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SoS:suspect a case of Creutafeldt-Jakob syndrome in china

mail1 at irix.xamu.edu.cn mail1 at irix.xamu.edu.cn
Mon May 17 10:38:25 EST 1999


Dear Sir:
    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.
PE:
  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 :(+) .
       Neck: soft
       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,
            Sugar   150mg/L
            Cl   126mmol/L
            Total cell count: 25¡A10^6 /L ;WBC 0¡A10^6/L
            Viral series:
                 CMV;  HSV ¢ñ/¢ò;Toxoplasma;Rubella
IgM and IgG are all negative.
             14-3-3 ¦A protein: (-)
             TB-DNA:  (-)
  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?




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