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NEUROLOGYNEUROLOGY
Post Graduate CMEPost Graduate CMEOSCE - NeurologyOSCE - NeurologyBai Jerbai Wadia Hosital !or C"idre#$Bai Jerbai Wadia Hosital !or C"idre#$
Mu%baiMu%bai
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&' ( yrs old$ boy $ )*N+$ Nor%al %otor$
%e#tal a#d la#guage %ilesto#es,Co%lai#ts !ro% s"ool . aggressi/e$
a#t sit i# o#e lae$ restless$
!orget!ul a#d i%atie#t$ roble%s 0it"!rie#ds$ !ig"ts
• Diagnosis?• 3 cardinal features of this condition
• 2 treatment options
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• ADHD
• Inattention, hyperactivity and impulsivity
• Medications (methylphenidate,
amphetamines, fluoitine, atomoitine!,
"ehavior therapy
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1'*"is "ild "as
eilesy$ MR$ ata2ia$
u#ro/o3ed laug"ter$
se/ere see" delay
• Diagnosis?
• #hromosome involved
• Inheritance
• $ame a syndrome %ith
the same chromosomala&normality
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• Angelman syndrome
• ')''*2+'2
• niparental disomy - usually maternal (./+
01! + occ* paternal
+ normal chromosome
? Dominant mutations
• rader illi - ')''+'3 (paternal origin!
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3! 1 yrs old "ild bor# re%aturely 41503s6o%es 0it" o%lai#t o! delayed
a"ie/e%e#t o! %otor %ilesto#es,E2a%i#atio# s"o0s "yerto#ia 0it" bris3re!le2es i# all ( li%bs %ore %ar3ed i# lo0erli%bs,
• hat is the diagnosis? Define*
• hat is the M4I picture?
• hat is the treatment and %hen do you start it?• $ame 2 conditions this child %ill &e at ris5 for?
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• 6pastic Diplegia* Increased tone in all 7etremities and face* 88989 face
• eriventricular leu5omalacia
• :arly intervention
• 8earning disa&ilities,
• ;isual concerns
• :pilepsy
• Delayed mental development
• "ehavior concerns
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(,7 !ull ter% $%ale "ild de/elos 8au#dieo# day 9 o! li!e$ :S, bilirubi# .9( %g;'a#du#dergoes a# e2"a#ge tra#s!usio# *
• hat is the immediate complication li5ely tooccur?
• here is the anatomical a&normality?
• hat are the long term complications?• $ame ' investigations you %ould insist on
after discharge?
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• <ernicterus
• =lo&us pallidus, dentate nucleus, cere&ellarvermis, cochlear nuclei
• #horeoathetoid cere&ral palsy, dystonic >
dys5inetic #, 6ensorineural deafness,
• Audiometry testing
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<, 7 "ild 0it" aare#tly #or%al birt""istory =MS- soial s%ile at 9 %t"s$Sitti#g at 5 %o#t"s$ rt "a#dre!ere#e at > %t"s$ 0al3i#gi#dee#de#tly at &,? yrs,
• hat is your diagnosis?
• hat investigation %ill you do?
• hat relevant maternal history %ill youas5 for?
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• #ongenital hemiplegia
• M4I &rain
• Maternal inury, threatened a&ortion, A# resistance, coagulation pro&lem
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.* +iag#osis
• 3 Differential diagnosis
• 3 differentiating features
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• 4ing enhancing lesion
• $##, tu&erculoma, tooplasma,
• a&scess, tumor
• eripheral vs central
• single vs multiple
• perilesional edema
• smooth vs irregular
• 6cole seen• all thic5ness
• Intensity of cystic material
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0* Diagnosis
• $ame 2 other s5in lesions
• Inheritance
• @ther systems you %ould eamine
• $ame 3 lesions seen in the &rain
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• Ash leaf macule in u&erous sclerosis
• Adenoma se&aceum , shagreen patch,
cafB au lait spots
• Autosomal dominant
• :yes, heart, 5idneys, &rain
• u&ers, su&ependymal nodules,su&ependymal giant cell astrocytomas
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8. This 5 yr old comes with unsteadiness of
gait with recurrent respiratory infections.• Diagnose the condition
Inheritance
3 salient features
2 la&
investigations
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• Ataia elangiectasia
• Autosomal recessive
• C:A4:6C:A4:6 Ataia, telangiectasia,immunodeficiency, recurrent sinopulmonaryinfections, E ray hypersensitivity, malignancies
• 8A"8A" Alpha feto proteins, Immunoglo&ulins,(lo%
IgA, Ig=, high IgM!, chromosomal &rea5s %itheposure to radiation*
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F* 5 yr old boy 0it" "eada"e$
/o%iti#g$ ata2ia$ diloia,
• #linical diagnosis?
• M4I sho%s 6@8
• *$ame 2 supra and infra tentorial 6@8s
• Cor this 6@8 %hat other a&normalities
%ill you epect
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• 4aised I#
• 6upra+ #raniopharyngioma, astrocytoma, =liomas,ganglioglioma
• Infra+ medulo&lastoma, glioma
• #raniopharygioma
• anhypopitutarism, Hypothyroidism, =H deficiency,
=onadotrophin deficiency, cortisol def*
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'/* CS) iture
Protei# &?(@%g; $
sugar (<A>1 $
ells &<@ P(@ L?@
• Identify the condition
• 3 pathological etiologies
• 3 features on # scan
• reatment options
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• "M
• Ischaemia, edema, eudates
• Infarct, &asal eudates, hydrocephalus (communicating >non communcating!tu&erculomas,
• 7 drug A<, steroids, anti edema, shuntsurgery,
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''* C"ild 0it" !e/er$ "eada"e $/o%iti#g a#d altered se#soriu%,
• CS) iture =rotei#s &9@$
sugar (@A>@$
ells 1(@ P(@ L ?@
• =ive 3 differentials
• =ive 2 other investigations
• reatment options
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• artially treated pyogenic meningitis,
early "M, aseptic, viral meningitis
• 8ate agglutination, H6; D$A #4, #
scan %ith contrast, ::=
• Anti&iotics, acyclovir '/ mg>5g G hrly for
'7 days, A<, supportive
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&1,C"ild 0it" !e/er $ o#/ulsio#s$
altered se#soriu% CS) iture = rotei# ?1$
ells >< P&@ L>@$
sugars (<A5@$RBCs 5@A "!
• Additional 2 investigations of choice
• reatment %ith dose
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• ::= + 8:D6+periodic lateraliingepileptiform discharges
• H6; #4,( ? H6; Ig=, IgM !, # scan >M4I %ith contrast - &i> uni temporalhyperintensities
• Acyclovir '/mg>5g>dose G hrly for '7 +2'days
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&9'&,< yrs old "ild 0it" !e/er a#do#/ulsio#s,
CS) iture = rotei#s 5@$
sugar 1@ A?@$
ells <@@ $ P >@ L &@,
• Diagnose > 3 common organisms
• #hild develops tense AC %ith focal
convulsions on day G* ro&a&le causes?• 3 8ong term se)uelae
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• yogenic meningitis pneumococci, Hinfluena, meningococci
• 6u&dural empyema, cere&ral a&sess,infarction*
• 6ensorineural deafness,hydrocephalus, #, M4, :pilepsy,
&ehavioural pro&lems
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&('&& %o#t"s old o%es 0it"
#euroregressio# !ro% < %o#t"s o!age, He is "yoto#i o# e2a%i#atio#,
• $ame 3 systems you %ould li5e toeamine?
• Differentials
• =ive 7 DDs of cherry red spots• Investigation of choice
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• #$6, a&dominal, ophthalmology,
• <ra&&es, =6D JompesK, ay sachs,gangliosidosis
• =M '=angliosidosis, sialidosis, taysachs, niemann pic5s, sandhoffs, M8D,
mucolipidosis• 8eucocyte enymes, fi&ro&last culture
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&<'&5 %o#t"s old "ild o%es 0it"reurre#t stro3es $ tosis $ ata2ia a#d%yoat"y,
• Diagnosis
• Investigation of choice
• Inheritance
• $ame 2 more conditions of the samemeta&olic category
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• M:8A6
• Mitochondrial deletion study
M4I %ith spectroscopy
#6C 8actate, "lood 8actate*
A"=+Anion =ap
Meta&olic 6creen
• Maternal transmission
• M:44C6, <earn 6ayers, 8eighs, Alpers
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'.! ? %o#t"s old !e%ale "ild$ )*N+$"as asy%%etri i#!a#tile sas%s$
de/elo%e#tal retardatio# a#d"oroidal lau#ae, MR li#"es t"ediag#osis,
• hat is the diagnosis?
• hat is the M4I picture?
• hat is the inheritance pattern?
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• Aicardi syndrome
• #orpus callosum agenesis
• osterior choroidal cysts,
• choroidal lacunae
• E lin5ed Dominant
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'0! 7 boy rese#ts 0it" t"is s3i#
lesio#-
=ive 7 differential diagnosis
hen and ho% much %ould youinvestigate?
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• #afB au lait spot*
• DD+ $C,Mc cune al&right, 6, Ataia
telangiectasia, Maffuci, anypha5comatosis, #hedia5 Higashi
• If asymptomatic -$o treatment
;:+eriodic intervals if
a&normal do M4I foroptic glioma
• If symtomatic -;:, M4I,
::=
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'G! • $ame the sign
• 3 DDs
• 3 investigations in
se)uence ofimportance
• Inheritance> locus
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• =o%ers 6ign
• Duchenne, 6MA III, 8im& girdledystrophy, "MD, myopathy
• Dystrophin gene study, :M= >$#;,#< levels
• E lin5ed recessive> Ep 2'
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'F!
• Diagnosis
• 2 associateda&normalities
• #lassical #$6 picturein # scan and isdescri&ed as
• Inheritance
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• 6turge e&er syndrome
• =laucoma, intracranial calcification,
intracta&le epilepsy, hemangioma
• ram trac5 calcification
• 6poradic inheritance
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• 2/!
• Identify
• 2 salient features
• #riteria for diagnosis
in ne%&orn periodare called
• #hild develops
spastic)uadriparesis*hat%ill you suspect?
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• Do%ns syndrome
• Cacies, congenital heart defects,hypothyroidism ,deafness, hypotonia
• Halls #riteria
• Atlanto+aial dislocation
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• 22!
• Diagnose
• Inheritance
• 2 associateda&normalities
• Investigation for
4aised I#
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• Apert syndrome
• Autosomal dominant
• roptosis, syndactyly, M4
• 3 D #6can
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• 23!
•*"is "ild a%e0it"#euroregressio#!ro% %o#t"s o!age 0it" ab# "a#d%o/ts
• Diagnosis > DD
• 3 salient features• hich milestones
are delayed?
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• 4etts syndrome > Autism
• Autism, stereotypies,microcephaly, loss of
fine purposeful handmovements
• 6ocial, language
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27! &@ %o#t" old $ %ale$ rese#ts 0it"rogressi/e dereased ati/ity si#e 9%o#t"s o! age$ breat"i#g di!!iulty si#e5 %t"s a#d #eu%o#ia, E2a%i#atio#s"o0s alert "ild 0it" "yoto#ia a#dabse#t re!le2es,
• hat is the diagnosis ?
• Ho% do you confirm?
• hat is the inheritance?
• $ame 3 antenatal > natal features to as5 for?
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• 6MA '
• :M=>$#;, =ene studies
• 6MA+ A4,
• Decreased fetal 5ic5 count,
polyhydramnious, previous fetal loses,%ea5 cry, resp pro&lems at &irth*
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2! 9 yrs old girl o%es 0it" drooi#g o! bot"eyelids a#d sDui#t #otied si#e &< days,7b#or%ality see%s to i#rease i# t"ee/e#i#gs,
• Diagnosis?
• Any 2 relevant )uestions?
• Ho% do you confirm? $ame 2 relevantinvestigations*
• Drug of choice*
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• Myasthenia gravis
• Any other activity tires %ith time eg*:ating, %al5ing, speech,
• :drophonium test > $eostigmine test
• Anticholinesterase anti&ody, :M= %ith
repititive stimulation, #6can chest
• yridostigmine
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•
• 2. ! 8a&el the
parts sho%n
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• #orpus callosum
• 8ateral ventricle
•7 th ventrricle
• ons
• medulla
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:I8:6L
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&'( Yrs old boy 0it" #or%al %otor a#d
%e#tal de/elo%e#t beo%es a"asi !orlast &< days, He "ad a si#gle G*C at 9,?
yrs,CNS e2a%i#atio# is #or%al,
• Investigation of choice
• Diagnosis
• reatment options
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• ::=
• 8andau <leffner syndrome
• I; Ig , 6teroids, A:Ds
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2! ? yrs old girl 0it" delayed de/elo%e#t,
Peri#atal "yoglye%ia, Sy%to%atii#!a#tile sas%s at %t"s, *"e# !oalseiures$ ge#eralised$ %yolo#i a#d #o0to#i,
• Diagnosis?
• Drugs used?
• A:Ds to &e avoided?
• $on pharmacological treatment modalities
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• 8enno =astaut syndrome
• ;alproate, 8amotrigine, opiramate,clo&aam
• #ar&amaepine, pheno&ar&itone
• #orpus callosotomy, <etogenic diet,vagal nerve stimulation
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3! &@ yrs old boy rese#ts 0it" li s%a3i#g$
!aial a#d eye de/iatio# o# le!t lasti#g 1%i#utes a!ter !alli#g aslee, Si%ilar"istory 9 a#d ? %o#t"s ba3,
• Investigation of choice?• Diagnosis?
• hich investigation you need not do?
• rognosis?• Drug of choice?
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• ::=
• 4olandic epilepsy> ":#6
• M4I>$euroimaging
• =ood,
• #ar&amaepine >ocar&amaepine
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('( days old #e0bor#$ )*N+ rese#ts
0it" %ultile %ulti!oal seiures, He"as a #or%al syste%i e2a%i#atio#,
• DD+ 3 most imp
• If strong family history of neonatal
seiures present, %hat %ill u thin5
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• Hypocalcemia,
• hypomagnesemia,
• th day fits,
• "C$6,
• hypoglycemia
• "C$6
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! ? yrs old boy o%es 0it" le!t sided
!oal seiures reeded by aura o!!ear!ul#ess, He "as "istory o!rolo#ged !ebrile seiures at & yr o!age, EEG s"o0s rig"t te%oral
eileti!or% ati/ity,
• hat M4I picture do you epect?
• hich virus has &een suspected for thesame lesion?
• hat are the treatment options?
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• Mesial temporal sclerosis
• Human herpes virus .
• emporal lo&ectomy
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.! hat does this ::= sho%?
• hat is the drug of choice?
• rognosis?
• @ne @D procedure to confirmdiagnosis
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• 3 H spi5e and %ave activity in A&senceseiure - childhood >uvenile
• ;alproate, 8amotrigine, clo&aam
• =ood in childhood, slightly less for uvenile
• Hyperventilation
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er5 er5
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0! Diagnosis
• #onfirmatory test
• 3 therapeutic options
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•66: -&urst suppression pattern
• #6C measles Ig=
• 4i&avarine, interferons, inosiple,
amantidine
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G! Diagnosis
• #ommonest etiology
• Drug of choice
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• 8:D6• Herpes encephalitis
• Acyclovir
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F! ? yr old %ale ad%itted 0it"rolo#ged ge#eralised seiures,
• Immediate A:Ds -name 2 %ith dose• 2nd line A:Ds -2 %ith dose
• 3rd line - name 2
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• 8oraepam J/*/mg>5gK,
• Diaepam J/*3mg>5gK,
• midaolamJ /*2mg>5gK
• henytoinJ 2/mg>5gK,
• pheno&ar&J2/>5gK
• Midaolam drip,
• propofol,• thiopentol
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'/! ? %o#t"s old "ild 0it" "Ao
eri#atal i#sult o%es 0it"regressio# o! soial %ilesto#es a#dlusters o! startles o# a0a3e#i#g ,
• Diagnosis
• $ame 2 investigations you %ill as5 for?
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• Infantile spasms, est syndrome
• ::=, M4I
• Hypsarrhythmia
• A#H > steroids, ;iga&atrin, ;alproate>opiramate > $itraepam
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''! SO+UM F7LPRO7*E
• :ach ml of syrup contains
• #ommonest 3 side effects
• #ontraindicated in• 6ynergistic activity %ith
• Dosage range
• ith 8amotrigine %hat precautions Areneeded?
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• 2// mg, or 7/ mg>ml
• eight gain, hair loss, hepatotoic,#@D, hirsutism, hyperammonemia
• Inherited Meta&olic disorder, underlyingliver dysfunction
• 8amotrigine
• '/+7/ mg>5g>day
• #ut the dose of ;A %hen adding 8M
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'2! *oira%ate
• 2 indications
• 2 side effects
• #ontraindicated in
• a&let strength? 6yrup? Any otherpreparation 5no%n?
7/21/2019 Neuro Case Scenarios
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• =eneralised, partial, Infantile spasms, 8=6
• t loss, %ord finding difficulty - speechregression, &lurred vision, renal calculi,
hyperthermia
• thin children, speech concerns
• 2 > / > '//mg* $o syrup* 6prin5le
7/21/2019 Neuro Case Scenarios
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'3! Carba%aei#e
• #ommonest side effects
• $ot used in• Higher derivative %ith advantage
• 6yrup strength
7/21/2019 Neuro Case Scenarios
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• Ataia , diplopia, rash, hyponatremia,cognitive decline, &ehavior concerns
• A&sence, myoclonic
• @car&aepine, less side effects
• ml N '//mg
7/21/2019 Neuro Case Scenarios
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&('La%otrigi#e
• Indication, Dose
• 6ynergistic %ith?• hat dosage modification done?
• 8ife threatening side effect?
7/21/2019 Neuro Case Scenarios
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• artial, generalied, 8=6, M:
• 3+0 mg>5g>d
• ;alproate,
• 4educe the dose of ;A prior tostarting
• 6teven ohnson syndrome
7/21/2019 Neuro Case Scenarios
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• '!$ame 3 ne%er A:Ds
• $ame a car&onic anhydrase inhi&itor used
as A:D
7/21/2019 Neuro Case Scenarios
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• @car&amaepine, opiramate,8amotrigine, ;iga&atrine, =a&apentine,Oonisamide, 8eviteracetam, Cel&amate,
iaga&ine
• Acetaolamide, opiramate
7/21/2019 Neuro Case Scenarios
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'.! 9 %o#t"s baby "as i#tratableeilesy si#e birt" a#d delayedde/elo%e#t
• $ame 2 vitamins you can try
• Does not respond to them - %hat
investigation net
7/21/2019 Neuro Case Scenarios
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• yridoine
• "iotin
• Meta&olic %or5up, M4I - dysplasia,meta&olic
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'0! &st u#ro/o3ed ge#eralised seiure
i# a 1 yr old "ild
• :ssential investigation
• @ptional investigation
• reatment of choice
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• ::=
• M4I, &iochemistry, #6C
• $il , unless specific indication
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http>>oscepediatrics &logspot in
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