I'm a neurologist,I need your help! I met a strange patient who was
suspected Creutzfeldt-Jakob syndrome.But 14-3-3 beta protein in CSF was
negative.What's the diagnosis on earth? any ideas? How to treat the
patient?If you have answer ,You can send E-mail to
lidan at irix.xamu.edu.cn.
The case abstract is as follow:
48 YO Chinese F presant ,who hospitalized with the CC:
progressive amnesia and stupidity for 3 Mons, unconsciousness and
limbs tic for 4 days.
3 Mons prior to admission ,she was becoming
forgetful,less-effective in house-working, spontaneous nonsense,eating
without feeling hungry or full and defecation ,urination everywhere.
There was no clear reason. 4 days prior to admission ,she was
unconscious ,extremities tic ,myoclonus seizure, excretion complete
incontinences. No fever ,no headache and no vomitting during all the
course of disease. She was initially treated as the diagnosis of
mental disturbance, but it didn't work.
She was in her USOGH(usual state of good health). No vaccination
,trauma,toxication history and no special family/social history.
Vital Sign: stable
CV, Pulm, Abd and Ext examination is N.A.D (no abnormal detected)
Neurology System :
Superficial coma state, primitive defence reflexes could be
seen. Bilateral pupils were in same size,D m,showed a sensitive light
reflex. Doll eye sign :(+) .
Motor: Automatical movement and myoclonus seizure could be seen
after stimulation. Extremities muscular hypertonia ,tendon
hyporeflexia. Babinski's sign (-),other pathologic reflex: (-).
Meningeal irritation sign (-).
Sensory and co-ordination :can not be checked because of
inability to co-operate.
Lab: CBC: WBC 7.8¡A10^6/L,N:0.65,L:0.34
CSF: protein 400mg/L,
Total cell count: 25¡A10^6 /L ;WBC 0¡A10^6/L
CMV; HSV ¢ñ/¢ò;Toxoplasma;Rubella
IgM and IgG are all negative.
14-3-3 ¦A protein: (-)
EEG: progressively increased periodical slow-apex and slow-spinal
waves on the background of diffused slow wave.
Brain biopsy: brain tissue mild congestion,focal lymph cell
infiltration around small vessels, neuroglia mild hyperplasia ,neuron
cells has no obvious alteration.
Cranial CT and MRI :(-)
Electrolytes ,liver and kidney function: All are normal.
Up to now,the patient is being coma for near 2 Mons. She was not
getting worse or better ,even though many heteropathies had been taken.
Diagnosis? Therapy? Any ideas?