involvement was negative. The temporomandibular joint (TMJ) was not contributory.Name your best diagnosis.
What is tension headache
200
Obstructive hydrocephalus may result from all the following, except: A. Choroid plexus papilloma B. Subependymal giant cell astrocytoma (SEGA) C. Aqueductal stenosis D. Vein of Galen malformation
What is A. choroid plexus papilloma
300
What are the risk factors for the development of epilepsy in children with febrile seizures?
What is 1. family history of epilepsy. 2. complex febrile seizure 3. Duration of fever <1 hour before seizure onset. 4. developmental delay
300
What medication should you avoid in children with POLG mutation?
What is depakote
300
A 2 year old girl has global developmental delay and intractable infantile spasms. Her MRI showed corpous callosum agenesis, gray matter heterotopia, and polymicrogyria. Name other associated symptoms/signs with this disease.
What is coloboma, microphthalamia, short philtrum, flat nose, large ears, sparse eyebrow, spine and rib abnormalities, small hands. MRI can also show cerebellar hypoplasia, choroid plexus papillomas, posterior fossa and other extraparenchymal cysts
300
You are evaluating a previously healthy 15 yo girl who presents with complaints of worsening headaches for 3 weeks and blurred vision for 2 days. The headache began several weeks ago and has gotten more severe over the last several days. She now describes a constant, pounding sensation all over her head. Two days ago she also developed blurred vision. She does not have a prior history of headaches or migraines. She denies photophobia, phonophobia, nausea, and vomiting. What medications can cause this?
What is oral contraceptives, steroids, vitamin A, Isotretinoin, lithium, growth hormone, nitrofurantoin, sulfa drugs, minocycline, Tamoxifen, thyroid replacement, tetracycline
300
Skin lesions associated with tuberous sclerosis complex involve all of the following, except: A. Shagreen patches B. Ash leaf spots C. Café-au-lait spots D. Angiofibromas
What is Café-au-lait spots
400
Name the three diagnostic criteria for Lennox-Gastaut Syndrome
What is 1. Multiple seizure types. 2. Intellectual disability. 3. 1.5-2.5 Hz slow spikes and waves on EEG
400
How do lamictal and depakote interact with each other?
What is depakote inhibits metabolism of lamictal, increasing its level
400
A 10-year-old boy complained of leg cramps and clumsiness. His motor development was normal. He was in the fifth grade and was performing well academically. At age seven, he had begun to walk on his toes. He had sprained his right ankle twice in the last year and had also complained of hand cramps after long periods of writing. His maternal grandfather had "weak feet" and his maternal uncle had leg braces. His mother had no complaints, except for occasional leg cramps after long walks. Two younger siblings had no neurological complaints. He appeared healthy, and his gait showed toewalking and, that he was unable to walk on his heels. His heel cords were tight and there was weakness (4/5) of the dorsiflexors of both feet. The legs showed mild atrophy of the anterior tibialis and peroneal muscles. There was no atrophy or weakness of the intrinsic hand muscle. The stretch reflexes were absent in the upper and lower limbs. The plantar responses were flexor. There was decreased pricking sensation distally in stocking distribution. The great auricular nerves were enlarged on both sides, but the left was more visible. When palpated, the ulnar and peroneal nerves were found to be enlarged. He had mild pes cavus deformity.
What is Charcot-Marie-Tooth
400
Who should you avoid prescribing propanolol
What is patients with asthma or depression
400
The brain is myelinated by what age?
What is second year
500
What is the enzyme that is deficient in pyridoxine-dependent epilepsy?
What is α-aminoadipic semialdehyde (α-AASA) dehydrogenase, also known as antiquitin
500
Which of the following has the longest half-life? A. Felbatol B. Rufinamide C. Leveitracetam D. Oxcarbazepine
What is A
500
An 18 mo girl is being evaluated in clinic for abnormal gait after a minor fall 2 months ago. Mom reported to you that she tripped and fell onto a carpeted floor. There was no LOC. Over the next few days mom noted a limp. Over the next few weeks she refused to walk and now crawled exclusively. In addition, she has had recurrent vomiting and has lost 3 pound in the past few weeks. Two weeks ago she had a URI associated with stridor and she continues to have somewhat stridulous respiration in sleep. Developmentally she did well the first year. Per mom everything was on track. She continues to gain new words but she is very difficult to understand. Her receptive language is on track. Perinatal history and family history negative. On neurologic exam, her head circumference is at 95%tile, her weight is 30%tile and height is 40%tile. There is frontal bossing but no other dysmorphic features. CN is notable for decreased facial movement, decreased gag. Motor: axial hypotonia and appendicular hypertonia. Decreased movement and strength with spontaneous tasks in proximal upper and lower extremities with some asymmetry in the lower extremities. Only antigravity movement noted in the right leg. Sensory: grossly intact. Reflexes: 3+ in the upper extremities and bilateral ankles, cross adductor in the patellar, upgoing toes bilaterally. Coordination: dysmetria R>L. Gait: required assistance with standing up, circumduction of the right leg. What is the diagnosis?
What is Alexander disease. Leukodystrophy caused by mutation of GFAP on chromosome 17. Characterized by extensive white matter abnormalities that primarily affect the frontal lobes. Infantile form commonly presents before age 2 with macrocephaly and frontal bossing, bulbar dysfunction, seizures, spasticity, and motor delays with regression. It is typically rapidly progressive, with death by age of 10. In juvenile form they may live into their 40s. The adult form presents during adolescence or early adulthood with a variety of neurologic symptoms that can mimic MS. Palatal myoclonus may be present.
500
A 10 yo girl told her mom that about a month ago she had an episode where she thought she had shrunken to 10 inches tall when she was walking. Objects appeared to be much farther away than they were. She has also lost track of time. This lasted for about 5 days and resolved spontaneously. She was not disturbed by them. She is otherwise developmentally normal. Which viral infection can cause this syndrome?
What is EBV (Alice in Wonderland syndrome)
500
An elongated skull (scaphocephaly) is caused by synostosis of which suture.
What is sagittal suture synostosis. Also called dolicocephaly Plagiocephaly- coronal suture Trigonocephaly-metopic suture
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