NEUROLOGY
Post Graduate CME
OSCE - Neurology
Bai Jerbai Wadia Hospital for Chidren,
Mumbai
1) 4 yrs old, boy , FTND, Normal motor,
mental and language milestones.
Complaints from school aggressive,
cant sit in one place, restless,
forgetful and impatient, problems with
friends, fights
Diagnosis?
3 cardinal features of this condition
2 treatment options
ADHD
Inattention, hyperactivity and impulsivity
Medications (methylphenidate,
amphetamines, fluoxitine, atomoxitine),
Behavior therapy
2)This child has
epilepsy, MR, ataxia,
unprovoked laughter,
severe speech delay
Diagnosis?
Chromosome involved
Inheritance
Name a syndrome with
the same chromosomal
abnormality
Angelman syndrome
15q11.2-12
Uniparental disomy usually maternal (6075%)
- occ. paternal
- normal chromosome
? Dominant mutations
Prader Willi 15q11-13 (paternal origin)
3) 2 yrs old child born prematurely [28wks]
comes with complaint of delayed
achievement of motor milestones.
Examination shows hypertonia with brisk
reflexes in all 4 limbs more marked in lower
limbs.
What is the diagnosis? Define.
What is the MRI picture?
What is the treatment and when do you start it?
Name 2 conditions this child will be at risk for?
Spastic Diplegia. Increased tone in all 4
extremities and face. LL>UL> face
Periventricular leukomalacia
Early intervention
Learning disabilities,
Visual concerns
Epilepsy
Delayed mental development
Behavior concerns
4.A full term ,male child develops jaundice
on day 3 of life, (S. bilirubin 34 mg%)and
undergoes an exchange transfusion .
What is the immediate complication likely to
occur?
Where is the anatomical abnormality?
What are the long term complications?
Name 1 investigations you would insist on
after discharge?
Kernicterus
Globus pallidus, dentate nucleus, cerebellar
vermis, cochlear nuclei
Choreoathetoid cerebral palsy, dystonic /
dyskinetic CP, Sensorineural deafness,
Audiometry testing
5. A child with apparently normal birth
history :MS- social smile at 3 mths,
Sitting at 8 months, rt hand
preference at 9 mths, walking
independently at 1.6 yrs.
What is your diagnosis?
What investigation will you do?
What relevant maternal history will you
ask for?
Congenital hemiplegia
MRI brain
Maternal injury, threatened abortion,
APC resistance, coagulation problem
6. Diagnosis
3 Differential diagnosis
3 differentiating features
Ring enhancing lesion
NCC, tuberculoma, toxoplasma,
abscess, tumor
Peripheral vs central
single vs multiple
perilesional edema
smooth vs irregular
Scolex seen
Wall thickness
Intensity of cystic material
7. Diagnosis
Name 2 other skin lesions
Inheritance
Other systems you would examine
Name 3 lesions seen in the brain
Ash leaf macule in Tuberous sclerosis
Adenoma sebaceum , shagreen patch,
caf au lait spots
Autosomal dominant
Eyes, heart, kidneys, brain
Tubers, subependymal nodules,
subependymal giant cell astrocytomas
8. This 5 yr old comes with unsteadiness of
gait with recurrent respiratory infections.
Diagnose the condition
Inheritance
3 salient features
2 lab
investigations
Ataxia Telangiectasia
Autosomal recessive
FEATURES: Ataxia, telangiectasia,
immunodeficiency, recurrent sinopulmonary
infections, X ray hypersensitivity, malignancies
LAB: Alpha feto proteins, Immunoglobulins,(low
IgA, IgG, high IgM), chromosomal breaks with
exposure to radiation.
9. 8 yr old boy with headache,
vomiting, ataxia, diplopia.
Clinical diagnosis?
MRI shows SOL
.Name 2 supra and infra tentorial SOLs
For this SOL what other abnormalities
will you expect
Raised ICT
Supra- Craniopharyngioma, astrocytoma, Gliomas,
ganglioglioma
Infra- meduloblastoma, glioma
Craniopharygioma
Panhypopitutarism, Hypothyroidism, GH deficiency,
Gonadotrophin deficiency, cortisol def.
10. CSF picture
Protein 1640mg% ,
sugar 45/92 ,
cells 150 P40 L60
Identify the condition
3 pathological etiologies
3 features on CT scan
Treatment options
TBM
Ischaemia, edema, exudates
Infarct, basal exudates, hydrocephalus (
communicating /non communcating)
tuberculomas,
4 drug AKT, steroids, anti edema, shunt
surgery,
11. Child with fever, headache ,
vomiting and altered sensorium.
CSF picture :proteins 130,
sugar 40/90,
cells 240 P40 L 60
Give 3 differentials
Give 2 other investigations
Treatment options
Partially treated pyogenic meningitis,
early TBM, aseptic, viral meningitis
Latex agglutination, HSV DNA PCR, CT
scan with contrast, EEG
Antibiotics, acyclovir 10 mg/kg 8 hrly for
14 days, AKT, supportive
12.Child with fever , convulsions,
altered sensorium
CSF picture : protein 62,
cells 95 P10 L90,
sugars 45/80,
RBCs 80/ hpf
Additional 2 investigations of choice
Treatment with dose
EEG - PLEDS-periodic lateralizing
epileptiform discharges
HSV PCR,( ? HSV IgG, IgM ), CT scan /
MRI with contrast bi/ uni temporal
hyperintensities
Acyclovir 10mg/kg/dose 8 hrly for 14 -21
days
13)1.5 yrs old child with fever and
convulsions.
CSF picture : proteins 80,
sugar 20 /60,
cells 500 , P 90 L 10.
Diagnose / 3 common organisms
Child develops tense AF with focal
convulsions on day 8. Probable causes?
3 Long term sequelae
Pyogenic meningitis; pneumococci, H
influenza, meningococci
Subdural empyema, cerebral absess,
infarction.
Sensorineural deafness,
hydrocephalus, CP, MR, Epilepsy,
behavioural problems
14)11 months old comes with
neuroregression from 5 months of
age. He is hypotonic on examination.
Name 3 systems you would like to
examine?
Differentials
Give 4 DDs of cherry red spots
Investigation of choice
CNS, abdominal, ophthalmology,
Krabbes, GSD [Pompes], Tay sachs,
gangliosidosis
GM 1Gangliosidosis, sialidosis, tay
sachs, niemann picks, sandhoffs, MLD,
mucolipidosis
Leucocyte enzymes, fibroblast culture
15)18 months old child comes with
recurrent strokes , ptosis , ataxia and
myopathy.
Diagnosis
Investigation of choice
Inheritance
Name 2 more conditions of the same
metabolic category
MELAS
Mitochondrial deletion study
MRI with spectroscopy
CSF Lactate, Blood Lactate.
ABG-Anion Gap
Metabolic Screen
Maternal transmission
MERRFS, Kearn Sayers, Leighs, Alpers
16) 6 months old female child, FTND,
has asymmetric infantile spasms,
developmental retardation and
choroidal lacunae. MRI clinches the
diagnosis.
What is the diagnosis?
What is the MRI picture?
What is the inheritance pattern?
Aicardi syndrome
Corpus callosum agenesis
Posterior choroidal cysts,
choroidal lacunae
X linked Dominant
17) A boy presents with this skin
lesionGive 4 differential diagnosis
When and how much would you
investigate?
Caf au lait spot.
DD- NF,Mc cune albright, TS, Ataxia
telangiectasia, Maffuci, any
phakcomatosis, Chediak Higashi
If asymptomatic No treatment
VEP-Periodic intervals if
abnormal do MRI for
optic glioma
If symtomatic VEP, MRI,
EEG
18)
Name the sign
3 DDs
3 investigations in
sequence of
importance
Inheritance/ locus
Gowers Sign
Duchenne, SMA III, Limb girdle
dystrophy, BMD, myopathy
Dystrophin gene study, EMG /NCV,
CPK levels
X linked recessive/ Xp 21
19)
Diagnosis
2 associated
abnormalities
Classical CNS picture
in CT scan and is
described as
Inheritance
Sturge Weber syndrome
Glaucoma, intracranial calcification,
intractable epilepsy, hemangioma
Tram track calcification
Sporadic inheritance
Identify
20)
2 salient features
Criteria for diagnosis
in newborn period
are called:
Child develops
spastic
quadriparesis.What
will you suspect?
Downs syndrome
Facies, congenital heart defects,
hypothyroidism ,deafness, hypotonia
Halls Criteria
Atlanto-axial dislocation
Diagnose
22)
Inheritance
2 associated
abnormalities
Investigation for
Raised ICT
Apert syndrome
Autosomal dominant
Proptosis, syndactyly, MR
3 D CTScan
23)
This child came
with
neuroregression
from 7 months of
age with abn hand
movts
Diagnosis / DD
3 salient features
Which milestones
are delayed?
Retts syndrome /
Autism
Autism, stereotypies,
microcephaly, loss of
fine purposeful hand
movements
Social, language
24) 10 month old , male, presents with
progressive decreased activity since 3
months of age, breathing difficulty since
8 mths and pneumonia. Examination
shows alert child with hypotonia and
absent reflexes.
What is the diagnosis ?
How do you confirm?
What is the inheritance?
Name 3 antenatal / natal features to ask for?
SMA 1
EMG/NCV, Gene studies
SMA- AR,
Decreased fetal kick count,
polyhydramnious, previous fetal loses,
weak cry, resp problems at birth.
25) 3 yrs old girl comes with drooping of both
eyelids and squint noticed since 15 days.
Abnormality seems to increase in the
evenings.
Diagnosis?
Any 2 relevant questions?
How do you confirm? Name 2 relevant
investigations.
Drug of choice.
Myasthenia gravis
Any other activity tires with time eg.
Eating, walking, speech,
Edrophonium test / Neostigmine test
Anticholinesterase antibody, EMG with
repititive stimulation, CTScan chest
Pyridostigmine
26 ) Label the
parts shown
Corpus callosum
Lateral ventricle
4 th ventrricle
Pons
medulla
EPILEPSY
1)4 Yrs old boy with normal motor and
mental development becomes aphasic for
last 15 days. He had a single GTC at 3.6
yrs.CNS examination is normal.
Investigation of choice
Diagnosis
Treatment options
EEG
Landau Kleffner syndrome
IV Ig , Steroids, AEDs
2) 6 yrs old girl with delayed development.
Perinatal hypoglycemia. Symptomatic
infantile spasms at 7 mths. Then focal
seizures, generalised, myoclonic and now
tonic.
Diagnosis?
Drugs used?
AEDs to be avoided?
Non pharmacological treatment modalities
Lennox Gastaut syndrome
Valproate, Lamotrigine, Topiramate,
clobazam
Carbamazepine, phenobarbitone
Corpus callosotomy, Ketogenic diet,
vagal nerve stimulation
3) 10 yrs old boy presents with lip smacking,
facial and eye deviation on left lasting 2
minutes after falling asleep. Similar
history 3 and 6 months back.
Investigation of choice?
Diagnosis?
Which investigation you need not do?
Prognosis?
Drug of choice?
EEG
Rolandic epilepsy/ BECTS
MRI/Neuroimaging
Good,
Carbamazepine /oxcarbamazepine
4)4 days old newborn, FTND presents
with multiple multifocal seizures. He
has a normal systemic examination.
DD- 3 most imp
If strong family history of neonatal
seizures present, what will u think
Hypocalcemia,
hypomagnesemia,
5 th day fits,
BFNS,
hypoglycemia
BFNS
5) 6 yrs old boy comes with left sided
focal seizures preceded by aura of
fearfulness. He has history of
prolonged febrile seizures at 1 yr of
age. EEG shows right temporal
epileptiform activity.
What MRI picture do you expect?
Which virus has been suspected for the
same lesion?
What are the treatment options?
Mesial temporal sclerosis
Human herpes virus 6
Temporal lobectomy
6) What does this EEG show?
What is the drug of choice?
Prognosis?
One OPD procedure to confirm
diagnosis
3 Hz spike and wave activity in Absence
seizure childhood /Juvenile
Valproate, Lamotrigine, clobazam
Good in childhood, slightly less for
juvenile
Hyperventilation
Jerk
jerk
7) Diagnosis
Confirmatory test
3 therapeutic options
SSPE burst suppression pattern
CSF measles IgG
Ribavarine, interferons, inosiplex,
amantidine
8) Diagnosis
Commonest etiology
Drug of choice
PLEDS
Herpes encephalitis
Acyclovir
9) 6 yr old male admitted with
prolonged generalised seizures.
Immediate AEDs name 2 with dose
2nd line AEDs 2 with dose
3rd line name 2
Lorazepam [0.05mg/kg],
Diazepam [0.3mg/kg],
midazolam[ 0.2mg/kg]
Phenytoin[ 20mg/kg],
phenobarb[20/kg]
Midazolam drip,
propofol,
thiopentol
10) 6 months old child with h/o
perinatal insult comes with
regression of social milestones and
clusters of startles on awakening .
Diagnosis
Name 2 investigations you will ask for?
Infantile spasms, West syndrome
EEG, MRI
Hypsarrhythmia
ACTH / steroids, Vigabatrin, Valproate
/Topiramate / Nitrazepam
11) SODIUM VALPROATE
Each 5ml of syrup contains
Commonest 3 side effects
Contraindicated in
Synergistic activity with
Dosage range
With Lamotrigine what precautions Are
needed?
200 mg, or 40 mg/ml
Weight gain, hair loss, hepatotoxic,
PCOD, hirsutism, hyperammonemia
Inherited Metabolic disorder, underlying
liver dysfunction
Lamotrigine
10-40 mg/kg/day
Cut the dose of VPA when adding LMT
12) Topiramate
2 indications
2 side effects
Contraindicated in
Tablet strength? Syrup? Any other
preparation known?
Generalised, partial, Infantile spasms, LGS
Wt loss, word finding difficulty speech
regression, blurred vision, renal calculi,
hyperthermia
thin children, speech concerns
25 / 50 / 100mg. No syrup. Sprinkle
13) Carbamazepine
Commonest side effects
Not used in
Higher derivative with advantage
Syrup strength
Ataxia , diplopia, rash, hyponatremia,
cognitive decline, behavior concerns
Absence, myoclonic
Oxcarbazepine, less side effects
5ml = 100mg
14)Lamotrigine
Indication, Dose
Synergistic with?
What dosage modification done?
Life threatening side effect?
Partial, generalied, LGS, JME;
3-7 mg/kg/d
Valproate,
Reduce the dose of VPA prior to
starting
Steven Johnson syndrome
15)Name 3 newer AEDs
Name a carbonic anhydrase inhibitor used
as AED
Ocarbamazepine, Topiramate,
Lamotrigine, Vigabatrine, Gabapentine,
Zonisamide, Leviteracetam, Felbamate,
Tiagabine
Acetazolamide, Topiramate
16) 3 months baby has intractable
epilepsy since birth and delayed
development
Name 2 vitamins you can try
Does not respond to them what
investigation next
Pyridoxine
Biotin
Metabolic workup, MRI dysplasia,
metabolic
17) 1st unprovoked generalised seizure
in a 2 yr old child
Essential investigation
Optional investigation
Treatment of choice
EEG
MRI, biochemistry, CSF
Nil , unless specific indication
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