Dear collegies,

please help us to make the diagnosis to one patient.
She is girl, 9 years old. She has entered to our clinic - Kazak
Scientific Centre of Pediatrics and children's surgery of Ministry of
Public Health of Kazakhstan on 15th day if the disease.
Her complaints were: high temperature during long time, weakness. She
falled ill acutely, during first 10 days the temperature was 37-37,5 C
, her condition of health became worse, the temperature became higher
(up to 39-40 C), she had headache, vomiting was periodically.
Her {*filter*} analysis at that time: RBC - 3,7 , HGB-132 g/l, PLT - 310,
reticulocities -7%, WBC - 6,5 , NE (nature neutrophilies) - 60%, LY
(lymphocites) - 35%,  MO(monocytes) - 5%, the velocity of RBC setting
- 14mm/hour.
On 28 th day of disease her condition of health became progressively
worse: headache became stronger, diplopia and dimness of sight
appeared, vomiting became more frequent, severe ataxia, disorders of
speech, disturbed memory appeared, left fold from nose to month became
smooth.
On the 29th day of disease meningeal sings appeared.
{*filter*} analysis: RBC -7,0 , HGB -120, WBC - 9,7 , NE -85 %, Ly - 15%,
the velocity of RBC setting - 31 mm/hour.
Urine analysis : protein 0,066 , Erythrocytes -entirely (many).
Spinal liquour: chloridies -116 mm/l, cytosis -320, neutrophilies
-88%, limphocytes -12%, protein -0,49 g/l.
Magnetic tomography of brain: white and grey substances of brain are
not changed. The signs of tumor are absent. The truncus cerebri and
cerebellum haven't any local changes of structure. Extracerebral
liquor cavities are not widen. Ventricles of brain are enlarged.
On the 60th day of disease the disorders of speech, right hemiparesis,
amnesia appeared. After 6 days the hemiparesis disappeared.
The analysis of {*filter*}: RBC - 3,1 , HGB - 103, NE - 64%, LY -32%, the
velocity of RBC setting - 41 mm/hour.
Spinal liquour: glucose -2,77 , cytosis -98, lymphocities -97%,
protein -2,04.
On the 63 rd day of disease spinal liquor: cytosis -56, lymphocities
-98%, protein - 13,6.
The function of pelvis organs is keeped.
On the 70th day of disease spinal liquor: cytosis - 3, protein -2,04.
On the 72th day of disease the condition of girl's health became again
worse: right hemiparesis, amnesia, disorders of speech, pain before
gulp appeared. Anemia (1-st degree),  neitrophilia, gradual growth of
the velocity of RBC setting to 60 mm/hour. in the {*filter*} was at this
time. Considerable hematuria was in the urine analysis.
Other laboratory analysises:
- Bacterial sowing  of {*filter*} was negative.
- The serological investigation to cytomegalovirus was negative.
- The analysis of {*filter*} to typhus-paratyphus infections - was
negative.
Magnetic tomography in the dinamics: in the white substance of
hemispheres were single little parts of increased signals in the T2
image.
Infectious diseases, which are accomparied with high temperature were
excluded: listeriosis, leptospirosis, leishamaniosis, brucellosis,
tuberculosis, pseudotuberculosis, yersiniosis.
AIDS and veneral diseases were excluded.
Tick-borne encephalitis was also excluded. The analysis of {*filter*} to
toxoplasmosis was negative. Bilirubin, ALT, AST, thymoltest were
normalduring all course of disease.
Electrocardiography: sinus tachycardia (115-120 beats per one minute),
disorders of metabolism in myocardium.
Reoencephalography: spasm of brain's vessels.
Ultrasound investigation on organs of abdominal cavity:
- Liver -is enlarged, perivascular reaction
- Gallbladder - sings of normal sizes (lenth=58mm, width=21mm).
- Duodenum and Spleen are without changes.
- Kidneys - signs of pyelonephritis.
Tomography of organs of abdominal cavity - without pathology.
Electroencephalography - without changes.
Coagulogram: the lowering of coagulation in first, second and fourth
phases, the inhibition of fibrinolysis by internal way.
The course of disease is wavy: the periods of improvement of health
condition replaced by periods of changes to the worse. And all this
occurs at the background of keeped fever during 2,5 months.
The treatment was performed (antibacterial, antivirus,
antituberculosis, antiintoxication, dehydration therapy, hormones,
antioxydant, symptomatic, immunopotention therapy).

We think, that this girl has meningoencephalitis. But the ethyology of
this case of disease is mysterious for us.
Your opinion ?

Thank,
d. Imanculova G.

Dr. Imanculova,

Please refer to "Case Records of the Massachusetts General Hospital"
_NEJM_ 1988; vol.319 (no.17): pages 1139 - 1148

While certainly not an identical case, there are some intriguing parallels.

I would certainly test for celiac disease, on the basis of the similarities
in the waxing and waning of aphasia, similar body temperatures, memory
disturbance, and neuropathies. The renal tubular necrosis in the
Massachusetts case may have some parallel with your patient's urinary
hematuria and signs of pyelonephritis.

Vascular abnormalities in the cerebral cortex are also reported in the
literature as commonly associated with celiac disease.

An enlarged liver is also common in celiac disease.

The waxing and waning of severity of illness is also quite consistent with
celiac disease.

I hope this is helpful.
Best Wishes,
Ron Hoggan