More Neuro PREP Questions

Neurology Rotation Lecture Series

Last Updated by Lindsay Pagano

Summer 2013

PREP question

A mother bring in her otherwise healthy 4 year old son because of problems walking. He has not wanted to walk since this morning and only stands on tip-toe or crawls. He had a URI with a fever 1 week ago. On PE, he appears well, but complains of leg pains bilaterally. He reports no back pain. His calf muscles are tender to palpation, but joints are not warm, red or swollen. Patellar and ankle jerk reflexes are present bilaterally, and there is no clonus.

Of the following, the MOST appropriate initial diagnostic procedure is:

A.Antinuclear antibody measurement

B.Lumbar puncture

C.Magnetic resonance imaging of the spine

D.Nerve conduction studies/electromygraphy

E.Serum creatine kinase measurement

E. CKEvaluating weakness•Symptoms localize to the muscle (no reflex changes, no bowel or bladder involvement, no sensory changes, no focal unilateral findings)

•Acute myositis: CK can be >1000 u/L; MC after URIs, especially influenza; self limited, no specific treatment

•Regarding the other choices:

A. ANA: no chronicity, no joint involvement, no rash

B. LP: no current signs of infection

C. MRI spine: does not localize to spine, as above

D. NCV/EMG: acute pathology, likely unrevealing, no other findings on exam

PREP question

You are working in the newborn nursery when the nurse asks you to evaluate a girl who has just been admitted. According to her records, her mother had good prenatal care and results of prenatal laboratory evaluations were normal. The infant is vigorous and pink.

PE: normal except for the following skin finding

Of the following, the condition that is most likely to be associated with this skin lesion is:

A.Kasabach-Merritt syndrome

B.Neurofibromatosis type 1

C.Osler-Weber-Rendu disease

D.Sturge-Weber syndrome

E.Tuberous sclerosis complex

D. Sturge-Weber syndrome

Sturge Weber

•Port wine stains: 5-10% of children with these have this condition; V1 distribution

•IL brain findings

•60% with glaucoma

•PWS can be treated with pulsed dye laser

•Regarding the other choices:

A. Kasabach-Merritt: large, atypical hemangiomas (eg hemangioendotheliomas), thrombocytpenia

B. NF1: no associated hemangiomav (café au lait)

C. Osler-Weber-Rendu: hereditary hemorrhagic telangiectasia, recurrent epistaxis

E. TS: no associated hemangioma (hypopig macules)

PREP question

During the health supervision visit for a healthy 4 month old male, you notice that his head circumference is 46 cm (>98th percentile) and his height and weight are at the 50th percentile. He has mild frontal bossing and widely split cranial sutures. The fontanelle is flat. Arm and leg movements, tone and reflexes are normal. In reviewing prior growth parameters, you note that his head circumference was at the 75th percentiles at birth and the 90th percentile at 2 months.

Of the following, the MOST helpful next diagnostic procedure is:

A.Electroencephalography

B.Head ultrasound

C.Lumbar pucture with manometry

D.Plain radiography of the skull

E.Three-dimensional head computed tomography scan

B. HUS

Macrocephaly

•Crossing growth curves

•No other sign of neurologic disease

•DDx per PREP: hydrocephalus, arachnoid cyst, parenchymal brain lesions, subdural hematomas, neurodegenerative disease

•Regarding the other choices:

A. EEG: no periods of altered awareness or concerning spells

C. LP: no clinical signs of increased ICP, no infectious symptoms, sutures open

D. Xray: only assesses bone

E. 3D Cthead: no signs of craniosyostosis

PREP question

A 15 year old boy presents to the emergency department after an apparent seizure. He had a sudden arrest of normal activity at school, with posturing on the left side, eye deviation, and loss of consciousness for about 1 minute, followed by confusion.

On PE, he is fully oriented and answers questions appropriately. He is afebrile. Results of cranial nerve examination, motor examination and gait evaluation.

Of the following, the procedure that is MOST likely to establish the cause of the seizure is:

A.Brain magnetic resonance imaging with contrast

B.Electroencephalography

C.Lumbar puncture

D.Noncontrast head computerized tomography scan

E.Urine toxicology screen

A. MRIbrain with contrast

First time seizure

•Concerning features: age, unprovoked, focal

•Workup: EEG, MRI

•Regarding the other choices:

B. EEG: first seizure, known focal seizure, need to discern potential causes before confirming localizing interictal findings

C. LP: returned to normal mental status, no signs of meningitis

D. noncon CThead: back to baseline

E. urine tox: focal seizure, not MOST important

PREP question

During the health supervision visit for an 18 month old boy, his parents express concern that he is vocalizing but not saying any real words. He is holding a small piece of string that he moves back and forth repeatedly. When you call his name, he does not respond. You point to the light in the room and say “look,” but he continues to look at the string with a sideways glance. You try to get him to look at you, but he avoids eye contact.

Of the following, the MOST likely diagnosis for this boy is:

A.Asperger disorder

B.Autistic disorder

C.Expressive/receptive language disorder

D.Obsessive-compulsive disorder

E.Rett syndrome

B. Autistic disorder

Autism Spectrum Disorder•Impairment in 3 areas: reciprocal social interactions, verbal and nonverbal communication, range of activities or interests

•Social interactions: unable to share interests, share play

•Communication: speech delay or regression, echolalia, repetitive speech

•Activities: repetitive play, limited imaginative play, focus on sensory features of object

•Other: difficulty with transition, repetitive hand or body movements (stereotypy)

•Regarding the other choices:

A. Asperger disorder: preserved cognitive and language functioning

C. Expressive/receptive language disorder: doesn’t account for social limitations

D. Obsessive-compulsive disorder: doesn’t account for language, social impairment

E. Rett syndrome: 6-18 months of age onset with slowed motor development, cognitive and speech changes at 1-4 year olds

PREP question

A 12 year old male comes to your clinic for his health supervision visit. He has been healthy in the past and is doing well in special education classes at school. His only complaint at this visit is the development of “bad acne” over the last year. He has tried over-the-counter benzoyl peroxide without relief.

PE reveals multiple 1-2 mm reddish-brown, firm papules over his nose and cheeks and extending up to the lower eyelids.

Of the following, the MOST likely underlying cause of this finding is:

A.Acne vulgaris

B.Neurofibromatosis type 1

C.Periorifacial dermatitis

D.Systemic lupus erythematosus

E.Tuberous sclerosis complex

E. TSC

Tuberous Sclerosis Complex

•Skin: angiofibromas (aka adenoma sebaceum), hypopigmented macules (ash-leaf macules), periungual fibromas

•Regarding the other choices:

A. acne vulgaris: comedones, papules, pustules

B. NF1: café au lait

C. periorificial dermatitis: young women, discrete erythematous papules and pustules in the nasolabial folds and chin

D. SLE: malar erythema (well circumscribed plaques)

PREP question

A well nourished, healthy child comes to your office for his two year health supervision visit. You find that he has failed an autism-specific screening tool (the Modified Checklist for Autism in Toddlers [MCHAT]) completed by his parents while in the waiting room. Results of a presvious audiology evaluation are normal. His head circumference and growth parameners are unremarkable, and the neurologic exam produces nonfocal findings. Muscle mass, strength and tone are within normal limits. His DTRs are normal.

Of the following, the MOST appropriate next step is:

A.Early intervention referral

B.Genetics evaluation

C.Head magnetic resonance imaging

D.Metabolic evaluation

E.Sleep-deprived electroencephalography

A. Early intervention referral

Autism spectrum disorder– MCHAT: sens 0.85, spec 0.93

– Associated medical disorders: TS, fragile X, FAS, Angelmans, Rett

•Regarding the other options:

B. Genetics referral: appropriate, esp with family history, but early intervention will improve prognosis

C. MRIbrain: not indicated with normal PE

D. Metabolic evaluation: few reports of association with ASD

E. sleep-deprived EEG: not indicated, no concern for seizure