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WorksheetsiSMART quiz on Nervous System & Rehab disease
Total questions: 20
Worksheet time: 20mins
A 7-year old boy was seen in your clinic due to double vision that worsens during the day. On PE he had unilateral ptosis but the pupillary reaction to light was normal. He had difficulty sustaining an upward gaze. What diagnostic procedure will you do to confirm your diagnosis?
repetitive nerve stimulation
serum creatinine kinase
serum potassium
muscle biopsy
Which is TRUE of Duchenne muscular dystrophy?
histologic evidence of myopathy at birth
normal creatine kinase at birth
short stature seen in symptomatic phase
honeymoon period is due to improvement in motor milestone
Which of the following cases qualifies a diagnosis of epilepsy?
An 8-year-old male who had recurrent tonic clonic seizures during his sleep 2-3 months apart.
A 3-year-old girl who had 4 seizure events since 6 months old. In all events, she had fever.
A 10-year-old having recurrent seizures after falling off his bike.
An 11-year-old girl had one afebrile seizure prior to consult. She had two febrile seizures when she was 3yr old.
A 5-year old boy complains that objects are moving towards him. He is frightened every time he experiences it. This was also associated with diaphoresis, nausea, vomiting and on and off clicking sound on his right ear. What is your consideration?
Focal aware seizure
Benign paroxysmal vertigo of childhood
Focal to bilateral tonic-clonic seizure
Focal aware seizure and Benign paroxysmal vertigo of childhood
A 4-year old girl was brought to the clinic due to recurrent seizures. You noted several skin lesions. What is the most likely diagnosis?
Tuberous sclerosis complex
Aicardi Syndrome
Neurofibromatosis Type 1
Sturge Weber Syndrome
A 3-day old baby girl was admitted due to jaundice. she was delivered uneventful to a 20-year old G1P1 (1001) via NSD at home assisted by a midwife. On the 2nd day of life, she started to have jaundice in the face and eventually progressed all over her body. She was immediately brought to the hospital. At the ER she developed generalized tonic-clonic seizure for about 5 minutes. Your consultant asked for TBB1B2 and revealed extremely high B1. Your plan is to do double exchange transfusion. What will be the possible outcome of this neonate?
Cerebral palsy hemiplegia type
Cerebral palsy athetoid type
Cerebral palsy diplegia type
Cerebral palsy paraplegia type
A 5-year old previously healthy boy was having difficulty doing monkey bars and climbing up the stairs. On examination, he had proximal weakness, big calves and normal reflexes. He was seen by a general physician who suspected a neuromuscular condition. Which neuro-diagnostic procedure will you request initially?
serum creatinine kinase
multiplex ligation probe amplification for dystrophin gene
muscle biopsy
electromyography with rapid nerve stimulation (RNS)
A 12-year- old girl was brought to the clinic for evaluation due to seizures. You noted her unique facial findings. What is your diagnosis?
Aicardi Syndrome
Tuberous sclerosis
Von Recklinghousen disease
Sturge-Weber Syndrome
Clinical picture not consistent with a peripheral cause of hypotonia in a child
Lethargy
Feeding impairment
Respiratory distress
Profound weakness
A 15-year-old girl was brought to ER for visual loss. This was preceded by fever, headache, and eye pain. She had EBV infection 10 days ago. What is the most likely diagnosis?
central retinal artery occlusion
retinal migraine
optic neuritis
non-organic visual loss
The following are the Risk Factors NOT associated with Poor prognosis of Bacterial Meningitis :
high bacterial titers with the gram positive bacteria
absence of fever
delayed CSF sterilization
neonate age group
Which of the following is consistent with patients with Bacterial infections of the CNS?
The CSF may show Pleocytosis with monocytic predominance with increased protein and decreased glucose
Hyponatremia , dehydration and inappropriate of antidiuretic hormone secretion are not common to occur.
Seizures complicate 20-30percent of bacterial meningitis in children.
Deafness is highest risk as complication if meningitis is caused by Streptococcal pneumoniae.
A 4 weeks old baby boy was admitted due to 3 days fever and focal seizures. As the attending Pediatrician, part of your differential is Meningitis. What is the most plausible etiologic agent would you consider for you to institute proper therapeutic management.
H. influenza
E. coli
Listeria Monocytogenes
N. Meningitidis
About Peroxisomal disorders, which of the following statement is TRUE?
X Linked adrenoleukodystrophy is a common autosomal recessive perosixomal disorder.
Increasing number of peroxisomes are pathognomonic for Disorder of Peroxisome Biogenesis.
The nervous system displays 2 types of ALD lesions. In the mild cerebral form, demyelination manifested by the accumulation of perivascular lymphocytes and in the rapidly progressive adult form, adrenomyeloneuropathy is the main finding .
The adrenal dysfunction is a direct consequence of the accumulation of VLCFAs.
Perinatal Arterial Ischemic Stroke does NOT correctly described in which of the following statement?
Acute symptomatic neonatal AIS presents with focal seizures within 24-28 hr of birth.
Hand dominance within the first year of life is abnormal and may be the result of perinatal stroke.
Neuro Imaging reveals focal encephalomalacia in an arterial territory, typically as lesions in the anterior cerebral artery.
Stroke recurrence rates in subsequent pregnancies are extremely low in the absence of a familial prothrombotic disorder.
Cerebral Sinus Veno-Thrombosis is NOT associated with which of the following finding?
Cerebral venous drainage occurs via the cortical veins, superior sagittal sinus and the internal cerebral veins, straight sinus converge at the torcula to exit the cranial vault via the paired transverse and sigmoid sinuses and jugular veins.
In CSVT, the thrombotic occlusion of these venous structures can create increased intracranial pressure, cerebral edema, and venous stroke.
CSVT may be more common in children than in adults, and risk is greatest in the early toddler period.
Anticoagulation therapy with unfractionated or Low Molecular weight Heparin plays an important role in childhood CSVT treatment.
A 2 month old baby was admitted due to poor suck. On physical exam at the emergency room you noted the patient to be underweight, microcephalic and with failure to thrive. Gross motor exam showed patient is hypotonic and muscle mass is thin in the trunk and extremities. Pupils are equally brisk reactive to light . Head control is poor with noticeable neck weakness and marked axial hypotonia. Tendon stretch reflexes are absent. Based on these clinical findings, which of the following is your most plausible impression:
Acute Flaccid paralysis – Polio
Acute Disseminated Myelitis (ADEM)
Fukuyama Muscular Dystrophy
Dejerine Sottas Disease ( HMSN Type III)
This is consistent with Herpes Simplex Viral Meningoencephalitis?
Spike and slow waves epileptiform discharges seen at the frontal and superior temporal lobe of the brain as the most common sites of predilection.
Age, disease duration, and level of consciousness at the onset of therapy were proved to be the major determinants of clinical outcome.
In patients with HSE, CSF findings are non diagnostic, being similar in patients with confirmed disease or diseases that mimic HSE.
A completely normal CSF does NOT rule out encephalitis
Treatment modalities Employed in patients with brain abscess include:
Glucocorticoids cannot be given routinely to patients with brain abscesses. Dexamethasone should be tapered as rapidly as possible to avoid delaying the natural process of encapsulation of the abscess.
Patients should not receive prophylactic anticonvulsant therapy because of the high risk (~35%) of focal or generalized seizures.
Anticonvulsant therapy is continued for at least 6 months after resolution of the abscess, and decisions regarding withdrawal are then based on the EEG.
Anticonvulsant therapy can be slowly withdrawn, with close follow-up and repeat EEG after the medication has been discontinued if clinically no seizures with prior Abnormal EEG.
A 4-month-old girl was admitted because of severe hypotonia. She was born with a birth weight 2,100 g at 38 weeks and 5 days of gestational age. Her Apgar score was 7 at 1 minute and 9 at 5 minutes.
She was a child of nonconsanguineous parents. After birth, she was admitted for a workup examination of intrauterine growth retardation, but discharged because no specific abnormality was observed.
At the age of 4 months, consulted due to poor neck muscle tone. She looked alert, showed social smiling and babbling, and could contact her eyes with her mothers eyes, but her posture was severely
hypotonic. There was no sign of ophthalmoplegia or of equinovarus. Deep tendon reflexes were absent. Upper extremity power was grade 3 and lower extremity power was grade 2. Tongue fasciculation was observed. Most closest explanation we can think of as differential would include:
Acute motor and sensory axonal neuropathy
Due to abnormal continuation of fetal apoptosis of spinal anterior horn cells
Demyelinating process
Ongoing central nervous system inflammatory process
