Interesting case of abnormal lip movements
11 year old girl who was healthy till one week back had low grade fever followed by focal seizures on right side on same day. Seizure lasted five minutes.
She regained consciousness but seizure recurred after few hours. Fever subsided with one dose of paracetamol and lasted one day only.
- No past history of significant illness,
- No contact with Tuberculosis ,
- no history of ear discharge,
- No history of head trauma.
Development all normal,
Immunized update.
No family history of any neurological disorders.
LP done , CSF clear ,normal pressure.
With this Possibility of Encephalitis was high in the list and considering treatable encephalitis patient was put on Acyclovir and supportive measures..
With the blood and CSF result also was not favoring. we did nt put the patient on antibiotics.
( May be this decision can be criticised . In an ICU setting with altered sensorium focal seizures and CSF showing cells it is dictum to put patient on treatable drugs ,saving life is more important and time too short. So usually we take a policy to start management of pyogenic meningitis and treatable antiviral , acyclovir .
At the same time send for virological studies, cultures.
Usual policy is to start imperically and stop later once the possibility is disproved. That ll be a better decision in an ICU . Here many points arguing against pyogenic or tuberculous and we took a decision not to start empirical antibiotics or ATT , but acyclovir only.
With evidence of diffuse brain involvement ,no fever possibility of biochemical /metabolic , toxic /system function derangement also to be ruled out.
Here biochemical report did not support any of them . ie blood sugar ,electrolytes ,RFT ,LFT .
Considered possibility of poisoning also but no circumstantial or clinical supporting evidences.
With the CSF finding of Lymphocytes possibility again increased for possibility of infection . But this may be misleading , the cell number is low , only lymphocytes. This can happen following any seizure.
Argument for Herpes encephalitis usually are cells , lymphocytes but more important is RBC in atraumatic LP . Here RBC were not there. Protein minimal elevation and sugar normal.
We sent the CSF for Virological studies,
Next day patient developed this movements
(consent taken from parents in writing )
These were abnormal movements ,(not seizures ) repetitive involving left hands picking hair and dress. Abnormal movement around mouth was prominent.
Encephalitis with extra pyramidal movements were suggestive of Jap B encephaltis . But this movement is more suggestive of another entity - NMDA receptar encephalitis . In fact many cases earlier were diagnosed as Jap B encephalitis before this entity was not known.
With this possibility in mind we send the CSF for NMDA receptar antibody and
She was Put on Methylprednisolone and IVIG ( better to give both in this condition )
MRI was taken next day.
This was the report from radiologist , but i think there is some basal ganglion hyper intensity in flair images
NMDA receptar antibody result came as high titre positive.
Herpes , Jap B Dengue , westnile viruses negative. in CSF (virological study results from ICMR lab Manipal )
Sensorium improved , but she had seizure on the third day . She was put on Levetirazetam.
Since the last two days the abnormal movement worsened and sensorium deteriorating. Considering the possibility of levetirazetam worsening the situation it was stopped and she was put on dilantin sodium . Short period she was on midazolam drip.
Movements improved , but the sensorium same.
She was put on Rituximab. 375 mg per meter square . 500 mg per day IV.
For the abnormal movement she is put on Tetrabenazine 25 mg half tab BD.
Supportive measures continued. Nutrition fluid ,electrolyte balance maintained mainly be IG tube feeding .
*****************************************************
(This report was made two and half years back, She came to OP today.)
She recovered slowly with sequel e . We investigated for the possibility of underlying tumors , which came negative
She was put on long term oral steroids and Azathioprim.
She is under follow up clinically and USG.
She is now admitted for repeat titres.
She lost her speech. She understands try to read and copy letters.
we reported case series in Indian pediatrics last year
Immunized update.
No family history of any neurological disorders.
On admission
- Altered sensorium. EMV 10
- breathing normal,
- BP 110/70 ,pulse 80/min,afebrile.
- No skin bleeds,skin rash,No jaundice.
- Eye movements normal ,pupils normal size and reaction fundus normal.
- other Cranial nerves normal.
- Motor system ,power normal ,tone lower all limbs reflexes sluggish ,plantar up going.
- No signs of meningeal irritation.
- Provisional diagnosis of ADEM /Herpes encephalitis was made.
First level investigations done.
LP done , CSF clear ,normal pressure.
With this Possibility of Encephalitis was high in the list and considering treatable encephalitis patient was put on Acyclovir and supportive measures..
Teaching points.
Patient had mild fever at onset only , possibility of bacterial /tuberculous meningitis not considered high in the list.With the blood and CSF result also was not favoring. we did nt put the patient on antibiotics.
( May be this decision can be criticised . In an ICU setting with altered sensorium focal seizures and CSF showing cells it is dictum to put patient on treatable drugs ,saving life is more important and time too short. So usually we take a policy to start management of pyogenic meningitis and treatable antiviral , acyclovir .
At the same time send for virological studies, cultures.
Usual policy is to start imperically and stop later once the possibility is disproved. That ll be a better decision in an ICU . Here many points arguing against pyogenic or tuberculous and we took a decision not to start empirical antibiotics or ATT , but acyclovir only.
With evidence of diffuse brain involvement ,no fever possibility of biochemical /metabolic , toxic /system function derangement also to be ruled out.
Here biochemical report did not support any of them . ie blood sugar ,electrolytes ,RFT ,LFT .
Considered possibility of poisoning also but no circumstantial or clinical supporting evidences.
With the CSF finding of Lymphocytes possibility again increased for possibility of infection . But this may be misleading , the cell number is low , only lymphocytes. This can happen following any seizure.
Argument for Herpes encephalitis usually are cells , lymphocytes but more important is RBC in atraumatic LP . Here RBC were not there. Protein minimal elevation and sugar normal.
We sent the CSF for Virological studies,
Next day patient developed this movements
(consent taken from parents in writing )
These were abnormal movements ,(not seizures ) repetitive involving left hands picking hair and dress. Abnormal movement around mouth was prominent.
Encephalitis with extra pyramidal movements were suggestive of Jap B encephaltis . But this movement is more suggestive of another entity - NMDA receptar encephalitis . In fact many cases earlier were diagnosed as Jap B encephalitis before this entity was not known.
With this possibility in mind we send the CSF for NMDA receptar antibody and
She was Put on Methylprednisolone and IVIG ( better to give both in this condition )
MRI was taken next day.
This was the report from radiologist , but i think there is some basal ganglion hyper intensity in flair images
NMDA receptar antibody result came as high titre positive.
Herpes , Jap B Dengue , westnile viruses negative. in CSF (virological study results from ICMR lab Manipal )
Sensorium improved , but she had seizure on the third day . She was put on Levetirazetam.
Since the last two days the abnormal movement worsened and sensorium deteriorating. Considering the possibility of levetirazetam worsening the situation it was stopped and she was put on dilantin sodium . Short period she was on midazolam drip.
Movements improved , but the sensorium same.
She was put on Rituximab. 375 mg per meter square . 500 mg per day IV.
For the abnormal movement she is put on Tetrabenazine 25 mg half tab BD.
Supportive measures continued. Nutrition fluid ,electrolyte balance maintained mainly be IG tube feeding .
*****************************************************
(This report was made two and half years back, She came to OP today.)
Her follow up.
She recovered slowly with sequel e . We investigated for the possibility of underlying tumors , which came negative
She was put on long term oral steroids and Azathioprim.
She is under follow up clinically and USG.
She is now admitted for repeat titres.
She lost her speech. She understands try to read and copy letters.
we reported case series in Indian pediatrics last year
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