Interesting case of encephalitis

History in short 

One year and eight month old girl born to a blind couple were bought with fever and seizure.( Both of them
were blind at birth,otherwise intelligent and normal)
She developed fever and at the height of fever she developed generalized seizure for which they were
hospitalized. Seizure was controlled and she was playful for a while.At night again she developed seizures.
lasting for two and half hours which was not controlled with usual anti convulsants and she was referred here. In between the seizures she vomited twice.She was bought deeply comatose, seizing movements controlled but occasional twitching persisting on left side, mother said the movements were more on left side.
She was second child was born at term after an uneventful pregnancy.
She was normal up to this age gained all milestones normally.
Elder sibling born at term was normal. She developed fever,developed seizure and vomiting. Got hospitalized with intractable seizures and coma,she died in hospital within 24 hours.No more details about the condition or treatment available with them.Mothers words there were no rashes,no bleeds .No history of fall, drug intake,bites which can be attributed to the events.
Non consanguineous marriage.
No other members in family affected with similar or other neurological problems

She was immunized up to age.

Discussion at the end of history

Previously normal girl child developing fever followed by seizure from which she recovered initially first possibility considered was febrile seizue .
But it recurred with longer duration and severity, developed vomiting and deteriorating level of consciousness other possibilities were considered.

Possibility of trauma, toxins poisons were less likey.

Fever persisting possibility of infections or infection triggering some inborn error of metabolism were considered.
Other possibilities of malignancies or connective tissue disorders were less likely .

She was normal up to this age does nt rule out possibility of inborn error of metabolism as few of them may present later milder variants.
Argument for this entity was higher in view of the sibling also had almost similar problem almost same age.

Common things always common, we thought of ruling out simple biochemical abnormalities and infections of brain or meninges.

On examination,

breathing was normal,circulatory status normal Blood pressure normal for age.
Mild pallor ,no lymph nodes,no focus of infection, No obvious rash or bleed.No bite mark or injury obvious.
EMV 8.Movement of limbs on pain,paucity of movement left upper and lower limbs
No ear discharge,
No vesicles visible on child or parents.Parents were healthy,no neurocutaneous markers on their body.

Nervous system examination

Pupils normal , reacting. Fundus normal

Deviation of face to right side 

Tone on left upper and lower limb lower than right side (on day of admission) 

DTR sluggish on day1

Plantar upgoing bilaterally.

Video taken on second day

Skull and spine normal
No signs of meningeal irritation
Other systems normal . No hepatosplenomegaly

Next day

.

INVESTIGATIONS

Blood routine, ESR ,platelets were normal

Platelets normal

Serum electrolytes,Serum calcium, Blood sugar, Liver function tests,Renal function tests were all within normal limits.

Serum ammonia, Lactic acid done normal

ABG normal

Lumbar puncture done, CSF normal pressure ,clear. Microscopy showed 8 cell all lymphocytes. Protein 15mg.Sugar 60,

CSF send for virological studies , negative for Herpes and Jap B

NMDAR antibody negative

MRI done

Bilateral symmetrical thalamic and basal ganglion involvement. Right tempero occipital cortex involved.
Diffusion restriction in thalamic and basal ganglia
Infarction of above area bilateral symmetrical manner, how to explain?
The above areas are not part of a vascular territory
Bilteral symmetrical involvement with a family history of sibling same clinical features leading to death strongly suggest possibility of neurometabolic problem triggered by fever
But that can not explain the infarct.bilaterally
With the above possibility in mind patient was put on supportive measures and megavitamins (high dose of Biotin, Thiamin,Pyridoxin,Coenzyme Q and carnitin)
As the patient deteriorated we put the patient on Methyl prednisolone and IV gammaglobulin 1gm /kg/day in two days
This is the response


There is response.
So how to explain the entire problem?
This presentation strongly suggest possibility of metabolic disorder , but good response to modulators.
If immune mediated , how to explain the ischemia ?
If immune mediated how to explain the family history , that too strong family history
We searched for answer

Necrotising Encephalitis............

infection triggering immune mediated injury, there can be genetic susceptibility.
Infections like influenza well known to cause
She improved but
weakness of left upper and lower limb and left LMN facial weakness plus
Drooling of saliva ,difficulty in swallowing
No speech
Above all , she cant see.
She can understand , obey command.
I dont know how much improvement i can expect.
She is on oral steroid 1mg/kg of prednisolone now.

incidentally another case was referred from Coimbatore last week with almost same presentation except for the fact that kid had high liver enzymes and renal shut down
She had loosestools severe enough to lead to shock. Whether the multiorgan failure was secondary to the shock or due to the basic problem we dont know.
H1N1 of that kid was positive
Had a discussion with the intensivist of that institution. They had two more similar cases last month
So
Is there a possibility of more of this presentation as a complication of H1N1  in the coming days