Board2017 MCQs

Questions and Answers

A patient with hypokalemic periodic paralysis should avoid which of the following triggers?

Beta-blockers

Low sodium intake

Physical rest after exercise

High carbohydrate meals (correct)

Which of the following best describes the "second wind" phenomenon seen in McArdle disease?

Improved exercise tolerance after a brief rest during activity (correct)

Sudden onset of muscle weakness after meals

Immediate fatigue with minimal exertion

Muscle cramps that worsen with continued activity

Regarding a trauma patient with right-sided cerebellar signs and cranial nerve palsy, which area is likely affected?

Left pons

Right medulla

Right midbrain (correct)

Left cerebellar hemisphere

In a patient with Horner syndrome, which of the following is a correct statement?

Anhidrosis occurs in lesions of first and second-order neurons Signup and view all the answers

Which antiepileptic drug is excreted in breast milk?

Ethosuximide Signup and view all the answers

Regarding treatment for a child with Juvenile Myoclonic Epilepsy (JME), which of the following is correct?

Treatment is usually lifelong Signup and view all the answers

Which of the following increases the risk to develop another febrile seizure in a child with febrile seizures?

All of the above Signup and view all the answers

Regarding NF1, a child with Café au lait spots, what should be done next?

Ophthalmology examination to assess for optic pathway gliomas Signup and view all the answers

What is the best management for a patient with an arachnoid cyst causing intractable seizures?

Surgical removal or fenestration of the cyst without VP shunt Signup and view all the answers

In the case of a child post-RTA with a CT brain showing a skull fracture, what should be done next?

Neurosurgery consultation Signup and view all the answers

What is the most probable diagnosis for a child who developed headache and photophobia after being hit on the head with a ball?

Concussion Signup and view all the answers

A patient with a history of worsening verbal development and EEG showing epileptiform discharges during sleep is most likely diagnosed with?

Landau-Kleffner syndrome Signup and view all the answers

A patient presenting with frequent headaches that are relieved by rest and exacerbated by lack of sleep should be advised to?

Improve sleep hygiene practices Signup and view all the answers

What is the most likely diagnosis for a child who presents with an inability to abduct the left eye and retraction of the globe upon attempted adduction?

Duane syndrome Signup and view all the answers

In a patient undergoing chemotherapy for leukemia who develops seizures and visual symptoms, the most probable cause of PRES is?

Methotrexate toxicity Signup and view all the answers

A patient with unexplained behavioral changes shows improvement with steroids. Which investigation is most appropriate?

Anti-thyroid antibodies (TPO, thyroglobulin) and TSH levels Signup and view all the answers

Which antiepileptic medication should be avoided in a patient with Dravet syndrome (SCN1A mutation)?

Carbamazepine Signup and view all the answers

If a patient with anti-NMDA receptor encephalitis did not improve with high-dose steroids, what is the next best step?

Start plasma exchange Signup and view all the answers

A child with esotropia and impaired vertical gaze after trauma is most likely diagnosed with?

Basal skull fracture Signup and view all the answers

What is the EEG pattern seen in a patient with Ohtahara syndrome?

Burst suppression pattern during both sleep and wakefulness Signup and view all the answers

A neonate has episodes of apnea and sudden jerky movements with touch. What is the most likely diagnosis?

Hyperekplexia Signup and view all the answers

In the context of Tuberous Sclerosis, which of the following statements about everolimus is correct?

It is an mTOR inhibitor used for treating subependymal giant cell astrocytomas (SEGAs) Signup and view all the answers

Which of the following syndromes can be treated with cannabidiol (marijuana)?

Dravet syndrome Signup and view all the answers

A trauma patient with right-sided cerebellar signs and cranial nerve palsy shows which area is likely affected?

Right midbrain Signup and view all the answers

Regarding patient identification in the hospital, which method is correct?

Use both name and MRN Signup and view all the answers

An 8-year-old girl had two attacks of abnormal movements after minor trauma. What is the likely diagnosis?

Reflex anoxic seizures Signup and view all the answers

If an infant presents with seizures and an EEG showing burst-suppression patterns, what is the most likely diagnosis?

Ohtahara syndrome (Early Infantile Epileptic Encephalopathy) Signup and view all the answers

In the case of a previously normal 18-month-old girl with developmental regression, what is the most likely diagnosis?

Rett syndrome Signup and view all the answers

A patient with a large brain abscess on MRI had a negative lumbar puncture. What should be done next?

Aspiration and culture/sensitivity Signup and view all the answers

What is the treatment for a child diagnosed with dopa-responsive dystonia?

Levodopa-carbidopa (Sinemet) Signup and view all the answers

Which of the following is associated with dopa-responsive dystonia (DYT5)?

GTP cyclohydrolase 1 gene mutation Signup and view all the answers

After a minor head injury during sports, when can a patient return to playing football?

After being symptom-free for at least 5 days and following a gradual return-to-play protocol Signup and view all the answers

What should be done for a child post-RTA with a CT brain showing a skull fracture?

Neurosurgery consultation Signup and view all the answers

In a child diagnosed with metachromatic leukodystrophy (MLD), what is the deficient enzyme?

Arylsulfatase A Signup and view all the answers

When a child presents with seizures and neuroimaging shows holoprosencephaly, what investigation should be ordered?

Endocrine evaluation including pituitary hormones and electrolytes Signup and view all the answers

A junior resident talks about a senior who humiliates her in rounds. What is the correct course of action?

Take the issue seriously and address it with appropriate supervisory staff or department leadership Signup and view all the answers

If a colleague asks you to prescribe an antipsychotic medication he is using, what should you do?

Refuse and advise him to seek appropriate medical care Signup and view all the answers

For a patient with tension-type headaches, which of the following treatments is correct?

Preventive therapy includes amitriptyline Signup and view all the answers

What is the next step for a patient reporting frequent headaches worsening with lack of sleep?

Advise to improve sleep hygiene Signup and view all the answers

What is the most appropriate step for a patient with medication overuse headache?

Abruptly discontinue the overused medication Signup and view all the answers

What affects cognitive function prognosis for a child diagnosed with medulloblastoma?

It depends on several factors including age, treatment modalities, and postoperative complications Signup and view all the answers

For a child in the PICU post-neurosurgery who is extubated but not talking, what is correct regarding this condition?

It is a transient condition known as cerebellar mutism Signup and view all the answers

What is the risk of recurrence for future pregnancies in a child born with myelomeningocele?

2-5% Signup and view all the answers

If the mother of a neonate born with myelomeningocele indicates that her husband hit her during the 34th week of gestation, what should you tell her?

An injury at that gestational age has nothing to do with the presentation Signup and view all the answers

A child presenting with headaches and MRI shows cerebellar tonsillar herniation 6 mm below the foramen magnum, what type of Chiari malformation is this?

Chiari I malformation Signup and view all the answers

MRI spine shows an extensive lesion involving the cervicothoracic area. What would you expect to find on examination?

Weakness in upper limbs and muscle atrophy in hands Signup and view all the answers

The treatment for a patient with Guillain-Barré Syndrome (GBS) is?

Intravenous immunoglobulin (IVIG) Signup and view all the answers

For a 15-year-old patient with cognitive decline, myoclonic jerks, and an EEG shows periodic complexes, what investigation will help confirm the diagnosis?

Elevated anti-measles IgG antibodies in CSF Signup and view all the answers

A 15-year-old patient has been having academic difficulties accompanied by quick jerking movements. The EEG shows background slowing and generalized periodic epileptiform discharges every 4 seconds. Which of the following is least likely to be found in this condition?

Cowdry B bodies Signup and view all the answers

In Duchenne muscular dystrophy (DMD), which statement regarding the genetic mutation is correct?

DMD is caused by an out-of-frame mutation in the dystrophin gene Signup and view all the answers

In Horner syndrome, anhidrosis occurs in lesions involving which neurons?

First and second-order neurons Signup and view all the answers

In a neonate with episodes of apnea and sudden jerky movement with touch, what is the most likely diagnosis?

Hyperekplexia Signup and view all the answers

Which of the following syndromes can be treated with cannabidiol (CBD)?

Dravet syndrome Signup and view all the answers

What are you going to do for a child with multiple types of seizures including myoclonic, difficult to control on levetiracetam and lamotrigine with high LFT?

Add carbamazepine Signup and view all the answers

What to give to a patient who deteriorated after tonsillectomy under anesthesia due to Central Core Disease?

IV Dantrolene Signup and view all the answers

What to do next for a 10-year-old girl diagnosed with MS who did not improve after high dose steroids?

Plasma exchange Signup and view all the answers

What to do for a patient with opsoclonus myoclonus diagnosed with Neuroblastoma who presented again with the same symptoms?

IV Ig Signup and view all the answers

What to do next for a patient with recurrent Staphylococcus aureus meningitis who also has rhinorrhea?

MRI brain Signup and view all the answers

What to do for a patient with a sore throat and high ASO titer who developed chorea?

Infectious Disease Signup and view all the answers

When to start treatment for a 6-year-old child diagnosed with BECTs?

Older age group Signup and view all the answers

What is the most probable cause for a patient with leukemia who develops bilateral leg pain even with touch?

Side effect of chemotherapy Signup and view all the answers

What to do next for a child with macrocephaly and normal development?

Measure parents' head circumference Signup and view all the answers

Where is the localization of the aura of palpitation and sweating?

Insular cortex Signup and view all the answers

What are you going to do for a patient with ADHD on methylphenidate who developed tics?

Discontinue methylphenidate Signup and view all the answers

Which AED to start for a patient post renal transplant who developed focal seizures with automatism?

Phenytoin Signup and view all the answers

What to do next for a neonate found to have Café au lait spots?

Echocardiogram Signup and view all the answers

What is the correct answer for a neonate with myoclonus, not in clusters?

KCNQ2 channel mutation (Benign familial neonatal seizures) Signup and view all the answers

What is the most probable diagnosis for a patient with episodes of one-sided headache with lacrimation that improves after indomethacin?

Paroxysmal hemicrania Signup and view all the answers

Which of the following is most likely the cause of leg paresthesias in a patient with inflammatory bowel disease?

Selenium deficiency Signup and view all the answers

What is the diagnosis for a child who developed febrile and afebrile seizures on the right and left sides?

Dravet syndrome (SCN1A mutation) Signup and view all the answers

What is the most likely diagnosis for a neonate with apnea and sudden jerky movement upon touch?

Hyperekplexia Signup and view all the answers

Which antiepileptic drug is excreted in breast milk?

Ethosuximide Signup and view all the answers

What is the treatment for dopa-responsive dystonia?

Levodopa-carbidopa (Sinemet) Signup and view all the answers

What is the diagnosis for a patient with episodes of alternating weakness aggravated by crying?

Moyamoya disease Signup and view all the answers

Which of the following is a major criterion for Tuberous Sclerosis?

Hypomelanotic macules (≥3), Angiofibromas (≥3), Shagreen patch, etc. Signup and view all the answers

In which condition is the 'second wind' phenomenon seen?

McArdle disease (Glycogen storage disease type V) Signup and view all the answers

Myokymia is seen in which conditions?

Episodic ataxia type I Signup and view all the answers

What to do next for a patient with Guillain-Barré syndrome who started to improve without treatment?

No need for treatment currently Signup and view all the answers

What to do for a colleague who asks you to prescribe an antipsychotic medication he is using?

Advise him to seek appropriate medical care and refrain from prescribing. Signup and view all the answers

What should you do if a junior resident talks to you about a senior humiliating her in rounds?

Take the issue seriously and address it with appropriate supervisory staff or department leadership. Signup and view all the answers

What is the next step for a patient with a headache related to lack of sleep?

Advise to improve sleep hygiene Signup and view all the answers

What is the next step for a patient with medication overuse headache?

Abruptly discontinue the overused medication Signup and view all the answers

What is the most probable diagnosis for a child with a history of febrile seizures who developed afebrile seizures occasionally on different sides?

Dravet syndrome (SCN1A mutation) Signup and view all the answers

What is the most likely diagnosis for a neonate with myoclonus and a sibling with similar history but now normal?

KCNQ2 channel mutation (Benign familial neonatal seizures) Signup and view all the answers

What is the most probable cause of transient cerebral arteriopathy?

Varicella zoster virus Signup and view all the answers

What should be ordered for a patient with visual symptoms following an URTI who requires MRI brain?

R/O demyelinating disease Signup and view all the answers

What is the deficient enzyme in metachromatic leukodystrophy (MLD)?

Arylsulfatase A Signup and view all the answers

What is the treatment for a patient with Guillain-Barré syndrome?

Intravenous immunoglobulin (IVIG) Signup and view all the answers

What test to order for a patient with behavior changes responsive to steroids?

TSH and antithyroid antibodies (TPO, thyroglobulin) Signup and view all the answers

What is the most probable diagnosis for a patient with anemia, hepatosplenomegaly, and vertical gaze palsy?

Niemann-Pick disease type C Signup and view all the answers

Which syndrome can be treated with marijuana (cannabidiol)?

Dravet syndrome Signup and view all the answers

What is the diagnosis for an 8-year-old girl with two attacks of loss of consciousness triggered by minor injury?

Reflex anoxic seizures Signup and view all the answers

What is the recurrence risk for future pregnancies for a child born with myelomeningocele?

2-5% Signup and view all the answers

What do you tell a mother of a neonate born with myelomeningocele whose husband hit her during pregnancy?

An injury at that gestational age has nothing to do with the presentation Signup and view all the answers

What is the correct inheritance pattern for Neurofibromatosis type 1 (NF1)?

It is autosomal dominant with high penetrance and variable expression Signup and view all the answers

What is the correct answer regarding Wilson disease and its serum levels?

Low serum ceruloplasmin levels and decreased serum copper concentration Signup and view all the answers

What is the most common cause of transient cerebral arteriopathy?

Varicella zoster virus Signup and view all the answers

Which patient requires PICU admission for spinal injury post RTA?

Injury above T10 Signup and view all the answers

What is the diagnosis for an 11-year-old girl presenting with unsteadiness, seizures, and impaired upward gaze?

Cataplexy Signup and view all the answers

What causes a patient with anemia, hepatosplenomegaly, and vertical gaze palsy?

Niemann-Pick disease type C Signup and view all the answers

What is the diagnosis for a child with two attacks of abnormal movements following minor injuries?

Reflex anoxic seizures Signup and view all the answers

What is the correct approach for a patient with medication overuse headache?

Abruptly discontinue the overused medication Signup and view all the answers

Which of the following conditions is associated with loss of previously acquired language skills in children?

Landau-Kleffner syndrome Signup and view all the answers

What are the features of anti-NMDA receptor encephalitis?

Orofacial dyskinesias Signup and view all the answers

What is a major criterion for Tuberous Sclerosis?

Cardiac rhabdomyoma Signup and view all the answers

What differentiates Duchenne from Becker muscular dystrophy?

Duchenne is caused by an out-of-frame mutation, Becker by an in-frame mutation Signup and view all the answers

In Horner syndrome, anhidrosis occurs in lesions of which neurons?

First and second-order neurons Signup and view all the answers

What is correct regarding treatment for a patient with tension-type headache?

Abortive therapy includes acetaminophen or NSAIDs Signup and view all the answers

What is correct regarding a mute postoperative patient with cerebellar tumor?

It is transient and typically improves over weeks to months Signup and view all the answers

What to tell a mother of a neonate with myelomeningocele after a traumatic event in pregnancy?

An injury at that gestational age has nothing to do with the presentation Signup and view all the answers

What is the inheritance pattern of Neurofibromatosis type 1?

It is autosomal dominant with high penetrance and variable expression Signup and view all the answers

What is the serum concentration characteristic of Wilson disease?

Low serum ceruloplasmin levels and decreased serum copper concentration Signup and view all the answers

What is the most common cause of transient cerebral arteriopathy?

Varicella zoster virus Signup and view all the answers

What is required for a patient with spinal injury post RTA who has an injury above T10?

Injury above T10 Signup and view all the answers

What is associated with Niemann-Pick disease type C?

Anemia, hepatosplenomegaly, and vertical gaze palsy Signup and view all the answers

What is the diagnosis for a child with two attacks of abnormal movements triggered by minor injury?

Reflex anoxic seizures Signup and view all the answers

What should be done for a patient with medication overuse headache?

Abruptly discontinue the overused medication Signup and view all the answers

Which syndrome is known for loss of previously acquired language skills in children?

Landau-Kleffner syndrome Signup and view all the answers

Which features indicate anti-NMDA receptor encephalitis?

Orofacial dyskinesias Signup and view all the answers

What is a major criterion for Tuberous Sclerosis?

Cardiac rhabdomyoma Signup and view all the answers

What informs the distinction between Duchenne and Becker muscular dystrophy?

Duchenne is caused by an out-of-frame mutation, Becker by an in-frame mutation Signup and view all the answers

Anhidrosis in Horner syndrome is identified in which neurons?

First and second-order neurons Signup and view all the answers

In treating tension-type headache, what statement is accurate?

Abortive therapy includes acetaminophen or NSAIDs Signup and view all the answers

What is accurate about a mute patient following cerebellar tumor surgery?

It is transient and typically improves over weeks to months Signup and view all the answers

Study Notes

Seizure Management and Investigation

Difficult-to-control seizures in a child with myoclonic types require maximizing levetiracetam therapy.
Post-anesthesia complications in Central Core Disease are managed with IV Dantrolene.
In multiple sclerosis diagnosed cases presenting with extensive MRI lesions, plasma exchange is preferred when high-dose steroids are ineffective.
Recurrent opsoclonus myoclonus in neuroblastoma patients should be treated with IV immunoglobulin.

Infection and Consultation Protocols

In patients with recurrent Staphylococcus aureus meningitis presenting rhinorrhea, a thin cuts CT brain is indicated for investigation.
Following sore throat and high ASO titer with resultant chorea, initial consultation should be directed to cardiology.

Pediatric Seizure Disorders

In cases of benign epilepsy with centrotemporal spikes (BECTs), treatment is indicated if seizures are frequent.
Development of chorea post-streptococcal infection warrants consideration for autoimmune etiology and potential rheumatology involvement.

Asymptomatic Conditions and Diagnostics

In a child with macrocephaly and normal development, measuring parental head circumference can provide valuable context.
Café au lait spots in a neonate necessitate checking parents for similar manifestations to assess genetic implications.

Neurological Symptoms and Treatments

Hyperekplexia is characterized by neonatal myoclonus without clusters and normal prior development.
Paroxysmal hemicrania diagnosis is likely when headaches improve after indomethacin administration.
Vitamin B12 deficiency is indicated by leg paresthesias and other neurological signs in patients with inflammatory bowel disease.

Pediatric Disorders and Their Assessments

Reflex anoxic seizures should be diagnosed in a child with unintentional injury causing loss of consciousness and drowsiness.
Investigating holoprosencephaly requires an endocrine evaluation to assess associated hormonal disturbances.
Patients with metachromatic leukodystrophy show deficiency in arylsulfatase A enzyme.

Management and Treatment Protocols

Abrupt discontinuation of overused medications is critical in medication overuse headache cases.
Intravenous immunoglobulin is the recommended treatment for Guillain-Barré Syndrome, while steroids are not indicated unless severe.
In Neiman-Pick disease type C, diagnosis involves identifying anemia and hepatosplenomegaly alongside neurological signs.

Genetic and Inheritance Considerations

Neurofibromatosis type 1 follows an autosomal dominant inheritance pattern with high penetrance and variable expression.
Duchenne muscular dystrophy results from an out-of-frame mutation; Becker muscular dystrophy involves in-frame mutations.

Other Significant Findings

The second wind phenomenon in McArdle disease is associated with improved exercise tolerance after brief pauses, illustrating a unique body response during physical activity.
Anhidrosis in Horner syndrome is indicative of first and second-order neuron lesions, affecting sympathetic pathways.

Prognosis and Risk Assessment

Myelomeningocele recurrence risk is approximately 2-5% in subsequent pregnancies.
Cognitive function prognosis post-medulloblastoma treatment relies on various factors including age, treatment modalities, and complications.

Emotional and Behavioral Concerns

Behavioral changes responsive to steroid therapy may require TSH and antithyroid antibody tests to rule out underlying thyroid disorders.
Junior residents facing bullying must report the incidents to supervisory staff for proper intervention.

Epilepsy and Antiepileptic Drugs

Long-term treatment is usually necessary for Juvenile Myoclonic Epilepsy, as seizures typically do not resolve without ongoing management.
Ethosuximide is notably excreted in breast milk, requiring caution in breastfeeding mothers with epilepsy.

This condensed view emphasizes critical information regarding clinical conditions, treatments, neurologic assessments, and familial contexts, ensuring a comprehensive understanding for medical students and professionals.### Febrile Seizures and NF1

First febrile seizure occurs before 12 months of age, may indicate risk of epilepsy.
Complex febrile seizures exhibit features that increase risk.
Family history of epilepsy is a significant risk factor.

Neurofibromatosis Type 1 (NF1)

In children with Café au lait spots, the next step is an ophthalmology examination to check for optic pathway gliomas.

Arachnoid Cyst and Seizures

Best management for an arachnoid cyst causing seizures is surgical removal or fenestration, rather than VP shunt placement.

Head Trauma and Imaging

Post-RTA children with skull fractures and no neurological deficits should receive a neurosurgery consultation.
Symptoms following minor head trauma, such as headache and photophobia, suggest a concussion.

Seizure Disorders

EEG showing epileptiform discharges during sleep and language regression indicates Landau-Kleffner syndrome.
Burst suppression pattern on EEG is characteristic of Ohtahara syndrome (Early Infantile Epileptic Encephalopathy).

Medications and Treatment

Carbamazepine is contraindicated in Dravet syndrome (SCN1A mutation).
Everolimus, an mTOR inhibitor, is used for treating subependymal giant cell astrocytomas (SEGAs) in Tuberous Sclerosis.
CBD (cannabidiol) is effective for treating Dravet syndrome.

Behavioral and Psychological Issues

In cases of unexplained behavioral changes with steroid response, testing anti-thyroid antibodies is appropriate.

Neurological Assessment

Cerebellar signs and cranial nerve palsy post-trauma likely indicate right midbrain injury.
Persistent speech loss in a PICU patient post-neurosurgery is often transient, known as cerebellar mutism.

Chiari Malformation and Myelomeningocele

Cerebellar tonsillar herniation of 6 mm below the foramen magnum indicates Chiari I malformation.
Myelomeningocele has a recurrence risk of 2-5% in future pregnancies.

Treatment for Conditions

Intravenous immunoglobulin (IVIG) is the treatment for Guillain-Barré syndrome.
Levodopa-carbidopa (Sinemet) is indicated for dopa-responsive dystonia.
Abrupt discontinuation of overused headache medications is necessary for medication overuse headache.

Genetic and Infectious Disease Insights

Duchenne muscular dystrophy results from an out-of-frame mutation in the dystrophin gene.
Elevation of anti-measles IgG antibodies in CSF helps confirm diagnosis in patients with cognitive decline and myoclonic jerks.

Eye and Neurological Conditions

Symptoms of esotropia paired with vertical gaze abnormalities suggest a basal skull fracture.
Apnea and sudden jerky movements in newborns are indicative of hyperekplexia.

Psychiatric Considerations

In situations of workplace bullying, it is advised to escalate the issue to supervisory staff rather than ignoring it.

Laboratory and Imaging Protocols

When faced with a brain abscess, aspiration and culture/sensitivity testing follow a negative lumbar puncture.
Initial endocrine evaluation is appropriate for children with seizures and holoprosencephaly.

Miscellaneous

Chiari malformation types vary, and clarity on the correct type is essential for appropriate management.
Understanding of symptom patterns and the body’s response to injury is crucial for accurate diagnosis and treatment pathways.

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Description

Test your knowledge on the management of pediatric seizure disorders and related investigations. This quiz covers treatment protocols for conditions like myoclonic seizures, post-anesthesia complications, and the role of consultations in related infections. Ensure you understand the nuances in treatment and diagnosis for these cases.