Appendix e-1: Clinical features of one VGKC Ab encephalitis patient (Case 4)

clankflaxMechanics

Feb 22, 2014 (3 years and 5 months ago)

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Appendix e
-
1
:

Clinical features of one VGKC Ab encephalitis patient (Case 4)

A 14 year old girl of Vietnamese origin presented with headache and dizziness followed
by new onset secondary generalised tonic
-
clonic

seizures (GTCS) and confusion. On
examination she was febrile, encephalopathic and combative, but no focal neurologic
deficit. She had frequent short lived GTCS every 30
-
90 minutes without intervening
return of consciousness. She was intubated, ventilated

and commenced on a midazolam
infusion.

Brain MRI at presentation and three weeks into her admission was normal. The
initial EEG had left periodic epileptic discharges whilst subsequent EEGs showed
generalised slowing and alternating right and left tempora
l electrical seizures. CSF
revealed six white cells, normal protein and glucose, and negative PCR for herpes
simplex virus and enterovirus. CSF neopterin was elevated at 87.9 nmol/L (normal 7
-
28),
and oligoclonal bands were negative. Her erythrocyte sedime
ntation rate was 40 mm/hr
(normal <20), and C
-
reactive protein was 42 mg/L (normal 0
-
10). There was no
hyponatraemia at any stage of the illness.

Her acute VGKC antibody titre was 640 pM (<100). Serology for mycoplasma,
cytomegalovirus, Epstein Barr virus
, human herpes virus 6, toxoplasma, and cat scratch
disease were negative. Anti
-
nuclear antibody (ANA) titre was elevated at 1:640 with a
speckled pattern, although further autoimmune testing was normal. Pelvic ultrasound
revealed no ovarian teratoma.

Duri
ng her 18 days of intensive care she had hyperthermia up to 41.5
o
C. Despite
midazolam infusion, sodium valproate, phenytoin and levetiracetam, she continued to
have up to 20 focal and generalised seizures per day. In the third week of her admission
her sei
zures abated and she was extubated and discharged to the ward, although she was
emotionally labile and had word finding difficulties. Formal neuropsychology assessment
at three months revealed impairments of cognition and higher executive function,
includi
ng problems with emotional control, attention and complex thinking. In view of
the ongoing cognitive concerns three months after discharge, she was given one course of
intravenous immunoglobulin and oral steroids for 1 month. At 15 month follow
-
up, she is
back to her pre
-
morbid functioning according to her family and school, and has been
weaned off all anti
-
convulsants.