A case of creutzfeldt-Jakob disease

1st hospt. West China Univ. of Med. Sci. hxyy at public.cd.sc.cn
Fri Oct 11 20:20:48 EST 1996

Hi, Dear colleague,
This is a testing esnding E-mail, We look forward to your replay
about the case as follow:


This is a 44-year-old female who entered the department of
neurology of West China University of Medical Sciences on Jun 20,
1996 with chief complaint of increasing unstable steps and slurred
speech for one month. She had been perfectly will until early April
1996 when she noted mild dry cough. One month later chest X-ray
showed interstitial pneumonia which disappeared after two weeks
treatment. But the frequency of the cough increased.
One month before admission, she developed clumsiness. She walked as
though she were drunk and had a tendency to fall. Her speech became
slurred. Although she heard what people said she complained of
decreased sense of hearing. All above symptoms became increasingly
sever. 20 days before admission brain MRI revealed normal.
Physical examination on admission: The patient was alert, oriented
and cooperative. There was horizontal nystagmus. Visual acuity and
sense of hearing were normal. She had scanning speech. Her gait was
ataxic, nose-finger-nose test, heel-knee-shin test were abnormal.
There was titubation of her trunk when she or walked. muscle
strength was normal, muscle tone was decreased. Deep tendon
reflexes were normal. There was no pathologic reflexes, Sensation
was intact to pinprick, light touch, proprioception and vibration.
Laboratory examination: CSF was normal including total protein
content, gamma globulin content, gamma globulin index. Oligoclonal
bands, VEP was abnormal bilaterally. BAEP was normal. Routine blood
counts and chemistries were normal, blood immunochemistry also
normal. EEG showed periodic complexes of sharp or slow wave
activity with irregular intervals.
The tentative diagnosis was multiple sclerosis. Dexamethasonum 20
mg intravenously daily for ten days then used prednisone started
with 60 mg daily tapered in 10 days then stopped. Regardless of the
treatment, she was getting worse. She lost the ability to speak and
was unable to move her limbs. Myoclonus developed, started in limbs
then generalized involving her head and trunk which responded to
verbal stimulus. There was a decerebrate posture with open eyes.
She also has had epileptic attacks which response to phenobarbital.
Up to now our tentative diagnosis is Creutzfeldt-Jakob disease.
If you could help us with the diagnosis and treatment, we would
greatly appreciate it.

Zhang yuping
Professor of Neurology

Dept. of neurology
The First Teaching Hospital
West China University of Medical Sciences
Chengdu, sichuan 600041
People's Republic of China

E-mail address is: hxyy at public.cd.sc.cn  

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