NEW ONSET REFRACTORY

STATUS EPILEPTICUS (NORSE)

MISS SS, 25 YEARS OLD LADY
• NO KNOWN MEDICAL ILLNESS
• NON ALCOHOLIC, NON SMOKER
• ADL INDEPENDENT
• STAFF NURSE WORKING IN QUEEN ELIZABETH HOSPITAL
 PRESENTATION COMPLAINT

PRESENTED TO CASUALTY ON 30/9/16

• UNWELL SINCE 24/9/16
• FEVER ON AND OFF WITH CHILLS AND RIGORS
• URTI SYMPTOMS: PRODUCTIVE COUGH-YELLOWISH SPUTUM, RUNNING NOSE, SORE THROAT
• GUM BLEEDING-BRUSHING TEETH
• WENT TO PRIVATE GP ON 26/9/16-GIVEN T. PARACETAMOL, T. LORATADINE, SYRUP
BENADRYL, T. AMOXICILLIN
ON EXAMINATION, ALERT CONSCIOUS
• BP: 128/72
• PR: 80
• T: 38 CELCIUS
• SPO2: 100 UNDER ROOM AIR
• NEUROLOGICAL EXAMINATION NORMAL
• LUNGS: CLEAR, GOOD AIR ENTRY
• ABDOMEN: SOFT NON TENDER
• CVS S1S2 NO MURMUR
 IN GENERAL MEDICAL WARD

• INITIALLY TREATED AS DENGUE FEVER IN FEBRILE PHASE.

DDX: VIRAL FEVER
• FBC: WBC: 2.35/PLT 120/HCT 40.6/HB 12.5
• DENGUSE NS1: NEGATIVE, DENGUE IGM: NEGATIVE
• BSMP: NEGATIVE
• PLT GOING DOWN IN TREND AND STILL HAVING FEVER
• STARTED ON IV CEFTRIAZONE TO COVER FOR ATYPICAL INFECTION
ON 4/10/16 AT 7.30AM (AFTER 4 DAYS ADMITTED TO MEDICAL WARD),
• FOUND LESS RESPONSIVE IN BATHROOM. SHE APPEARED RESTLESS AND CONFUSED. GCS
E4V1M3. DXT STAT: 9.1. OTHER VITAL SIGNS STABLE.
• WAS INTUBATED FOR AIRWAY PROTECTION AND TRANSFERRED TO ICU
 ICU

IN ICU, 4/10/16 AT 7.30PM,

• PATIENT HAD SUDDEN BRADYCARDIA (HR 40BPM). THEN ASYSTOLE FOR ~10 SEC WITH APNEIC
SPO2 85%. THEN SPONTANEOUS REVERSION TO SINUS RHYTHM.
• NOTED TWITCHING OF TONGUE, UPROLLING OF EYEBALLS, CLONIC MOVEMENT OF LEFT LOWER
LIMB, PUPILS BILATERAL 3MM REACTIVE
• ABORTED WITH IV DIAZEPAM.
• SEIZURE CAME BACK AND WAS LOADED WITH IV EPILIM 900MG STAT AND STARTED ON IV EPILIM
200MG TDS.
• TREATED AS NON CONVULSIVE STATUS EPILEPTICUS. COVERING FOR MENIGOENCEPHALITIS
 Date Investigation Result
5/10/16 CSF cell count Clear and colourless
WBC nil
RBC 32
CSF FEME Glucose 4
Protein 0.36
CSF gram stain NOG
CSF Torula stain Negative
CSF Virology (HSV 1, HSV 2, Negative
EBV, VZV, JE
CSF AFB Negative
CSF MTB C&S Negative
CSF C&S NOG
CSF NMDAR Negative
ON 5/10/16,
• EEG: SEVERE BACKGROUND ENCEPHALOPATHY, NON CONVULSIVE STATUS EPILEPTICUS WITH
MULTIPLE SPIKES AT F3.
• LOADED WITH IV KEPPRA 1G AND STARTED ON T. KEPPRA 1G BD
ON 6/10/16:
• STILL HAVING TWITCHING ON LEFT LOWER LIMBS AND RIGHT FACE. LOADED WITH IV EPILIM
900MG STAT AND INCREASED T. EPILIM 400MG TDS.
• IN THE EVENING, HAD ANOTHER EPISODE OF FIT. LOADED WITH IV PHENYTOIN 750MG STAT
AND MAINTENANCE IV PHENYTOIN 100MG TDS. IV MIDAZOLAM WAS INCREASED FROM 4 TO
6ML/HR.
ON 7/10/16
• MRI BRAIN: CHANGES IN BILATERAL MESIAL TEMPORAL REGION LEFT > RIGHT
• LP RESULT: NORMAL. AUTOIMMUNE INVESTIGATION: PENDING
• COVER FOR AUTOIMMUNE ENCEPHALITIS.
• STARTED ON IVIG 18 G OD FOR 5/7 AND IV METHYLPREDNISOLONE 1G OD FOR 5/7
• 8/10/16
• 7 EPISODES OF SEIZURE.
• STARTED ON IV PROPOFOL 10ML/HR.
ON 9/10/16
• ANOTHER 7 EPISODES OF FITTING DESPITE INCREASED IV PROPOFOL. STARTED ON T.
TOPIRAMATE 200MG BD.

ON 10/10/16
• IV PROPOFOL WAS DISCONTINUED IN VIEW OF BRADYCARDIA AND PRIS. STARTED ON IV
MIDAZOLAM 30MG/HR (DOUBLE STRENGTH)
ON 11/10/16
• EEG: DOCUMENTED CLINICAL AND ELECTROGRAPHIC SEIZURES ORIGINATE FROM RIGHT
TEMPORAL THEN GENERALIZED TO LEFT SIDE WITH BURST SUPPRESSION PATTERN BUT NOT
LONG ENOUGH
• NO IMPROVEMENT DESPITE COMPLETED IVIG AND IV METHYLPREDNISOLONE FOR 5/7.
• TREATED AS NORSE
• STARTED ON IV PHENOBARBITONE 90MG/HR THEN IVI PHENOBARBITONE 0.5MG/KG/HR
(22MG/HR)
• HAD ANOTHER FEW EPISODES OF SEIZURE AND LOADED WITH IV PHENOBARBITONE 60MG/HR
AND INCREASED IVI PHENOBARBITONE TO 1MG/KG/HR (44MG/HR) AND INCREASED IVI
MIDAZOLAM 40MG/HR (20ML/HR)
• 12/10/16,
• EEG: NO MORE CLINICAL SEIZURE. MORE BACKGROUND SUPPRESSION BUT STILL CANNOT
ACHIEVE TARGET OF 6 SEC SUPPRESSION.
• GIVEN IV PHENOBARBITONE BOLUS 50MG X3
• INCREASED IVI PHENOBARBITONE TO 150MG/HR (3.5MG/KG/HR)
• PERCUTANEOUS TRACHESOTOMY WAS DONE IN VIEW OF PROLONGED VENTILATION
ON 13/10/16,
• AUTOIMMUNE WORKUP: NEGATIVE
• EEG: BURST SUPPRESSION 4-5 SECONDS
• TDM PHENOBARBITONE: 101.3
• CONTINUE IVI PHENOBARBITONE 150MG/HR

ON 14/10/16
• EEG: BURST SUPPRESSION 5-6 SECONDS (ON IVI PHENOBARBITONE 150MG/HR)
• DECREASED IV PHENOBARBITONE 120MG/HR
ON 15/10/16
• TDM PHENOBARBITONE 218.8
• DECREASED IVI PHENOBARBITONE 80MG/HR

ON 16/10/16
• TDM PHENOBARBITONE 197.3
• DECREASED IVI PHENOBARBITONE TO 50MG/HR
ON 17/10/16
• ABLE TO OFF IVI PHENOBARBITONE. STARTED ON MAINTENANCE IV PHENOBARBITONE 60MG
TDS

ON 23/10/16
• FIT FREE SINCE 17/10/16 AND THEN NOTED HAD FASCICULATION OF TONGUE AND LEFT SIDE
JERKING OF FACE, LEFT LOWER LIMBS AND UPPER LIMBS
• STARTED BACK IVI PHENOBARBITONE 0.5MG/KG/HR (22MG/HR)
ON 24/10/16,
• LOADED WITH IV PHENOBARBITONE 225MG
• INCREASED IVI PHENOBARBITONE 88MG/HR (2MG/KG/HR)

ON 26/10/16
• STILL HAVING SPIKE OF FEVER. NO SEIZURE.
• CT TAP: NO MALIGNANCY
• CONTINUE IVI PHENOBARBITONE 88MG/HR (2MG/KG/HR)
ON 27/10/16,
• EEG: SLOW THETA WAVE, BURST SUPPRESSION < 1 SEC. NO CLINICAL SEZURE
• CONTINUE IVI PHENOBARBITONE 88MG/HR (2MG/KG/HR)

ON 28/10/16
• EEG: BURST SUPPRESSION. NO CLINICAL SEIZURE
• TDM PHENOBARBITONE: 240
• DECREASED IVI PHENOBARBITONE 66MG/HR (1.5MG/KG/HR)
ON 30/10/16
• TDM PHENOBARBITONE: 270
• REDUCED IVI PHENOBARBITONE 44MG/HR (1MG/KG/HR)

ON 31/10/16
• TDM PHENOBARBITONE: 250
• REDUCED IVI PHENOBARBITONE 22MG/HR (0.5MG/KG/HR)
ON 2/11/16
• FITTED 2 EPISODE
• ON IVI PHENOBARBITONE 44MG/HR (1MG/KG/HR)
• TDM PHENOBARBITONE: 194.6
• GIVEN IVI PHENOBARBITONE 44MG/HR (1MG/KG/HR)
 HDW

ON 11/11/16 – TRANSFERRED TO HDW

• EEG: GENERALIZED BACKGROUND SLOWING WITH GPLEDS. NO CLINICAL SEZURE
• TDM PHENOBARBITONE: 125.9
• INCREASED IVI PHENOBARBITONE 66MG/HR (1.5MG/KG/HR)

ON 14/11/16
• TDM PHENOBARBITONE: 187
• CONTINUE IVI PHENOBARBITONE 66MG/HR (1.5MG/KG/HR)
• INCREASED PERAPAMEL 6MG ON AND T. TOPIRAMTE 100MG OM, 200MG ON
ON 21/11/16
• OFF IVI PHENOBARBITONE
• CHANGED TO T. PHENOBARBITONE 480MG TDS

ON 24/11/16
• TDM PHENOBARBITONE: 236.8
• DECREASED T. PHENOBARBITONE 360MG TDS
ON 28/11/16
• NOTED MACULOPALPULAR RASH OVER TRUNL/ABDOMEN/ ANTERIOR CHEST WALL
• STOPPED PHENYTOIN. (SUSPECT CAUSE OF RASH)
• REDUCED T. PHENOBARBITONE 120MG TDS

ON 2/12/16
• REDUCED T. PHENOBARBITONE 60MG TDS
ON 11/12/16
• ANOTHER EPISODE OF SEIZURE.
• IV PHENOBARBITONE 200MG BOLUS
• INCREASED T. PHENOBARBITONE 120MG TDS

ON 12/12/16
• ANOTHER BOLUS OF IV PHENOBARBITONE 200MG
• CONTINUE T. PHENOBARBITONE 120MG TDS
ON 13/12/16
• INCREASED T. PHENOBARBITONE 180MG TDS
• INCREASED T. TOPIRAMATE 400MG OM, 200MG ON

ON 15/12/16
• HAD SEIZURE. LIP SMACKING NOTED (?AUTOMATISM)
• STARTED T. LQCOSAMIDE LOADING 400MG STAT AND 100MG BD
ON 18/12/16
• HAVING ORAL DYSKINESIA
• INCREASED T. LACOSAMIDE 150MG OM, 100MG ON

ON 29/12/16
• INCREASED T. LACOSAMIDE 150MG BD
 NEUROMEDICAL WARD

ON 6/1/17
• TRANSFERRED TO NEUROMEDICAL WARD

• EEG (12/1/17): BACKGROUND SLOWING OF THETA RANGE, LEFT TEMPORAL DISCHARGE BUT
LESSER COMPARED TO PREVIOUS
• NO MORE SEIZURE.
• TRANSFERRED TO KOTA BELUD HOSPITAL FOR CONTINUATION OF CARE
DISCHARGE MEDICATIONS:
• T. TOPIRAMATE 200MG BD
• T. LACOSAMIDE 150MG BD
• T. EPILIM 800MG TDS
• T. PHENOBARBITONE 180MG TDS
• T. KEPPRA 150MG BD
No. Date Seizure Medication Remarks
1 4/10/16 Seizure  IV epilim 900mg loading
 T. Epilim 200mg TDS
2 5/10/16 Seizure  IV Keppra 1g loading
 T. Keppra 1g BD
3 6/10/16 Seizure  IV Epilim 900mg loading
 ↑ T. Epilim 400mg TDS
Seizure  IV Phenytoin 750mg loading
 IV Phenytoin 100mg TDS
4 7/10/16  IVIG 18g OD Cover for autoimmune
 IV Methylprednisolone 1g OD encephalitis
5 8/10/16 Seizure IV Propofol 10ml/hr
6 9/10/16 Seizure T. Topiramate 20mg BD
7 10/10/16 Seizure  Off iV Propofol Discontinued in view of
PRIS and bradycardia

 IV Midazolam 30mg/hr (double strength)

8 11/10/16 Seizure  IV Phenobarbitone 90mg/hr loading

 IVI Phenobarbitone 0.5mg/kg/hr (22mg/hr)

 IV Phenobarbitone 60mg/hr loading

Seziure
 ↑ IVI Phenobarbitone 1mg/kg/hr (33mg/hr)

 ↑ IV Midazolam 40mg /hr (20ml/hr)

9 12/10/16 Seizure  Bolus IV Phenobarbitone 50mg x3

 ↑ IVI phenobarnitone 3.5mg/kg/hr

(150mg/hr)
10 14/10/16 ↓ IVI Phenobarbitone 120mg/hr
11 15/10/16 ↓ IVI Phenobarbitone 80mg/hr
12 16/10/16 ↓ IVI Phenobarbitone 50mg/hr
13 17/10/16  Off IVI Phenobarbitone
 IV Phenobarbitone 60mg TDS
14 23/10/16 Seizure  IV Phenobarbitone 225 mg

 IVI Phenobarbitone 0.5mg/kg/hr (22mg/hr)

15 24/101/6 Seizure  IV Phenobarbitone 225mg

 ↑ IVI Phenobarbitone 2mg/kg/hr (88mg/hr)

16 28/10/16 ↓ IVI Phenobarbitone 66mg/hr (1.5mg/kg/hr)

17 30/10/16 ↓ IVI Phenobarbitone 44mg/hr (1mg/kg/hr)

18 31/10/16 ↓ IVI Phenobarbitone 22mg/hr (0.5mg/kg/hr)

19 2/11/16 Seizure  ↑ IVI Phenobarbitone 44mg/hr (1mg/kg/hr)

 IV MgSo4 4g bolus the 2g/hr

20 11/11/16 Seizure ↑ IVI Phenobarbitone 66mg/hr (1.5mg/kg/hr

21 14/11/16 Seizure  Continue IVI Phenobarbitone 66mg/hr (1.5mg/kg/hr)

 ↑ T. Perapamel 6mg ON
 ↑ T. Topiramte 100mg OM, 200mg ON

22 21/11/16  Off IVI Phenobarbtone

 T. Phenobarbitone 480mg TDS Tapering 120mg dose per week

23 24/11/16 ↓ T. Phenobarbitone 360mg TDS

24 28/11/16 ↓ T. Phenobarbitone 240mg TDS
25 1/12/16 ↓ T. Phenobarbitone 120mg TDS
T. Phenytoin off Off due to rashes
26 2/12/16 ↓ T. Phenobarbitone 60mg TDS Noted patient shallow breathing and
CO2 retention with compensatory
metabolic alkalosis

27 11/12/16 Seizure  IV Phenobarbitone 200mg loading

 ↑ T. Phenobarbitone 120mg TDS
28 12/12/16 Seizure  IV Phenobarbitone 200mg loading
 Continue T. Phenobarbitone 120mg TDS

29 13/12/16 Seizure  ↑ T. Phenobarbitone 180mg TDS

30 15/12/16 Seizure  T. Lacosamide 400mg loading

 T. Lacosamide 100mg BD
31 18/12/16 Oral dyskinesia ↑ T. Lacosamide 150mg OM, 100mg ON

32 23/12/16 ↑ T. Epilim 800mg TDS TDM Epilim low

33 29/12/16 ↑ T. Lacosamide 150mg BD
34 6/1/17 ↓T. Perapamel 4mg OD
35 13/1/17 ↓T. Perapamel 2mg OD
36 15/1/17 Off T. Perapamel No more stock
37 24/1/17 ↓ T. Topiramate 200mg BD

38 6/3/17  ↓ T. Phenobarbitone 120mg TDS

TCA neurology  Off T. Lacosamide
No more stock
39 6/6/17 ↓ T. Phenobarbitone 120mg TDS
TCA neurology
 INVESTIGATIONS
Date Investigation Result
30/9/16 Dengue IGM Negative
30/9/16 NS1 Negative
5/10/16 Urine Zika Virus PCR Not detected
5/10/16 Serum Zika Virus PCR Not detected
6/10/16 ANA Negative
6/10/16 C3, C4 Normal
14/10/16 Paraneoplastic panel Negative
14/10/16 Tumour markers (AFP, CEA, Negative
Ca125)
14/10/16 Autoimmune markers Negative
 IMAGING
Date Imaging Result
4/10/16 CT Brain No evidence of intracranial bleed
7/10/16 MRI brain High T2 signal is seen at the left medial temporal lobe, which is not suppressed
on FLAIR and is hypointense on T1.
Midly hyperintense on T@, not suppressed on FLAIR and isointense on T1.
Impression: Limbic encephalitis
No abnormal beaded appearance or aneurysmal dilatation

MRA brain
13/10/16 USG abdomen Ascites
IVC thrombosis
Urinary bladder debris
26/10/16 CT TAP Lung changes in keeping with active infection
Hepatomegaly. No focal lesiom
17/11/16 USG abdomen No evidence of collection
28/11/16 MRI brain Reduced signal intensity in DW1 sequence indicates response to treatment
Cerebral atrophy

10/12/16 ECHO No vegetation

14/12/16 USG abdomen No significant findings
16/12/16 CT TAP No evidence of collection or mass
IN NEURO CLINIC ON 6/3/17
• HAVING CONTRACTURE. NO SEIZURE
• DECREASED T. PHENOBARBITONE 120MG TDS. OFF LACOSAMIDE

IN NEURO CLINIC ON 6/6/17

• NO SEIZURE
• DECREASED T. PHENOBARBITONE SLOWLY EVERY MONTH UNTIL 30MG OD
THANK YOU