west syndrome case presentation

Welcome

to

Clinical MeetingPresented by

DR. Amlendra K. Yadav

DR. Chandra Shekhar Bhagat

Resident (phase-A)

Particulars of the Patient

Name : Ahona

Age : 3 month

Sex : Female

Address : Narayangonj

Religion : Islam

Date of Admission : 05/01/2014

Date of Exammination : 06/01/2014

Chief Complaints

• Seizure for 2 months

History of Present illness

According to the statement of informant mother , her child

was reasonably well two month back then she developed

seizure. The seizure was sudden, brief contraction of neck

and extremities. It occurred in cluster (3 – 4) episodes per

day and lasts for (3 - 5) minutes and during awakening.

There is no history of passing urine or defecation during

the episode of seizure.There was no postictical phemenon

.Mother also complainted that her child had no interest to

surroundings . There was no history of fever, vomitting,

respiratory problem, loss of consciousness or trauma.

For above mentioned complaints she visited general

pediatrician and was treated with anti epileptic drugs

for 4 weeks but the seizure didn’t improve .Then they

brought the child to BSMMU for better evaluation and

management.

Birth HistoryAntenatal : There was no history of fever, rashes,abortion

or sibs death.

Natal : Delivered by LUCS due to oligohydriamnios at

term .

Postnatal: Baby cried immediately after birth.

Immunization HistoryImmunization as per EPI schedule started.

Feeding HistoryThe child is on Exclusive breast feeding.

Milestones of Development

Gross motor :- no neck control

Fine motor :- palmar grasp present

Language :- cooing

Social :- smile present

Consanguinity

No H/O consanguinity.

Family History

She is the only issue of her parents.

Other family member are healthy.

History of Past Illness

Nothing significant.

Drug History Phenobarbitone for 2 weeks.

Sodium Valproate for 2 weeks.

Socio-Economical History

Belongs to poor socio-economical status

family , stays in pakka house , drink tubwell

water .

DRUG REACTION HISTORY

No past drug reaction history present .

Physical ExaminationGeneral

Appearance : alert, playfull

Pallor

Edema

Jaundice

Cyanosis Absent

Clubbing

Koilonychia

Dehydration

Neck vein : Not engorged

Lymph Node :- Not enlarged

Skin survey : – BCG mark present.

Signs of meningeal irritation : – Absent

Bony tenderness : – Absent

Fontanelle : - open but not bulged

Vital Signs

Temperature – 98o F

HR – 120 beats /min

BP – 70/40 mm Hg

RR – 40 breaths /min

ANTHROPOMETRY:

Weight - 5.5 kg, ( lies on 50th percentile)

Length - 62cm, ( lies on 25th percentile)

OFC - 38cm ( lies on 10th percentile )

Nervous system

• Patient is playfull

• Cranial nerve - Intact as per I exammined

• Motor :- Bulk of muscle – normal

Tone - normal

Jerk – Normal

• Sensory - normal

• Plantar – b/l extensor type

• Primitive reflex – normal

Inspection• Shape of the chest : normal• R/R : 38 breaths/min• Visible vein & Pulsation : absent• Scar mark : absent

Palpation• Trachea : centrally placed• Apex beat : left 5th ICS, medial to

midclavicular line

Respiratory System:

Percussion note

• Resonant all over the lung fields

Auscultation

• Breath sound : vesicular

• Added sound : absent

Inspection: No visible pulsation

Palpation:• Apex beat : left 5th ICS, • Thrill : absent • P2 : Not palpable• Lt. parasternal heave : absent

Auscultation:• 1st & 2nd heart sounds audible in all 4 areas • Murmur : absent

Cardiovascular system examination

Mouth & Oral cavity:

Tongue : NormalGum : NormalBuccal mucosa, tonsils & fauces : Normal

Alimentary system examination

AbdomenInspection:

Umbilicus centrally placed & inverted Engorged vein/visible peristalsis – absent

Palpation:

Soft, non tender, non-distendedLiver just palpable .Spleen not palpable

Percussion:Upper border of liver dullness : Rt. 5th ICSShifting dullness: Absent

Auscultation: Bowel sound : PresentBruit (Hepatic / Renal) : Absent

Salient FeaturesAhona 3 months old female child only issue of

non-consanguinous parent came from narayangonj,

was admitted with a complain of seizure for 2 month.

The seizure was sudden , brief contraction of neck

and extremities.It occurred in cluster (3 – 4) episodes

per day and lasts for 3 -5 min and during awakening.

There is no history of passing urine or defecation

during the episode of seizure . There was no post

ictical phemenon . Mother also complainted that her

child was not responsive to surrounding . She was

treated with anti-epileptic drugs for 4 weeks but the

condition didn’t improve. The child was delivered by

LUCS , there is no H/O perinatal Asphyxia.

On examination Ahona was alert ,playful , BP –

70/40 mm of hg , RR – 40/min , HR – 120 beats/min,

temperature – normal, BCG mark present, fontanels –

open but not bulged, signs of meningeal irritation -

absent ,lymph node – not enlarged , cranial nerve –

intact , bulk- normal , tone – normal , jerks – Normal,

plantar – b/l extension , lungs – B/L clear, P/A-Liver

just palpable . Other systemic exammination reveals

normal findings.

Provisional Diagnosis

???

Provisional Diagnosis

West-Syndrome

Differnential diagnosis

1) Early Myoclonic infantile encephalopathy

2) Early infantile epileptic syndrome

Differential diagnosis

D/D Point in favor Point against

West Syndrome

Seizure in cluster during

awakening

Developmental delay

Severe myoclonic

epilepsy in infancy

Age less than 2 month

Seizure in cluster

myoclonic jerks

Early infantile epileptic

syndrome

Age less than 2 month

Seizure in cluster

Tonic spasm

INVESTIGATION

AND

MANAGEMENT

E.E.G - 17/12/2013

Normal Findings

TORCH screening

CMV IgG - Positive

CMV DNA - Positive

USG of Brain :- Normal Study.

Inv./Date 06/01/2014 15/01/2014

C.B.CHb% 9.2 g/dl 9.4 g/dl

ESR 05 mm in 1st hr

T.C 17,000/cumm. 10,000/cumm.

D.C N=13%

L= 80%

N= 10%

L= 86%

Platelet count 3,00,000

SGPT 23 u/l

Sr.Creatinine 0.3 mg/dl

Eye Evaluation chorioretinitis

Hearing Test Normal

Final Diagnosis:

West syndrome with CMV positive

Follow-up on 16/01/2014

Subjective :- No new complain

Objective :- G/C – alert, playful, afebrile

respiratory rate – 38/min

heart rate – 110/min

temperature – 98o F

Blood pressure – 80/40mmhg

Asessment – Improving

Plan – stop inj. Gancyclovir

Rx after admission

Counseling

Breast feeding

Inj Gancyclovir-6mg/kg/dose- 12 hourly

Tab.Vigabatrin-100 mg/kg/day

Syp.Cefixime- 8 mg/kg/day

Developmental therapy

Follow-up PLAN

Weekly CBC examination

Repeat EEG before discharge

CT scan of brain

Thank You..